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Spencer
Idiopathic GP
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Spencer was born on Valentine’s
Day, 1989. His entry into this world was far from how I had anticipated.
Instead of having a quiet peaceful birth, his birth was surrounded by
all kinds of medical technology. He had to be suctioned as soon as his
head was out to avoid aspirating meconium. He was then taken to a warmer
bed to be suctioned some more. He was in distress so he was just about
immediately taken to the NICU where he spent the next 3 weeks recovering
from sepsis caused by a fever I developed during labor. He was quite ill
with respiratory distress, septic shock and kidney failure.
When they began to feed him, it was obvious that there were problems
there as well. He had a difficult time taking formula and would spit up
frequently. He was diagnosed with gastroesophageal reflux. I didn’t
know much about it but thought it wasn’t too big of a deal as all
babies spit up.
After being at home for about 10 days, I learned otherwise. During a
middle of the night feeding, Spencer refluxed and aspirated. He stopped
breathing and turned blue. Again we were back to the hospital with a
very sick baby. This time with the diagnosis of aspiration pneumonia. He
recovered well and came home. At home, I continued to do all that I
knew. Every time he spit up, I re-fed what he spit up. Most of my day
revolved around feeding Spencer. Fortunately, there weren’t any more
episodes to scare me like that night.
At about a year old, I noticed that Spencer had stopped spitting up! We
were so happy. I thought that things were finally settling down. He had
frequent ear infections and other respiratory infections but I didn’t
think much about that nor did the pediatrician. As time went on, he
started having symptoms that should have made all of us think some more.
He started wheezing and coughing at night. I would go into his room an
find him blue. I continually brought him to the ER and to the
pediatrician but when he was there he was fine so they didn’t do
anything. The doctor I worked for finally convinced Spencer’s
pediatrician that there was something wrong that forced me to sleep
sitting upright so Spencer could lean against me as he couldn’t sleep
lying down. We then began home monitoring with an apnea monitor and
pulse oximeter.
The monitoring was recorded by the apnea monitor. It showed frequent
oxygen desaturation. Oxygen saturation should be at or above 95%.
Spencer frequently dropped into the 60s and 70s. He was then being
treated for asthma but that wasn’t helping the problems at
night.
Spencer had his adenoids removed when he was 2 thinking that his
respiratory problems were due to large adenoids. It didn’t help. He
then had his tonsils removed when he was 3. Again, it didn’t help.
Finally after recovery from the tonsillectomy, we saw a pulmonologist
for his respiratory problems. Immediately, the pulmonologist suspected
reflux as being his problem despite the fact that he didn’t spit up.
His suspicions were confirmed when we saw a pediatric
gastroenterologist. A pH probe was done which showed that Spencer was
refluxing 25% of the time. He was immediately started on medication and
an endoscopy was done. This showed that he also had esophagitis due to
long term refluxing of acid. We tried different med combinations. We
tried changing the meds and raising the doses. Nothing worked. Finally
all he would eat was yogurt. He was losing weight and was
miserable.
I changed GI doctors and he almost doubled the meds. Things still didn’t
get any better. Finally, after having three more endoscopies that showed
worsening esophagitis, it was decided that due to the degree of
esophagitis as well as Spencer’s ongoing respiratory problems, that
surgery was the best choice at this time.
Off the surgeon’s office we went. Determined and relieved that this
was indeed the answer for my almost 4 year old. We saw the surgeon who
explained that they would make an incision and take the top portion of
the stomach and wrap it around the esophagus to tighten the sphincter
muscle that wasn’t functioning and was allowing the reflux. The
surgery, called a Nissen fundoplication, was done. Recovery was hard.
Then it was time to let Spencer eat. He drank and vomited. He ate and
vomited. He swallowed saliva and vomited. This went on for several days.
We couldn’t take him off IV fluids because he couldn’t get anything
down. So, at almost 2 weeks after surgery, the surgeon did an upper GI
to check things out. It was found that the “wrap” was too tight. He
only had a 1 mm opening for things to go through. So, the next morning,
Spencer went back to the OR to have his wrap dilated. Almost immediately
afterward he could eat. No problems at all.
As time went by, it was obvious that not only could Spencer eat. He
could reflux too. It was very upsetting to watch this happening. The
symptoms weren’t as bad as they had been but things weren’t
pleasant. I took Spencer back to the doctor many times. The doctor told
me he couldn’t be refluxing because he had just had a fundoplication.
He had the pH probe and endoscopy repeated and the testing showed that
he was indeed refluxing again. I think the doctors really took things to
heart when Spencer had a GI bleed in December. Only 11 months after
surgery. At only 4 yrs old, he came into my room at about 5 AM to tell
me that he was burping up blood. My poor baby. All we went through to
have the initial surgery done and here he was in horrible shape
again.
