08/18/2005
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Devin's Story: Titanium Rib Helps Child with Multiple Disabilities Danbury, CT-----Despite having three sonograms during her pregnancy, Rixys Diaz was never told there was anything wrong. But when her son, Devin, was born eight years ago, he had multiple medical problems. Six of his ribs and his left kidney were missing. One lung was so compressed by his twisted backbone that at first it was thought to be missing. His heart was pushed to one side, and he was experiencing respiratory distress. "They told me he might not live through the first night," said Rixys, a single mother living in Florida. For about a week, Devin stayed in the hospital intensive care unit, but each day seemed to bring more bad news. Medical exams revealed a heart murmur, numerous skeletal deformities, cranial asymmetry, and malfunction of the bladder and colon. Many of the problems stemmed from his severe scoliosis - curvature of the spine. And, as Devin grew older, this condition became even worse because the active toddler struggled to walk and play in spite of his many disabilities. As she watch her little boy try to do the things other children did, Rixys became increasingly desperate to help him. She sought help from a local orthopedic surgeon who told her there was nothing more he could do. However, he also remembered "a fellow from Texas" he had seen at a recent seminar who might be able to help. He gave her the name of Dr. Robert Campbell. "I went online to find Dr. Campbell," Rixys said. She read about his work at the University of Texas Health Science Center at San Antonio, where he was developing an expandable titanium rib for children with a condition known as thoracic insufficiency syndrome. That occurs when there is insufficient room for the lungs to grow because of missing ribs or skeletal malformations. After learning about Dr. Campbell's research, Rixys took a daring step. She went on leave from her job and launched a fundraising campaign that took her door-to-door and included local TV appearances. With determination born of desperation, she raised the money to go to Texas with Devin. When she first met Dr. Campbell, Rixys laid it on the line: "You HAVE to help us," she said. "You're our only hope." To ensure that Devin met the requirements for the clinical trial, she followed Dr. Campbell's instructions precisely, including getting up several times each night to feed Devin, whose weight was too low. Ultimately, those efforts paid off. Devin was scheduled for surgery to implant the titanium rib. The procedure took eight hours, but Devin came out of it 60 degrees straighter. When Dr. Campbell held up an X-ray showing the difference, "We all jumped on him and hugged him," Rixys said. Most amazed of all was Devin himself. While recovering in the hospital after the surgery, he never saw himself. It was only later when he saw his reflection in a hotel mirror that he realized how different he looked. "Mom, I'm not sick anymore!" he triumphantly reported. Now Devin returns to Dr. Campbell twice a year for expansion of the implanted rib. He also continues to receive other medical services. But straightening his spine has resulted in marked improvement of many of his problems. His left lung is working better, and his heart has moved closer to where it should be. Other internal organs are better aligned than previously. This spring - three years after Devin's surgery - the titanium rib was approved by the U.S. Food and Drug Administration (FDA), a fact that can be attributed to Dr. Campbell's persistence and devotion to babies and children like Devin. When, as a young surgeon several years ago, Dr. Campbell first got the idea for the expandable rib, he had trouble finding anyone to fund his research. The National Organization for Rare Disorders (NORD), a non-profit patient organization, saw the value of his work and provided the first small grant to get it started. Later, when the research produced promising findings, Dr. Campbell was able to secure funding through the Office of Orphan Products Development at the FDA. Now a well-known medical device company in Pennsylvania, the Synthes Spine Company, has agreed to produce the titanium ribs, even though sales will be restricted to a small market. The device will help babies and children with various rare medical problems, including a condition known as "Jeune syndrome", that result in too little room for the lungs to expand as the child grows. Many of the children who will be helped by the titanium rib would die without it. Devin, a third-grader, has been going to a rehab center and loves to do the things other kids his age do. As for Rixys, in addition to her work as a community fundraiser for a substance abuse treatment facility, she also has a new calling: She has become a volunteer advocate and the voice of experience for parents of children with medical problems similar to those Devin has experienced. "They are worried and afraid. I know just how they feel. I speak their language," she explained. Rixys also hopes someday to start a foundation to raise money to "help other kids like Devin". For more information about rare diseases and improving the lives of those affected, go to NORD's Web site at www.rarediseases.org. For information about the new Titanium Rib Foundation:
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