To Tube or Not to Tube

 

 

Our son Neil turned six on January 28.

He’s just over three feet tall and weighs just over 31 pounds. Even considering the small stature of his birth parents, he’s still a small (but wonderful) person.

We shy away from the “percentile” conversations with other parents. Neil is on the growth charts (thanks to the Centers for Disease Control), but barely. He’s below the lowest curve, chugging along at what appears to be less than the first percentile for both height and weight.

Because he sees so many doctors at A.I. DuPont Hospital for Children, we get plenty of data to obsess about.

Neil gains weight incrementally – a few ounces here, a few there. But when he gets sick, and his appetite declines, the weight seems to fall away quickly. We nearly had a party when he topped 32 pounds a few weeks after his birthday.

Good thing we didn’t spend money on streamers; thanks to a recent cold, he was back down to 31 pounds.

Every ounce he gains is a monumental victory – winning American Idol and hitting a game-winning home run rolled into one. The struggle for these tiny gains keeps our own stomachs churning, as we test-drive the latest calorie-enhancing tactic designed to outsmart the poor muscle tone (hypotonia) in his mouth and a severe but fairly well-managed case of gastrointestinal reflux (GERD).

Tops on our list these days is adding a tablespoon or more of melted butter to his food.

The Dentist’s Chair

Feeding him is as tense as a lengthy stint in the dentist’s chair - and that’s for us. We’ve come up with an array of distractions that qualify us for Ringling Brothers clown school. We dance, we sing, strum the guitar – let him strum the guitar – shine the flashlight in patterns on the wall, open and close the refrigrator, just to get the calories into him.

We’re lucky if he takes in 1,500 calories in a day. As a kid with CF, he needs more. Eventually, he’ll need to take in more than 3,000 calories per day to sustain himself.

When Neil was about 3, his CF doctor, Raj Padman, a strong advocate of proper nutrition for CF patients, suggested that we think about subjecting Neil to surgery to install a feeding tube (the Department of Surgery at the University of Michigan offers a detailed explanation of the procedure).

At the time, Neil was spending most of his time on his back and belly. He’s a pretty energetic little person, as this video shows.

We cringed at the possibility that he might tear the tube out of his side.

The Debate Begins

And so the debate began – it would go on for nearly four years. On one side, there was Dr. Padman and Neil’s endocrinologist, Dr. Daniel Doyle, who told us that until Neil was bigger, a meaningful test to determine if Neil needed growth hormone shots could not be performed.

On the other side was Dr. Stephen Shaffer, then Neil’s G-I doctor. Neil’s body-mass index (BMI) was pretty good, he said, and Neil looked sturdy, so we should wait.

On the same side, but showing a more centrist tendency was Neil’s excellent pediatrician, Dr. Anna Kolano. She too wanted to wait, but gently nudged us toward treating the tube as inevitable.

In March, we met Neil’s new G-I doctor, Dr. Jose DelRosario. A gastric emptying test done in the weeks after the meeting revealed that Neil’s stomach empties very slowly, more slowly, in fact, than it had during a previous test. “Normal” emptying occurs when two-thirds of stomach contents leave the stomach in a predetermined period.

A little over a quarter of Neil’s stomach contents emptied during the test.

Dr. DelRosario explained that Neil’s mild case of cerebral palsy could be the cause of the delayed emptying. The valve that sits at the top of his stomach simply isn’t working as well as it should, he said.

We were given several options during a follow-up meeting with Dr. DelRosario – do nothing and continue the feeding struggle, which would only make Neil less inclined to eat other foods; insert the feeding tube; and a surgical procedure called fundoplication combined with insertion of the feeding tube.

Maybe I was exhausted; maybe I was just tired of cleaning up Neil’s vomit, usually laced with thick phlegm. Whatever the reason, I practically leapt out of my chair to endorse the surgery, during which the upper part of the stomach is wrapped around the esophagus and stitched into place, thereby stopping the GERD he’s suffered from since he was an infant. The size of the stomach decreases, accelerating the emptying of its contents.  

Neil would likely never vomit again, the doctor told us. That’s all I had to hear. Sheila came around gradually, but eventually agreed the benefits of the twin procedures – more foods in his repertoire, reduced GERD, fewer food fights – and yes, no vomiting – outweighed, for the most part, the minimal risk.

