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Physical health and well being 

Physical health and well being

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Physical health and well being
DOI:
10.1093/med/9780199533220.003.0006
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Introduction

Physical ill health and challenges to well-being are common in people with ID. Consequently it is important that they have early access to assessment, treatments, and interventions, thereby reducing complications, maintaining physical health and well-being, and leading to an improved quality of life.

As a population, children, adults and older people with severe ID all have higher health needs compared with the general population. They also have a different pattern of physical health needs, many of which go unrecognized and remain untreated. However, in the absence of a specific syndrome, the health profile of people with mild ID is the same as the general population once socioeconomic factors are taken into account, although they may experience more difficulty in accessing health promotion and primary and secondary care services. Such difficulties may contribute to increased health problems for some individuals.

As a result of a range of factors, which include unmet health needs, many die unnecessarily at a premature age. This section provides comprehensive coverage of the very many factors that can compromise the health and well-being of this group of people, along with a range of strategies that ID nurses can adopt to support them.

Further reading

Mencap (2007). Death by Indifference. Mencap: London.

Michael J (2008). Healthcare for All: Report of the independent inquiry into access to healthcare for people with learning disabilities. Department of Health: London. Physical health and well being www.iahpld.org.uk/

Health inequalities

Definition

Health inequalities may be described as the gap in access to healthcare, or in the health status, between different and discrete populations. These populations may include people from different ethnic communities and social groups, as well as people with ID or people living in defined geographical localities. Many people with ID are known to experience persistent and multiple factors that can result in health inequalities and social exclusion. These factors may include:

  • Living in poverty and greater physical and mental health needs

  • Difficulties in accessing primary and secondary healthcare

  • Lower nutritional status and poorer quality of healthcare provision

  • Limited access to good health information and health education

  • A higher likelihood of living in poor quality housing and reduced access to reliable transportation

  • Less employment opportunities

  • Increased experiences of hate crime and organizational/individual discrimination.

Tackling health inequalities

The need to tackle health inequalities is identified in numerous documents, which are directed across various agencies and departments, as being central to key government policy. These documents include all 4 UK learning disability policies: in Wales, Fulfilling the Promises,1 in Scotland, The Same as You,2 in Northern Ireland, Equal Lives,3 and in England, Valuing People.4 The focus of delivery of programmes developed to tackle health inequalities is directed towards local action. Leadership for these is expected through local authorities, education departments and health services, including PCTs. Within England, LSPs have a key role in tackling health inequalities.

What are Local Strategic Partnerships (LSPs)?

An LSP is a single body that brings together people from the public, private, voluntary, and business sectors, to provide an integrated approach to tackling health issues, at a local level. Each local government area in England has an LSP. Their role is to identify shared priorities and write joint targets as a means of tackling local health inequalities.

Tackling health inequalities in people with intellectual disabilities

Everyone working with people with ID, whether in health, social care, leisure or housing, has a role in reducing health inequalities. The following four principles underpin how health inequalities are tackled in practice. These are presented along with examples of actions that have been taken when applying them to reducing health inequalities for people with ID.

Working through general services making all services more accessible to the needs of disadvantaged groups

Ensuring that accessible health information on chronic health conditions and health treatments are available at GP surgeries and hospital outpatient clinics. Providing training on the health and communication needs of people with ID to all primary care and hospital care staff

Preventing inequalities from getting worse by addressing underlying causes of ill health

Introducing annual health checks and the opportunity for health action plans to all people with ID. Running regular health information groups for people with ID, to help them gain greater knowledge and personal control. Supporting people with chronic health conditions access the Expert Patient Programme.

Finding new ways of meeting needs, particularly in areas that are resistant to change and targeting interventions at these

Working with acute hospitals to develop acute liaison nurse posts, and support the development of resources to improve the care of people with ID in hospitals. Working with local police departments, to help them understand the needs of people with ID, in relation to the management of local hate crime.

Developing policies and supporting coordinated action on these from a central point

National guidance on the NHS Cancer Screening Programme on breast and cervical screening for women with ID, highlights the importance of the issue, informed consent and sets national standards for practice.

Further reading

Department of Health (2003). Tackling Inequalities: A Programme for Action. Department of Health: London.

Department of Health (2004). Choosing Health: Making Healthy choices Easier. Public Health White Paper. Department of Health: London.

Mencap (2007). Death by Indifference. Mencap: London.

Michael J (2008) Healthcare for All: Report of the independent inquiry into access to healthcare for people with learning disabilities. Department of Health: London.

Principles of health promotion

Health promotion comprises a broad span of activities that work towards achieving the positive health and well-being of individuals, groups, and communities.

The focus of health promotion work includes health education, lifestyle and preventative approaches, alongside environmental, legal, and fiscal measures.

Key health promotion principles

  • Equity

  • Participation

  • Collaboration

  • Empowerment

  • Sustainability.

What health promotion barriers do people with intellectual disability experience?

    • Limited skills of primary and secondary care staff in accessible communication and in the knowledge of the population’s health needs

    • A lack of policy promoting health to the ID population

    • Reduced access to health screening and health education

    • A lack of accessible health promotion resources

    • Low priority given to the health of the population

    • Reduced health ownership and control, with less opportunity for decision making and informed choice.

Everyone who works with people with ID has a role to play in tackling health inequalities through health promotion activities. Health promotion comprises a complex and wide ranging area of activities, which at times may require radical thinking. It facilitates opportunities to focus on developing choice, promoting independence and rights, and enabling inclusion. Health promotion activities may take place with individuals or collectively within small or large groups. It may be necessary, in order to meet the needs of people with ID to the same level as that of the general population, to adopt different kinds of solutions.

The following list identifies 5 key principles of health promotion that may be used in work relating to people with ID.

Equity

  • Promoting the need for and supporting GPs and their staff in developing and introducing annual health checks

  • Highlighting and working with public/private/voluntary sector employers in raising the need for developing an accessible application process to help people with ID to apply for and get jobs

  • Keeping up to date with new projects at a local level. Working with staff in education, housing, health and leisure projects to help make projects accessible to people with ID.

Participation

  • Informing people with ID on the role of links and other local consultation groups. Supporting them to participate in these groups

  • Employing people with ID as co-trainers in the delivery of training to health and other staff

  • Involving people with ID in the recruitment of staff

  • Ensuring meetings and their minutes are accessible.

Collaboration

  • Working in partnership with people in local advocacy groups in developing local strategies and policies

  • Working across service boundaries to ensure the needs of people with ID are incorporated into local services e.g. older people’s services, sexual health, drug and alcohol, mental health

  • Working with health visitors in the delivery of training to staff on the needs of parents with ID.

Empowerment

  • Promoting and using person-centered approaches to care

  • Organizing and delivering health education related activities, either individually or in groups

  • Consulting with user-led groups, e.g. black and minority ethnic groups, on their needs in local service development

  • Involving people with ID in the commissioning strategy

  • Sharing responsibility.

Sustainability

  • Developing and using accessible health education resources to help people understand and take greater control of their health

  • Undertaking inclusive research activities (see Physical health and well being Working with people with intellectual disabilities in the research process, p.[link])

  • Supporting self-advocacy groups to run health information related activities

  • Being innovative in the collection of evidence to promote the need for new service developments

  • Ensuring that the needs of people with ID are included in disability equality schemes.

Promoting public health

Definition

The Faculty of Public Health has described public health as, ‘The science and art of preventing disease, prolonging life and promoting health through the organized efforts of society’.

Overview

The role of public health is strongly driven towards the reduction of health inequalities. Public health may be referred to, and considered from, four different approaches. These are:

  • A multidisciplinary profession, whose wide range of skills are focussed on, predominantly, proactive work, directed towards improving the health of the population

  • A set of diverse skills, including specific skills in working with populations, as well as collecting, interpreting, and using evidence to direct, and develop programmes of service delivery, to meet identified need

  • A range of desired outcomes originating from both individuals and populations, relating to the aim of achieving lasting improvements across mainstream health service delivery

  • Factors that contribute to health and illness, focused on tackling the root causes of ill health. These include addressing personal lifestyle issues such as diet, exercise, and smoking, as well as wider determinants, which may include housing, or the quality of healthcare.

Public health delivery

The assessment and delivery of public health initiatives are increasingly being delivered in working partnerships. At a local level an increasing number of directors of public health are joint posts, which cover both the NHS and local authorities that share geographical boundaries. This joint approach is reflected in some shared delivery of health promotion and public health practice. Coordinated approaches to public health are set out in LAAs.

What are Local Area Agreements (LAAs)?

An LAA is a 3yr agreement. It sets out the priorities and objectives for a local area, represented by a local strategic partnership of representatives of all key organizations, and central government. LAAs are designed to:

    • improve central and local government relationships

    • improve efficiency in public services

    • strengthen partnership and enhance leadership of local authorities on a range of public service outcomes.

Public health in practice

People working in public health consider their role, and its application in practice, in 3 domains:

  • Health protection and prevention

  • Improving health and social care

  • Health improvement.

People with ID experience extreme and multiple health inequalities; however, their needs are often neglected in the work of public health departments. This has been recognized and there is currently a strong national push to rectify this.1 Initiatives include public health departments employing a staff member to take a lead in ID, or ID staff working with multiagency services to include the needs of people with ID in local audits, strategies and projects. However, public health departments cannot be considered the only agency responsible for meeting the health inequalities of people with ID. The promotion of public health needs to be considered as central to everyone working with this population. The above domains can be used as a framework to list examples of work that may be required.

How do the three public health domains translate into examples of work related to intellectual disability?

Health protection and prevention

    • Facilitating accessible sex education to children and adults

    • Educating and supporting women with ID to access breast andcervical screening

    • Providing education on home/road safety.

    • Promoting and supporting annual health checks

    • Working with police in addressing management of hate crime.

Improving health and social care

    • Developing and auditing quality standards in primary/acute hospitals

    • Conducting local needs assessments to inform local service delivery

    • Facilitating groups to enable people with ID to understand local service provision and contribute to local consultation

    • Undertaking local/national research and reporting on findings.

Health improvement

    • Ensuring availability of accessible health promotion resources

    • Developing and facilitating health, housing, employment projects

    • Influencing/working with public health specialists to ensure ID is included in local strategies e.g. older people, exercise, diet, and obesity.

Further reading

Care Services Improvement Partnership (CSIP) (2007). The Role of Public Health in Supporting the Development of Integrated Services. National Institute for Mental Health in England: Leeds.

Promoting physical well-being of individuals

Good health is the foundation on which a good quality of life can be built. Good physical well-being, that includes a sense of personal ownership and a personal locus of control, is strongly linked with positive mental well-being. People with ID experience many barriers in the achievement of optimum physical health. Multiple sources of evidence from the UK, and wider, have found that people with ID experience worse physical health, due to many different causative factors. In addition they receive worse treatment from health services, with both primary and acute hospital system failures. It is also widely acknowledged that insufficient priority is given within health services in addressing the specific health needs of this population.

What do we know about the health of people with intellectual disabilities?

    • They have an increased risk of an early death, and have a different picture of health needs, than the general population

    • Some syndromes associated with ID (e.g. Down syndrome, Prader–Willi syndrome) are accompanied by an increased range of specific health conditions

    • They have high levels of unmet physical and mental health needs and higher rates of some chronic or long-term health conditions, e.g. epilepsy

    • They have higher rates of vision and hearing difficulties and are less likely to have received regular physical health, dental, vision, and hearing checks

    • Women with ID are much less likely to access breast mammography or cervical screening

    • They are more likely to experience mental health problems

    • They are less likely to have access to health education or to live healthy lives, and they have reduced ownership and control of their personal health needs.

The aim of developing local approaches in meeting the health needs of people with ID in England was identified in Valuing People that stated the aim to be:1

To enable people with a learning disability to access a health service designed around their individual needs, with fast and convenient care, delivered to a consistently high standard, with additional support, where necessary

Practical steps to consider when promoting physical well-being

Individual

  • Be pro-active in considering and promoting health needs within all working roles and care environments; facilitate a healthy living environment, ensuring availability to accessible information; facilitate each individual making informed decisions on health treatments, health screening, and lifestyle choices.

  • Enable each individual to select someone they know and trust to be their health facilitator (HF), to support them in thinking about their health, building knowledge, health ownership, and responsibility.

  • If not initiated by the local GP practice, the HF should support each individual to arrange and attend a health screening check. This should help each person to identify individual health needs, and consider how these will impact on their wider lifestyle aspirations and future plans

  • Working with the individual, record the needs identified by the health check. Consider how these needs will be met, by whom, and in what timescale. Support the person to put these into a plan, where possible, in a style and format accessible to the individual. This may be known as a health action plan (HAP).

  • Plan and prepare for any health or hospital visits, where possible, in advance. Write any special requirements down in a patient passport, to share and leave with medical staff.

Organizational

  • Raise the health profile of people with ID across commissioning, health, social, and voluntary agencies at local levels. Also offer support and initiate partnership working with local primary care teams, acute hospitals, and dental and other heath agencies.

  • Ensure health strategy planning group has multi-agency membership. Also offer and deliver staff training to a wide range of health staff and health students. Employ people with ID to share this role.

  • Develop a resource base that contains a wide range of accessible resources on physical health conditions, health screening, and medical procedures. Make these resources or the links to them easily available to all. Also build a local resource of examples of HAPs, to support training, and work in partnership with self-advocacy groups in building health knowledge in people with ID.

  • Link with national networks to share and learn from national best practice.

  • Develop surveys, research, audit or other approaches that record evidence of local health needs.

Further reading

Disability Rights Commission (2007). Equal Treatment: Closing the Gap–One Year On. Equality and Human Rights Commission

Michael J (2008). Healthcare for All: Report of the independent inquiry into access to healthcare for people with learning disabilities. Department of Health: London.

Physical health assessment of people with intellectual disability

People with ID experience a wide range of inequalities in relation to their physical health. These include an increased prevalence of a number of specific health conditions along with poor health outcomes. Other evidence suggests that they are less likely than the general population to access regular health checks or health screening. This inequality leads to increased likelihood of suffering from a range of specific conditions, and poor health outcomes. There is a sound rationale for introducing systematic physical health assessments for this population. An additional legal rationale, the Disability Discrimination Act, Disability Equality Duty, requires all public bodies to address meeting these inequalities within local disability equality schemes; failure to do this could make them liable to prosecution.

