By Melissa Creary
I was three years old when I was diagnosed. My parents did not know what was wrong, when after a day at the pool, I became very ill and nothing seemed to pacify me. I went through test after test. At one point, the doctors even thought I had leukemia. It was not until my aunt, a nurse, asked my mother if I had ever been tested for sickle cell anemia, that my family finally had an answer. Both of my parents had emigrated from Jamaica and unfortunately neither of them had even heard of such a disease. Neither of them knew that they each carried a gene for altered hemoglobin, which in turn had drastically affected their first offspring.
The hemoglobin portion of my red blood cells (the part that carries oxygen to all of my body parts) was altered so that sometimes it caused my normal blood cells to sickle. The most common form of sickle cell disease (SCD) is sickle cell anemia, but there are other forms of altered hemoglobin that may combine to form different types of SCD, such as sickle cell SC and sickle cell beta-thalassaemia. These can be sometimes milder forms of the disease. My parents were told that I had the SC variant.
When there is not enough oxygen present in my blood, my cells become sticky and hard and can not squeeze through small blood vessels. The cells can block the vessels, not allowing oxygen to get to where its needed. This can lead to severe pain and organ damage. When I was in the 5th grade I had to run a mile and instead of telling my instructor that I was tired, I pushed myself and as a result missed the rest of the school year. My Junior year in college, I was so stressed out about a test that I ended up in the ER due to a sickle cell crisis. Painful episodes can be alleviated by avoiding:
- Over exertion
- Getting cold/wet
- Stress/emotional upset
- Sudden temperature changes
- Poor eating habits
Other problems that may occur include stroke, growth problems, chest pain, and ulcers. The disease does not affect everybody in the same way or with the same severity. It affects people in a wide age range and is usually unpredictable in the times a crisis may arise. An episode may last a few hours to several weeks, with excruciating pain or mild discomfort. I have been hospitalized twice, while others have experienced pain and hospitalizations all of their lives.
An important part of knowing about SCD is knowing about the sickle cell trait. The trait is when an individual carries a small amount of sickle hemoglobin, but not enough to cause complications. If a person with the trait has a child with someone else who also has the trait, it is possible to have a child with SCD.
In Jamaica about 10% of the population have the sickle cell trait, but in other islands the frequency varies from 7% in Barbados to as high as 13-14% in Dominica and St. Lucia. This compares to about 8% in the Black American population, and frequencies of 20-30% in Black populations of West Africa and of some populations in Saudi Arabia, India, Greece, and Italy.
You can find out if you carry the sickle cell gene by getting a simple and painless blood test called hemoglobin electrophoresis. Pregnant women or people thinking of having a child should be tested to find out for sure if having a child with SCD is a possibility. Tests can be arranged by your general practitioner or at your local sickle cell center or foundation. Newborn screening is mandatory in most states for SCD so that key early diagnosis can be detected. The only way SCD can be prevented is to detect the sickle cell trait and to make knowledgeable decisions about family planning. Sickle cell disease can be treated however. Pain is treated as it arises and anti-sickling drugs are now available. Transfusions can also be utilized if the SCD crisis is severe enough. Regular immunizations and good nutrition will make those who have the disease less susceptible to infections. Healthy eating includes more grain products, fruits and vegetables, and less food with sugars and fats. Lots of water and juices and less caffeine are also important parts of the diet. Yams, herbal products, and foods that contain zinc and omega fatty acids may also help. Folic acid may assist in blood formation, perhaps improve growth and is found in dry beans, many vegetables such as spinach, cabbage, and okra, and fruits which include kiwi, plantains, orange and pineapple juice.
When I was three, there was not a lot of advancement made in the sickle cell world. Twenty years later, the outlook for someone who has the disease has improved tremendously. In the past, the survival beyond the age of 30 was not likely, but it is now realized that many with the disease live well beyond that age. Easy access to medical care is still not the best it could be in our community, but improvements are occurring, and the development of the disease is being researched continuously. I am currently one of the many worldwide who are involved in researching the disease to improve the quality of life for those whom it affects. It is still important however, to make the public aware of a disease that affects, quite disproportionately, Blacks all over the world. There is still not enough public knowledge and awareness of SCD or public funding for advancements. It is still important to dispel the myth of not only my disease, but of the 50,000 Blacks in the U.S. alone it affects annually. It is not contagious, but a genetic disorder you receive from birth. It is not cancer. The mind is not affected. I completed a college degree, am working, and will be going to graduate school next year. It is not just a “Black” disease as it affects Hispanics and people of some Asian and Mediterranean origin as well. It is not “bad blood” or a family curse. It is a disease that exists just as any other except in a way unlike any other. In its uniqueness, it offers challenges to both those who treat it and those who live with it. It is a disease that has molded my life and made me who I am and hope to be.
About the Author: Recent graduate of Emory University (B.S. in Biology), currently working in the Biochemistry Division of MSM. Has been a part of sickle cell education, advocacy, and research for over eight years, and has the disease. Future plans include attending Emory University for graduate school to obtain a Masters in Public Health to continue the strides made in sickle cell development.
If you would like to help the cause or want more information you can contact Melissa at the Morehouse School of Medicine, 720 Westview Drive, S.W., Atlanta, GA 30310.