My first father’s day was certainly not as I had imagined. We were in a strange city, in a huge hospital and within the upcoming days my wife, Tammy and I, would be making the most difficult decision so far in our lives. Our only child, Madelyn Kate made her entrance into our world ten months earlier on her due date August 22, 2007. We had prayed to conceive a child for four years. Maddie was conceived with our third (and decidedly final) IVF attempt. We knew we had been blessed with a miracle. I felt so ecstatic holding her that I found it difficult to breathe.
We realized that something was seriously wrong when Maddie began vomiting bile her second day of life and was unable to tolerate any feeding. Our roller coaster ride had begun. We were told that she “must have some sort of intestinal obstruction” and that surgery would likely be required. She was immediately transferred from Willow Creek to the NICU at Northwest Medical Center in Springdale.
My wife was having blood pressure issues and was recovering from a c-section and I would spend the next four days agonizing as I needed to be in two hospitals at once. They had Maddie ingest some barium dye and took multiple x-rays as it passed through her digestive system. To everyone’s surprise no true obstruction was found, but the transit time through her intestines was markedly delayed. Her intestines functioned as if she were a 30 week old premature infant. We were told that it should simply be a matter of weeks for her intestines to mature before we could take her home.
Within days her tummy became so distended it looked like she had a water melon in it and she screamed in pain. A tube was inserted down thru her nose and taped to her face to drain the stomach contents so that she wouldn’t have to vomit them. When the vomiting would quell for an hour or more they attempted feeding thru this same tube, a few drops at a time to no avail. We could no sooner get her clean and dry that she would vomit again. We felt helpless, desperate and frustrated!
After 3 weeks with no improvement, Maddie was flown to St. Louis Children’s Hospital, one of the top 5 children’s hospitals in the country. We spent another few weeks trying to increase feeds by mouth and feeding tube. Maddie was tested for every disease and disorder they could think of. They tried numerous medications but nothing helped. One positive word or sign or test result would give us the strength to pull ourselves out of the next ditch we were thrown into with bit of bad news. We overheard one doc tell another, “we’ve given up feeding her” and we were heartbroken.
My wife set up a caring bridge site in order to communicate with our extended family and friends as reliving the stories of each days events just became too painful for her to repeat over and over on the phone. She spent much of her time weeping while rocking Maddie for hours in the dark lonely NICU room.
In the absence of a cause for Madelyn’s intestinal failure, a tentative diagnosis of chronic intestinal pseudo obstruction was made. Chronic intestinal pseudo-obstruction (CIP) is a rare disorder of gastrointestinal motility where coordinated contractions (peristalsis) in the intestinal tract become altered and inefficient. When this happens, nutritional requirements cannot be adequately met.
At 6 weeks old she had 4 full thickness small intestine biopsies and her first central line placed. A central line is a tiny catheter that is place in a vein just under the skin in the chest and then threaded through the blood vessel all the way to the tip of the heart. All of her nutrition would now go through this IV line. It was around this time that my wife asked one of the doctors “is pseudo-obstruction something that could kill her?” The reply was, “I don’t think we can answer that just yet”.
Madelyn had been on TPN (total parental nutrition or IV nutrition) 24 hours a day since shortly after birth. This was causing liver damage and she was shockingly jaundiced. The catch 22 with TPN and a central line is that although it gives the nutrition needed to survive, it can cause the liver to fail and/or life threatening blood infections, metabolic bone disease and anemia. Incredibly, we found a pediatric GI motility expert (one of a handful in the country) about 2 hours away in Kansas City, who we were able to speak to at length on the phone. When Maddie was 10 weeks old we transferred to Kansas City. The doctor was able to perform a test that would give us a definitive diagnosis of intestinal pseudo-obstruction. We felt relief that we knew what to call it, but devastated that there is no cure and that she would be “chronically ill” for life.
She was in surgery the next day to have a permanent feeding tube put in her stomach, an ileostomy placed and more intestinal biopsies. The “feeding” tube allows us to drain the bile and formula (or food) that has backed up into her stomach as needed to hopefully prevent vomiting. She had a very difficult time recovering from surgery. Two days before Thanksgiving we were able to take her home for the first time. She was on 20 hours of TPN per day and pre digested formula mixed ½ strength continuously running through her feeding tube.
