September is Sickle cell disease awareness month. The medical condition affects 20 million people worldwide, most commonly those of African descent. Sickle cell disease is a group of inherited red blood cell disorders, which affect the body’s hemoglobin, the protein that transports oxygen through the body. The red blood cells take on a crescent shape, making it difficult for these cells to move freely, leading to blockages or occlusions in blood vessels. When hemoglobin cannot do its job appropriately, certain areas of the body may lack the oxygen needed to function. Those living with sickle cell disease are at increased risk for stroke, infections, and vision problems, and may experience extreme episodes of pain.
Symptoms, Diagnosis, and risk factors
Sickle cell traits are usually passed from both parents. Because sickle cell disease is inherited, it’s important to monitor babies for signs of sickle cell disease. A yellowish skin color, swelling and pain in hands and feet, and tiredness are the most common symptoms. In adults, symptoms can include fatigue, fever, cough, chest pain, shortness of breath, and symptoms of a stroke. Complications of sickle cell disease include vaso-occlusive crises, which occurs when sickled red blood cells block blood flow to the point that tissues aren’t getting enough oxygen.
Usually, newborns are screened for sickle cell disease prior to leaving the hospital. Adults may get a blood test to confirm a sickle cell diagnosis. Treatment for sickle cell disease can include medications and blood transfusions as a way to deal with the long-term pain. The only cure for some with sickle cell disease is a blood and bone marrow transplant.
Sickle Cell Disease and Quality of Life
Quality of life can be reduced among those living with sickle cell disease. According to a research study surveying sickle cell survivors using the Sickle Cell World Assessment Survey, more than half (62%) reported that they avoided intense exercise, and 41% reported sickle cell disease affected their family or social life. Additionally, 38% of respondents reported that sickle cell disease impacted their ability to perform household daily activities such as oral hygiene and food preparation. Only one-third of study participants were employed (either full-time or part-time). Respondents also reported that the three most common symptoms in the month before survey completion were fatigue, bone aches, and headache. Additionally, nearly all patients experienced at least one or more vaso-occlusive (pain) crises in the 12 months before survey completion, with the study sample experiencing an average of 3 of these crises per year. Sickle cell infusion centers may be a viable option for people grappling with these crises, but there are few in the U.S.
Sickle Cell Disease and COVID-19
Those living with sickle cell disease may be at an increased risk for complications following COVID-19 infection. One study sampled 66 people (8months-69 years old) with sickle cell disease and COVID-19 diagnosis at the time of the study. Researchers found that the majority of the sample (76%) required hospitalization. Hospitalized patients also had a greater prevalence of chronic kidney disease (44%) compared to non-hospitalized participants (35%). Additionally, one-third of hospitalized patients developed hypoxia, a condition in which the body’s tissues and organs are not getting enough oxygen.
Minimizing COVID infection risk and increasing quality of life
Limiting infection risk is critical for patients with sickle-cell disease. People living with sickle cell disease should see their primary care provider at last every 3-12 months, and stay on top of vaccinations. Because of the potential for pain crises, it may be more difficult to maintain a job, but it's not impossible. When a pain crisis is starting, it’s important to hydrate and try anti-inflammatory medications. In addition, it may be beneficial to find a provider who specializes in sickle cell disease. These providers may have more knowledge about other interventions to help someone with sickle cell disease have a better quality of life.
References
Lanzkron, S., Little, J., Wang, H., Field, J. J., Shows, J. R., Haywood Jr, C., ... & Varadhan, R. (2021). Treatment of Acute Pain in Adults With Sickle Cell Disease in an Infusion Center Versus the Emergency Department: A Multicenter Prospective Cohort Study. Annals of internal medicine, 174(9), 1207-1213.
Minniti, C. P., Zaidi, A. U., Nouraie, M., Manwani, D., Crouch, G. D., Crouch, A. S., ... & Klings, E. S. (2021). Clinical predictors of poor outcomes in patients with sickle cell disease and COVID-19 infection. Blood advances, 5(1), 207-215.
Osunkwo, I., Andemariam, B., Minniti, C. P., Inusa, B. P., El Rassi, F., Francis‐Gibson, B., ... & James, J. (2021). Impact of sickle cell disease on patientsʼ daily lives, symptoms reported, and disease management strategies: Results from the international Sickle Cell World Assessment Survey (SWAY). American Journal of Hematology, 96(4), 404-417.
National Heart Lung and Blood Institute
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