Operation Exacerbation

Welcome back!

Rule Number One that I hear myself telling people who are bummed out about their illness, whatever it is, is to remember, “there is more right with you than wrong with you.“

And that’s true, right?  Think about it, by some miracle you are here on this earth, breathing, most likely able to walk around, enjoy food, listen to good music, pet your dog, watch Survivor…

Why is that?  Because most of the 100 trillion cells in the body, each of which are probably performing thousands of events per second, are actually doing their jobs perfectly well right now.  If not; well, you would not likely be reading this.

Now I’m not preaching here.  I am mostly writing this for my own edification, because I just learned that I am now infected with MRSA in my lungs.  I really can’t convey in words what happened in my consciousness when I learned of this last week.  Maybe you know the feeling that really bad news carries with it.  The sinking feeling in the pit of the stomach, the tight chest and throat, the heart racing and the blood draining from seemingly everywhere…  Sh_t!

Then the anger, and the self-pity arrive.  Sh_t (again)!  Why me, why now?  This is a REALLY bad time for this.  I’ve got plans, Universe!

And then, the tears.

So, now I’m officially a CF Pariah.  Gown, glove and mask everyone…here comes Julie.  Man, I’m pissed.  And of course, really what I am is scared.  What does this mean?  Is my CF going to get worse?  Will the freakin IV’s even work this time?

So, I allowed this to go on for a couple of hours.  Then, I was better.  Then, it came back with a full and serious vengeance over several days.  And now, finally, I’ve decided to respond instead of reacting.  Or at least…to try.

The first thing that came to my mind was to write the post I was going to write before the fateful telephone call.  It seemed to me that writing about having a “positive exacerbation” could only be credible if it were to be created during an exacerbation.

So, even though this is a slightly unusual (for me) situation, it is most certainly a “CF adverse event” and just as certainly would be best handled with a positive attitude.

Here are a few things I do to make my three weeks of IV’s…my “home vacation:”

1) First, since I can’t exercise strenuously, I don’t.  I legitimately and compassionately stop pushing myself.  A gentle walk every day that I feel like it is about as hard as I’ll push these days.  After the port goes in and the PICC comes out today, my arms will be free at last, free at last.  So as I feel stronger…I’m back to those kettlebells!  Have I told you about my favorite new exercise?  Something else to post about.

2) I have incredible friends who have been incredibly kind.  I will appreciate them every day, in some way.  By telling them what they mean to me, I will add positive energy to their world and mine.

3) Sometimes it takes a real blow to the illusion of stability to wake me up.  This has been a great one.  I will resolve to appreciate what is good in my life.  The best way I’ve done that in the past is to keep a gratitude journal, and vow to write in it every night three good things that happened that day.

4) I have a post that I have been writing in my head for weeks now about the growing importance of meditation in my life.  I have been trying to figure out how to stress this without sounding like a preacher…hence the silence about meditation.  But it has been SO key for me this last week, that I will write this post.  It will be the next one.  I will pour my soul into it, for both myself and for those two or three readers I have:-).

So if you like this plan, or if you have some suggestions, or if you’d like to share how you deal with your own “operation exacerbation,” please leave a comment!

A GIANT LEAP FORWARD?

Up until very recently, therapy for CF has been directed at correcting the consequences of the defective CFTR protein (thinning mucus, improving clearance, treating infection, calming inflammation, improving nutrition, etc).  Yes, when the gene was discovered in 1989, there was a flurry of research in the area of gene therapy…finding a safe mechanism to insert a copy of the “normal” CFTR gene into the targeted cells, and getting it to work.  This proved to be quite an undertaking, and while there is still much being done in this field, the exciting research making news today is from companies like Vertex Pharmaceuticals. With the Vertex drugs and others like them, this is the first time that a therapy—a small-molecule, not gene therapy—is actually directed at trying to correct the defective protein.

In a recent article in Xconomy, Dr. Bonnie Ramsey (who should really be in the CF-caregiver Hall of Fame) responded in part to a question about the Vertex drug VX-770, “Whether it turns out that Vertex is 100 percent successful or not, this is such a giant step forward, it’s like a man walking on the moon.”  Walking on the moon…. I remember that day.  It was huge.  It is my mission with this article to try to explain as best as I can what she is talking about.

