top of page


Sickle cell Disease in Children

Sickle Cell Disease (SCD) refers to a group of disorders that are characterized by the presence of sickle haemoglobin (Haemaoglobinopathies). The commonest SCD syndrome is Sickle Cell Anaemia (SCA).

Diagnostic Criteria

  • Pallor

  • Jaundice

  • Painful swelling of hands and feet (dactylitis)

  • Bossing of the skull

  • Splenomegaly

  • Recurrent infections

  • With evidence of Hb SS on Hb Electrophoresis

Note: Patients are symptomatic from 3 months


  • FBP

  • Peripheral smear

  • Reticulocyte count

  • Sickling test and Hb Electrophoresis.


For children with sickle cell disease

  • Give booster doses of Pneumococcal polysaccharide vaccine at two years and five yearly thereafter, for life.

  • Give Folic acid, Mebendazole and Penicillin V as shown in the table below

Folic Acid, Mebendazole and Penicillin V Dosage


Management of specific conditions


Mild pain:

  • Reassure, reposition for comfort, massage, distraction (stories, play) AND

  • Paracetamol PO 15mg/kg 6 hourly

Moderate pain: ADD

  • Ibuprofen PO 5mg/kg 8 hourly OR

  • Diclofenac IM or PO 1mg/kg 8hourly

Severe pain: ADD

  • Liquid morphine PO 0.5mg/kg (max 20 mg) 3 hourly OR

  • Pethidine IM or IV 1mg/kg 8 hourly

  • Reassess after 1hour and give further analgesia if needed


  • Oral fluids should be encouraged.

  • For IV infusion: Recommended IV fluids are DNS or half strength NaCl 0.45%. If not available use NS, RL as shown in the table below.

Fluid requirement in 24hrs



  • Divide total daily volume by 24 to obtain hourly rate.

Acute Chest Syndrome/Acute Chest Crisis

It is a complication of SCA resulting from vaso occlusive crises of pulmonary vasculature.

Diagnostic Criteria

  • Pleuritic pain (worse with breathing)

  • Respiratory distress.

  • Fever

  • Wheeze

  • With evidence of new pulmonary infiltrates on chest x-ray


  • Blood grouping and cross match

  • ABG analysis if SPO2<90% in room air

  • Chest X-ray

  • FBP

  • Serum Electrolytes

  • Serum Creatinine and BUN

  • Liver Function Tests


Non pharmacological treatment

  • Admit the patient

  • Give oxygen 2L/min to maintain SPO2>95%

  • Manage pain (see dose above)

  • Give fluids as indicated above

  • If SPO2<95% Give top-up transfusion to achieve Hb 10g/dL and HbS<70%

  • If SPO2<90% Give exchange transfusion to achieve Hb 10g/dl and HbS<30%

  • Do HPLC to monitor HbS concentration

Pharmacological treatment


Give antibiotics IV as in severe pneumonia (refer to section on severe pneumonia)

  • If above 5 yrs add

    • Erythromycin 12.5mg/kg 6 hourly OR

    • Azithromycin 10 mg/kg once daily



Diuretics are contraindicated even if signs and CXR may mimic pulmonary oedema.

Refer to the next level facility with adequate expertise and facilities

Sequestration Syndromes

Splenic Sequestration is a sudden onset of splenomegaly or enlargement of a pre-existing splenomegaly commonly in under-fives. Hepatic sequestration can also occur, usually in children over 4 years old. All these conditions can lead to hypovolaemic shock.

Diagnostic Criteria


  • Acute anaemia (fall of Hb ≥2g/dL from the steady state).

  • Premature cells (NRBC) on film, thrombocytopenia of varying degree.

  • High or normal reticulocyte count.

  • Bone marrow hyperplasia.

  • Regression of splenomegaly after transfusion.


  • FBP

  • Peripheral smear

  • Reticulocyte count


Non pharmacological treatment

  • Give high flow oxygen 2L/min to keep SPO2>95%.

  • Give 20ml/kg bolus 0.9% NS while waiting for blood.

  • Give Top-up transfusion to steady state Hb immediately (within 3 hours of admission)

  • Give fluids orally / IV (see above).

  • Provide pain relief (Refer to pain management section).


Pharmacological treatment

  • Give Ampicillin IV 50mg/kg 6 hourly for 5 days AND

  • Gentamicin IV 7.5mg once daily for 5 days.


This is a hyperactive splenic disorder which leads to premature destruction of blood cells and progressive enlargement of the spleen from vascular congestion, phagocytic hyperplasia, and cellular infiltration.

Diagnostic Criteria


  • Enlargement of the spleen.

  • Cytopenia: Reduction in more than one cell lines in the peripheral blood.

  • Compensatory marrow hyperplasia.


  • FBP

  • Peripheral smear

  • Reticulocyte count


  • Manage Cytopenia accordingly.

  • Definitive treatment is splenectomy.



Indications for splenectomy are:

  • Severe Hypersplenism: Splenomegaly>10cm and Neutrophils <0.5x109/L, Hb <5g/L, Platelet <50x109/L

  • At least 2 episodes of acute splenic sequestration crisis.



Defined as the persistent painful unintentional erection. If left unattended can lead to infarction subsequent penile amputation

Diagnostic Criteria

  • Prolonged and persistent painful penile erection more than 4 hours

  • Not associated with sexual intention, interest or stimulation


  • Encourage patient to pass urine.

  • Give IV fluids as above.

  • Analgesia

  • Aspiration of retained blood and irrigation of the corpus cavernosum.


Refer to the next level facility with adequate expertise and facilities if the condition is not resolved within 2 hours of initiation of treatment.

Neurological Manifestations


These are vaso occlusive events which occur in the major arteries supplying the brain leading to ischaemic or haemorrhagic infarcts.

Diagnostic Criteria

  • Unilateral weakness

  • Seizures

  • Other focal neurological deficits


  • Neuroimaging (Preferably MRI/MRA, if not available Cranial CT),

  • Trans cranial Doppler Ultra Sound (TCD)


Non pharmacological treatment

  • Support Airway, Breathing and Circulation.

  • Give oxygen 2L/minute.

  • Give IV fluids as shown above.

  • Give 5mls/kg of 10% Dextrose if RBG if <2.5 mmlo/l and maintain normoglycaemia

  • Provide Physiotherapy & occupational therapy.

Pharmacological treatment

  • Give Paracetamol PO 15mg/kg if temperature is > 38.5°C

  • Give Phenobarbitone PO 5mg/kg/day for 1 month if the patient has a seizure

  • Give IV antibiotics, if meningitis is suspected. (Refer to the section on meningitis)

  • Give Hydroxyurea PO 15mg/kg once a day for life to prevent further infarcts


  • Refer to next level facility with adequate expertise and facilities

Last updated on 01.10.2020

Go back to main menu

Go back to previous session

Go to next session


  1. ​Tanzanian Standard treatment guideline for children 2017 edition page 114-119

  2. MSD manual. sickle cell disease. Accessed October 1, 2020

Neurological manifestations
bottom of page