complications
Last reviewed 01/2018
- 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)
- growth impairment becomes evident after 6 months of age and is usually
caused by
- 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)
- epilepsy
- 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 sickle lung disease
- 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)
- 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)
- proliferative retinopathy
- gallstones
- seen in over 50% of children with SCD
- usually asymptomatic or commonly presents with intermittent abdominal pain (3)
Reference:
- 1. Sickle Cell Society 2008. Standards for the clinical care of adults with sickle cell disease in the UK
- 2. Claster S, Vichinsky EP. Managing sickle cell disease. BMJ. 2003;327(7424):1151-5.
- 3. NHS Sickle Cell and Thalassaemia Screening Programme 2009. Sickle cell and Thalssaemia. Handbook for laboratories
acute haematogenous osteomyelitis