The Milwaukee Community Journal’s Year-long Health Focus
by Dr. Patricia McManus–Pres., Black Health Coalition of Wisconsin
When you mention “Sickle Cell Disease (SCD),” most black people recognize the name as something that “Black people get” but not much else. It is strange that more African Americans do not want to learn about this disease, especially since it occurs mainly, but not totally, in the African American population.
This is clearly one condition that the community needs to know more about. Even though new- born screening now includes sickle cell anemia, this is still after the factor. There has been little to no push for African Americans to learn their sickle cell status. It is very important to know if you have the trait so that you do not have children with someone who also has the trait. There is a one in four chance of your baby having the disease. So let’s begin to learn more about this disease that can be so devastating to children and families in our community.
What is Sickle Cell Disease (SCD)?
Definition: Sickle cell disease is caused by a genetic disorder of hemoglobin in red blood cells. People with sickle cell disease commonly experience “crises” due to stoppage of flow in their blood vessel, which can result in injury to any of the body’s tissues or organ systems. This includes stroke in approximately 20% of children with sickle cell disease. The development of vascular obstruction in sickle cell disease is complicated and likely to involve many different abnormalities of the circulating blood vessel wall. Sickling decreases the cells’ flexibility and results in a risk of various complications.
The sickling occurs because of a mutation in the hemoglobin gene. There is currently a poor understanding of how “vaso-occlusive crisis” occur. Because of this, there are very few choices for effective treatment.
SCD affects an estimated 70,000 to 100,000 Americans. The disease occurs in about 1 out of every 500 African American births. Sickle cell trait occurs in about 1 in 12 African Americans.
Life expectancy is shortened, with studies reporting an average life expectancy of 42 in males and 48 in females. It usually presents in childhood, and occurs more commonly in people (or their descendants) from parts of tropical and sub-tropical regions where malaria is or was common.
One-third of all indigenous persons of Sub-Saharan African carry the gene because in areas where malaria is common, there is a survival value in carrying only a single sickle-cell gene (sickle cell trait).
Those individuals with only one of the two genes that cause malaria are more resistant to Sickle Cell. Since the infestation of the malaria plasmodium is halted by the sickling of the cells that it infests.
People with SCD have less access to comprehensive team care than people with other genetic disorders, such as hemophilia and cystic fibrosis. Sickle cell-related death among African American children less than 4 years of age fell by 42% during 1999-2002. This coincided with the introduction of a vaccine that protected against invasive pneumococcal disease in 2000. (CDC)
Issues with care
According to Bonny Johnson, RN, MSc from Minority Nurse, “a lot of nurses and health care professionals in general have preconceived erroneous ideas that sickle cell disease patients are just drug seeker”. She goes on to state that many of these patients are hurting, and it’s often up to nurses to make sure they receive the pain control and disease management care they urgently need.
As the crescent shaped or sickle cells travel through the bloodstream, they tend to get stuck in narrow blood vessels, cutting off the flow of blood and causing excruciating pain.
The only cure for SCD is a bone marrow transplant, but it’s a risky procedure that’s not available to most patients. The alternative is to simply manage the chronic discomfort.
What can families and the communities do?
1. Families must become advocates for their relatives with this disease.
2. They must support those with this disease who are advocating for themselves.
3. Families can help by creating a home environment that eliminates trigger for sickle cell crisis, such as extreme heat, cold or increased stress. Managing stress for African Americans in this environment is very difficult.
4. Family members should be on the bone marrow donor registry. Family members are usually the best match.
5. Everyone should know their sickle cell status. You should also know the status of anyone you might have children with. On the average of 40% of babies who test positive for sickle cell had parents who knew their status. That means most of the parents had no idea of their own status or that of their partner. Both parents must have at least the trait for the baby to be born with the disease.
6. The community needs to advocate for more funding. There are three times more babies born with sickle cell than cystic fibrosis. However, according to the National Institutes of Health, for every baby born with cystic fibrosis, there is $2733 research dollars spent to find a cure. In contrast, for every baby born with sickle cell disease, only $345.58 is spent.
Another disparity or equity, however you want to call it, it is indicative of the situation for African Americans in this county. If we do not think we are good enough to fight for, who will?