Harmony was first treated at NCH when she was only 3 weeks old. She was having feeding issues and not gaining weight as she should have been. She was admitted for observation of her feeding schedules. She gained minimal weight while in the hospital for about 4 days so we got to go home. She was followed closely and was readmitted shortly after Thanksgiving, at about 6 to 7 weeks old. This time, tests were ran and specialists in GI were brought onto our team. No answers this time either, but minimal weight was gained and we were allowed to go home. We were then followed by GI and our pediatrician. Weight gain continued to be a problem and many medications were trialed. Eventually, we were admitted to get more testing and an NG tube was placed. She was tested for a few disorders and her test results came back in March 2013 definitively that she had CDG 1a, a genetic metabolic disorder that is incredibly rare and not much information was available about it. We were going to treat the symptoms and hopefully, with a diagnosis, be able to move on in treatment and continued good health.
Unfortunately, about a month after her diagnosis, she aspirated some of her formula and developed pneumonia. She was admitted and placed on oxygen. Eventually she weaned off of it and was preparing to get discharged, when she got worse. The PICU team was called in, she was transferred to the PICU for intubation and ventilation where she could be managed easier. She got worse before better, but she got extubated after about 2 1/2 weeks of being supported to allow her lungs to heal. She was doing well enough to talk of leaving to go home, when her team and her dad and I decided that she should probably have a g-tube placed while there. She had the surgery, it went well, she was monitored, then that night, her incision opened up and disintegrated, leaving no good skin to close the area. She was introduced to the wound care team and stayed in the hospital thru the summer trying to manage and heal the area. As time went on, she got worse, gaining fluid on her belly and needing ventilation or CPAP/Bi-Pap. She did get discharged but only went home for 5 days before developing a clotting problem and being readmitted. She passed away October 3, 2013 with all of our PICU team, family, and friends present. Harmony’s daddy now works at NCH and is going to nursing school to help other families the way the doctors and nurses helped our family. Harmony is now a big sister angel to her rainbow baby brother, Lincoln. We can’t thank the staff at NCH enough for helping our family.