Katie's Story - The First Year

Our daughter’s story starts in September of 2001. It started out innocently enough with a trip to the Women’s Clinic for the 20-week ultrasound of our second child. We found out we would be having a girl!

The ultrasound went fine; however, there was one little thing the doctor wanted us to be aware of. In the ultrasound, it appeared that our daughter had a small bowel obstruction in the jejunum of her small intestine (jejunal atresia). The doctor said not to worry too much, but he did want to do a follow-up ultrasound in two weeks just to be sure. We had an amniocentesis done to rule out any chromosomal defects (which are often associated with these kinds of intestinal problems). The amniocentesis came back fine - no Down Syndrome, no Cystic Fibrosis (at least not yet). Now, we were just left with knowing that our daughter would need to have abdominal surgery after she was born to correct her intestinal problems.

Not long after the 20-week ultrasound, my wife was diagnosed with polyhydramnios (excessive amniotic fluid), a tell tale sign confirming that our daughter did indeed have an abdominal obstruction (atresia). When a baby in utero has an intestinal obstruction, the baby is not able to drink and process all of the amniotic fluid that they normally would. Therefore, the mother’s womb fills up fuller and fuller with the amniotic fluid that the baby would normally be digesting and excreting. Polyhydramnios often leads to premature labor and delivery and this is exactly what happened in our situation.

Our daughter was born near the end of December, weighed 5 pounds 12 ounces and was six weeks early. We held her long enough to get about five pictures, and then I carried her in my arms down to the Neonatal Intensive Care Unit (NICU). Upon arrival, she was immediately hooked up to oxygen and oxygen sensors, blood pressure cuffs and a machine that tracked her respiration, pulse and oxygen saturation. After pumping all the excess, undigested amniotic fluid out of her stomach, her weight was actually 5 pounds even. An IV was started in the soft spot on her head, and a nasal gastric tube (NG-tube) was placed in her nose and down into her stomach. Since her condition was very unstable, she was not fully covered in the isolette but left open for easy and if necessary emergency access by nurses and doctors.

The pediatric surgeon had an emergency surgery that kept her well into the early morning hours so our daughter would not be seen by her until the following day. The surgeon requested x-rays of her small bowel which included the kidney, ureter and bladder (KUB) before doing anything else. Once we had the results back and a diagnosis of jejunal atresia was confirmed, the neonatologist approached us and after asking him to give us the worst case scenario, he quietly told us that our daughter might not have enough healthy bowel/intestine to live on. I won’t go into the details of the anguish that bit of news brought us, but let me just say that it was the longest day of my life! The neonatologist left our hospital room at about 10:00 AM. He explained that we would not know the full details of our daughter’s injury until the surgeon had completed a laparoscopy and had a full assessment of her intestinal tract.

The first slot to open up in the O.R. was not until 6:00 PM that evening. We had nothing to do for the rest of the day but wait. We passed the time with close friends and family while our daughter was in surgery. Three-and-one-half hours later the surgeon had an intern call up to our room and explain that our daughter did indeed have enough healthy bowel to live on - we were ecstatic! The surgeon removed the obstruction (the term for this is called anastomosis) and repaired the parts of our daughter’s bowel that could be comfortably reached. The pediatric surgeon later explained that the bowel above the obstruction was very, very distended (stretched) and that a portion of her bowel was difficult to reach as it was behind her liver. She explained that a follow up surgery later in our daughter’s life would be necessary to clean up that hard-to-reach portion of her bowel. Our daughter would need to grow more in order for her bowel to lengthen to a point where the surgeon would be able to get to repair the rest of it.

One other side note worth mentioning is that our daughter had a ‘text-book’ condition called apple-peel bowel. At some point in our daughter’s development in utero, the major blood supply coming down from the aorta into the small bowel was destroyed. The body responded amazingly well by wrapping the small bowel around the blood supply coming up from the colon creating an apple-peel twist around the major artery.

