In the United States, an estimated 100,000 people live with sickle cell disease. For most, living with SCD, an inherited disease 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. And with annual health care costs that can reach into the tens of thousands, for many, how can one see hope, when no horizon is yet to be found? Or in this case, be afforded.
What Is Sickle Cell?
Sickle cell disease is a genetic blood disorder, meaning it is passed down from parents to child. The name sickle cell is derived from the very characteristic of the disease being sickle-shaped red blood cells. 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 more of 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. Sickle cell is an inherited disease that is passed down from parent to child. A genetic disorder, sickle cell is not contagious.
For those who are carriers of the sickle cell gene, they either have sickle cell disease or have sickle cell trait. Sickle cell trait, or SCT, is not a disease and people who carry it generally experience no symptoms. Carriers of sickle cell trait don’t have the disease, but as a carrier, can pass it on to his or her 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?
With a sickle cell crisis, pain that ranges from moderate to severe, is a result from red blood cells becoming obstructed within blood vessels. This is called a vaso-occlusive crises. 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. Coincidentally, in 1992, Brookoff also conducted a study focused on treating sickle cell pain like cancer pain. In this, he concluded that a pain-control program modeled on regimens used to treat chronic cancer pain reduced hospital use by adult patients with sickle cell pain.
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
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 gene defect that could lead to sickle cell disease in the baby. For certain, early diagnosis is beneficial.
What Is The Life Expectancy For SCD Patients?
According to Kathryn Britos-Swain, a state sickle cell coordinator, the median life expectancy for SCD 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, or as it is also known, 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
Sadly, only two medications have been developed to treat SCD: hydroxyurea and Endari. 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.”
Is There Hope On The Horizon For Sickle Cell Disease Patients?
In February 2018, Rice University provided an update on progress in pursuit of a sickle cell cure where Bioengineers used gene editing to correct the mutation responsible for sickle cell disease in up to 40 percent of patients’ cells used for lab testing. In the report, Rice University bioengineer Gang Bao states, “Scientists have successfully used gene editing to repair 20 to 40 percent of stem and progenitor cells taken from the peripheral blood of patients with sickle cell disease.” You can read the report from sciencedaily.com here.
But until that day comes, one thing is for sure, for those who live with SCD, this is not a game. This is their life. And if you are a sickle cell patient, we at PSI want to tell you something, you are not alone.
Patient Services Inc., Providing A Horizon Of Hope
Dr. Dana Kuhn, the founder of PSI who shaped the very first non-profit patient assistance model in 1989, knows the pain firsthand of watching a loved one fight-and-lose their battle against chronic illness; he knows, too, the crippling financial burden often carried by those left behind.
Since our inception in 1989, Patient Services has been a pioneer leading the charge to find solutions to the challenges that face the chronically ill in the United States. We provide financial support and guidance for qualified patients with specific, rare chronic diseases. Through PSI assistance programs patients and their families rediscover hope and health.
Patient Services, Inc. continues to provide peace of mind to patients living with specific chronic illnesses by providing financial assistance to eligible persons. To learn more about our types of assistance, click here. You can also learn about the various programs PSI has available based on your type of insurance as well as our supported illnesses by clicking here.