Our sweet Grace was born two days after her due day on November 15, 2001. After much ado with her heart rate dropping due to the umbilical cord being wrapped around her neck, she arrived with a loud cry! I'll never forget as the nurse handed me my sweet baby girl and reassuringly said, "Look at her she's pink and healthy". Little did we know our Grace would show signs of distress at her two week check-up with labored breathing, a heart murmur, and a coloboma in her left eye. We were immediately sent to Emanuel hospital in Portland, Oregon where we were rushed into ICU as Grace's coloring looked much more blue than pink. Later that day, it was determined after running many tests that Grace had a Congenital Diaphragmatic Hernia.
Looking at the x-ray with the surgeon, my husband and I felt somewhat relieved that it wasn't more serious. After all people get hernias everyday and have surgery and then are just fine- or so we mistakenly thought. You see, we had no idea how serious CDH is and further, had no idea the problems that seem to follow these little ones long after their initial hernia repair.
Grace was one of the lucky ones. She sailed through the surgery and was out of ICU and off oxygen just days after her operation. The surgery had gone very well and we were home in less than one week. What a relief to have it behind us and I, her mother, felt she would thrive at home. The difficulty began with feeding her. She would fall asleep within the first five minutes of nursing, had difficulty gaining weight, and it started to appear as if she was uncomfortable as she would squirm and arch her back.
We were referred by our pediatrician's office to a developmental pediatrician. She observed Grace nursing and suggested a NG tube because she felt she wasn't strong enough after surgery to do it herself. She wouldn't take a bottle so there was no other way of increasing her calories. We were very hesitant to pursue this, but it was becoming apparent that she needed some type of supplementation and we felt that if she put on some weight she might be strong enough to nurse on her own. Grace had the NG tube for about six weeks. We tried to remove it once but she had difficulty gaining weight. She started to arch more during her feeding and spit or vomit frequently. To our surprise, the developmental pediatrician seemed to have no explanation for this and instead referred us to a gastroenterologist for a gastrostomy tube (G-tube).
We were stunned. What did she mean she didn't know? It was second opinion time. In the meantime, it was evident Grace needed the G-tube although we had no idea what the diagnosis for needing it was. It was apparent she couldn't take in enough calories on her own and it was explained to us that prolonged use of an NG tube could cause oral aversion. We decided to schedule the G-tube procedure while awaiting a second overall opinion with a gasteroenterologist connected with a feeding team that consisted of a dietician, speech therapist, and nurse. It was there that we finally got the answers we were so desperately searching for.
"Reflux is common in CDH kids", the GI doctor explained. A barium swallow test confirmed the reflux and medication was prescribed. A malrotated bowel was also observed and was perhaps contributing to Grace's rather bilious vomit. The GI doctor also felt the malrotation might be why the medications for Grace's reflux seemed to be insufficient and referred us back to her surgeon.
While malrotation isn't always corrected in CDH kids, the Surgeon believed it might make a difference in Grace's case. He went on to say that we wouldn't know how much malrotation was contributing until it was corrected and rather than correct the malrotation and do a Nissen/fundoplication to correct reflux it seemed more logical to take it one step at a time.
We agreed. Grace was completely miserable in the month leading up to surgery. She was now crying at every feeding. Nighttime was worse. She would cry and vomit for hours until an exhausted Grace would finally fall asleep only to be awakened when the feeding pump turned on for her nighttime drip. We thought the surgery would help her; and it did for a time. Unfortunately, reflux was the root of the problem. We struggled over the course of a year counting calories and ounces and doing almost daily weight checks. We wondered how many times one should try to put the same feeding Grace had just thrown up back through her tube. She was no longer vomiting an ounce here and there, it was three or four ounces sometimes four to five times a day and even in the middle of the night. Weight gain was an ongoing issue. All the while we kept in touch with our GI doctor and feeding team trying every possible suggestion. Ultimately, Grace was maxed out on three different types of medications to control the reflux that were giving her little, if any relief.
Working with a GI doctor, dietician, and speech therapist, we had explored every conceivable medication and feeding approach for Grace. Nothing seemed to make a difference. Grace still threw up. It was becoming a consensus that reflux was not something Grace would outgrow. She was a severe case.We stuck to a strict feeding schedule but Grace would still lose weight. If we went out to dinner and Grace was sitting upright she would likely vomit due to pressure on her abdomen. We had to be very careful to not expose her to public places because she would invariably catch a cold which would make her reflux even worse. Our little girl who I was so sure would thrive was now considered "failure to thrive".