To understand why VX-770 and its partner in crime VX-809 make such as giant leap forward for mankind, we first must have two small refresher courses.

CF Mutations 101

There are more than 1,600 known mutations of the gene that causes CF.   We now know that each of these mutations fits into one of five “classes.”  Each member in a class of mutations causes a disturbance in the sequence from gene (DNA) to CFTR (protein) to functioning CFTR protein at the membrane of the cell (electrolyte transport into and out of cell) in characteristic ways.

In a Class 1 mutation, there is no synthesis of CFTR protein at all.  Zilch.  This can be the result of a “nonsense” mutation, where a STOP message is read on the mRNA (transcribed from the gene) somewhere along the line, and synthesis of the protein is aborted.  Or, a Class 1 type of mutation can lead to a misread of the gene because of a “frameshift”.  Think of a frameshift as what happens when you forget to answer ONE question on a multiple choice exam where you have to fill in the answers by coloring in ovals on a separate sheet…all the answers after the one you forgot are wrong…chaos ensues).  No CFTR protein…pretty severe CF.

A Class II mutation is one where the gene codes for a protein that is constructed by the cell machinery, but because of the error from an amino acid deletion in the gene, the processing of the resultant protein is messed up. As a result, the protein is defective in folding, stability, and channel gating (the opening for chloride ions is not regulated properly).  Because it is unstable, not much of it makes it up to where it is needed at the cell membrane.  Our friend, delta F508 is a Class II mutation.

Class III mutations allow for the gene to code for a CFTR protein which makes it up to the membrane, but as a result of this “milder” mutation, the CFTR channel is not regulated or activated properly.  G551D is an example.

Class IV mutations are similar to Class III in that a protein is made and gets up to the surface of the cell, but it has “altered conductance.”  The ion channel just doesn’t work as well as it should.  R117H is an example.

Finally, Class V mutations are those where there is simply reduced synthesis of the CFTR protein.

Clinical Trials 101

You often read or hear about newly developed drugs being tested on humans in “clinical trials.”  These trials occur in a series of steps, or phases, that are designed to answer different questions.

Phase I trials are when researchers test a new drug in a small group of people for the first time.  These studies evaluate overall safety of the drug, look to find effective dose ranges, and document any side effects.

Phase II trials are designed to evaluate effectiveness of the drug and are generally performed with a much larger group of people.  Safety continues to be monitored closely.

Phase III trials are done with very large groups of people to confirm effectiveness, monitor side effects, compare it to commonly used treatments, and collect information that will allow the drug to be approved for use.

Now, back to regular programming:

When gene therapy was not proving to be wildly successful, some companies started to ask if the defective protein could be fixed.  Fortunately, a technique known as “high-throughput screening” was being developed just as the need to find ways to tweak the CFTR protein was becoming glaringly apparent.  Very simply, high-throughput screening uses automation (robotics and high-speed data processing and control software) to rapidly test hundreds of candidate “small molecules” to find the ones that show a specific biologic activity.  In the case of CF, they were looking for molecules that could assist with translation of the RNA “message” to form a CFTR protein normally, or molecules that could assist CFTR in getting up to the membrane, or molecules that could open the dang thing up and let the chloride ions flow as they should.

One company, PTC Therapeutics, found a compound called PTC124, which could to “read through” the STOP sign on the Class I nonsense CF mutations.  PTC124 (now called Ataluren) only works for Class 1 (nonsense) mutations, of course, but clinical studies so far are looking very promising.  Phase I and II studies have confirmed that Ataluren is safe, orally tolerated, and showed encouraging efficacy.  A much larger and long-term phase III trial is scheduled for this summer.

In the case of CFTR protein modulation, Vertex Pharmaceuticals looks for small molecule correctors and potentiators.  Simply put, a corrector gets the CFTR protein to the membrane in larger numbers.  This would be helpful in the Class II CF mutations such as delta F508.  A potentiator works on the protein already at the membrane, increasing its effectiveness.  This kind of drug could potentially be beneficial in several of the mutation classes.

