Youth-focused solutions are desperately needed as the transition from pediatric to adult care can make or break a patient’s successful management of the disease. The disorder’s symptoms are cumulative, and mismanagement of the disease can lead to drastically diminished outcomes and life expectancy for young adults.
Overall, the lives of people with sickle cell disease are typically 20 to 30 years shorter than those of people who do not have it.
And with a nationwide focus on opioid abuse, adult patients often encounter skepticism when they seek pain relief at the emergency room.
St. Jude offers a transition program to introduce teen patients to local adult care providers and set them up for success in ongoing treatment. The program includes mentoring and support groups, a venue for teenagers to process their fears about the future.
And death is the issue that they’re thinking about. At 16, 17, they’re thinking about dying. We want to allow children to know that there are people with sickle cell disease who live longer and who live successfully. But we also want to find out why, in their mid-20s, that the mortality and morbidity rate increases. Nobody really knows the answer to that question.
To get those answers, St. Jude is conducting a comprehensive cohort study that follows patients with sickle cell disease from birth to end of life.
“We look at all of the consequences of sickle cell disease in the body, all the things that could potentially be damaged from the disease,” said Jane Hankins, M.D., associate member of the St. Jude Department of Hematology.
The Bridges girls, Khirsten and Kaitlyn, take medication daily, and when pain crises arise, they are hospitalized and receive blood transfusions. Things that might seem like an afterthought for most, like staying hydrated and getting enough sleep, are also critical tools for staying on top of the disease and preventing pain crises.
As much as St. Jude is doing to advance cures and treatment for sickle cell disease, Nichole and Chris have to prepare their girls for the day when they will leave the hospital’s care, and they are starting early. For Nichole, that means arming her daughters with medical terminology and teaching them to advocate for themselves.
Until then, the Bridges are prepared, and wait hopefully.
“What we try to instill in our daughters every day is, ‘You know, I can have sickle cell and I can overcome. Even through the pain I can make it through this and make it just one more day,’” Nichole explains. “But ultimately we are praying for a cure.”
Through funds provided by the Legacy Grant, researchers at St. Jude will also pursue a health-worker education program in Nigeria.
Globally, more than 300,000 babies are born with sickle cell disease every year, and Nigeria accounts for roughly 50 percent of these births. In the U.S. and other high-resource countries, testing all newborns for sickle cell disease has dramatically reduced the number of children who die from the disorder, and approximately 99 percent of children born in the U.S. survive into adulthood.
In Nigeria, 50 percent to 90 percent of children who are born with sickle cell disease die before reaching their 5th birthday. Most of these deaths are preventable by simple interventions such as family education, vaccinations, giving children penicillin to prevent infections and starting medication early to prevent malaria.
To make a global impact on sickle cell disease, St. Jude Global and the Department of Hematology are working with Nigerian doctors to implement a newborn screening program to ensure children born with sickle cell disease in Nigeria receive these proven interventions.
“We want to implement the simple but effective lifesaving therapies, pioneered in the U.S. and at St. Jude, for newborns with sickle cell disease in Nigeria,” said Jeremie Estepp, M.D., assistant member of the St. Jude Department of Hematology.
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