After surgery, our daughter returned to NICU where she had a recovery that was very disheartening to watch. She was on morphine for 4 days to help with the abdominal pain. During the time she was on morphine she was not able to breathe on her own, so they had her on a respirator. Nothing that we observed brought us any level of comfort. When I think back on it, I don’t know how we made it, but here we are. They expected bright green residual (green fluid and mucous coming from the NG-tube) for about 7 days. Our daughter’s recovery was slower than they expected and it was about 14 days until they had trace amounts of green residual. At this time the decision was made to start giving her oral feedings of pumped breast-milk. They gave her 20 cc’s (cubic centiliters) every four hours (1 ounce = 30 cc’s). Over the following days and weeks, the volume of feeds was slowly increased and her stools were closely observed to check for absorption. I should mention that in a situation like this where a child is not able to take in enough calories orally to sustain life, a Broviac (central-line IV) is placed into an artery just above the heart and the patient is put on Total Parental Nutrition (TPN). This is great because it keeps the patient alive! From what I understand, our daughter would have died had she been born even just 25 years ago because this technique has only recently been perfected for medical use. The only real drawback to TPN is that it can ruin the excretory system - the liver and kidneys take a beating.

Our daughter continued to get oral feeds and the volume was gradually taken up to 90 cc’s. At this point, our daughter, now six weeks old, appeared to be doing well enough to come home. They removed the Broviac IV since it appeared that she was consuming and absorbing enough calories to thrive and sent her home. Once home, our daughter was on a very strict dosage of Pepcid, Glutamine, Fer-Gen-Sol, Reglan, Imodium, Actigall and Phenobarbitol. We had her home for only 9 days when she started having many episodes of vomitus (throwing up). All of her vomit was bright yellow. She threw up usually right after a feeding. At 6:00 PM on February 6, 2001 we took our daughter to the Emergency Room to be evaluated by a physician. The extensive amounts of throw-up were just too much. They took another x-ray and attempted a small bowel follow-through using barium, but nothing would go through her system and the decision was made to admit her for further observation.

Our daughter was back in the hands of the nurses on the pediatric floor of the hospital. They kept her under observation and we were frequently told by nursing staff that she was doing just great and there did not appear to be a problem; however, my wife and I had instincts that something was not right. We were told by nursing staff that she probably just had the flu or something and that we were worrying about nothing. But, three days into observation, our daughter had quite an episode of forceful and projectile vomiting that coated a computer screen and filled a keyboard at the nurses’ station. The color was recorded as being greenish-yellow (this is important because it indicates the presence of bile in the vomitus). Bile is one indicator used in diagnosing potential trouble.

After a few more x-rays were taken, the decision was made to operate again. By this time, our daughter had grown enough where the surgeon was able to repair a portion of her bowel that had been previously unreachable. During the operation the surgeon also corrected two problems. The worst problem (which is fairly common following abdominal surgery), is lysis of adhesions (scar-tissue). The second problem she corrected was a new kink that had formed in her lower colon. The surgeon also repaired the size of her upper bowel so that it more closely matched (in diameter) the bowel above the obstruction (which was still very stretched out). The section of bowel below the obstruction had not been stretched as much as the bowel above the obstruction, since the part above was constantly filled and distended so extensively with fluid.

Three weeks later, our daughter came home again. This time she stayed home much longer, but that is not to say that things got better for ANY of us. Our daughter had (and still has) a problem with motility (her bowel does not move food through as quickly and efficiently as it should). Motility is very difficult to diagnose or measure, but in some cases, nerve damage has taken place and the affected bowel does not contract at all or it contracts in wrong direction. Whatever the problem is with our daughter, it is a significant problem and one that plagued us from February 2002 until October 2002. These 8 months will be one of the hardest times in our lives. Our daughter had forceful projectile vomiting at least once every other day (on average) during the entire time. There were times where we went up to two weeks between her scary throw-up episodes and there were times were we had three episodes per day. Some of the episodes were terrifying and resulted in paramedics being called to our home because our daughter would inhale the vomit back into her airway and choke on it. During these times, the only thing that would get her to quit retching and choking and to start breathing, was to put her quickly into her car seat and swing her gently. This rhythmic movement was usually the only thing that would get her to calm down. As a precaution, Our daughter spent many months sleeping the night away in her swing. My wife and I were often in tears and were scared, frustrated and generally just felt helpless through all of the chaos. Needless to say, the carpet in our house was absolutely ruined. Thank God for homeowner’s insurance! As I type, we are 2 weeks away from getting our entire house re-carpeted.

