10 on Health
10 things to know about Sickle Cell Disease
1. Sickle cell anemia is a disease passed down through families where red blood cells form an abnormal crescent shape (Red blood cells are normally disc shaped) at low oxygen levels. These sickle shaped cells clog small blood vessels and disrupt normal blood flow to organs.
2. Sickle cell is more common in people of African and Mediterranean descent. It can also be seen in people from South and Central America, the Caribbean and the Middle East.
3. Sickle cell anemia is inherited from both parents who are blood type AS to give an SS child. A child who inherits the S gene from one parents and a normal A gene from the other parent will have sickle cell trait. These children do not have symptoms of true sickle cell anemia
4. Symptoms of sickle cell usually do not occur until after age 4 months since before this time the blood type is still in its fetal form (Hemoglobin F). After 4 months it changes to a more mature type (Hemoglobin S) that is capable of sickling at low oxygen levels
5. Symptoms of Sickle Cell Anemia
- Hand-Foot Syndrome: This is when small vessels to the hands and foot are blocked causing pain and swelling. This may be the first sign in infants.
- Fatigue, paleness and shortness of breath: This is due to low oxygen carrying ability of the sickled red blood cells
- Pain (Crises) – unpredictable pain in joints and body wherever sickled cells blood blood supple to a tissue. The frequency and amount of pain varies and can last minutes or hours.
- Eye Problems
- Jaundice (Yellowing of Skin and Eyes) – due to rapid break down of the weak sickled cells which release bilirubin that colors the skin ad eyes
- Delayed growth and puberty – Due to shortage of cells
- Infections – due to damaged immune system organs (spleen)
- Stroke – due to damaged brain blood vessels
- Acute chest pain – this life long problem is similar to pneumonia and is caused by trapped sickled cells in the lungs.
6. A pregnant woman with sickle cell disease is at increased risk of preterm labor and needs careful monitoring throughout pregnancy. If the baby’s father has sickle cell trait, the baby has a 50 percent chance of having the disease. If he does not, the baby will have only the trait
7. Hydroxyurea is a drug that has shown promising results in adult sickle cell patients. It reduces the frequency of severe pain, acute chest syndrome and the need for blood transfusions.
8. In 1954, Anthony Allison hypothesized that sickle cell trait offered protection against malaria. He suggested that those with the trait did not succumb to malaria as often as those without it; but, when they did, their disease was less severe. The sickling process destroys the malaria parasite and prevents it from spreading through the body. Therefore in regions repeatedly devastated by malaria, people who carry the sickle cell trait will have a greater chance for survival than other individuals.
9. Sickle cell disease is a global health problem, however, in Nigeria one in every four persons are carriers of the sickle cell gene. It is estimated that over 150,000 babies are born with symptomatic sickle cell anemia each year in the country.
10. It’s especially important to find ways to control and cope with pain of sickle cell. Different techniques work for different people, but it might be worth trying heating pads, hot baths, massages or physical therapy. Prayer, family and friends also can be sources of support
MedStud Tip: As recently as 1973, the average lifespan for people with sickle cell disease was only 14 years. Currently, life expectancy for these patients can reach 50 years and over. Women with sickle cell live longer than their male counterparts – # RIP SAZZY #
Sources: Medline Plus, EverythingSickleCell.com, MayoClinic.com, WHO, NYTimes Health Guide