Why her?

That question bubbled up right from the day of her extraordinary birth. Why did this happen to our daughter? Why her?

We’re not smokers, and we drink infrequently. Lisa knew she was pregnant from early on, and over the nine months she followed every rule. She didn’t take a single Tylenol. But from the moment the doctors removed her via C-section, her physical challenges were obvious. A club foot and a vertical talus. Two dislocated hips. Severe contractures of her joints. And so we asked.

Why her?

Sometimes the question reeked of self-pity. ‘Why her?’ translated to ‘Why us?’

Part of the allure of getting a genetic diagnosis was an explanation for what had happened. Syndromes have different causes, and some are anomalies. When the geneticist told us our daughter had Opitz trigonocephaly, we became consumed with the complications and the implications. As in a fairy tale, the diagnosis brought three consequences: she would have profound mental delays, she wouldn’t live to be a year old, and there was a one-in-four chance that the condition would recur in any future pregnancy.

But the diagnosis proved wrong and the geneticist recanted. And so the question rose up again in the shadows of our lives. Why, then? Why had this happened to her?

I went through a difficult period in my relationship with God, and this question weighed at its core. My faith suffered. It felt personal, as if our family had been singled out. Again I wondered why. We had always tried to be good Catholics. So why her?

People offered support, saying ‘God only gave you this burden because He knew you could handle it.’ (They didn’t realize that this seemed more like a punishment for our faith and fortitude, but we saw that they meant well.)

It took a pilgrimage to Lourdes to make my peace with God. I stopped seeing God as a thunderbolt-hurling deity and instead sought mercy.

After that, my blame was more aimless. If it wasn’t God, and it wasn’t us, was it fate? Was it just meant to be? But when bad things happen, fate is an unsatisfying explanation. It seems like another way of saying we have no idea.

Somewhere along the way, my perspective changed again. There wasn’t a moment of epiphany. Perhaps my sense of acceptance broadened. Whatever the cause, I stopped asking ‘Why her?’ Instead I thought ‘Why not her?’

I like to believe this might stem from the humbling we had experienced in our lives. Once I thought others more likely than us to have brought such a situation upon themselves by engaging in dangerous activities while pregnant. But did we deserve it less?

Then I realized that the whole notion of ‘Why her?’ is based on the assumption that what happened to Gabriella is unfortunate. But when I considered ‘Why not her?’ I also recognized that her situation, her life, is not a curse, but a blessing.

And for me, being Gabriella’s dad was an opportunity. I credit our life together with making me a better person, if only because I became more sensitive to the needs of those different from me. With making me a better leader, because she taught me to prioritize and to achieve balance. With making me a better writer, because she gave me rich material and helped me find emotional honesty. And, counter to my expectations, with making me more optimistic. Enough so that sometimes, on my best days, I ask ‘Why her?’ in a whole new way. As in, how did we get so lucky?

It’s now a cliché to say it takes a village to raise a child, but in no case is it truer than with a child born with disabilities. Our village has become multi-faceted over two decades.

In the early years, our community was made up of medical professionals. Gabriella’s birth left us blindsided, but an intuitive nurse helped me through the first few hours while my wife recovered from her Cesarean. Suddenly faced with the unexpected, we relied on her pediatrician and a few specialists as we navigated dehydration and cataracts and clubfoot surgery and a tumor on her skull. Even today, I become nostalgic when I meet one of those early mentors on our unlikely journey.

As our daughter’s health stabilized, our team evolved. Lisa took her each week to Early Intervention, where she worked with a special education teacher and a nurse and three therapists (PT, OT and speech). We found the same model at Lakeview School, where the staff helped Gabriella blossom with dedication and patience. Lakeview also introduced us to a community of parents facing similar challenges, an invaluable resource for the scary times.

When she turned 21, Gabriella aged out of school. In New Jersey, adult programs (it’s still hard sometimes to think of her as an ‘adult’) are county-based. We’re fortunate that Monmouth County provides good alternatives. She takes part in a day program that offers a community feel, and we are thrilled that she has now moved into a room led by a former special ed teacher that provides an atmosphere much like a classroom. Again we’ve met a group of administrators, therapists and families that has widened our circle.

On weekdays, Gabriella’s nurses come early, get her ready, spend the day with her, and then transition her back into the home environment. On weekends, they arrive later and care for her until bedtime. In all cases, they manage her medical needs. Our daughter forms an attachment to many of these women, which makes it more challenging to replace them when their careers and circumstances change. Three weeks ago, we were discussing how pleased we were with her four nurses; then two had changes in their situations. As has often happened, we are again working on identifying and training new nurses, recognizing that some might not pan out. But those that do become a vital part of her community.