Just putting in the feeding tube would not speed up the emptying. Trying other stomach emptying medicines similar to Reglan, the medicine he takes now, wouldn’t either. Installation of another type of tube, a G-J tube, would require that Neil not try to dislodge it for 16 hours while food was being sent into his small intestine.

Not much chance of that.

So in August, the fundoplication will take place. His GERD will end, for five years anyway, and he won’t vomit. He’ll keep food – and calories – in.

 

 

Making it Through the IEP

Sheila and I visited Neil’s school in mid-January for an Individualized Education Plan (IEP) meeting with his teachers and therapists. Under the Individuals with Disabilities Education Act (IDEA), an IEP must be drawn up for every special needs child who attends public school.

IEP’s are updated each year. Every change to a student’s goals is dutifully recorded, and an updated plan supplied to the parents. Sheila and I have joked that parents end up with quite a collection of folders featuring the school district’s logo.

The IDEA, amended by Congress in 2004, requires that children with special needs “have a free appropriate public education the emphasizes special education and related services designed to meet their unique needs and prepare them for further education, employment, and independent living.”

The law also established an “early intervention system” to make services available to families with special needs children. Funding was also earmarked for training and recruiting qualified teachers, and for conducting meaningful research on the progress of special needs students.

Making Progress

The meeting gives us a chance to talk at length with Neil’s teachers, to hear about how cute he is, to hear the assessments of his progress. Last year at this time, crawling was difficult for Neil. Now, he jets around the house, especially when he sees something he wants, or if the front door is open.

And with the help of his walker, he gets around quickly, and maneuvers pretty easily around large pieces of furniture.

Last year, his menu was limited – stage 3 baby food and PediaSure, for the most part. Today, he partakes primarily of selections from the school’s regular lunch menu. Sometimes it’s an adventure (particularly if his GERD is acting up), but he tolerates (“likes” is still not quite accurate) more types of food. 

Neil has mastered signs for “want” (patting his chest) and “more” (clapping his hands). He’s almost got “go” (tapping his thighs), but is a whiz at “all done” (think of an umpire’s `safe’ sign). Last year, he communicated only by babbling and raising his voice.

Then why do we dread the IEP so?

More Challenges

For starters, there’s so much further to go, so many more challenges for Neil to face. Our hope is that he becomes self-sufficient – able to take care of himself and, eventually, live on his own.

Listening to the teachers and therapists as they painstakingly discussed last year’s goals for Neil, noted his progress toward meeting them, and then laid out this year’s goals – some of which were scaled back because he hadn’t made enough progress - we felt as though we hadn’t done enough.

We felt like bad parents – bad parents who had been sent to the principal’s office.

I thought of the times that I graded papers, or read a newspaper, or even went to the bathroom when I could have been interacting with him. But there I sat, making notes, promising Neil and Sheila I would do better, when I knew that life simply intervenes, making this kind of full engagement impossible.

We came up with at least one defense mechanism: Sheila and I would periodically examples of Neil’s behavior that made us laugh, or that we thought were evidence of at least modest progress.

The teachers and therapists, in full skilled professional mode, verbally patted us on the head – the equivalent of saying “isn’t that nice”- before moving on to the next goal.

At times, the tone of the meeting was a stark contrast to the happier, more playful interaction between teacher, therapist, us, and Neil during the yearly home visit set up by the school district.

It’s probably just a matter of being on “our turf.” They observe Neil here, as he cavorts around the house, either on his scooter, hands and knees, or in his walker. A lot less tension.

We came away from the IEP with a list of instructions for the coming year: encourage him to put together large Legos, get him to sit with his knees directly under his rear end, rather than splayed in a “W,” try a “wheelbarrow walk,” where we lift his legs up behind him and cajole him into walking on his hands, and add some new signs to his repetoire.

Sliding a hand up and down an outstretched arm means “music.” A cupped hand placed in front of the mouth means “drink?”

The dread subsides only when the meeting ends. It is replaced by hope, by a strong willingness to try all of the things the team has suggested.

We only hope we can get to at least some of them in the coming year.