VX-770, an investigational CFTR potentiator, is intended to increase chloride ion transport through the defective CFTR protein.  Vertex chose to specifically look at people with the Class III G551D mutation in the early phase trials of VX-770, because in this mutation, the protein is already where it needs to be on the membrane.  It just needs to be tweaked to open properly.  They figured that although only 4% of people with CF carry this mutation, the odds of showing effectiveness would be best in this small group of patients.

And, indeed, they were right!  Not only did Phase II trials show a marked (10%) improvement in lung function after only two weeks of treatment, they also showed that both nasal potential difference (PD) and sweat chloride levels moved distinctly toward normalized values (this is exciting because no treatment ever has shown to change the sweat chloride levels).  Importantly, when people stopped taking the drug, lung function values, sweat chloride values and nasal PD values returned to their baseline values.

Based on these positive results, Vertex is now initiating larger, Phase III trials.  These are designed to look at larger numbers of children and adults with the G551D mutation over a longer period of time.  In addition, a Phase II study of VX-770 in patients with CF aged 12 years and older who are homozygous for delta F508 is planned to start in the third quarter of 2009.   The hope is that VX-770 will measurably increase the effectiveness of the small amount of CFTR protein that actually makes it to the membrane in Delta F508 CF. If so, then all we need is a corrector to get more of the protein to the membrane, and throw in a dash of VX-770 to create a “Vertex-cocktail” of sorts.

Vertex is hoping that VX-809 is just that corrector (and so am I).  This molecule is designed to increase the amount of deltaF508 CFTR protein on the surface of cells lining the airway.  It is one phase behind VX-770.  So far, Phase I studies have not shown any safety or tolerability issues.  A Phase II study of this drug is now underway.  Where can I sign up?

In summary, I think the message is this:  There is serious cause for hope that one day soon, we will take yet another daily pill (or two…) that is going to improve our lives beyond anything that has yet been discovered.  Is it going to “cure” CF?  Not likely.   A scarred pancreas is not suddenly going to produce enzymes or insulin.  Damaged lung tissue is still damaged.  I am not suddenly going to have a normal FEV1.  But if I knew that a daily pill might slow or even halt the downward slide of lung function that has up until now seemed inevitable…I’d be pretty psyched!  I might even volunteer to write an article about it.  I only have one suggestion for Vertex Pharmaceuticals.  Will you please give these things proper names?

Control Trumps Fear When it Comes to Adherence to Exercise in Cystic Fibrosis

I’m getting a lot of ideas for posts as I prepare for this talk in a couple of weeks at the NACFC in Minneapolis.  I am speaking about motivation and exercise, one of my favorite subjects, and am quite happy to be doing it.

Today I reviewed an article published in Thorax 2004; 59: 1074-80, by Moorcraft et al, entitled Individualized Unsupervised Exercise Training in Adults with Cystic Fibrosis: a 1 year randomized controlled trial. Here are a few reasons why this is a well designed study and one to believe:  1) it is (in CF terms) a pretty long term study.  Most others are only weeks to a few months in duration. 2) It was randomized, a short-fall of many other exercise in CF studies. 3) After an initial training session, it was unsupervised and the exercises (though structured by a trainer) were done at home–so the positive results are  good news about adherence and sustainability of a program.  The patients were, however, given frequent contact by phone and/or clinic and were actively encouraged and motivated to continue.

The results were indeed positive.  After a year, a significant training effect was shown in the training group and there was a lesser decline in lung function in those trained when compared to controls. But, as important as that is, that is not why I am writing this.  The most important point of the article to me was in the summary, where the authors state:

“Every effort must be made to adapt the exercise to fulfill the wishes of the patients and integrate it with their lifestyle.  This study shows that benefit can be obtained with an individualized home-based programme.  In the long term, motivation must be sustained by the individual and the clinician must strive to engender an exercise habit.  A flexible approach to encouraging exercise and an enthusiastic approach from the staff should not be underestimated.  A feature that favours exercise adherence in CF is that the patients perceive it as an area over which they have control and that, unlike other treatments, fear of their disease does not drive adherence to exercise (my emphasis).  Instead, they have a positive outlook on exercise regarding it as a normal activity which they can enjoy.”

