My excuse for taking an extended break from the blog is we were preoccupied the whole summer with traveling in between regular work and play and preparations for the girls’ upcoming school transitions. On two big car journeys (out West and up North, respectively) we covered thousands of miles to traverse 28 states in the course of visiting 5 national parks, a number of scenic state parks, and our favorite coastline. While cf accompanied us as usual along every leg and stop of the way, I’m grateful to say it stayed quiet enough in the backseat for us to enjoy plenty of unfettered driving, exploring, trekking, sightseeing, camping, packing, and family time. Yep, our summer was bright and busy and fun. It was a bucket checklist kinda summer.
Now as we welcome fall routine, Lou is settled comfortably, growing and thriving in first grade. Av has found her tribe at an exceptional public magnet arts high school. Our days demand a lot, but mostly, everything is just swell.
Everything was just swell. I was beginning the long exhale of having both daughters finally back in school when cf wreaked havoc once more.
Av is dealing with an adverse drug reaction, a complication that surely lands high on the list of alarming setbacks patients with chronic illness and their parents face. Its onset comes fraught with questions, concerns, and regrets. At the heart of the matter is my child’s acute physical pain and safety; potential long-term effects from a course of medicine that was only supposed to help, not hurt; and a steadily shrinking list of available treatment options for the future.
We’re convinced the culprit was a heavy-hitting two-week course of oral antibiotics, previously tolerated, but which has left Av saddled with mind-numbing, debilitating tendinitis of the wrists and knees. Since when did a kid treated for a lung infection wind up in wrist splints and knee braces? Talk about a reckoning with risks versus benefits.
Life interrupted by tendinitis means her participation in JV tennis and orchestra programs halt; a relatively fit 14 year old takes the elevator instead of stairs; and she struggles in first quarter of high school to keep pace with note taking, essay writing, art projects, and a CPR unit. All because of her medical treatment. It’s frustrating and disheartening to stand by knowing she’s in pain as she tries to sleep, write, draw, play cello, play sports, catch the bus, do therapy, and otherwise move normally about her life. It seems so unfair.
Yet when have we ever known cf to be just, considerate, or discriminate.
I hope eventually we conclude, ‘this ill-timed adverse drug reaction rudely interrupts her regularly scheduled cf life to bring lots of extra strength and resilience.’
My remarks during the opening ceremony of our cf walk yesterday…
Happy Great Strides day, everyone! This looks and feels just like a big birthday party! We are here to celebrate Great Strides with all our CF fighters…including my daughter. Instead of wearing a party hat, I think I’m going to stick to my old well-worn Aviary hat that says ‘cure cf.’
This morning, I actually want to talk with you some more about hats. Like many of you, I wear many hats. I wear the hat of daughter, sister, wife, and friend. I also wear the hat of mom to my two daughters, Av and Lou. I wear the hat of a CF mom for Av. I wear the hat of her caregiver. And now that Av is a teenager, I am proud to say I wear the hat of her partner. We have reached the incredible milestone where she and I now share in many of the responsibilities of her care.
As for her care, it’s never a party. It’s no fun for her to be constantly fighting aggressive infections. It’s no fun watching your daughter’s disease progress with every passing year.
But there is some good news, too, in the world of CF. Av would tell you, since she started taking one of the breakthrough treatments called Orkambi, she is breathing a little bit easier. It’s the CF Foundation that drove the research and development behind Orkambi in large thanks to fundraisers just like this one. Breathing easier is a gift. And that’s the best reason I can think of to be celebrating on Great Strides day.
Let’s circle back for a few more moments to hats! One of the most meaningful hats I wear is the hat of an advocate. I used to think being an advocate meant going to speak with our congressman about CF, and so we did that. Later I learned that being an advocate also meant standing up and speaking up for my daughter when she was sick in a hospital bed. I’ve done that.
But do you know what else makes an advocate? An advocate wears the hat of a walker. The hat of an awareness raiser. The hat of a fundraiser. An advocate is a champion who raises his or her voice for a cause she or he believes in. Do you know what that makes you? Because you are standing here today celebrating Great Strides with me and my daughter, you, too, are a CF advocate. Together we are amplifying the call for even better treatments for our loved ones, who valiantly fight cystic fibrosis, and one day, for their cure.
Fellow advocates, to borrow a line I hear often from our walk director: thank you for all you do! Hats off to you and happy Great Strides!
I won’t forget a time, years back, when I recoiled at the notion my daughter would always be sick. We were at a routine cf clinic visit. After the doctor had seen her, one of the therapists waltzed into the exam room and greeted Av by saying sympathetically, “I hear you’re sick.” Geez, really. Did she have to use that word? I wished this person hadn’t planted a single seed of concern in my young gal’s psyche. Could she not see how very well she looked?
Granted, Av happened to be coping with a little extra cough that day. The doc was putting her on more antibiotics. But despite some pesky symtoms, she hadn’t slowed down a bit. She was keeping up with school and activities as usual. She wasn’t home in bed with a fever or something. She would take her medicine and be good as new within days. Sick? Av was not living life like a sick girl. I simply could not equate a little increase in cough with the state of her being sick. Also…denial ain’t just a river in Egypt. I guess even a cf mom is susceptible to falling down the invisible illness trap.
