Sickle Cell Disease - Infection management/ prophylaxis

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ICD10: D5

Management

Chronic Management

Treatment

General Measures

  • Regular follow up and education of patients and families (Family support)
  • Always keep well-hydrated
  • Give folic acid 5 mg daily for life
  • Promptly assess, and treat any fever with antibiotics until source of fever is identified
  • Ensure complete immunisation using the UNEPI programme, which includes the pneumococcal vaccine for all infants
    • Plus, if available, immunisation against meningococcus (to be given in regions within the meningococal belt) and influenza
  • Prophylactic penicillin V (up to 5 years of age)
    • Child 3 months-3 years: penicillin V 125 mg every 12 hours
    • Child 3-5 years: penicillin V 250 mg every 12 hours
  • Malaria prophylaxis with monthly sulphadoxine pirimetamine (SP)
    • Child 2-5 years: ½ tab monthly
    • Child 5-10 years: 1 tab monthly
    • Child 10-15 years: 2 tabs monthly
    • Child >15 years: 3 tablets monthly
      • For those with sulphur allergy consider use of erythromycin 250 mg every 12 hours

Management of Acute complications

Acute Chest syndrome

  • Restricted IV fluids use, always use calculated required amounts of IV fluids. NB: limit in cases of pulmonary oedema
  • Oxygen therapy
  • Pain management as described here
  • Salbutamol inhaler (2-4 puffs prn) or nebulisation 5 mg (2.5 mg for children <5 years)
  • Ceftriaxone 1-2 g once daily for 7-10 days
    • Child: 80-100 mg/kg once daily
  • Plus erythromicin 500 mg every 6 hours for 7-10 days
    • Child: 5-10 mg/kg every 6 hours
  • Transfuse if no improvement, and/or Hb falls <9 g/dL. Start incentive spirometry or blowing of a balloon early in acute chest syndrome.

Acute Abdomen/Mesenteric crisis

  • IV fluids
  • Nil by mouth 
  • NGT tube on free drainage
  • Antibiotics
  • Ceftriaxone 1-2 g once daily for 7-10 days
    • Child: 80 mg/kg once daily
  • Plus metronidazole 500 mg IV every 8 hours for 7-10 days
    • Child: 10 mg/kg IV every 8 hours
  • Red cell transfusion
  • Plain abdominal X-ray to rule out obstruction or stool impaction
  • Surgical review

Infections

  • Prompt assessment and treatment of cause (osteomyelitis, pneumonia, chronic leg ulcers, cellulitis, etc.)
  • Treat according to cause. If no localising focal symptoms, and no malaria, give:
  • Ceftriaxone 1-2 g once daily for 7-10 days
    • Child: 80 mg/kg once daily

If osteomyelitis or septic arthritis

  • Or Cloxacillin 500 mg 6 hourly IV or orally
    • Child: 50 mg/kg 6 hourly for at least 21 days
  • or Ciprofloxacin 500 mg 12 hourly for at least 21 days
    • In child: Ceftriaxone 50 mg/kg IV once a day for at least 21 days

Prevention/health education

  • Patient, family and community education
  • Timely initiation of hydroxyurea (see full Uganda Clinical Guidelines) 
  • Periodic comprehensive evaluations, and other disease- specific health maintenance services
  • Periodic evaluation for sickle cell complications for example urinalysis and renal function for sickle cell nephropathy, cardiac echo for pulmonary hypertension, transcranial doppler in children for early detection of stroke risk. Patient with these complications should be referred to a specialist
  • Timely and appropriate treatment of painful crisis and acute illness
  • Genetic counseling (for couples planning to have children)
  • Antenatal screening
  • Early recognition/screening of children with low Hb
  • Vaccination (pneumococcal vaccine, H-influenza vaccine, Hepatitis B vaccine evaluation)
  • Antibiotic (oral penicillin twice a day in > 5 years), and antimalarial chemoprophylaxis