Living with sickle cell disease, or sickle cell anemia, a specific form of sickle cell disease, is a reality for thousands of people across the world.
September is National Sickle Cell Awareness Month, which is recognized by Congress to help focus attention on the need for research and treatment of sickle cell disease.
In the United States, an estimated 70,000 to 100,000 people live with sickle cell disease.
For most living with sickle cell disease, an inherited condition with only one potential cure, life can be a game of survival in seeking to navigate pain episodes, infections and various complications that frequent their path every single day.
With annual health care costs that can reach into the tens of thousands, how can one see hope on the horizon?
What Is Sickle Cell Disease?
Sickle cell disease is defined as a genetic blood disorder, meaning it is passed down from parents to their child. The name sickle cell is derived from the sickle or C-shaped red blood cells, an important disease trait.
Taking on this unique shape, these sickle red blood cells have a challenging time navigating through blood vessels.
These sickle cell blood cells are further characterized by being hard, sticky and stiff in contrast to normal red blood cells which are round, soft and flexible, allowing for an easier time maneuvering through blood vessels.
How Does A Person Get Sickle Cell Disease?
In order for someone to get sickle cell disease, each of their parents must carry the sickle cell gene or trait. Sickle cell is an inherited disease that is passed down from parent to child.
For those who are carriers of the sickle cell gene, they either have sickle cell disease or have sickle cell trait.
Sickle cell trait (SCT) is not a disease and people who carry it generally experience no symptoms; however, they can pass it on to their children.
What Happens To A Person With Sickle Cell Anemia?
As a blood disorder, sickle cell disease can cause complications in any part of the body, most commonly anemia, infections and sickle cell crisis.
What Is A Sickle Cell Crisis?
A sickle cell crisis is pain that ranges from moderate to severe and is a result of red blood cells becoming obstructed within blood vessels. This is called a vaso-occlusive crisis.
These crises, or pain episodes, can last anywhere from minutes to months and can range from dull to excruciating. Generally, a sickle cell crisis will be treated in a hospital setting with various pain killers, such as narcotics.
According to Daniel Brookoff from Howard Smith’s 2009 book, Current Therapy in Pain, studies in both children and adults have characterized the vaso-occlusive crisis as having four phases, with the typical painful episode lasting an average of 10 days.
What Are The Symptoms For Sickle Cell Disease?
As a result of the uniquely sickle-shaped red blood cells, which block the flow of blood as it circulates through the tissues of the body, these tissues, due to the impaired circulation, suffer damage from lack of oxygen.
Various signs or symptoms of sickle cell disease can include:
- Episodes of pain
- Swelling of hands and feet
- Delayed growth
- Frequent infections
- Vision problems
- Diagnosis For Sickle Cell Disease
In most developing countries, screening is available for sickle cell disease shortly after birth.
Furthermore, pregnant women may also be offered a screening test as well, to test for the presence of a gene defect that could lead to sickle cell disease in the baby.
What Is The Life Expectancy For Sickle Cell Patients?
According to Kathryn Britos-Swain, a state sickle cell coordinator, the median life expectancy for one living with sickle cell disease is 42 for men and 48 for women.
Is There A Cure For Sickle Cell Disease?
For those with sickle cell disease, there is currently only one potential cure, and that is by way of bone marrow transplant, also known as a stem cell transplant. In light of this, for the sickle cell patient, treatment is generally aimed three different ways:
- Avoiding Sickle Cell Crisis
- Relieving Symptoms
- Preventing Complications
Coincidentally, according to the journal “Blood,” a 2013 study reported that cystic fibrosis, which affects 30,000 people across the United States, receives seven to 11 times more funding per patient than sickle cell disease.
What Is The Cost For Health Care For People With Sickle Cell?
In a report written by Carolina Henriques for Sickle Cell Anemia News, Henriques writes, “Another challenge is the cost of sickle cell treatments. There are elevated medical costs associated with the disease at around $1 million for patients who reach 45 years, with annual costs of $10,000 for children and $30,000 for adults. These costs may cause some patients to discontinue treatment.”
What Is PSI Doing For Sickle Cell Patients?
We are doing everything we can to assist those living with sickle cell disease, which includes in-house programs and advocacy efforts.
Sickle Cell Budget Amendment
After PSI’s efforts and the crucial help from Sen. Lionell Spruill (D-VA) and Delegate Dawn Adams (D-VA), the Virginia State Legislature voted in favor of a $500,000 sickle cell disease patient assistance program in March. We are looking forward to assisting patients with sickle cell disease.
The Virginia Department of Health will administer the program, which provides health insurance premium assistance and cost-sharing assistance for sickle cell disease patients who do not qualify for Medicaid.
In turn, PSI will work with the department in providing cost-sharing assistance to sickle cell disease patients and to help make this program a success by helping families obtain the new treatments and therapies to treat this long-ignored disorder.
As this program develops, be looking for more announcements from PSI.
What We Think About the Budget Amendment
This is what our director of government relations Jim Romano had to say about the budget amendment:
“PSI is very excited to work with leaders in the legislature such as Sen. Spruill and Delegate Adams to create a program to ensure families with sickle cell disease have access to coverage and the treatments they deserve.”
This is what our government relations and advocacy associate Nathan Thomson had to say:
“Sickle cell is a disease that disproportionately affects the African American community. For too long, this community has been marginalized and sickle cell has not received the proper attention from the medical community. Thankfully, there have been advances in the recent past to help treat this costly and painful disease. We would like to thank the state of Virginia for its leadership on this vital topic. By adopting this budget item and creating a new sickle cell program they will be allowing patients access to life-altering medication while simultaneously saving the commonwealth money.”
Sickle Cell Established Program
PSI also recently established both a private and public insurance sickle cell patient assistance program.
We are currently seeking funding, and look forward to serving the sickle cell population in the near future.
We Support the Sickle Cell Disease and Anemia Community
For those living with sickle cell disease and anemia, you are not alone. We support you and will fight for you.
If you’re affected by sickle cell disease, it can be helpful to find a support group.
Check out Sickle Cell Disease Association of America Inc.’s website for a list of member organizations you can join.