Sickle Cell Disease Guidelines

First visit

Every visit

Hydroxyurea

Infection

Thrombosis

Acute chest syndrome

Priapism

Indications for blood transfusion

Indications for referral

First Visit 

• Parental education about the disease 
• Most importantly about compliance and the recognition of early signs of infection 
• Screening siblings (Hb electrophoresis at 3/12) and parents 
• Medic alert bracelet. See websitewww.medicalert.co.za 

Every Visit 

• FBC and reticulocyte count 
• Pen VK 125mg bd from 8 weeks until 3 years of age. 
  • Increase to 250mg bd until 5 years of age. 
  • Can be stopped after 5 - 6 years 
  • Keep a penicillin ‘crash pack’ at home to be administered in the case of fever. 
  • Erythromycin 10mg/kg bd if penicillin allergic. 
• Folate 2,5 mg or 5 mg daily. Smaller doses may be required for neonates. 
• Immunization 
  • Routine immunisations (especially Hib and Hep B) should be followed as usual. 
  • Pneumovax [>2years old and every 5 years]. Pneumovax is not sufficiently immunogenic in children younger than two years. 
    • Schedule: 
    • Give conjugate vaccine initially, followed by the polysaccharide vaccine once the child is 2 years old. 
    • If < 2 years, give 3 doses of conjugate vaccine at monthly intervals, followed by 1 dose of polysaccharide vaccine at 2 years of age. 
    • If > 2 years, give 2 doses of conjugate vaccine at monthly intervals, followed by 1 dose of polysaccharide vaccine one month later. 
  • Influenza (annually in the autumn) 
• Growth and development (especially if on hydroxyurea). 
• Blood pressure monitoring (at risk for stroke). 
• Intellectual development and school performance. 
• Ophthalmological evaluation 
  • Start at school going age. 
  • Repeat every few years. 
• Regular dental care / good oral hygiene. 

Hydroxyurea 

• Indicated after several / recurrent major thrombotic episodes. Initiation of therapy should be discussed with a paediatric haematologist before starting. 
• Hydroxyurea increases Hb F in SS disease / reduces the proportion of dense cells / increases the MCV and MCH of sickle cells. 
• Main complications: Neutropaenia, teratogenesis and retarded growth and development. 
• Start at an oral dose of 15mg/kg. (500mg per capsule). May have to opt for alternate day doses in smaller children or even less than that (e.g. 500mg 2X per week). 
• Monitor Hb F levels. Aim for an Hb F level of 10%. 

Infection 

• Perform FBC, reticulocyte count, urine dipstix and cultures (urine/blood/throat). 
• CXR only if clinically indicated. 
• If the child is ‘toxic’ with a high fever (>38oC) admit and start IV Ceftriaxone 50mg/kg. Oral Augmentin is adequate if the child is not systemically unwell or febrile. For acute chest syndrome choose an antibiotic which will cover possible Mycoplasma infection i.e. erythromycin or clindamycin. 
• Treat blood culture positive patients for seven days with IV antibiotics. 
• Treat CSF positive cases for 10 days with IV antibiotics. 
• Children who are improving and are culture negative after 48 – 72 hours can be switched to an oral equivalent and can be sent home. 
• Suspected salmonella osteitis can be appropriately investigated with a bone scan. Any collection should be drained by an orthopaedic surgeon. 

Thrombosis 

• Hydrate with IV fluids at maintenance doses. 
• Cover empirically with antibiotics as above. 
• Continue oral folate. 
• Provide adequate analgesia. Start with an oral paracetamol/codeine combination every 6 hours. Change to IV opiates if pain not totally resolved on weaker analgesics. Most children will respond to oral analgesia and adequate hydration. 
• If pain persists or worsens try IV methylprednisolone 15mg/kg per day for two days. 

Acute chest syndrome (this may be due to pulmonary sequestration or pneumonia) 

• Admit to high care. 
• Vigilant monitoring. Blood gases may be required if hypoxic. 
• Hydrate and analgese as above. 
• Transfuse. 
• Try trial of bronchodilator therapy which may be very effective. 
• Administer O₂ if hypoxic. 
• Exchange transfusion may be necessary if hypoxia worsens, clinical deterioration continues despite packed cell transfusion or the haematocrit is high. 
• Cover possible Mycoplasma infection i.e. erythromycin or clindamycin. 

Priapism 

Home management: 

• Urinate often 
• Do some vigorous exercise 
• Increase fluid intake 
• Soak in a warm tub 
• If no result after all these measures the patient should come to hospital 

Hospital management 

• Hydrate, analgese, transfuse 
• If no result within a few hours consult a surgeon for shunting or corporeal irrigation 

Indications for blood transfusion 

• Splenic sequestration crisis 
• CNS infarction 
• Aplastic or haemolytic crisis 
• Preparation for surgery (aim for a Hb of 10g/dL) 
• Acute chest syndrome 

Indications for referral to a tertiary centre or d/w a haematologist 

• Acute chest syndrome 
• Priapism 
• CVA 
• Pulmonary hypertension or systemic hypertension 
• Commencement of hydroxyurea 
• Assessment for hypertransfusion programmes 
• Preparation for surgery or anaesthetic 
• Assessment for bone marrow transplant