Sickle-cell Disease - Signs and Symptoms - Complications

Complications

Sickle-cell anaemia can lead to various complications, including:

• Overwhelming post-(auto)splenectomy infection (OPSI), which 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, which can result from a progressive narrowing of blood vessels, preventing oxygen from reaching the brain. Cerebral infarction occurs in children and cerebral haemorrhage in adults.
• Silent stroke is a stroke that causes no immediate symptoms but is associated with damage to the brain. Silent stroke is probably five times as common as symptomatic stroke. Approximately 10–15% of children with sickle cell disease suffer strokes, with silent strokes predominating in the younger patients.
• Cholelithiasis (gallstones) and cholecystitis, which may result from excessive bilirubin production and precipitation due to prolonged haemolysis.
• Avascular necrosis (aseptic bone necrosis) of the hip and other major joints, which may occur as a result of ischaemia.
• Decreased immune reactions due to hyposplenism (malfunctioning of the spleen).
• Priapism and infarction of the penis.
• Osteomyelitis (bacterial bone infection); the most common cause of osteomyelitis in sickle cell disease is Salmonella (especially the non-typical serotypes Salmonella typhimurium, Salmonella enteritidis, Salmonella choleraesuis and Salmonella paratyphi B), followed by Staphylococcus aureus and Gram-negative enteric bacilli perhaps because intravascular sickling of the bowel leads to patchy ischaemic infarction.
• Opioid tolerance, which 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 haemorrhages and retinal detachments, resulting in blindness. Regular annual eye checks are recommended.
• During pregnancy, intrauterine growth retardation, spontaneous abortion, and pre-eclampsia.
• Chronic pain: Even in the absence of acute vaso-occlusive pain, many patients have chronic pain that is not reported.
• Pulmonary hypertension (increased pressure on the pulmonary artery), leading to strain on the right ventricle and a risk of heart failure; typical symptoms are shortness of breath, decreased exercise tolerance and episodes of syncope.
• Chronic renal failure due to Sickle cell nephropathy—manifests itself with hypertension (high blood pressure), proteinuria (protein loss in the urine), haematuria (loss of red blood cells in urine) and worsened anaemia. If it progresses to end-stage renal failure, it carries a poor prognosis.