CDH baby under the watchful
eye of Dr. David Kays
& Ray Bunch)
I was having
a pretty "normal" pregnancy with no problems initially. My AFP
test had come back with an elevated risk of Downs so it was recommended
that I have an amnio. I was 32 so I wasn't going to automatically have
one but I opted to do it due to the result of the AFP. (When I was pregnant
with my daughter three years prior, I had the same thing happen so I just
thought, "here we go again.") My amnio was normal and the detailed
ultrasound they did prior to it and during it revealed no abnormalities.
very relieved and happy I was carrying a healthy baby, so my pregnancy
proceeded with me feeling very well. I knew I was carrying a boy and my
husband and I felt very lucky and were trying to prepare our two year
old daughter for the arrival of her little brother.
About a week
before Christmas, I was 31 weeks by then, I went in for my normal checkup
and the doctor measured the height of my uterus. He said I was measuring
35 weeks along and that he wanted to do an ultrasound. I told him that
my first baby was big and that I wanted one done at 38 weeks so I could
decide if I wanted another cesarean. He said I could have one then, but
he wanted one now. (Thank God for him. This was the first step to saving
my baby's life.)
My husband and I went in a few days later. The ultrasound revealed what
was initially believed to be a problem with the heart. Our baby's heart
was shoved way to the left of his chest. We were devastated and when we
saw my OB, she gave me a big hug and said that she didn't know what was
wrong with the baby, but she was going to send us to specialists and that
she was going to do everything to help our baby. It took a couple of visits
with level 2 ultrasounds to narrow the problem down. The doctor couldn't
get a good picture of the baby and it was either a type of mass in the
chest, or a diaphragmatic hernia. (Initially they thought there might
be something wrong with the structure of the heart, but it was because
the heart was being smashed up against the wall of the chest.) The doctor
recommended to deliver at a hospital that has ECMO and explained to us
why. He gave us a couple of options,
the closest of which was Gainesville, FL (we live in Jacksonville.) He
had also mentioned the in-utero surgery they do in San Francisco but we
did not like the success rate percentage.) We met with the OB specialist
who did another ultrasound. He definitely could diagnose it as a diaphragmatic
hernia. He said that it was right sided and the intestines were probably
up there and most of the liver. We then met with Dr. David Kays who is
one of the pediatric surgeons at Shands Hospital and who also specializes
and has done many repairs of CDH. He went through all of the scenarios
that could take place. We all decided on a cesarean set for 38 weeks.
I would be given two steroid shots starting two days prior to my cesarean.
All this time was devastating. I was trying to be cheerful over the holidays
for my daughter's sake, plus I didn't think that crying 24 hours a day
would be good for the baby, however, I couldn't control my emotions at
night. I would pray and cry for hours every night until my pillow was
soaked. The one thing the doctors were able to get across to me was the
seriousness of this defect.
At my weekly visits and non-stress tests I would have at my OB's, I asked
her what I should do if I go into labor and she had said to call and come
right away and she would send me by helicopter if necessary to Gainesville.
I laughed at the idea of me going by helicopter and even told my cousin
to "pray my water doesn't break and I don't have to ride in some
damn helicopter!" At 37 weeks, that's exactly what happened.
I was at my mom and dad's with my daughter and I started leaking amniotic
fluid. I called my doctor and went to the office which was located at
Baptist hospital in Jacksonville. She verified I was in labor and called
Dr. Kays' office in Gainesville. They asked her to give me a shot of Beta
Meth and send me on my way. I was already dilated two centimeters, so
she decided to get me there as quickly as possible. My husband, mother,
and daughter, were there and said they would meet me in Gainesville since
nobody was able to ride with me. As soon as I was at Shands hospital,
they set me up on a magnesium sulfate drip to slow the labor. Dr. Kays
came in to talk to me and said they were going to hold me off for quite
a while because I needed some time to pass and then another steroid shot.
The had to put me on the maximum of the drip to finally get my labor to
slow down. The next day they were able to reduce it a little so I could
be a little more comfortable. The doctors would check me minimally so
as not to cause anymore dilating. I had been admitted on a Tuesday and
on Thursday morning, I was in a lot of pain and felt like I had dilated
more. They checked me and I was five centimeters and they said it could
not be stopped now. Dr. Kays came in and said that they would do the cesarean
at 11:30am and he would be in the delivery room ready to help my baby.
