Megan, born 2004

Megan Grace was born full-term on September 12, 2004, our sixth child. She looked perfectly healthy at birth; she was pink, and beautiful. Within the next 28 hours, however, I noticed that she was breathing much too hard and fast. She was taken to the special care nursery and then eventually transferred to YNHH for more tests. We were shocked to hear the diagnosis of Hypoplastic Left Heart Syndrome.

 

The doctors told us about the three-staged surgeries immediately after the diagnosis, and we met the surgeon. After her first surgery was postponed due to less-than-optimal labs, she had the Norwood (Sano type) procedure at 10 days old. The Lord blessed her with a good recovery and she was able to come home from the hospital two weeks later. Megan was able to nurse and gained weight slowly, but adequately. Three months later, she started having difficulty feeding and she started to desat. 

 

She had her pre-Glenn cath January 3rd. She had a very difficult time after the cath as her sats dropped down into the 40's and 50's. So she had her bi-lateral, bi-directional Glenn surgery two days later. The surgery went well but she had a very difficult recovery. The doctors had seen moderate to severe tricuspid regurgitation during the echo and surgery and considered going back in to fix it. Megan was extubated, then re-intubated for a week. During her month-long stay in the PICU, it was determined that she had a paralyzed left local cord, making it very difficult for her to feed. She came home with an ng tube.

 

The following month was a nightmare. Megan had much difficulty nursing; she was gagging and vomiting much of the time. Despite my best efforts to get calories into her through the ng tube, she was not gaining weight. Another visit to the cardiologist revealed that there was an aortic obstruction, along with tricuspid regurgitation. After an unsuccessful attempt to balloon open the arch in the cath lab, Megan headed back to the OR for an aortic arch reconstruction, and tricuspid valvuloplasty; her third surgery in six months. Despite a left lung collapse following extubation, Megan had a good recovery and was home 8 days after surgery. She did well, and began to grow on a regimen of breastfeeding, ng feeds and baby food. After a few months, we were able to remove the ng tube altogether.

 

Around her first birthday, she began to refuse to nurse. The next few months were nerve wracking. She stopped eating, began losing weight, part of her left lung kept collapsing and she just looked sicker and sicker. They finally diagnosed her with congestive heart failure and increased her medications. We headed for Boston Children's Hospital for a cardiology consult. Soon after, Megan ended up in the hospital for a week with a respiratory illness; that probably saved her life. We were finally able to get her to keep the ng tube in again and get her some nutrition. After many tests, we headed back to Boston for a catheterization. Three days later, she went in for her 4th surgery - a tricuspid valvuloplasty and annuloplasty band. She recovered well but stayed in complete heart block and ten days later had a pacemaker implanted. Once again she recovered quickly and we were home a week later. Six weeks later, in February 2006, Megan finally got her g-tube. She is dependant on it for most of her nutrition but we continue to work on her feeding skills.

 

One year after her surgery in Boston, a PC check-up revealed a narrowing above the tricuspid valve, restricting blood flow going through it. A follow-up two months later revealed a little more narrowing. So in March, she had an exploratory pre-Fontan catheterization at BCH. She tolerated it well and we came home the following the day. The results showed that her pressures are a little high making it questionable whether she is a candidate for the Fontan.

 

After months of gathering information, meetings with the transplant team in Boston, and a meeting with the surgeon, it was determined that there was risk in proceeding with the Fontan, but it was a reasonable risk. She turned out to be a poor candidate for a transplant. Megan had her Fontan surgery on November 28, 2007. She had a longer recovery, with some pleural effusions, but overall she did well, and went home three weeks later. In the months that followed, she was finally able to talk more, sing, and became much more mobile, as her energy increased. The following year, when she was four, we went to a 4-week Intensive Feeding Therapy program in Hershey, PA to help her with her feeding skills. We were able to get her off her daytime tube feedings; she only gets supplemental feeds at night.

 

The next few years were relatively quiet. Megan started homeschooling, and is doing well. She has no learning difficulties. She is now 8 years old, and is such a happy, funny girl. This summer, she had her pacemaker replaced, along with an EP catheterization. Following that, she started to get puffier, and her belly got even more distended (it had been getting progressively bigger the last year or so). Blood work showed that she was losing Protein, Albumin and IgG. She was admitted to Yale Children’s Hospital and diagnosed with and treated for Protein-Losing Enteropathy. We have changed up her medications, and are hoping to get things under control. After a follow-up appointment in Boston, we have decided to start researching the possibility of a Heart/Lung transplant. The next course of action is uncertain at this time but the Lord has been so faithful during Megan's journey that we know she is in His hands, and we will trust Him to direct our path.

 

Story by Megan's mom, Cheryl - Connecticut