Here to Stay -- Living With Sickle Cell Disease

03/19/2015 09:24 am ET | Updated May 19, 2015
Noah Williams

My name is Noah Alexander Williams and I am living with sickle cell anemia disease. Sickle cell isn't really a widely talked-about disease but it's so real and epic that it should be.

Sickle cell anemia disease is a grasping chronic sickness that doesn't let go. Unfortunately I have it and have had it since I was born. I don't know life without sickle cell disease and therefore I don't know life without pain -- the daily aches, the crucial crises that come out of nowhere. Sickle cell is never predictable. Of course this disease has impacted my life beyond words. I've learned to cope with it.

Just to be clear, sickle cell is a genetic trait. Sickle cell disease is a severe illness that occurs in people who inherit the sickle cell gene from both parents.

When I was a few months old, my pediatrician predicted that I would have my first sickle cell crisis at six months. She was right. A sickle cell crisis happens because the red blood cells aren't the normal round shape. They are long and pointed, like a sharp edged knife (or a sickle). When those sharp edges get wedged into blood vessels, they block the flow of blood and cause excruciating pain throughout the body.

I don't remember my first sickle cell crisis, but I remember a lot of them. I've been living with this disease ever since.

There are three levels of sickle cell: SS, SC and SB. Each of these levels refers to a specific gene inherited from one's parents. SB is the mild side of the disease -- referred to as Thalassemia. SS is the most severe form of sickle cell. It is the kind I have. The treatment is blood transfusions or blood exchanges.

When I was six years old I had my first massive stroke. It blew out the two main vessels in my brain. During this episode I became paralyzed and blind. It was a miracle that I regained my eyesight and snapped out of my paralysis. At six years old I wasn't sure what was happening to me. I was in a maze of fear and confusion.

I thought I was going to die -- and I wasn't the only one who thought that. After the stroke, doctors informed my parents that I would probably only live to be about nine years old.

Today I'm 17. I didn't know I was supposed to have died until I'd outlived the prediction.

I wish I could tell you that was the end of it -- that I've beat this disease. Actually, when I was 15 another doctor told my parents I wouldn't live to be 20. She didn't bother to keep this dire prediction from me, but I know that predictions aren't reliable. With God's help I intend to prove her wrong as well.

Hope is really all I have. Hope for tomorrow. Hope that I won't go to sleep in pain or wake up in pain. Holding on and not letting go is my approach.

At eight years old I had a splenectomy -- a surgery in which a part or all of the spleen is removed. Surgeons usually remove the whole spleen, but mine was only partially removed. I'm not sure why. I've had many other operations. My gall bladder was removed and I've had procedures on my heart for acute chest syndromes. Fortunately I did not require open-heart surgery.

God is good though. I feel like I have come a long way, but the truth is my journey has just began and I'm okay with that. I did not choose this disease, but it has chosen me. I'm a survivor like many other survivors. We will keep battling this disease for our own lives and for the lives of people in the future. Hopefully my struggle will lead me to a better life and hopefully it will somehow help spare future people from the pain I've experienced. God is good. That is something I will never stop believing.

My doctors have stopped giving me blood transfusions because they were creating too much iron in my blood stream. Instead, at least for now, I am being treated with blood exchanges. During a blood exchange, a person's blood is completely removed and replaced with new blood. Of course, this is a slow process. The blood cannot all be drained and replaced at once. So far the blood exchanges have helped and, along with medication, have made the disease manageable, but this disease can be very deadly. It's known in some cases to cause sudden death especially with an acute chest syndrome. An acute chest syndrome can severely affect breathing.

There is a misconception that sickle cell anemia and sickle cell anemia disease are only found in people of African descent. The fact is that many other people whose ancestors are from Asia and Europe have the gene that causes this disease.

If someone were to ask me what's my worst nightmare I would describe a sickle cell crisis. It brings an unbearable amount of pain throughout the body. Sometimes it is localized. Other times it seems like I'm being stabbed with a thousand razors. I recently had an acute chest syndrome. I was afraid I would suffocate.

I am grateful for all the medical advances that have helped me live and manage this disease, but sometimes I wish there were more. Sickle cell disease affects about 100,000 people in the U.S. and I'm sure that all of them have the same hope. Current total NIH spending on sickle cell anemia research is around 75 million dollars. That may sound like a lot, but not compared to the billions spent on some other illnesses.

Sickle cell is screaming loud but no one is listening. This disease is terrifying and it grabs the souls of the young, helpless and defenseless. Sickle cell anemia disease is overlooked, a forgotten hell that a lot of people need to be rescued from.