🩸 Men's Health

Sickle Cell Disease — Adult Management (Nigeria)

Living with SCD as an adult in Nigeria — managing crises, preventing complications, and optimising health

Nigeria has the highest number of people living with sickle cell disease (SCD) in the world — approximately 150,000 infants are born with SCD annually. Thanks to improving care, many more Nigerians with SCD are reaching adulthood. Adult SCD is distinct from childhood SCD — new complications emerge (leg ulcers, avascular necrosis, pulmonary hypertension, chronic pain), and the approach to pain management and prevention shifts. This guide focuses on the adult Nigerian patient with SCD.

Signs and Symptoms

✓ Vaso-occlusive crisis (pain crisis) — severe pain in bones (back, chest, limbs) from blocked blood flow

✓ Acute chest syndrome — chest pain, fever, falling oxygen levels — a life-threatening emergency

✓ Stroke — sudden weakness, speech difficulty — occurs in children and adults with SCD

✓ Priapism — painful persistent erection lasting >4 hours — urological emergency

✓ Leg ulcers — chronic, painful ulcers above the medial malleolus (inner ankle)

✓ Avascular necrosis — hip or shoulder pain from bone death due to ischaemia

✓ Chronic anaemia — haemoglobin typically 6–9 g/dL at steady state in HbSS

✓ Pulmonary hypertension — breathlessness on exertion, a serious complication in adults

Risk Factors

HbSS genotype — most severe. HbSC and HbS-beta-thalassaemia — milder phenotypes
Low fetal haemoglobin (HbF) level — high HbF is protective (explains hydroxycarbamide benefit)
High baseline white cell count — predicts more frequent crises
Dehydration — reduces blood oxygen saturation and triggers sickling
Cold temperatures — causes vasoconstriction and sickling
High altitude — low atmospheric oxygen
Infection — major trigger for crises and acute chest syndrome
Emotional stress

When to Seek Help

EmergencyAcute chest syndrome: chest pain + fever + falling oxygen saturations — call for help, give oxygen, go to hospital immediately

EmergencyStroke symptoms — sudden weakness, facial droop, speech difficulty in any SCD patient

EmergencyPriapism lasting >4 hours — urological emergency; give fluids, analgesia, and seek urgent urological drainage if not resolving

Same dayFever >38.5°C in a patient with SCD — sepsis risk is high; blood cultures and IV antibiotics should not be delayed

Same dayPain crisis not responding to analgesia at home after 2 hours

RoutineSix-monthly SCD clinic review — haematology assessment, complication screening, medication review

Tests & Diagnosis

🧪 Full blood count (FBC)

Baseline haemoglobin — know your steady-state Hb. A drop of ≥2 g/dL from baseline = significant — check for aplastic crisis (parvovirus B19), splenic sequestration, or haemolytic crisis

🧪 Reticulocyte count

Low in aplastic crisis (parvovirus B19). High in haemolytic crisis. Parvovirus IgM if aplastic crisis suspected

🧪 Blood film

Sickle cells (drepanocytes), target cells, Howell-Jolly bodies (functional asplenia)

🧪 Transcranial Doppler (TCD) ultrasound

Annual screening for stroke risk in children. Adults with prior stroke or symptoms need CT/MRI brain

🧪 Urine albumin:creatinine ratio + eGFR

Sickle cell nephropathy — very common in adults; progressive proteinuria leads to CKD. Annual screening essential

🧪 Echocardiogram

Pulmonary hypertension screening — tricuspid regurgitant velocity (TRV) ≥2.5 m/s requires right heart catheterisation to confirm

🧪 LFTs, hepatitis screen

Chronic liver disease from repeated transfusions and iron overload. Biliary stones (pigment) common — MRCP if suspected

🧪 Serum ferritin + MRI liver T2*

Iron overload monitoring in transfused patients — ferritin >1000 µg/L or liver MRI evidence of overload indicates chelation

Treatment Options

Hydroxycarbamide (hydroxyurea) — the most important disease-modifying therapy

Increases fetal haemoglobin (HbF), reduces sickling, decreases crisis frequency by 50%, reduces acute chest syndrome, and may reduce mortality. All adults with HbSS should be considered for hydroxycarbamide. Starting dose: 15 mg/kg/day. Increase by 5 mg/kg/day every 8 weeks (monitoring FBC) to maximum tolerated dose. Available in Nigeria — prescribable by haematologists at teaching hospitals

