Sickle cell anaemia:
most common among people originating from areas in which malaria is endemic. Due to altered types of haemoglobin. Patients typically have a low Hb level (typically 8–9 g/dl) but compensate well. Consider all patients to be hyposplenic.
Haemophilia and other clotting factor deficiencies:
bleeding → joints or muscles is often delayed following trauma. If untreated, results in permanent damage. Pressure effects occur if bleeding takes place into a confined space, e.g. intracranial bleed. Severity of bleeding is related to levels of clotting factors.
treatment can be ‘on demand’ or ‘prophylactic’. All haemophiliacs should have long-term follow-up via a specialist haemophilia centre.
• Transfusion of factor VIII or IX preparation as soon as possible after bleeding has started—most administer it to themselves
• Symptomatic treatment of bleeds, e.g. rest, analgesia ± physiotherapy for bleeds in muscles/joints.
yellow pigmentation due to excess bile pigment (clinical jaundice when serum bilirubin >35 μmol/l). May be pre-hepatic (due to haemolysis), hepatic (hepatitis, cirrhosis) or post-hepatic (obstructive due to carcinoma, gallstones, sepsis, primary sclerosing cholangitis). All jaundice needs investigating to establish a cause, if unwell or rapidly progressive -admit, else refer for urgent out-patient appointment.
presents with sudden onset of severe illness.
• Hepatic encephalopathy (ranges from mild confusion and irritability through drowsiness and increasing confusion to coma)
• Haemorrhage—due to deranged clotting factors
• Ascites—hepatosplenomegaly and ascites are not usually prominent.
Breakdown of a surgical wound – usually abdominal. May be partial or complete. Readmit for further assessment except if very minor—when refer for urgent out-patient review. If complete dehiscence, cover with a sterile pack soaked in saline.
Acute intermittent porphyria:
Rare, inherited metabolic disorder. Porphyrins are important in the manufacture of haemoglobin. Deficiency of enzymes in the porphyrin pathway results in build up of intermediary which are toxic to skin and nervous system.
fever, GI symptoms (vomiting, abdominal pain – can be severe); neuropsychiatric symptoms (hypotonia, paralysis, fits, impaired vision, peripheral neuritis, odd behaviour – even psychosis). Urine may go deep red on standing.
English law does not require a doctor
• To confirm death has occurred or that ‘life is extinct’. A doctor is only required to certify what, in their opinion, was the cause;
• To view the body of a deceased person. There is no obligation to see/examine a body before issuing a death certificate;
• to report the fact that death has occurred.
English law does require the doctor who attended the deceased during the last illness to issue a certificate detailing the cause of death. Certificates are provided by the local Registrar of births, marriages and deaths. A special certificate is needed for infants of <28 d old.
Death in the community:
1/4 occur at home.
in all cases, advise to contact the undertakers and ensure the patient's own GP is notified.
• Patient's home: visit as soon as practicable.
• Residential/nursing home: if possible the GP who attended during the patient's last illness should visit and issue a death certificate. The ‘on-call’ GP is often requested to visit. There is no statutory duty to do this but it is reassuring for the staff at the home and often necessary before staff are allowed to ask for the body to be removed.
Unexpected and/or ‘sudden’ death:
if called, advise the attendant to call 999. Visit and take a rapid history from any attendants. Then:
• Resuscitate if appropriate—drowning and hypothermia can protect against hypoxic neurological damage; brains of children <5y. old are more resistant to damage.
• Report the death to the coroner—If any suspicious circumstances or circumstances of death are unknown/unclear—call the police.
Alternatively if police or ambulance service is already in attendance and death has been confirmed, suggest the police surgeon is contacted.
The Cremation Acts of 1902 and 1952 require 2 doctors to complete a certificate to establish identity and that the cause of death is not suspicious before a person can be cremated. A fee is payable to each doctor by the person arranging the funeral. It has 2 parts:
• Part B: completed by the patient's usual medical attendant—usually his/her GP.
• Part C: completed by another doctor who must have held full GMC registration (or equivalent) for ≥5 y and is not connected with the patient in any way nor directly connected with the doctor who issued part B—usually a GP from another practice.
Further information on completing cremation forms
Notification of death to the coroner:
the coroner can be contacted via the local police. Reporting to the coroner does not automatically entail a post mortem. The coroner, once circumstances of death are clear, may advise the GP to tick and initial box A on the back of the certificate which advises the Registrar that no inquest is necessary. Deaths which MUST be reported to the coroner are listed in Box 16.1.
In Scotland deaths are reported to a procurator fiscal. The list of reportable deaths is the same with the addition of deaths of foster children and the newborn.
Recording deaths in primary care:
death registers are useful. Routine communication of deaths to all members of the primary healthcare team and other agencies involved with the care of that patient (e.g. hospital consultants, social services) avoids the embarrassing and distressing situation of ongoing appointments and contacts being made for that patient. Record the death in the notes of any relatives/partner registered with the practice.
Patient advice and support
Department of work and pensions (DWP) Leaflet D49: What to do after a death in England and Wales. Available from www.dwp.gov.uk
Scottish Executive What to do after a death in Scotland. Available from www.scotland.gov.uk
Office of Fair Trading. Arranging funerals. www.direct.gov.uk