Sickle cell disease involves more than just pain

My first two children, Tyson and Jordyn, were born in 2013 and 2015, respectively, with sickle cell disease type HbSS.

Tyson was diagnosed about a week after birth. The doctors called me and told me I needed to bring him in for his first hematology appointment. After that visit, they gave me a thick, red, three-ring binder full of information about sickle cell and wished us luck.

I read that binder from front to back several times, and vaso-occlusive crises comprised only a portion of it. While crises are the best-known complication of sickle cell, there are many more I’d like to talk about.

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Other complications of sickle cell disease

Immunodeficiency is one of the most dangerous complications that sickle cell warriors, especially children, have to deal with. Tyson and Jordyn had to take penicillin every single day until they were 5 to help fight any infections they might get. Hygiene became a focus in our household. My kids always traveled with little book bags containing hand sanitizer and antibacterial wipes. When Tyson started school, he wasn’t even allowed to share the classroom supplies.

In 2020, when we first started hearing about COVID-19, I pulled him out of school a week before everything shut down. Jordyn has never attended school in person, as her first year started in the fall of 2020.

Infections are one possible trigger of acute chest syndrome, another frequent complication of sickle cell disease that can look similar to a severe case of pneumonia. Unfortunately, both Tyson and Jordyn have had acute chest syndrome. In 2022, my daughter experienced it twice, and both times, she ended up in the intensive care unit on a BiPAP machine.

Sickle cell warriors are also at a higher risk of stroke, so much so that my children undergo a transcranial Doppler ultrasound every year. This kind of scan can detect blockages in the brain’s blood vessels, helping us prevent a stroke. Unfortunately, it cannot catch blockages in the smaller retinal arteries.

In 2024, Jordyn had an eye stroke, also known as a retinal artery occlusion, which left her with only partial vision in her left eye. The optometrist explained to me that she had essentially experienced a vaso-occlusive crisis in her eye.

Organ failure is another complication. Among those with sickle cell, it’s more common in adults, but the damage can start in childhood. When people have a very high percentage of sickled red blood cells, it can cause damage even if no blockage occurs. Because sickled cells are more rigid than normal blood cells, they can damage the inner lining of blood vessels, potentially leading to organ failure over time.

Avascular necrosis is another common sickle cell complication, though it’s not talked about enough. It refers to the death of bone tissue caused by a lack of blood supply. This issue is also more prevalent among adults with sickle cell, but in 2022, Jordyn developed it. She had to have knee surgery and do outpatient occupational therapy to help her walk again.

The last complication I want to mention is blood clots. Sickled red blood cells are prone to clumping together and creating blockages due to their misshapen form. And those blockages can easily clot, resulting in a life-threatening situation.

Unfortunately, this list only scratches the surface of the many hardships sickle cell warriors face. But I hope that in reading this, you have learned something new and maybe even share this information with a friend or family member. Sickle cell is often invisible and overlooked, so before Sickle Cell Awareness Month ends, do something to shine a light on all the warriors out there.

Note: Sickle Cell Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Sickle Cell Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to sickle cell disease.