Analgesia for vaso-occlusive crisis

Mild-moderate: paracetamol + ibuprofen/diclofenac (NSAID) at home. Severe (hospital): IV morphine by patient-controlled analgesia (PCA) or regular IV morphine 0.1 mg/kg. Do not delay adequate analgesia — undertreated pain prolongs hospitalisation. Oral opioids for discharge. Avoid pethidine (norpethidine seizure risk)

Hydration

Oral fluids (2–3 litres daily) at steady state. IV normal saline or 5% dextrose in crisis. Avoid overhydration in acute chest syndrome (pulmonary oedema risk)

Penicillin prophylaxis

Phenoxymethylpenicillin 500 mg twice daily lifelong for HbSS (functional asplenia — high infection risk). If penicillin allergic: erythromycin. Pneumococcal, meningococcal, Hib, and influenza vaccinations essential

Blood transfusion

Top-up transfusion for symptomatic anaemia (Hb <5–6 g/dL or drop >2 g/dL from baseline). Exchange transfusion for stroke, severe acute chest syndrome. Chronic transfusion programme (monthly exchange) for primary/secondary stroke prevention. Use matched blood — anti-E, anti-C, Kell negative — to prevent alloimmunisation

Acute chest syndrome management

Oxygen to maintain SaO2 >95%. Incentive spirometry. IV antibiotics (cover atypicals — azithromycin + ceftriaxone). Bronchodilators. Blood transfusion if Hb falls or oxygen requirement increases. Exchange transfusion if deteriorating. ICU if rapid deterioration

Avascular necrosis (AVN)

Physiotherapy for pain and function. Core decompression (surgical drilling) in early disease. Hip or shoulder arthroplasty for advanced AVN — available at Nigerian orthopaedic centres

Leg ulcers

Wound dressings, debridement, zinc sulphate supplements, compression bandaging (with caution in SCD — risk of ischaemia). Hydroxycarbamide helps some patients. Skin grafting for refractory ulcers

Pulmonary hypertension

If confirmed by right heart catheterisation: sildenafil (PDE5 inhibitor) or bosentan (endothelin antagonist). Hydroxycarbamide and chronic transfusion also help. Refer to specialist cardiology

Pregnancy in SCD

High-risk pregnancy — manage jointly with haematologist and obstetrician. Hydroxycarbamide must be stopped before conception (teratogenic). Folic acid 5 mg daily. Prophylactic low-molecular-weight heparin. Transfusion threshold lower in pregnancy. Partner testing for sickle trait essential

Frequently Asked Questions

Can adults with SCD live a full life?

Yes. With hydroxycarbamide, modern crisis management, and regular specialist follow-up, life expectancy in SCD has improved dramatically. Many Nigerians with SCD work, marry, have children, and live active lives. Consistent adherence to hydroxycarbamide and regular clinic attendance are the most impactful things a patient can do.

Should I take hydroxycarbamide?

If you have HbSS or HbS-beta0-thalassaemia with frequent pain crises (2+ per year), acute chest syndrome, severe anaemia, or other complications — yes, you should discuss hydroxycarbamide with your haematologist. It is the single most effective treatment for SCD. It is not chemotherapy, despite appearances — it is a disease-modifying drug.

Is it safe to have children if I have SCD?

Yes, with planning and specialist care. Before pregnancy: stop hydroxycarbamide (at least 3 months before trying to conceive). Test your partner for sickle trait. If both partners carry the sickle gene, there is a 25% chance each pregnancy has SCD. Preimplantation genetic diagnosis (PGD) is available for families who want to avoid affected pregnancies.

Why do I keep getting infections?

SCD causes functional asplenia (the spleen becomes non-functional from recurrent infarctions). The spleen is important for fighting bacteria with polysaccharide capsules (Pneumococcus, Meningococcus, Haemophilus). Without a functioning spleen, you are at high risk of overwhelming sepsis from these organisms — which is why penicillin prophylaxis and vaccinations are lifelong requirements, not optional.