Travel with Gabriella has always had its limitations, but we’ve gotten more adventurous over the past few years. Last week we took perhaps our boldest step yet, a seven-night cruise.

It’s been three years now since our inaugural cruise, two nights up the Atlantic seaboard and back. That trip was simple, with no ports to navigate and just enough time on-board to reassure ourselves that our daughter could handle the motion.

This was a bolder step. Rather than departing from nearby Bayonne, the Norwegian Escape left from Midtown Manhattan. The ship stopped in four ports, only one of which used “tenders” that prevented Gabriella from disembarking. And the longer duration meant we had to rethink her care. Her huge pink duffel bag overflowed with her supplies.

Also, we learned from our prior experience, when bending over the low sofa-bed resulted in backaches, and we arranged for a hospital bed in our cabin. This offered not only the ability to raise and lower, but to elevate our daughter’s head at night to reduce the risk of gastric reflux.

One thing I looked forward to most was getting Gabriella in the pool. This remains a favorite activity, but we now feel much more secure using an accessible lift to transfer her from her wheelchair into the water. The ship provided such a mechanism, so on our first day out of port, she and I put on our bathing suits. It was chilly, but we were able to use a heated spot in an indoor area. She loved it, and so did I. For a while.

Unfortunately, that pool was less than four feet deep, which put a strain on my back. Further, the accessibility crew that operated the lift took longer to return than expected. By the time we got out, I was glad I had scheduled my massage for that afternoon.

Another thing that made us eager for this trip was the opportunity to set sail with our travel agent, Debbie from Cruise Planners. It was her presentation several years ago on travel for people with disabilities that prompted our first cruise, and when she offered us the chance to join a couple dozen others on this ship, we were thrilled. We dined with the group, and Gabriella welcomed our new friends.

Unlike our earlier “cruise to nowhere”, this itinerary included four ports, the first two in Maine, the latter two in eastern Canada.

Portland was a charming city and the day was unseasonably warm. Lisa, Gabriella and I meandered about in the morning, setting a pattern that would recur in most of the ports when we found excellent book stores (for me) and yarn shops (for Lisa). We returned to the ship to change and feed our daughter, anticipating the afternoon excursion we would take in town with Debbie’s group. Sadly, one bus had a problem with its lift and the tour company had to cancel the outing. We were disappointed to miss the chance to see the local lighthouses, but this outcome is not unusual even for experienced travelers with disabilities.

Bar Harbor was our tender port. This meant the ship was unable to dock close enough to set up a ramp for easy access on and off. Instead, the cruise line used small boats to ferry passengers to shore, and those in wheelchairs had to stay on the Escape. Lisa and I had prepared for this, and I spent the morning on an excursion to Acadia National Park, while she did the same in the afternoon. It was a beautiful location, and we enjoyed the vibrant foliage, even if we regretted having to visit individually and without our daughter.

Between Maine and Canada, the weather changed. Both St. John and Halifax were cold and rainy, but that didn’t stop us from pulling on our ponchos and grabbing umbrellas and venturing out to enjoy both cities. As is often the case, Gabriella most loved being in motion, enjoying the vibrations on the cobblestones in St. John and the steep climbs in Halifax.

On this, our second cruise, we learned several lessons. The hospital bed made a huge difference, and our backs felt better after seven nights on the Escape than they had after two on the prior ship. We also worked with the crew to get one meal pureed for her each day, allowing her to share the tasty food we enjoyed.

Gabriella showed a strong interest in several of the evening musical performances, including a couple piano shows in bars, a first for her. While the music was loud, her noise-reducing headphones enabled her to sit and watch with a huge smile for over an hour each night.

We also benefited from watching Debbie’s group in action. My instinct was to hang back in the doorway, but in entering a bar behind them, we followed into the middle of the room.

As always, I discovered a need for patience, of which I am often in short supply. More than once, we wolfed down our meals to rush out of a quiet dining room while Gabriella complained loudly. While we experienced kindness among our fellow travelers, we remained self-conscious when she expressed her discontent. As a result, we had food delivered to our cabin several times to avoid eating in public.

Our adventure drove one other conclusion: for our next cruise, we will consider bringing along a nurse. Even in a vacation environment, the 24×7 demands of care can become overwhelming. While this would still offer the joys of cruising with our daughter, it would also allow us to share some experiences as a couple.

