Sickle Cell Anaemia

Management:

Medicine	Adult Dose	Frequency	Duration
folic acid po	5mg	once a day	for life
penicillin V po	250mg	once a day	for life
For malaria prophylaxis in endemic areas:
pyrimethamine/ dapsone po	One Tablet 12.5mg/100mg	once a week	continual

In painful crisis, intravenous rehydration, regular and adequate analgesia, oxygen by mask where available and possibly in some cases antibiotics are required.

Morphine is necessary to control severe pain. Weaker opiates (codeine) or non-steroidal anti-inflammatory medicines (e.g. aspirin) may be used for less severe pain. See chapter on Pain in main Zimbabwe STG.

Antibiotic therapy

Medicine	Adult Dose	Frequency	Duration
amoxicillin po	500mg	3 times a day	5 days

Other antibiotics may be required according to site of infection/causative organism.

Osteomyelitis: see section on Septic Arthritis and Osteomyelitis

Other types of crises:

In aplastic and haemolytic crisis, red cell transfusion may be required to treat anaemic heart failure. Sequestration or splenic pooling requires splenic massage and less often, blood transfusion.

Note that over transfusion worsens the sickling crisis and may cause iron overload. Special precautions must be taken during anaesthetizing sickle cell disease patients. Adequate hydration and oxygen exposure are essential to avoid red cell sickling.

Avoid debridement of leg ulcers as these are due to poor circulation rather than mere dead tissue.

Treat priapism conservatively with hydration and analgesia before resorting to surgery.

Patients with frequent crises need to be started on hydroxy urea where possible, refer to provincial or central hospital. The objective is to increase the HbF to at least 20%, levels which do not lead to haemolysis.

Medicine	Adult Dose	Frequency	Duration
hydroxyurea po	500mg	once a day	indefinitely

Note: Use hydroxyurea 500 mgs daily indefinitely , titrate against HbF. Monitor white cell and platelets counts.

Sickle cell trait requires no treatment, and does not cause anaemia.