Delivering palliative care in the midst of a humanitarian crisis poses numerous challenges, among them being the provision of acute care during a palliative emergency. These emergencies may differ from those encountered in typical hospital or hospice environments outside of resource challenged zones. Potential emergencies include bleeding, pneumothorax, pleural and pericardial effusions, seizures, superior vena cava (SVC) syndrome and malignant spinal cord compression. The goal of this chapter is to provide some guidance in the approach to and management of these urgencies in the context of limited access to diagnostic and therapeutic tools.
Bleeding is commonly encountered in humanitarian crises, but in the context of treating patients on a palliative trajectory (end-stage cancer, liver disease), the management of these clinical situations may be challenging. Bleeding may occur in the range of 10–15% of patients with cancer alone.1 The presence of a brisk bleeding episode is usually quite distressing to the patient, family, and caregivers. Understanding the patients’ wishes for their care and their prognosis will assist the practitioners in their approach. Utilizing techniques that are accessible, inexpensive, and easy to use will be paramount in the care of active bleeding.2
Causes of bleeding may include tumor invasion, thrombocytopenia, carotid blowout syndrome, gastrointestinal sources (such as esophageal varices, ulcers, diverticulosis), nutritional deficiencies (vitamin K, folate, vitamin B12), medications (anticoagulants, antiplatelet agents), and treatment side effects (from radio- and chemotherapy, graft versus host disease).
Depending on the availability of resources and time constraints, the workup may be quite limited. Identifying reversible causes of bleeding such as medications (Coumadin) or bloodline pathologies (disseminated intravascular coagulation, thrombocytopenia, leukemia) may be possible depending on the situation.
Acute presentation of a bleeding emergency may present as epistaxis, decrease in level of consciousness due to intracranial hemorrhage, hemoptysis, hematemesis, melena, hematochezia, vaginal bleeding, hematuria, cutaneous bleeding from wounds or tumors, or internally (thoracic, peritoneal, retroperitoneal).3
Management of the bleeding emergency will depend on several factors, including the patient’s goals of care and the trajectory of their disease process. Clear communication to the patient and family is a priority in these situations, as the patient may decline within minutes. Having a stated plan will enable the opportunity for the patient to be optimally supported. This may be as basic as providing a private area with suction, dark towels (to offset the visual stress of the blood), and medications for pain and sedation (SC/IV benzodiazepine, opiate, or both), in addition to psychosocial support. For detailed information on medication and dosing for pain associated with bleeding, please refer to Chapters 4, 9, and 13 of this book.
Other potential bleeding scenarios and their management are as follows:
Bleeding wounds: Consider topical epinephrine, tranexamic or aminocaproic acid (powder, IV), direct pressure dressings, cautery (silver nitrate, thermal), and over-sewing small blood vessels.
Brisk hemoptysis: Position patient in lateral decubitus with the affected side (e.g., bronchial tumor) down. Consider radiotherapy, if appropriate, once the patient is stable.
Hematemesis/vaginal bleeding/melena: Attempt to slow rate of blood loss with a trial of tranexamic acid 500–1000 mg (IV/SC/topical with packing).
Medication reversal: If applicable, assess for reversal of agents causing bleeding (e.g., vitamin K, fresh frozen plasma for Coumadin).
• Octreotide (variceal bleeding)
• Vasopressin infusion
• Antifibrinolytics (tranexamic and aminocaproic acid)
• Platelet transfusion (thrombocytopenia)
Pneumothorax/Pleural and Pericardial Effusions
Dyspnea is a common presenting symptom in patients with palliative needs. Sources of dyspnea may present in a patient in crisis that requires rapid assessment and treatment. The workup of these patients will hinge on the availability of resources (e.g., diagnostic imaging) and other factors, such as rate of deterioration. The approach to caring for the patient in distress will, of course, be dictated by the patient’s wishes for care and the discrete clinical scenario. For details regarding medications and dosing, please refer to Chapters 5 and 13.
Patients with cancer and non-cancer diagnoses (e.g., COPD or heart failure) may be in distress due to either or both of these lung pathologies. Having a high clinical suspicion for these conditions will help direct the practitioner to address the problem quickly. The diagnosis can be made clinically (history and physical exam) and with the assistance of diagnostic tools, if available. Point-of-care ultrasound (POCUS)4 is increasingly being used as an extension of the physical exam in the assessment of patients with dyspnea. The portability, ease of use, and lower cost have made POCUS a reasonable option for many practitioners in resource-poor environments. Ultrasound can help direct the diagnosis and facilitate safe procedures (thoracentesis and tube thoracostomy placement).5 Traditional modalities, including X-ray and computed tomography (CT) are often not accessible in humanitarian disasters, owing to cost, electricity limitations, and personnel. If applicable, safe placement of tube thoracostomy, thoracentesis, or both may relieve dyspnea and hypoxia in patients in distress.
Pericardial effusions (PCE) can complicate many disease processes such as cancer (malignant effusion with breast, thyroid cancers), infections (viral, bacterial, parasitic, tuberculosis), metabolic and endocrine disorders (uremia, hypothyroidism), post–myocardial infarction (MI) (Dressler’s), and others (trauma, connective tissue disease). Clinical assessment can include Beck’s triad (muffled heart sounds, elevated jugular venous pressure [JVP], hypotension) and pulsus paradoxus. POCUS may assist in the diagnosis (transthoracic or subxiphoid views) and treatment (pericardiocentesis +/- pericardial window). Utilization of simple tools such as long spinal needles may be an option where expensive pericardiocentesis kits are not available.
