I’m veering off course in my blog today in honor of CDH Awareness Day. CDH stands for congenital diaphragmatic hernia. Though it’s a term most people have never heard, it’s as common as cystic fibrosis and spina bifida. The hole in the diaphragm allows for the abdominal organs to enter into the chest cavity leaving little room for the affected lung(s) to develop. To this day, 50% of the babies born with CDH do not survive and those who do face a lifelong battle with complications.
CHERUBS, one arm of CDH International, is a charitable support organization for those affected by this affliction. To help spread awareness, CHERUBS asks us to share our story. Read on if you would like to know Hillary’s story told through her mother’s eyes. I imagine most everyone reading this blog has played a role in her life—I thank God for each of you.
“Mrs. Leipold, your baby has a diaphragmatic hernia.” Those were the words delivered by a doctor following an echocardiogram in March 1996. Those words were game-changing. An ultrasound in January had picked up a suspected problem and resulted in a referral to a specialist in Pittsburgh.
Following the CDH diagnosis we had about a month to digest it before meeting with the pediatric surgeon and chief neonatologist at Allegheny General Hospital in Pittsburgh. Things were much different 23 years ago in terms of technology. I was just starting to use email at that time and “googling” was not yet a thing, at least not to me. Like most parents of CHERUBS, my husband and I had never heard of CDH. Our primary means of educating ourselves about CDH was through the doctors. When we met with the neonatologist and surgeon in April, I didn’t even know what questions to ask because I knew so little about it. That appointment was completely overwhelming because the medical jargon went right over my head. Being a math teacher, there was one thing I could understand at that appointment—percentages. My big take away that day was that our baby girl had a 50% chance of surviving IF she was born in the right place at the right time. The odds were substantially worse if she wasn’t. We set a date for a c-section that day with the hope that Hillary would stay put until June 14. She was expected on June 30th. At that time, we lived about 2.5 hours from Pittsburgh. One thing that reinforced the severity of the situation was being told that if I went into labor before the scheduled c-section, they would take me by helicopter to Allegheny General. Yes, that got my attention! I remember crying the whole way home from that appointment.
The two months following that appointment were the longest two months of my life. There was just so much uncertainty. We were (and still are) blessed with a wonderful support system in the form of family and friends. They rallied around us during the wait for Hillary’s arrival and have been with us every step of the way since. A few weeks before she was born, I experienced my first panic attack. I was anxious for her to come into this world, yet scared to death what might happen once she did. There were a lot of “what if” scenarios playing out in my mind.
When we flipped the calendar to June, I went to stay with my sister and her family, who lived near Pittsburgh, in order to be closer to the hospital just in case Hillary decided to come out and play before the scheduled c-section. Thankfully, she was content right where she was. Hillary Jayne was born, as scheduled, on June 14, 1996, my parents’ 43rd wedding anniversary.
Again, we did not have the technology that we have today back in 1996. After Hillary was born, she was whisked away and put on a special oscillating ventilator immediately. A nurse brought me my “first picture” of Hillary once she was stabilized a few hours later. It was a Polaroid. Those are the only pictures I have to tell of Hillary’s early days. I was not able to see her with my own eyes until the following day.
Hillary was paralyzed by narcotics for the first week of her life in order to help stabilize her in preparation for her repair surgery. That surgery took place when she was 6 days old. It’s interesting what I remember nearly 23 years later as I write her story. I’ll never forget the words of the anesthesiologist just before they wheeled Hillary off to the OR. She said, “I suppose I don’t need to tell you how sick your child is.” The stress-o-meter was through the roof BEFORE she opened her mouth. I wanted to slap her, but thought better of it! When the surgeon came to the waiting room to talk to us following Hillary’s repair surgery, I simply burst into tears. I didn’t even know what he had to say yet. Dr. Hechtman was able to relocate the organs that had moved up into her chest cavity to the general vicinity of where they were intended to be and patch her diaphragm together. She was able to keep her spleen, but we found out in the last few years that it is located front and center rather than in the upper left quadrant of her abdomen under her ribs.
