Raya was born full-term with no complications. She weighed about 6 lbs 13 oz. Shortly after birth she began experiencing symptoms of reflux and seemed to have difficulty gaining weight. She often fell asleep while nursing and it was difficult to keep her awake to feed her enough.
By one month of age, Raya had gained less than one pound. She weighed 7 lbs 8 oz. She was becoming more and more difficult to soothe in the evenings. She was placed on Prevacid for her reflux at that point and we tried remedies like adding rice cereal to her breastmilk to help make it heavier & hopefully stay in her stomach better, and feeding her breastmilk from a Dr. Brown's bottle because that had helped her older sister. Nothing really seemed to help.
At the time of her 2 month check-up, Raya was crying every evening for 3-4 hours straight. She weighed 8 lbs 8 oz, which was less than a 2 lb weight gain since birth. She was extremely difficult to soothe and was still experiencing a great deal of discomfort from reflux. She spit up frequently and had started projectile vomiting as well. In an effort to help reduce her symptoms, I had eliminated all foods that could possibly have been bothering her from my diet but it didn't seem to help. Her pediatrician was concerned about her poor weight gain and continued reflux symptoms and referred us to a pediatric gastroenterologist.
During our first visit with the GI doctor, I was asked literally dozens of questions about how I was caring for and feeding Raya. I answered them as best I could and as the appointment went on, I felt more and more concern over the condition she was in. When the doctor looked at her, she commented about how underweight and malnourished Raya was. She pointed out that Raya's general disposition seemed to be one of discomfort and discontent and that there was literally no fat on her body. She mentioned hospitalization but instead allowed me to take her home over the weekend and modify her feeding regimen in the hope of getting her to gain a few ounces by Monday.
I stressed over every detail of both of our diets all weekend. I made sure I was eating sufficient calories and drinking enough fluids and followed the GI doctor's feeding plan to a T but it didn't work. The vomiting got worse over the weekend. At her weight check on Monday she had lost an ounce so the doctor called us that night and asked us to take her in to the hospital.
She stayed for 5 days and had several tests done. They did a pyloric ultrasound which ruled out pyloric stenosis. She also had an upper GI, which showed "significant reflux" and distension of the intestines (i.e. excess buildup of gas in her GI tract). She was switched from breastmilk to Enfamil Pregestimil formula. She had no desire to drink it (I didn't blame her, it was just plain foul) so they placed an NG tube so that we could get the formula and medications in whether she wanted them or not. :) They watched her for a couple days to make sure she was starting to gain weight and then sent us home. That was the beginning of our life with a feeding tube.
The vomiting continued without explanation or relief. At the age of 5 months, we suspected that she was aspirating her feeds & reflux so she had a modified barium swallow study done to evaluate that. Unfortunately, they were booked solid at all the hospitals for a month and during that time, she was NPO (nothing by mouth) for safety reasons. By the time she had the MBS, she no longer remembered how to swallow liquids other than her own saliva and didn't care to try so she continued to be 100% tube fed.
After about 6 1/2 months with the NG tube, we were finally able to get her in for surgical placement of a G tube in August 2010. (Read about our decision to get the G tube here.) At that point, she was still vomiting anywhere from once to 6 or 7 times a day with gagging & retching episodes mixed in here and there. She also successfully completed and failed her 2nd MBS which meant that had she been drinking any liquids by mouth at that point, we would have had to thicken them to nectar consistency, but she wasn't so we didn't. :)
About a month after her G tube placement in the middle of September, she started to have bloody fluid leak from her G tube (from inside it, not from around it) and she was vomiting brown blood. She had an endoscopy and her GI doctor found a large lump of granulation tissue that had built up in her stomach right next to the bumper on her G tube, which was the source of the bleeding. At this point, we had been discussing possibly putting in a GJ tube to see if that would help with the vomiting, so they attempted to place one that day in interventional radiology (IR). After about 10 minutes, the radiologist informed me that without doing a revision of her G tube site (i.e. new surgery), they would not be able to get a GJ tube placed. We went home that day with a regular Mic-key button instead. Her doctor put her on carafate (used to treat ulcers) to help with the bleeding.
A month later, she was vomiting more and the amount of blood was increasing. Her discomfort level was higher and things were just starting to get a little out of control, so her GI doctor admitted her to the hospital again so she could go on gut rest for 24 hours. This time, IR was successful at placing the GJ tube but immediately after that, Raya started vomiting bile and ended up being at the hospital for 15 days while we attempted to get the output of bile from her stomach under control.