This time we went to a different surgeon who did a different type of
surgery for this problem. Just 10 days short of a year after Spencer’s
first abdominal surgery, he had his second abdominal surgery. This time
it was an uncut Collis-Nissen fundoplication which also involved using
part of the stomach to lengthen the esophagus. He also had a
pyloriplasty done to aid with stomach emptying. This surgery was longer
and more difficult than the first. However, after he recovered, it
seemed like the surgery was helping. Eating was difficult again but
there was no anatomic reason.
Things were progressing nicely until about 9 months after surgery when
Spencer started having painful episodes of vomiting. This was very
unusual and very different from anything I had ever seen with him. We
went back to the GI. He did another endoscopy. When the doctor came out
after the endoscopy, he told me that there was a 2nd hole from the
stomach to the esophagus which allowed Spencer to reflux. There was a
lot of inflammation around it since acid probably just sat there all the
time. This needed to be corrected.
We went back to the same surgeon who had done the 2nd surgery and
Spencer had his 3rd abdominal surgery in less than 2 years. This time,
he had also been losing weight so the doctor placed a feeding tube.
Recovery was a little bit easier this time thanks to the feeding tube.
We didn’t have to worry about Spencer’s eating as much. However,
even with the G-tube, Spencer didn’t gain weight well. At 6 years old,
he only weighed 36 lb.
Spencer had several severe infections of the G-tube site during the
first several months and was hospitalized 3 times for cellulitis and
once for a necrotic abscess and peritonitis thanks to a little boy who
pulled his tube partially out. The doctor did an endoscopy while in the
hospital about 5 months after the 3rd surgery and found that Spencer
still had esophagitis. The 3rd surgery didn’t work at all.
We then spent the next 2 years trying to figure out why fundoplications
weren’t successful for Spencer. We were told that the success rate is
typically >90%. Why didn’t Spencer fall into this category? We
tried high dose steroids to see if it made any difference in his oral
intake and then repeated an endoscpy to see if there was inflammation
present while on steroids. There was. We also tried an elimination diet.
Spencer had a couple of foods that we knew he wasn’t allergic to and
was able to eat them. Otherwise, all he had was a formula called Neocate
1+ through his G-tube. We did this for several weeks and repeated the
endoscopy. Still no improvement.
Finally, the last thing I could think of was to go to the best Children’s
Hospital in the country. I found Boston Children's Hospital. I took
Spencer there in June 1997. We met a GI doctor there who was very
interested in Spencer. In a whirlwind week, Spencer had the full scope
of GI testing done. He had lab work, an upper GI, pH probe, esophageal
mamometry, and an endoscopy. They found that his previous fundoplication
was completely disrupted. His esophageal sphincter muscle was so far
open the doctor could see out Spencer’s mouth with the scope while in
the stomach. There continued to be reflux, esophagitis, and esophageal
dysmotility. The GI doctor there suggested that we try medications for
the dysmotility first in addition to the reflux meds he was already
taking. We tried one which helped the dysmotility but made the horrible
reflux worse. Then we tried another which had the same effect. When
those medications failed, it was again time to consider surgery.
The 4th abdominal surgery was done by a surgeon at Boston Children's. He
was kind and caring and explained the possible procedures he was
planning. He wouldn’t know just what he would do until he was in the
OR. After 12 hours in the OR, Spencer went to the ICU after having a
partial esophagogastrectomy, new gastrostomy, and modified Belsey
fundoplication. Recovery was more difficult than any of the other
surgeries and perhaps harder than all 3 combined. However, the surgery
was successful. After several months, Spencer was weaned from G-tube
feedings and was doing well. The G-tube was removed 10 months after
surgery. Spencer was medication free and we were thrilled!
He started to experience some abdominal pain, diarrhea and dizziness
about 15 months after the 4th surgery. He had had episodes of dizziness
in the past as well which we could never find a reason for them. The GI
doctor in Boston tested Spencer for dumping syndrome which is when the
stomach is emptying too rapidly. This happens frequently when the
stomach is small which Spencer’s by that point was very small. It
causes explosive diarrhea, pain, and hypoglycemia. That is what the
problem was and we were able to easily treat it with diet and cornstarch
which helps to control blood sugar. Things were good again. Spencer went
to summer camp for the first time and loved it! He stayed 4 weeks. After
camp however, he was burping and hiccupping which were always signs of
reflux for him.
We called the GI doctor and started Zantac. That didn’t help so he
started Prilosec. Testing was done yet again. The pH probe shoed that
despite not eating for most of the test, that Spencer was indeed
refluxing and there was mild esophagitis. The doctor added medications.
Things were under control for a little while and summer came. Spencer
went back to camp. This time for 8 weeks! I went to see him after 2
weeks. He looked horrible. Dark circles, hoarseness, coughing, and most
of all, he didn’t want to eat even though we were taking him out for
some fast food!
