Written by Dana Werner (Sam’s Mom)
Samuel Jon Werner was born on January 4, 2000 in Austin, TX. I started leaking amniotic fluid four days earlier, and was put on bed rest in the hospital. That was the day we first had an inkling something might be wrong with him. The doctor saw an enlarged bladder on an ultrasound. It wasn’t until after he was born that we knew the bowels were involved. Shortly after his birth a geneticist evaluated him and delivered the diagnosis of MMIHS to my husband, Dennis, and me. Later, a NICU doctor told us that the syndrome was usually fatal and Sam had about six months to live — he would die of liver failure due to the TPN and not being able to process anything orally (no omegaven in those days). At day three of life he had surgery to place an ileostomy.
My husband and I quickly decided we were not going to accept the grim prognosis, and had him transferred to Texas Children’s Hospital (TCH) in Houston. Doctors there were much more positive and gave us hope. He stayed at TCH for three months. We learned during that time that he likely had little-to-no intestinal motility, as he threw up almost all the breast milk/formula he ingested. We had several close calls, as he had many line infections and became seriously ill.
TCH referred us to a wonderful GI in Austin who managed his TPN/lipids and meds. He had several surgeries to “untwist” the bowels and to remove parts of them. Again, several close calls.
When Sam was seven months old, at our GI’s suggestion, we took him to Pittsburgh for a motility study. Several tests indicated that he had basically no motility in the stomach or small intestines. The doctor who performed the motility study recommended placement of a g-tube and j-tube, so at eight months of age he underwent that surgery.
He was unable to take anything by mouth the first three years of his life. 24/7 TPN. Gradually, he became sicker and sicker due to sepsis. The doctors referred to it as “bacterial translocation.” He had “intestinal non-function causing bacterial overgrowth escaping the intestines.” Rotating antibiotics to address the problem was not a long-term solution. So, our GI got us in touch with Pittsburgh again, and Sam was placed on the transplant list. Transplant was the only option to save his life. After six months on the list, we got the call.
In March of 2003 (at age three), Sam underwent the 14-hour surgery. The stomach and small intestines, along with the pancreas, were transplanted. The spleen, gallbladder, and colon were removed, as they were necrotic — diseased beyond the point of function.
Sam spent two months in the NICU and two additional months in a Ronald McDonald House in Pittsburgh following the transplant. He had several occurrences of high fevers (no permanent damage). He was on a fairly high dose of prograf to prevent rejection. In all, he was on about ten different meds.
Soon after he was discharged, and we were back home in Austin, we received a call from Pittsburgh that we needed to return, because he had contracted Hepatitis C, likely through a blood transfusion. We stayed in Pittsburgh for about two weeks. The doctors there decided that our local GI could continue the treatment at home. He received treatment for one year and was cured of the Hep C.
Sam had started eating and processing food while in Pittsburgh. He was started on soft foods and progressed to Cheerios (one-at-a-time!) fairly quickly. He showed no oral aversion. He retained his central line for six months, and was on one bag of hydration per day. After six months, the line was pulled, and all that remained was his ileostomy bag.
Over time, Sam’s meds were reduced to only the three he takes today — prograf, bactrim, and penicillin. He has been hospitalized only once since the transplant. When he was twelve, he contracted RSV, which was treated and cured after six days of hospitalization.
Sam’s medical routine today consists of labs every three months and a few doctor visits a year — one with his GI, one with the transplant surgeon in Seattle (his surgeon moved from Pittsburgh to Seattle Children’s Hospital, and we followed him), one with his urologist, and one with an Infectious Disease doctor.
Today, Sam, at 17, is a happy, healthy, young man. He eats anything and everything, with pizza being his favorite! He takes care of his ileostomy bag himself. He self-caths three times a day. We do nothing for him medically, other than ensure that he takes his meds. He goes to school, church, the movies, and other social activities. He is currently running on his school’s cross country team.
Sam’s long-term prognosis is good. At one appointment (annual transplant follow-up) I told our doctor that I wanted Sam to outlive me. His response was, “That will happen.” Doctors tend to emphasize that if a patient makes it 5 years post, they are pretty much out of the woods. No doctor has ever mentioned to us the possibility of another transplant. There are possible future complications from only one med he takes — the prograf (the anti-rejection med). Since it causes him to be immunosuppressed, he is more susceptible to infections/illnesses such as viruses, bacterial infections, the flu, etc. If he catches something, it may take him longer to fight it off. He does get a flu shot every year, and has never had the flu or any bacterial infection! I think the low dose of the two antibiotics he is on (one is because he has no spleen) protects him from such things. Docs have told us that down the road he may be more prone to cancer. But, the risk is low, and I just try not to think about that.
Our family life today is so much easier and more pleasant than before the transplant. We are so glad he had the transplant, and blessed that it was successful. Our family has a strong faith. Sam was prayed for by people from all over the world. We know that God’s grace helped us through the journey, and is responsible for Sam’s health today. Sam is our “miracle son.”