Personal Story: Shantá

Shantá's story

Shantá and her husband Derek are the parents of three sons, two of whom have sickle beta thalassemia +, a type of sickle cell disease (SCD): their 21-year-old son Khari and their 19-year-old son Mikaili. Their oldest son, Ryan, is 31 years old and Shantá’s son from a previous marriage; he does not have SCD.A

"When my first son was diagnosed, I had the most naïve reaction. But with my second son, we knew it was a possibility," said Shantá. "My husband's brother had sickle cell disease, so we knew he had the trait. I had thalassemia minor and never had any problems growing up. It never occurred to us that our kids would have sickle cell disease. It was a surprise. I got a letter from the state saying my child has sickle cell disease. I read the information – that scares you. As a mom, I went through every emotion. I wanted the best for my baby. I wanted to learn what to do to make sure my child has the best care, is managed well, and how to protect my children."

"My husband and I never considered genetic counseling," she continued. "It didn't occur to us. We thought we should be good. At most, we thought our kids might have sickle cell trait. In hindsight, we should have had more conversation around that. We should have found out what type of sickle cell disease my husband's brother had and I should have looked at my own family history as well. But regardless, we are in a space of total gratitude for having our children. This information would not have changed our decision to have children together."

Finding resources

Shantá first learned her children had SCD from state newborn screening results. At the time, most of the available information and resources about SCD was in hard copy form. She found information on genotypes, management and care, the importance of seeing a hematologist, vaccination information and the importance of starting penicillin early, as well as information on the importance of breastfeeding to build health-protecting immunities. While Shantá found these resources helpful, what was most helpful was having access to the expertise provided by her child's hematologist, Dr. Keith Hoots, and the stakeholder (interested groups) community.

"I was fortunate to know many hematologists who are top notch in sickle cell disease management [through my work and my husband's work in the healthcare industry). They let me know that sickle cell disease is manageable and gave us hope. They taught us what penicillin meant to disease care, as well as the importance of treatment compliance, hydration, and a healthy diet, which helped us develop good habits early on."

When they moved to Maryland, Shantá and her family also found support from the sickle cell community by getting connected to, and taking part in, activities sponsored by the Sickle Cell Disease Association of America (SCDAA). In 2006, Shantá and her husband co-founded their very own community-based organization, the Maryland Sickle Cell Disease Association (MSCDA), and began providing services to help and support other families like them.

Community work

Shantá and her husband are very involved in the sickle cell community. They do a lot through MSCDA and have recently helped develop Maryland state guidelines (recommendations) for the management of SCD in schools. They are also working with major teaching institutions in their area to connect people to specialty providers. In addition, they connect people to resources, hold an annual patient and provider conference to discuss relevant sickle cell topics like eye care and the Cure Initiative, and have formed grass roots support groups.

According to Shantá, "The future of sickle cell disease is extremely bright. It's motivating to see how much is being addressed on different fronts and among different stakeholder groups. By continuing to work very closely together as a sickle cell disease stakeholder consortia, we can get even more done."

Challenges

Staying healthy in school

To help her kids stay healthy in school and to make sure appropriate care would be available, if and when needed, Shantá asked the school principal to hold an in-service training session with teachers, coaches, and nurses. She worked with nurses at Johns Hopkins University, who came to the school and educated staff about SCD, including why hydration is so important, why pain complaints should be taken seriously, and what to do if the child has a fever.

Transition

Transition is the process of young people learning to become more responsible for their health, including transferring their care to an adult healthcare provider. The biggest concerns Shantá had regarding transition were around the loss of control over her sons' treatment compliance and health choices. When her children were young, Shantá would cook for the family, making sure they got what they needed. For example, she would cook with turmeric and other spices to reduce inflammation (swelling). But when they grew older and more independent, she often wondered whether her kids were taking their medications and getting enough rest and proper nutrition.

According to Shantá, "For the person transitioning, it's a big move. They need help with this. Parents can benefit from information on how to handle this change."

To help with this in her own family, she would check in with her sons to make sure they were doing what they needed to be doing (such as taking their medication) and issue helpful reminders. For example, she would remind them of upcoming doctor appointments, as well as remind them, about a week before their medications were due to run out, that they would need to call the pharmacy to request a refill.

Impact

When asked how SCD has impacted her family's life, Shantá said, "Sickle cell has been a part of our lives, part of our family dynamic and norm for so long. The demands of sickle cell disease are unpredictable. I've had to get comfortable with that in terms of the plans we make (for example, vacation, events). Planning a vacation without travel insurance is not an option for us."

The most difficult thing for Shantá was the challenge of caring for two children with SCD who required very different approaches to care and management due to how the disease uniquely affected each child. Her youngest child, Mikaili, had more issues with pain crises, and eventually had to have surgery for avascular necrosis (bone tissue death). Her middle child, Khari, has only had one crisis to date, which was due to lack of adequate hydration during a basketball practice, but has experienced retinopathy issues (eye damage). Shantá handled these difficulties with grace by prioritizing "what needed to be done at that moment," by "not taking too big of a bite," and by "taking challenge by challenge—one thing at a time".

Shantá acknowledges, however, that this has not always been an easy task, and that sickle cell has had a profound impact on her family's life. "Our faith in God is core to everything we do. Practicing our faith keeps us grounded and focused on managing the current challenge.

"Sickle cell has had an impact on every aspect of our lives. Our work life, our personal life, we even socialize in the arena of sickle cell disease if some [sickle cell] events are being held. Now that my boys are young men creating their own lives, I am working on defining my life and maintaining a healthy balance.

In many ways, though, sickle cell has had a positive influence in their lives. "It has made us a well-connected family. It's made us closer. When something happens, we have an unspoken, informal process of sharing information with each other, and we work together."

Advice for others

Shantá's tips for caregivers and family members of someone with SCD:

• "Arm yourself with knowledge and education about sickle cell disease on every level that you can. Position yourself as an expert."
• "Connect with the medical, research, and sickle cell community-based organizations and become involved."
• "Take care of yourself. If you wear yourself down, you will not be there for the ones you love the most. You won't be able to give them the care they need."

Where they are now

Shantá's oldest son, Ryan, is a young professional living and working in Seattle. Her middle son, Khari, is an undergraduate studying theatre and acting at Towson University in Towson, Maryland. And her youngest son, Mikaili, is an undergraduate student studying clinical psychology, with an interest in sports psychology, at Hood College in Frederick, Maryland.

CDC would like to thank Shantá for sharing her story.