Stephanie

Stephanie's father, Terrill Sebastian, has written a wonderful tribute to Stephanie and her experience called A Purple Heart for Stephanie which he created from daily journal entries he made throughout his daughter's near-fatal bout with NF. The courage of this beautiful little girl, as well as the faith, love, and determination of her parents springs from every page of this tribute. To help other parents of child-victims of NF, Terrill has agreed to share his very personal story. If you would like a copy, please email Terrill at tsebcsstn@juno.com. THE NIGHTMARE BEGINS Three days before she turned 7 on February 11, Stephanie came home with chickenpox. Covered from head to toe with itchy lesions, she developed a typical chickenpox symptom - high fever - and a not-so-typical symptom - stomach pains. "My stomach hurts all over," she told me. Thinking this was constipation, I took her to the bathroom where I found two purple patches on her lower abdomen - one softball size. A blood blister had formed under there and the top layer of skin had come off. I called her mom, " Ann, we've got a serious problem here." The patches began to grow as we watched them. We whisked Stephanie to the hospital, where doctors started IV antibiotics and admitted her to monitor her condition. Stephanie's patches grew together. Her blood pressure plummeted and her pulse skyrocketed, all of which are signs of streptococcal toxic shock syndrome. Wasting no time, doctors sent Stephanie on a helicopter to Children's Hospital in Denver. Stephanie remembers the helicopter ride and landing on the roof of Children's Hospital. But most everything after that is a blur. For a time, she believed the helicopter had landed directly in the emergency room. " This place doesn't have a roof." Stephanie told the doctors. In the emergency room doctors frantically worked to save Stephanie's life. As Ann and I arrived at the emergency room, we could hear her screams of pain. "Make them stop hurting me!" They were sticking IVs everywhere they could stick them. But they couldn't get them in because her blood pressure was too low. Later that night Stephanie was transferred to ICU where she was monitored continually by the doctor. LEARNING THE TRUTH Stephanie was in critical and unstable condition. The first few days provided little sleep for Ann and I. We felt we had to spend every minute with her because we thought it was our last days with our daughter. The doctors would never give us hope of her survival. They would only inform us of the progress the medicines were making. She began to show improvement after a few days. There were no signs of live Strep A bacteria. It was time to begin the process of removing the dead tissue and start skin grafts. We thought we would be home within a couple of weeks. Little did we know at that time that not only would we not be home soon, but we might not take our daughter with us. After the removal of the necrotic tissue, which was about one third of her abdomen, Stephanie began running a fever. The doctors couldn't find the cause for the fevers and decided to hold off skin grafts until they had an answer. Stephanie's high fevers refused to come down. For three weeks she endured x-rays, CAT scans, blood test and the poking and prodding of the doctors. Every blood test, x-ray and every other test was negative. Stephanie's condition, however, began to decline. She started showing signs of toxic shock. Her face was swollen until her eyes were unable to be opened. Her little body grew with the fluid that leaked from her blood vessels. HIT AND MISS SURGERY The doctors operated again and again, a hit-or-miss process. Each time they'd remove dead tissue until they reached live tissue, then they'd stop. After each operation, Stephanie would improve slightly, then two days later relapse. The doctors were baffled. This cautious approach usually worked. But they learned from Stephanie's case that deadly flesh-eating bacteria sometimes needs to be treated more aggressively. By mid-March, Stephanie's condition was deteriorating. Dangerous amounts of fluid were collecting around her heart. Before undergoing an operation that required a needle to be placed dangerously close to her heart to remove the fluid, she was placed on a respirator. She stayed on it 5 1/2 weeks, during which she was unable to speak. Already in a fog from the painkillers she needed for dressing changes on the widening wound on her abdomen, Stephanie communicated with us by nodding her head and mouthing the words "I love you." LIFE vs. DEATH Stephanie's condition continued to worsen with no hope in sight. The surgeons finally decided to open the abdominal wall to see what damage had been done to her internal organs. Stephanie had become so unstable that her lungs were filling with fluid and her kidneys shut down. After a dialysis treatment, her doctor told us, we have to go to surgery now, if we wait we may not be able to take her. The surgery proved to be successful. While