“Pain is everywhere throughout my body, stabbing, cutting, stinging, people do not know this pain, as long as I am living I am going to fight Sickle Cell, I am going to die eventually, but I will fight the pain, while I live. I get upset at people who don’t understand. I am not here to abuse pain medication. White folks especially don’t understand that I am in pain and I need medication. I refuse to be seen as a E.R. hopper. I have been dependent on medication since I was 13 years old, I am now 37, yes I am addicted to it, it takes away my pain.”

—Sickle Cell patient Morgan Taylor, Cedar Sinai Hospital ER

It is exactly 3:30 p.m. and Morgan Taylor is pulling into his driveway after receiving four hours of intravenous fluids and pain medication. He sounds like he is feeling okay, you can hear the energy in his voice.

This is a ritual he undergoes three to four days a week.

Taylor is one of the estimated 100,000 individuals suffering from Sickle Cell Disease (SCD).

According to the National Institute of Health (NIH), SCD is an inherited red blood cell disorder. People with SCD have abnormal hemoglobin, called sickle hemoglobin, (due to their shape resembling a sickle rather than their typical round shape. Hemoglobin in red blood cells carries oxygen throughout the body. The sickle shape can cause blood vessel blockages that are very painful and can deprive organs of oxygen leading to organ damage and death.

SCD is called a disease predominantly affecting people of African heritage, however it is also found in persons of Middle Eastern, Indian, and Mediterranean heritage because those geographic regions are most prone to malaria. The gene variant for sickle cell disease makes the patient less susceptible to malaria.

In 1910, Dr. James B. Herrick, a physician at Presbyterian Hospital and professor of medicine at Rush Medical College in Chicago, Ill., published an article on the case of an anemic West Indian dental student, Walter Clement Noel. Herrick’s laboratory findings of the patient’s “peculiar elongated and sickle-shaped” red blood cells were the first description of sickle cell disease in Western medical literature.

The west has been dealing with fighting SCD for approximately 117 years and has only produced hydroxyurea, which treats sickle cell anemia by helping to prevent formation of sickle-shaped red blood cells.

West African countries have used indigenous concoctions of herbs and plants (plant therapy) to combat the disease long before the introduction of modern medicine, according to Ibrahim Muazzam, Ethnobotanist for the National Institute for Pharmaceutical Research and Development. This use of herbs continues today due to biomedical scarcity in rural communities and African countries suffering from civil war or no health infrastructure.

Muazzam stated that among the Hausa-Fulani of northern Nigeria, where sickle cell anemia is called “sankara-miji,” the disorder is perceived to be paranormal and incurable. Once a relationship between patient and healer is established herbs are used that have the same properties and impact on the SCD as found in western medicine, and although the patient is informed that sankara-miji (SCD) is incurable, there is a spiritual trust.

A plant found in Nigeria, known as West African pepper or Ashanti pepper, has naturally processed into the drug Niprisan, which reduces the production of sickle shaped red blood cells.

Researchers in Burkina Faso claim to have found a cure for SCD utilizing root bark extracts which have been used in West Africa for generations. Root bark extract acts as a vasodilator that widens the walls of blood vessels causing relaxation of smooth muscle cells within the vessel walls, and preventing a sickle cell crisis.

A specific regimen consisting of roots of the Fagara tree has been associated with producing fetal hemoglobin, similar to GBT440 which according to a spokesperson at Global Blood Therapeutics (GBT) is being developed as an oral, once-daily therapy for patients with SCD.

GBT440 is designed to work by helping hemoglobin hold onto more oxygen as the red blood cells travel through the body. This keeps red blood cells in their normal shape and helps stop sickling. The treatment is still in the clinical trial phase.

Pharmaceutical economist Alex Lash believes GBT440 could be the cure for SCD calling it “the biggest medical advance in over 100 years.”

Taylor says he’s heard of GBT440, but was informed by his hematologist that the drug would not enter the market until at least ten years from now.

According to the NIH, “While there is no widely available cure for sickle cell disease, there are treatments for its symptoms and complications. Over the past several decades, scientists and doctors have learned a great deal about sickle cell disease. They know its causes, how it affects the body, and how to treat many of its complications. Thanks to improved treatment and care, people who have sickle cell disease are now living into their 40s or 50s or longer. The NHLBI continues to support efforts to find new and better treatments for sickle cell disease.”