Learning to handle her IV nutrition, tube feeds and ostomy care was terrifying, even with the help of 5 grandparents, other family, and many friends. We rarely slept more than 2 hours at a time and it was very hard to think straight. The first year she threw up about 30 times a day, around the clock. You have never seen two people jump out of bed and change a crib, a onesie and move some laundry through with the speed and teamwork we have. We were told that if Maddie made it through the first year of life that she would probably do well. The statistics are that 1/3 of infants with pseudo-obstruction on TPN die within the first year due to liver failure or blood infection.
Maddie’s liver disease was significant enough that we were referred for a small bowel/pancreas/liver transplant evaluation in Omaha, NE when she was 10 months old. They were very concerned that her liver was near failure. It was during this transplant evaluation that we “celebrated” my 1st Fathers Day. The transplant surgeon said point blank, “your daughter has a fatal disease, not unlike cancer, and a transplant is her best chance at long term survival.”
We were completely shocked. The one year survival rate for this transplant was 90% and five year survival rate 60%. We were in the process of getting FDA approval for two investigational drugs used only for emergency or life saving purposes. After much careful consideration, prayer and counsel from our two GI doctors we chose not to list her for multi-organ transplant at the time. We chose to continue to “limp along” as we had been. Over the next three months Maddie’s bilirubin and liver function tests miraculously started to normalize. Although, we had finally received FDA approval for both emergency drugs we only needed one of them.
The first year of her life wasn’t marked by weeks or months of age, but by which hospital we were at. Ultimately, we spent the first year of her life in 8 different hospitals in 5 different states. It was the most difficult year of our lives. We pray to never suffer the pain that we experienced during that first year, ever. We have been fortunate that an intestinal failure team has been set up at Arkansas Children’s Hospital and they work closely with our GI motility specialist (who is now in New Orleans) as needed.
Maddie is 3.5 now and is on 18 hours per day of TPN. She is allowed to eat small amounts of certain foods by mouth for pleasure. It is important that she not develop any food or eating aversion. We have never been successful with tube feeding even a teaspoon per hour. She is treated for her anemia with weekly IV Iron Infusions and is on constant rotating antibiotics for small bowel bacterial overgrowth. She has emergency approval for a drug called Zelnorm which she gets 2 times per day.
We drain her feeding tube overnight and when she tells us she is “sick”. She hasn’t needed a blood transfusion in over a year. We see our GI doctor in Little Rock every two months and as needed. She is very susceptible to fracture and has suffered a femur fracture and most recently a broken bone in her foot.
Some people can tolerate TPN for many, many years. Some children have a difficult time growing while on it. So far Maddie has not. We take it one day at a time and sometimes five minutes at a time. We try not to live in constant fear but sometimes it is very difficult. Each fever takes our minds on an odyssey to its darkest corners where no parent should have to travel. A place that most parents don’t ever have to experience and those that do usually don’t speak of. Is this the fever that points to a line infection that leads to a sepsis “the number one killer of children on TPN” that kills MY child?
The what if’s take over, more so when we are tired and feeling desperate. She sleeps in the bed next to ours and we love having her so close to us. We do not ever want to regret not spending even five minutes with her. Our life is about her and we thank God daily for giving us the opportunity to take care of her and for all the joy she brings to our family and friends. She has changed many lives. We pray for better transplant outcomes, a stem-cell breakthrough or cure, and better drugs to treat motility disorders.
This father’s day, my 4th, certainly won’t be as I had imagined. We have just returned from a strange city, after staying near a magical place called Disney World. We are so thankful to Make A Wish Foundation and Give Kids the World for making Maddie’s wish to meet princesses, like herself, in person.
Will pseudo-obstruction kill her? We don’t know the answer to that yet. What we do know is that miracles do occur, dreams do come true and wishes are granted. And we are so thankful for our family and friends who have helped us immensely along the way.