I had sent my family home since I didn't think I would be delivering this
early and the only reason my husband agreed is so he could work more so
that he could stay for a long time after the baby was born. I called him
to come back to Shands but there was not much time before I was taken
to the operating room. When they lifted my baby out, I heard someone say,
"Judy, look quickly!" I saw my son for a split second before
they handed him to Dr. Kays' team. I'll never forget that big "waaaa"
face with no sound coming out, except for a quick little noise that I
can't explain, sort of like what it sounds like when someone gets the
breath knocked out of them. I looked over to the little bed where Ian
was but I could not see because there were too many people around it working
on Ian. The two anesthesiologists were talking to me to keep my mind off
things and they would look over and then try to reassure me. I kept hearing
someone call out each minute that passed, and knew things weren't going
well. All this time I was praying as fast as I could and straining to
see my son. At one point, one of the anesthesiologists went to look at
Ian and then I saw him look at the other one and he was white as a ghost.
When four minutes had passed I still hadn't heard anything and then finally
Dr. Kays came up to me and said that Ian was very serious but they finally
got him stable enough and were taking him to NICU. After they took him
out of the room, someone came to tell me that my husband just arrived
and got to see Ian for a second, and that he would be waiting in the recovery
room for me.
I was taken back to my room to recover from the surgery. After an hour
or so, someone brought me a Polaroid of my son. He was on a ventilator
and almost his entire body was covered with wires and tubes. At two hours
old, we were told we could go to see him. I sent my husband and my mother
to go look at him and come back and tell me how he was. When they came
back, I begged my recovery nurse to let me go to see. She helped me to
the wheelchair and I was taken to see him. When I went in, Dr. Kays explained
to me how serious Ian was. Ian's apgar score had been 0 and 4. He said
he went a long time without oxygen in the beginning because he was very
hard to intubate. When I looked at Ian, the vent was working his body
so hard that his chest was dramatically rising and falling. The nurse
was there to answer any questions about all the wires or machines that
we needed to ask. I couldn't stop crying every time I looked at him. He
was so beautiful. He looked so
much like our daughter when she was born. He was a good size (7lbs. 14
* oz) which the Dr. said was in our favor, and also that it seemed he
had a strong heart. I would visit again and again. I couldn't stay away
from him. It was like I was trying to engrave his face in my mind because
I thought I would lose him. At about 3 o'clock in the morning, I went
to see him again, and there I saw the surgeon, Dr. Kays, still by Ian's
side. That is when I realized that we were in the best place we could
The next day, only 24 hours after he was born, Dr. Kays came to tell us
that Ian was not doing well and that he had a small window of time to
get his surgery done. He really didn't want Ian to go on ECMO without
having done his repair yet, so he was going to do the surgery even though
Ian was so marginal. He did Ian's surgery right in NICU because he was
too unstable to move. Ian made it through the surgery. Dr. Kays said that
85% of his liver and almost all of his intestines were in his chest. He
said that Ian basically had no diaphragm and he showed us the gortex that
he used as a patch. He said it was the biggest patch he's ever had to
do. The X-rays revealed that Ian's right lung was extremely small, about
30% of what it should have been, and his left lung was about 70% of what
it should be. Dr. Kays said Ian's lung and liver were bruised somewhat
because the vent was causing them to hit each other.
The following day, Ian's blood gases were so poor, he had to be put on
ECMO. That was the worst day of my life. Even though the ECMO program
was the initial reason we went to Shands, I was hoping all along that
it would not be needed. I was told that Ian's lungs had been worked too
hard and ECMO allowed them to turn down the vent and give Ian's lungs
a rest and that hopefully, his lungs would grow and expand with the room
in his chest recently created by the repair surgery.
I can't explain ECMO. It was the worst sight I've ever seen. I thought
seeing him with all the tubes, vent, and other stuff was bad, however
it was nothing compared to this. A huge machine was parked right next
to Ian's bed, with big fat tubes filled with blood going from the machine
surgically attached to his jugular vein. The only redeeming quality was
that for the first time, my son looked more peaceful because the vent
had been turned down and it was not pumping his lungs like it had been.
