Courtney Stinnett: Real Stories from People Living with Sickle Cell Disease

Courtney’s Story

Living with sickle cell can be challenging and unpredictable, but I believe it makes us some of the strongest people on earth.

The type of sickle cell disease (SCD) that I have is called hemoglobin S/beta(+)-thalassemia (also called sickle beta-thalassemia or HbS beta-thalassemia). Although it is a milder type of SCD, I still have pain episodes and other health issues. When I have a pain crisis, it can occur at any moment. It’s unpredictable. I can literally just start hurting all of a sudden. The only way I can describe a pain crisis is to compare it to a migraine headache, but it’s 10 times worse and located in the joints. I have gotten up in the morning feeling fine, driven to work, and could not get out of the car because of a pain crisis. The pain can be that sudden.  I try to do things to avoid going into a crisis, but I cannot always prevent it from happening.

Sickle cell disease is an inherited (genetic) condition, present from birth, that causes red blood cells to stick together, blocking oxygen and blood flow in the body. It can cause pain and other serious health problems.

Certain things can help manage pain crises, such as staying hydrated, staying warm in the winter, staying cool in the summer, trying to avoid colds and infections by getting flu and pneumonia shots every year, and of course, taking my medicines and listening to my doctors.

It can be very frustrating to have a pain crisis, go to the emergency department, and not be believed or treated as a priority because I am not ‘behaving’ like I am in pain. My biggest fear is having a stroke.
My son has beta-thalassemia intermedia, also known as Cooley’s anemia. In Cooley’s anemia, the red blood cells have too little of the hemoglobin protein to adequately transport oxygen throughout the body, and the red blood cells don’t live as long. He does not experience pain crises, but he does need regular blood transfusions. Since the age of 4, he has been transfused every 5 to 8 weeks. Signs and symptoms related to Cooley’s anemia include fatigue, headache, difficulty concentrating, reduced ability to exercise, and slow physical growth. He is short, looks young for his age, and he has an individualized education program at school. He has had a splenectomy (a surgical procedure to remove the spleen), which means he has a greater risk of infection, so he takes penicillin every day. Believe it or not, it is easier for him to tell his friends that he has sickle cell rather than trying to explain his disease.

The transfusions can be overwhelming at times. Before each transfusion, there has to be a type and crossmatch done (two blood tests to determine whether blood from the donor is compatible with the person receiving the blood). The type and crossmatch is good for only 72 hours. If the transfusion has not begun within the 72 hour window, then another type and crossmatch needs to be done before he can receive blood. The amount of time between transfusions varies. As of now, the doctors have determined that my son needs a transfusion every 5 weeks. Regular transfusions can cause a dangerous buildup of iron in the body so he needs chelation therapy (medicine to lower the amount of iron in the body). He now takes five different medicines every day for his condition.

Between the two of us, keeping up with prescription refills and doctors’ appointments can be a part-time job. There have been times when I have had an appointment in the morning, only to return later in the evening or the next day for his appointment and vice-versa. Getting and/or keeping a job or maintaining school work can be difficult.

Thanks to certain medicines, such as hydroxyurea (a medicine that can reduce sickle cell-related health problems), people with SCD are able to live longer, healthier lives.