I don’t know about you, but I think that fear sucks.  It doesn’t feel good.  It incapacitates me when it comes to rational thinking, and over the long haul, it frankly shrinks my brain.  It is true that sometimes fear works to motivate.  If that weren’t true, I probably wouldn’t have made that phone call to my doctor when I coughed up blood.  I feared for my life, and a phone call was made.  Fear works in acute situations.  It is the flight aspect in the fight or flight response to the mountain lion on the bike path.  Ok, bad analogy.

The point is that as a long term motivator, fear is a BAD choice.  Chronic fear leads to increased stress hormones which lead to depression and brain shrinkage.  Neither helps with adherence to any kind of program, let alone one where you must insert significant energy, as in an exercise habit.

Control, however…now THAT is powerful.  To me, seeing and feeling my body respond to exercise over the long haul is not so much about control as it is empowerment.  I feel actual empowerment over at least part of my body…and this is not a common feeling for one living with a disease such as cystic fibrosis.  This empowerment leads to confidence in other areas as well, and makes one think twice about negating the effects of all that work by, for instance, missing treatments.

Thinking about going to the gym or going out for a run just like any other “normal” person makes me feel more “normal.”

Now think about a kid…an adolescent with body image issues and control issues who is angry and in denial about living with CF.  How helpful do you think a little dose of empowerment and normalcy might be?  Trying to instill a little fear into him or her would lead one direction…the one you don’t want to go.  Helping them to feel good about how well they respond to an exercise program and encouraging them to exercise because it is what we ALL should do…that works!

Running From Depression

A recent study by Cruz et al, “Anxiety and Depression in Cystic Fibrosis,” (Semin Respir Crit Care Med 2009; 30: 569-578) came across my desk the other day.  Oddly, I was in the middle of creating a talk about exercise and was on the exact slide where I talk about my favorite book on the subject of exercise, Spark, by Dr. John Ratey.

Ratey is a psychiatrist at Harvard and one of his areas of expertise is the neurochemistry of exercise.  I read this book when it first came out and have practically forced most of my clients and several family members to do the same.

The Cruz study points out in grave detail the increased prevalence of both anxiety and depression in people with cystic fibrosis, noting that these have important consequences, including poorer disease outcomes and lower scores on measures of quality of life.  Depression especially wreaks havoc on adherence.   It makes sense, really.  When you are depressed, it is very easy to blow off treatments.  When you don’t care, why bother? When you are consumed with anxiety, a trip to the gym is not the first thing you think about doing.

After these grave facts are discussed, the authors conclude that better screening for depression and anxiety should be done on patients with CF, and treatment given to those with symptoms, including medication or therapy (or both).  I would add one more tool to the bag, one that in fact would also likely work as a preventative measure.  And…it’s free (a big plus these days).

If Ratey is to be believed (and he gives sound reasoning and research to back up his material), the BEST time to head to the gym or lace up your walking shoes is when you are blue and don’t feel like doing anything.   The reason is that exercise acts as an antidepressant.  In fact, exercise is nature’s perfect antidepressant…with no side effects.  The reasons are complex, but I am going to try to simplify:

First and foremost, to call “depression” a disease is like calling a “cough” a disease.  A cough is a symptom that something is wrong.  Perhaps you have asthma.  Maybe you are choking on a marble.  It could be that the air is extremely polluted and all sentient beings are suffocating.  The bottom line is the cough tells you that something is wrong.  The underlying cause is yet to be named.

In the same way, depression is a symptom.  Many things can cause depression: pain, stress, medications, trauma, addiction, AND altered neurochemistry, to name a few.  Just looking at the last one, the brain’s chemistry can be messed up in completely different pathways and yet the final result can look similar.  This is why a medication that blocks the re-uptake of serotonin may work on me, but not you.  Someone else may only respond to a medication that increases Dopamine…or Norepinephrine.  The names are not important.  What is important is to understand that the whole thing is very complex.

And yet, one thing that we all can do at some level, exercise, seems to be able to jolt the brain back into balance.  It seems to regulate the neurotransmitters that antidepressants target….all of them…and at just the right dose.