What is physical health assessment?

Physical health assessment is the process of gaining a fuller understanding of the health needs of an individual, in order to detect, treat, and manage any health conditions.

National recommendations are that physical health assessment may be best managed in people with ID, by the provision of individual health checks. It is recommended that these are offered annually. Health checks should be offered by GP practices to all adults with ID registered with the practice. Annual health checks should include the key areas of health risks known in the ID population.

To ensure an integrated approach between each person’s health check and well-being, the outcomes of the health check, with any actions, by whom and the timescale to be achieved, should be recorded within the HAP.

Preparing people with intellectual disability for ahealth check

People with ID may need support in preparing to have a health check so that they can give informed consent for it. Prior to the appointment, work should be undertaken with the person to help them understand what investigations may be required during the health check. It may be helpful to obtain accessible information leaflets.

Accessible information may be available from:

  • The GP surgery

  • The local NHS health information resource centre

  • The local community learning disability service

  • Health information websites e.g. Physical health and well being www.easyhealth.org.uk

What areas of physical health warrant assessment in a health check?

    • Family history and risk factors

    • Immunization record

    • Drug and alcohol use

    • Vision and hearing

    • Review of chronic illness including epilepsy

    • Physical examination including skin condition, mobility and posture

    • Syndrome specific check

    • Medication review

    • Oral health

    • Sexual health

    • Continence

    • Review of mental health and emotional needs

    • Behavioural disturbance, sleep issues

    • Carer details and constraints

    • Referrals made and any follow up

    • Identified areas of risk and actions to be taken.

Supporting physical health assessment in practice

The implementation of annual health checks for people with ID across the UK is extremely patchy. The Welsh Assembly introduced regular health checks into their GP contracts from 2006/2007. In other parts of the country, different areas have introduced them using Local Enhanced Services payments to GPs.

Resources

A number of resources have been developed to promote and support the introduction of health checks. These include:

  • Primary care service commissioning framework July 2007

  • The management of healthcare for people with ID in primary care

Physical health and well being www.primarycarecontracting.nhs.uk/204.php

This is an extensive package of practical resources to support the development and implementation of health checks. It includes:

  • Health checks—a summary of steps and support

  • Templates for GP IT systems

  • Job description of a HF

  • GP practice step-by-step guide at a glance

  • Health checks—role of the CLDT

  • Health checks—role of the PCT

Blood pressure, temperature, pulse

Blood pressure

Blood pressure is a measure of the levels of force exerted on the walls of blood vessels, due to the flow of blood from the heart. Blood pressure is dynamic, and varies from minute to minute. It is measured by an instrument called a sphygmomanometer, a gauge that measures the pumping (systolic) and resting (diastolic) stages of the heart. Readings of blood pressure are usually performed by trained health professionals.

Blood pressure is influenced by a large number of factors. These include temperature, respiration, environmental noise, pain, fear, anxiety, exercise, smoking, eating, and drinking. Body position can also affect blood pressure readings.

High blood pressure is known as hypertension, and low blood pressure is hypotension. Consistent readings of 140/90 mmHg and above are likely to be associated with hypertension. Hypertension is an increasing medical concern, being commonly associated with obesity, diabetes, and high levels of fat in the blood. If left untreated, a combination of the above can lead to cardiovascular (heart) disease, stroke, kidney failure, dementia, and early death. Treatment is usually based on lifestyle changes and drug interventions.

People with ID are known to have higher rates of obesity than the general population, and receive fewer health checks. In meeting the health needs of people with ID, carers have a key role in the prevention, detection, and management of hypertension. It can be difficult to detect without measuring blood pressure, therefore blood pressure measurement is required in any regular health check. In preparing a person for the procedure, people need to be aware of the discomfort of a tightening cuff on the upper arm—distraction interventions may help. There are a number of less uncomfortable wrist readers, but these are less accurate.

Preventative lifestyle measures include healthy eating, eating a well balanced, low-salt diet, maintaining a healthy weight, regular exercise, low alcohol intake, reduced caffeine intake, stopping smoking, stress reduction, and learning free relaxation techniques.

Temperature

Body temperature varies from person to person—the normal range is 36–37.5°C. It is always useful to have a record of each individual’s normal temperature, as what may be a normal temperature for one person, may be high or low for another. Normal body temperature is slightly lower in the morning than in the evenings.

A wide range of factors influence body temperature. These include age, exercise, circadian rhythms, hormonal actions, stress, and environmental factors. Hypothermia, or low body temperature, develops when the body temperature becomes too cold, and when the usual body mechanism of heat production cannot maintain a thermal balance. This is most often seen in older people, or people overly exposed to very low temperatures for a long time. A high temperature, known as pyrexia, or hyperthermia, is symptomatic of infection, neurological injury, extreme drug reaction, or heat exhaustion.

Body temperature is recorded using a thermometer. There are different types of thermometer, including glass thermometers, tympanic (ear) battery operated devices, fever brow (chemical disposable strips), and electronic readers.

The most commonly used thermometer, in the home, is the glass thermometer. Under the tongue, in the mouth, is the most commonly used site for recording temperature. The mouth, however, should never be used for infants, small children, people with poorly controlled tonic clonic epilepsy, or people with ID who are confused or have poor cognitive understanding. In these situations the thermometer should be placed, and held, in the axilla (arm pit), or another type of thermometer should be used. On rare occasions, body temperature may be taken using a special rectal thermometer.

Pulse

The measurement of pulse rate is another aspect of a person’s health assessment, closely associated with temperature, respiration, and blood pressure. Taking a pulse reading is one common technique used to assess how well the heart is working, and therefore, to assess the general health of the patient. A pulse reading measures the force of blood pumped from the ventricle, in the left side of the heart, into the aorta, the main artery that then sends blood around the body. Pulse points are places in the body where an artery can easily be felt when pressed against a bone. There are 10 common pulse points, the most common being the wrist (radial pulse), the neck (carotid pulse), and the inner arm of the elbow (brachial pulse). Placing a finger or a stethoscope over a pulse point is referred to as ‘taking a pulse’. This gives information on the number of times the heart beats in a min, the rhythm and regularity of the pulse, and its strength. If taken over several pulse points, it can also measure consistency of blood flow throughout the body.

A normal adult pulse rate is 60–100 beats/min. Depending on the age, a child’s pulse rate is significantly faster. Exercise, temperature, anxiety, pain, body posture, and drugs or medication can all influence pulse rate.

Respiration and oxygen saturation levels

Respiration

When a body is at rest, and the person is in good general health, respiration, or breathing, should be regular, require minimal effort, and be quiet. By discreetly observing a patient’s respiration, much can be indicated relating to general health. The measurement of respiration involves counting the rate, depth, and pattern of chest wall movement. Breathing rates are extremely variable, being influenced by age, emotion, and pain. The normal adult respiratory rate is 12–18 breaths/min.

What is respiration?

Respiration is another word for breathing. It involves a process of gaseous exchange in the lungs. This involves air constantly moving in and out of the lungs, bringing oxygen into the body and removing carbon dioxide.

The observation of respiration is something that can be done by anyone working with people with ID, and may be the first observation to identify physical ill health. Changes in respiration may include coughing, either dry or productive; that may be different at different times of the day, or in different postural positions. This could indicate the presence of infection, asthma, or allergy. Coughing during eating or drinking may suggest that the person is aspirating (breathing) food or liquid into their lungs, and should be referred to the GP as a matter of urgency. In observing seizures in epilepsy, particular attention needs to be given to ensure that the airway to the lungs does not become blocked. Halitosis, bad smelling breath, may indicate poor oral hygiene, infection, or severe constipation. Sweet smelling breath may indicate diabetes.

Oxygen saturation levels

The body cannot function well without high levels of oxygen in the blood. Oxygen is carried in the haemoglobin of the red blood cells, which take oxygen to all parts of the body. Levels of blood gases may be monitored using a noninvasive method of pulse oximetry, in which a probe is placed on the end of a finger, covering the fingernail. If more detailed information is required, a blood test known as ABG (arterial blood gases) is undertaken to measure oxygen, carbon dioxide, and acidity levels of blood. These levels relate to how well body organs, predominantly the lungs, are working.

An ABG test is carried out on blood taken from an artery. It is likely to be requested to:

  • check for breathing problems in lung disease, such as asthma, cystic fibrosis or chronic obstructive airways disease

  • check how well lung treatments are working

  • check whether more oxygen is needed if the person is on oxygen treatment

  • measure the acid levels in the blood of people with heart failure, uncontrolled diabetes, severe infections, or drug overdoses.

Epilepsy

Epilepsy is a condition where a person has a continuing tendency to have seizures resulting from an abnormal electrical discharge from a group of brain cells, caused by a disruption to the electrical and chemical balance of neurons. The type of seizure the person experiences depends on which part of the brain the discharge originates, and how far and quickly it spreads.

Diagnosis of epilepsy

Diagnosis of epilepsy involves identification of seizure type, and relies on a description of the episode provided by the individual, where possible, and/or an eyewitness. Detailed information, before (what the person was doing, environment, time of day, how did they feel/appear, warning or partial onset, any potential ‘trigger’), during (sudden onset, fall, body movements, eyes open/closed, incontinence, gum/tongue bite, level of consciousness, behaviour, duration), and after (recovery time, confusion, behaviour, injury) is essential. Video recording is increasingly being used to assist with diagnosis. Tests including EEG, MRI, and blood screening may be performed to support a diagnosis of epilepsy and/or assist in differential diagnosis. If possible, epilepsy syndrome should be identified, the disorder defined by the type(s) of seizures seen, EEG findings, age of onset, family history, response to treatment, and prognosis (e.g. Lennox Gastaut Syndrome).

Main seizure types

Focal or partial seizures

affect only part of the brain.

Simple partial seizures—consciousness is not impaired, and duration is less than a minute. Can present with:

  • localized motor signs e.g. twitching of mouth (may spread to other areas), head turning, lip smacking, rhythmic jerking of one part of body

  • special sensory symptoms e.g. taste, smell, tingling, light flashes, sound

  • autonomic symptoms e.g. epigastric sensation, pallor, sweating, flushing

  • psychic symptoms e.g. déjá vu, distortion of time, fear, anger, dreamy.

Complex partial seizures—consciousness is impaired, duration approximately 1–3mins, difficult to tell exactly start and finish of seizure, some confusion after, and no memory of event. Can present with complex automatic behaviour e.g. dress and undress repetitively, sucking, chewing or swallowing movements, inappropriate urination, frantic running, and uncontrollable laughing. May develop from simple partial seizures, and may evolve to secondary generalized seizures.

Generalized seizures

affect more or less all the brain.

Absence seizures—brief impairment of consciousness, will stare normally for less than 10s, may be some mild motor movements e.g. eye blinks, mouth movements, rubbing the fingers together.

Atypical absence—similar to above but last longer, may be somewhat responsive, and motor movements are more pronounced.

Myoclonic seizures—very quick muscle jerks usually of arms, legs or trunk, may be one sided or both sides, frequently happens soon after waking.

Clonic seizures—rhythmic jerking movements of the arms and legs, sometimes on both sides of the body, rare seizure type.

Tonic seizures—sudden stiffening of limbs, body, and facial muscle, will fall if standing, lasts seconds, can cause injury, fairly quick recovery.

Atonic seizures—sudden loss of muscle tone, person drops to the floor, lasts a few seconds, can cause injury, quick recovery.

Tonic–clonic seizures—may be preceded by partial seizure activity, may experience a ‘prodrome’ (feeling unwell for some days before). Sudden contraction of muscles (tonic stage), will fall if standing, may cry as air is expelled from lungs, may bite gum or side of tongue. Intermittent relaxation and contraction of muscles follows (clonic stage); results in rhythmical jerking of body. Person may become blue, usually around the lips, exaggerated by dilation of blood vessels in the face caused by pressure from the contraction of the chest. May be excess saliva, snorting noises may be heard, and the person may be incontinent. Periods of relaxation become more prolonged, eventually contractions stop, usually within 1–2mins. Full consciousness may not return for 10–60mins, confusion and tiredness may persist for hours or days.

Unclassified seizures

—seizures may present which are unclassifiable.

Treatment of epilepsy

The effective treatment of epilepsy is important in order to provide opportunities for an enhanced quality of life for the person, and reduce the risk of sudden unexplained death in people with ID (see Physical health and well being Supporting people with epilepsy, p.[link]). AEDs are the mainstay of treatment for epilepsy; the aim of their use is to maintain a normal lifestyle by complete seizure control without drug-related side effects. Some 70–80% of newly diagnosed cases of epilepsy have a good outcome with AEDs; however, complete control may not always be achievable. Choice of AED depends on seizure type and individual circumstances. One prescribed AED at a time is preferable; however, more than one may be required. Published guidelines provide recommendations for AED treatment.1

Non-pharmacological approaches to the treatment of epilepsy include psychological and alternative therapies, and avoidance of obvious ‘triggers’, and these may help seizure control for some individuals. Generally a healthy lifestyle approach is recommended to help seizure control; consideration should be given to diet, exercise, and compliance with medication, along with a regular sleep pattern, and controlled alcohol intake. Vagal nerve stimulation or neurosurgery, under certain circumstances, may be considered if satisfactory control cannot be obtained with AEDs.

Supporting people with epilepsy

Up to 25% of people with ID have epilepsy, compared with 0.7% of the general population. The more severe the ID the higher the prevalence of epilepsy will be. The aim of epilepsy management is to maintain or improve seizure control without drug-related side effects, or if seizures are unavoidable then to reduce physical and psychological injury as much as possible.

Epilepsy management plan (EMP)

Know the individual’s seizures—having an individual EMP will assist carers in recognizing and managing the person’s epilepsy on a day-to-day basis. The EMP should include the following.

Description of seizure

—what happens before, during and after the seizure, and recovery details (see Physical health and well being Diagnosis of epilepsy, p.[link]). It may take time to observe and record seizure activity in various settings.

Usual duration of seizure

—care should be taken to detail the time of actual seizure activity and recovery period separately.

Management of seizure

—detail the first aid required for particular seizure types (see Physical health and well being First aid for seizures, p.[link]) and consider individual needs.

Emergency management

—this should detail after how many minutes or the number of seizures when emergency services should be contacted. Alternatively when emergency medication should be administered, if the individual is prescribed them, and staff are trained to administer—this will require an individual emergency management plan.