I could understand the concept of what ECMO could do for Ian. It would
oxygenate his blood so his lungs could be given a rest and hopefully start
to grow and work. I asked Dr. Kays how long he thought Ian would have
to be on ECMO. He said given how sick Ian was, probably somewhere between
10 and 14 days.
Life on ECMO was extremely hard. Ian would have one good day, two bad.
Then have two good days and one bad. At one point we were very encouraged
because a blood gas showed more oxygen that what they were giving Ian
which meant his lungs were beginning to work. He had to go through two
circuit changes (that's when they change out the whole machine with another
one filled with all new blood.) They would have to do that because the
blood in the machine would start to clot after about five days, which
would be extremely dangerous. After both circuit changes, Ian would have
a very bad day and a half, probably because it was a shock to his system.
At about day 10, Dr. Kays tried a second attempt of trial off ECMO. That's
when they fix the machine so they can see how Ian would do without it.
Again, Ian's blood gases were too poor and he had to put him back on.
Eventually, on day 16, it was time for another circuit change, but Dr.
Kays had been preparing the machine, turning the flow down for a few days,
hoping that this time Ian was going to do it. He made it off and the next
time I went in, the machine was gone. The next nine days, Ian was slowly
weaned off the vent. When he was 22 days old, I walked into NICU and Sandy,
Ian's nurse, had him dressed in a little shirt and had washed his hair
for the first time. She and Michele, the nurse practitioner, asked if
I wanted to hold him. I had never asked any of the nurses when I would
be able to hold him because I was afraid I would not like the answer,
but mainly because I knew I wouldn't be able to ask the question without
breaking down and crying. I would cry when I would see other parents in
NICU get to hold their babies because I so much needed to hold him.
They put an oxy-hood on him at first, then switched him to a nasal canula.
This is the first time I saw my baby's beautiful mouth. His cry was raspy
and weak, but it was music to my ears. At this point, he started making
progress in leaps and bounds. It was a continual positive progression
from here. They did an upper GI to see how bad his reflux was going to
be. The test showed minimal reflux. We started slowly introducing feeds
by mouth. I had been pumping breast milk and when they first placed the
NG tube, that was the first real food (not IV) he received.
Increasing the feeds by mouth was a painstaking process. Eventually I
was able to breastfeed. Since breastfeeding is not an exact science, every
day I anticipated when he would be weighed hoping for a nice gain. At
the same time the nurses were teaching me how to give Ian his meds by
mouth since I would be doing that once I took him home. After Dr. Kays
was satisfied with Ian's weight gain and progress, after 50 days in NICU,
we were able to take Ian home. He came home on portable oxygen by nasal
canula. I had a huge tank set up in his room and he had a 20 foot tube
so I could walk around the house a little with him. He was also on a heart
monitor constantly for the first few months and then just at night. He
had to take many meds by mouth every day. Nifedipine he took initially
because he had pulmonary hypertension, which he was off in a couple of
months. He also took Cisipride, Pepcid, which helped with reflux, and
Lasix. He remained on oxygen until his first birthday. In his first year
and a half he was hospitalized for respiratory infections, none of which
were RSV. As he gets older, he seems to get sick less often, and tolerate
the viruses better.
He is now
six years old. He is developmentally where he should be with
others his age, runs and plays with his older sister, and is in the 50%
for height and weight of normal children his age. Nobody can believe he
was as sick as he was. He looks so healthy and is extremely active like
any six year old.
Ian would not be here if it weren't for his surgeon, Dr. David Kays, who
is a pioneer in changing the way babies with Congenital Diaphragmatic
Hernia are treated. He has the best published diaphragmatic hernia data
with a survival rate of 92% of babies that are born at Shands. He and
the wonderful doctors, nurses, and therapists provided Ian's little body
with everything that it needed to grow and work and heal. We are so lucky
we found our way to Shands. We are so lucky in many ways, and knowing
how different it could have been, makes us count our blessings every single
day for our miracle baby.
Judy Bunch firstname.lastname@example.org
Are you a parent carrying a child with CDH? Click here to
contact Dr. Kays and his staff