Katherine Kay Blevens chooses to celebrate the purest joys in her life – husband, children and grandchildren, despite being diagnosed with sickle cell disease as a baby. When she was a teenager, she was told that she would not live past 21.
The reason why sickle cell disease carries such a grim prognosis is because the red blood cells in patients with the disease are markedly abnormal. Normal red blood cells are very pliable. They can travel through the narrowest of blood vessels to ensure that oxygen, which is carried on hemoglobin, the red pigment inside the cell, reaches all the tissues in the body. In sickle cell disease, the oxygen carrying pigment is abnormal and the red cells are rigid. Their shape is deformed. As a result, travel through the smaller blood vessels is restricted and tissues are starved of oxygen. For some sickle cell disease patients, tissue oxygen starvation can cause chronic pain while for others there are pain crises when, as Katherine describes it, “the pain goes from a hammer to a jack hammer”. The emotional burden of a disease characterized by chronic pain and the uncertainty of when another crisis might occur is inescapable.
Katherine’s fortitude, her reliance on her faith and the support of family, doctors, and nurses have combined to ensure an outcome that nobody would have predicted when she was first diagnosed, 50 years ago, with sickle cell disease.
Care for Sickle Cell Disease
While cures for sickle cell disease are still under investigation, Dr. Inna Shmerlin, who is Katherine’s hematologist at Methodist Health System, frequently orders red cell exchanges in the management of patients with sickle cell disease.
Red cell exchange is effective because the patients’ abnormal cells are replaced with healthy red blood cells from volunteer donors. This takes about two to three hours and may require up to 12 units of donated red blood cells. At best, these transfused red cells will live their normal life span of 120 days. During that time, the sickle cell patients’ abnormal cells, which were discarded during the exchange, will start accumulating, oxygen delivery to the tissues will be impaired, and pain crises could be imminent.
Dr. Shmerlin ordered Katherine’s first exchange more than a year ago and Katherine is undergoing this procedure now every other month*. “It gives me better quality of life with my family”, said Katherine. She reports that she has only had one crisis that necessitated an emergency room visit since she started exchange treatments.
Katherine will soon celebrate her 53rd birthday. Despite sickle cell disease, she said there are too many things to smile about. “Whatever comes, we have to deal with it head on”, she said. She is grateful to be a wife, a mother, and a grandmother.
*Katherine receives her red cell exchange transfusions from Carter BloodCare’s therapeutic apheresis program. Due to regulatory considerations, a current, signed contract is required to initiate apheresis services with Carter BloodCare. In addition, Carter BloodCare’s technical or nursing staff must be credentialed to enter and work in your facility. If these arrangements are not in place, emergency privileges can be established for professional staff performing the procedure. Call 972-788-0650 for more information.