Note:

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

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Acute Chest Syndrome/Acute Chest Crisis

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

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Diagnostic Criteria

• Pleuritic pain (worse with breathing)

• Respiratory distress.

• Fever

• Wheeze

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

Investigations

• 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
   

Treatment

Non pharmacological treatment

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• 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

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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

 

Note:

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

Refer to the next level facility with adequate expertise and facilities
 

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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.
   

Investigations:

• FBP

• Peripheral smear

• Reticulocyte count
   

Treatment
 

Non pharmacological treatment

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• 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.
   

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Hypersplenism

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.

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Diagnostic Criteria

 

• Enlargement of the spleen.

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

• Compensatory marrow hyperplasia.
   

Invesigations

• FBP

• Peripheral smear

• Reticulocyte count
   

Treatment

• Manage Cytopenia accordingly.

• Definitive treatment is splenectomy.
   

Note:

 

Indications for splenectomy are:

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

• At least 2 episodes of acute splenic sequestration crisis.

 

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Priapism

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

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Treatment

• Encourage patient to pass urine.

• Give IV fluids as above.

• Analgesia

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

Refer

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

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Neurological Manifestations
 

Stroke

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
   

Investigations

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

• Trans cranial Doppler Ultra Sound (TCD)

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Treatment

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
   

Note:
 

• Refer to next level facility with adequate expertise and facilities

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Last updated on 01.10.2020

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References

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

2. MSD manual. sickle cell disease. https://www.msdmanuals.com/professional/hematology-and-oncology/anemias-caused-by-hemolysis/sickle-cell-disease. Accessed October 1, 2020