Just three days after becoming a new mother, Rosa received the letter in the mail that would change everything.
She sat down and cried. Fear. Guilt. Tears.
Rosa had no idea she carried the sickle cell trait. She had never had a sign. People with only one sickle cell gene generally have no symptoms. But when a child inherits a sickle cell gene from both parents, he or she develops sickle cell disease.
Jamira, her beautiful new baby girl, tested positive for the blood disorder. Rosa knew nothing about the disease and felt the need to educate herself. Johns Hopkins All Children’s Hospital has helped along the way. For virtually all of her 16 years, Jamira has been treated by the sickle cell team at the hospital.
Early on, Rosa was given a sickle cell explanation that made sense: normal cells are round, sickle cells have sharp edges that get caught and bang into organs as they travel through the body. It creates havoc and can cause damage off and on throughout a person’s life. It affects about 100,000 people in the United States, most commonly African Americans, according to the Centers for Disease Control and Prevention.
“Sickle cell is the most common genetic disorder known,” explains E. Leila Jerome Clay, M.D., director of the hospital’s sickle cell program
. Clay has had the benefit of being mentored by George Dover, M.D., former pediatrician-in-chief of the Johns Hopkins Children’s Center in Baltimore, Maryland, and director of hematology at the Johns Hopkins University School of Medicine. Dover is a pioneer in the area of pharmacologic manipulation of fetal hemoglobin—which can greatly benefit sickle cell patients—and has made many substantial contributions to the field while at Johns Hopkins. Clay is pleased to be working closely with Dover and the Baltimore campus on finding ways to improve the treatment and success of sickle cell patients such as Rosa’s daughter, Jamira.
Sickle Cell in the Jackson Family
Rosa knows now that there are triggers to look out for in Jamira. Things to avoid. Ways to plan. Rosa was overwhelmed at first, but she said out loud what she has been saying ever since to anyone who will listen: “There will be no negative talk in this house.”
Rosa’s older daughter, Jasmin, a teen when Jamira was born, turned out also to carry sickle cell trait. Jasmin has a daughter of her own named Antonesha, now 9, who was born with sickle cell disease.
That’s four members in the same core family connected by sickle cell. Four strong, brave, stubborn females.
“This is a family affair,” Jasmin explains. She watched her sister go through it, now her own daughter. Both are treated at Johns Hopkins All Children’s. “We all pitch in to lift each other up because one day they are going to beat this.”
“People with sickle cell disease have normal intelligence just like we see in Jamira,” Clay explains,
“however, with the sickle damage that can cause in the brain, they may have some cognitive deficits and may need some support in school. Jamira uses our tutoring services here at the hospital to help her progress in school. We are fortunate to have school teachers available to us for her to take this advantage. Many sickle cell programs do not have such a program available to them. Education is important for young kids like Jamira. People with sickle cell disease achieve all goals they have in life. There are scientists, doctors, lawyers, educators, advocates, entertainers with sickle cell disease.”
A Little Help Along the Way
Rosa suspected Jamira was struggling, but she was too proud, or maybe too shy, to ask for help. Rosa requested some testing and was not surprised to find out the effects of the disease had taken some toll on Jamira’s learning. They were immediately connected to the Patient Academics Services team, part of the Child Life program
at Johns Hopkins All Children’s, where Jamira began a customized plan to help her accelerate in class. Her instructor, Annette Pagliaro, was determined to get her back on track and even went as far as visiting Jamira’s school to find out where she needed the most help.
The gesture brought Rosa to tears. “I just can’t believe how much people at this hospital, like Annette, have gotten involved and really given us incredible support for Jamira and now Antonesha,” Rosa recalls.
“I work with Jamira several times a week on math, reading and anything she needs help with,” Pagliaro explains. “We are here to do whatever it takes to make these kids successful and I often meet with teachers to put together a plan. I am seeing Jamira’s commitment to tutoring and it is definitely improving her self-confidence. That’s a win-win for me.”
Along with Jamira’s learning struggles, both Jamira, now 16 and her niece Antonesha, now 9, have faced medical issues and receive multidisciplinary care from hematologists, pulmonologists, cardiologists, neurologists and ophthalmologists. They get regular lab work done, scans and more.
“It’s a lot, but God placed them in the same situation in order to get each other through it,” Jasmin, Antonesha’s mom, explains in her determined way. It must come from Rosa. “I’m glad it turned out this way because it has brought us closer as a family.”
Rosa agrees. She’s seen it with the younger girls. They share appointments. “They both hate needles, so they are brave for each other,” she says, shaking her head. They are like a comedy team and goof their way through all those appointments. “They make it fun. They have to.”
The girls have their limits, and it has been a hard road finding them.
“It’s different having sickle cell,” Jamira explains shyly. “I can’t run track or cheer. I can’t do sports, but I did join dance,” she adds with a smile. Jasmin nods. “You should have seen her face when she finished her performance. So much pride. She was so happy she could do it especially after getting her heart broken over cheerleading.”
Rosa has always been well aware of the limits. “You have to watch them. Jamira’s eyes get yellow. She needs lots of fluid. They can’t overheat. You watch them, you listen to them. Pay attention to your child and find their normal.” Jasmin keeps her own eye on Antonesha. “She rolls her eyes and says, ‘Why are you staring at me, mom,’” Jasmin laughs.
Both girls go into what’s called “crisis.” Often from being dehydrated. It is extremely painful for the patient—and almost as painful for the parent.
“A proactive parent is quite important in the livelihood and success of a sickle cell patient,” Clay explains. “We educate the parent first and their advocacy for their child will help greatly in the success of that patient. Our transition team works with patients as they transition into the next phases of their disease and eventually into adulthood. Family is a big part of this process.”
Rosa has learned much about sickle cell over the years and as you can imagine, she has some advice.
“Let these kids know you love them and that it’s going to be OK. Jamira is going to live to 100. I tell her she can be anything she wants to be. The hospital is helping us make that possible.”