Stability Ball vs Lower Body

Welcome back!

Try out these exercises with your ball.  They are great for your legs and abs! And don’t worry, you can do them with a PICC in your arm!

Another Great PICC-time Exercise: The Lunge

This is a little experiment I am doing in anticipation of the upcoming Stanford/UCSF research project I’m involved with.  “MRSA-schmeRSA,” I say.  So what if I can’t coach the little guys in person? This is 2010 and I have YOUTUBE!

Have you ever thought it would be kind of cool to be able to meditate, but then a tiny little voice in your head would say, “Are you kidding?  Spend 30 minutes focusing on my breath?  I’d rather stick a needle in my eye!”

I’ve been there.

But then, 13 years ago, in the midst of one of the more stressful periods in my life, I signed up for a class called Mindfulness-Based Stress Reduction (MBSR).  It was an eight-week class that met weekly for two hours, and included one all day “retreat” toward the end of the class, where we practiced in silence what we had been working on over the previous weeks.  This was one of the best decisions of my life, and meditation has become one of the most effective tools I have as I continue to live a full and happy life with cystic fibrosis.

This class made such an impact on me, that I have now learned how to teach it.  The reason I took the time and spent the money for this training is that I want to teach others with CF how this simple practice can make a difficult and sometimes complicated life just a bit easier to handle.

I took the class (twice) in person (both times in hospitals), and co-taught another eight-week session with my mentor in a hospital in San Jose.  Why meditate in hospitals, you might ask?

Actually, the MBSR program originated at the Stress Reduction Clinic, which was founded in 1979 at the University of Massachusetts Medical Center by Jon Kabat-Zinn. Now, it exists in over 250 medical centers across this country as well as in numerous locations internationally.  Consistently, graduates of the program report:

• Coping more effectively with both short- and long-term stress
• Greater self-respect, energy, and enthusiasm for life
• Lasting improvements in physical and psychological well-being

You know that having cystic fibrosis does not define you. Yet, it can be hard to find yourself in the midst of treatments, medications, doctor visits, hospital stays, and constant concern over that magic number, the FEV1. Having a chronic illness like cystic fibrosis is stressful.  This is just a fact of life.

What is often forgotten is that there is much more that is right about us than is wrong! Using the techniques taught in Mindfulness-Based Stress Reduction, we can develop skills that will help us stay afloat in times of chaos, and get more in touch with aspects of ourselves that are untouched by problems with an epithelial chloride channel!

Common Questions

What is Mindfulness?

Mindfulness is simply purposefully paying attention to what is happening in the present moment, without judgment. The present moment is where life unfolds, and it is only here where choice is possible. By cultivating the practice of mindfulness, you can begin to see where you tend to be on “autopilot,” and learn to use compassion and courage to make conscious choices about how you allow life to unfold, rather than feeling completely out of control. Mindfulness practice is ideal for cultivating greater awareness of the interconnection of mind and body, as well as of the ways our unconscious thoughts, feelings, and behaviors can undermine emotional and physical health.

I can’t focus on my breath…How can I meditate?

The good news is that the leader of the class (me) also has CF and understands this dilemma.  There are other ways to use mindfulness to better cope with stress.  One does not need to focus on the breath.  There are many other ways to anchor the mind.  Breath is just a very easy one to teach, and it’s always there.  Because I understand that attention to the breath can provoke anxiety, we will explore other ideas.

I can’t go to a class.  I have a multi-resistant bug. Or, the corollary:  I don’t want to get multi-resistant bug.

The best news yet:  This class takes place in a virtual classroom.  All you need to attend is a computer with Internet access.  If you would like to be able to speak (and this is encouraged), a computer headset is recommended.

What are the details?

This class will be an 8-week intensive training in mindfulness based on ancient healing practices. In addition to the weekly classes, there will be one full day retreat scheduled toward the end of the course.  The price of the course is $350, but no one will be turned away for lack of ability to pay.  If you would like a scholarship, please contact Julie Desch at Julie@newdaywell.org.

Registration can be completed here.

The mind and body are linked. We know this now through innumerable well-designed scientific studies, and we are learning more every day about how this works. Don’t kid yourself into thinking that you have no input into your health simply because your disease is genetic.  When you learn the practice of mindfulness, you begin to experience exactly what this means, and with that understanding, you can begin to see some wiggle-room around unhealthy habits of the body and mind.