In the end, despite the challenges, we all enjoyed our latest Escape.

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

All summer, Gabriella had been coughing. She was often congested. And her respiratory complications raised alarms about aspiration.

It was the week before Labor Day, eight years ago. More than a decade had passed since those tumultuous early days of dehydration and surgeries and the malignant frozen section. Much of that time was calm, even if the monotony became disheartening. But be careful what you wish for…

My wife made the rounds among our daughter’s specialists, adding an allergist and a pulmonologist. We became concerned about asthma, which ran in my family. After a trip to the ER in March, we were giving her respiratory treatments at home with a nebulizer. This involved pulling a clear plastic mask over her mouth and nose, squeezing liquid Albuterol from narrow tubes into the apparatus, dispensing it in gas form.

These treatments became a daily ritual, which largely fell to Lisa. I had a long workday, and she was the medical professional. Plus, the new routine intimidated me. What did I know about dispensing medicine?

Only later did I realize how stressful it was for her. Years of education and training and experience as a practitioner don’t prepare a mother to treat her own child.

All of this made me feel a bit useless. At the beginning, it had all been so enormous. We had gone from having our first baby to discovering how unique were our challenges, from a crushing diagnosis to ten surgical procedures. I was there for all the hospitalizations and the meetings with the geneticist, and I attended appointments with the orthopedist and the brain surgeon. When the bank I worked for got sold in 1996 and I was laid off, I took the opportunity to spend six weeks caring for Gabriella while Lisa was having Alexander.

By 2010, life was different. I headed to the train station each morning at 5:30 and arrived home around 8 p.m. Most of the developments came during visits with the pediatric specialists. I was left with a dislocated anxiety.

The physicians involved were different, too, focused on digestion and breathing as opposed to eyes and bones. There was something more subtle about these complications, something more mysterious.

On the Tuesday before Labor Day, Lisa went for a consult with the new pulmonologist. She shared the x-ray of Gabriella’s lungs, which showed markings consistent with chronic inflammations, similar to asthma. The congestion and coughing increased her chance of aspirating, she confirmed. And she added a second medicine, Pulmicort, to the daily respiratory treatments, while prescribing chest PT to loosen the accumulating mucus. She even recommended a device that would administer this therapy by itself: the Vest.

Chest physical therapy consists of rapid hits with a cupped hand on the patient’s back and upper torso. During her hospitalization the previous March, Gabriella discovered how much she hated chest PT. Some combination of discomfort and insult over those repeated light body blows caused her to pout and cry. Even more, she had hated her experience with the Vest. Neither Lisa nor I relished inflicting such misery on our child, but as usual my wife got stuck with most of the burden. But we opted against reprising the Vest.

In the meantime, we explored whether her respiratory issues had a GI component. A few days after seeing the pulmonologist, Lisa took her to the gastroenterologist. She expressed hope that the lung treatments would prevent further infections and pneumonia, but warned that if they didn’t, she would seek a pH probe to test for reflux, one possible cause of aspiration.

Not long after, Gabriella missed school with a wet cough and a low-grade fever. The pulmonologist ordered more x-rays. We increased the frequency of her nebulizer treatments and her chest therapy. Later that week, she remained very congested with a thick sputum. On the third day, Lisa made the rounds of the specialists.

That evening, she briefed me on a most ominous morning. Gabriella looked pale and she weighed 55 pounds, down 7 in two months, unsurprising since she hardly ate. The GI was concerned. She prescribed an immediate suppository, with another that night. Within forty-eight hours, if our daughter hadn’t resumed eating, she would have to be admitted to the hospital and fed through an NG-tube. The gastro also raised the possibility, that she might need a permanent G-tube. We had discussed this previously, hoping to avoid it if at all possible.

The lung doctor added something else. While the x-ray of her chest was clearer than expected, the view of her abdomen showed an unexpected development. Gabriella had kidney stones.

Suddenly, all was spinning. I had a difficult time processing all of this information. It was likely at least some of these symptoms interacted with one another, but how was unclear. I had no ability to distinguish among different types of feeding tubes, but all of them brought a foreboding. And kidney stones? I didn’t know a lot about them, but I knew they were painful.

It pained us that Gabriella couldn’t tell us herself where she hurt. And we prepared ourselves for a new period of the unknown.

To be continued…