Snow, Richard Carl

[left:a887e08c63]http://www.cherubs-cdh.org/Album/new/snow2.jpg[/left:a887e08c63]My husband John and I spent a year and a half trying to become parents. Finally, with the help of a reproductive endocrinologist, we found ourselves expecting twins. Because of my age and because there were two babies, the high-risk OBs kept watch over our progress with frequent ultrasounds. We found out that our twins were a boy and a girl—perfect!

About 20 weeks into the pregnancy, the doctors swept away the happy blue and pink fog we were living in. They noticed that our son’s heart was on the right side of his chest rather than on the left. After a couple of weeks of observation and speculation, they informed us that he had a left-sided congenital diaphragmatic hernia (CDH), and that some of the contents of his abdominal cavity were protruding into his chest and pushing his heart over. The literature they gave us on CDH, (pages photocopied from a medical textbook), described the surgical repair and estimated a 50% chance of survival. It also gave a long list of possible complications and after-effects, some of which could last a lifetime.

Next, the doctors began closely examining Richard’s tiny heart. They thought they could see a hole in the ventricle separating the two lower chambers, or a ventricular septal defect (VSD). They explained that a VSD can be quite serious, but that it is at least a birth defect with a longstanding solution. Successful surgical repairs have been done for decades. Provided the hole is not too large or awkwardly located, and provided the surgery is done well, the baby usually gets well quickly and has few to no long-term problems.

The senior OB had just returned from a conference where he had seen a presentation by a pediatric surgeon who claimed to have a high survival rate with CDH babies. Luckily, this surgeon was at Shands Hospital in Gainesville, Florida, only 75 miles south of where we live in Jacksonville. The OB also explained that many CDH babies need a bypass machine called ECMO, which they have in the NICU3 at Shands but do not have at any of the hospitals in Jacksonville.

Shortly thereafter we visited Dr. David Kays in Gainesville. He impressed us with his confidence and energy and with his record when it comes to diaphragmatic hernia kids. He has a 92% survival rate with CDH babies, as opposed to the usual 50-60% rate. Dr. Kays attributes his success not so much to the way he performs the surgery—many good pediatric surgeons can repair a diaphragm—but to the timing of the surgery, the timing and administration of other treatments both before and after the surgery, and especially to the “gentle” ventilation he uses to avoid damaging delicate, neonate lung tissue.

The delivery of the twins is a story in itself--suffice it to say that at 34 weeks gestation, both babies were delivered in Gainesville via C-section at 12:04 and 12:05 A.M. October 17, 1998. Isabel weighed 3’14” to Richard’s 4’13”. As the OB lifted him out and swooped him over for me to give a quick kiss, Richard gave one hoarse cry. I had been told he wouldn’t be able to make a sound. Dr. Kays, who had been called at his home at 11:30 P.M., was in the delivery room. He and a respiratory therapist ventilated Ricky immediately.

After birth, we found out that Richard had a considerable amount of bowel in his chest cavity along with his spleen. The heart defect stopped being a possibility and became a certainty. He had a medium-sized hole in his septum. Each defect was bad enough to be considered serious on its own, which made the combination grave.

Dr. Kays literally spent days at Ricky’s bedside in the beginning. He used a patch to repair Ricky’s diaphragm when he was about 34 hours old. The surgery went well, but the pulmonary hypertension that is usual with CDH kids set in with a vengeance. Within hours of the surgery, Dr. Kays had to hook Ricky up to VA ECMO to give his lungs a chance to rest.

The dangers of ECMO had been described to us, especially the potential for brain damage. The nurses relieved our anxieties a bit on this score, because we could see that they were extraordinarily careful. Around the clock they checked equipment, looked for clots in tubes with a flashlight and, when Dr. Kays wasn’t in the unit, phoned blood gas numbers to his beeper. He read them, day and night, and phoned back with adjustments for ECMO and/or ventilator settings. Ricky never had a problem. ECMO looks intimidating with its garden hose-sized, blood-filled tubes, especially to someone unused to hospital sights. We became grateful for it anyway, because it definitely helped save our son’s life.

Towards the end of Ricky’s third week on ECMO, Dr. Kays left to speak at an out-of-town conference, and another pediatric surgeon took over our baby’s care. Dr. Langham had just returned from the same conference to which Dr. Kays was going. On his Friday evening round, I told him that we were eager for Ricky to get off of ECMO.

Earnestly, Dr. Langham told me that he had presented Ricky’s case at a roundtable discussion at the conference, and that the pediatric surgeons there had said “to a man” that they would “withdraw therapy” and let him die. He wanted to let me know that we “shouldn’t necessarily expect a positive outcome.” Ricky’s CDH problem was serious on its own, and few babies with an additional heart defect had ever survived.

I was distraught at first, especially with Dr. Kays out of town. When I told my husband what Dr. Langham had said, we commiserated, then pulled ourselves together a bit and decided that, for some reason, we both still believed that Ricky would get well. Dr. Kays had said before he left that he thought Ricky was getting stronger and could make it, and we thought so too—we just wished we could know for sure. We were concerned, however, about having a doctor who felt pessimistic about our son’s outcome in charge of his care.