Give it a try by registering now

Class description

Class schedule

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Operation Exacerbation

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!

“Port”al

Eckhart Tolle likes to talk about “portals” to the Now.  My favorite of his suggested portals is focusing on the body sense.  It is a very simple exercise:  you simply ask yourself, “Without moving or looking at my left big toe  (or whatever body part you choose), how do I know that it is there?”  Immediately, you are connected to the feeling present in the body, and when this remains in your focus, you are in the present moment.  Try it.  Pick some part of your body, close your eyes and ask yourself, “How do I know that ______ is there?”  Then, let your attention move to feeling the entire body this way, as a whole.  This is using the body as a portal into the Now.  And of course, the beauty of being in the Now is that you can’t be uselessly rehashing the past, or pointlessly rehearsing the future.  Life is always Now anyway, and this exercise places you right smack in the middle of it.

Shifting focus….I was thinking about ports the other day.  Central ports…you know the ones.  The things we hate to think about needing, because it means we need antibiotics frequently enough to justify the risk of an indwelling central line.  A central port provides immediate and easy access for administration of life saving medication as we watch our lung function diminish.  I don’t know about you, but I have always had a visceral reaction to the idea that I may need such a port someday.

So when my partner mentioned the other day that maybe I should consider getting a port, imagine my surprise when my immediate thoughts  (really) were about Tolle, and how “port” and “portal” clearly come from the same root.  So now I’ve looked it up and, sure enough, the Latin root, porta, means “gate.” Tolle’s portals are gates to the Now, and a central port is a gate to, well, your heart and circulatory system.  The next thoughts I had were about the bright side of having a central port. In other words, I didn’t freak out.

There are definite pros to having a port.  No more PICC lines, for one!  My PICC’s always have to go into the right arm (clot in the left–from a PICC, of course), and always have to be put in by Interventional Radiology (I love those guys, but really…it’s another appointment, it’s more radiation, and they SEW the sucker in so it’s hard to pull out yourself:-)).  Not only that, but as you know, you can’t lift weights when you have a PICC (did I mention the clot in my left arm?).  So no PICC, means no three week layoff from one of my favorite ways to stay in shape.

Maybe it’s my age.  Maybe it’s wanting things to be simpler.  Maybe this just means I don’t care as much about what “other people will think.”  But I’ve been thinking about it in a very “accepting” kind of way, and will likely talk with my doctor about this the next time I need IV’s.  (He’ll probably say, “Are you crazy?”)

Which brings me back to Tolle.  Full circle.  Maybe a central port could be viewed as a sort of metaphor for a “portal” to Acceptance-with-a-capital-A.  There’s no denying or fighting the fact that the lungs are needing some serious help when you submit to a port.  It would be a daily visible reminder of my mortality staring back at me in the mirror each day.  It would be hard to ignore evidence like that.  Still, I’m not freaking out for some reason…

I’m liking this metaphor.

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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?

Mindful Shaking

Thirteen years ago, I was a member of a Mindfulness-Based Stress Reduction (MBSR) group at the hospital where I was working.  MBSR is an 8-week program, first developed at the University of Massachusetts Medical Center by Jon Kabat-Zinn, which teaches the practice of mindfulness.  Since it’s inception in 1979, over 200 medical centers in this country offer this course, as well as several international locations.  The program continues to grow because it works.  The beauty of MBSR is that it is evidence based.  Many published studies in medical journals have shown the benefits of mindfulness meditation in working with medical challenges.

WAIT!  Don’t stop reading.  I know I used the “M” word.  I can practically hear you saying, “I don’t want to read about meditation.  There is no way in the world I can, or want to, meditate!”   I get that.  But just humor me for a moment.

So back to thirteen years ago:

I was STRESSED!  I was at a job that I loved doing…but couldn’t stand doing with the people I was doing it with.  Not all people of course.  Just two.  But they were making me miserable.  (Actually, in retrospect, I now see that I was making me miserable, but that’s another story.)  So, the environment was toxic for me.  I hated going to work.  When I was there, all I wanted to do was leave.  I was in an emotional heap on the floor most of the time.  In addition—quite probably as a consequence, my health was spiraling in a downward direction for the first time in my life.  And to boot, I was about to become a mother!  A good thing, yes, but as we know, even good stress is stressful.

I knew of Jon Kabat-Zinn’s work, and when I saw the class offered, I jumped at the chance to do it.