When both my daughters were about four years old, they were obsessed with reading a thin paperback storybook about The Berenstain Bears, which still remains dogeared on our bookshelf. You Berenstain fans may know the cubs (along with Papa Bear) always learn morals, and in this particular story, Mama teaches “that appearances can be deceiving.” She explains, “It’s just a grown-up way of saying that things aren’t always what they look like.” I admit, for me; as a grown-up, parent, and caregiver, all three; she makes a pretty ominous point pertaining to chronic illness. One of the most confounding aspects of cf is remembering that inside the beautiful facade, a war rages on. Some small battles are won from time to time, while others are lost forever to the triple threat we know as persistent infection, insidious inflammation, and permanent scarring.
What does sick with cystic fibrosis really look like? Sometimes sick shows its cards with its hallmark thunderous, juicy, body-quaking coughing fits, accompanied by globs of sticky mucus. At other times, sick is subtle, sneaky even. Sick is a breath a touch too crackly and constricted; a complexion a mite more translucent and sallow; a voice puffing from deeper within the windpipes. Sick is tired from snoring and waking up already fatigued. Sick is throbbing pain to the head, the tummy, and chest. Sick summons up anxieties about time. What time it is going to take her to fight yet another battle. What time she will lose to the chronic illness lurking inside.
Appearances can sure be deceiving. Cf fooled me once, but no more. While the battles continue to ravage lungs, pancreas, and sinus on the inside, if you happen to blink, you might think you’re only seeing a picture of health.
On the eve of her big journey to the Netherlands, according to plan, I was spreading on the dining room table all the contents to be packed into Av’s therapy backpack, when it hit me like a brick: the refill she needed of Orkambi had never shown up. It was already after hours on Friday evening! I had been expecting that medication on Thursday morning! Since overnight packages tended to be reliable, I previously checked off both mental and written packing lists. What could possible have happened between Thursday and Friday to cause me to overlook something as essential as a missing Orkambi delivery?
Over the last 18 hours, I had single-handedly juggled the end of my first week in a new office, the kids’ early release for the start of spring break, an eighth grade conference, a kindergarten conference, a high school parent meeting, and a Thai dinner out. That all happened successfully, yet no delivery.
I scurried to login to myChart, and shot a concerned message to our pharmacist who manages Av’s drug orders. I stated the predicament flat out, “Hello, I realize there’s nothing to do about this on a Friday night, but…”
Orkambi is one of the groundbreaking treatments known as a cftr modular, which to some extent corrects the genetic defect that causes cystic fibrosis. Since Av added it to her regimen after she turned 12, we give it credit for a boost in her lung function, and even more, for stabler health. The thing to understand about Orkambi is, it’s a deluxe, precision medicine exclusively for select cf patients; its annual price tag could buy you a decent house in a small town. Unfortunately, you can’t just pick this stuff up at CVS.
We were in a pickle. She was in a pickle. For all the well-laid preparations, she would have to go to the Netherlands with a gaping hole in her safety nets. I could continue flipping out, I could seethe, or stress, or I could:
Exhale and let Av know about the missing medication. When I told her, she launched into a rendition of a Scout song she made up a few years back on a camping trip. The refrain goes, “Iiiifff we die we die together…” I joined the chorus, which made us giggle for about half a minute. I then asked her, assuming she would be traveling with what little Orkambi she had on hand, whether she thought it better to take the regular dose for five days and quit cold turkey or halve the dose and spread it out over ten days, almost the length of the trip. She would decisively opt for the latter.
Dial up the pediatric pulmonologist on call at the hospital over Friday night to ask if there was any shot whatsoever in hell of acquiring an Orkambi refill from a specialty pharmacy before 10:30 a.m. on Saturday morning? The answer: highly unlikely, but he would check first thing and get back to me. We discussed the dosing plan (he agreed with Av) and he reassured me she would survive this, too.
Exhaust my limited courses of action, and then put the problem in perspective and accept the change in circumstance. Av traveling without enough Orkambi is pretty much a nightmare come true, but it could be even worse. Missing digestive enzymes, for instance, would be a far more painful prospect. So there. The whole trip is one giant leap of faith anyway.
What lesson, aside from the dark side of procrastination, was I reminded of on the eve of Av’s trip? (All you cf parents out there: order those refills early and often!) No matter what, we can’t let our foe get the better of us. We must balance the endless string of challenges cf presents us with enough levity for our own sanity. Take it from my gal. When she boarded the plane, she had a brave smile on her face, maybe a tear in her eye, my kiss on her cheek, and a ridiculously adorable campfire song in her heart… “Iiiifff we die we die together…”
We’ve got traveling on our minds. Among other exciting trips our family has planned this year, Av herself soon embarks on an adventure to the Netherlands over spring break with her granddad (aka Opa) and cousin. While she’s a seasoned traveler, this is her first time going away anywhere for any length of time without me coming along. And there’s only one way she can pack: heavy. I’ll try the translation in Dutch: dat is zwaar. You see, with cf, I know of no humanly possible way to travel light.
