Moving from childhood to adolescence into adulthood brings its own milestones, freedoms and responsibilities of growing up. For a young person with a chronic or an inherited health condition, such as sickle cell anemia, the transition can be overwhelming going from pediatric care to adult health care. That transition is being made smoother by the Adolescent and Young Adult Transition Clinic for Sickle Cell Disease, located at Christian Hospital and the Center for Advanced Medicine.
“As they are getting older and going into that adult side, we have found that that’s when the mortality and the morbidity start to rise,” said pediatric oncologist Cecelia Calhoun, M.D. She and Allison King, M.D. treat young adult patients at the transition clinics.
“Transitioning to an adult as a teenager is hard, and then you add on a chronic disease – one that causes pain, one that has very limited therapies and cures – it’s a challenging space for our teens,” Calhoun said. “We are trying to capture as many patients as we can, because a lot of people fall off. Consequently, they get a lot of their care in the emergency department, which is not good.”
Transition readiness is not just an age, she said. Under pediatric care, children become more actively involved in conversations about their care as they age, making sure that youth understand their medications – even as early as 12 years of age. Using patient input from a transition readiness assessment form from the American Society of Hematology and the patient’s full medical history, doctors determine if the teen is at the right the maturity level to go to the adult clinic. That could be at age 18 for some young adults and age 20 for others.
“We make sure they feel comfortable talking on their own and are mostly comfortable with tasks that are necessary for self-management,” Calhoun said. She said the transition clinic also sees young adult patients who were in pediatric care at other health facilities as well. When young adults visit the clinic, they learn expectations and set goals for their managing their own care. The transition clinic sees 18- to 26-year-olds.
Sickle cell disease, diagnosed during infancy through newborn screenings, is an inherited blood disorder that occurs when normally round red blood cells are sickle-shaped and don’t carry oxygen as well throughout the body. The sickled cells sometimes gather and become lodged in smaller blood vessels – causing extreme pain episodes and blocking blood flow, which can lead to serious health complications – higher risk of stroke, organ damage and shorter life spans.
Sickle cell disease is not limited to painful episodes. Calhoun said inside the body, sickle cell anemia damages the organs and affects brain cognition.
“The most common presenting complaint is pain, but sickle cell patients are at super-high risk for stroke,” Calhoun said, adding that seeing an 8-year-old girl who had a stroke and could not move one side of her body brought her into this field of medicine.
“We can’t just say you are turning a certain age, and ‘we’re good’ because we know that even if you have no changes on your MRI, you can have a loss in IQ points that can affect your quality of life – and all because of your sickle cell disease – not because you have any other thing going on.”
A child is born with sickle cell disease if he or she inherits the sickle cell gene from both parents. If each parent carries the sickle cell trait, the child has a one in four chance of having full-blown sickle cell disease. It is more prevalent among persons of African, Middle Eastern and Mediterranean descent.
Monica Hulbert, M.D., a pediatric hematologist at St. Louis Children’s Hospital, said treatment and care for persons with sickle cell anemia has improved over the years, changing the approach to treating pain episodes by using maintenance medication to prevent them altogether or to at least lessen pain crises.
Hulbert said, “Since 2014, it’s recommended that all children with severe manifestations of sickle cell disease should be treated with a medication called hydroxyurea, which is a daily oral medication that kids can take at home that reduces the likelihood of the red blood cells to sickle, reduces the chances of having a pain crisis, and also helps to protect their organs – like their kidneys, their lungs and their heart.”
Hulbert said there are other treatments in development that are options for some patients with sickle cell disease.
“Some patients get blood transfusions regularly, some patients can even be eligible for a bone marrow transplant, and now we have gene therapy as a potential treatment that is still in research phases,” Hulbert said.
Calhoun said the transition clinic is a space where young people living with sickle cell can get knowledge, gain independence and learn how to take the best care of themselves.
Hulbert said progress is being made for persons living with sickle cell disease.
“We are able to offer much better care than what was around 20 or even 10 years ago,” Hulbert said. “And with everybody – researchers and physicians who take care of patients and patients and families themselves – we are all able to work together to make some progress and help people live longer and healthier with better quality of life.”