Cystic Fibrosis Pediatric Patient Stories

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Michelle's story

Man, oh man, oh man, where to begin?

Isn’t that what some of you reading this would ask yourself if invited by Boston Children’s Hospital to “share your story,” the story of being a parent to your amazing child?  Your amazing child because he or she is yours and had to be hurting at some level to even be in a hospital in the first place, right? 

That’s what I asked myself when I first visited with the folks here about my Michelle and our experiences together as we’ve charted the waters of her myriad medical complications. 

Michelle is 12 years old and beautiful.  Sweet as can be.  Not a mean bone in her body. 

Though unable to speak, her eyes dance and her face lights up a room.  Occasionally she grimaces or whimpers, but oh-so-rarely, and only for very good cause. After all this time, I can figure out just about everything she “says.”  She is also unable to walk, her delays—and the mild seizures she suffers nearly every day—were caused by a rare, genetic form of intellectual disability known as Lissencephaly, diagnosed just after she turned one.

But it was the very day after Michelle was born that life as I knew it changed forever (which happens after giving birth to even a healthy child, no?).  With a distended abdomen and an unwillingness to eat, she was flown from our small rural town to Children’s.  I’d had a C-section, so she had to go alone.  Torture.

Three days later, still apart from my teeny precious love, I learned of her diagnosis of cystic fibrosis, according to the Web, “A life-threatening disease…” Life-threatening.  Stop right there.  That thought was paramount for days.  Days?  Try months.  Life-threatening.

Other diagnoses were to follow.  One of them:  hip dysplasia.  Surgery scheduled in 2006 to correct it was overruled when uncontrolled seizures took precedence and the risks of so difficult an operation outweighed its benefits.

But the brilliant Dr. Brian Snyder, who has followed Michelle for many years, predicted—astutely—that eventually he’d have to go in and fix what was amiss.  When Michelle’s left hip became dislocated last December, that time had come.

In the intervening years, Michelle was diagnosed with CF-related diabetes.  At the time, I thought, “Oh, God, please, not something else.  I just won’t be able to handle it.”  But when I reflect on that time, I recall the wise words of my faith-filled mom:  “The human mind can get used to just about anything in 10 minutes.”  My initial reaction to her CF diagnosis notwithstanding, blood sugar checks and insulin shots simply became just another part of the very-busy-but-really-not-that-complicated routine.

And once you overcome that hurdle of learning a new medical regimen (and, of course, the gut-wrenching feeling of what does this mean for my child now?), what’s left is the adoration and purity of a living, breathing angel who is your very own daughter.  Unbelievable.

Anyway, I didn’t know but should have known—the most significant dilemma of the “hospital” parent—that this latest CFRD diagnosis would impact Michelle’s recovery of the surgery that was to come.

As I sat in the comfortable suite of OR waiting rooms that March afternoon, I never dreamed it would be 1 a.m. before I would finally see Michelle again, in the Intensive Care Unit (ICU).  Had she not been a patient there before, I honestly believe that at the sight of her I would have crumbled to the floor with that mix of despair and exhaustion many parents know all too well.  (But, somehow, we remain standing.)  There she was, breathing tube down her throat, puffed up from excessive fluid retention, both legs in a giant double cast, and completely and totally in another world.  Though her nurse looked all of 19, it was clear within minutes she knew her stuff.  She administered meds, checked “PICC” lines, monitored monitors, consulted pain management specialists, doctors, and fellow nurses, chatted comfortably and reassuringly with me, and simply “did her job” (oh, what a job) with an efficiency and alacrity that would astound Donald Trump. 

I crashed.  In clean sheets on the bed for me in Michelle’s room. 

Later that morning, my number one priority was coffee.  Well, that’s the case on the best of days, so you can only imagine.

I returned to the ICU to doctors of all stripes (students, residents, fellows, attendings—you get the hierarchy down after awhile), pow-wowing outside Michelle’s door. 

