Sophie Grace Nelson was on her way into the world when it was discovered in June 2008 that her organs appeared to be backwards and her heart was on the wrong side. During further sonograms it was found Sophie suffered from Situs Inversus, a condition where organs are in the opposite side of where they should be; mirrored image. Following several trips to Fairfax pediatric doctors, we were advised Sophie was in severe danger of having a terminal congenital heart defect common in children with Situs Inversus. Thus, we began visiting a cardiologist and following three appointments learned Sophie had a strong heart with only a 6mm hole inside; we were told it would most likely close on its own within months. In August 2008, we were advised Sophie did not have complete Situs Inversus, but rather partial Situs Inversus; a condition where only particular organs were reversed causing abnormalities in the formation of organs. The prognosis appeared to be getting worse because Sophie’s type of Situs Inversus is linked to other congenital birth defects. As time passed, we continued our check- ups and awaited Sophie Grace as her due date approached. Shortly before her birth, we were informed Sophie had three veins in her umbilical cord instead of the normal two! We were told it was very interesting; this was becoming the word of the pregnancy, "Interesting"Sophie Grace Nelson was born September 5, 2008 at 3:18am at Fairfax Hospital weighing 7 pounds 12 ounces and 20 inches in length. After every possible test, and dodging what seemed to be every imaginable issue, Sophie was allowed to go home with our family with only a slight concern of having a high biliruben level. Sophie had very high direct and indirect levels in her liver and we were told to have it monitored at the pediatrician's office. Following several visits in the following week, Sophie's levels remained the same and her baby poop was a cream color; this implied her liver was not putting bile into her small intestines causing the poop to be colored brown and green. In addition, Sophie was suffering from severe acid reflux and was prescribed multiple medications for her constant spitting up. The following week on September 18th, we were on our way to Fairfax Hospital where we would be checked in and remain for a week while they monitored Sophie and ran assorted tests on her liver. During our time in Fairfax, we began to notice Sophie had a funny looking belly and her stomach appeared to be swelling and becoming uneven on each side. After a week in the hospital, Sophie was transferred to Georgetown University Hospital on September 23rd to be evaluated for Biliary Atresia. Upon checking into Georgetown, we were immediately placed on the "Children’s Organ Transplant Unit" where we remained for two days prior to her surgery. Based on the test results and evaluation by the doctors, it was determined Sophie suffered from Biliary Atresia and they needed to operate immediately; studies show that operations after 90 days of birth were 50% less successful than those within weeks of birth. During discussion with doctors, we learned the operation was called a "Kasai," named after the Japanese doctor who pioneered the process. The operation called for Sophie's liver being cut open, new bile ducts being formed, and a piece of her small intestines being attached to her liver to help the liver drain into the intestines and deliver bile into the digestive track. Research shows 1/3 of operations are unsuccessful, 1/3 work for about a year, and another 1/3 work for years; However, all patients would eventually need a liver transplant. Thus, Sophie went into surgery on Friday afternoon at 2:30pm and was out by 7:15pm. Little did we know at the time Sophie was suffering from maul rotated intestines which had not been detected at the time of the evaluation and therefore her operation was scheduled for the following Monday. It was only by chance the surgeon penciled Sophie in on Friday for her surgery. In meeting with the surgeon following the operation, we learned Sophie had issues beyond the Biliary Atresia and the operation became complex to the point where the surgeon called for assistance from another doctor at Washington D.C. Children’s Hospital to complete the surgery. It was explained to us Sophie had been suffering from maul rotated intestines causing the bloating we saw in her stomach due to two herniated areas in the small bowels. Another issue was Sophie's intestines were wrapped around her liver and other organs, her gallbladder was not functioning; it too was removed along with her appendix. It was assumed her intestines were tangled due to her partial Situs Inversus. Lastly, Sophie had one major artery running into her liver instead of two. This was the first the surgeon has ever seen and he took pictures to show us during the surgery debriefing. Following the operation, Sophie remained at the hospital for two weeks while they tended to her 7 pound body and supplementing her diet. On Saturday, Sophie was released from the hospital and allowed to come home with a feeding tube inserted into her nose and a liquid food pump attached to her at all times. We were told, "the truth is in the poop," and to pray for colored poop. Well, after a week, no colored poop and it has been determined the Kasai operation was not successful; Months following the operation we have learned the Kasai is working and Sophie will be stable for a few years to come.In July 2009, Sophie was again admitted to Georgetown Hospital where she was diagnosed as having a rare breathing condition where her system was making it difficult for her to process oxygen throughout her body. Initially she was breathing very hard and her lips appeared blue. In addition, she looked almost purple and her eyes look sunken and bruised. After several weeks in the hospital, Sophie received a Liver transplant from a close family friend and she is now awaiting to recover from her breathing condition. More can be read about Sophie' rare breathing condition on the home page. The story of Sophie and Judy
As Sophie laid in bed on her ventilator she had a respiratory specialist named Judy Geberegzabher watching her and studying her breathing habits. 5 weeks after transplant, Sophie's condition was steady getting worse and she was starting to bleed from her lungs to the point of suffocation within hours if not resolved. At this point the doctors from Georgetown, Boston, Philadelphia, and London had no answers and it was assumed Sophie was just going to pass due to her lung and blood shunting. As we all stood by her side Judy came into the room, and after a short firm talk with doctors, reset the ventilator and attempted to try a vent setting never used before on a baby.....we heard doctors tell Judy they were out of options and it couldn't hurt
See, Judy cared for Sophie so much she was doing her own studies at home on the Internet and in medical journals. Since Sophie's hepatipulminary syndrome was very rare there was little information on her condition and the first of her kind seen here at Georgetown Transplant Center. Furthermore, Judy had seen where a few cases worked on this new vent setting only used on adults. After several failed attempts with doctors in previous attempts, Judy was allowed this one chance as doctors were out of options; only eventual suffocation from her bleeding lungs. To everyone's amazement, within two hours Sophie began to improve and within a week she improved so well it has been described as a medical miracle.
Today Judy has been tasked with writing the hospital guidelines and protocol for the future use of this breathing treatment and assisting in teaching others in the utilization of such drastic therapy. Judy was nominated for a award and was publicly recognized for her professionalism, dedication, and ability to solve a medical condition that perplexed six (6!!) of the top transplant doctors around the world!! Judy will be included in a published study and documented case in medical journals around the world. Because of Judy other children will be helped with the use of APRV vent settings using Sophie as the example.Please allow me to publicly thank Judy for not giving up on Sophie during her darkest hours and having the courage and persistence with skeptical doctors regarding her knowledge as doctors apologized for Sophie's declining condition without answers. That night Judy was the second living angel to embrace Sophie! Thank you for saving our daughter Judy!