Almost immediately when starting to exercise vigorously, norepinephrine is elevated.  This is the wake up and get going chemical that also works mysteriously on boosting self-esteem.  Also, dopamine, the brain’s attention system and regulator of feelings of well being, is elevated.  Finally, the well-known chemical serotonin, important for mood, self-esteem and impulse control, bumps up.  And to add frosting to this cake, endorphins are made within the brain upon exercise, and we all know what endorphins do…

So that’s the chemical story.   But there is an architectural one as well.  Exercise causes release of something called BDNF (brain-derived neurotropic factor), which Ratey calls “Miracle-Gro for the brain.”  This, and other neurotropins cause the brain to a) make new cells, and 2) create and foster new connections between brain cells.  As he explains, depression is caused not just by a lack of neurotransmitters, but also by a lack of connections within the brain itself.  BDNF fixes this.  Exercise releases BDNF.

Yes we are at high risk for depression.  And yes, depression is very bad for compliance with a complicated medical regimen.  Sure, we could take another pill or two or three (and some of us may need to) to combat depression.  But one easy thing to try right now is to move.  Every day.

How Important is “Quality of Life” in CF?

A couple of months ago, I asked very distinguished panel of CF care providers whether they thought, 1) that the scientific literature was compelling enough to persuade them that exercise should be a routine part of CF care, and 2) do they promote exercise in their clinics?

I was a bit surprised to hear that most were not convinced by the evidence to date, but happy to hear that all thought that exercise was “a good idea.” Not many had formal exercise programs or promoted it vigorously (other than Dr. Strandvik from Sweden). But they all agreed that it was good to move. Hard to disagree with that.

Now, I understand the caution engendered by this lack of conviction. After all, I was a scientist once. It takes multiple, repeated long-term studies that are designed with a matched control group, huge numbers of patients that are randomized as to who gets the intervention and who are the controls, and double blinded (neither the patient nor the doctor knows who is getting the “intervention,” to convince this crowd. But a study looking at exercise and CF can’t be huge (think about it…how many of us are there?), can’t be “blinded” for obvious reasons, and are extremely hard to create and fund for “long term studies.” The longest so far is a 3-year study from Toronto (oh yeah…that’s the one that showed pretty convincingly that regular aerobic exercise DELAYED decrease in pulmonary function in CF; Schneiderman et al, 2000). Most of the reported studies are 3 to 6 months in duration.. It’s hard to show much after only 3 months, and yet, it’s been done. Just not enough for this crowd.

So, instead of looking for irrefutable evidence that exercise improves lung function in CF, or that it increases mucus clearance, or that it prolongs life (all great questions that deserve more study), let’s ask a basic question that I believe HAS been answered. Does regular exercise improve the quality of life in someone with cystic fibrosis?

The answer is a resounding YES. Several well-designed studies have shown that increasing exercise capacity and tolerance in those with CF improved measures of QOL (quality of life) or QWB (quality of well-being). (Kaplan et al 1989, deJong et al 1997, Klijn et al 2004, Enright et al 2004). These studies include both children and adults.

To me, quality of life is worth improving. Yes, it is great to fund study after study looking for small molecules which correct the basic defect, or to find better antibiotics, or better ways of combating over-exuberant inflammation, or ways to thin mucus and clear it more effectively. But while all of these (and more) are in the pipeline, what about those of us in the trenches? Some of us may not be able to wait. If there are ways to improve our lives, our self-image, our body-image, our self-esteem and self-efficacy RIGHT NOW, shouldn’t these ways be encouraged? Shouldn’t studies about this be funded? Shouldn’t this very simple (I said simple, not easy) and inexpensive intervention be pushed HARD in our clinics?

I think so, and I’d like to hear what you think. Please leave a comment.

From Outside the World Of CF Research

Just randomly surfing while “puffing on my pipe” just now (I hope you all realize this mean a nebulizer), I ran across this.  Now let’s figure out how to attach this sucker to Pseudomonas…

Kary Mullis\’ next-gen cure for killer infections

Amazing stuff.

