complications

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  • chronic sickle pain
    • is defined as an episode of pain of greater than 3-6 months (1)
    • often caused by orthopaedic conditions such as avascular necrosis, vertebral collapse, or chronic arthritis (2)

  • bones and joints
    • vaso occlusive episodes resulting in infarcts are often seen in bones and joints
    • "fish mouthing" (abnormalities of the vertebrae) are characteristic of SCD
    • growth disturbances and osteopenia may occur due to hyperplasia of the bone marrow
    • avascular necrosis of hip and shoulder causes chronic pain (particularly in adults)
    • SCD patients also are at an increased risk of infection and failure of prosthesis
    • osteomyelitis is also more common (may be difficult to differentiate from infarction) (2)

  • impaired nutrition and growth
    • growth impairment becomes evident after 6 months of age and is usually caused by
      • decreased absorption of nutrients and/or
      • an increase in the metabolic rate
      • poor appetite associated with febrile or painful episodes
    • a delay in puberty may be seen - by about 6 months in HbSC patients and by 2-3 years in HbSS patients
    • pituitary and/or primary gonadal deficiencies may be seen in patients who are on long-term transfusion programmes (due to iron overload) (3)

  • neurological conditions
    • epilepsy
      • seizures occur in 10-15% of patients with SCD (10 times the incidence of general population)
      • associated with cerebrovascular disease and silent infarction o chronic headaches
      • common in SCD patients
      • may be secondary to migraine, benign intracranial hypertension, hypertension, sleep apnoea or tension (1)
    • cognitive impairment
      • mild cognitive impairment may be seen in children with silent infarcts (seen on MRI scan) (3)
      • in adults evidence of cognitive abnormalities is thought to be due to covert infarctions (1)

  • chronic lung disease
    • chronic sickle lung disease
      • may be associated with a history of acute chest syndrome or with low level pulmonary damage which occurs during painful episodes (1)
      • divided according to the presence of chest pain, degree of hypoxia, chest X-ray and lung function test findings (1)
    • obstructive sleep apnoea
      • common in SCD and may be caused by tonsillar hypertrophy or other causes of sleep disordered breathing
      • increased painful episodes and increased neurological events can also be associated with overnight hypoxia
    • pulmonary hypertension
      • one third of adults with SCD will develop pulmonary hypertension (2)
      • is thought to be due to chronic haemolysis which releases free haemoglobin, causing a deficiency of nitric oxide. This in turn leads to acute and chronic pulmonary vasoconstriction (1)
      • both chronic sickle lung disease and pulmonary hypertension are predominantly seen in adulthood although it is increasingly being recognised in older children and adolescents (3)

  • chronic leg ulcers
    • relatively uncommon in children, commonly seen during adolescence, prevalence increases with age
    • almost all ulcers are seen in the ankle region (near the malleolous) and are bilateral.
    • can be painless or very painful (3)
    • recurrence is high (1)
    • HbSS genotype is more likely to develop leg ulcers

  • chronic renal disease
    • renal complications are common specially with increasing age
    • renal failure primarily due to SCD is rare in childhood (3)
    • sickle nephropathy
      • presentation may range from painless haematuria, proteinuria and progressive loss of function to end stage renal disease (ESRD)
    • renal medullary carcinoma
      • occurs in patients with sickle cell trait and young patients with sickle cell anaemia (2)

  • priapism
    • very common and 89% of males with sickle cell anaemia will have had at least one episode of priapism by adulthood
    • obstruction of the venous drainage of the penis by vaso cocclusion leads to painful, persistent erection (2)
    • can present as an "acute" attack (which requires hospital admission) or a self limiting "stuttering" episode (1)

  • eye complications
    • proliferative retinopathy
      • accounts for around 73% of sudden visual loss in SCD patients
      • caused by recurrent microvascular occlusion which leads to ischemia and growth of new blood vessels
      • can lead to vitreous haemorrhage and retinal detachment
      • commonly seen in young adults between the ages 15-29 years
    • non proliferative retinopathy (1)

  • gallstones
    • seen in over 50% of children with SCD
    • usually asymptomatic or commonly presents with intermittent abdominal pain (3)

Reference:

Last reviewed 01/2018

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