Anesthesiology.  A tricky practice indeed.  The physician conducting this particular train (yet another OK-you-are-so-astonishingly-brilliant-but-very-kind human being typical of Children’s Hospital) had run into trouble with this critical component of Michelle’s procedure:  the epidural intended to manage Michelle’s pain locally didn’t work.  After three attempts.  So that meant heavy-duty narcotics and continued intubation. 

The huddled doctors called me over to inform me that when drawing from the failed epidural, the nurse discovered spinal fluid, meaning a 2-3% chance of spinal chord damage.  A small percentage, yes, but not to me.  Could my love have had double hip surgery only to end up paralyzed from the waist down??

Knowing it would be weeks before a neurological exam could reveal any response to touch, Dr. Snyder ordered an MRI for that day (thank you, God).  It took a full hour and a half to ready Michelle to simply leave her room, so connected was she to a vast array of monitors, IVs, and oxygen, all of which had to STAY connected while she underwent the procedure in the narrow MRI chamber.  Hours later, great news:  no compression of the spinal chord.  No leaked blood or other fluids. 

The next day I had to go home, to return to work and Michelle’s eight-year-old sister, Simone.  Now, nothing is more important for a parent than helping “quarterback” the flow of communication among multiple medical professionals, let alone after a complicated procedure with so many different services at play (ortho, pulmonary, endocrine, neuro) working toward a difficult recovery.  But I knew that in the ICU, Michelle was too sedated to have any awareness of my absence and that she was in the best of hands; after a certain point, I was a bit in the way. 

So home I went. 

Then back again.  Back and forth.  A lot.  With frequent calls to Michelle’s nurses in between. 

After nine days, she’d finally been “extubated” and soon thereafter transferred to 9 South, where she’s been for most of her stays at Children’s. How shall I describe the environment there?  Let’s just say that once, when telling my sister about it, she said, “Boy, I bet you’re hoping Michelle ends up in the hospital again soon.”  Well, not quite.  But when you can count among the nurses, CAs, receptionists, and custodians a multitude of lifelong friends, you know it’s pretty special indeed.

And the extra comfort it gave me to have Michelle in the care of 9 South’s extraordinarily skilled and unbelievably kind nurses made all those trips back home a little more bearable.

As Michelle’s parent, I’ve frequently found myself torn between letting the experts do their thing and being, frankly, a pain in the ass.  The trick is to do the latter while maintaining mutually respectful relationships.  I’ve learned that at the risk of losing it a bit from time to time, I am Michelle’s foremost advocate, her mother lion, and if noses get bent out of joint in the process of assuring her maximum well-being and comfort, I nonetheless do what I must do. 

Fortunately the people who know me best understand that Michelle is my primary focus, and that if it means letting off a bit of steam, insisting on swift, clear answers to countless questions, or answers to questions I’ve not yet even formulated, well, that’s cool with them.  (It’s even okay to cry; there is always a shoulder available if I need it.)  I think the nurses especially would far prefer to see a parent passionately engaged than dutifully polite, too intimidated to challenge, or (the saddest of all), remote and uninvolved. 

It’s been a long road, and she’s a ways to go with the healing of that nasty wound.  I could question endlessly:  How could this happen?  Should the cast have been removed sooner?  Could I have more aggressively intervened?  But everyone did more than their best, and my little girl and I shall soldier on. 

It was Michelle’s most recent admission, but it won’t be her last.  It’s been another learning experience for Mom, and a further strengthening of the respect, gratitude, and sincere fondness I feel toward the people that make up the best pediatric hospital in the country – the best, to me, in the whole wide world.

How immeasurably blessed we are, despite the challenges we face, that these amazing, gifted, dedicated men and women play such an important role in our lives.

Who would ever have known?  Who would have known that this story would be . . . our story.

- Ann Davis

We are grateful to have been ranked #1 on U.S. News & World Report's list of the best children's hospitals in the nation for the third year in a row, an honor we could not have achieved without the patients and families who inspire us to do our very best for them. Thanks to you, Boston Children's is a place where we can write the greatest children's stories ever told.”
- Sandra L. Fenwick, President and CEO

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