Sickle cell anemia, often misunderstood and surrounded by misconceptions, is a condition that individuals can live with while maintaining a fulfilling life. According to the National Guidelines for the Control and Management of sickle cell disease in Kenya report NATIONAL-GUIDELINES-FOR-CONTROL-AND-MANAGEMENT-OF-SICKLE-CELL-DISEASE-IN-KENYA-.pdf (ncdak.org) Sickle cell disease affects 20-25 million of people globally, of which 12-15 million live in Africa. It is estimated that 75-85% are children born in Africa where 50-80% of children born with the disease die before the age of 5 years. Sickle cell disease (SCD) encompasses a group of inherited red blood cell disorders, where red blood cells contain abnormal hemoglobin, a protein responsible for oxygen transportation. Unlike healthy red blood cells, which are round and smoothly navigate the bloodstream, SCD produces crescent-shaped or sickle-shaped red blood cells. These cells have a shorter lifespan, resulting in a constant shortage of red blood cells. Furthermore, their irregular shape causes them to get stuck in small blood vessels, obstructing blood flow and leading to various health complications such as pain, infections, acute chest syndrome, and stroke.
There are several types of SCD, determined by the specific genes inherited from parents. Common types of sickle cell disease include Hemoglobin SS disease, Hemoglobin SC disease, Hemoglobin SB+ (beta) thalassemia, Hemoglobin SB O (Beta-zero) thalassemia, Hemoglobin SD, Hemoglobin SE, Hemoglobin SO, and sickle cell trait.
Symptoms of sickle cell anemia typically manifest at a young age, often appearing in babies around the six-month mark, although some may present as early as four months old. While the severity may vary, the symptoms remain consistent across different types of SCD. These symptoms include excessive fatigue or irritability resulting from anemia, fussiness in babies, bedwetting associated with kidney problems, jaundice (yellowing of the eyes and skin), swelling and pain in hands and feet, frequent infections, and pain in the chest, back, arms, or legs.
Complications associated with sickle cell anemia include:
1. Pregnancy complications: Sickle cell anemia increases the risk of high blood pressure and blood clot formation during pregnancy, along with an elevated likelihood of miscarriage, premature birth, and low birth weight babies.
2. Stroke: The blockage of blood flow to a specific area of the brain by sickle cells can cause a stroke. Warning signs of a stroke include seizures, weakness or numbness in the limbs, sudden speech difficulties, and loss of consciousness.
3. Pulmonary hypertension: Individuals with sickle cell anemia may develop high blood pressure in their lungs. This complication primarily affects adults and is characterized by symptoms such as shortness of breath and fatigue. If left untreated, pulmonary hypertension can be fatal.
4. Acute chest syndrome: This life-threatening complication arises from a lung infection or blockage of blood vessels in the lungs due to sickle cell traits. Symptoms include chest pain, fever, and difficulty breathing.
5. Priapism: Men with sickle cell anemia may experience painful, prolonged erections. Sickle cells can block blood vessels in the penis, potentially leading to impotence over time.
To manage sickle cell disease effectively, individuals should maintain a healthy lifestyle. This includes drinking 8 to 10 glasses of water daily, consuming a balanced diet, and avoiding extreme temperatures and excessive fatigue. While physical activity is encouraged for children with sickle cell disease, it is important to strike a balance and ensure adequate rest when fatigued, along with proper hydration.
It is crucial for individuals with sickle cell disease to recognize that they can lead full lives and engage in activities enjoyed by others. Finding a supportive community-based organization or support group can provide valuable information, assistance, and emotional support throughout their journey with sickle cell anemia. By fostering a positive mindset and implementing appropriate self-care strategies, individuals with sickle cell disease can thrive and embrace life’s opportunities.
