AustinRath

From ShortGut - Wiki

• Austin's Blog: [1] www.carepages.com page name: AustinRath

• Initial Diagnosis:

Austin was diagnosed with Hirschsprung's Disease about 1 week after birth. He was initially sent to the NICU immediately after birth because he was having difficulty breathing. After he didn't pass stool for several days and was vomiting after feeds, the doctors became worried. But all the tests were determined to be normal. The medical team thought he has severe reflux and were going to send us home. My husband has HD and we were both worried that Austin's symptoms were also due to HD. We asked to be transfered to Children's Hospital of Pittburgh from Magee Woman's Hospital, where Austin had the biopsy and was diagnosed with Hirschsprung's Disease. The doctors at Children's told us they believed that Austin had a mild case of HD and planned to do the traditional pull-through surgery. But what was suppose to be a 3 hour surgery turned into 9 hours. In the end, Austin was diagnosed with "ultra long segment" Hirschsprung's Disease. We were told that a case as severe as Austin's occurs only once every 5 years or so. Although Max was born just 5 days after Austin with the same severity.

• The First 4 Months:

Austin spent most of his first 4 months of life in and out of the NICU and PICU related to repeated line infections, sepsis and colitis. The uncertainty of what was going to happen to Austin was really challenging. There were several occasions that I thought Austin wasn't going to make it, starting from immediatly after birth when he wasn't breathing, to the initial surgery, to when he was septic and had to be intebated. Things now are much better and Austin is much more medically stable and doing well. During the first 4 months, Austin had 2 Broviac central lines placed surgically in his chest for TPN, he had a g-tube placed, his non-functioning intestine removed and an ostomy was created.

• 5 Months to 12 Months:

Despite a very rocky start, Austin's health since Aug. of 2006 has been rather stable. He's had 2 hospitalization and a few ER visits, but otherwise has done well at home. Austin had a transplant (liver and bowel) evaluation and was officially listed in Feb. of 2007. While he remains relatively healthy and doesn't need an organ at this time, he will acrue time on the list and have a better chance of getting an organ if he needs on in the future. Austin's care was initially managed by the surgery dept. of Pittsburgh, but transferred to The Intestinal Care Clinic (ICC), led by GI in October of 2006. We were disappointed in the change in care style and transferred Austin's care to Boston Children's Hospital in May of 2007.

• The Second Year

Austin is now followed by Boston Children's. Austin is eating more and doing well. He had a hospitalization for a line infection in July/August of 2007 and ended up having his line replaced. He is now at the 28th percentile for height and 44th percentile for weight. With the extra TPN calories, his liver numbers have remained the same.

• Therapy

Austin was discharged from OT, PT and Developmental and now gets Speech Therapy.

• Feeds

Currently Austin gets TPN 12 hours a day, with lipids every other day. He receives g-tube feeds 12 hours at night. He gets 6 bottles per day, ~50ml. per bottle and about 6 oz. of table food per day. Progress with feeds has been very, very slow.