Reflux was making it impossible for her to lead any kind of a normal life and hindering her development. As a baby Grace never crawled. She never had the upper body strength because if we put her on her stomach she would throw-up. Instead she pursued walking to get upright as she gradually gained the strength to pull up to stand. She finally walked at fifteen months. Walking was a huge milestone for Grace but a dangerous one as she wouldn't bend at the waist to sit down. Instead she would fall flat either forward or backward and falling always meant throwing up and of course possible injury. It took her almost two years to get into a sitting position from lying down and it was almost another year to get from sitting to standing. Looking back now we realize she simply lacked the strength. It broke our hearts to see her not be able to participate like most toddlers in daily living. Her older brother who was only three at the time was also in tune with her. We would be trying to give her a bolus through the pump and I would get up to answer the phone and he would say, "Mom, there she goes again." And Grace would be throwing up. Our GI doctor referred us back to her surgeon to discuss a fundoplication,(the surgical correction for reflux). We researched the Nissen/fundoplication surgery and educated ourselves on the procedure as well as the post-op risks and long-term outcome. We wanted to do whatever possible to avoid surgery. However, we knew our little girl was completely miserable and the surgery, although with a definite down side, would eliminate the reflux.
Even though the surgery might create new complications, we were ready to face them and work on solutions to what we hoped would lead to a better quality of life for our daughter. We already knew the surgeon would be opposed to the surgery, but since she hadn't gained any weight in almost a year he reconsidered. We proceeded with the surgery after careful consideration. The fundoplication was done laproscopically and thereby shortened Grace's recovery time. We were out of the hospital in just a few days.
Once at home, Grace began to experience one of the most common and dreaded side effects of fundoplication – retching. Grace seemed to do okay with her feedings at first, but had begun to wretch within days of being home. While it was wonderful to see her keep her feedings down, they still seemed to cause her great pain. The retching was almost worse than the vomiting because she would strain until her face turned purple oftentimes breaking blood vessels on her face. We tried medications suggested by her GI doctor which provided little, if any, relief. We also experimented with different formulas, but she still retched constantly until it was just a part of her feeding routine. We were at the end of our rope and felt there had to be someone, somewhere, who knew of a solution to the constant retching.
We decided to take an aggressive approach to the problem. The question to Specialists became "If not you, than who?" To be more specific: If one professional didn't have a solution, who else could they recommend? We asked every medical professional we came in contact with and some others we barely knew. We also utilized the Cherub's (Congenital Diaphramatic hernia Association) database. We emailed all parents whose kids had the Nissen/fundoplication surgery asking two simple questions: Did your child retch, and if so, what did you do about it? It was in one of those emails we finally found some answers. Several moms spoke of a Speech Language Pathologist named Polly Tarbell who they credited with helping their kids to become oral feeders. Polly was a part of a feeding program at Kluge Children's hospital and Rehabilitation center in Charlottesville, Virginia called the Encouragement Feeding Program which boasts a 75% rate of getting kids off their g-tubes. We set up a time to talk over the phone.
Polly made two suggestions that made an immediate impact on Grace's retching. Polly felt Grace was suffering from intolerance to formula and suggested a blended diet for Grace that consisted of things she would normally be eating at this stage of her development. She also recommended a drug called periactin that helped with mucous secretions and stimulated a feeling of hunger. Both suggestions worked immediately and Grace was like a completely different child. We worked with Polly for four months over the phone and email getting Grace ready for the program. Getting ready for the program required getting Grace's feeding schedule arranged so that she would have more normal hunger/full stomach cycles. No more nighttime bolus as that is not what is normal for an oral feeder. It all made sense. Grace had to learn how to regulate her own hunger cycle, which meant we had to allow her to get hungry and resist the natural urge to feed our child. Letting go and letting Grace do it herself after such a long struggle with counting calories has been the most difficult part of the process, but we believed in the program at Kluge and we had grown to trust Polly. It was all up to Grace now. When we began the two week program Grace ate nothing by mouth. By the second day she was eating 30% of her calories by herself. Like learning a new skill, eating had become her new favorite thing to do! By the end of the first week Grace was taking in 80% of her calories by mouth!
Grace is now a healthy, happy 11 year old!