I was born with sickle cell anemia, a hereditary blood disease that deprives normally round cells of oxygen, and causes them to collapse into a “sickle” shape. This results in restricted movement of blood through vessels, and further deprives the body of muchneeded oxygen. The condition is chronic, lifelong and causes periodic attacks of pain, as well as strokes and damage to internal organs. In my case, both parents passed the sickle cell trait down to me and my sister. (Actually, it can be passed down if only one parent has it.) And yet I have resolved to live a life that is not defined by my illness.
I was introduced to this ticking bomb inside my body in the 1960s when I was a child. Always sickly and skinnier than most kids, I still played outside, kicking balls and climbing trees, sometimes into the night. I had a strong will, even if my body didn’t always back me up. When I came down with a cold or the flu, I often felt an ache deep in my bones. Sometimes, during the night, I felt pounding pain in my legs or arms, and all I could do was cry. I’ve gotten used to the pain, however, and over time, have developed a high tolerance to it.
When I was growing up, there was not only no cure, but there were few treatment options. Doctors could help ease the pain, but the weakness that sickle cell caused was something I had to learn to live with.
Sometimes I could literally hear the pounding of blood trying to flow through my limbs. I listened, as my body tried to keep up with the demands of my sickling blood, and my blocked veins and arteries groaned from the lack of flow. All I could do was moan.
My favorite escape from this reality was my annual summer camping trip with my sisters. I loved it because I met new friends and experienced wonderful adventures. This particular summer, we were going to a new camp, sponsored by a Catholic church and managed by nuns. It was at 6,000 feet in the mountains, and I was so excited.
The morning started off like any other first day of camp: We packed one suitcase full of shorts and another full of T-shirts, and drove to the church pick-up point. The buses were there, motors running, and children waited in lines, eager to board. I jumped out of our still-running car, said “goodbye” to my mother, and ran towards the person holding a sign-in sheet for our group. I gave them my name and they motioned me towards the correct bus. I hopped up the three bus steps.
The bus ride along oak-lined winding roads with river views was so exciting. I felt the weight of the world lift from my young shoulders and flow out of the window into the wilderness. I was happy and pain-free.
When we finally pulled up to the camp grounds, we immediately dropped off our suitcases and rushed to the mess hall for lunch. Smells of ham, turkey, mashed potatoes, green beans and hot rolls wafted through the room. After such a long ride, I was hungry, and the food was laid out like a banquet.
After that delicious meal, we went to our cabins to settle in and get ready for the rest of the day’s events. But while unpacking, I threw up. I lay down on my bunk bed and threw up again. In fact, I couldn’t stop. The camping staff carried me to the nurse, who laid me down in her office. Unfortunately, my condition got worse. I began to shiver. Finally, I blacked out. Just an hour or two after my arrival, camp staff had to call my mother and tell her to come and take me home.
This was my introduction to the disastrous combination of high-altitude and sickle cell disease. The higher the altitude, the thinner the air. As a result, my blood could not get enough oxygen to function normally, which caused me to have a sickle cell “crisis.” Luckily, the nurse knew to get me out of the high-altitude quickly, so I would not sustain damage to my internal organs.
Later that year, my mother discovered that there was a newly formed sickle cell clinic at a local hospital that planned to conduct a study in the Los Angeles area on people who had the condition. My sister and I were included. Each week, kind and knowledgeable doctors greeted us, took our blood, talked to us about pain, and told us why we were experiencing it. For the first time in my life, I could tell someone how it felt, and they understood. They finally helped me identify the cause of my pain, and taught me how to manage it. It was comforting to hear that it wasn’t my fault.
As a teenager I began to develop a new relationship with my body through dance. I was introduced to it by my older sister, who was a modern dance major at UCLA. One day, she turned on some music and started to sway and move in rhythm with the beat. I was mesmerized by her mastery of her own body. I loved music, but had never seen anyone move to music with such grace and beauty. Each beat seemed to have a meaning, and each change in tempo a purpose.
I discovered I could use my body to tell the stories of its struggles. Dance gave me the assurance that my body could take care of itself. Beyond hopelessness and pain, dance offered me hope, strength, love and respect. Moving and stretching also helped me find peace.
While there is still no cure for sickle cell, universities and other research centers around the world continue to study the disease. I don’t know if I’ll see a cure in my lifetime, but I will dance on.
A Closer Look
Let’s break down the components of sickle cell anemia. Anemia is a general term meaning abnormally few red blood cells. There are a few different types of cells in the blood: The white ones, for example, which fight off infection, and the red one—typically disc-shaped—that attach themselves to oxygen molecules called hemoglobin to carry oxygen to the cells that make up our organs.
Due to an inherited gene, the sickle cell anemia hemoglobin molecule is defective. The defective hemoglobin causes several problems. While normal red cells, which are made constantly in the bone marrow, last a few months, the abnormal sickle cell red blood cells live only a few days. The shorter lifespan of the red blood cells leads to anemia.
The sickle shape of the red blood cells poses another problem. Because they’re malformed, they don’t flow well through blood vessels. They tend to clump, which results in poor circulation and inadequate oxygen to the organs. The resulting symptoms are myriad: fatigue and shortness of breath. Headaches, chest, arm and leg pain are often common. Complications may include stroke, blindness, lung damage, and multiple organ failure. Infection can be a problem, too.
Sickle cell patients are often hospitalized at least once and sometimes several times a year. Blood transfusions and intravenous hydration are usually used to relieve pain and improve blood flow. Occasionally, the disease is cured with bone marrow transplantation. Researchers are now exploring gene therapy as a possible cure for the disease.
P. Allen Jones