The Summer of ’96 was quite the rollercoaster ride. Dr. Null, the chief neonatologist, described Hillary’s journey quite well as the summer wore on. He said it was a series of “two steps forward, one step backwards.” That, it was. She made pretty good progress during the first month following surgery. I was thrilled to hold her for the first time on July 9. Then it got a little rocky. The surgeon decided to perform a bronchoscopy in an attempt to open her left lung a bit. We THOUGHT it was an overwhelming success only to find out a few hours later that it was just the opposite. She had a hole in her lung! She went back on the special oscillating ventilator temporarily and inherited a chest tube as a result. Shortly thereafter we learned that her formula was leaking into her chest cavity and there was yet another hurdle to jump. The forward progress trumped the setbacks and Hillary was discharged from the hospital on September 9 when she was 88 days old. My second panic attacked occurred the weekend before her release when I realized that my husband and I would now be responsible for her care. I wasn’t qualified!! That brings me to one of my favorite quotes—“God doesn’t call the qualified, He qualifies the called.” He called my husband and I to be Hillary’s parents and has been equipping us for that role ever since.
We brought Hillary home with a monitor, but thankfully she did not need oxygen. There is nothing quite like hearing that monitor go off in the middle of the night! It is pretty hard NOT to be a basket case as a parent of a CDH baby. The antennas go up with every cough, sniffle and sneeze. Any unusual pain puts us on high alert. You just want to put them in a bubble so harm cannot reach them. However, since that’s not realistic, my faith has served me well. I’ve never been in charge anyhow! I rely on God to show me where my responsibility ends and His begins. I can only do my part. I must trust Him with the rest.
There has been no shortage of speed bumps along the way, but, I’m happy to report that Hillary is looking and feeling better than ever as she approaches her 23rd birthday. Again, that summation “two steps forward, one step backward” has pretty much applied to her whole life. We are blessed that Hillary has never been admitted to the hospital since her initial discharge. Her diaphragm is held together by gore-tex and it has served its purpose up to this point. As is fairly common with CDH children, weight gain has been a struggle for Hillary for most of her life. Most people only wish they had that problem! If you saw her eat, you would be pretty amazed at how much she can put away. There were a few things the doctors told us to expect as she grew up—asthma and scoliosis. Their advance notice was correct, but thankfully neither issue has required any extraordinary measures. The scoliosis causes its share of musculoskeletal pain, but the curve itself isn’t severe. I’ll never forget when I first noticed it. It was a picture from her Confirmation when she was in 8th grade. I lived with her all that time, but it took a picture for me to notice that her shoulders go “downhill” from right to left. Perhaps it only became noticeable during a growth spurt in adolescence.
The biggest hurdle, and most recent, took quite some time to diagnose. Two years, in fact. As I recall, it started as a cough that just would not end in 2014. Her cough was accompanied by chest pain. That always puts CDH parents on high alert. After a few unsuccessful attempts to treat Hillary’s cough, her pediatrician heard a “rub” while listening to her heart and referred Hillary to a cardiologist at Children’s Hospital in Pittsburgh. He diagnosed Hillary with acute pericarditis in the spring of 2014. There was fluid around her heart. She had lost a substantial amount of weight by this time and was having frequent weight checks at her pediatrician’s office. The fluid had resolved by early in the summer only to resurface at the end of the year. The following July, a different cardiologist wasn’t so sure it was pericarditis at all. He suspected that it might have something to do with her pectus excavatum and referred us to a pediatric surgeon. In the meantime, she had a CT scan and it showed lesions on her right lung that would indicate infection. Even though Hillary’s pectus is fairly severe, the surgeon did not feel that a repair would alleviate her symptoms and did not recommend proceeding to surgery. The ball was thrown back into the hands of her pulmonologist in October. That led to a bronchoscopy in January of 2016. Though the preliminary pathology was negative, eventually Hillary tested positive for mycobacterium avium. A second sample verified that diagnosis. That put Hillary on the fast track to the Infectious Disease department in March, just over two years after the cough/chest pain began. She had many doctors stumped along the way, including the ones in the Infectious Disease department. “Lady Windermere Syndrome” (Hillary’s mycobacterial avium infection) almost always occurs in older, thin women with a history of pectus excavatum. Hillary was prescribed 3 high powered antibiotics to treat her infection. She took all three for an entire year. Thankfully, she responded beautifully without too much fallout in terms of side effects. Three years from the start of it all, she was symptom-free. Hillary has really done well since that difficult season of her life. She put all that lost weight back on and even added to it once she was finished with the antibiotics. Her primary issues these days are related to her scoliosis. That creates havoc with her musculoskeletal system. She finally looks healthy though!
Hillary will be celebrating her 23rd birthday in June and we thank God for every one of them. She is currently pursuing a doctorate degree in Occupational Therapy at Gannon University’s Ruskin, FL campus. Since she benefitted from occupational therapy as an infant, she’d like to focus on children in need of such services as a way of giving back.
