There are people 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. Sickle Cell is an “invisible” disease. 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). In Texas, 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. I was a wreck and cried for days. I went through a mental checklist of what could I have done differently: changing my diet or taking more vitamins. I know my thoughts weren’t logical; sickle cell is a genetic disease, and none of those things would have changed the outcome. But I still struggled with the overwhelming feeling that I could have done better or done more.
When my oldest daughter was diagnosed, 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! Admitedly, iIn the beginning, I was mildly paranoid that she would become a sickly child. I spent a lot of hours researching the disease and praying that my daughter would be on the “low end” of the scale. Since babies can’t tell you how they are feeling or where it hurts, I had to be diligent about watching her more closely.
What is Sickle Cell?
Sickle cell is an “invisible” disease. 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 of a person that can tell you the story on 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 (i.e. a pain crisis). Miss Double Digits’ first pain crisis happened when she 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 the emergency room of Texas Children’s Hospital. After several tests and x-rays, we discovered that it was actually a pain crisis. Blood was pooling in the joint at her elbow which was the source of her pain. It was unreal to think that this is what the disease looked like… in my three-year-old. And unfortunately, this would not be our last trip to the ER as she experienced several more pain crises until she turned five.
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 not long from now she will be out on her own.
As for my son, 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. Miss Double Digits started hydroxyurea when she was five; Stinker started it at 14 months. They both have had a very positive responses 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. It took a few visits before we realized that we needed a specialized kind of help. Insert big sister, Miss Double Digits, 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.
Education is the key.
I learned what the disease was and its extremes, and then taught others. I had to become an advocate. For healthcare, equal treatment, and awareness. For my daughter and my son. I had to do what it took to protect my children and prepare others. Our family needed to know what to do just to babysit. At school, administrators got the Cliff Notes about sickle cell too. I made sure to have meetings with nurses and counselors and the 504 team. 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 pain management medication. (And just a 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.)
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 – World Sickle Cell Day is June 19 and September is Sickle Cell Awareness Month.