Case Study 1

Sickle Cell Disease Jaila Stanley Prairie View A&M University HLTH 4306: Health & Communities Dr. Kentya Ford February 7 th , 2023 Sickle Cell Disease is a red blood cell disorder that causes the red blood cells to become abnormally shaped. It is caused by a mutation of the protein hemoglobin, which carries oxygen through the RBC's. “This causes pain and discomfort in the joints and chest (called pain crisis), dizziness, fatigue, infections, stroke, etc.” Normal red blood cells take the shape of a circle, so that they can easily carry oxygen through the blood vessels to the rest of the body. In individuals with sickle cell disease, the red blood cells abnormally form, become hard and rigid, and mimic the shape of a sickle which makes it harder for oxygen to get to different parts of the body. Sickle cells die quicker, causing a lack of an adequate amount of healthy red blood cells, in turn causing anemia. Sickle Cell Disease is inherited when both parents are carriers of the mutated gene. The disease has origins in east, west, and central Africa; as well as India and the Arabian Gulf. Research has shown that the mutation of the hemoglobin gene, which is linked to the cause of sickle cell, was to give protection against malaria. Individuals in countries along the coast and in central Africa are affected by SCD the most because malaria is more prominent there. In turn, there is evidence that individuals of African descent are more affected by this disorder. There is more than one type of SCD, and it depends on different factors such as your ethnicity, geographical location, genetics. There is HbSS sickle cell disease, it is when two abnormal are inherited from both parents and causes the blood cells to take the sickle shape, it is one of the most severe and talked about forms of sickle cell disease. HbSC is when one abnormal “S” hemoglobin gene is inherited, and another abnormal C gene is inherited, this is usually not as severe as HbSS. Then there is HbS beta thalassemia. This is when one gene of the abnormal “S” hemoglobin is inherited from one parent and the abnormal beta thalassemia gene is inherited from the other parent. In addition, there are two types of the beta thalassemia gene, beta thalassemia zero and beta thalassemia plus. Individuals with beta thalassemia zero have a more severe form of sickle cell disease, but those with beta thalassemia plus have a less severe form of the disease.

Sickle Cell Disease in all Ages Age Specific Potential Risks Potential Solutions Neonatal/Infants Infants with severe sickle cell are at higher risk of infection. Infants with severe forms of sickle cell, are recommended to take antibiotics twice a day until they are five years old. Children Children with severe sickle cell are at higher risk of infection. Up until five years old, children with severe forms of sickle cell, are recommended to take antibiotics twice a day. Adolescents Acute and chronic pain associated with the disease can lead to missed school time; changes in the transition from pediatric to adult care can cause complications with the disease due to pause in care; children with sickle cell may have delayed growth or puberty. Adolescents with severe sickle cell can create care plans in school to accommodate the effects of the disease. This includes, ensuring they have enough water, frequent bathroom breaks, etc. Frequent checkups and follow ups with PCP are necessary as well; emotional support during crisis may also help adolescents dealing with sickle cell disease. Adults Adults can develop high blood pressure in the lungs, acute chest syndrome, anemia, fever, blood clots, avascular necrosis, etc. Treatments for adults with sickle cell disease include bone marrow transplants and blood transfusions. These are the only known treatments, but there are other options to manage symptoms such as using antibiotics to manage infections, constant hydration, vitamin supplements, pain medications, etc. Older Adults Older adults can develop high blood pressure in the lungs, acute chest syndrome, anemia, fever, blood clots, avascular necrosis, etc. Treatments for adults with sickle cell disease include bone marrow transplants and blood transfusions. These are the only known treatments, but there are other options to manage symptoms such as using antibiotics to manage infections, constant hydration, vitamin supplements, pain medications, etc.