Sickle Cell Disease: Living with Sickle Beta Zero Thalassemia

Sickle Cell Disease comes in different forms of the condition as well as different degrees of complications. My husband Charles Jewell Francis, IV was born with Sickle Cell Beta Zero Thalassemia and due to his excessive physical activity, it was a constant threat to living a healthy life.

CJ as his family and friends called him was a very high-strung active young men who marched to the beat of his own drum. Therefore it was sometimes hard to manage his constant Sickle Cell crisis. Like Sickle Cell Anemia, sometimes there is an increase risk of getting infections. This is because most people with Sickle Cell are more susceptible to infections.

There were times that CJ would be playing tennis or engaging in rigorous activity and it would case a crisis. When a crisis would arise due to overexertion CJ would sometimes endure mild pain to very serious pain that resulted in him being hospitalized for up to a week or more. Some people with Sickle Cell have a risk of contracting Pneumonia or encounter severe pain cased by clogs caused by red blood cells of ST0 disease which are stiff and don't move easily, hence causing them pain. Also, other Sickle Cell suffers have larger spleens than other people without the condition.

CJ suffered constantly with his Sickle Beta Zero Thalassemia most of his life as he had a mild stroke in his 20's that left him with a slight crooked smile. However, he maintained an upbeat attitude about it and typically lived his life as a normal man. That was until April of 1992, when after taking a ride on his motorcycle that he came home complaining of a headache.

The headache seemed to get increasingly worst as the evening progressed. When CJ would have crisis in the past we would opt for heat therapy in a hot whirlpool or though the use of several heating pads. However, this evening nothing seemed to relieve the pain, so we had to go to the Emergency Room at the Hospital to have prescription medicine administered to relieve the pain. Once the pain medicine was administered, it seem to relieve the pain somewhat, but the headache was still there. CJ was later admitted to the hospital for observation and further testing. Overnight it appears that CJ had a stroke, which affected his speech and was disoriented by the morning. Later that day things turned for the worst and began a downward spiral where his lungs began to fail and he ultimately was put on a respirator.

Charles Jewell Francis, IV lost his fight with Sickle Beta Zero Thalassemia and he died at the age of 33. CJ's story does not have to be yours or your child's story as some people have no pain at all with this condition and have a normal lifestyle and life span. If you or your spouse has the Sickle C trait or the Beta Zero Thalassemia there is a 25% chance of having a baby with sickle beta zero thalassemia with each pregnancy, so just know that you should have your child receive regular checkups and vaccinations. Also, you may need to make several visits to a hematologist or blood specialist to monitor the condition.

Remember knowledge is power, so do your research and find out all you can about Sickle Cell and take care of you and your family.