Sickle cell disease (SCD) is a serious inherited chronic blood disease that mostly affects Africans and African-Americans, and it the red affects blood cells; people with sickle cell disease have red blood cells that contain only haemoglobin “S”, an abnormal type of haemoglobin. Sometimes these red blood cells become sickle or crescent shaped, and because of the abnormal shape they will have difficulty passing through small blood vessels thereby causing a blockage in the vessel.
The sickling of the red blood cells also cause them to break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice. Painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain. A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure.
Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.
The painful crisis caused by the sickle-shaped red blood cell can be likened to a heavy hammer blow smashed on the bones continuously for days and nights consecutively, sometimes weeks or months, depends on how soon the tissue responds to treatment and the red blood cell becomes normal. Occasionally, acute painful episodes may occur affecting the bones of the back, the long bones, the chest and the internal organs.
In between episodes of sickling, people with SCD are normally well. Good treatment, started early in life, can prevent complications. So, early diagnosis and specialist treatment are advised for SCD.
SCD can be properly managed with medication and the right diet, but this is very expensive and can hardly be afforded by the less priviledged in society. The only possible cure for SCD is bone marrow transplant which is very expensive and very risky, a lot of the patients cannot afford it. More often than not, children die daily from SCD.
Noah’s Ark Foundation is born out of the zeal of the trustees to ensure that indigent SCD sufferers can live a healthy normal life where possible by giving them access to drugs, nutritious food, required daily medications and medical care which can help to increase the timeline between episodes of sickling.