Dr. Langham may have thought Ricky was losing the battle, but to his credit he did not stop helping Ricky fight. Over that weekend, he managed to get Ricky off of ECMO. Instead of reducing the flow of blood, he reduced the amount of oxygen going into the blood. (I may have that backwards.) After 21 days of dependence, Ricky hung in there and got rid of the machine. Gone were our reservations about Dr. Langham!

Ricky’s next achievement was supposed to be getting off the ventilator. Everybody hoped he would be able to breathe on his own before he had surgery to repair the hole in his heart. He could not do it. The VSD caused his heart to shunt extra blood to his struggling lungs, which made them too wet and heavy for him to breath unassisted for any length of time. This meant that Dr. Kays had to lobby the cardiac surgeons to go ahead with the VSD repair as quickly as possible.

Like most organizations, hospitals are endowed with a certain measure of politics, a pecking order, territories, etc. The two cardiac surgeons who helped Ricky, Dr. Knopf and Dr. Alexander, are wonderful people and fantastic doctors, but they did not relish having a pediatric surgeon telling them when to perform a surgery on a patient, nor did they want him managing that patient’s ventilator settings during and after the procedure. It took delicate persuasion and persistence—Dr. Alexander informed me that “if Dr. Kays tells me one more time about that baby’s lungs, I’m going to throw him out that window over there”—but Dr. Kays convinced them to go ahead when Ricky was about six weeks old.

The heart surgery went well. It left Ricky wiped out, of course, and there were niggling issues about keeping his heart in rhythm, but Dr. Knopf (another of Ricky’s saviors!) had done an excellent job, and within a month Ricky got off of the ventilator for good. He grew stronger, more alert, and gained weight. We thought it would be smooth sailing from there, and in many ways it has been--he has never been in danger again--but we never reckoned on the feeding problems.

Like many CDH babies, Ricky had gastroesophogeal reflux. Because he had been on a ventilator during his first two-and-a-half months of life, he hadn’t had a chance to suck and swallow at a crucial time. In fact, he had been on IVs and had never even had anything in his stomach until after he got off of the ventilator. To make an extremely long, frustrating story short, we tried everything to get him to eat on his own. He did eat, badly, reluctantly, but he didn’t gain weight.

The solution offered to us was another surgery, a Nissen Fundoplication, which would prevent him from vomiting. This would not only help to make him more willing to eat, but would protect his lungs from damage caused by stomach acid. During this procedure, Dr. Kays would also insert a G-tube into Ricky’s abdomen that would enable us to put formula and/or breast milk directly into his stomach.

The Nissen sounded like mutilation to me. Occasionally, during a lifetime, a person needs to throw up and frequently needs to burp. I wanted my son to be able to do both of these things on an as-needed basis. The G-tube didn’t sound so bad because it was a measure to temporary help him grow and make up for lost time.

After six weeks of nurses, occupational therapists, and us trying to feed Ricky enough, John and I finally agreed to the surgery. Dr. Kays gave him the Nissen, inserted a G-tube, and circumcised him to boot.

Ricky recovered rapidly, and all four of us went home as a family when he was 4½ months old, March 6, 1999. He weighed 8 pounds 4 ounces.

Thanks to Dr. Kays’ gentle ventilation, Ricky’s lungs are in good shape. He never needed oxygen at home. Although it takes him about a day longer than his sister to recover from a cold with a cough, he’s never had any serious respiratory complications and has no asthma. At 33 months of age, he does not appear to have any brain damage, neurological damage or hearing loss as a result of being on ECMO for such a long time.

Ricky’s eating has slowly improved, and his G-tube was removed in November of 2000. He now enjoys food. In fact, he samples new things with more eagerness than does his sister. Unfortunately, he still doesn’t like to eat the quantities he should, so he remains thin. However, he is gaining weight (26 pounds and some change now) and growing (36 inches tall) without a G-tube.

He has had some mild developmental delays but is catching up rapidly. At two-and-a-half he was discharged from speech therapy and will finish physical therapy and occupational therapy when he’s three. He’s mostly where he should be or even ahead of himself, but there are small gaps here and there in what he knows and in what he is able to do.

I was afraid that all that time in the hospital, three surgeries, needles, tubes, etc. so early in his life would make him mistrustful and withdrawn, but he’s just the opposite. He’s an affectionate kid who loves to cuddle, likes to meet new people and talk to them, and who even gets along with his sister most of the time. He tells jokes. Last week he turned to me after watching a movie and, as slyly as is possible for a 33-month old, said “Mary Poppins changed my diaper!”

Ricky had the following strikes against him in addition to his CDH; he had a serious heart defect, he was six weeks premature, he was a twin.

Our son was terribly, terribly sick, but he isn’t now. The care he received in Gainesville certainly saved his life. John and I thank God every day that we found Dr. Kays. Our hope is that the techniques Dr. Kays uses at Shands will soon become more widely understood and accepted so that more CDH babies can go home with their families.


Written by Richard's Mom, Catherine Snow (Florida)
2001