|Expert Cardiology and Thrombosis Collaboration Helps Save an ACH Patient|
Eleven-year-old Joralys Morales is no stranger to All Children's Hospital Johns Hopkins Medicine (ACH JHM) and the life-saving care provided by the cardiology team.
She was born in Puerto Rico with a heart murmur. But it wasn't until she was 3 years old - after her family moved to Florida - that All Children's cardiologists examined her and discovered a small hole in her heart. What's more, the hole appeared to be growing, not sealing by itself as her parents had originally been told might happen by doctors in Puerto Rico.
"So that's when All Children's became a part of our life," said her father, Rafael Morales. "Joralys had heart surgery and it went great. She's been receiving follow-up treatment ever since."
It was during one of those visits from their home in Port Charlotte last year that her cardiologist, Alfred Asante-Korang, M.D., noticed something that would require expert collaboration with the ACH JHM thrombosis program led by Neil Goldenberg, M.D., Ph.D.
Dr. Asante-Korang, who had done the surgery on Joralys and continued to care for her in long-term follow-up, performed an echocardiogram and spotted what looked like either a scar or a blood clot in the major vein returning blood flow from the lower body to the heart, called the inferior vena cava. It was in an area where she'd had a catheter inserted during her heart surgery several years earlier. The decision was made to closely follow the abnormality, and a follow-up echocardiogram confirmed that it had grown - that new clot was growing on an older chronic clot .
"We were scared that the clot was growing and would come loose, so it was urgent that Dr. A-K do something to help her," said Morales, whose wife, Johany, speaks limited English. "We were like, ‘What's going to happen next?' But Dr. Goldenberg and Dr. A-K explained everything in a way that we could understand, and everyone was so nice to us."
"This is one example of how a collaborative effort has saved a young child."
Suddenly, a new treatment regimen was required for Joralys. Drs. Goldenberg and Asante-Korang formulated a plan of action involving anti-clotting medications ("blood thinners") injected under the skin twice daily. "The blood clot could have progressed and reached a point where the entire lumen of the vessel was completely occluded," said Dr. Asante-Korang. "This is one example of how a collaborative effort has saved a young child."
"That's why we love All Children's," Morales added. "I had opportunities to go out of state for work but because her health is first to us, that's why we haven't moved."
Morales moved his wife and daughter to the United States out of a desire to provide a brighter future for the family, which would grow to include a son, Noel, now 7 years old. "It was bad back home and I didn't want my children to go through everything I went through," he said. "The rough life. It's very tough to live over there. I was looking for a better life. We've been here 10 years now and I love it."
He worked in the distribution center at Wal-Mart and as a produce inspector, but now stays home to devote his time to helping Joralys, who also has some developmental delays. He takes her to and from school, and to her doctor appointments, while Johany works at Walgreens.
And now the family has even more reason to savor life - and appreciate the care All Children's has afforded Joralys.
In addition to overseeing the blood thinner treatment, Dr. Goldenberg performed a comprehensive evaluation of Joralys' blood clotting system. "Coagulation lab testing and interpretation is challenging, particularly in children," remarks Dr. Goldenberg. "This, and the rising incidence of thrombotic disorders in pediatrics, is why most major children's hospitals like ACH JHM have developed thrombosis programs, led by hematologists who focus on the care of children with blood clots and clotting disorders."
Dr. Goldenberg added, "For Joralys, we tested the level and function of a number of proteins important in the clotting system, as well as some genetic risk factors. Fortunately, her test results showed no evidence for an inherited or persistent abnormality in clotting that would put her at increased risk for blood clots. This made us optimistic that we would be able to safely take her off blood thinners soon enough."
But Joralys' case was tricky. Her scenario required someone like Dr. Goldenberg who not only helped write the international guidelines for care of children with blood clots, but who had the experience to know how to best treat kids like Joralys who have unusual situations.
Dr. Goldenberg explained: "Joralys' scenario was unusual in that, while her older clot was provoked by a venous catheter (often called a ‘central line'), the fresh clot that developed atop it was unprovoked-meaning that there had been no recent repeat catheterization, no traumatic injury to the area, no recent hospitalization, etc. The fact that the fresh clot was unprovoked meant that we should treat with blood thinners for six months, which is three months longer than the usual course for a provoked blood clot.
"Coagulation lab testing and interpretation is challenging, particularly in children. This, and the rising incidence of thrombotic disorders in pediatrics, is why most major children's hospitals like ACH JHM have developed thrombosis programs, led by hematologists who focus on the care of children with blood clots and clotting disorders."
"It also meant that we need to use a special blood test, called the "D-dimer," to help us have even greater confidence that 6 months was enough for Joralys. The D-dimer gives us a sense of how activated a patient's clotting system is at a given time. For Joralys, we made sure her D-dimer was negative before stopping blood thinner injections at six months, and then we checked it again after she came off therapy, to make sure it stayed negative. This, combined with what we saw on the follow-up imaging of her clot, made us feel great about her long-term outlook and low risk for developing a clot in her inferior vena cava again."
A follow-up scan performed in early March revealed that all of the fresh clot had disappeared, and some of the older clot had gotten smaller as well, just leaving a small scar on the margin of the vein-but with excellent blood flow through the whole inferior vena cava. "The doctors presented us with all the information and the options," Morales said. "They recommended that we stop the blood thinner injections and continue to be followed closely.". And so, Joralys is back to being her regular happy self, and receiving regular follow-up care from both Drs. Asante-Korang and Goldenberg.
"She's doing very well," said Dr. Asante-Korang. "It was great that we were able to diagnose and treat the clot before it could cause her harm, and without requiring another surgery. If the clot had blocked off the vena cava-the critical pipe for blood to get back to the heart from the lower body-it could have been catastrophic." Asante-Koran added: "It's also very important that we were able to get her safely through blood thinner treatment without any bleeding complications. The team, including Dr. Goldenberg, did a wonderful job for this young girl."
Wonderful doesn't even begin to convey how Joralys' parents feel.
"We can't even describe it," Morales said. "We're so grateful for the collaborative and expert care at All Children's."
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