September is Sickle Cell Awareness Month

September is sickle cell awareness month, and Medical News Bulletin is here to give you the latest information on the condition. This article will delve into the causes, symptoms, and available treatments for sickle cell disease.

What do I need to know about sickle cell disease this September?

1. If you are of African descent and don’t know your sickle cell status, book yourself in to get tested. It can help both you and your kids to know whether you are at risk.
2. People with sickle cell disease depend on blood donations to stay healthy. AB- blood types are under-represented in stocks, so if you would like to help, consider donating blood.¹
3. In some regions, like England and Wales, specialist nurses play a crucial role in managing sickle cell disease. Their expertise ensures faster, more effective treatment during crises. We need this level of care everywhere. You can call your representative today and ask them to fund sickle cell disease as a healthcare specialty.²
4. Racial bias plays a significant role in the under-recognition and inadequate treatment of sickle cell disease in the global North. In healthcare settings, this can lead to misdiagnosis, delayed treatment and negative patient experiences. 

You can find resources and events to help educate yourself and others about sickle cell disease at these organizations:

• Sickle Cell Society (UK): https://www.sicklecellsociety.org/
• Sickle Cell Disease Association of America: https://www.sicklecelldisease.org/

What is sickle cell disease?

Sickle cell disease (SCD) is a group of inherited red blood cell disorders that affects millions of people worldwide.³ It’s a serious condition that can lead to a variety of health complications including anemia, organ damage and jaundice

In individuals with sickle cell disease, the red blood cells, normally round and flexible, become rigid and take on a crescent or “sickle” shape. This change in shape is caused by a mutation in the gene that produces hemoglobin, the protein responsible for carrying oxygen in the blood.⁴

The misshapen cells can stick together and block blood vessels, leading to reduced blood flow and oxygen delivery to tissues.

Symptoms and complications

Symptoms of sickle cell disease can vary widely and range from mild to severe, as well as cause episodes of severe pain known as “sickle cell crises,” 

• Anemia: a lack of healthy red blood cells to carry sufficient oxygen can cause tiredness and irritability.⁵
• Jaundice: sickle cells have a shorter lifespan than healthy red blood cells, and they die faster than the liver can clean out. This leads to a buildup of bilirubin, causing yellowing of the skin and eyes.⁶
• Swelling of hands and feet
• Frequent infections due to damage to the spleen
• Vision problems
• Delayed growth and development in children
• Organ damage, particularly to the spleen, kidneys, and lungs

Treatment and management 

While there is no cure for sickle cell disease, various treatments and management strategies can help individuals live longer and healthier lives. These may include:

• Medications to manage pain and prevent infections
• Blood transfusions to increase the number of healthy red blood cells
• Stem cell transplants, which are the closest thing to a cure in some cases
• Supportive care, including lifestyle modifications and regular checkups

Living with sickle cell disease can be challenging, but with proper management and support, individuals can lead fulfilling lives. It’s important for people with the condition to work closely with their healthcare provider to develop a personalized treatment plan.

Causes and risk factors

Sickle cell disease is an inherited condition passed down through families. It can happen when a child inherits two copies of the sickle cell gene, one from each parent. If a child inherits only one copy, they have sickle cell trait, which usually doesn’t cause symptoms but can be passed on to their children.People of African, Hispanic, Mediterranean, Middle Eastern, and South Asian descent are at a higher risk of inheriting the sickle cell gene.^3,4

Where can I find more information?

If you have concerns about sickle cell disease or are at risk of carrying the sickle cell gene, it’s important to talk to your doctor. Early diagnosis and management are crucial for living well with sickle cell disease.

References 

1. Telfer P, Agodoa I, Fox KM, et al. Impact of voxelotor (GBT440) on unconjugated bilirubin and jaundice in sickle cell disease. Hematol Rep. 2018;10(2):7643. Published 2018 May 22. doi:10.4081/hr.2018.7643
2. Rare blood types (no date) NHS Blood Donation. Available at: https://www.blood.co.uk/why-give-blood/demand-for-different-blood-types/rare-blood-types/#:~:text=AB%20negative%20is%20the%20rarest,both%20rare%20and%20in%20demand. (Accessed: 03 September 2024). 
3. Shortage of sickle cell nurses putting patients at risk (no date) BSH. Available at: https://b-s-h.org.uk/about-us/news/shortage-of-sickle-cell-nurses-putting-patients-at-risk (Accessed: 03 September 2024). 
4. What is sickle cell disease? (no date) National Heart Lung and Blood Institute. Available at: https://www.nhlbi.nih.gov/health/sickle-cell-disease (Accessed: 18 August 2024). 
5. Sickle cell trait (no date) National Heart Lung and Blood Institute. Available at: https://www.nhlbi.nih.gov/health/sickle-cell-disease/sickle-cell-trait (Accessed: 18 August 2024). 
6. Mandal AK, Mitra A, Das R. Sickle Cell Hemoglobin. Subcell Biochem. 2020;94:297-322. doi:10.1007/978-3-030-41769-7_12