In the context of patients with a palliative diagnosis, seizures are not uncommon. They may be due to intracranial metastases or hemorrhage, meningoencephalitis, metabolic abnormalities, or drug overdose, withdrawal, or interactions. Seizures may be generalized or focal, or both. Sustained seizure activity meets the definition of status epilepticus after 5 minutes of generalized or 30 minutes of focal convulsions. The diagnosis can be confirmed with electroencephalogram (EEG) studies, but this is often not a practical approach in a humanitarian-crisis scenario.
The treatment options are largely anchored on cessation via the utilization of benzodiazepines, which are inexpensive and accessible.6 For further details on medications and dosing, please refer to Chapter 13. Common options include the following:
Midazolam can be administered via multiple routes (IV/SC/buccal/intranasal). Its advantage is rapid onset and duration of action (2.5 hours half-life [t1/2]).
Lorazepam has a longer duration of action than midazolam (t1/2 10–15 hours) with an onset of approximately 3 minutes.
Diazepam may have a more rapid onset than lorazepam because of its ability to cross the blood–brain barrier (more lipophilic).
Other anticonvulsants: Ongoing therapeutic options can include phenytoin/fosphenytoin and phenobarbital. Phenobarbital may be a good option for seizure prophylaxis as it can be used in the SC route.7
SSVC syndrome is usually caused by SVC obstruction (most commonly extra luminal), intraluminal or invasive tumor, or thrombosis. The patient may present with an indolent or more acute symptom profile including dyspnea, facial edema, headache, cough, chest pain, or visual disturbance. There is often a positional element to the symptoms (i.e., sitting up).8
Physical examination may reveal facial plethora/cyanosis, proximal vein dilation, and edema to the arm.
Access to definitive diagnostics may be limited in resource-limited environments, thus the diagnosis may be clinical. Chest X-ray and POCUS may assist in clinical decision-making when CT is not available.
Definitive treatment options can be considered, such as stenting, radiotherapy, chemotherapy, and anticoagulation, if possible. Reasonable options for treating a patient with suspected SVC syndrome during a crisis include the following:9
• Elevation of the head of the bed
• Symptomatic treatment of dyspnea with low-dose opiates
• Steroid administration (e.g., dexamethasone). This may be sufficient therapy in some patients.
• Diuretics may help to decrease preload.
Dosing details for medications can be found in Chapter 13.
Malignant Spinal Cord Compression
A very commonly missed diagnosis in the face of cancer is malignant spinal cord compression (MSCC). It is a true palliative emergency because if it is not recognized promptly and treated urgently, it leads to significant morbidity, including permanent paralysis, sensory loss, and autonomic dysfunction as well as sphincter loss. Ability to ambulate prior to treatment is a positive prognostic sign, as there is an 80% chance of maintaining this function after treatment.10 Significant loss of motor function (including walking) before treatment predicts a more dismal rate of recover to walk (approximately 10%).
Commonly associated cancers include breast, lung, prostate, renal, and thyroid. The most common level of compression is the thoracic spine (70%), followed by lumbar and cervical. Multiple levels of compression occur about 30% of the time, thus imaging of the entire spine is strongly recommended.
Pain (dull, sharp, radicular) is the most common presenting feature and most often precedes any neurological deficit (although pain may be rarely absent). On examination, lesions found to be above the L1 level will often demonstrate upper motor neuron signs (hyperreflexia and motor weakness and increased tone), and those lesions below L1 more commonly show lower motor neuron signs (motor weakness, hyporeflexia, and decreased reflexes). Loss of bowel and bladder function is often a late finding and is a poor prognostic indicator.
If MSCC is considered, immediate treatment should be initiated in the form of steroids (e.g., dexamethasone—refer to Chapter 13 for dosing details). Unfortunately, the diagnostic gold standard is full spinal magnetic resonance imaging (MRI), which is likely not an option during a humanitarian crisis. Plain X-rays are thought not to be useful, though a CT myelogram can be considered if available.11
Definitive therapy includes radiotherapy and surgery, if possible. Optimally, MSCC would be an indication for transfer to a referral center.
Ethics of Medical Intervention During an Emergency
In the setting of a humanitarian crisis, the ability to respond to an emergency in a patient with a palliative trajectory may be limited. Often patients with a limited prognosis such as end-stage cancer or organ failure will be subject to triage bias in the face of competing interests (e.g., patients with a reversible medical crisis). Such patients are at risk of suffering in the context of an overwhelmed humanitarian response. The concept of triage in crisis is addressed in detail in Chapter 2.
A key priority would be to make suffering part of triage criteria so that these patients do not go without having their symptom needs addressed. Even if a patient is not expected to survive, quick attention to pain, dyspnea, confusion, nausea, and other distressing symptoms can be easily tackled with low technology options, such as the following:
• Morphine as needed for pain, dyspnea. If available, fentanyl provides rapid onset and is likely a better option for end-stage renal and liver disease, owing to lack of metabolite accumulation.
• Midazolam is often offered for end-stage agitation and delirium, seizures, and dyspnea. One can also utilize infusion for palliative sedation.
• Haldol may be used for agitation and delirium as well as nausea.
• Methotrimeprazine (also known as levomepromazine) is a phenothiazine neuroleptic that may be considered for treating nausea and hyperactive delirium, as well as having some analgesia qualities.
Please refer to Chapter 13 for medication dosing details.
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