Real Hope for CDH
Affectionately known as "KAYS' KIDS"
Learn about a 92%
survival rate for CDH babies born here at Shands
Learn about a 92%
survival rate for CDH babies born here at Shands
Dr. Kays' Team Has a 92% Survival Rate for CDH babies born at Shands
CDH baby under the watchful
eye of Dr. David Kays
Ricky Snow (Cathy & John Snow):
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 CDHs (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 aftereffects, 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 to say that at
34 weeks gestation both babies were delivered in Gainesville via C-section
at 12:04 and 12:05 AM 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 PM, 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 his
life. 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, fairly well at first, then not so well, and he did
not 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 temporary measure to 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 4_ 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—nothing
out of the way for a preemie who had three major surgeries during his
first three-and-a-half months.
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.
Cathy Snow cathybsnow@comcast.net
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