Other relevant information

—e.g. how often do seizures occur, what time of day, are they in a particular environment, are there any triggers and how can these be avoided.

Epilepsy and risk

When specific concerns are raised, individual risk assessments should be carried out. This is because a balance is required to minimize risk while simultaneously achieving optimum quality of life for the individual. There may be a tendency to over protect, so individual choice, capacity, and consent are issues must be considered.

Supporting individuals to maintain or improve seizure control

Correct diagnosis

—diagnosis should be reviewed by a specialist if seizure control does not improve. Observation and detailed description of seizures is essential for correct diagnosis; involve individual, family, and consider video recording to assist with this. Note that challenging behaviour, repetitive stereotyped behaviour, and behaviour relating to mental ill heath can make diagnosis difficult. A structured approach to differentiation is needed and may require behavioural analysis. Communication difficulties further complicate the diagnosis and treatment of epilepsy.

Regular review

—individuals should have a yearly review, or more frequently if seizure control does not improve, deteriorates, or AED side effects occur.

Medication compliance

—make sure education is provided and memory problems are identified. Records should be inspected for missed medication; consider use of alarms, pill dispensers, timing, and slow-release preparations to increase compliance.

Identifying triggers

—isolate factors unique to the individual if apparent e.g. physical stress—pain, pyrexia, constipation, lack of sleep, emotional stress, missed meds, alcohol, drugs (prescribed and illegal), and hormonal impact on women.

Comorbidity

—be aware of drug interactions and their effect on seizure control or other treatments, particularly drugs used for mental ill health.

Complementary therapies

—some find these helpful although no evidence suggests they are successful in controlling or curing epilepsy.

Lifestyle choices

—a healthy lifestyle approach is recommended to help seizure control, including diet, exercise, regular sleep pattern, structured routine, avoidance of drugs, and controlled alcohol intake.

Education

for individuals with epilepsy, their family and support staff, including information on voluntary organizations e.g. Epilepsy Action, National Society for Epilepsy.

First aid for seizures

Simple partial seizures

—simply stay with person and offer reassurance.

Complex partial seizures

—stay with the person, speak calmly and reassure, do not restrict movements (as it may be misinterpreted as aggression and they may respond aggressively), guide the person away from danger if necessary. Even if confused for a short period most people recover quite quickly and can continue with their work/activity. If complex partial seizures persist (non-convulsive status epilepticus), the person will require medical assistance and possible hospitalization.

Absence seizures

—usually no help is needed; if walking they may require guidance and reassurance.

Myoclonic seizures

—usually so short lived little can be done other than reassure when over; may be unbalanced, help to steady them, if they fall check for injuries.

Clonic

—stay with person, prevent injury from jerks, reassure.

Tonic and atonic seizures

—these are over very quickly therefore little can be done during the seizure; check for injuries, which may need medical attention, stay with the person and reassure. Tonic–clonic seizures—try to stay calm, time seizure, assess danger to self and client, and only move if in danger. Protect the person’s head, loosen tight clothing, and remove glasses. Move objects away if you can or try to protect all from injury. When seizure has ceased, if the person does not regain consciousness place them in the recovery position. Monitor breathing, and skin colour, maintain airway. Wipe away excess saliva, do all you can to minimize embarrassment, quietly reassure, keep others from gathering around. Assess for injury. Allow to rest if they indicate need. Observe as they may be confused. Inform carer where appropriate.

See Physical health and well being Emergency management of a person in a seizure, p.[link].

Cardiorespiratory disorders

This section outlines some of the health challenges that some people with ID face because of cardiac and or respiratory problems. Cardiac disorders experienced by some people with ID may result from the underlying cause of their disability. For example people with Down syndrome experience a range of cardiac disorders that require effective management. People with Down syndrome have a high prevalence of cardiac septal defects, Tetralogy of Fallot, ductus arteriosus, mitral valve disorder, and aortic insufficiency. Some may go on to experience pulmonary hypertension as a consequence of their cardiac disorder, with associated further complications such as heart failure. It is important, therefore, that cardiorespiratory disorders are assessed effectively and managed across their lifespan.

Respiratory disease is the most common cause of death for people with moderate or severe ID.1 This point is significant and is a major factor in the premature death of some people with ID, and nurses who practice in all care settings should recognize this. For example, gastric aspiration is associated with swallowing, specific nutrition, and feeding problems—and this is experienced by some in this population. Gastro-oesophageal reflux disorder (GORD) is common and is experienced by some 70% of people with ID.2 , 3 , 4 As can be seen in Physical health and well being Gastrointestinal disorders, p.[link] this may lead to them regurgitating the acidic gastric contents back up the oesophagus, resulting in inflammation of the respiratory tract that in turn leads to infection. Another example is illustrated in a recent study that identified respiratory disorders as common reasons for hospital admission for people with Prader–Willi syndrome.5

Cardiovascular disease is the second most common cause of death of people with moderate or severe ID.6 This contrasts with the general population, where it is the most common cause. Congenital heart disease is common in people with ID, and as this population is now living longer, it is anticipated that more will experience the complications of cardiovascular disease associated with the ageing process. As a consequence, some people with ID will experience conditions such as hypertension, vascular dementia, myocardial infarction, and cerebral vascular accident. Therefore it is important for nurses working in primary care, general hospital, and specialist assessment and treatment services, to be aware of the increased likelihood of these physical disorders being present.

As a consequence of these physical disorders, children, adults and older people with ID will be regular users of primary care and general hospital services.7 , 8 Nurses working in these care environments must familiarize themselves with the distinct needs of people with ID in their care to ensure that their needs are appropriately addressed.1

Some people with ID who have cardiorespiratory disorders will also require assessment and treatment from specialist ID health services, because of the nature of their complex health needs. Nurses practising in such services need to be familiar with the physical health problems that can be experienced by people with ID in their care. They have an important contribution to make in supporting them during the assessment and treatment of their physical healthcare, and have a responsibility to liaise and communicate effectively with colleagues in primary and general hospital care settings, to ensure that issues relating to communication, behaviours, mental illness, and ASD are reflected within nursing care plans.

Obesity

Obesity is a growing issue in developed countries. The level of obesity is higher in people with ID than in the general population.1 There are a range of factors that contribute to this situation, which include limited physical activity, poor nutritional practices, and the lack of nutritional knowledge, that collectively results in a population that is overweight.2 Obesity is a significant risk factor among people with ID for the development of coronary heart disease, cardiovascular disease, cerebrovascular accident, and type II diabetes.3 Obesity is linked to genetic conditions such as Prader–Willi syndrome and women with Down syndrome. Men with Down syndrome have been found to be obese, but less so than in people with ID without Down syndrome. Obesity results in a decrease in life expectancy and an increase in health needs.4 In recognition of the increasing life expectancy of people with ID, obesity is a significant health issue that needs to be addressed.

Inactivity plays an important part in contributing to obesity in this population. People with ID lead more sedentary lifestyles when compared with the general population, and have limited opportunities to exercise. Many people with ID are dependent on a carer, be it family or paid, to provide living support. Studies have found that people who receive care in care homes and from family carers are more likely to be obese.5 Evidence further suggests that increasing the amount of vigorous exercise, reducing carbohydrates, and improving the daily diet can bring about benefits.6

Following assessment that includes identifying dietary habits and other risk factors, it is necessary to monitor and record weight regularly, and to set weight parameters appropriate to the individual. There is an opportunity to work collaboratively with people with ID and their carers to effect longer term change. Treatment needs to include a focus on providing education for carers and others involved in the care.

Primary care has an important role in assessing for diabetes, hypertension and cardiovascular disease. Preventative strategies are important, and effectiveness is increased when delivered in partnership with ID specialists such as nurses, physiotherapists, and dieticians. Nurses have an important role in working in partnership with dietitians to provide support, advice, and regular monitoring, thereby enabling reduction or maintenance of weight. Medications used to treat mental illness need to be reviewed, as they can contribute to obesity.7 Structured approaches need to be in place, including the development of weight management services that can provide a focus for supporting weight maintenance, and where possible weight reduction.

Nutrition

Nutrition plays an important part in everyone’s life. Food has a central role in relation to socialization, and is generally an enjoyable experience. It is recognized that nutrition plays an important part in the lives of people with ID, and there are high rates of obesity when compared with the general population.1 Obesity contributes to a range of important risk factors, such as the development of coronary heart disease, reduced mobility, musculoskeletal problems, and diabetes.2 This emphasizes the need to develop opportunities for people with ID to participate fully in community activities, and experience regular physical activity and exercise.3 Ensuring health promotion information is fully accessible is vital in supporting people with ID and their carers to make informed food choices.

The nutrition issues impacting on the lives of people with ID have important public health significance as the population ages and more people live into old age.

Whereas there is clear evidence of the prevalence of obesity within the ID population, for others, particularly those with profound and multiple impairments, there is the risk of being underweight or malnourished.4 The prevalence of being underweight increases with the severity of the ID, and is common in conditions such as cerebral palsy. People with profound and multiple ID are at increased risk of swallowing and feeding problems such as choking, regurgitation, vomiting, and gastric aspiration.5 , 6 As a consequence, comprehensive assessment is required of the gastro-oesophageal tract and swallowing mechanism, and of nutritional needs to maintain weight and appropriate hydration.

Nurses have a role to play in providing additional support during hospitalization when investigations are necessary. The issue of consent to treatment is important, particularly in those with profound intellectual impairment, and where capacity may be lacking. Effective individual nutrition plans are required to minimize the possibility of dehydration, gastric aspiration and pneumonia. The prevention of aspiration pneumonia is important as it is the highest cause of death in this population, and with appropriate assessment and management can be prevented. Nurses have an important role in working collaboratively with people with ID, their families, and carers, to promote healthy lifestyles that includes a focus on improving nutrition and physical activity wherever possible.

Mobility

There are high rates of mobility problems experienced by people with ID. Some mobility problems will be evident, such as those experienced by people with cerebral palsy and more severe forms of ID. Mobility may be reduced as a consequence of falls and injuries that result in deformity and pain which impacts on the ability and confidence to mobilize. Some antipsychotic and anti-epileptic medication can impair mobility, thereby contributing to falls and injuries.1 , 2 People with ID participate in lower levels of physical activity, well below those recommended to protect and improve health. As a result for the population there is an increased prevalence of obesity. Exercise, as part of the structured preventative approach, has the potential to increase life expectancy and improve health and well-being.3

In people with severe physical disabilities and those in older age, the role of postural management is relevant, and plays an important role in minimizing and preventing deformities and breathing difficulties in order to maximize mobility potential.4 Providing appropriate access to assessment and management by specialists such as ID nurses, physiotherapists, occupational therapy, and specialist services contributes to promoting and maintaining mobility.5 Ensuring regular attendance at podiatry services is important because of the higher prevalence of foot and toenail problems in this population. Access to specialist footwear may also be required. Addressing these issues helps to reduce pain, falls, and accidents, and offers people with ID increased mobility, which in turn creates the opportunity to participate in community activities.1 , 6

Some people with ID experience visual and hearing disorders that can be treated easily, which helps to improve mobility and self-confidence.7 Nurses have a role to play in facilitating access to audiology and ophthalmology services, orthotics and wheelchair clinics, and the necessary aid to promote mobility. Regular review of medication used to treat mental illness and epilepsy is important in this population to minimize possible side effects.

Exercise

Regular exercise is important for everyone and brings about positive benefits to both physical and mental well-being. Undertaking regular exercise increases community visibility and presence, and offers opportunities to establish and develop new relationships and friendships.

Ensuring that people with ID of all ages have access to and participate in regular exercise has become an issue of increasing importance and concern.1 As a population, people with ID are unhealthy, overweight, and undertake limited exercise.2 It is recognized that physical activity among people with ID offers an effective way to improve health and well-being.3 Inactivity is common within this population and there is lack of light, moderate, and vigorous exercise.3

Many of the health conditions and restrictions resulting from living environments impacts significantly on the ability of people with ID to exercise regularly.1 , 4 Many people with ID live in supported living or with families, and continue to attend congregated day activities with limited opportunities for physical activity.3 , 5 As the population ages and increases there is an opportunity to develop and incorporate physical activity within the supports and services offered to people with ID. This is important, because few people with ID achieve the recommended daily level of physical activity.4 , 6

Nurses and others working with people with ID have an important part to play in supporting the development of healthy lifestyle choices. It is important to ensure that regular exercise appropriate to the individual is incorporated within their daily activities. Identifying opportunities to participate in local leisure and exercise activities offers the potential to bring about the benefits of regular exercise, which in the longer term impacts on physical and mental well-being. By developing collaborations with people with ID, their carers, and creating opportunities whereby people with ID can be supported and enabled to undertake regular daily physical activity is vital in promoting and improving their overall health and well-being.

Diabetes

Glucose is necessary for normal body functioning, and circulates in the blood, where it is used by the cells and muscles as energy. Insulin is a hormone produced by the Islets of Langerhan’s in the pancreas and is necessary for the uptake of glucose by cells, to enable their normal functioning. Diabetes is an endocrine disorder affecting glucose metabolism. In diabetes, the pancreas produces little or no insulin or, for reasons that are poorly understood, the body cannot effectively use the insulin that is produced. As a result there is a build up of glucose in the bloodstream, which is disposed of by the body in the urine.

There are two main forms of diabetes, type I and type II. People with type I diabetes have difficulty manufacturing insulin; it is more common at a younger age. Insulin is required for treatment. In type II diabetes there is an inadequate amount of insulin produced to enable glucose metabolism. This form of diabetes is more common in adults, particularly those who are overweight. The clinical features of diabetes can include fatigue, an increase in micturition, glycosuria, and an increase in thirst and hunger.

There are a range of risk factors associated with the development of diabetes, including obesity, coronary heart disease, and poor diet.1 The link with obesity and the development of type II diabetes is clear, and this is an important issue for people with ID.2 Within the ID population, where diagnostic overshadowing is possible,3 the existence of diabetes may be overlooked, and therefore go untreated. It is important, therefore, to recognize the possibility of diabetes within the ID population, and support the opportunity to access health screening, appropriate management and, if required, treatment.

