Sickle-cell disease natural history, complications, and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aarti Narayan, M.B.B.S [2]
Overview
Natural History
Complications
Sickle-cell anemia can lead to various complications, including:
- Vaso-occlusive crisis (otherwise known as painful crisis): Most patients with sickle-cell disease have periodic intensely painful episodes called vaso-occlusive crises. The frequency, severity, and duration of these crises vary tremendously. Painful crises are treated with hydration and analgesics; pain management requires opioid administration at regular intervals until the crisis has settled. For milder crises a subgroup of patients manage on NSAIDs (such as diclofenac or naproxen). For more severe crises most patients require inpatient management for intravenous opioids; patient-controlled analgesia (PCA) devices are commonly used in this setting. Diphenhydramine is effective for the itching associated with the opioid use. Incentive spirometry, a technique to encourage deep breathing to minimise the development of atelectasis, is recommended.
- Acute chest syndrome is a life-threatening condition characterised by chest pain, shortness of breath, fever, hypoxaemia and pulmonary infiltrates on chest X-ray. It can be triggered by pain crisis, respiratory infection, bone-marrow embolization, or possibly by atelectasis, such as can be caused by opiate administration, or surgery.
- Overwhelming post-(auto)splenectomy infection is due to functional asplenia, caused by encapsulated organisms such as Streptococcus pneumoniae and Haemophilus influenzae. Daily penicillin prophylaxis is the most commonly used treatment during childhood with some haematologists continuing treatment indefinitely. Patients benefit today from routine vaccination for H. influenzae, S. pneumoniae and Neisseria meningitidis.
- Stroke can result from a progressive vascular narrowing of blood vessels, preventing oxygen from reaching the brain. Cerebral infarction occurs in children, and cerebral hemorrhage in adults.
- Cholelithiasis and cholecystitis (gallstones) may result from excessive bilirubin production and precipitation due to prolonged haemolysis.
- Avascular necrosis (aseptic bone necrosis) of the hip may occur as a result of ischemia.
- Decreased immune reactions due to hyposplenism (malfunctioning of the spleen)
- Priapism and infarction of the penis.
- Osteomyelitis (bacterial bone infection) - Salmonella is noted much more commonly than in the general population, although Staphylococcus is still the most common.
- Opioid tolerance can occur as a normal, physiologic response to the therapeutic use of opiates. Addiction to opiates occurs no more commonly among individuals with sickle cell disease than among other individuals treated with opiates for other reasons.
- Acute papillary necrosis in the kidneys.
- Leg ulcers
- In eyes, background retinopathy, proliferative retinopathy, vitreous hemorrhages and retinal detachments can occur. Regular annual eye checks are required.
- During pregnancy, intrauterine growth retardation, spontaneous abortion and pre-eclampsia are the possibilities.