One mother's story: our baby's congenital heart defect

By Tricia Garcia-Dergay

riley_photoRiley arrived on July 6, 2007, via c-section at a hefty 9 pounds and 10 ounces. She was gorgeous, plump and hairy. The doctors mentioned she had a heart murmur, but probably nothing we needed to worry about.

Fast-forward two weeks. Riley is home but has been fussy for a few days and not eating. She’s grunting and her color is off. Her dad and mom (who is herself dealing with a bout of Bell’s Palsy) take her to her pediatrician. He takes one look and goes into full “I’m more heroic and ‘take charge’ than Dr. Green from ER!” mode and rides with Riley (now in respiratory distress) in an ambulance to New England Medical Center (NEMC).

Riley spent the new two weeks at NEMC. While there, she underwent a cardiac catheterization to better diagnose the underlying problem. We knew she had pulmonary hypertension and mitral valve regurgitation. But we were still uncertain about the underlying cause. We contacted Dr. James Lock at Children’s Hospital Boston and he graciously offered to have his team review Riley’s records to date. They were as concerned as we, and encouraged us to bring Riley in so they could evaluate her in person. At first we resisted—on a subconscious level, perhaps we just didn’t want to admit that the problem was so severe as to require the kind of treatment that only Children’s can provide. We so badly wanted a healthy baby that for a few days we managed to convince ourselves we were on the right path at NEMC, and ignored much evidence to the contrary.

But we were fooling ourselves. We finally came to the realization that our child was profoundly ill and likely needed more help than NEMC could offer. We transferred Riley to Children’s. We were immediately impressed with the level of attention, the resources, and the team approach that the Children’s Cardiac ICU staff brought to bear. Additional tests confirmed that Riley was in rough shape, and mitral valve repair would likely be necessary. The team hoped to help Riley gain weight and size before any surgery—she was only a month old! But it soon became clear that her malformed heart simply wouldn’t give us the time we would have liked. Prior to the surgery, we sat with Pedro del Nido, MD, chief of Cardiac Surgery, as he explained the plan for the operation and answered our questions. We stared at his hands, dumbfounded that they would soon be manipulating tiny flaps of skin in our baby’s walnut-sized heart. riley_secondpic

During the bypass procedure that followed, Riley’s breastbone was cracked and the surgical team operated on both her tricuspid and mitral valves. Things were looking up for a while, but then she regressed, and had difficulty breathing. She dropped down to a little over 8 pounds in weight and wasn’t able to eat on her own.
It’s really no fun remembering all the stuff that followed during the remainder of Riley’s 109 days of hospitalization. Suffice to say Riley had another bypass, and once again she started to look very bad soon afterwards. At this point, our baby had gone through two bypass operations and three cardiac catheterizations. She’d spent the vast majority of her life in intensive care units, and lost several weeks of development to sedation. Often we couldn’t do anything other than read to her unconscious, helpless form, as she breathed via a respirator and was nourished via a dozen lines snaking into her body. It was hell. We thought she was going to need an artificial valve, and the prospect frightened us. There are many replacement valve success stories, but the process entails a life time of blood thinners and additional surgeries to put in larger artificial valves as the child grows.

When Riley went in to her third bypass surgery, we fully expected her to come out with an artificial valve. We were dumbfounded when Dr. del Nido was able to make a repair without having to go that route.

Since that last surgery, we have watched with wonder and gratitude as our little girl has blossomed. It was as if a switch was turned, from “fighting for her life” to “thriving.” There is no doubt in our minds that without Children’s we would not be what we are today: ridiculously proud parents of a beautiful, vibrant, healthy two-year old.

When we share Riley’s story with new friends, they’re eager to hear the resolution.  But this is not a neatly scripted Hollywood tale with a nice, tidy ending.  The story is ongoing, and while we’re enjoying the latest chapters, there may be conflict yet to come.  Children’s has become a part of our lives, and it brings us great comfort to know that they’re in our corner, continuing to fight for Riley and doing all that they can to make sure she has the quality of life we all deserve.

Read about another patient, Ava LaBarge with pulmonary hypertension, and check out this blog about living with the condition by 14-year-old Shannon.