For the next 9 1/2 months, Raya was fed into the J port anywhere from 18-23 hours a day and at the same time we drained bile from her G port to keep her from vomiting the bile. She continued to vomit multiple times a day despite all of the things we tried to help with the vomiting. I took her to Nationwide Children's Hospital in Columbus, OH in December 2010 to have testing done to determine whether or not she had a motility disorder. In short, she was not diagnosed with a motility disorder (like gastroparesis) but we were told that her vomiting was most likely due to visceral hyperalgesia, or hypersensitivity in her gut. We were already treating her with the medications used for this condition and so we continued with that course of action. (To read about the trip & the results of the testing, see here and here.)
About 2 months before our trip to Ohio, Raya had been formally diagnosed with mild spastic diplegic cerebral palsy. This diagnosis was a blessing to us because it qualified Raya to receive therapy services beyond what she was getting through the state's early intervention program, and it also qualified her for the state's long term care insurance. In January 2011, Raya started occupational therapy with the purpose of working at reducing the effects of her sensory processing disorder. This therapy has made a world of difference for her in her ability to cope with her surroundings and has helped her to learn how to be calm in situations that her body & brain have difficulty processing well.
After 9 1/2 months of having the GJ tube, Raya was finally able to go back to a regular G tube in the end of July 2011. About a month or two before that, she had finally started to nibble on crunchy things that dissolve easily, like crackers. A couple of weeks after having the GJ tube taken out, she started to eat 100-200 calories a day of pureed foods (oatmeal and beans).
In August 2011, Raya had gone almost 3 whole months without vomiting with the exception of once or twice when she got fed a little too quickly for her stomach to handle. Since things were getting SO much better, I asked her GI doctor if we could wean her off of one of her medications that she was on to control the gagging/retching/dry heaving she had been experiencing. We tapered her dosage for the space of 3 or 4 weeks and then on September 4th, she had her final dose. About a week later, all hell broke loose and Raya turned into a crying, crabby, anti-social mess for a few days. Her reflux went crazy, she quit eating anything that she had been interested in, and she had mood swings exponentially more dramatic than a typical 22 month old. Fortunately, things improved with time and we were able to leave her off of the medication.
She turned 2 in November 2011 and continues to work hard in OT, PT and feeding therapy. She is eating small amounts of food every day but is still 75-90% dependent on the feeding tube for her nutrition, hydration, and medications.
Aside from her GI issues (which have always been at the forefront), Raya was born with congenital muscular torticollis and congenital plagiocephaly resulting from positioning in-utero. You can read more about those two conditions here. Her treatment for torticollis has been a LOT of physical therapy appointments along with stretching and exercises that we've done with her at home. To treat the plagiocephaly, she had a DOC Band orthotic done by Cranial Technologies. It was expensive and they aren't contracted with most insurances so it was all out-of-pocket, but it was absolutely worth every penny spent and every mile driven to the appointments. Other companies will make a head orthotic for less money, but they don't compare. *stepping off of soapbox*
She has also seen a cardiologist for a heart murmur that turned out to be "innocent", a geneticist, and an orthopedic specialist who evaluated her for cerebral palsy-related care. Her legs and hands are doing well though and she does not need any corrective braces or supports. Genetic testing showed that Raya has a duplication at 18p11.31, which means that there is a small amount of extra genetic material on her 18th chromosome. The significance of this duplication is unknown and we are currently waiting to find out if our insurance will cover testing for the two of us. If either one of us has the same duplication, that would mean that it's a familial trait and not significant. If neither of us has the duplication, then it is anomalous to Raya and could be the missing piece of the puzzle that's responsible for at least some of her health issues.
During her first year, Raya had a LOT of tests done. She had a full neurological workup, including an MRI of her brain and spinal cord, ultrasound of her neck, EEG, and genetic labs to test for chromosomal defects. She had 2 or 3 endoscopies during which biopsies were taken and tested for eosinophilic esophagitis, celiac disease, and other similar conditions, all of which were negative. She had a gastric emptying study, a couple of modified barium swallow studies, a 24-hour pH study, several more upper GIs for various reasons, and many, many x-rays and labs. She was diagnosed with spastic diplegic cerebral palsy and sensory processing disorder in October 2010. In December 2010, Raya was evaluated at Nationwide Children's Hospital in Columbus, Ohio to determine whether or not she had a motility disorder. They did 2 gastric emptying studies, antroduodenal manometry, and an impedence test and determined that she does not have a motility disorder. The quest for a diagnosis that would explain all of Raya's medical issues has been long and frustrating and we may never find that "golden diagnosis" that we would like, but we are grateful for the excellent medical care she has received and the progress she has made.
Here is a video about our feeding tube journey:
Untitled from Brandis G on Vimeo.
Raya is now 5 years old and still nearly 100% tube dependent. She has gone through more testing in the past year or two, and while we have added a few more pieces to the puzzle, she is still a bit of a mystery.
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