Chronic Lung Disease for now

Diagnosis: CLDFN - Chronic lung disease for now. 

She does not have asthma, although she uses inhalers to reduce inflammation in her lungs and in her trachea because...
She does have chronic lung disease  and the experts expect her to grow out of it (yes please).
She seems to have some seasonal allergies (who doesn't this year I'd like to know).

Why has Grace gone from overall healthy to in and out of coughing jags, in and out of pulmonary clinics, and all the things with the steroids, the inhalers, the Zyrtec, and respiratory therapy...

"She was doing so well..."

For those of you playing along at home with inquiring minds, and for those of you playing along at home who also have a child with naughty lungs and airways here's the scoop:

Since June of 2013, just following heart surgery, Grace switched from Pulmocort to Qvar. Both are inhaled corticosteroids to reduce and keep away inflammation in the lungs. She also then, switched from Albuterol to Atrovent which she uses as necessary as an "emergency" inhaler to quickly open airways. Albuterol affects the heart rate (not great for heart patients) and has been shown to make tracheomalacia worse (not good for Grace and her floppy trachea). Atrovent has been shown to not affect the heart rate (as much) and actually helps tracheomalacia in most patients. Win-Win. From the time she started those inhalers until May of this year (almost a year) she had only one lung infection and there ain't no inhaler that would have helped with that one. It was a doozy.

The plan we worked out with our pulmonary/ENT/cardiac-league of justice, was to wean her off of the Qvar once cold and flu season was over. We decided to wait until after returning from a vacation and began to wean after Easter. After about a week I noticed no change in her breathing, but I did notice some drainage and clearing her throat. I was mostly listening for any breathing issues so I didn't think much of it. After two weeks it was still there. After 3 weeks a pesky cough started and it worsened quickly. Because of our very very mean winter and subsequent delayed spring, this year's allergy season is among the most offensive in recent years. Mid-may the pesky cough was so persistent that I took her to Urgent care on a Sunday morning and the doctor listened, examined the x-ray and gave us a prescription for antibiotics, calling it pneumonia. She did tell me that the radiologist disagreed with her diagnosis - but I was to follow up with her pulmonologist the next day. When I did, she said that after reviewing her x-ray and comparing it with prior films it was not pneumonia. The coarse breath sounds the urgent care doc heard are Grace's normal but abnormal coarse lung sounds and I was to stop the antibiotic and start a course of steroids. Why does she have coarse lung sounds? Because her lungs fought a battle of being deprived of oxygen for almost 21 months, endured chronic pneumonia, and horridly filthy air. They can heal, they can regenerate, they are expected to heal. This is the time in her life when lung tissue regenerates at the fastest rate, and we know that they have improved some in the year she has been with us.

After managing to squeeze ourselves into a visit with our pulmonologist face to face a few days later, about 100 questions, and reassurance that this was an unfortunate byproduct of seasonal allergies and postnasal drip, we returned home with two more days of steroids, zyrtec, and were a little wiser. No more weaning off of the inhalers right as allergy season hits. We plan to try again next year during the summer.

After a week on allergy meds, being back on inhaled steroids, and a course of an oral systemic steroid (prednisone) for a few days - she was good as new, and better all around than she had been in weeks. For about 3 weeks while being off the inhaler, she had not been herself. Her whole personality was just off. She probably felt lousy, her lungs inflamed, and without the vocabulary to explain it to me had been acting out, throwing fits, not sleeping well, having night terrors - it was tough on all of us. It was a relief to have her back to her best self.

After being on twice the recommended dose of Zyrtec for 2 weeks and seeing her return to healthy, non-hacking-like-crazy Grace, I started giving her less (the routinely recommended dose) of zyrtec. Her pulmonologist had said that twice the recommended dose was good for her at the time and that I could give half of that once she was better. After a few days half the dose - the cough returned. It returned for two days and then a fever joined the party. Then her tiny nose stuffed up. Lord have mercy. X-ray was clear, pulse ox was good but she was exhausted and miserable. Back up went the Zyrtec, we doubled the Qvar, and after our 4th clinic visit in 6 days I feel like we are heading toward "normal" again.

There have been some victorious moments, however. We had our orthopedic check Monday to check on the congenital scoliosis, in the hope that it had not worsened. We learned that although congenital scoliosis rarely improves, her degree of curve HAS improved both the lower curve and the upper curve! Praise the Lord! The doctor is happy and surprised and I am happy and not that surprised because she looks straighter to me than she did a year ago. Her muscles are doing their job pulling things in her spine where they need to be and while she will likely always have curvature because she her two wonky vertebrae, improvement is to be celebrated. After that visit, I happened to walk past the pulmonary clinic. I was waiting for a call back from them about the crazy-coughing and since-I-was-there....thought I'd pop in and see if anyone had a minute to hear my hacking child.

Of course I did.

They did not, the docs had already gone home or were on rounds...but telling a lot of details to the receptionists who did not seem enthusiastic about my visit, they did gave me an appointment with a different available pulmonologist the following morning and I happily took it. Our pediatrician is off on Mondays, so a completely new urgent care doc listening to Grace would very likely repeat the same scenario detailed above (listen, hear coarse sounds, view x-ray, see cloudy business that is just her lung tissue not pneumonia and say she has pneumonia and prescribe antibiotics) and since I'm not a fan of starting and stopping antibiotics willy-nilly I decided to be the squeaky wheel. You're darn right I did.

The squeaky wheel really does get the grease. Squeaky wheels unite!

That visit Tuesday morning, was such a valuable opportunity to get yet another perspective, another explanation, some reassurance, and further absorb the reality that it most likely won't always be this way. We are now aware of things we weren't aware of before (allergies, for one), and this pulmonary battle is complex. It is exhausting. It's a "let's try this and she how she does" approach because the pulmonary system is complex and there's no one thing that works for each situation and each kid. So we try and we adjust and we stay with the things that work until they don't work any more. Then we try new things and hope they work.

It's times like these when I'm reminded of an analogy a friend of mine shared when he was facing cancer treatments. He said,

"I want to have a faith so anchored to Jesus that although it may life may be in tumult on the surface, 50 feet below it's tranquil." ~ Mark Deboth

It has not been the worst of times, it has not been the best of times. The not knowing what to do and how to help her is, in some ways, worse than knowing even if the plan for recovery is rough. There have been many days when it has felt very rough on the surface. Plans have to be cancelled, trips to urgent care are a challenge, sleepless nights pull the rug right out from under us it seems; and we have to dig deep to what we are anchored to: hope, healing, eternity, restoration, our Redeemer.

Again, and again, and again.

So if for the last 6 weeks or so we have been a little scattered, a little twitchy, a little absent from things we were supposed to be not-abesnt-from, a little late in responding, a little more forgetful than usual...

It won't always be this way.