Sickle Cell Disease - Infection management/ prophylaxis

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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 above
  • 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
  • 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, transcra-nial doppler in children for early detection of stroke risk. Patient with these complications should be referred to a specialist
  • Timely and appropriate treatment of 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), and antimalarial chemoprophylaxis