Making Sure Neil Gets His Medicine

I’ve created a very basic podcast detailing our journey to refill Mister Neil’s many prescriptions. I did this by creating a podcast on odeo.com, recording the audio, and then placing the audio in the podcast. Click on the bar below to play the podcast, and be sure to let me know what you think:

Taking on George Will

Please take a moment to read this column by Newsweek’s George Will about how parents deal with the news that their unborn baby has Down’s syndrome, and my response, sent to Mr. Will on January 29:

Dear Mr. Will:

There’s a spot in our local supermarket, at the end of the aisle where they sell small appliances on one side and cake mixes and frosting on the other, where I learned in January 2002 that our son, Neil, had cystic fibrosis. A genetic screening test, required for newborns in Pennsylvania, revealed that both of his birth parents were CF carriers, and that he indeed had the defective gene.

My wife struggled with disbelief and fought back a flood of tears as she told me about the test results, which she had received minutes earlier from the wonderful social worker who had shepherded us through the adoption process.

Then came the question – the question that neither one of us wanted to ask, but had to answer: would we give him back? Our social worker told my wife that she and the agency would understand completely if we decided to cast Neil to the bitter winds of the foster care system. During the preparations for his birth we had made clear our willingness to adopt a child with any of a number of health-related or family-related challenges. But still, the social worker said, they would understand. We could change our minds.

In a second, I tried to even barely comprehend what Neil would go through, how his life might unfold, armed at the time with only a cursory knowledge of CF. My wife was stunned, even angry, that the social worker would even ask us about backing out on our promise. We talked only for a moment or two, shared our doubts and fears, and even sketched out the framework of a plan to make it through the next few months.

Then the answer came: not a chance. He was our son, and we would deal with the challenges together. We still cannot imagine making a different choice, but continue to acknowledge that ours might not be the right path for every family. We continued to love and push forward even after learning that Neil suffers from a form of cerebral palsy, and also is thwarted in his efforts to gain weight by a severe case of gastro-intestinal reflux disease.

Which brings me to your January 29 Newsweek column. Your unfeeling assessment of the motivations behind the decisions made by some families to abort Down’s syndrome babies reads like the well-worn passage from the GOP’s election playbook that asserts that any woman who chooses to have an abortion must have made that decision lightly. You know the one: silly, flightly women – they give the same amount of thought to aborting a baby as they do to picking a new hairstyle, keeping tabs on Paris Hilton, or where to go to lunch with their friends so they can mimic the characters on that horribly raunchy show Sex and the City. They should find their way back to the kitchen, so that we can keep an eye on them while we men reestablish the sanctity of the American family.

Thanks to some skilled spinning from the Christian right (which has busily circulated your thoughts on the subject at hand), and some cowardice and calculated side-stepping on the part of Democrats, this line of reasoning has pushed itself on to the national agenda.

But it is nonsense.

The American College of Obstetricians and Gynecologists promotes screening only as a means for families to reach an informed decision about the direction their lives will take once a child with special needs enters their hearts and home; the group’s policy is not a how-to manual for mounting a “search and destroy mission,” as you assert. Wouldn’t it be better if families carefully considered these decisions, rather than try to build a family while possibly dealing with the anger and resentment produced by their lack of understanding?

Shouldn’t we applaud, or at least respect, a couple who takes the time to decide that they might not be ready to embark on such a daunting journey? The clear “God will provide” subtext in your column is outdated and irrelevant to these experiences. Love is powerful, and can overcome a great deal, but only if it’s partnered with information and common sense.

Steps taken by our elected leaders to improve the communication process between families and physicians are encouraging. Senators Kennedy and Brownback in 2005 co-sponsored the Prenatally Diagnosed Condition Awareness Act, which, if passed and signed into law, would “increase the provision of scientifically sound information” to parents. One of the catalysts for the bill was a study done by a Harvard Medical School student who found that physicians at Children’s Hospital in Boston often offered inadequate information to families facing a Down’s syndrome diagnosis, and in a few cases, pressured them to abort their pregnancies.

The bill calls for the National Institutes of Health and the Centers for Disease Control to establish an array of resources for parents trying to compile information on Down’s syndrome, including a telephone hotline, support groups, and a national registry of families willing to adopt a child with Down’s syndrome.

All necessary changes, to be sure. But the tone of your column suggests that even with all of this information, women would blithely choose to abort. It’s a long way, rhetorically, from reining in an imperious physician to suggesting that women almost automatically participate in the “casual destruction of pre-born babies.”