they had to remove the last bit of tissue covering her abdomen and the muscle that protects her organs, the organs themselves were untouched. A LONG RECOVERY Finally, more than two months and 10 surgeries later, doctors were certain they'd gotten all the dead tissue. About 35 percent of Stephanie's skin had been removed. Her abdomen and lower back were raw. By late April, she was ready for skin grafts. Thin slabs of tissue were taken from her thighs, stretched by a machine and grafted onto her abdomen. On April 28, Stephanie's respirator was removed. Yet the respirator had been used so long that doctors feared Stephanie wouldn't be able to breathe on her own. They also worried her throat would be so scarred she'd need more surgery to correct it. But Stephanie surprised them. After the tube was out of her throat, she said in a whispery but determined voice, "I'm hungry." REHABILITATION The next two weeks the rehab staff worked Stephanie hard. She'd lost much of her abdominal muscle and had to learn how to sit up by shifting to her side. Getting to her feet was agony. She'd lost the top skin layer from the bottoms of her feet and they were as tender as a baby's. Ann witnessed Stephanie's first attempt at walking. "She took three little steps toward me," Ann told me later. Stephanie found a companion in a doll she named Stephanie. Everywhere Stephanie had a tube, we put it on the doll. Stephanie was able to remove a tube from the doll every time she had one removed from her. She finally had someone who really understood. By mid-May we brought Stephanie home on a two day pass. The once warm loving home was now a strange and unfamiliar place. " I want to go back to the hospital," she told me. The house was so much bigger then her small hospital room. There seemed to be so much more noise that disturbed her and a lot of action from her little brother that was happy to see her again. We made the trip back to the hospital for another day or two and was discharged to home. The trip home the second time was much better and she was glad to be home. GRADUATION DAY On May 28, Stephanie was the star of her kindergarten graduation. After a special announcement of her recovery from this terrible disease, Stephanie received a standing ovation from the audience that prayed for her recovery. She was assisted as she carefully walked across the stage to receive her diploma and a special purple heart from her teacher. The purple heart, her teacher explained, was for her bravery and because her 3 month ordeal that began from chickenpox she received from a school friend. CURRENT ISSUES Stephanie's condition continued to improve after the initial scare from the flesh eating disease. While major muscles were lost in the abdomen, causing major herniation, she continued to improve over all. In 1997, Stephanie went back to surgery to have tissue expander put in. Two out of the six implants became infected causing another long two week hospital stay while being flooded with massive amounts of antibiotics. After eights months of weekly needle sticks to have the implants inflated Stephanie returned to Children's Hospital again on March 25, 1998 for more surgery. The latest surgery included the transplant of muscle from her legs to her abdomen for strength. They removed her tissue expanders and stretched her upper chest tissue as far as possible to cover her grafted areas. Amazingly, 50 percent coverage was achieved. Unfortunately, Stephanie became infected again and another surgery had to be performed to removed a synthetic mesh that caused the infection. The muscle flaps had to be left open to heal from the inside out and the tips of the muscle had to be removed. While Stephanie continued to have complications we were able to get her home and continue the antibiotics and wound packing. A home health care nurse is available for question but the wound packing and drugs are all done by her mother and I. Stephanie will soon have to have an ENT doctor look through a scope into her trachea. Because she had the tube in for so long, scar tissue has developed and continues to shrink the size of her air way. Any activity, short of walking, causes her to struggle with getting enough air. There may be a laser surgery procedure that will open her air way to accommodate her needs. Stephanie's attitude is unbelievable. Words cannot express how strong and determined she is. Her faith in God is what I believe has helped her so much while trying to deal with this disfiguring process. Stephanie is 10 years old now and her doctors says he believes he can cover the full abdominal area by the time she graduates from high school. She will have her CVP line out in a couple of days and she will be able to enjoy the last few weeks of school. She is very excited about returning and getting back to being a little girl again. Stephanie is especially thankful to God for her recovery and her doctors, friends and church family for their support and prays during this trying time.