Sickle Cell Disease

Subsets

Sickle Cell Trait:

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

Sickle Cell Disease:

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

Making the Diagnosis

Important points in history

Important points on examination

Investigations

Treatment (general)

Anaemia

Painful & vaso-occlusive crises

Analgesia - Regular Medicine, not PRN

( Pain Management )

Infections and Fever (functional asplenia from 6 months)

Others

Specific Management

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

Discharge & Long-term follow-up

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