I talked to the GI doctor who felt that it was probably time to consider
surgery yet again. I thought and thought and thought some more. It just
sounded so complicated. Much more complicated than any of the prior
surgeries. I consulted with surgeons all around the country. There was
one in LA, one in Salt Lake City, the surgeon who did Spencer’s 4th
surgery had moved to Madison, WI, and we also consulted a very seasoned
surgeon in Boston. The surgeon in LA wanted to remove Spencer’s
stomach and attach it to his small intestine. The surgeon in Salt Lake
City wanted to replace his esophagus with colon. The surgeon in Madison
wanted to replace the bottom portion of his esophagus with small
intestine since the small intestine has motility which would push
anything that Spencer refluxed back into his stomach. However, he would
only do it if the surgeon who trained him flew out to help. The surgeon
who trained him was the surgeon in Boston. The doctor in Madison
suggested that we go straight to Boston as it would be better for
Spencer to be in Boston.
Shortly after Labor Day 2000, we went to visit Dr. Hardy Hendren at
Boston Children's Hospital. We had spoken in early August so he was
familiar with the case. He suggested the same surgery that his former
protégée in Madison had suggested. Obviously that was going to be the
procedure we would pursue. He also felt that he may take part of Spencer’s
colon to enlarge his stomach as his stomach was “tiny”. Dr. Hendren
is a very busy man even though he is much older than your typical
pediatric surgeon at age 75. The first day he had an open day in the OR
was December 13, 2000. Spencer was scheduled.
Early in the morning on December 13th, Spencer’s prep for the surgery
ended. It had started more than 12 hours earlier. At about 7:30 AM,
Spencer, his father, and I went to the OR. Spencer went into the OR at
about 8 AM. Dr. Hendren then came to talk with us. He writes his own
consent forms so that nothing is ever left out. After that, Spencer was
in Dr. Hendren’s hands. He remained there for almost 16 hours. At
about 12:00 midnight, Dr. Hendren emerged from the OR and immediately
told me we had done the right thing as our GI doctor had changed his
mind and hadn’t been in agreement with surgery at that time. Spencer’s
esophagus had been “destroyed”. He wasn’t able to do the stomach
as he felt he’d already been there too long. Obviously, this recovery
was far more difficult. Spencer had problems with pleural effusions,
pneumonia, a leak at the atastamosis where the esophagus and small
intestine were joined, and a GI bleed. He remained in the ICU for about
a week and was on a ventilator for the first 3 days. He continued to
require a lot of oxygen for about 3 weeks. Things finally improved and
he went home on G-tube feedings at the end of January.
In February, Spencer told me he was refluxing a LOT. Back to the
hospital we went where Spencer stayed for another 2 weeks. It was then
decided that Spencer definitely needed the surgery to enlarge his
stomach. We then went home again and tried it again. Things weren’t
good and Spencer was losing weight so we went back again to have a new
kind of feeding tube (GJ tube) placed that although you could feed into
the stomach it also had a port that bypassed the stomach. It took about
2 weeks to increase his feedings but once there, he gained weight like
crazy! 25 lb in 3 months! Spencer went back to school in mid April. He
managed to pass all of his 6th grade advanced classes and was allowed to
progress to all 7th grade advanced classes!
In late July, after being able to enjoy several baseball games, a trip
to the Jersey shore, and a trip to Fredericksburg, VA, we headed back to
Boston to have the gastric augmentation. I wasn’t feeling very good
about things. I had no clue if the surgery would work or not but I had
to give Spencer every chance to live life as normally as possible. So,
another huge surgery prep begun. Spencer went into the OR this time at
about 11:00 AM and was out by midnight. His surgical recovery was smooth
this time! He and I were very excited! However, when it was time to
start feeding, it didn’t go well. Spencer wasn’t hungry and wouldn’t
take anything orally. They tried his G-tube at a low rate. However, what
was left after 3 hours of feeding was more than what had gone in! With
medications added and time, things got good enough that Spencer could be
fed and we could go home!
Remarkably, Spencer is doing very well especially considering the road
we have traveled despite persistent obstacles. He continues to have
problems with reflux, bile reflux, dysmotility which is possibly
throughout his gut, chronic pain, partial small bowel obstruction and
asthma. He remains on a lot of medication but is doing quite well
despite that list of diagnoses. There are good times and bad times. Our
bad times are bad but we truly appreciate the good times. He is doing
very well in school. He recently took the SATs as an 8th grader and
scored 1040! Dr. Hendren is an amazing surgeon and an amazing human
being. He will always hold a very special place in my heart. I can’t
even begin to imagine where Spencer would be today without Dr. Hendren.
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