Sickle Cell – the facts

Jul 18, 2017 News

---

By Robert E. Fraser RN, BS

World Sickle Cell Day was recently marked on June 19. Sickle Cell is an inherited condition. It is more common in people of Afro-Caribbean, African, Mediterranean or Asian heritage.
We inherit two copies of every gene – one from each parent. If you have one sickle cell gene, you would not be affected by sickle cell disease but you have the sickle cell trait. If you do have any symptoms, they are much milder than sickle cell disease, but you could pass the gene on to your children.
If you inherit the sickle cell gene from either parent; (or one sickle cell gene and another related -abnormal gene) you will be born with Sickle Cell Disease. Your red blood cells which contain a chemical called hemoglobin, and deliver oxygen to your body’s tissues can change from their normal doughnut shape into a sickle or crescent shape.
Symptoms don’t usually start until you are three to six months old, but from then on you can get episodes of ‘Sickle Crisis’ from time to time. This happens when a lot of your red blood cells change to a sickle shape at the same time, causing blockage in blood vessels.
The most common place for this to happen is in the small blood vessels in the bones and joints. Along with pain which comes on suddenly, there may be joint swelling. If blood vessels in the lungs are affected, the result can be breathlessness (shortness of breath), fever and chest pain.
The commonest type of sickle cell disease is sickle cell anemia, which affects people with two sickle genes. As the name suggests, one of the symptoms is anemia or low levels of hemoglobin. Like other forms of anemia such as iron deficiency anemia seen in women with heavy periods, it can cause tiredness, breathlessness and dizziness.
Triggers for sickle cell crisis include being dehydrated, generally unwell or cold. Infections can be more severe if you have sickle cell disease, and can also trigger a crisis.
World Sickle Cell Day aims to raise awareness of the importance of regular medication and immunizations and seeking help quickly for possible crisis. But equally important, it’s about alerting people to the possibility of passing on the sickle gene before they have children. It is possible to have sickle cell trait without ever knowing it, so the only way to know for certain is to be screened, ideally before you get pregnant.
“The End is at the Beginning.” I invite your attention to the aforementioned quote. Because I do believe that although the statement seems contradictory and absurd, it captures an essential truth that is so self evident that we may not grasp the full meaning unless we take a moment to focus our attention on the key message that it contains.
The goal and end point vision should be to raise and increase the public’s awareness and consciousness of this devastating disease.
Each can play a very meaningful part to intensify focus on the care of sickle cell anemia. It is education and legislation that will have a positive effect.
For that reason I am advocating the introduction and passage of legislation – THE SICKLE CELL TREATMENT ACT. This critical and potentially life – saving legislation should aim to provide preventative medical strategies, treatment and services to juveniles and adults with sickle cell disease.
It also should encourage and support testing to determine carriers of the sickle cell trait to prevent the spread of the disease.

The sickle cell Treatment Act will place Guyana in line with, for example, England, where all babies are screened for sickle cell as part of routine tests within one week of birth. Every state in the United States, The District of Columbia and the U.S Territories require that all new born babies receive screening for Sickle Cell Disease (SCD).
When a child has SCD, the parents are notified before the child develops symptoms. If a person has SCD, it is present at birth. Medical findings indicate that most infants do not experience any problems from the disease until five or six months of age.
I urge the citizens of Guyana to petition their Members of Parliament to introduce and pass the Sickle Cell Treatment Act. It will prove to be a significant life saving strategy. I urge you to spread the word and encourage relatives, friends and neighbors to seek out all available resources to educate themselves about this disease.
The author of this presentation is a graduate from the New Amsterdam School of Nursing and has practised nursing in the New York Area in Clinical and Administrative areas in reputable teaching institutions. He is also involved in an ongoing research in Sickle Cell anemia in the ‘Family.’
He also conducts in-service education for Nurses in Regions Five and Six annually.

---

---