Each April I participate on a panel of parents of kids with disabilities for future special education teachers. Over the years, I’ve heard other panelists discuss the state agencies responsible for managing benefits for disabled adults. What struck me was that the rules seemed always to be evolving. Now that Gabriella qualifies, we have experienced the system for ourselves. We work with a service coordinator who has helped us navigate the latest changes. One outcome is that because our daughter attends her program only four days per week, we have funding to put toward other activities. We are excited about an art session we’ve begun exploring.

Most important are our family and friends, most of whom have been with us since the beginning of our odyssey. Their love has provided the support we’ve leaned on in difficult times, and the camaraderie we’ve enjoyed when things have gone well.

All these medical professionals and educators and families and loved ones make up our village, and every village needs a mayor. For us, that has always been Lisa. For all those years I worked long hours, she coordinated all the different constituencies that make up Team Gabriella.

It says a lot about our society that so many have contributed to the care and development of this one special person.

All of us remember where we were on 9/11. Like all of our family’s stories these past 24 years, my version is intertwined with our life with Gabriella.

On the morning of September 11, 2001, I found myself waiting on the tarmac at Newark Airport. My flight to Dulles was about to take off, in advance of my meeting with the SEC. Oblivious to the horror to come, I sat wondering how I would make it through the day after the night we had just experienced.

Our daughter had come home from school the prior day on the bus, but as Lisa pushed her up the driveway something didn’t seem right. By the time they reached the door, Gabriella started screaming.

While non-verbal, our daughter uses a variety of sounds to communicate her needs and wants. None of them involve screaming.

Lisa got her onto the sofa (her bedroom was still upstairs then), but she continued to sob. Every movement of her legs made her anguish worse. When my wife called the school, they said they had noticed nothing out of the ordinary; they let us know that she had spent time on the prone stander. (The stander is a piece of equipment that replicates the act of standing for people unable to support their own weight.) But she hadn’t cried at all. She rarely did except following a pout when her feelings were hurt, and those jags lasted less than a minute.

On September 10, the crying and screaming continued throughout the afternoon.

Lisa called me at work and I rushed home. On the way, I considered my meeting the following day. Four attorneys (two inside and two outside) were to accompany me to the SEC to discuss an innovative new product we were developing. As the lone business person attending, I felt I needed to be there. Just in case though, I arranged with a colleague to take my place in case I couldn’t go.

I arrived home to find Gabriella in obvious pain. She was pale and continued to cry. Unable to calm her, we drove to the emergency room. It was our first trip to the hospital in more than a year, the longest period in the seven years since her birth. We hadn’t missed it.

Our daughter has osteopenia, resulting in very thin bones. In the ER that night, we confirmed Lisa’s suspicion that she had suffered hairline fractures on both legs.

They splinted her legs, and inserted an IV to give her painkillers. After several hours, they discharged her. We arrived home at 2:30 a.m., and we didn’t get Gabriella to bed until after 3. The car service would arrive soon to pick me up. Lisa and I discussed me transitioning the trip to my colleague, but I was the product manager. I slept for forty minutes, then rose and showered.

Exhausted, I landed in Washington, D.C. When I reached the terminal, I called Lisa to check on Gabriella and to let her know we had touched down. (After that day, this became a routine for me, completed most times while still taxiing.) Then I headed to the offices of our outside law firm, Debevoise and Plimpton. In my haze, my thoughts kept returning to my daughter. Two broken legs!

When I got upstairs, I found the others hadn’t yet arrived, so I prepped for my meeting. Then the first plane hit the World Trade Center and the world changed. Someone called me into a conference room and we watched the image over and over. At that point, we all still thought it was an accident.

My colleagues arrived. The second tower was hit. Well before the third hijacked plane struck the Pentagon, we knew the United States was under attack, that 9/11 would become more than a date.

Minutes later, we stood watching the South Tower collapsing on the TV screen in the conference room. My fears for my daughter’s well-being suddenly seemed small, but they remained no less vivid to me.

At home, Lisa was trying to reach me again, but with the cell towers out, phone service was sporadic. The morning passed, surreal. I experienced spikes of adrenaline and grogginess after my endless night. At one point I remember a local news correspondent saying the USA Today building had been bombed. In retrospect, I’m not sure whether the media got that wrong or I was delirious with exhaustion.