We are destined to pack large. Our dog weighs 70 pounds. Lou still needs a booster seat. Phil usually wants to bring along a canoe or a chainsaw or something. Since Av is a cellist, we’ve figured out precisely how to wedge the cello case into the back of our small sportwagon without crushing the bridge, which leaves room for…not much of anything else. On our big road trips, I’m typically the one in charge of packing up the whole cf show. Having over a decade of practice, and given an uninterrupted hour or two or half a day, I can expertly compress every element Av needs for her cf care into just three pieces: a 66-quart storage bin, a 15-pound roller duffel, and a cooler. These vessels take up merely three-quarters of the rear cargo hold. Who cares about suitcases anyway?
In truth, the heaviest baggage when traveling with cf is not so much the luggage as the forethought and fortitude required. The dream would be to send my gal off to bike along the canals of Holland carefree. Visiting her Dutch relatives with nothing more than a sketchbook in hand and a passport in her pocket. The reality is, traveling and cf aren’t that compatible. If she ever wants to go to away, whether on a sleepover, to a sleepaway camp, or to college for that matter, the predicament is how to depart from the regular home routine of cf therapies. Seems like we always scramble for pills and a modified treatment schedule because, as well prepared as we try to be, spontaneity and cf still conflict. The central problem boils down to: cf never stays home.
Of course, cf also never takes vacation. And so, off it must go as Av’s constant companion. If you’re curious about how much daily responsibility she’s going to have over the course of her Netherlands stay, check out the “quick” care tracker she and I drafted together. It may be a hassle for her to fit in so much therapy while traveling, but at least it will come second nature.
Av’s first solo voyage is bound to be not only her own rite of passage, but ours. Can you hear the echoes of “time to let go, let go, let go…?” Indeed, we’ve no choice but to trust in her abilities to care for herself. We trust she’ll return home to us in good, if not good enough, health. Should cf so happen to prevail and she gets sick, well, I’m sure we’ve got extra treatments for that. What’s most important at this juncture is her embracing an awesome adventure she can cherish for a lifetime. The world beckons to our intrepid young traveler. We are wishing her not only a goede reis, but blessings on her journey.
I recently connected with a local mom who, like me, has two beautiful daughters. Her baby girl was born with cf. She invited me as an “experienced” cf mom; along with a social worker and respiratory therapist from our center, and staff person from our chapter; to join in an evening gathering at her home to talk about what life is like with cf in the presence of her closest extended family and friends.
The purpose of the evening was to inform the inner circle about how best they could support this family and their little daughter. But the benefits of the conversation were exponential. I can’t think of a smarter, more inspired idea than calling in your reinforcements during those early days of cf parenting. Those are the days when you’re still fretting over your baby’s every gurgle and wondering what warrants calling the doctor. Those are the weepy days of wondering when or if you’ll ever feel (back to) normal. Frankly, you’re wondering if anyone else can adequately learn how to care for your child with so many complex medical needs. You’re seriously doubting you’ll ever get another date alone with your spouse.
This family welcomed me warmly into their home on an icy winter night. We shared in food and wine before settling comfortably in the living room to talk. We gazed upon the baby who kicked and cooed happily on a blanket on the floor. While we can predict certain things about cf, we can’t know what’s in store for this child, what challenges her future holds. Only one thing’s for sure. The goodwill of the exceptional people who gathered together in that living room is sure to carry her through.
Here are the thoughts I shared with them:
You can still be the parent, or family member, or friend you are meant to be. You just add on an extra layer for cf. Think of cf like a blanket, albeit sometimes a wet and scratchy one! My advice is to stay true to your parenting style. Hold fast to your family traditions. Keep the pastimes you love in your life. Keep traveling. You just have to bring along the blanket.
Your child can still be the person she is meant to be! Remember, cf is now a manageable condition people survive with thanks to advances in treatment and care. Grown-ups with cf often say their disease does not define them, but is only a part of them. Give your child any opportunity to pursue her dreams. She just has to bring along the blanket. For a long while, you’ll be there to help her carry it.
There is no denying cf requires a lot of extra time, work, emotional and physical energy, organization, money, and more. These factors will become your new normal. Be ready. Because before you know it, you’ll be cooking nebulizer stew every night for at least the next eighteen years. And you’ll get so good, you could do it with your eyes closed. Guaranteed. You’ve got this!
Cf will absolutely change any old easy-going ideas you used to have about germs and cleanliness, and illness and medication. You will panic every time you hear about a sick classmate or cousin. You will never again forgo a flu vaccination. You will never leave home without having enzymes and hand sanitizer in your possession. Never ever. Welcome to the hyper-vigilant club.
Cf will change you forever. It brings the whole family plenty of heartache, but also silver linings, and maybe even a greater purpose. While much of cf feels uncontrollable, remember the parts you own. You can:
Establish a good routine early-on for your child and your family;
Make adjustments as you learn. Once you know better, do better; and
Jump into cf events to feel like you are actively helping your child.