In brief, the practice I developed during that 8-week course probably saved my life.  It certainly helped me to calm down enough to make the rational decision to remove myself from an environment that was literally killing me.  Ultimately, I retired.

And, I kept up the practice of meditation.  I will admit, my practice waxed and waned.  I didn’t always have a daily practice.  In fact, at times I went months without a formal practice.  I even played around with different types of meditation.  What I noticed was that when I was diligent with just doing it, whatever type I used, life went smoother.  No, that is wrong.  Life was the same…constantly changing when I didn’t want it to (read: health declining), or presenting me with “opportunities” for growth (read: emotional pain).  What went smoother was my response to life.

Fast-forward thirteen years:

I am now in the midst of taking a teaching practicum in MBSR.  Yep, I want to teach it.  In fact, I want to teach it to YOU.

So what is this about Mindful Shaking?  Well, first let me just point out that mindfulness is pretty simple.  Easy? No.  Simple?  Very.

Mindfulness is the simple act of being present, non-judgmentally, to the experience of living in this moment, right now.  For instance, when you are mindful of breathing, you bring your awareness to the in breath as you inhale…you simply feel it, whatever that brings.  Then, you watch your out breath, as you exhale.  You don’t try to control your breath.  You just watch it.  And you watch whatever accompanies it:  emotions, thoughts, sensations, whatever, in a non-judgmental, detached sort of way.

For me (at first), this brought a lot of anxiety!  Learning to be mindful of the breath is not always the way to start with someone with lung disease!  In fact, I don’t recommend it.  Many people that I have discussed this with (most with CF) think that this means that they “can’t meditate.”  This is unfortunate, because so much can be gained from the act of being mindful!  You can practice being mindful of ANYTHING!  It doesn’t have to be the breath.  The breath is just easy, because it is always there.  It is the path of least resistance for most people.  Because of this, it is the default way of first teaching this practice.  But this is clearly not the way to go for those of us with CF.

So here is what I propose:  The next time you strap yourself into your Vest, instead of watching TV, or getting a headache trying to read a vibrating page of print, simply close your eyes, and notice the shaking.  Notice the feeling of the Vest expanding and contracting as you breath.  Notice the intensity of the shaking.  It is always changing, depending on whether you are breathing in or breathing out.  Notice how the shaking extends to the different parts of your body and how those sensations are always changing.  Talk about impermanence!  A vibrating Vest is the PERFECT vehicle to focus on to understand the concept of “always changing, always moving.”  In fact, mine starts and stops 14 times per second!

If the Vest is doing what it should, it is quite likely that you will need to cough.  This can be challenging, but what the heck?  See if you can be mindful while you are coughing.  If you pay close attention, you can sense the feeling of the cough-to-be.  Watch it, and then you can watch what your body naturally does in response.  Afterwards, it is interesting to watch how your body calms down again after multiple spasms of intense activity.  Slowly…very slowly, it comes back into balance.

Notice your thoughts about all of this (and you WILL have thoughts).  When you notice a thought, simply acknowledge it, and go back to the sensation of the vibrating.  Thinking is what your brain is made to do.  It is a normal process.  So just because you catch yourself thinking a lot (and you WILL), this doesn’t mean you are not doing this RIGHT.  It simply means you have a human brain.  If you had a bird’s brain, this would not likely be a problem.  However, then you would likely have other issues.

Luckily, as a human with a frontal cortex, you have a choice of whether you get lost in your thoughts, or whether you pull back as you notice the thoughts, and go back to awareness of the shaking.  It’s tempting to go with the thoughts.  After all, that is our pattern—our habit developed over years and years.  But just for fun…just for a minute or two, try the lesser-known path, and go back to sensing.  Not much happens if you do it once or twice and then quit.  This practice takes some perseverance before you begin to reap the benefits.

Then, one day it will dawn on you that the You that is watching the sensations is not the sensations, nor the body that is experiencing the sensations.  The You that is watching the thoughts is not, in fact, the thoughts.  The You that watches the fear and anxiety is separate from fear and anxiety.  And, the best part is that it ALL changes!  Everything…all the time…is in flux.  The only thing that doesn’t change is the Watcher.

One last suggestion:  Make sure that for at least the last minute of the session, you are very focused on being mindful of shaking, eyes closed…very focused.  The reason is that the moment of transition from shaking to non-shaking is so cool, that it is really hard to describe.   It simply has to be experienced.

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!

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.

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