🔴 Men's Health

Sickle Cell Disease — Adult Guide

Living well with sickle cell disease — what adults need to know.

Sickle cell disease (SCD) is a lifelong genetic blood disorder that affects millions of Nigerians — the country with the world's highest SCD burden. With proper management, regular medical care, and understanding of triggers, adults with SCD can live longer, healthier, and more fulfilling lives than previous generations.

Signs and Symptoms

✓ Acute painful crises — sudden, severe pain in the bones, chest, abdomen, or joints

✓ Chronic pain — even between crises, some adults experience persistent pain

✓ Fatigue and anaemia — haemoglobin is typically 6–9 g/dL

✓ Acute chest syndrome — chest pain, fever, breathlessness (a medical emergency)

✓ Strokes — particularly in younger patients

✓ Splenic sequestration (more in childhood) — sudden abdominal pain and anaemia

✓ Avascular necrosis — bone death, especially in the hip joint, from blocked blood supply

✓ Leg ulcers — chronic, painful ulcers on the lower leg

✓ Priapism in men — prolonged, painful erections (a medical emergency if lasting over 4 hours)

✓ Eye complications — retinopathy (damage to blood vessels in the eye)

✓ Kidney and lung complications with age

Risk Factors

HbSS genotype — most severe form
HbSC genotype — generally milder but still significant complications
HbS-Beta Thalassemia — variable severity
Common triggers for crises: dehydration, cold temperatures, stress, infections, alcohol, overexertion, high altitude, and certain medications

When to Seek Help

NowAcute chest syndrome (chest pain + fever + breathlessness) — this is life-threatening and requires immediate hospital care.

NowStroke symptoms — sudden weakness, face drooping, speech difficulty, confusion — call emergency services immediately.

NowPriapism lasting more than 2 hours in men — medical emergency, seek immediate help to prevent permanent damage.

NowSevere pain crisis not responding to home medications within 2 hours, or fever above 38.5°C.

RoutineRegular clinic reviews every 3–6 months — even when well. Prevents complications and adjusts treatment.

Tests & Diagnosis

🧪 Full Blood Count (FBC)

Baseline and regular monitoring of haemoglobin, white cells, platelets. Most adults with HbSS have Hb of 6–9 g/dL — any drop from personal baseline signals a complication.

🧪 HbS Quantification (HPLC)

Confirms genotype and monitors the proportion of sickle haemoglobin — important when assessing response to hydroxyurea.

🧪 Kidney Function (eGFR/Creatinine)

Annual. Renal complications are very common in adults with SCD — early detection allows slowing of progression.

🧪 Liver Function Tests + Hepatitis Screening

Annual. Liver is often affected by SCD (from iron overload and small infarcts) and from blood transfusions.

🧪 Echocardiogram (Heart Ultrasound)

Every 1–2 years. Pulmonary hypertension is common in adults with SCD and is associated with high mortality.

🧪 Eye Examination (Retinal)

Annually — sickle retinopathy progresses silently and can cause blindness if not treated early.

🧪 TCD (Transcranial Doppler) Ultrasound

In adults with prior stroke history — to assess cerebral blood flow and guide transfusion decisions.

Treatment Options

Hydroxyurea (Hydroxycarbamide)

The most important disease-modifying treatment. Reduces the frequency of painful crises by 50%, reduces acute chest syndrome and transfusions, and may extend life. Under-prescribed in Nigeria due to myths — it is safe and effective.

Folic Acid

5mg daily for all people with SCD — the high turnover of red blood cells requires extra folate to prevent megaloblastic anaemia.

Penicillin Prophylaxis (or Amoxicillin)

Lifelong for people without a functioning spleen — prevents life-threatening infections from encapsulated bacteria (pneumococcus, meningococcus). Often continued into adulthood if spleen function is lost.

Vaccination

People with SCD have reduced spleen function and are extremely vulnerable to certain infections. Pneumococcal, meningococcal, and Hib vaccines are essential.

Blood Transfusion

Used for acute complications (acute chest syndrome, stroke) and chronic high-risk situations. In Nigeria, blood safety and availability remain significant challenges — Mascot Healthcare facilitates access to safe, tested blood.

Pain Management

NSAIDs (ibuprofen), opioids (for severe crises), hydration, warmth, and rest. Develop a personalised pain plan with your doctor — having a written plan reduces unnecessary hospitalisations.

Frequently Asked Questions

Can I have a normal life with sickle cell disease?

With modern management — hydroxyurea, regular reviews, and prompt treatment of complications — many adults with SCD live into their 50s and beyond with good quality of life. The key is not to manage it alone.

Should I take hydroxyurea?

Most adults with HbSS or HbSC disease would benefit from hydroxyurea. It is the single most evidence-based treatment for reducing crises. Discuss with your haematologist — many of the fears around it are unfounded.

Can I exercise?

Yes — moderate, regular exercise is beneficial. Avoid extreme exertion, dehydration, and cold exposure. Always hydrate well before, during, and after exercise.

What should I do during a crisis at home?

Drink plenty of fluids (water, warm drinks), apply warmth to painful areas, take your prescribed pain medication regularly (not waiting until severe), and rest. If pain is uncontrolled after 2 hours or you develop fever, go to hospital.

What happens during pregnancy with sickle cell disease?

Pregnancy with SCD is higher risk and requires specialist obstetric and haematological care. With proper monitoring and management, many women with SCD have successful pregnancies. Pre-pregnancy counselling is strongly recommended.