Sickle Cell Disease from a Public Health Perspective: Lessons from the National Heart,Lung and Blood Institute (NHLBI)

This is a second topic I am sure many have heard about. Yes, we are talking about Sickle Cell Disease ( SCD). Sickle Cell Disease is caused by a genetic abnormality in the gene for hemoglobin, which results in the production of sickle haemoglobin. Hemoglobin is a protein that carries oxygen. Once oxygen is released from a sickle hemoglobin it sticks together and forms long rods which damage the shape of the red blood cells (WebMD). Abnormal hemoglobin is called haemoglobin S, this causes sickle cell disease (SCD).

SCD, is passed on from parents to children, it is not contagious and no one can catch like they catch a cold or an infection. People with SCD inherit one abnormal gene from both parents. When the person has inherited one abnormal gene then they are said to have an abnormal hemoglobin S. When they inherit both genes they are then said to have hemoglobin SS. Hemoglobin SS is called Sickle Cell Anemia. This is the most severe and most common SCD.

June 19th of every year, is set aside as the National Sickle Cell Day.  This year ( 2015) Dr. Gary H. Gibbons of the  National Heart, Lung and Blood Institute (NHLBI), shared the story of  Afia Donkor who is one of a handful of people to have undergone a successful bone marrow transplant to cure her disease.

The NHLBI also set the stage for an opportunity at the campus of the National Institutes of Health in Bethesda, Md., that will brought together diverse members of the sickle cell disease community, including patients and their families, advocates, health care professionals, professional societies, government agencies, and others.  As a community, we will discuss and strategize about the future of sickle cell disease research.  The forum will include panel sessions and discussions that focus on issues affecting those living with sickle cell disease, including sickle cell research, pain management, disease treatment, and other timely topics.

According to the NHLBI Website, in the United States, most people with sickle cell disease (SCD) are of African ancestry or identify themselves as black.

• About 1 in 13 African American babies is born with sickle cell trait.
• About 1 in every 365 black children is born with sickle cell disease.

There are also many people with this disease who come from Hispanic, southern European, Middle Eastern, or Asian Indian backgrounds. Approximately 100,000 Americans have SCD.

SCD can manifest in many forms: blockage of blood vessels, known as vaso-occlusive crisis; obstruction of smaller vessels by sickled red blood cells; pain crisis; injury to internal organs due to blockage of blood flowing to them which can cause ischemic injury. There are 6- more episodes per person annually. Other forms of pain and complication are :  malaria exacerbates effects of SCD; dactylitis; avascular necrosis; acute joint necrosis; abdominal pain; liver infarction; papillary necrosis; inability to concentrate urine ( isosthenuria); deep bone pain; fevers; acidosis; dehydration and chirped lips.  What does this inform us?

Some suggestions to make life better:

1. Improve on reception services for those in acute pain or crisis.

2. Establishing community and national awareness drives addressing SCD.

3. Anti-malaria drives such as universal practices ensuring no mosquito bites.

4. Provision of mosquito repellants and insecticides.
5. Provision of Folic Acid Supplements.
6. Translating literature on SCD in format that is understandable.
7. Providing education on partner testing for abnormal genes.
8. Training resource persons to provide care, management and interventions.
9. Establishing cultures fr testimony giving following Bone Marrow Transplants.
10. Provide opportunities for community engagement

SOURCES:

1. NHLBI

2. WebMD

3. NIH