The ID population is increasing and ageing, and evidence further points to many of the population being overweight, which results in a reduction of their life expectancy and increases the risk of associated health complications, including diabetes.2 The effective prevention and management of obesity is therefore an important issue for many people with ID, and requires a range of coordinated interventions to improve health outcomes and quality of life.4

Interventions include the need to ensure that there are effective and systematic health screening programmes that support a treatment element, including weight reduction, behavioural interventions, and physical activity, and health promotion interventions.5 There is an important role for carers in the management of diabetes, and specific education and support needs to be available for them. Nurses have an important role in supporting people with ID and carers to manage their weight effectively as a means to reduce possible consequences of diabetes, coronary vascular disease, and obesity. Where diabetes is identified it is necessary to ensure that the treatment regimens in place are adhered to, and this could include compliance with hypoglycaemic medication and insulin. There is also a need to ensure effective monitoring and recording of weight, urinalysis, and blood results where appropriate. Effective management of diabetes can therefore require a coordinated approach to care, including the person with ID, their carers, primary care services, specialist diabetes services, and specialists for ID health services.

Thyroid

The thyroid gland is an endocrine organ responsible for making thyroid hormone, which is responsible for regulating some of the physiological functions of the body. The gland itself is situated behind the thyroid cartilage below the larynx in the neck. It comprises a right and a left lobe, joined in the middle by an isthmus. The thyroid gland produces the hormones thyroxin (T4) and triiodothyronine (T3), which are responsible for the regulation of metabolism and growth. T3 and T4 hormones are regulated by thyroid-stimulating hormone (TSH), which is released by the pituitary gland in the brain.

Thyroid disorders commonly take the form of hypothyroidism (myxoedema), where there is under production of the thyroid hormone, or hyperthyroidism (thyrotoxicosis), where there is over production of the thyroid hormone. Hyperthyroid disease can result in conditions such as Grave’s Disease, characterized by goitre (a swelling in the neck), tachycardia, arrhythmias, exophthalmus, over activity, sweating, and non-pitting oedema of the lower limbs.

Hypothyroidism is more common to people with Down syndrome, and the incidence of this condition increases with age.1 , 2 , 3 , 4 The clinical features associated with an under-active thyroid gland include;

  • Tiredness

  • Lethargy

  • Dry skin

  • Weight gain

  • Depression

  • Reduced concentration

  • Memory impairment

  • Constipation.

Diagnosis of thyroid disorders is made by measuring TSH T3 and T4 levels in the blood, and in some cases by biopsy.

In people with ID, hypothyroidism may not be detected until the later stage of the disease, and recognition is important to supporting health and well-being.5 6 Treatment is with levothyroxine tablets, and annual screening is recommended in people with Down syndrome.4

Nurses have an important role to play in the possible detection of hypothyroidism. A detailed history obtained from families and carers familiar with the person with ID can provide useful insight and background information regarding their usual behaviours and presentation, and changes that have occurred over a period of time. Regular recording of weight can indicate an increase, which, when coupled with other symptoms, may indicate the need for screening by primary care services. Health checks should include blood tests to check for thyroid disorders, and ongoing monitoring and review is required by those with active hypothyroidism and for older people with Down syndrome, where the condition is more common.4

Cancer

Cancer comprises a large number of diseases where abnormal cells divide uncontrollably and infiltrate normal body tissue. Cancer can spread throughout the body from the primary source, and is a disease that is found across age groups and genders. It is recognized that there are risk factors associated with the development of cancers, such as smoking, harmful chemicals such as asbestos, a family history, and conditions such as ulcerative colitis. Tumours may be benign or malignant. Malignant tumours can involve the invasion of tissues throughout the body, resulting in metastastic spread. Leukaemia is a cancer that involves blood, bone marrow, the lymphatic system, and the spleen, and does not form into a tumour.

People with moderate or severe ID experience a different cancer pattern compared with the general population that contributes to their mortality.1 , 2 In the general population respiratory cancer is the leading cancer for men and women, with breast cancer being prevalent in women and prostate cancer in men. People with ID experience lower levels of prostate cancer, respiratory cancer and urinary tract cancer, with evidence suggesting an increased risk of stomach, oesophageal, and gallbladder cancer.3 Cancers of the stomach and oesophagus may be related to the high prevalence of gastrointestinal disorders, and in particular GORD and Helicobacter pylori.2

Leukaemia is a cancer of the white blood cells, and there are two main types: lymphocytic leukaemia and myeloid leukaemia.

Lymphocytic leukaemia affects the lymphocytes, and myeloid leukaemia arises from an immaturity of the myeloid stem cell. White blood cells have an important role to play in protecting the body from infection. People with Down syndrome are at risk of leukaemia, with rates being significantly higher than the general population.4 People with Prader–Willi syndrome are at increased risk of myeloid leukaemia.5

Cancers are diagnosed by examining a biopsy of a tumour, blood analysis, by CT scan, or mammography. A range and combination of treatments may be used, including surgery, bone marrow transplants, chemotherapy, and radiotherapy.

Preventing cancer is important. Ensuring that women with ID are supported to access national cervical and breast screening services is important, as uptake is poor. Nurses have an important role in ensuring adjustments to care are made to help obtain consent and to ensure cooperation. Men with ID may need support and advice to assist with testicular self-examination. Screening for Helicobacter pylori should be undertaken, and treatment initiated. Some patients may require admission to hospital for further investigations, such as endoscopy. GORD should be treated with proton-pump inhibitors.

For those diagnosed with a cancer, additional support and information is required during investigations, diagnosis, and treatment.6 The issue of capacity to consent to treatment needs to be considered, and communication needs identified and addressed. With the population of people with ID there is a need to consider and identify their information and support needs, as some will require care from palliative care services as a consequence of cancer or other life-limiting conditions.7

Stopping smoking

People with moderate or severe ID tend to have a lower incidence of smoking when compared with the general population.1 , 2 This may in part be attributed to social and occupational factors, which could account for the lower rates.

Even though the overall level of cigarette smoking is lower, some people with ID are cigarette smokers and they require access to information, education, and support to assist them to stop.

Information available for the general population about the risks of cigarette smoking may be inaccessible to people with ID who may have difficulty reading. Therefore accessing health promotion information at an appropriate level and in an accessible format is necessary.

Alternative methods of providing health information are being developed by way of symbolized and pictorial health improvement material.3 , 4 , 5

Smoking cessation clinics and self-help groups have been developed for the general population. People with ID who smoke should have the opportunity to attend and participate in these.

It is important to recognize, however, that some people with ID may need additional support to enable them to access clinics and groups that support smokers to quit. Modified counselling may be necessary as part of a treatment programme tailored to individual needs.

CBT approaches have been developed to assist smokers to quit, and include preparation to quit, coping with the challenges after quitting, and becoming a long-term non-smoker.

Health screening programmes targeted at people with ID need to include a specific focus on smoking, and the possibility of associated respiratory problems. Primary care and specialist ID health services, as a result of the health screening, have the opportunity to identify people with ID who require education and support to enable them to stop smoking.6 Assisting people with ID to stop smoking will bring about wider improvements in their health.

Consideration and recognition needs to be given to the possibility of weight gain in a population that face challenges with their weight. It is appropriate, therefore, to consider support for stopping smoking, in the wider context of a plan aimed at supporting healthy lifestyles and choices for people with ID.7

Medication options to help people with ID to stop smoking need to be considered carefully, to ensure that they are appropriate to the individual, and that any additional support needs are identified, and clearly set out and agreed by them and their carers in the patient’s care plan. Ongoing review and monitoring is necessary.

Accidents

People with ID frequently experience accidents, falls, factures, and trauma.1 , 2 , 3 Accidents and falls are associated with epilepsy, the side effects of medication, such as anticonvulsants, visual impairments, impulsivity, and epilepsy, and are multifactorial. People with profound and multiple ID are at significant risk from fractures.4

Epilepsy is common in people with ID and can be related to specific syndromes and conditions such as Angelman syndrome, Tuberose sclerosis, Fragile X, Rett syndrome, and Down syndrome. Epilepsy has been implicated in contributing to injuries and sudden unexplained death in people with ID.5 , 6 Seizures can cause fractures, subluxation, joint dislocation, and soft tissue injury. Fractures to the skull may result from trauma to the head following a seizure.

It is now recognized that people with ID are more at risk of developing osteoporosis due to vitamin D deficiency and lower bone density than the general population.7 , 8 , 9 Osteoporosis can go unrecognized and may contribute to bone fractures that result from falls, nutritional issues, and the menopause.8 , 9 , 10 There is also the possibility that fractures can occur even with very minor injury, and this needs to be borne in mind if people with ID stumble, trip, or fall. Women with ID are at risk of fractures, and should be recommended to have bone density tests and medication to improve the bone’s density.10

Nurses working in unscheduled care services, such as accident and emergency departments, need to be aware of the possibility of fractures. The detection and treatment of fractures is important as it eliminates pain and suffering, as well as the possibility of deformity and reduction in functioning. Exercise, good nutritional supplements, and medication for the treatment of osteoporosis can reduce the progress of this disorder.

People with ID can experience mobility problems due to associated conditions such as cerebral palsy and severe intellectual disabilities.4 Mobility problems may also be associated with disorders of the foot, which if not treated can contribute to falls, and associated trauma and injury in this population.

Some people with ID are prone to become overweight, and ensuring the issue is addressed is important in terms of accident prevention and improving health. Poorly managed respiratory disease can impact on the ability of people with ID to mobilize, and conditions such as asthma may reduce the ability and confidence of people with ID to participate in community activities. As this population ages, many will experience cardiovascular disease such as diabetes, high blood pressure, and cerebral vascular accidents, and these conditions can impact on the mobility of people with ID, thereby increasing their disadvantage.11 Nurses have an important contribution to make in collaborating with dietitians, physiotherapists, primary care colleagues, carers, and people with ID to minimize the impact of health conditions and associated complications, thereby helping to reduce the possibility of accidents that in some circumstances result in further disability and can contribute to premature death.

Further reading

Kelly K, Stephen L, Sills G, Martin B (2002). Topiramate in patients with learning disability and refractory epilepsy. Epilepsia 43(4), 399–402.

Kerr M (2001). Clinical guidelines for the management of epilepsy in adults with an intellectual disability. Seizure 10, 401–409.

McGrowther C, Bhaumik S, Thorp C, Hauck A, Branford D, Watson J (2006). Epilepsy in adults with intellectual disability: Prevalence, associations and service implications. Seizure 15, 376–386.

Sexual health and personal relationships

People with ID are unique individuals who have their own relationships and friendship needs.1 , 2 They are sexual beings and have the right to express their sexuality in line with their peers and all other members of society, but their sexuality and sexual health needs have historically been ignored and their rights not recognized. They need to access information relating to their rights and responsibilities regarding relationships as well as their sexuality and sexual health.

Young people and their parents

There is a need to include young people with ID in sex education programmes within schools, and materials have been developed to assist in this regard. Regardless of age they have to have accessible sexual health information that enables and supports them to make choices within their relationships. Nurses have an important role in supporting and facilitating them to form relationships and express their sexuality. By working with small groups nurses can support and develop understanding of puberty, menstruation, masturbation, and contraception. It is important to recognize the needs of parents, and differences in views and opinions that may be expressed by them regarding relationships and friendships that their adult son or daughter may form. Parents may have anxieties regarding the ability of their son or daughter to enter into sexual relationships, and may be concerned about abuse and exploitation.

Women with intellectual disability

Women with ID have sexual and reproductive health needs. Additional support and education may be required by some young women at the onset of menstruation to enable them to understand and meet their personal care needs. Contraception and the additional information that may be required to enable choices to be made by these women offers opportunities for nurses in sexual health services, primary care teams, and specialist ID teams to support these women. Sexually active women should attend regular cervical screening; women with ID are sometimes not included because it can be distressing and embarrassing, so nurses working in specialist services should work collaboratively with colleagues in primary care and sexual health services to overcome this. Women should attend for breast screening, and as this group is now living longer, breast screening is indicated, because there is the possibility of an increase in cervical and breast cancer.3 Nurses should ensure that health screening programmes take account of their sexual health, and contraception needs, and that they are included in national screening programmes.4 , 5 As women age they will also experience the menopause. Literature highlights a delay or absence of puberty in some women with ID, whereas others, as a result of syndrome-based conditions, may fail to produce sex hormones.

Men with intellectual disability

Men with ID have their own distinct sexual health needs, and yet many are not included in well-men activities, and this is an issue that needs to be addressed. Testicular cancer can occur in young men and it is important to ensure that men with ID have the necessary knowledge and support to examine their testicles and seek help if indicated. As men age there is a need to recognize the possibility of prostate disease and prostate cancer; all social and healthcare workers must ensure that their care needs are addressed within screening and well-men programmes, and they are given information, advice, and support about relationships, sexuality, their sexual health, and options available to them to make informed choices.

Sexual health and abuse in intellectual disability

People with ID who are sexually active may be at risk of developing STIs, such as gonorrhoea, syphilis and Chlamydia, and the possibility of HIV and hepatitis. It is important to recognize that they may enter into same-sex relationships, and this may bring with it an increased risk of STI.6 , 7 , 8 People with ID have been victims of a range of abuses, and this includes sexual abuse.9 , 10 As a result of their cognitive impairment and communication difficulties, perpetrators may target such people and all social and healthcare workers need to be mindful of this.

Gastrointestinal disorders

Gastrointestinal disorders are common in people with ID and increase with the severity of their disability.1

GORD is experienced by ~70% of people with ID.1 , 2 Gastric aspiration and pneumonia are associated with the swallowing, nutritional, and feeding problems experienced by this population. As a consequence of GORD, people with ID, particularly those with cerebral palsy, scoliosis, and severe and profound ID, may regurgitate the acidic gastric contents up the oesophagus, which is then inhaled into the lungs. Material inhaled results in inflammation of the respiratory tract, which in turn results in pneumonia, which if not promptly and effectively treated and managed, results in death. Trial treatments with proton-pump inhibitors such as omeprazole and lansoprazole are indicated, and should be instigated without an endoscopy.3

It is important to identify and treat gastric disorders in people with ID, because they cause pain and distress, and have been linked to cancers.4 Nurses have an important role in working with carers, dietitians, physiotherapists, speech and language therapists, and colleagues in gastroenterology units in general hospital services, to ensure that risk assessments are undertaken, and plans of care developed and implemented, thereby ensuring that nutritional and care needs are met.