Rant

Ok, so sometimes it’s hard to be both sick and happy.  I will admit this.  Today, I am going to allow myself exactly the time it takes  to write this post to be less than happy.  So pardon me while I rant.

Sometimes having CF sucks.  There is no better way to say it.  This is one of those times.  Two weeks after pulling my IV from a three week course of antibiotics, I found myself in the Emergency Department with a fever and chest pain.   Bad chest pain…the kind that, when you have CF, you know is not going to end well.

Sure enough, the chest film shows an infiltrate and I need another course of meds.  So yes, at some point, I will get to “it’s a good thing there are antibiotics,” but right now, not so much.

Ok, enough.  I’m done ranting.

Sick and Happy Takes on New Meaning

I am going to be the first ever to blog about the negative effects of happiness in CF.  The following is an excerpt from an article from Stanford Medicine, published yesterday (the emphasis is mine).  In brief, the article reports the discovery of an exaggerated white blood cell response to inflammatory signals leading to lung destruction in CF lungs:

So what are the live neutrophils doing in patients’ lungs? The new findings surprised Tirouvanziam’s team. After collecting fresh neutrophils from cystic fibrosis patients’ sputum and analyzing them with fluorescence-activated cell sorting, the team discovered that signals from the patients’ lung tissue were reprogramming live neutrophils with conflicting messages. The first set of signals switches on what Tirouvanziam calls “an ancient happiness pathway” — a chain of commands that tell the neutrophils that nutrients are plentiful, and that it’s a good time to translate the cell’s library of genes into new protein. The second pathway is a cellular alarm system associated with inflammation and stress.

“They’re receiving a lot of signals at same time, and we think the happiness signals are messing them up completely,” Tirouvanziam said.

His team now suspects the inappropriate activation of the “happiness signal” — the molecular target of rapamycin, or mTOR, cell signaling pathway — may trigger neutrophils to release large quantities of human neutrophil elastase, the enzyme that destroys the elastic fiber of lung tissue. In healthy individuals, neutrophils never release destructive human neutrophil elastase into nearby tissue.

So maybe Sick and Happy should now be called Sick because I’m Too Happy????

THE TRYING, I MEAN ADOLESCENT, YEARS

My oldest son is about to turn 12, and I am getting a first taste of what is to come.  This will require fortitude….and the ability to dance.  No, I don’t mean really “dance,” I mean mentally and emotionally dance with him, as he comes into his own.  Now, if he had CF and I were trying to get him to exercise as a method of airway clearance and self-esteem enhancement, I would:

1. Not exactly phrase it that way.
2. Make sure it included other peers (unless this causes additional discomfort, embarrassment, etc…).
3. Introduce weight training as soon as he/she is capable of following direction and mature enough to be safe.
4. Strictly enforce the bike/walk/scooter/skate to school, the store, a friend’s house, etc… rule.
5. Hope that he/she liked to play soccer, basketball, baseball, or whatever team sport was available, so that a “coach” ordered the training, and not me.
6. Continue to use enticement, aka bribery, to encourage daily exercise.

Let’s take them one at a time, shall we?

The Wording and the Timing of the Wording
First, the word “exercise” has unfortunately taken on a negative connotation among many of our youth these days.  I don’t quite get it, frankly.  When I was a kid, the trick was in getting me and my friends to come in at night.  Now it is the exact opposite.  I suppose it has something to do with the myriad forms of indoor entertainment these days.  The problem has become that in trying to entice some form of movement away from electronic screens, we (and by we, I mean I) use the words “You need to get some exercise!”  Instant negative reinforcement.  “Exercise” is equated with  the taking away of something good…screentime.

If you are a psychology buff, you know that this negative reinforcement is not going to promote the behavior (exercise) that you want.  A more useful way to reinforce that behavior is to associate something positive with it.  Like Pavlov and the dog! Remember, bell…food.   So yes, get them away from the screen.  By all means.  But don’t repeat my mistake, and use getting exercise as the reason why.  Bad idea.

More on positive reinforcement later.

