Management of chronic complications of sickle cell disease

• chronic pain
  • often caused by orthopaedic conditions such as avascular necrosis, vertebral collapse, or chronic arthritis (1)
  • chronic pain is difficult to eradicate and the goal should be to minimise the suffering as much as possible
    • medium- to long-acting opiates (orally or transdermally) should be used
    • prescribe an alternative analgesia for "breakthrough" pain
    • if NSAID's are used, should be limited to standard doses and defined periods and if given for longer periods, monitor renal function every 3 months (2)
    • non pharmacological therapies such as massage, psychological coping strategies, including distraction techniques should be encouraged in children (3)
  • yearly (or more frequently in special cases) assessment, medication review, and health education should be provided to the patient
    • career/vocational and employment advice
    • occupational, psychological therapy (2)
    • inform about the importance of avoiding factors that may exacerbate pain e.g. - cold weather, high-impact exercise or sports, weight management and dehydration (2,3)
      
      
• nutrition and growth
  • height and weight should be measured at each visit
  • if the child is hospitalized for frequent and long periods, consider referral to a dietician for extra calorie input
  • zinc supplementation in case of growth retardation and vitamin D deficiencies should be treated (3)
  • folic acid supplementation is recommended for all patients with SCD (although it may mask megaloblastic anaemia caused by cobalamin deficiency) (2)
  • refer children with no physical signs of puberty at 14 years in a girl and 14.5 years in a boy to a paediatric endocrinologist
    
    
• nocturnal enuresis
  • inform the parents that nocturnal enuresis is common in SCD (specially in boys with HbSS) and most will resolve spontaneously
  • advice on techniques to achieve continence e.g - intermittent alarms and parental waking to achieve continence
  • if children do not respond to routine advice, consider oral or nasal desmopressin
  • refer to
    • an ENT specialist if the history is suggestive of obstructive apnoea and snoring
    • a specialist management (e.g. an enuresis clinic) if there is no response to basic measures after the age of 7 years (3)
      
      
• cerebrovascular disease
  • all children with SCD aged over 2 years should undergo annual transcranial Doppler imaging (3)
  • parents/carers should be educated about symptoms and signs of stroke and what action should be taken if a child develops neurological symptoms (3)
  • a first episode of acute severe headache, or a significant change in the type of headache may indicate intracranial haemorrhage or venous sinus thrombosis and these patients should be admitted for urgent assessment (2)
  • the mean blood pressure in SCD patients is lower when compared to age and sex matched controls hence more aggressive management of systemic hypertension is required on detection (2)
  • regular blood transfusion should be offered throughout childhood for secondary prevention of stroke (3)
    
    
• eye complications
  • prevention of developing complications is the most effective therapy
  • all SCD patients must be evaluated by an ophthalmologist annually
  • patients with acute loss of vision (due to occlusion of the central retinal artery) should be treated with immediate blood transfusion and referral to an ophthalmologist
    
    
• leg ulcers
  • should be managed by a multidisciplinary team with expertise in leg ulcer management
  • could be treated with frequent dressing, support bandages, physiotherapy and antibiotics if infected (Topical antibiotics should be avoided)(3)
  • blood transfusion could be considered in patients who fail to respond (2)
    
    
• priapism
  • boys and their parents/carers should be educated that priapism is a complication of SCD and to seek treatment early and should attend hospital as an emergency if priapism persists for more than 2 hours
  • for minor events (occurring for less than 3 hours) - bladder emptying, exercise such as jogging, warm baths and analgesia may be useful
  • for stuttering priapism oral etilefrine may be beneficial(3)
    
    
• chronic lung disease
  • in patients with chronic lung disease advice about smoking cessation, vaccinations (should be kept up to date), avoiding, or getting early treatment for chest infections (2)
  • Trans-thoracic echocardiography should be done to screen pulmonary hypertension every 1-2 years (2)
  • refer patients with suspected pulmonary hypertension to a specialist
    
    
• liver disease
  • an ultrasound of liver and biliary tree should be carried out in patients with recurrent abdominal pain
  • in symptomatic biliary disease, consider elective cholecystectomy

Reference:

• 1. Claster S, Vichinsky EP. Managing sickle cell disease. BMJ. 2003;327(7424):1151-5.
• 2. Sickle Cell Society 2008. Standards for the clinical care of adults with sickle cell disease in the UK
• 3. NHS Sickle Cell and Thalassaemia Screening Programme 2009. Sickle cell and Thalssaemia. Handbook for laboratories