Sickle Cell Disease

Subsets

HbSA (± 10% population) Heterozygocity for HbS (sickle cell trait): usually asymptomatic, lower parasitemia, higher Hb

HbSS (<1% population) Homozygocity for HbS or HbS+C/β: change in Hb structure, sickling if deoxygenated

There is a wide spectrum of severity
Suspect if patient is chronically anaemic and/or received a previous transfusion (>2 months of age)
Suspect in children who have suffered from one or more of the presentations or complications listed below
"Sickle cell test"
- sickledex or shaketest (>12 months) available at main lab
- Hb-electrophoresis: quantifies fraction of Hb-types. Often available at sickle clinic.

PCV and MPS
FBC + red cell morphology to look for characteristic sickle cells
Reticulocytes
Blood culture if febrile
Further tests guided by complaints (e.g. X-ray/ abdominal ultrasound/ CT-scan/ LP)

If shocked consider splenic sequestration (Give 10ml/ kg Normal Saline Shock protocol)
Suspect Parvovirus B19 infection if aplastic
Transfuse in acute chest syndrome or stroke

Oxygen: Oxygen stops the sickling of RBC's. All children presenting with a Sickle Cell Crisis should be started on Oxygen, even if saturations are normal
Fluids:
- Per 24 hrs give 1.5 x maintenance fluid IV
  e.g. 24kg
  1.5 x (10x 100ml + 10x 50ml + 4x 20ml)
  = 1.5 x 1580ml
  = 2370 ml/ 24 hours
- Encourage lots of oral hydration

Sepsis (usually S. pneumo): Ceftriaxone
Meningitis (usually S. pneumo): Ceftriaxone x 2 weeks
Acute Chest Syndrome: Ceftriaxone
Salmonella Osteomyelitis: Ceftriaxone (IV antibiotics for 2 weeks) then consider change to oral antibiotics (Ciprofloxacin). Aim for total duration of antibiotics 4-6 weeks
Always check for Malaria parasites

Cholelithiasis / Cholecystitis (>10yrs): AXR, abdominal ultrasound & surgical referral
Iron overload secondary to Transfusion (less if not transfused)
Stunted growth & Delayed maturation

	Age	Management	Differential
Hand-Foot Syndrome	0.5-2 yr	O2, fluids and analgesia. X-ray seldom necessary	Septic Arthritis
Bone infarct (Hips)	0.5-10yr	X-ray, Analgesia + Rest, Orthopaedic referral	Osteomyelitis (Salmonella)
Stroke/CNS complications	5-10 yr	MRI scan, treat seizures, physiotherapy referral	Cerebral Malaria / Abscess
Splenic Sequestration	3 yr	O2, treat shock, Ceftriaxone, transfuse
Acute Chest	0.5-10 yr	O2, fluids and analgesia, Cef, X-ray, transfusion	Pneumonia
Bowel infarct/ Abdominal Crisis		O2, Cef + Metro, Surgical review	Acute abdomen/ Cholethiasis
Priapism		Surgical review
Kidney infarct		Urinary output, electrolytes & BP	Nephritic Syndrome
Skin Ulcers		GV-paint/ Antibiotics	Kwashiorkor
Eye disorders	>10 yr	Ophthalmologists review

Send to sickle cell clinic.
In QECH it takes place Tuesdays at 13:30 in Paediatrics Under 5 for:

Malaria prophylaxis
- Sulphadoxine-Pyrimethamine (SP - Fansidar) once a month
  - < 2years: 1/4 tablet
  - 2 - 5 years: 1/2 tablet
  - 5 - 10 years: 1 tablet
  - 10 - 15 years: 2 tablets
  - >15 years: 3 tablets
- OR Chloroquine 5 mg/kg once a week
Folic Acid (1-5 mg daily)
Benzathine-Penicillin (>6 months): Monthly IM <30kg: 600 000 IU; >30kg: 1 200 000 IU
Pneumococcal vaccine (2 and 5 yrs) - if available
Consider Hydroxyurea for those with frequent painful crises (discuss with senior)

myelosuppressive agent - the only effective drug proven to
- reduces frequency of painful episodes
- raises level of HbF and haemoglobin level
usually decreases the rate of painful episodes by 50 %
also decreases the rate of acute chest syndrome episodes and blood transfusions by ~50 % (in adults)
Initial dose 10 - 15 mg/kg/day OD
Side effects includes neutropenia, bone marrow suppression, elevation of hepatic enzymes, anorexia, nausea, vomiting and infertility!