We’re lucky: Neil’s first pediatrician, whom we consulted on the advice of a friend, knew he was in over his head, having not treated a CF kid in 30 years, before the recent advances in treatment, and corresponding steady increase in life expectancy. He suggested we start a relationship with the CF Clinic at the A.I. DuPont Hospital for Children. The doctors there have treated Neil with expertise and great care.

Neil turned five this weekend. His CF symptoms are mild; his lungs have yet to be compromised by his ongoing struggle against the thick mucus that his body can’t properly expel. But thanks to the combined efforts of his remarkable mother, his doctors, therapists, teachers, his favorite babysitter Lindsey, and the rest of our family, he is doing remarkably well.

As someone once said, “It takes a village to raise a child.”

Thanks for reading my letter. My wife and I are thrilled to hear that your son is doing so well.

Sincerely,

Ron Bishop

CF on The View

This is my first shot at inserting a video clip in my blog, so bear with me.

In mid-December, the folks on The View, when they weren’t trying to show the world what a jerk Donald Trump is, stunned (in a good way) a little girl with CF when they brought the cast of the Broadway show Rent to the studio to sing for her. Check it out:

Fear of Fever

We kept Neil home from school today because he was running a slight fever (about 99 degrees), and was battling some serious nasal congestion.

In most cases, 99 degrees is not cause for alarm. But a fever for Neil triggers all kinds of alarms in our house.

Sheila and I tell ourselves that a fever is a good thing; it tells us the body is fighting, trying to heal. But for the parents of a CF kid, even a minor fever triggers fears that the increased body temperature is a signal of an oncoming infection.

For Neil, this means a round of antibiotics, a day or two out of school, and a quick recovery – so far. We are hoping desperately to hold off the day when the fever means that his lungs have been compromised by serious infection.

New and improved medicines mean that these infections can usually be stifled, but never completely knocked out. They usually mean that the CF patient can expect to embark on a sometimes lifelong cycle of hospital visits and more aggressive treatments.

Average Life Span on the Increase

If Neil had been born in the 1960’s, he would have probably lived only five years – that was then the average lifespan of CF patients. So instead of getting ready to celebrate his fifth birthday this Sunday at home with a party, we quite possibly would have been celebrating in a hospital, or trying desperately to brace ourselves for living without him.

Not that these thoughts ever completely leave your mind. Today, though, he’s doing well. His last CF Clinic visit at the A. I. DuPont Hospital for Children revealed that while he’s still struggling to gain weight (he’s nearly five, and weighs a little less than 30 pounds), his lungs are clear, and a throat swab detected no sign of bacteria in his lungs.

The inability to gain weight is a pretty typical struggle for CF patients. Following consultation with his nutrionist, we’ve been adding melted butter, cream cheese, and/or Carnation Instant Breakfast to his food in order to ramp up the calorie count. As Neil gets older, we’ll work hard to ensure that he gets the more than 3,000 calories he’ll need each day to help maintain his health. 

The cruel irony is that Neil also suffers from gastro-intestinal reflux disease (GERD) and has only recently developed sufficient muscle tone in his mouth to enable him to tolerate even slightly chewy foods. So it’s been baby and toddler food for him from the time he was two months old. We joke about how tired he must be of applesauce.

But like so many families who deal with CF, we’re thrilled when we read that median age for kids born today with CF has increased from 32 years in 2000, to 36.5 years in 2005. Forty percent of CF patients these days are 40 or older. All great news, courtesy of the Cystic Fibrosis Foundation.

But that still means that we will outlive him – a fact that we file in our short-term memory and only deal with from time to time when we’re feeling strong enough.

Next: I’ll take you through a typical day for Neil – his many medicines and treatments, but also his curious love of ceiling fans, swinging doors, and the song “Bingo.”

Welcome!

Welcome to my blog – I thought it only fair that since I’m asking all of you to create blogs, I’d create one, too.

After our discussion last week, I decided to narrow my focus a bit, from issues that touch the lives of special needs kids, to issues faced by families of kids with cystic fibrosis (CF).

The rest of the content is about the same – I’ll write about everything from insurance snafus to dealing with doctors, to linking with other families, to thoughts and emotions that families like mine encounter on their journey.

I’ll also try to include enough expert guidance to make this a valuable source of information for those who have special needs kids, and those who simply want to learn more about these issues.