People are struggling with diseases and disorders that you never know about. They look healthy and act “normal”. Often times they suffer in silence by choice, shame, or embarrassment. But many of these people are your friends, family, or loved ones. Today is no different. June 19th is World Sickle Cell Day, and while you may have heard of the disease, I want to put a couple of (cute) faces to it.
Two of my children, Miss Double Digits and Stinker, both have Sickle Cell Disease (SCD). Under Texas law, all newborns are tested for a total of 53 birth defects, conditions, and disorders. With one blood draw, you can find out what, if any, medical issues your baby might have. My kids were tested and their diagnoses were confirmed within 2 weeks of coming home from the hospital, and both times my reaction was the same.
When my oldest daughter was diagnosed, I cried for days. I felt that I didn’t do my job as her mother to protect her. I went through a mental checklist of what could I have done differently: changing my diet or taking more vitamins. I was a wreck. I know my thoughts weren’t logical; sickle cell is a genetic disease, and none of those things would have changed the outcome.
As a mother, it was hard for me to reconcile that this was going to be our life. Almost immediately, our lives, routine, and what was considered “normal” changed. Now, our baby had to take two different preventative medications twice a day. Preventative?! What exactly are we trying to prevent? I was freaking out! I spent a lot of hours researching the disease and praying that my daughter would be on the “low end” of the scale. In the beginning, I was mildly paranoid that she would become a sickly child. Since babies can’t talk about how they are feeling or tell you where it hurts, I felt that I had to watch her more closely to ensure that she was ok.
Sickle cell is invisible. You can’t tell who has it or who doesn’t. You can’t see which part of the body it is affecting. There is nothing on the outside that can tell you the story of the inside. SCD is an inherited blood disorder that affects red blood cells. People with sickle cell disease have red blood cells that contain an abnormal type of hemoglobin (hemoglobin S). These red blood cells can become sickle-shaped (like a C) and have difficulty passing through small blood vessels. Anywhere blood can get “trapped”, like in the joints, can create what is called a pain crisis.
Nothing prepares you for the things that you are unprepared for. One of our first pain crisis happened when my daughter was three. She was crying constantly and kept holding her elbow. Naturally we thought that she bumped into something while playing, but she just wouldn’t calm down. We eventually took her to Texas Children’s Hospital’s Emergency Room. After tests and x-rays, we discovered that this was actually a sickle cell event, a pain crisis. Blood was pooling in the joint at her elbow and was causing her pain. It was unreal to think that this is what the disease looked like. In my three-year-old. This would not be our last trip to the ER as she experienced several more pain crises until she turned five.
Education was the key to my child’s survival! I had to learn what the disease was and its extremes, and then, I had to teach others. Instantly, I had to become an advocate. For healthcare. For equal treatment. For awareness. For my daughter. I had to do what it took to protect her and prepare others. Our family needed to know what to do just to babysit. And once she was off to elementary school, administrators needed to get the Cliff Notes about sickle cell too. I have even had to write a couple of letters to a major drugstore about the treatment I received from a pharmacist in regards to her pain medication. If you want to see me become a “Momma Bear”, just wait until you hear about the negative experience I had at the pharmacy. (Side note – The opioid crisis in the news today also includes those who medically need these drugs to live a pain-free life, but I’ll save those thoughts for another post.)
Miss Double Digits is now her own advocate. She doesn’t need me to talk to the nurses or doctors about her care. She knows exactly what medication to take and when to take it. I am grateful that she is the type of child that can handle this type of disease. I allow her the space to help manage her own care, especially since the next seven years will disappear and she will be on her own. Don’t get me wrong, I am still Momma Bear when I need to be.
As for the boy, Stinker is only three so his journey with this disease is just beginning. Thankfully, he has not had any pain crisis since birth. I attribute this partially to a more aggressive course of treatment for him. Hydroxyurea is a medication that was formerly used to treat cancer patients until researchers figured out that it could be useful for SCD patients. It is known to lessen painful crisis episodes and prevent the need for blood transfusions. My daughter started hydroxyurea when she was five; Stinker started it at 14 months. They have a very positive response to this medication.
However, Stinker’s most pressing issue was the nurses. He would give them the blues! At one point, it would take four nurses to just get his blood pressure and a blood draw. FOUR. But then his big sister came to the rescue. She helped to calm him down and talk him through it. While I don’t think it made a big difference in the beginning, it has begun to get a lot easier. Now, we only need one (strong) nurse to make it through.
Sickle cell will be a part of our lives for the rest of our lives. While there is no cure, there is a lot of research and clinical trials (which my kids are a part of) that will make it one step closer to finding it. In the meantime, I’ll just do my part to help increase awareness of this disease. Remember – September is Sickle Cell Awareness Month.