Make it Social

Though not a universal characteristic of teenagers, most would rather hang out with friends than do pretty much anything else.  If I ask my son to please take the dog for a walk, I get a, “Why….?  I don’t want to….I had gym class today….I’m tired…etc…”  If I instead say, “Will you take the dog over to your friend’s house and see if he’ll walk his dog with you?”, he’s off like a flash.  It’s just (teenage) human nature.  So why fight it?  This is one of those Aikido moments…use the opponents force to get them to do what you want.

When I was a teenager, it was only by starting to hang out with active friends that I discovered my inner athlete.  My parents didn’t really encourage it….it just happened.  I still wonder what would have happened had I stayed in my shell.

Weight Training

It is an old wives’ tale that teenagers shouldn’t lift weights until they are fully grown for fear of damage to the epiphyseal plates.  The truth is that as soon as a kid is mature enough to follow instructions and be safe in a gym with a trainer, it is perfectly fine to start weight training.

And the teenage years are the best for starting this habit early on.  Why?  For one thing, body image issues become overwhelming at this age, as we all can remember.  Now imagine going through that again, but this time with CF.  As a teen with CF, you deal with growth delay, puberty delay, an “unpredictable” body when it comes to lung function and GI function.  Your friends see you take a handful of pills and wonder what is wrong with you.  You spend inordinate amounts of time in bathrooms, your fingernails look weird…you get the picture.

Wouldn’t it be nice to be able to do something that had a visible, positive effect on your body that CF didn’t really effect?

The answer is unquestionably, yes!  It had a profound effect on me, and I have spoken with many other adults who say the same thing.  This is an issue where a child can actually have an “internal locus of control.”  They can get stronger!  They can put on muscle!  They can start winning arm wrestiing matches!  And CF can’t touch this.

Make Use of Multitasking

We are all pretty good at multitasking, so this one should be a no-brainer.  We all have to go places…even our children.  School, friends’ houses, the store, downtown, ball games, church….whatever.  We travel.  It is easy (I know) to get locked into a pattern of driving to all of these places.  Certainly, when the weather is bad, we need to do this.  But how many times could we just say, “I’m not driving you today.  I’ll walk or ride bikes with you…but we are going to get there the low tech way today….just for fun.”

Just as it is with small kids, it is not necessary to get all of ones aerobic exercise for the day done in one session.  It works just as well to break it into two or three smaller chunks.  So that 15 minute bike ride (each way) to school, if done intensely, could be just what the doctor (or coach) ordered for the daily goal.

Defer to the Coach

This trick works if you son or daughter is on a sport team, and practices with the team.  Then your job is easy…the coach makes your kid work, and you are off the hook.  If this describes your situation, count your lucky stars.

Not all kids are “team” types, of course.  So then what do you do?  Well, here is my pitch for wellness coaching.  If your child is mature and appreciates the need to  establish an exercise habit, working with a wellness coach is a great idea.  There are a couple of caveats, though.  First, if your child is not into the idea and only you are…it doesn’t work.  Behavior change is tricky business, and one thing is for sure, the changee has to want to do the hard work of changing.  The coach doesn’t do it….the parent doesn’t do it.  Second, I’ve learned through doing this that until a child is in their teens, it is best to scheule “family coaching” sessions.  Taking on a new habit is a big job, and a child needs support from not just a coach, but also from their family members.  Everyone in the familly needs to understand the plan, and be ready with support and encouragement.

Bribery

Now, we could call this something else I suppose.  But the truth is that is isn’t such a bad thing for a kid to understand the concept of quid pro quo.  Just as I said this works for younger aged children (remember pedometer steps for video time, stickers for exercise), when kids become teens, the concept still works; the stakes just get to be a bit higher.  Now we may be talking going to the movies, getting the car keys, going out with friends…you name it.

When you start feeling guilty about this, remember that the ultimate goal is for your chilld to learn for themselves during this time that they actually feel better when they exercise, and will hopefully find some activities that they love to do, and will keep loving to do into adulthood.  This is a critical time..and it calls for some …unorthodox methods.

If you have great ideas that have worked for you in encouraging your teenager with CF to exercise, please share them here.

Do You Ever Have One Of Those CF Days?

There’s been a bit of a lull in my series on shaking up your CF child’s exercise routine.  But, I have good reason.  I promise, I’ll return…as soon as….it works.

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