In 2009, scientists discovered that a modified transplant of adult blood stem cells could improve SCD in 9 of 10 adults who had been severely affected by the disease. The research, carried out at the NIH Clinical Center in Bethesda, Md., was a milestone in the search to cure SCD. Dr. John Tisdale led the team of researchers.

In 2009, the National Heart, Lung and Blood Institute (NHLBI) launched an initiative called Exploratory Studies in the Neurobiology of Pain in Sickle Cell Disease. Studies funded under the initiative allow experienced pain researchers to learn more about the biology of pain in sickle cell disease and to lay the groundwork for the development of effective drug treatments. One study is examining whether African American adults with sickle cell disease have different ways of experiencing pain compared to matched healthy adults. Other studies are evaluating pain perception in animal models of sickle cell disease.

In 2010, a study found that neurologically normal adults with sickle cell disease scored lower on tests of brain function than neurologically normal adult participants who did not have sickle cell disease, suggesting that the disease may affect the brain more than previously thought. This finding was part of the first study to examine brain function in adults with sickle cell disease and was funded by the NHLBI. A second study is ongoing to evaluate the impact of a new treatment on brain function.

Some children with the disease have been successfully treated with blood stem cell, or bone marrow transplants. This approach, though, was thought to be too toxic for use in adults. High doses of chemotherapy are used to destroy all of a child’s bone marrow, which is then replaced with marrow from a donor. Stem cell recipients typically need to take immunosuppressants for months to a few years. These medications can cause serious side effects.

Physicians at the University of Illinois Hospital & Health Sciences System have cured 12 adult patients of sickle cell disease using a unique procedure for stem cell transplantation from healthy, tissue-matched siblings.

The transplants were the first to be performed outside of the NIH campus in Maryland, where the procedure was developed. Physicians there have treated 30 patients, with an 87 percent success rate. The results of the clinical trial at UI Health, in which 92 percent of treated patients were cured, are published online in the journal Biology of Blood & Marrow Transplantation.

The new technique eliminates the need for chemotherapy to prepare the patient to receive the transplanted cells and offers the prospect of cure for tens of thousands of adults suffering from sickle cell disease.

The U.S. Food and Drug Administration (FDA) recognized the lack of incentive for pharmaceutical companies to develop cures for rare diseases and established the Office of Orphan Product Development. This branch of the FDA provides incentives to companies that work toward curing rare diseases by exercising the rights given to them under the Orphan Drug Act of January 1983. One type of incentive is the orphan drug status, which provides tax reductions and the exclusive right to develop the cure for a specific condition, for a period of seven years to companies attempting to cure rare diseases.

The pharmaceutical industry is big business and the largest amount of money is made by developing drugs that patients may be dependent on for numerous years if not a lifetime.

Pfizer Inc. and the National Newspaper Publishers Association (NNPA), a trade association of more than 200 African-American–owned community newspapers from around the United States, are collaborating to raise awareness of SCD according to Steven Danehy, media contact for Pfizer Inc. According to a press release, Pfizer states the following: “Our commitment at Pfizer goes beyond clinical research to supporting the rare disease community through innovative collaborations. Working together, with the NNPA and Howard University, we hope to improve awareness and ultimately address the unmet medical needs of sickle cell disease patients.”

Taylor is hopeful of the work Phizer and the NNPA are doing. “Awareness leads to support, if my family members know more, they will become aware of my illness in depth. They will better understand me and the pain I experience. A lot of times a support group will make you stronger and a knowledgeable relative enhances their ability to support a patient. The more that individuals are aware of the disease the less lonely those of us who are suffering will feel.

Clinical trials have been a struggle for scientists. In a review of 174 sickle cell disease trials, difficulty enrolling patients was the stated cause in nearly half of the 30 percent of the trials that were terminated early.

Taylor’s response to this issue, “What for? Why does it have to be me? I do not want to be experimented on. The only person I trust is my doctor who has been treating me for most of my life,” he said, mirroring the distrust that so many in the African American community have for many medical professionals.

Taylor stated, “Lastly, lots of White hematologists should participate in these surveys and get even better informed because they are the ones who control our pain medication and some of them believe that African Americans can tolerate more pain than others, and therefore believe that we sometimes exaggerate our pain. I assure you that is not the case.”