Elizabeth Lynne arrived three weeks early, on January 20, 2014. Very early in my pregnancy we were sent to a Maternal Fetal Medicine (MFM) Specialist due to the fact that I was over 35. At roughly 14 weeks, the specialist noticed that Elizabeth’s bowel had some fluid in it. It was unusual but not alarming by itself. We were keeping an eye on it and having regular sonograms every couple of weeks. Then a few weeks later, we were unable to see the bowel anymore because the bladder was enlarged. It should have been about the size of a grape, but it was the size of a lemon. They kidneys were also very full of fluid due to the bladder not emptying and backing up into the kidneys. We waited nearly an hour that day, hoping that the bladder would drain but it did not. In November, we had a vesicocentisis where the specialist inserted a needle and drained as much fluid from the bladder as she could. The fluid was clear and we took that as a good sign that fluid was emptying from the bladder at some point. The fluid was sent to be analyzed and I was admitted to the hospital for about 3 hours for observation. At the end of the three hours, the bladder was still small and the kidneys were normal sized. However, 48 hours later the bladder and kidneys were enlarged again.
At the end of November we had an MRI hoping to rule out some of the worst case scenarios. The results of the MRI were generally discouraging. We were told that there was a 98% chance that Elizabeth would require at least some surgery immediately after birth. The MRI showed no meconium in the bowel, which should have had meconium from top to bottom at that point. We were told that the best case was that Elizabeth would require surgery or surgeries to correct the bowel and bladder issues, which may or may not involve a bowel transplant. Worst case scenario, Elizabeth would have something called megacystis microcolon intestinal hypoperistalsis syndrome. However, this could not be diagnosed for sure until Elizabeth was born.
The only information that the specialist could give us about MMIHS was scant and disheartening. I went home and googled MMIHS and everything I could find was doom and gloom. Needless to say we were crushed. The information we were finding was so devastating that I refused to even get Elizabeth’s room together until we had a release date from the hospital. I was so afraid we would never get to bring her home.
At the beginning of January we met with the doctors and staff from Children’s Medical Center Dallas. They all agreed that all of Elizabeth’s symptoms were pointing to MMIHS and they started work on a plan of care for after Elizabeth was born. The doctors were able to help us feel more comfortable and prepared for what the coming months would bring. I was also referred to a high risk OB who would deliver Elizabeth at a hospital closer to Children’s Medical Center. The OB also suggested it would be safer for Elizabeth and me if she were born via C-section.
Elizabeth surprised everyone by coming 3 weeks early. She weighed in at 10 pounds, 3 ounces. She was taken to the NICU at UT Southwestern immediately and transferred to the NICU at Children’s the next day. Elizabeth spent six weeks in the NICU. During that time she had a Ladd’s procedure to correct intestinal malrotation and her entire bowel was examined for any signs of physical blockage. She had a central line placed in her leg, and received a vesicostomy. The vesicostomy had to be revised several times and was eventually closed up because Elizabeth had so many problems with it. After six weeks, Elizabeth’s PICC line was moved to her arm and we were moved to the GI floor. We spent two weeks there trying to give Elizabeth feeds and learning to care for her central line and GJ button.
The first year was brutal. Within two weeks of being home we lost Elizabeth’s PICC line due to cellulitis and had to completely change the way we do dressing changes. We had hospitalizations every 4-6 weeks like clock work due to UTIs. The end of the first year culminated in an emergency surgery where half of Elizabeth’s colon was removed and she received an ileostomy. We were hospitalized for 17 days but we made it home just in time to celebrate Elizabeth’s first birthday.
Now, we take hospitalizations and challenges as they come and do what we can to minimize infection risk. She is tolerating feeds through her J port well and requires catheterization every 4 hours. She gets speech and occupational therapy twice a week to help with oral skills and sensory issues. We travel to Omaha once a year for transplant evaluation but so far Elizabeth is doing well enough not be be listed. Elizabeth is a happy, funny, busy, bossy three year old. She loves playing with her dolls, Paw Patrol, her iPad, and being outside. In most ways she is a typical toddler and doesn’t let all the lines and tubes slow her down one little bit.