Some people with ID may require nasogastric nutrition or a PEG tube (see Physical health and well being PEG and PEJ feeding, p.[link]). This requires the surgical insertion of a tube into the stomach where it remains in situ and is used to provide nutrition. Nurses need to be familiar with the care needs of people with ID who have additional nutritional needs, and the safe management of the equipment they require. They also have a key role in providing education and support for carers and family members, and in reassessing care needs.5

Helicobacter pylori is a Gram-negative bacterial infection of the stomach lining and small intestine, that if untreated results in gastritis and ulceration of the mucosal lining. It is more common in people with ID than the general population, and many do not present with symptoms.6 , 7 Diagnosis is made by breath, blood tests or endoscopy, and biopsy and obtaining specimens can be challenging.8 Treatment is with antibiotics; it can recur and further treatment may be required. Side effects are common.7 , 9 Evidence suggests a link with the increased risk of stomach cancer, oesophageal cancer, and gallbladder cancer within this population.3 Nurses need to be aware of the possibility of Helicobacter pylori within the ID population, and of the presence of symptoms such as vomiting, weight loss, haematemesis, and melaena, which may indicate infection. Good infection control measures are required, such as scrupulous attention to hand hygiene, and particular attention is required when dealing with faeces and salivary secretions to minimize the spread of infection.

Constipation

Constipation is difficulty in passing faeces or a change in the person’s usual pattern. Having a bowel movement is a routine activity of daily living, although having a bowel movement does not necessarily happen every day, and what is normal varies from individual to individual. People with ID are more likely to be constipated than those in the general population.

What are the causes of constipation?

  • Not drinking enough fluids

  • Not having enough fibre in the diet

  • Not being active enough

  • Side effects of medications

  • Being stressed

Why are people with intellectual disability at a greater risk?

  • They may be at risk of not eating a balanced diet.

  • They may not drink enough fluids and/or have limited food choices, particularly if they are dependant on others.

  • They may engage in less physical exercise than is recommended, the prevalence is greater if they are less mobile.

  • They may be taking medications that have side effects, including constipation.

  • They may be over reliant on laxatives and or PRN (as necessary) medications.

  • They may be unable to communicate the urge to defecate or of being hungry/thirsty.

  • They may be unable to sit on the toilet in the right position or to use toilet safely.

  • They may have problems with eating and drinking (dysphagia).

  • Their carers may lack understanding of the effects of food and fluid on the bowel.

Bristol Stool Chart

This is a recognized general measure of consistency and form that is understood by many. This tool classifies faeces into seven types, according to their appearance. Type 1 has spent the longest in the colon, type 7 the least time, with type 4 being the easiest to pass with little discomfort (see Fig. 6.1).

Fig. 6.1 Bristol Stool Chart. Reproduced by kind permission of Dr KW Heaton, Reader In Medicine at the University of Bristol. © 2000 Norgine Pharmaceuticals Ltd.

Fig. 6.1
Bristol Stool Chart. Reproduced by kind permission of Dr KW Heaton, Reader In Medicine at the University of Bristol. © 2000 Norgine Pharmaceuticals Ltd.

Think about?

Fluid intake

—a person needs on average 2L/day of fluid, depending on the person’s age and weight. On average a person needs 30ml/kg every 24hrs. For further details seek advice from a dietitian.

Food intake

—a person should eat regular balanced meals that contain both soluble fibre (porridge oats, fruit, beans, lentils, baked beans) and insoluble fibre (wholemeal bread/flour, pasta, wholegrain breakfast cereals, brown rice). Consider keeping a food diary over a 7 day period. Any increase in dietary fibre should be done gradually to prevent any abdominal discomfort. This could mean starting ¼ wholemeal products with refined white products and gradually increasing to ½ and then ¾. Further advice should be sought from a dietitian.

Position on the toilet

—a person should be able to sit on the toilet comfortably, with their hips at a right angle to the floor, with both feet on the floor or firm base. See a physiotherapist or occupational therapist for further advice.

Increasing exercise

—if a person has limited mobility, abdominal massage could be included as part of a total bowel management programme. This is performed to promote peristaltic action within the bowel. Seek further advice from a qualified physiotherapist or community nurse

Use of laxatives

—these can be prescribed while dietary or other measures begin to take effect, or for drug-induced constipation, or when other measures fail. Laxatives can be divided into 4 main groups; bulk forming, stimulant, faecal softeners, and osmotic laxatives. Discuss further with either a non-medical prescriber or the person’s GP. It is important that these are reviewed once the food and fluids, and/or exercise have begun to take effect on the bowel.

Privacy and dignity

—think how you would feel if were trying to defecate while someone was caring for you. Think about how you communicate with the person at this time.

The gastro-colic reflex

—this occurs 20–30mins following a hot drink or food hitting the gut; having a bowel movement is more likely at these times and should be encouraged. While straining should not be encouraged, blowing into a balloon can be helpful and fun for children at this time, as this uses the muscles needed to bear down and defecate.

Spirituality

Spirituality is defined as ‘the essence of our being, and it gives meaning and purpose to our existence’.1 Our spirituality gives us a sense of personhood and individuality. It is the guiding force that gives us our uniqueness, and acts as an inner source of power and energy, which enables us to ‘tick over’ as a person. Spirituality drives some of us to search for meaning and purpose, and establish positive and trusting relationships with others. Some people may use religion as a medium to express their spirituality and as a way of relating to the transcendent.2 Spirituality is a journey and religion may become the transport to help people through their journey in life.

Spiritual needs

Spiritual needs may be attained through meaning and purpose, loving and harmonious relationships, forgiveness, hope and strength, trust and personal beliefs and values, spiritual practices, concept of God/Deity, beliefs and practices, and creativity.3

Spiritual care

Nurses should use a problem-based approach to plan care to meet the spiritual needs of their patients. Concentrating on helping patients in their growth can improve patients’ overall well-being. The following steps of the nursing process may be used for spiritual care.

Assessment

Valuable information central to spiritual needs should be obtained from patients (for Spiritual Assessment Guide, see Narayanasamy 2001, 85–86).4

Planning

The information from a spiritual assessment may contribute to the formulation of spiritual care plans. When formulating the care plan, careful consideration should be given to the patient’s individuality, the willingness of the nurse to get involved in the spirituality of the patient, the use of the therapeutic self, and the nurturing of the inner person (the spirit).

Implementation (giving spiritual care)

Implementation is about spiritual care intervention based on an action plan that reflects caring for individual. It is necessary to develop a caring relationship, which signifies to the person that he or she is significant. It requires an approach that combines support and assistance in growing spiritually.

Evaluation

As part of evaluation, the following questions may be helpful:

  • Is the patient’s belief system stronger?

  • Do the patient’s professed beliefs support and direct actions and words?

  • Does the patient gain peace and strength from spiritual resources (such as prayer and minister’s visits) to face the rigours of treatment, rehabilitation, or peaceful death?

  • Does the patient seem more in control and have a clearer self-concept?

  • Is the patient at ease in being alone? In having life plans changed?

  • Is the patient’s behaviour appropriate to the occasion?

  • Has reconciliation of any differences taken place between the patient and others?

  • Are mutual respect and love obvious in the patient’s relationships with others?

  • Are there any signs of physical improvement?

  • Is there an improved rapport with other patients?

Further reading

Koenig HG (2001). Spirituality in Patient Care: Why, How, When and What? Templeton Foundation Press: Radnor, Pennsylvania.

O`Brien ME (2003). Spirituality in Nursing: Standing on Holy Ground. Jones and Bartlett: Boston.

Swinton J (2001). Spirituality and Mental Healthcare. Jessica Kingsley: London.

Profound and multiple learningdisabilities

Definition

In the UK, the most widely cited definition of profound and multiple learning disabilities (PMLD) was provided by Lacey,1 who suggested that the term is applied to a group of people who can be described as having; profound intellectual impairment, and additional disabilities which may include sensory disabilities, physical disabilities, autism, or mental illness. The WHO state that profound intellectual impairment refers to people who score <20 on an IQ test.2 They need high levels of support and supervision in all aspects of their lives. They will have significant difficulties with communication and be reliant on others to anticipate their needs and interpret their non-verbal communication.

Problems with labeling

A number of labels have been used to refer to this group of people including; ‘profound disabilities and complex needs’, ‘profound learning disabilities’ and ‘the most severely disabled’.3 This can lead to confusion, not least for parents and carers, and also to difficulties with accessing appropriate services. Although definitions and labels are arguably necessary for the planning and delivery of care, people with PMLD are all different and should be treated as individuals.

Prevalence

According to Mencap,4 PMLD was the fastest growing part of the population of people with ID, and this is thought to be due to developments in medical technology, better control of epilepsy, and an increase in the use of tube feeding. Between 1985 and 2001, the number of children and adults with profound and multiple ID living in England and Wales rose from 25,000 to 40,000.4

Assessment of needs

Assessment should be holistic and include physical, psychological, emotional, and spiritual needs. Some areas that may need to be addressed are:

  • Mobility—many people with PMLD have difficulties with movement and control of posture. Specialized equipment may be required to aid mobility, support posture, and protect and restore body shape

  • Sensory disabilities—specialist assessment of hearing and visual impairment may be necessary

  • Respiratory difficulties, epilepsy and mental health

  • Eating and drinking—some people with PMLD are unable to take food by mouth and require gastrostomy feeding; others may experience severe swallowing difficulties, which must be assessed by a speech and language therapist

  • Continence

  • The nature and complexity of the needs of people with PMLD, and/or lack of appropriate support may affect behaviour.

Learning

Despite the severity of the disabilities, people with PMLD can continue to learn throughout their life if they are given the right opportunities. Learning takes place slowly, and constant repetition may be needed to develop the most basic skills.

Services and multidisciplinary support

Assessment and care should involve a multidisciplinary team, which may include:

  • family and carers

  • school/education professionals and day services

  • respite care

  • GP, community nurse, physiotherapist, occupational therapist, speech and language therapist, psychologist, and psychiatrist.

When planning care, consideration should be given to issues of informed consent. Many people with PMLD will be unable to give informed consent, both for day-to-day decisions and major health and welfare decisions. Decisions should therefore be made in accordance with relevant legislation (Mental Capacity Act 2005; Human Rights Act 1998).

Attitudes and social exclusion

People with PMLD are one of the most marginalized groups in Western society. Clark and Gates have argued that there remains considerable ignorance both in the general public, and health and social care professions about this group of people.5 However, the PMLD Network have argued vehemently that people with profound ID and complex needs have a fundamental right to life,3 and the Human Rights Act 1998 has enshrined their right to life in law. Lacey1 and the PMLD Network3 have pointed out the positive contributions that such people make to the lives of people around them.

Dysphagia

Dysphagia is a disorder of swallowing that may involve problems with any of the phases of a swallow—the reception and oral preparation of a bolus, the initiation of the reflexive swallow, and the pharyngeal or the oesophageal phase.1 Dysphagia can cause increased risk of mortality, dehydration, malnutrition, respiratory tract infections, and aspiration.

The normal swallowing process

Oral

—food in the mouth is chewed, sucked, and mixed with saliva to create a bolus to allow food to be swallowed.

Pharyngeal

—the swallowing reflex is triggered, allowing the bolus to be moved into the oesophagus. The epiglottis lowers to protect the airway and prevent aspiration of food.

Oesophageal

—the bolus is moved along the oesophagus towards the stomach by peristalsis.

Signs and symptoms of dysphagia2

  • Difficulty chewing—food spills from lips, excessive mastication, tongue, jaw or lip weakness

  • Difficulty in swallowing—mouth dryness, lip/tongue weakness

  • Drooling—lip/tongue weakness, infrequent swallows

  • Swallow delay—food is held in the oral cavity for excessively long periods of time

  • Food sticking—food residue in mouth, pharynx or oesophagus after swallowing

  • Coughing/choking—because of food entering the airway instead of the oesophagus

  • Coughing when not eating—aspiration of saliva or lung abnormality

  • Regurgitation—undigested food in the mouth; food returning from the oesophagus to the pharynx, acid reflux

  • Weight loss—unexplained weight loss/weight below ideal standard.

Management of dysphagia3

  • Any concerns regarding the capacity of an individual to swallow effectively, should be referred to a speech and language therapist for a full assessment.

  • Dysphagia management procedures developed by speech and language therapists should be strictly adhered to. A multidisciplinary approach is required. Ensure that all members of the multidisciplinary team are aware of the risk factors and management procedures in place for those who present with dysphagia.

  • Correct positioning of the individual, in a chair with their head and trunk slightly forward will aid the passage of food through the pharynx and oesophagus.

  • Desensitizing the oral cavity prior to feeding may be beneficial for some individuals, by massaging the cheeks or tooth brushing to reduce hypersensitivity. Liaise with speech and language therapists to identify best approach.

  • Tilting the chin down, turning/tilting the head to the left/right may be beneficial for some people. However, a speech and language therapist should determine which position is best for each individual.

  • Ensure the individual is comfortable and free from distractions.

  • Use adapted equipment, spoons, and feeding cups to maximize food intake, as required.

  • Ensure food is presented in an appetizing manner, at an appropriate temperature. This may require preparation of small amounts of food at a time, particularly if the feeding process is protracted.

  • Allow enough time to feed the individual involved, pace the intake of food to meet the person’s needs, and give appropriate amounts of food at a time.

  • Determine the portion of food the person will eat.

  • Ensure the consistency of the food is right (pureed versus soft, thin versus thickened). If using commercial thickeners, follow manufacturer’s directions. Observe for changes in food bolus consistency; some foods become thicker/thinner as they heat up/cool down.

  • Use verbal/non-verbal cues to direct the person’s attention to eating.

  • Observe the individual closely during and following feeding. The person should remain upright for 30mins following feeding. Clear any remaining food from the person’s mouth when finished feeding.

  • Record dietary intake on appropriate recording charts, and monitor the person’s weight regularly.

  • Carefully observe the person’s preference for different foods, and reassess regularly as people’s tastes change over time.

  • Continuous assessment is required through direct observation and a diet history to monitor the condition.

  • Any deterioration in the condition, such as increase of incidences of aspiration, respiratory tract infections, weight loss, may be an indication of the need for enteral feeding.

