Madi's Story: Our Blessing in Disguise
By Debra Jaurigue
On Sunday, April 27, 1997 at 2:07 PM, our family was
blessed by the birth of a beautiful little girl, who we'd named Madison
Claire months before she arrived. Her delivery had been carefully
planned with the high risk maternity doctors at Fletcher Allen Health
Care in Burlington, Vermont, but Madi surprised us all by arriving in a
very quick delivery one week earlier than expected. Why were we dealing
with "high risk maternity doctors"?
Well, at the beginning of my pregnancy things had been
progressing without any complications or complaints. I'd taken the
triple marker test, which came back "normal," relieving our concern
that the baby would test positive for Down's syndrome or some other
"deformity." A routine ultrasound was scheduled for January 8, 1997,
and that test went smoothly as well.
I went home to play with our 18 month old daughter,
Tamra Kaye. About 45 minutes later, the phone rang. It was Beth, my
doctor. She said that she had received a call from the radiologist
about the ultrasound. He had detected an abnormality in the size of our
baby's left and right ventricles, which led him to believe she had
"hydrocephalus." My heart stopped.
"What is hydrocephalus?" I asked in a panic.
Beth told me what it was and said that she was
referring us to specialists in Burlington, Vermont—a 2 hour drive
from where we lived.
After the phone call, I sat crying, not knowing what
to do, wondering what this hydrocephalus really was. I was miserable
because we did not have a "perfect" child growing inside me. I called
my husband, Jeff, at work, and he quickly went in to shock too. In a
matter of minutes, our lives went from being comfortable, healthy, and
happy to lives of panic, uncertainty, and fear.
We called Jeff's parents, who are doctors, to get more
information about hydrocephalus and what lay ahead for us and our baby.
Unfortunately, no one we knew had any real firsthand knowledge of
someone with hydrocephalus. So we were left it the dark, until we met
with the specialists.
That was a new facet in our lives
now—specialists! We met with the high-risk maternity doctors and
radiologists, who performed a 2 ½ hour ultrasound on me.
After being given all the pertinent information about
our baby's situation, and the risks involved, we decided to have an
amniocentesis, so as to rule out any other complications or
deformities. We also decided to find out the sex of our baby.
During this initial specialists visit, we also met
with pediatric neurosurgeon Dr. Steven Wald. He explained hydrocephalus
and the shunt surgery in detail, and even showed us the tubing that he
would put in our baby.
"It's amazing what they can do today," I thought.
Dr. Wald offered us some hope when he said, "Your baby
could possibly grow out of the hydrocephalus by the time she's born,
and not require a shunt."
It was this knowledge that kept my spirits lifted and
enabled me to concentrate on taking care of myself during the
pregnancy, so that our baby would have the best chance possible.
The only unusual thing about Madi's delivery was the
number of doctors and nurses in the room: 13 of them in total. I
delivered her naturally (the painful, natural way) with the high risk
maternity doctors and neonatal doctors in attendance. Madi was
"healthy" and her Apgar scores were good. I was able to hold and nurse
her before they took her to the Neonatal Intensive Care Unit (NICU) for
observation.
Madison spent a week in the NICU, and on day 4 she was
intubated and sedated for an MRI. The results showed that both her left
and right ventricles were extremely dilated; there was a blood clot of
unknown origin in her right ventricle and she had a Dandy Walker
variant. The Dandy Walker is a cyst in the fourth ventricle. The cyst
can interfere with the body's ability to drain cerebrospinal fluid
(CSF) from the brain, resulting in hydrocephalus.
Her head size was in the 95th percentile for a newborn child.
We were allowed to go home for 3 days, then returned
for her shunt placement surgery. Madison was 1 ½ weeks old. The
surgery was a success, and after 2 days we were discharged, and sent
home to start loving and caring for our now "normal" child.
At least, that's what we thought. You have something
wrong with you, the doctors operate, fix it and you go home never to
have to deal with it again! Problem solved!
We managed to convince ourselves of this even though
Dr. Wald had told us Madison only had a 55% chance of not needing a
shunt revision. Boy, were we in for a rude awakening!
In 5 days, we saw Madi's head go from 38 cm to 42 cm.
Her forehead was stretched so tight it was shiny. Of course it happened
at the worst possible time. We were on vacation, visiting Jeff's
parents in Philadelphia. So, off to the Philadelphia Children's
Hospital we went, for a consultation with a pediatric neurosurgeon.
The doctor took one look at Madi and immediately knew what was happening—
her shunt was malfunctioning and she needed a revision. He assured us that
she would be fine until the following week when we'd be home from our vacation.
This was because the sutures in her skull were not yet sealed and could move
about, giving her more room to handle the pressure and extra CSF in her
brain. We called Dr. Wald, gave him the news, and scheduled a revision
for the following week.
This was only the first of many revisions. Madi had a
revision every 10 days from the time she was 1 ½ months old
until she was 3 ½ months. After the third revision, the reality
of hydrocephalus set in. This was our normal life! I needed to accept
it, learn how to deal with it, and move on.
This was not an easy thing to do. I kept wondering
what I had done wrong. The guilt of not being able to help her or "fix"
her kept gnawing at me. My mind kept struggling to find some way to
cope with all of this helplessness. I decided I had to try to make the
best out of a bad situation. Maybe I could start a research fund for
hydrocephalus. But that would take so much work! I pushed the idea on
to the back burner.