The federal buildings had been closed soon after the planes hit the Trade Center, our meeting postponed. Like so many, we struggled to find a way out of the city, with flights grounded and trains canceled, a shortage of rental cars and hotels occupied out to Bethesda. I considered staying with my brother in northern Virginia, but given Gabriella’s condition I was desperate to get home.

Then the Debevoise managing partner in D.C. offered to have his driver take us home. For this act of kindness, I am grateful to this day. This man drove us each to our doorstep.

As we came up an almost deserted New Jersey Turnpike, we saw thick smoke in the direction of Manhattan. That fog seemed symbolic.

Because we live in central New Jersey, our house was the first stop. I rushed from the car to find my family safe. Gabriella was just beginning her recovery, and it would require time and care to nurse her back to health. In the days and weeks to come, we would know confusion and frustration and anxiety. We would seek to understand how such a thing could have happened, and take steps to make sure it never recurred. But after the events of 9/11, the sight of her lying there among her mom and brother and her grandparents brought only relief.

We were among the lucky ones.

Conclusion of a four-part sequence (links to earlier parts below).

The first time Gabriella’s doctor suggested inserting a G-tube, enabling us to insert liquids straight into her stomach through a “button” on her belly, Lisa and I were adamant. No way.

Following her scoliosis surgery, our daughter had lost some of her basic skills, including rolling over and beginning to move an adapted spoon to her own mouth. While accepting food from us didn’t seem like much of a “skill”, we knew other children who had stopped spoon-feeding once they received their nutrition through a G-tube. It was a lot to give up.

Then Gabriella suffered a series of setbacks. Respiratory weaknesses that led to daily nebulizer treatments. The loss of over 10% of her body weight … down from a starting point of 62 pounds. Kidney stones that required shattering into tiny fragments. She had new pediatric specialists. New medicines. New routines.

We were more worried about her ability to thrive than we had been in a decade.

That day in early October 2010, as Lisa made the rounds among the gastroenterologist and the pulmonologist and the nephrologist and the urologist, Gabriella remained sluggish. She was still fighting a respiratory infection. The GI expressed concern that she wasn’t eating. Ultimately, we took her to the emergency room where they inserted an IV, then placed an NG-tube to make sure she got the proper nutrition and hydration.

It was hard to look at the NG-tube, inserted into her nostril and snaking down her throat into her stomach. In a way, it was a more temporary and visible version of the dreaded G-tube. Due to concerns that she would yank it out, she had a mass of tape around her nose. I tried not to think about the pain it would cause her sensitive skin when they pulled it off.

One cause of her kidney stones was insufficient hydration. For years, we had tried to get waterinto her mouth from a syringe, but she hated the sensation and we had difficulty getting down 50 milliliters at a time, a couple times a day. Now we learned that, to prevent future stones, she required a daily fluid intake of 1800 ml!

Gabriella also continued to present risks for reflux, which her doctors feared increased the risk of respiratory problems. While in the hospital, and for two weeks after her release, the NG-tube provided liquified food and water. But we needed a permanent solution.

***                      ***                      ***

The doctors discussed two alternatives, either of which required a feeding tube. One, again, was the G-tube. The other was known as a “G-J”, along with an operation called Nissen fundoplication.

The G-J tube was like the G-tube, except that it continued through the stomach to the small intestine, the jejunum. With the G-J, she would receive a continuous flow of food and water to her digestive system, ending any hope of feeding her ourselves. All I knew about fundoplication was that it meant sewing part of her esophagus to part of her stomach.

As a physician assistant, Lisa understood better than me the alternatives and their repercussions. But even I recognized that neither was good.

One thing I did know. To our surprise, the G-tube had become desirable.

Further testing was necessary to determine which option would be better for Gabriella. This required a pH probe to test her level of reflux and review her internal anatomy.

***                      ***                      ***

Two weeks after her discharge, Gabriella still had an NG-tube taped into her nostril. We were giving her formula “meals” and water boluses (one-time injections from a large syringe). We tried different stomach medicines in a vain attempt to regulate her on the spectrum between diarrhea and constipation.

Days later, we returned to the hospital for the pH probe. The results would be telling: if her reflux was minimal, they would insert a G-tube, but if high she would need the Nissen fundoplication.