  • It is essential to remember that not all individuals present with visible signs of aspiration. Silent aspiration is difficult to detect; individuals present with recurrent respiratory tract infections or pneumonia, which may be fatal. Video fluoroscopic swallow evaluation is required to identify silent aspiration.

  • Care of the oral mucosa is essential for people with dysphagia; food must be removed carefully after feeding.

PEG and PEJ feeding

PEG and PEJ feeding are forms of enteral feeding. PEG feeding requires the insertion of a tube through a surgically created opening, known as a fistula, in the abdominal wall (see Fig. 6.2). This creates an artificial tract into the stomach through which feeding can occur. A peg is a flexible silicone transabdominal tube, which following insertion is held in place by an internal retainer disc and external skin bumper disc. 6 months following insertion, a balloon tube or low profile button tube may be inserted. Some individuals who are at risk of severe GORD may need PEJ feeding, where the tube is placed in the jejunum, bypassing the stomach, thus reducing the risk of GORD. This is usually undertaken in association with gastroscopy, with jejunal extension (PEG-J) or direct percutaneous jejunostomy. Issues and care of PEG/PEJ tubes and feeding regimens are similar.

Fig. 6.2 Routes for enteral feeding. Reproduced with kind permission.©Burdett Institute 2008.

Fig. 6.2
Routes for enteral feeding. Reproduced with kind permission.©Burdett Institute 2008.

Indicators for use of PEG/PEJ feeding1

  • Aspiration and or anorexia, respiratory difficulties, dysphagia, limited gag reflex, poor appetite, cerebral palsy, cleft lip and palate, individual unable to meet nutritional requirements.

Advantages of PEG/PEJ feeding

  • An effective means of long-term nutritional support. Ensures a person is adequately hydrated, enables an individual to take in orally what they can while ensuring adequate nutritional intake is achieved, reduces the risk of aspiration, can be used in the presence of significant medical illness, and are concealed by clothing therefore are more cosmetically acceptable than nasogastric tubes.

Disadvantages of PEG/PEJ feeding

Gastrointestinal problems

  • Diarrhoea, constipation, nausea, vomiting, gastroesophageal reflux (associated more with PEG feeding).

Tube related

  • Invasive procedure, requires general anaesthetic, wound infection, blockage, kinking or dislodgement of tube, can be difficult to relocate (associated more with PEJ feeding).

Quality of life

  • Unnatural method of feeding, social element of mealtimes may be lost, loss of enjoyment, pleasurable experience of food, rapid or excessive weight gain, continuous feeding (12–20hrs/d) may limit mobility and level of activity, care of oral mucosa may be overlooked.

Feeding

With PEG tubes methods of feeding may include:

  • Bolus—given via syringe 3–6 times/d (feed is instilled by force of gravity)

  • Intermittent—via feeding pump (large volume 3–4 times/d)

  • Continuous—via feeding pump (12–20hrs/d).

With PEJ, feeding is continuous; PEJ feed needs to be given more slowly, and any water put into the PEJ needs to be sterile or boiled. Always confirm correct positioning of the tube using pH indicator strips; a pH of 5.5 or less indicates correct positioning of the tube. Before starting feeding, gain consent and reassure the individual. Never lay the person flat during a feed—elevate the head and upper body at least 30°. Maintain this position during and post feeding for 1hr. The feeding regimen developed for the individual should be adhered to, and manufacturer’s guidelines for storage and use followed. The tube should be flushed out after each feed using cool boiled water or sterile water, to prevent blockage. Carefully document feeds in appropriate recording charts.

Caring for PEG/PEJ tubes2

Use of standard infection control procedures is necessary. Observe for:

  • leakage of stomach contents, blockage of the tube (clear by flushing the tube with cool boiled water or pineapple juice), granulation of tissue at the site, which may indicate friction, bleeding around the tube, which may indicate tissue erosion from pressure or granulation tissue, bleeding through the tube,which may indicate gastritis/ulcer or tissue erosion caused by the tube, erythema around the tube caused by cellulitis or yeast infection, treatable with topical or oral antibiotics as required.

Nasogastric feeding

NG feeding is used to supplement feeding of individuals of all ages who have swallowing or feeding difficulties. It requires the insertion of a disposable plastic feeding tube into the nose, extending into the stomach1 (see Fig. 6.2).

Advantages

  • Ensures adequate nutritional intake, allows individuals to take in orally what they can while ensuring adequate nutritional intake is achieved, ensures a person is adequately hydrated, reduces the risk of aspiration, less invasive procedure than gastrostomy, and can also be used as a trial prior to gastrostomy.

Disadvantages

Gastrointestinal problems

  • Diarrhoea or constipation, nausea and vomiting

Tube related

  • Erosion of nasal and oral mucosa, sinusitis and/or nasal ulcers, skin irritation, from fixing the tube to the individual’s cheek, blockage, kinking or dislodgement of tube

Respiratory

  • Pulmonary aspiration, incorrect insertion of tube

Quality of life

  • More ‘visible’ than gastrostomy, can cause distress when inserted, unnatural method of feeding, social element of meal times may be lost, long-term use can impact on swallowing and oral feeding.

Nasogastric care and feeding

Types of nasogastric tubes

NG tubes come in varying sizes for children and adults. There are 2 main types of NG tubes:

  • Silicone tubes can be used for up to 1wk and are less expensive than silk tubes.

  • Silk tubes can be used for up to 6wks.

It is important to adhere to local health service providers and manufacturer’s guidelines for timescale for use and disposal of NG tubes.

Insertion of nasogastric tubes

  • Wash your hands prior to gathering required equipment, put on gloves, select the tube required, and take time to explain the procedure to the person. Gain consent, and offer reassurance, ensuring the person is in an upright position.

  • Identify the length of tube required by using the tube itself to measure from the person’s nose to their ear and down to the epigastric area. Make sure to lubricate the tip of the tube and gently insert the tube into the nostril in an upward and backward motion, and encourage the person to swallow or offer a drink if possible while passing the tube.

  • Continue to swiftly and steadily pass the tube through the nose and down into the stomach, until the desired length of tubing has been inserted. Then temporarily secure the tube to the person’s cheek and behind the ear prior to checking that the tube is positioned correctly in the stomach.

Confirming correct positioning of tube

It is essential to check the position of the tube before every feed. So using a syringe, aspirate fluid from the tube (~0.2–1ml of aspirate will suffice). Place a few drops of the aspirate on a pH indicator strip, and allow 10–15s for any colour change to occur. Match the pH indicator strip with the colour code chart on the strip box. A pH of 5.5 or below indicates that the tube is correctly positioned, and feeding can start. A pH of 6 or over suggests that the tube may be incorrectly positioned. Consider rechecking or re-passing the tube. Seek advice if the situation remains unchanged. Having confirmed correct positioning of the tube, it must be attached securely with tape to either the left or right cheek. Observe the person for any adverse reaction to the insertion of the tube.

Feeding

Feeding via an NG tube can take the form of a bolus feed, where the prepared feed is administered via a syringe barrel, held above the height of the person, and the feed is instilled by gravity. Feeding may also be continuous, where the feed is administered via a pump. On completion of each feed flush out the tube to ensure it is clean and to avoid blockages, and document input and output in appropriate recording charts.

Important points to remember

If the person can take fluids and food orally, then feeding via NG tube should only be used to supplement their oral intake as required. Particular attention should be given to oral hygiene, particularly to alleviate mouth dryness. Care of the nasal mucosa is also important.

Hearing

In developing and maintaining communication skills, information from all five senses is important. However, the main channels for communication are the distance senses of hearing and sight. Many people with ID have additional difficulties in communication because of sensory impairment. Clarke-Kehoe estimate that as many as 45% of people with ID may have some level of visual or hearing impairment.1 Hearing is vital for the development of spoken language. Those with a pre-lingual profound hearing loss will not have access to speech sounds and therefore will not develop verbal communication.

Behaviours that may indicate hearing loss

  • Pulling/poking the ears

  • Watching the speaker’s face and lips constantly

  • Tilting the head to one side towards the source of sound

  • Speaking loudly—this is typical of a sensorineural hearing loss, where people raise their voice so that it is audible to themselves

  • Speaking softly—this is typical of conductive hearing loss, where people match their own voices to the level at which they hear others voices

  • Appearing startled when someone approaches

  • Visible signs of discharge from the ears, or excessive wax

  • Dislike of loud sounds—this may be due to recruitment, where people have a reduced range between the point where they can just hear sounds and the point where sound is perceived as unbearably loud.2

Causes of hearing impairment

  • Conductive hearing loss—sounds have difficulty passing through the outer or middle ear; hearing loss is usually mild/moderate

  • Sensorineural hearing loss—the cause of the hearing loss is in the cochlea or auditory nerve; hearing loss is usually severe or profound.

Some people may have the same type and degree of hearing loss in each ear, or it may be different in each ear. A hearing test will identify the type of hearing loss the person presents with and the appropriate intervention that is required.

Levels of hearing loss

  • Mild hearing loss—falls within the range 25–40db. The sounds heard may be compared to listening to sounds when you have a heavy cold.

  • Moderate hearing loss—falls within the range 40–70db. This may be compared to listening to someone talking in the next room with the door closed.

  • Severe hearing loss—falls within the range 70–90db. Many sounds cannot be heard at this level. A person with a severe hearing loss would experience gaps in what they hear.

  • Profound hearing loss—exceeds 90db. The person will not hear normal speech, but would be able to detect rhythms in loud music and feel the vibration of sound.

Hearing aids

Hearing aids may be recommended post assessment. Nurses and carers will have a vital role in assisting the individual to maintain and use them effectively.

Communication

When communicating with a person presenting with hearing loss, it is necessary to follow certain measures:

  • Ensure you have the person’s attention and that they are looking at you. This promotes lip-reading and the use of facial expression to aid communication

  • Slow your speech

  • Do not shout

  • Cut out background noise where possible

  • Get reasonably close to the person. If a hearing aid is worn, the optimum distance apart is ~1m—beyond this point background noise will sound as loud as speech

  • Be aware in group settings; changing speakers may be confusing for the listener

  • Ensure that the person has had a recent ophthalmological assessment; when someone has a hearing impairment, it is particularly important to know the person’s visual acuity.

  • Use pictures, symbols, facial expression, gesture and Makaton signs to augment speech

  • Thorough communication assessment of the individual will allow for accurate intervention, using the most appropriate communication tool.

Integration of sensory experiences

There is an increased prevalence of sensory impairments within the population of people with ID when compared with the general population.1 , 2 , 3 Hearing and visual impairments are common, and may be apparent from birth. Others may be acquired in later life and may be associated with genetic conditions related to the cause of the ID, such as Down syndrome, where visual and hearing problems are common.3 , 4 Some people with ID can experience congenital cataracts, retinal abnormalities, optic atrophy, or abnormalities of the eye structure that result in visual impairment.5 Access to assessment and remedial prosthesis, and other treatments such as cataract surgery, can be beneficial, and therefore early detection, treatment, and regular review is important and will contribute to reducing the possibility of accidents and injury resulting from visual impairments. Attention should also be paid to the sense of smell and touch, and there have been developments in the provision of therapies such as reflexology and aromatherapy provided for people with ID and cerebral palsy. As people with ID are ageing and living longer they will also experience visual changes and hearing loss associated with the ageing process.1 , 6 Their possible changing needs must be recognized, and routine health screening programmes provide an ideal opportunity for nurses to incorporate visual and hearing screening at the same time. Effective screening and management can have positive benefits to the quality of life of the person with ID; barriers can exist, however, when undertaking assessment of vision and hearing.7 Nurses should play an important role in enabling access to visual and hearing assessments, and in ensuring there is support to adapt to their prosthesis, such as glasses and hearing aids, and in educating carers of their importance.3

Oral health

Oral health ‘is a standard of health of the oral and related tissues which enables an individual to eat, speak and socialize without active disease, discomfort and embarrassment and which contributes to general well-being’.1 People with ID experience poorer general and oral health than the general population. In the past oral disease has not been given as much priority as other complex medical problems experienced by people with ID, despite the severity of the impact of oral disease on this population.2

Impact of oral disease

  • Dental caries, coronal caries (of the crown part of the tooth)

  • Root caries (of the root part of the tooth)

  • Periodontal disease (of the gum and supporting bone)

  • Lower number of natural teeth

  • Higher level of tooth extraction and lower levels of restorations

  • Significant pain

  • Inability to consume nutritious food

  • Poor quality of life (destruction of self-image, lack of confidence).

Oral disease has been linked to other systemic diseases such as:

  • Cardiovascular disease

  • Cerebrovascular disease

  • Diabetes

  • Respiratory infection

  • Periodontal bone loss

  • Osteoporosis.

Specific factors such as bruxism (involuntary grinding of the teeth), altered salivary flow resulting in xerostomia (dry mouth), and hypertrophy or overgrowth of the gingival tissues, due to long-term use of certain medications need to be addressed.3

Effective oral care.

Oral assessment

—simple oral assessment can initially be carried out by carers; healthy gums are firm in texture and do not bleed when brushed. Inflamed gums are swollen and often bleed. Observe for any changes in teeth, such as cavities, signs of trauma, or loose teeth. Observe the lips, gums, tongue, palate, and soft tissue of the oral cavity for damage and mouth ulcers. A full assessment by a dentist needs to be undertaken to identify signs and symptoms of oral disease. An annual review with a dentist should be standard for each individual with and without teeth. People with ID may require an advocate or health facilitator to arrange dental visits and to ensure an effective oral care plan is developed.

An oral care plan

should:

  • Ensure a person’s teeth are brushed effectively, using an ordered approach to brushing to ensure no part of the mouth is missed. If an individual has difficulty tolerating this, brush different parts of the mouth throughout the day. Record oral care appropriately, including any abnormalities noted. If an individual can brush their teeth with assistance, this should be encouraged, to promote independence. Skills training programmes can also be developed to teach individuals to brush their teeth.

  • When brushing teeth, always explain to the individual what is to happen, and gain consent if possible. Brushing someone’s teeth is an invasive procedure and can be frightening for the individual involved. Be aware of how long a person can tolerate having their teeth brushed, brush more frequently and for shorter lengths of time if required. Encourage the individual to cooperate as much as he/she can.

