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