Due to Gabriella’s anatomy, two attempts to place the pH probe proved fruitless. Together with the gastro, we decided to go ahead with the G-tube the following morning, choosing the least invasive option.

The outcome was that Gabriella had a plastic valve on her belly. (This device needs to be changed every three months, and when we remove the old one it looks as if she has a second navel below the original.) Through a tube connected to this valve, we administer liquids. This again ushered in new routines, including changes to some we had adopted only weeks before.

But there was a silver lining. Instead of another hurdle, the G-tube became a godsend. Today we use it to give her most of her medicines, either in liquid form or dissolved. Every day, she gets 2000 milliliters of water and formula. From the time they installed it, she gradually gained weight, plateauing three years later at just over 90 pounds. (And while our backs suffer for the increase when we lift her, her health as a result is far better.)

Oh, and she continues to eat from the spoon, just as she always did.

The stormclouds receded. After the urologist dealt with the renal stones, the additional water and medicines prevented new ones from forming. While our daughter still battled respiratory issues, the nebulizer lessened their severity. The dread I had known for most of that year dissipated.

Most of all, because of the G-tube.

Prior posts in this sequence:

Part 1

Part 2

Part 3

We were in the midst of the most frenetic year our family had experienced since Gabriella was three.

This time we didn’t have frequent surgeries, each with weeks of anxiety beforehand, a sleepless night before, the drive to the hospital, a stay in the waiting room, new post-op routines…and then the slow build-up to the next operation on her skull or eyes or foot. Those operations were awful, but they were finite and they targeted well-defined problems. This time was different. This time none of us knew what was going on.

We had often watched the TV series House, where a cantankerous doctor solved medical mysteries that combined unusual symptoms and diseases. With her background in healthcare, Lisa guessed some diagnoses mid-show. To me, the series seemed dramatic and even far-fetched. Yet now we experienced something eerily similar.

After months of our daughter coughing through meals, and after multiple cases of pneumonia, her gastroenterologist had prescribed consults with an allergist and a pulmonologist. The latter intensified her daily respiratory treatments and other routines. The situation worsened, and in early October Gabriella stayed home from school with congestion and poor digestion. The pediatric specialists suggested a combination of factors, but it still stunned us when they discovered she had kidney stones, one of which was too large to pass.

The situation had become urgent.

Two days later, my wife got Gabriella in to see a nephrologist. (This was another specialty I barely understood, although I should have assumed there would be doctors focused on the kidneys.) She reiterated that the large stone caused no blockage, so far, but told Lisa they needed to do a work-up to consider the causes.

It turned out that our daughter was prone to several risk factors. First, she was non-ambulatory, being confined to a wheelchair. Second, she got a limited amount of water each day, however much we were able to squirt down her throat at odd moments through a syringe. Last, one of her two epilepsy medicines was a known cause; that raised a separate concern because it had taken time and tinkering to control her seizures.

The nephrologist prescribed a new pill and told us to weigh each diaper to ensure she was making enough water. The care routines multiplied.

The second doctor visit that day was with a urologist. He determined that the larger stone occupied a spot where it could cause future obstruction. One remedy was surgical, scoping through the incision from her scoliosis surgery and through to the kidney to remove it, but her thin bones and the shape of her hips increased the likelihood of fractures. So he recommended lithotripsy, an hour-long treatment using ultrasound waves to shatter the stone. The resulting fragments could more easily be passed.

I grew nervous at the idea of shocking our daughter’s kidneys with ultrasound waves strong enough to destroy a large kidney stone. Would it cause other damage? But compared with the surgery, it seemed less invasive and less risky.

The following week, we went forward. The urologist felt the procedure went well, pulverizing the larger stone, although we would wait for the sand-like pieces to know for sure.

Now we searched each diaper (already heavier due to the water pills) for bits of shattered renal stone. A few days passed, but with no sign. Lisa confirmed that they didn’t always pass right away. But a week went by, then another. Nothing.

Back to the urologist. He said the lack of “sand” just meant the fragments remained in her kidneys, but they were smaller and unlikely to form a blockage. But then, upon examining the x-rays, he found new stones. He proposed giving it some time, then considering another lithotripsy, since she had tolerated the first one well.

A few months later, when the scan showed no change, he shattered the remaining kidney stones. Again Gabriella came through swimmingly. This time we found fragments in her diapers.

With additional medicine and better hydration, we now believed we could manage her renal issues. This was because, by now, things had come to a head on the biggest decision we had faced in many years, the feeding tube.

Next week: the Conclusion