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. At the time we decided not to find out the sex of our baby.

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.

"Your baby is not cooperating," they said, explaining the time it was taking. "She keeps moving around."

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 4 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.

In addition, we've expanded our mission to include research into hydrocephalus and associated neurosurgical conditions, such as: spina bifida, myelomeningocele, syringomyelia, brain injury, and brain tumors. Children with these conditions may also have hydrocephalus as a secondary condition, due to an abnormal CSF flow through the ventricular system. Research into these conditions is essential to these children. They need specialized pediatric neurosurgical care to manage their health and to sustain a higher quality of life.

We have also created an endowment fund so that funding will always be available in the future for research into the causes, prevention, and treatment of these neurosurgical conditions. Our 3 year goal is to raise $1.8 million, which would provide over $65,000 annually for research and over $12,000 annually for financial aid to families. Doctors and researchers at UVM College of Medicine and throughout the country will be able to submit research proposals and grant applications to the Madi's Fund Research Review Board. This board will review the proposals and award research grants on an annual basis, once the endowment fund is attained.

With the help of many personal and corporate donors from Vermont and Northern New York, the USA, and around the world, we believe that this goal is attainable. 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.

Looking back, I am amazed at what has transpired from Madi's hydrocephalus. She is a very happy little girl 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's hydrocephalus has been, and still is, a blessing in disguise.