Our lives got busier and Madi got more complicated. At
3 ½ months, Madi needed one more revision. Unfortunately this
revision did not relieve the pressure and it landed her in the hospital
with one externalization of the shunt and 6 more surgeries. She was
negatively affected, the failed revision compromised her vision, and
left her with a right hemiparesis. After 6 weeks of multiple CAT scans,
x-rays, and the placement of 2 shunts, we took her home, not knowing
what the future held for her. She went into the hospital a normal 3
½ month old (with hydrocephalus) and came out looking like a rag
doll.
My heart was broken, and our life was uncertain—again!
Madi started physical therapy and early intervention
services. The doctors could not tell us what to expect. I slipped into
"day by day" mode. With a lot of support from family, friends and the
medical community we pushed on—trying to fit Madi into our
"normal" life. But we weren't living. Just surviving.
After 3 months passed, Madi returned for a scheduled
CAT scan. The results showed that the 2 shunts (in her right and 4th
ventricles) were working. However, her left ventricle was extremely
dilated, taking up most of her left hemisphere of the brain. To make
matters worse, the ventricle was separated into 3 compartments. Not
only did she need one more shunt placed to manage her hydrocephalus,
but neuroendoscopic surgery was needed to open up the compartments of
the ventricle so the CSF could be drained by one shunt—not three.
This procedure was a success—for only 2 months.
One week in February 1998, Madi had a slight change in
her mood. We had scheduled a neurology appointment in Burlington
because we thought she was having some 'absence' seizures. While we
were there, I decided to stop in at Dr. Wald's office to mention her
mood change to him and get his thoughts on this. He wasn't in that day.
The neurosurgery resident said it was probably nothing, but suggested a
CAT scan so we could return home (2 hours away) with some peace of
mind.
The scan showed a slight dilation of her left
ventricle. We all knew what that meant—yet another revision. She
went into surgery 2 hours later. The neurosurgeon replaced her
ventricular catheter which was completely obstructed. Luckily, we had
caught the malfunction in an early stage and Madi bounced back right
away.
During our 2 day hospital stay, I kept thinking, when
will this end? The idea of the research fund came to mind once more.
That was when I told myself I either had to go for it or stop thinking
about it.
After some phone calls to the Hydrocephalus
Association, the Hydrocephalus Research Foundation, and three local
hospitals, I spoke with Dr. Steven Wald about my idea. With the support
and nonprofit status of Fletcher Allen Health Care in Burlington,
Vermont, my research fund came to be in April of 1998.
Madi's Fund for Hydrocephalus Research and Financial
Assistance for Families with Children Hospitalized is, of course, named
after our daughter, Madison Claire. She's my motivator for all the
dedication and work I need to make Madi's Fund a success.
We had four Dinner & Silent Auction fund raisers. We
did mailings to family and friends. Support came from local businesses.
In just two years we managed to raise over $41,000.
In September 2000, Madi's Fund became affiliated with
University of Vermont (UVM) College Of Medicine in Burlington, Vermont.
This provided us with direct access to neurosurgeons; fellow resident
doctors; the latest research equipment, technology, and laboratories;
and the support of the head of surgery and neurosurgery at both
Fletcher Allen Health Care and UVM Medical School.
(Updated July 2009)
Since
Madi’s Fund conception in 1998, we have held 11 fundraisers which
to date has raised $200,000.00 to raise awareness of hydrocephalus, to
promote research and to encourage education and understanding of
hydrocephalus in the community at large. This has been done
through the help of many local and corporate donors, to whom I am
grateful to.
Madi’s Fund consists of a research fund that awards an annual
research award to a prominent doctor to study an aspect of
hydrocephalus. Research fund proposals are sent to doctors and researchers at UVM
College of Medicine and throughout the country in an effort to submit
research proposals and grant applications to the Madi's Fund Research
Review Board. This board reviews the proposals and awards a research
grant on an annual basis.
Additionally, Madi’s Fund has an endowed annual Lectureship at
UVM College of Medicine that invites prominent Pediatric Neurosurgeons
to give a lecture and round with the residents and medical students, in
an effort to bring the latest groundbreaking procedures and research to
the Vermont medical community.
What greater gift can I give to Madi and all her friends than creating
a healthier and better world to live in? Madi's Fund is my way of
giving to those in need and thanking the medical community for what
they have given back to me, a healthy and happy child—my Madi.
Since 2000, Madi has had a long road traveled in and out of
hospitals. In short, she is now 12 years old and will be starting
5th grade this fall at Rutland Intermediate School, being mainstreamed
with all of her friends and taking Special Education classes. She
has undergone a total of 50 shunt revisions and 8 posterior fossa cyst
surgeries. The longest time she has gone without a surgery was
just under 4 years. Amazingly, she has the strongest spirit and
cheerful smile. Madi loves school and all her friends. She
is active in skiing, swimming, loves crafts, books, TV, computer games
and jokes. From the time she wakes up in the morning until the
time she goes to bed at night, Madi does not stop enjoying life to its
fullest. She is an amazing teacher and mentor of “What is
truly important in LIFE!”
Looking back, I am amazed at what has transpired from Madi's
hydrocephalus. She is a very happy young lady who brightens our days
with her big dark eyes, her giggles, and her joy for life. You would
never know she has hydrocephalus, except for some developmental delays.
God has blessed us with Madi—His special gift—and has
enlightened my life with much knowledge, strength, courage, new
friendships, and a new understanding of what is important.
Looking back to that day when I was told about Madi's hydrocephalus,
the only thing that I'd change is who would have the
hydrocephalus—me.
Madi is definitely “My Blessing in Disguise.”
Fondly,
Debra Puplawski-Jaurigue
Madi's Fund Founder & Director
