The Sickle Cell Disease Center is leading the way in treatment with new and innovative approaches to caring for patients with sickle cell disease.
Dr. Sharl Azar, medical director of the Comprehensive Sickle Cell Disease Treatment Center at Massachusetts General Hospital (MGH), has hired a medical exercise expert to work on a fitness program that will help improve the lives of his patients.
“One of the myths about this disease has been that people living with sickle cell disease cannot or should not exercise, because exercise can worsen the symptoms of the disease.” Hazard told ABC News. “It can lead to the painful crisis that is characteristic of this disease.”
by Centers for Disease Control and Prevention According to the CDC, sickle cell disease is a disease of red blood cells in which an abnormality in the oxygen-carrying protein hemoglobin causes the affected person’s red blood cells to be abnormally shaped (sickle-shaped) instead of round. hold. Sickle cells die early and there is a shortage of red blood cells. It also blocks blood flow when it gets stuck in a blood vessel. Narrowing of blood flow can cause severe pain to the patient.
This harrowing crisis is all too familiar to Amy Diawara, 27, who has lived with sickle cell disease her entire life. She said her crisis felt like an excruciating throbbing pain buried deep in the muscles of her body.
Diawara, a patient at MGH, worked on a personalized training program with medical exercise specialist Jen Miramontes in preparation for running the Boston Athletic Association Half Marathon on Nov. 12.
“You need someone who wants to run with you to the finish line, and I’m so grateful to Jen and Dr. Azar,” Diawara told ABC News. “They encouraged me to get this far, and I’m so excited to run the Boston Half this Sunday.”
Miramontes, 59, not only trains Diawara, but he also agreed to run the race with her. She has competed in a total of 80 marathons throughout her life and has created her training program specifically tailored to Diawara’s needs.
“For the first two weeks, I’m going to stick to one mile three times a week,” Miramontes told ABC News, adding that she had to make a careful mistake while training for Diawara. “On the other hand, if we had trained someone who didn’t have sickle cell disease, the process to get to where we are now would probably have been two to three times faster for him. [today]. ”
Miramontes said other factors that Diawara had to be careful of were extreme heat and cold while running, as dehydration and extremely cold temperatures can cause a crisis. To avoid that, she had Diawara run on a treadmill for most of her training.
Azar said Miramontes was hired by the hospital through a grant. She was chosen to provide medically approved fitness programs tailored to each patient she sees. Jen, who lives in California, flies to Massachusetts monthly to evaluate patients and create exercise plans that meet each individual’s needs and goals. After that, Azar said, she remains in constant communication and guides patients through virtual care.
Scientists have known about the genetic causes of sickle cell disease for more than 100 years, Azar said, but population studies on the disease are limited to other chronic congenital diseases such as cystic fibrosis and hemophilia. It is said to be far behind. Azar said this is because sickle cell primarily affects people of color and, as a result, there are not enough resources to research the disease.
by CDC, 100,000 people live with sickle cell disease in the United States, but the data hasn’t been properly updated in decades, Azar said. One in 365 African-American girlfriends is born with the disease, and 1 in 13 African-American girlfriends are born with the trait inherited from one copy of the sickle cell gene. Masu. Azar says if you scale those numbers up to today’s African American population, the number would be well over 100,000.
“Embarrassingly, in 2023 we are putting together the first program to investigate the role of exercise in patients with sickle cell disease.”
Most people suffering from the disease have always been told not to exercise too much due to the risk of danger, but Diawara and Miramontes are bucking that trend.
“I was shocked and very disappointed to learn that there is so little research on exercise and sickle cell disease,” Miramontes said. “If you look into cancer and MS, [multiple sclerosis], or Parkinson’s disease, if you try to find research on how fitness affects the disease and how it positively impacts the disease, you’ll find thousands of studies. I found two [for sickle cell]. And at least it’s certainly groundbreaking. ”
Azar said the Food and Drug Administration is expected to approve the first gene therapy product in December to help improve the lives of people with sickle cell disease. As for Diawara, he does not think he will undergo gene therapy because he is unlikely to be in any more critical condition. She is more focused on training and achieving her goal of running her first half marathon.
“Again, this is something I never thought I would be able to do, so I’m really excited,” Diawara said. “It was always just a dream, but now it’s like a real goal that’s about to be achieved.”
