Understanding Sickle Cell Anemia and Hemoglobin Variations
In a world where health is paramount, understanding diseases like Sickle Cell Anemia (SCA) is crucial. This condition, primarily affecting those of African, Middle Eastern, Indian, and Mediterranean descent, is characterised by the production of abnormal haemoglobin, leading to the distortion and premature death of red blood cells.
Diagnosis and Types
Newborns in countries with access to medical care receive a diagnosis through routine screening. However, for those who did not undergo newborn screening or live in regions without this facility, blood tests as adults can detect SCA. It's important to note that not everyone with SCA experiences symptoms, and some, like those with sickle cell trait, carry only one gene for the disease and do not manifest symptoms or suffer from lowered hemoglobin levels.
Complications and Symptoms
SCA complications can be severe, including dangerous blood clots, heart disease, infections, stroke, pulmonary embolism, kidney failure, eye health issues, splenic sequestration, bone damage, aplastic crisis, and a shortened lifespan, particularly if medical care is inaccessible. SCA sufferers may experience symptoms such as fatigue, weakness, dizziness, and shortness of breath due to low hemoglobin levels, which impair oxygen delivery to tissues.
Other symptoms include paleness of the skin and fingernails, jaundice, delayed growth and puberty, dizziness and nausea, rapid heart rate, and swelling and pain in hands and feet in infants and young children. Complications associated with low hemoglobin levels can be severe, including severe anemia crises, organ damage, stroke, pain crises, and increased susceptibility to infections.
Management and Prevention
Management of SCA primarily focuses on symptom relief, such as drinking plenty of water, washing hands often to reduce the risk of infection, avoiding high altitudes, and taking pain-relieving medication during sickle cell crises. Hematopoietic stem cell transplants show potential as a cure for SCA, but they are not yet widely available.
People with SCA should also prioritise vaccinations and safe food handling to minimise the risk of serious complications from infections. Maintaining a healthy lifestyle and knowing the signs of an emergency are essential for seeking prompt care when necessary.
References:
[1] National Heart, Lung, and Blood Institute. (2021). Sickle Cell Disease. Retrieved from https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease
[2] Mayo Clinic. (2021). Sickle cell anemia. Retrieved from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20368498
[3] Centers for Disease Control and Prevention. (2021). Sickle Cell Disease. Retrieved from https://www.cdc.gov/ncbddd/sicklecell/index.html
[4] American Society of Haematology. (2021). Sickle Cell Disease. Retrieved from https://www.hematology.org/patients/diseases/sickle-cell-disease
[5] MedlinePlus. (2021). Sickle cell anemia. Retrieved from https://medlineplus.gov/ency/article/000474.htm
A proper and timely diagnosis of SCA can be achieved through newborn screening or blood tests in adulthood, especially for those living in areas with limited medical facilities.
SCA, a medical-condition that can lead to various health-and-wellness issues such as blood disorders, can have severe complications like dangerous blood clots, heart disease, infections, and even a shortened lifespan if left untreated.
