Sickle Cell-001

Singer Tionne “T-Boz” Watkins, member of the ‘90s girl group TLC has lived beyond the years her physicians predicted with sickle cell disease, and has even been able to enjoy motherhood, which the condition compromises greatly.

When Tionne “T-Boz” Watkins, one-third of the girl group TLC, announced a decade ago that she suffered from Sickle Cell Disease, those with an understanding of the condition counted her fortunate.  Characterized by fatigue and excruciating pain, Watkins, like most with the disease, had not been expected to live past age 30.  Now living comfortably, in her 40s, Watkins is among the millions of African-Americans living with an inherited, genetic condition that has proven devastating to those living with it.

Sickle Cell Disease is a group of disorders that impact hemoglobin, the molecule in red blood cells that delivers oxygen to cells throughout the body. The cells turn sickle shaped, compromising oxygen flow to various parts of the body and causing irreparable damage to organs.  Organ failure and death can occur as a result.

And while a concerted effort to test and bring awareness to the nation stymied in the late 1970s, sickle cell continues to impact the lives of 90,000 to 100,000 Americans – and approximately 1 in 12 African-Americans.  Sickle Cell Disease is a chronic, inherited condition for which there is no cure, and one that disproportionately affects those whose families come from Africa, South or Central America (especially Panama), Caribbean islands, Mediterranean countries (such as Turkey, Greece, and Italy), India, and Saudi Arabia.  So why do most Americans believe Sickle Cell Disease (SCD) is no longer an issue in the Black community?

According to Sophie Lanzkron, director of the Adult Sickle Cell Center and associate professor of medication at Johns Hopkins University in Baltimore, many Americans believe the condition has been eradicated largely due to the success of early detection rates.

“The biggest misunderstanding is that if it is not in the public view anymore that it is not still a chronic and terrible condition,” Lanzkron said, noting the aggressive grade school screening process that took place in the region in the 1970s was less of a necessity with the advent of mandatory newborn screenings.  “The newborn screenings are amazing and allow each child screened to be followed until age 6.”

Though newborn screenings have become universal and keep a child plugged in to health agents, Lanzkron said that those parents who are initially notified that their children are carriers of the trait, while not having the disease, are not necessarily following up on this information later.  The inherited, genetic disease occurs when two people who carry the trait for sickle cell produce a child who develops the disease.

“If you have traits and got a letter, but have not communicated it to your child, no one knows that child is a carrier for the condition until they get married or during the childbearing years,” Lanzkron said.  “Too often, adults are unaware they have sickle cell traits until after pregnancies are confirmed, which is too late to keep the unborn child from being impacted.”

According to data from the Sickle Cell Disease Association of America, the most common signs and symptoms of SCD are linked to anemia and pain. Other signs and symptoms are linked to the disease’s complications, and include shortness of breath, dizziness, headaches, coldness in the hands and feet, paler than normal skin or mucous membranes (the tissue that lines your nose, mouth, and other organs and body cavities) and jaundice (a yellowish color of the skin or whites of the eyes).

Watkins, who began to feel sharp, dagger-like pains in her arms and feet around age 8, said,

“It would stop me from walking if the pain was in my legs or feet; if it was in my hands, I’d be unable to hold a pencil or glass…that’s a crisis.  When a crisis happens your blood clots and they have to give you IV fluids to flush your blood so you can get oxygen to your organs.”

Almost all people who have sickle cell experience painful crises at some point in their lives. Some have these crises less than once a year. Others may have crises once a month or more. Repeated crises can damage the bones, kidneys, lungs, eyes, heart, and liver. This type of damage happens more often in adults than in children.

In 1996 during the CrazySexyCool tour, Watkins passed out on stage.

“I chose to be in a profession where I stayed stressed and never ate right, so it took a toll on my body.  I never made it through a tour where I did not end up in the hospital.  You know we danced hard and sometimes my lungs were weak,” said Watkins, who claims her body was just worn out.

“I was tired and my body gave out.  I’ve been through so many things including her heart almost stopping.  Our immune systems are really low.”

Watkins, according to sickle cell experts, took many unnecessary risks with her condition that could have proven fatal.  In addition to putting her body through rigorous pacing, despite the pain she experienced, Watkins’ oxygen levels and general health had been compromised.  Too often, even among average sickle cell patients, needless risks are taken.

“The problem is that with sickle cell anemia, the person needs more iron, and they need to listen to their bodies.  There is not much that can be done to fix the condition, but it is important that through dietary and lifestyle recommendations, infections, some issues like anemia can be addressed.  Each patients’ care should be specialized and individualized,” Lanzkron said.

Medications used to treat sickle cell anemia include: antibiotics therapy, which are administered to children with sickle cell anemia may begin around 2 months of age and continue taking it until 5 years old, to ward off infections like pneumonia.  Hydroxyurea (Droxia, Hydrea) Therapy is often quite effective, when taken daily, in reducing the frequency of painful crises and need for blood transfusions. Hydroxyurea seems to work by stimulating production of fetal hemoglobin — a type of hemoglobin found in newborns that helps prevent the formation of sickle cells.

“Hydroxyurea may be the first drug to effectively address SCD and is the first FDA-approved medication for this genetic disease. There is no other drug to offer patients.  The only other options are transplants,” Lanzkron said.

Current therapies for adults include: blood transfusions to increase the number of normal red blood cells, supplemental oxygen to help with acute chest syndrome or crises; stem cell transplants, also called a bone marrow transplant, wherein, bone marrow affected by sickle cell anemia is replaced with healthy bone marrow from a donor. Healthy stem cells from the donor are filtered from the blood. The healthy stem cells are injected intravenously into the bloodstream of the person with sickle cell anemia, where they migrate to the bone marrow cavities and begin generating new blood cells.

Lanzkron said that as therapies improve the life expectancy of those living with SCD has increased from about 17, to the late 40s, accommodations are being made to better understand how the disease effects the lives of military personnel, college and professional athletes, and those women who were told they could not manage pregnancies.