  • Stand behind and to one side of an individual and gently draw back the lips with thumb and forefinger on one side of the mouth to gain access to the teeth. Commence with the upper teeth, brush teeth with short motions paying particular attention to the gum margins. Brush the inner and biting surfaces of all teeth as far as possible. If possible, gently brush the tongue. Assist the person to rinse their mouth, or clean around the mouth with a damp swab if required.

  • Proper nutrition is required to ensure good oral health; sugary snacks and drinks should be kept to a minimum. Self-cleansing foods such as apples and celery should be given as snacks between meals, for those who can tolerate them. No drinks (other than water) should be given after tooth brushing at night. It is important to remember that people who are fed via enteral feeding devices also require effective oral healthcare.

  • Medication can impact on oral health; more than 400 medications cause xerostomia (dry mouth), and anticonvulsant therapy has been linked to gingival overgrowth. Medications can often have a high sugar content, ‘sugar free’ options should be used where available.

  • Denture care is important, and individuals with dentures should be assessed regularly to ensure that their dentures fit properly. When cleaning dentures, follow manufacturer’s instructions when using cleaning agents. Do not use very hot water as this may warp dentures. They should be stored in a container of cool water, as they can become dry and warp. They can break easily if dropped onto a hard surface, so care must be taken when cleaning.

Healthy skin

The skin is the largest organ of the body, covering 1.5–2.0m2. It is made up of a number of layers and contains nerve endings, sweat glands, and blood capillaries.1

Functions of the skin

  • Protects the body against noxious agents and infectious organisms

  • Helps to regulate body temperature

  • Assists with the body’s fluid and chemical balance

  • Sensitivity to pain, temperature, pressure, and touch

  • Production of vitamin D with exposure to sunlight

  • By being visible to the outside world, the skin is also integral to an individual’s personal identification.

People with ID are at greater risk of suffering from skin complaints than the general population.2 For example, dryness and eczema is common in people with Down syndrome, and a variety of skin lesions and abnormalities are associated with phenylketonuria and tuberose sclerosis. Good assessment and care is therefore essential for this group.

Assessment

Skin care is integral to person-centred health action planning. Assessment should be individualized and holistic, and take account of personal preferences and cultural factors. Carers should be mindful of the impact of skin colour on the presentation of skin conditions. For example, lesions that appear red or brown in white skin may appear black or purple in pigmented skin, and mild degrees of redness (erythema) may be masked completely in black skin. Personal and intimate care can provide an opportunity for carers to assess the skin. People with ID who are able to carry out their intimate care independently should be educated to examine their skin and to report any worrying changes they notice to a carer or their GP. Assessment of the skin should include consideration of:

  • Family history of skin disease

  • Medication and its side effects

  • Seasonal effects

  • Allergies and changes in the use of cleansing agents, washing liquid and cosmetics.

Use of skin care products and cleansing agents

A balanced diet and drinking plenty of water is thought to be important for the health of the skin. Good personal and intimate care is also essential for maintaining the health of the skin, and the use of appropriate skin care products can promote healthy skin. Products should be chosen according to an individuals’ particular skin type, and based on individual assessment and preference. Soap and perfumed products should be used with caution because they can cause dryness and irritation, and soap substitutes, known as emollients, are more suitable for many people. Dry skin can be itchy, sore, and unsightly. This can be reduced and prevented with the use of appropriate emollients and moisturizers. Excessive washing, the use of detergents (such as soap), failing to remove soap from the skin, extensive soaking, and very hot bath water, can cause dryness and lead to dermatitis.

Common skin conditions and their symptoms

  • Eczema—dry, red, scaly skin that can be intensely itchy

  • Psoriasis—the most common forms appear as patches of silvery scales on top of areas of red skin

  • Leg ulcers—swelling, brown discolouration of white skin, presence of eczema

  • Pressure sores—initially appear as an area of red skin and feel warm and spongy or firm to the touch. In people with darker skin, the mark may appear to have a blue or purple cast, or look flaky or ashen. If pressure is not relieved, an open sore may develop that looks like a blister, and in advanced stages can appear as a deep crater-like wound.

  • Herpes simplex—ulcers or sores, found particularly on the face, hands and genitals

  • Dandruff—white, oily-looking flakes of dead skin appearing on the hair and shoulders, and an itchy, scaling scalp

  • Cellulitis—a bacterial infection resulting in redness, swelling, warmth, pain and tenderness.

A GP should be consulted for advice on specific skin complaints.

Care of the skin in the sun

The risk of skin cancer as a result of over-exposure to the sun has been well documented and established. The risk can be greatly reduced by taking the following precautions:

  • Stay out of the sun during the hottest part of the day. In the UK, this is usually 11–3pm in the summer months. Particular caution should be taken for people who are on psychotropics, or any medication, that can cause skin sensitivity to sunlight

  • Wear clothing made of cotton or natural fibres that are closely woven and offer good protection against the sun

  • Protect the face and neck by wearing a wide-brimmed hat

  • Use a high-factor sunscreen (SPF 30 or above) whenever you are exposed to the sun. Follow the instructions on the bottle and reapply as recommended, particularly after swimming

  • Never allow the skin to burn

  • Report mole changes or unusual skin growths to the GP promptly.

Personal and intimate care

Personal and intimate care includes: bathing and showering, dressing, changing continence pads, helping someone use the toilet, changing sanitary towels or tampons, shaving, skin care, hair care, and brushing teeth.

Good personal and intimate care is essential for health, hygiene, psychological well-being, and self-esteem. Many people with ID are dependent on others for their personal and intimate care. This might be as a result of deficits in cognition, sensory disabilities, or physical disabilities, which restrict movement and the ability to carry out fine and gross motor skills that are required for self-care. Personal and intimate care is carried out for hygiene, comfort, pleasure, and personal appearance.

Good hygiene

Skin cleanliness reduces the number of microorganisms on the skin that can cause infections. The genital and anal areas are particularly prone to bacteria and bodily secretions. Thorough hygiene is necessary to prevent infections and unpleasant odours in these areas.

Male hygiene

Perineal-genital care for an uncircumcised male involves retracting the foreskin to expose the glans or head of the penis, washing and drying the glans, and then replacing the foreskin. Inadequate hygiene and over washing with soap is associated with an increased risk of balanitis (inflammation and itchiness (of the head of the penis)), and viral infections.

Female hygiene

The vulva and perineal area should be washed daily, although the use of strong solutions of any kind in this area should be avoided because these can damage the mucus membrane. The use of deodorants, or clinging or synthetic clothes in this area can also lead to irritation and potentially vulvitis. Females should always be washed from cunnus to anus (i.e. front to back) in order to avoid transferring germs. Skin creases, such as beneath the breasts of women, are areas that are particularly prone to skin problems, and it is important that these areas are washed and dried gently but thoroughly, and with care.

Assessment and planning

Planning should be based on assessment of the individual’s physical and cognitive abilities, as well as their personal preferences. Other issues that need to be considered when assessing and planning care are:

  • Informed consent and capacity to consent

  • Maintaining and promoting independence

  • Particular needs around continence care

  • Choice of products (men and women with black and dark skin should use oils or moisturizers after bathing to keep the skin supple and smooth).

Cultural and religious considerations

In Hindu, Muslim, and Sikh religions, physical cleanliness is extremely important. Many people who belong to these religions have a preference for washing under running water; for these people showering is more appropriate than bathing. Modesty is important, particularly for women; legs and upper arms may need to be covered. The necessity for washing rituals prior to prayer time may need to be considered. In the Muslim faith genitals are washed with the left hand, because the right hand is used for eating. In Sikhism, hair and beards may need to be kept uncut; men and women fix their hair on their head with a plastic comb known as a ‘kangha’. Hair should not be cut without permission of the person or family.

Risk assessment, policies, and training

Areas that need to be addressed in risk assessments, policies and training include:

  • Prevention of abuse, and cross-gender care

  • Epilepsy and environmental safety (e.g. water temperature, need for slip mats)

  • Moving and handling

  • Infection control (consider use of gloves, aprons and good hand hygiene).

Good practice tips

  • Be discrete when talking about incontinence to others and in front of others; prepare the environment and ensure all items are close to hand.

  • Respect dignity at all times, e.g. do not undress the person until the last min when they need to be undressed, and use appropriate communication strategies, which may include short simple sentences and objects of reference. Allow the person time alone in private to masturbate, if they indicate by touching themselves that they would like to, and if it is safe for them to do so.

  • Intimate and personal care can be used as an opportunity for one-to-one communication and intensive interaction.

  • Clothing and appearance are important for self-expression.

Further reading

Cambridge P, Carnaby S (2000). Making it Personal: Providing Intimate and Personal Care for People with Intellectual Disability. Pavilion Publishing: Brighton.

Clark J (2006). Intimate care: theory, research and practice. Learning Disability Practice 9(10), 12–17.

Continence

Definition

Continence is the voluntary control over urinary and faecal discharge. Failure to achieve continence is referred to as ‘incontinence’ and is the involuntary loss of urine or faeces. Incontinence ranges from occasional leakage to an inability to control voiding.

Prevalence

Prevalence of incontinence is difficult to determine because a variety of definitions have been used, and also because of the stigma associated with it. Incontinence can happen to anyone and occurs across all age groups. It is, however, more common in older age. In people with ID, continence is more likely to be delayed later into childhood and some people remain incontinent throughout adulthood. Incontinence is more common in people with ID than the general population, and presents a greater problem to those with more severe disabilities.

Causation

Continence is dependent on hormonal, muscular, and neurological control. Incontinence is therefore associated with a variety of conditions, and the primary cause for people with ID is likely to be related to the nature of their learning and/or physical disability. Neurological and physical disabilities can be the root causes of incontinence and urinary tract infections, and renal abnormalities can exacerbate the problem. Many people with ID may have problems achieving continence if they are unable to understand the biological signals for urination and defecation. There may also be psychological and behavioural reasons for failing to urinate and defecate appropriately.

Assessment

Assessment should:

  • Be individualized, holistic and lead to person-centred treatment package

  • Be made by a multidisciplinary team, which might involve a GP, community nurse, continence advisor, physiotherapist, carers and social workers

  • Identify underlying problems and the type of incontinence

  • Include consideration of mobility, physical and mental health

  • Involve the measurement of fluid and dietary intake and output

  • Determine bladder and bowel habits and patterns.

Consider also the following factors, which may contribute to incontinence:

  • Medication

  • Constipation/diarrhoea

  • Lack of access to toilet facilities and lack of privacy

  • Difficulties with psychomotor skills needed for undressing and using the toilet independently.

Treatment

Incontinence is often viewed as an inevitable consequence of having ID, and therefore treatment is not always prioritized or even considered. However, with assessment, planning, and appropriate support, many people with ID have a significant chance of becoming continent.

A training programme for incontinence might include:

  • Positive reinforcement for appropriate voids

  • Teaching skills required for dressing and undressing

  • Regulating fluid and dietary intake

  • Making toilet facilities accessible

  • Training in pelvic floor exercises to increase muscle control for continence.

Management of incontinence

Good, person-centred personal and intimate care is essential when caring for someone who suffers from incontinence. This involves prompt skin care after defecation to reduce odour and skin problems. Brisk scrubbing should be avoided to prevent damaging the skin, and moisturizing cream or ointment can be applied to protect the skin from future exposure to urine and stool. Appropriate use of continence aids, such as the correct size of pads is important. Catheterization can be considered, and may be successful for people with neurological conditions.

The impact of incontinence

Incontinence can have serious effects on physical, psychological, and emotional well-being, both for the person and their carers. Some of the potential impacts are:

  • Increased risk of skin infections and perineal dermatitis and discomfort

  • Reduced independence and problems with access to social opportunities, school and work, and difficulties with accessing the community for extended periods of time due to limited changing facilities

  • The effects of stigma from a society that tends to undervalue people who have not mastered continence

  • Shame, embarrassment, depression and anxiety, lowered self-esteem, and negative body image

  • Barriers to sustaining relationships and leading a sexually fulfilling life

  • Physical, emotional and financial burden to carers and family; dealing with incontinence can be unpleasant and is a factor that leads to request for residential care.

Further reading

Stenson A, Danaher T (2005). Continence issues for people with learning disabilities. Learning Disability Practice 8(9) 10–14. Physical health and well being www.continence-foundation.org.uk

End of life care, preferred place of care

Planning for the future

As people approach the end of life, it is important to consider preferences regarding where they wish to be cared for and ultimately where they would prefer to die. While it is recognized that most people would prefer to die at home, many do not manage to do so and are often admitted to hospital in the last few days or hours of life.1 Reasons for this in the general population are complex, but are compounded for marginalized groups (such as people with ID or elderly people) who may lack the vocal ability to understand, make informed choices, and make their personal needs and wants known at this difficult time.

End of Life Care Programme

In recognition of the needs that all patients who are suffering and dying should have equivalent access to palliative care and opportunities for a good death, in the place of their choosing, the Department of Health launched their End of Life Care Programme. This initiative recognizes the importance of service user involvement in healthcare and aims to reduce the number of emergency admissions to acute care for those wishing to die at home, and it supports nationally recognized tools for end of life care. These include the Liverpool Care Pathway and the Preferred Place of Care (PPC). The PPC originated in Lancashire and South Cumbria Cancer Network, as part of a District Nurse education programme. From 2008, the PPC document will be known as the Preferred Priorities of Care (PPC), reflecting the importance of discussing priorities, preferences, and wishes with the patient and their family.

Preferred Priorities of Care

The PPC is a patient-held tool to record patient and carer preferences and priorities in relation to care and ultimate place of death, following the patient into different care settings as required. It recognizes that views are likely to change over time, and encourages discussions among the patient, the family, and other professionals associated with care delivery and support. Indicative to this process is the knowledge and ability of the care staff involved to facilitate the process in a meaningful fashion, that the individuals involved have the ability to communicate in a fairly sophisticated manner (both at staff and patient levels), and patients being supported to make informed, sometimes difficult, choices about sensitive and important issues. Such tools have been developed for the general population, and as such may not be easy to translate across to those within ID services.

Issues for people with intellectual disability

Marginalized groups such as people with ID have an increased risk of disenfranchised death resulting from a lack of autonomy, choice, and social exclusion, particularly at the end of their life. Challenges to reciprocal communication, an inability to comprehend complex information, and limited writing skills, are all factors that compound to make end of life choices around PPC difficult for both the staff facilitating the process and the patient who has an ID who is at the centre of that care. The Government’s White Paper, Valuing People,2 has advocated a person-centred approach to planning care for people with ID, which is a useful framework as end of life approaches.

