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.

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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.

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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…

The feeling lurks within like a sleeping beast. It’s not so much a spike of fear as a lingering dread, ready to be roused at any moment. By a phone call, a blurring over the eyes, a bump on the scalp. Or a retching cough.

We had settled into a routine. Those first few years – the dehydration, the cataract and clubfoot surgeries, the cancer scare – had indoctrinated us into a life of constant anxiety. A decade later, our daughter’s spinal fusion surgery had exploded any sense of normalcy. Now came the reign of tedium.

Lisa bore most of the burden, the hurry-up-and-wait activities of doctor visits and lab tests and billing questions. I worked. Gabriella went with delight to Lakeview School, and we tried not to dwell on the pace of her development. Or on the fact that her IEPs (the educational plans documenting her goals and her progress), hardly changed from year to year. Her health was stable, our primary concern.

But there were worrying signs.

For one, in September 2005 she weighed 57 pounds. Her height was already in the fifth percentile for eleven-year-olds, and her scoliosis surgery that month effectively stunted any further growth. The awful complications following that procedure weakened her and over the ensuing year, she dropped ten more pounds. The low point for me was looking at a photograph taken on the beach with her brother and cousins, finding that her arms and legs looked skeletal. It took another twenty-four months for her to regain what she had lost since the operation. After that, she plateaued between 55 and 60 for two more years.

One reason was that Gabriella remained a picky eater. I had reasonable luck in feeding her on the weekends, but it was still a struggle to get down a couple jars of baby food and a dish of applesauce. Before the scoliosis procedure she had begun using a customized curved spoon, laboring to control it to her mouth and needing lots of help, but finding pleasure in the act itself. She also toyed with drinking thickened juice from a cup, lifting the two-handled vessel to sip; this was important because she drank little water except when squirted between her lips with a syringe. But these burgeoning skills were among the things she lost when they straightened her back, never to return. We resumed holding the spoon ourselves.

She ate pureed food, and Lisa put meat or vegetables in the blender to vary her flavors, but sometimes she would choke, unable to process anything but the bland mush in the Gerber and Beechnut jars. Too much coughing interrupted a meal and often signaled its end. We heard similar frustrations from her teachers and speech therapists at school.

The coughs weren’t limited to mealtime. Our daughter became prone to respiratory infections, often succumbing around her birthday in late November. More than once, we spent Thanksgiving at home, trying to get her fever down, to ease her discomfort, to keep her from gagging herself into vomiting. She contracted bronchitis a few times, and pneumonia several more, always in her undersized right lung.

The confluence of these symptoms – her flatlined weight, her limited water intake, her frequent coughing and her respiratory setbacks – worried Lisa. It was a busy time at my job, and she took our daughter to nearly all of her pediatric specialist appointments by herself. Perhaps because I tried to stay optimistic, I rejected the idea of more comprehensive problems. But even within me, the dread began to stir.

What I never suspected was that this would soon become the second most stressful and medically-involved period of our daughter’s young life.

To be continued…