Between her totally unpredictable and scary throw-up episodes, choking and really no consistent weight gain from February to October 2002, we ultimately decided to surrender. Our daughter went from April to October and did not gain a single ounce of weight. In April 2002 she weighed 12 pounds, 5 ounces and in October 2002 she STILL weighed 12 pounds, 5 ounces. We were devastated and exhausted. Her non-stop throw-up episodes were preventing her from gaining any weight and she was not thriving and we were starting to worry about this affecting her development.

We finally agreed to something the surgeon and our pediatrician had been encouraging us to do for at least three months. We made the decision to do a surgery where the surgeon wraps the esophagus with a portion of the stomach (called a fundoplication). For our daughter, we decided on a toupee wrap which is not a full wrap of 360 degrees, but a 3/4 wrap of 270 degrees. With the toupee wrap, she can still throw-up if she really needs to. It is pretty much standard that a G-tube placement also includes a fundoplication. What we really wanted to do was put her on a feeding pump only and not do the fundoplication surgery. The major drawback to this is that she would very likely throw-up most of what we pumped in via the feeding pump. That is why the fundoplication is so important. With a fundoplication you get one immediate benefit in that the child can no longer vomit. Another great benefit is that by keeping the food in the stomach, it forces the stomach to stretch. In stretching the stomach and bowel, you force it to tone up and in so doing, you eventually will improve motility of the bowel. The G-tube goes directly into the stomach through the wall of her abdomen (via a MIC-KEY button). The G-tube hooks up to a feeding pump at night while our daughter sleeps. Right now, the feeding pump cycles through 16 ounces (480 cc’s total) per night (40 cc’s per hour). We “hook her up” at approximately 8:00 PM and she gets up at approximately 8:00 AM. Our daughter eats whatever she can during the daytime hours by mouth (baby food, crackers, soft adult food and her formula - Peptamin Jr.). The feeding pump is really just to supplement her calorically at night so that she continues to gain enough weight to thrive.

This G-tube/fundo surgery was the single best decision we ever made and I often struggle with guilt that we jeopardized our daughters life by not agreeing to do it sooner. She had the G-tube/fundo surgery in October of 2002 and by her first birthday in December of 2002 she had gained 4 pounds! She looked beautiful and was beaming with health and even ate a small piece of her birthday cake (a total no-no).

We have the feeding pump to thank for saving our daughter’s life. To this day, I wish that we would have listened to our surgeon and had the G-tube inserted sooner. Right now, it is nearly midnight in February of 2003. Our daughter is in her room hooked up to her feeding pump and she is sleeping very peacefully. It’s great knowing that she is being pumped full of calories while she sleeps. We still have a long road to walk with our daughter and we have no idea where and when it will end, but we keep going.

The story to this point covered our daughters life from birth to just past her 1st birthday. Two days after I finished that text above our daughter went back into the hospital for about 10 more days. She came home from nearly dying and two days later my company laid me off. A direct result of this is that we lost our home and had no way to pay for feeding our daughter other than credit cards and her formula was costing us over $3000 a month and that expense didn’t account for other medical care we had to obtain. But we clung to the hope that we would survive and somehow overcome this situation we had found ourselves in and now almost 4 years later we realize that if things don’t change we won’t be surviving. We desperately need help because the work has just become more than we can do on our own.