Person-centred approaches to end of life care

Discussions regarding end of life preferences with people with ID should not be left until death is imminent, but as early as is possible and feasible after a prognosis is known. Person-centred approaches to care planning promote inclusion, civil rights, and independence for people with ID. Planning should start with the individual, not with services, taking into account their wishes and preferences. To this end, person-centred planning and palliative care share similar philosophies with regards to taking a holistic approach, incorporating active listening to the patient and those who know them best. Health action plans or essential lifestyle plan are well suited to facilitating holistic, palliative/end of life care.3

Supporting the individual who has an ID to make complex choices at the end of life may not be easy for those nurses involved. Shared value bases across different professional groups will promote collaborative working to support this initiative and help individuals to die in places of their own choosing. Research around this topic will provide a sound evidence base for future development and support.

Further reading

National End of Life Care Programme Physical health and well being www.endoflifecare.nhs.uk

Medicines management

For a range of reasons, people with ID are sometimes prescribed a lot of medicines. This may partly be because of their poor physical health, e.g. high prevalence rates of mental health problems and challenging behaviour, and comorbidity, e.g. epilepsy, which often require long-term use of medication. New standards for medicines management, recently published by the Nursing and Midwifery Council,1 outline 26 standards that nurse registrants need to consider and apply in whatever setting they work. However, it is also important to recognize and understand that these standards will not cover every eventuality that nurses will face with respect to medicines management, and that they will continue to be required to apply professional judgement and expertise in decision making. The use of medicines for people with ID presents a number of significant challenges/problem areas. These include polypharmacy (the use of two or more drugs), the detection of adverse/side effects of drugs, consent, choice and compliance, the high use of PRN medication, inadequate regular and/or routine monitoring and review, and the provision of information to service users and carers. Nursing staff can improve medicines management by reflecting on their competencies in the following 6 key areas.

1. Knowledge of drugs

People with an ID will be prescribed the same range of drugs as the general population, e.g. antipsychotics, analgesics, hypnotics, corticosteroids, laxatives, antidepressants, and anticonvulsants. Such medicines may be prescribed to be administered via a range of routes, e.g. topical, oral, and parenteral. If a nurse is involved in the administration of any medication to anyone with ID, then they must ensure that they have adequate knowledge, and have access to information about indications for use, normal dose parameters, expected effects, contraindications, precautions, side effects, and interactions with other prescribed medicines.

2. Delegation of care

Most medicines are administered by unqualified carers. Nursing staff will be expected to develop, enhance, maintain, and assess competence in the unqualified workforce, and should ensure that they have access to robust systems, guidelines, and policies to do so.

3. Consent

Individuals with an ID have the right to accept or refuse their medication. Significant difficulties can occur in the administration of medicines to this population due to refusal, or inability to express consent. For consent to be legal and valid the individual in question must:

  • have adequate competence to make the decision

  • be given adequate information

  • not be placed under any form of duress or coercion.

In obtaining consent, all three of these components may present significant challenges to everyone involved in caring for people with ID. It is vital, therefore, that nurses have adequate understanding of consent and capacity principles, and relevant law in this area.

4. Information to service users and carers

Nurses must be responsive to the information needs of service users and carers. By doing so, efficacy of medicines will be enhanced, and adverse effects reduced. Information about medicines should be provided in a format that can be understood. The Elfrida society (Physical health and well being www.elfrida.com) provides a series of easy to read information leaflets about a range of medications that could be used for this purpose.

5. Regular review and monitoring

Due to the potential for polypharmacy for people with ID, nurses should ensure that adequate reviews of medication take place on a regular basis and are undertaken by the appropriate physician. This should occur at least yearly, and twice yearly for individuals taking 4 or more medicines. Nurses must also equip themselves to use side effect rating scales for neuroleptic medication, and 2 examples are provided below:

Dyskinesia Identification System: Condensed User Scale (DISCUS)

is a well-established measure for the assessment of tardive dyskinesia among individuals with an ID.2

Liverpool University Neuroleptic Side Effect Rating Scale (LUNSERS)

provides information relating to a range of side effects of neuroleptic medication.3

6. Adherence to fundamental standards

Nursing staff should be guided by a range of policy guidance documents in this area, and should ensure that they are aware of, and comply with, key standards in respect of e.g. transporting medicines, use of controlled drugs, PRN prescriptions, self-administration, documentation, and the management of adverse events and errors.

Independent nurse prescribers in intellectual disability

The Department of Health and the Nursing and Midwifery Council state registered nurses with 3yrs post-registration experience may train to be nurse prescribers. Higher education institutions provide specific training courses with assessments, and the nurse has supervision by a medical practitioner. Independent nurse prescribers are now entitled to prescribe from the same formulary as medical practitioners.

  • This allows nurses to prescribe any licensed medicine for any medical condition that a nurse prescriber is competent to treat, including some controlled drugs.

  • It is essential that nurses are prescribing within their scope of practice and it has been negotiated appropriately with their employing authority.

  • It is the responsibility of the individual nurse prescriber to be updated through continued professional development in accordance with the Nursing and Midwifery Council nursing regulations.

The nurse independent prescriber within the ID profession enables the nurse to provide holistic patient-centred care to improve patient outcomes and quality of life.

Occasions for prescribing in intellectual disability

People with ID often lack access to appropriate healthcare. They are also more vulnerable than average to a number of physical health problems, e.g. gastrointestinal disorders related to constipation, and UTI.

  • For people with ID it can be more difficult to diagnose these conditions.

  • Many clients are unable to articulate their complaints of illness because they may have communication problems or a deficit in understanding.

In these situations they may present signs of restlessness or behaviour problems, and their symptoms may be camouflaged. If these minor ailments are not treated they could lead to life-threatening situations. This is when it is important for the nurse to use their skills and knowledge during the assessment criteria, which is a fundamental part of nurse prescribing.

Prescribing in intellectual disability

In the instance of UTI it is essential that the independent nurse prescriber identifies and treats the cause with a course of appropriate antibiotics. The same principle applies if a client is constipated, the problem must be identified and the relevant treatment applied. This could be a laxative medication. Often ID individuals have other co-morbid health conditions such as mental heath, dementia, or epilepsy. Patients are often on several medicines (polypharmacy) for various states i.e. neuroleptic medication for mental health and challenging behaviour, and antiepileptic drugs for epilepsy. A number of these drugs have side effects that cause constipation.

Areas where nurses may work using nurse prescribing

Many practising nurse prescribers are based within an ID specialist multidisciplinary health and social team in the community or acute assessment settings. Subsequently they work alongside a medical practitioner who supports the nurse prescriber with their practice. Supplementary prescribing ethos is very useful for specific long-term health conditions. This systematic prescribing approach is applicable for ID using the process of an agreed patient specific CMP.

The advantages of nurse prescribing for people with intellectual disability

  • Having a nurse available who is an independent nurse prescriber would be valuable for the service in the event a patient became ill and there was no medical practitioner accessible.

  • Often the nurse has developed a therapeutic relationship with the patient. A reason for this is the nurse will see the patient more frequently than the doctor and have more time to spend in the consultation. Nurses may identify possible barriers or influence concordance i.e. lifestyle, work.

  • The nurse can ensure that there is regular dialogue with the medical practitioners. Therefore it fosters better links with the doctor, and response to patient treatment intervention is more effective.

  • Nurses can prescribe emergency medication in the event of crisis, i.e. minor ailments, prophylactic anti-epileptic, or antipsychotic drugs, enabling the patient to have a quicker response to treatment.

  • The nurse can set up nurse-led clinics, enabling more autonomy in their practice.

Further reading

Allsop A, Brooks L, Bufton L (2005). Supplementary prescribing in mental health and learning disabilities. Nursing Standard 19(30), 54.

Department of Health (2005). Improving Health Services by Extending the Role of Nurses in Prescribing and Supplying Medication: Good Practice Guide. Department of Health: London.

Beckwith S, Franklin P (2007). Oxford Handbook of Nurse Prescribing. Oxford University Press: Oxford.

British Medical Association & Royal Pharmaceutical Society of Great Britain (Regularly updated). British National Formulary. BMA: London.

Courtenay M, Butler M (2003). Essential Nurse Prescribing. Greenwich Medical Media: London.

Department of Health (2006). Medicines Matters: a Guide to Mechanisms for the Prescribing, Supply and Administration of Medicines. Department of Health: London.

Nursing and Midwifery Council (2008). Standards for medicines management. Nursing and Midwifery Council: London.

Prasher Vee P, Janicki MP (2002). Physical Health of Adults with Intellectual Disability. Blackwell Publishing: Oxford.

Notes:

1 Welsh Office (2001) Fulfilling the Promises. Welsh Assembly: Cardiff.

2 Scottish Executive 2000 The Same as You? A Review of the Services for People with Learning Disabilities. Scottish Executive:Edinburgh.

3 Department of Health, Social Services and Public Safety (2005). Equal Lives. Department of Health, Social Services and Public Safety: Belfast.

4 Department of Health 2001 Valuing People. A New Strategy for Learning Disability for 21st Century. Department of Health: London.

1 Michael J (2008). Healthcare for All: Report of the independent inquiry into access to healthcare for people with learning disabilities. Department of Health: London.

1 Department of Health (2001). Valuing People. A New Strategy for Learning Disability for 21st Century. Department of Health: London.

1 Stokes T, Shaw EJ, Juarez-Garcia A, Camosso-Stefinovic J, Baker R (2004). Clinical Guidelines and Evidence Review for the Epilepsies: Diagnosis and Management in Adults and Children in Primary and Secondary Care. Royal College of General Practitioners: London.

1 Hollins S, Attard MT, von Fraunhofer N, McGuigan S, Sedgwick P (1998). Mortality in people with learning disability: risks, causes, and death certification findings in London. Developmental Medicine & Child Neurology 40, 50–56.

2 Galli-Carminati G, Chauvet I, Deriaz N (2006). Prevalence of gastrointestinal disorders in adult clients with pervasive developmental disorders. Journal of Intellectual Disability Research 50(10), 711–718.

3 Böhmer C, Klinkenberg-Knol E, Niezen-de Boer R, Meuwissen S (1997). The prevalence of gastro-oesophageal reflux disease based on non-specific symptoms in institutionalized, intellectually disabled individuals. European Journal of Gastroenterology & Hepatology 9, 187–190.

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2 Hamilton S, Hankey C, Miller S, Boyle S, Melville C (2007). A review of weight loss interventions for adults with intellectual disability. Obesity Reviews 8, 339–345.

3 van Schrojenstein Lantman-de Valk H, van den Akker M, Masskant M et al. (1997). Prevalence and incidence of health problems in people with intellectual disability. Journal of Intellectual Disability Research 41(1), 42–51.

4 Melville C, Cooper S-A, McGrother C, Thorp C, Collacott R (2005). Obesity in adults with Down syndrome: a case-control study. Journal of Intellectual Disability Research 49(2), 125–133.

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2 van Schrojenstein Lantman-de Valk H, van den Akker M, Masskant M et al. (1997). Prevalence and incidence of health problems in people with intellectual disability. Journal of Intellectual Disability Research 41(1), 42–51.

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4 Bhaumik S, Watson J, Thorp C, Tyrer F, McGrother C (2008). Body mass index in adults with intellectual disability: distribution, associations and service implications: a population-based prevalence study. Journal of Intellectual Research 52(4), 287–298.

5 Galli-Carminati G, Chauvet I, Deriaz N (2006). Prevalence of gastrointestinal disorders in adult clients with pervasive developmental disorders. Journal of Intellectual Disability Research 50(10), 711–718.

6 National Patient Safety Agency (2004). Understanding the patient safety issues for people with learning disabilities. National Patient Safety Agency: London.

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3 Hamilton S, Hankey C, Miller S, Boyle S, Melville C (2007). A review of weight loss interventions for adults with intellectual disability. Obesity Reviews 8, 339–345.

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5 NHS Health Scotland (2004). People with Learning Disabilities in Scotland: The Health Needs Assessment Report. NHS Health Scotland: Glasgow.

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1 van Schrojenstein Lantman-de Valk H, van den Akker M, Masskant M et al. (1997). Prevalence and incidence of health problems in people with intellectual disability. Journal of Intellectual Disability Research 41(1), 42–51.

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5 Hamilton S, Hankey C, Miller S, Boyle S, Melville C (2007). A review of weight loss interventions for adults with intellectual disability. Obesity Reviews 8, 339–345.

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5 Evenhuis H, Henderson C, Beange H, Lennox N, Chicoine B (2001). Healthy ageing – Adults with intellectual disability: Physical Health Issues. Journal of Applied Research in Intellectual Disability Research 14(3), 175–194.

6 McCarron M, Gill M, McCallion P, Begley C (2005). Health co-morbidities in ageing persons with Down syndrome and Alzheimer’s dementia. Journal of Intellectual Disability Research 49(7), 560–566.

1 Hollins S, Attard MT, von Fraunhofer N, McGuigan S, Sedgwick P (1998). Mortality in people with learning disability: risks, causes, and death certification findings in London. Developmental Medicine & Child Neurology 40, 50–56.

2 Patja K, Molsa P, Livanainen M (2001). Cause-specific mortality of people with intellectual disability in a population-based, 35-year follow-up study. Journal of Intellectual Disability Research 45, 30–40.

3 NHS Health Scotland (2004). People with Learning Disabilities in Scotland: The Health Needs Assessment Report. NHS Health Scotland: Glasgow.

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5 Davies H, Leusink G, McConnell A et al. (2003). Myeloid leukemia in Prader–Willi syndrome. Journal of Pediatrics 142, 174–178.

6 Tuffrey Wijne I, Bernal J, Jones A, Butler G, Hollins S (2006). People with intellectual disability and their need for cancer information. European Journal of Oncology Nursing 10, 106–116.

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1 Tracy J, Hosken R (1997). The importance of smoking education and preventative health strategies for people with intellectual disability. Journal of Intellectual Disability Research 41, 416–421.

2 Robertson J, Emerson E, Gregory N et al. (2000). Lifestyle related risk factors for poor health in residential settings for people with intellectual disability. Research in Developmental Disabilities 21, 469–486.

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