Other manifestations of sickle cell disease besides bone pain crisis

By Dr. Chinonso Anyanwu-Yeiya

When most people think of sickle cell disease (SCD), they picture the intense bone pain crises. But do you know that SCD affects the body in many other serious ways? 

Here’s a closer look at how this complex condition manifests beyond bone pain.

Why Sickle Cells Cause Damage

Sickle cell disease is caused by an unusual type of hemoglobin— the protein in red blood cells responsible for carrying oxygen. This faulty hemoglobin easily loses oxygen, causing the red blood cells to change shape, become rigid, and stick to each other. This creates blockages that can obstruct blood flow and reduce oxygen supply, leading to intense swelling, heat, and pain. But the effects don’t stop there.

Other Major Complications of Sickle Cell Disease

1. Chronic Leg Ulcers

   • Painful, slow-healing wounds, often around the ankles, can result from blocked blood vessels and low oxygen in the legs. Treatment can include elevating the affected foot, antibiotics, and sometimes skin grafting for recovery.

2. Priapism

   • For men with SCD, priapism— a painful, prolonged, and unintended erection—can be an extremely uncomfortable and distressing experience.

3. Kidney Problems

   • Many children with SCD experience bedwetting due to kidney complications, as the kidneys struggle to retain fluids and filter blood properly. Recurrent obstructions can worsen kidney function over time.

4. Abdominal Crisis

   • This painful complication affects the abdomen, causing severe pain, fever, swelling, and sometimes constipation, which results from obstructed blood flow and oxygen deprivation in the abdominal area.

5. Gallstones

   • Rapid breakdown of red blood cells produces waste that can accumulate as stones in the gallbladder, leading to severe abdominal pain and jaundice when blockages occur.

6. Sequestration Crisis

   • In young children, blood can pool in the spleen or liver, causing rapid enlargement of these organs and a dangerous drop in blood levels. This condition requires immediate medical attention as it is life-threatening.

7. Acute Chest Syndrome

   • Doctors are particularly wary of this complication, as it presents with chest pain, cough, fever, and breathing difficulties. It can resemble pneumonia and is marked by a drastic drop in oxygen levels.

8. Pulmonary Hypertension

   • Different from regular hypertension, this condition involves high blood pressure in the lungs due to repeated blockages, causing lung damage and scarring. It’s a serious risk for adults with SCD.

9. Eye Complications

   • Frequent blood vessel blockages in the eyes can lead to gradual, often unnoticed damage. Regular eye check-ups are essential to catch and manage these issues early.

10. Stroke

    • Even children as young as one year old with SCD are at risk of stroke, as blocked blood vessels starve parts of the brain of oxygen. Strokes can impair vision, hearing, movement, and cognitive abilities.

11. Recurrent Infections

    • SCD weakens the immune system, making people with the disease—especially young children—more prone to frequent infections. Regular precautions and medical support are vital to reduce these risks.

Progress in Managing SCD Complications

Fortunately, many of these complications can now be managed more effectively with lifestyle adjustments, medications, and medical support. However, much remains unknown, and there is significant room for advancement in understanding and treating SCD. Continued awareness and research are crucial for improving life with this condition.

Thank you for reading.