🔴 Men's Health

Sickle Cell Disease — Adult Guide Nigeria

Q: My partner is AS — should we worry?

If you are SS and your partner is AS, every pregnancy has a 50% chance of producing an SS child. Prenatal diagnosis (chorionic villus sampling at 11–14 weeks) can identify the fetal genotype. Pre-implantation genetic diagnosis with IVF allows selection of unaffected embryos — available at a small number of Nigerian centres.

Q: Is hydroxyurea safe long-term?

Yes — over 30 years of data in SCD patients shows no increased cancer risk and significant benefit. The main concern is teratogenicity — women on hydroxyurea must use effective contraception. Stop 3 months before attempting conception.

Q: Can I exercise with SCD?

Yes, moderately. Avoid extreme exertion, dehydration, or cold environments. Swimming (heated pool), walking, and cycling are generally safe. Competitive sports that involve prolonged intense exertion carry higher crisis risk — discuss with your haematologist.

Q: What genotype should my partner be?

If you are SS (sickle cell disease), a partner who is AA (normal) means all children will be AS (trait, not disease). A partner who is AS gives each child a 50% chance of SS. Genetic counselling before having children is strongly recommended.

Living with sickle cell as an adult in Nigeria

Sickle cell disease (SCD) is the most common severe inherited blood disorder in Nigeria — the country with the highest burden in the world, with approximately 150,000 affected births per year. While much focus is on children with SCD, adults face unique challenges: recurrent pain crises, organ damage accumulated over years, complications of long-term hydroxyurea or transfusion therapy, reproductive decisions, and the psychological burden of a lifelong condition. With comprehensive care, people with SCD in Nigeria can live into their 40s, 50s, and beyond.

Signs and Symptoms

✓ Pain crises (vaso-occlusive crises — VOC): severe pain in bones, chest, abdomen, or joints lasting hours to days

✓ Acute chest syndrome: chest pain + fever + breathlessness — a medical emergency

✓ Stroke: sudden weakness, speech difficulty, facial droop — SCD increases stroke risk 300x

✓ Avascular necrosis: progressive hip or shoulder joint pain from bone death

✓ Leg ulcers: non-healing sores above the ankle — particularly common in adults

✓ Priapism (males): prolonged, painful erection not related to sexual arousal

✓ Splenic sequestration: sudden left-sided abdominal pain and pallor (more common in children)

✓ Chronic pain: persistent pain between crises in many adults

✓ Pulmonary hypertension: breathlessness on exertion from high blood pressure in lung vessels

Risk Factors

Genotype HbSS: most severe form — 'sickle cell anaemia'
Genotype HbSC: milder but still significant complications
Genotype HbSβ thalassaemia: variable severity
Dehydration: triggers sickling — inadequate fluid intake is the most common precipitant
Cold temperatures, stress, infection, or extreme exertion
High altitude travel or unpressurised aircraft
Alcohol and smoking — worsen vasculopathy
Pregnancy — high-risk; needs specialist obstetric haematology care

When to Seek Help

Fever above 38°CPeople with SCD and spleen dysfunction are at high risk of overwhelming bacterial sepsis — fever is always an emergency. Go immediately

Acute chest syndrome signsNew chest pain, breathing difficulty, falling oxygen saturation — ICU-level emergency requiring exchange transfusion

Signs of strokeFace drooping, arm weakness, speech difficulty — call for help immediately. Time to treatment is critical

Priapism lasting more than 2 hoursProlonged priapism causes permanent erectile dysfunction — needs emergency urological treatment

Pain crisis not controlled with home medicationsSevere pain unresponsive to oral analgesia needs IV opioids and fluids in hospital

Severe anaemia: pallor, extreme fatigue, rapid heartbeatHaemoglobin may be critically low — needs blood transfusion

Tests & Diagnosis

🧪 Full blood count

Baseline haemoglobin typically 60–90 g/L in HbSS. Reticulocytes elevated. Regular monitoring for aplastic crises

🧪 Haemoglobin electrophoresis or HPLC

Confirms SCD genotype — essential for genetic counselling and diagnosis

🧪 HbF level

Fetal haemoglobin percentage — higher HbF is protective. Hydroxyurea works by raising HbF

Ultrasound of brain vessels — screening for stroke risk in children (and some adults). High velocity = high stroke risk

🧪 Echocardiogram

Screens for pulmonary hypertension and cardiac complications — recommended every 1–3 years in adults

🧪 Renal function

Chronic kidney disease is common in adult SCD — annual creatinine and urine albumin

🧪 Liver and iron studies

Transfusion-related iron overload — serum ferritin and liver MRI if regularly transfused

🧪 Retinal examination

Proliferative sickle retinopathy causes blindness — annual ophthalmology review in SC and Sβ+ genotypes

Treatment Options

Hydroxyurea — the most important treatment

Reduces pain crises, acute chest syndrome, and transfusion needs by raising HbF. 15–35mg/kg/day orally. Significantly under-prescribed in Nigeria. Requires monitoring for bone marrow suppression (monthly FBC initially)

Pain crisis management

Oral: ibuprofen + paracetamol + tramadol or codeine. Severe: IV morphine, IV fluids (2–2.5L/day), oxygen if SpO2 < 95%. Avoid pethidine (meperidine)

Blood transfusion

For severe anaemia, acute chest syndrome, stroke, or pre-operative preparation. Top-up or exchange transfusion depending on indication

Folic acid

5mg daily — all adults with SCD. Increased red cell turnover increases folate demand

Penicillin prophylaxis

V 250mg twice daily — recommended until adulthood due to functional asplenia. Many Nigerian centres continue indefinitely

Malaria prevention

Proguanil or chloroquine prophylaxis — malaria triggers crises and is more severe in SCD. Use insecticide-treated nets

Stem cell transplant

Curative — but requires a matched sibling donor and specialised centre. Available at UCH Ibadan and LUTH. Suitable for severely affected patients with a matched donor

Gene therapy

Emerging curative option — not yet available in Nigeria but actively being studied

Frequently Asked Questions

My partner is AS — should we worry?

If you are SS and your partner is AS, every pregnancy has a 50% chance of producing an SS child. Prenatal diagnosis (chorionic villus sampling at 11–14 weeks) can identify the fetal genotype. Pre-implantation genetic diagnosis with IVF allows selection of unaffected embryos — available at a small number of Nigerian centres.

Is hydroxyurea safe long-term?