Unless you are a newborn, when you walk into a gym — quite a feat for a newborn — you are going to have some muscular tightness…some tension or “knots” in muscles that occur from chronic movement patterns that are less than ideal.  We all have less than ideal movement patterns.  This is not a judgment…it’s just a fact.

Those of us with chronic coughs especially develop tightness and tension in the thoracic spine, and neck.  Those who sit at work or at home at a computer for many hours a day can also develop muscular tension in the neck and back, as well as tightness in hip flexor muscles and hamstrings.  A good way to think about this is that over a long time sitting in a chair, your body tends to become “chair-shaped,” i.e. short and tight hamstrings and hips.  If that’s not scary enough to imagine (a chair-body), the ugly truth is that muscles don’t live in isolation from their neighbor muscles.  The entire soft tissue system (muscles, ligament, tendons and fascia) is all interconnected.  So a tight, knotted muscle in one area very definitely affects muscles, tendons, etc. both upstream and downstream from it.

This is why a knot in the middle of your back can cause pain all the way up into the back of your head.  Or in my case, this explains why spending a few minutes rolling the bottom of my foot around on a lacrosse ball can help me loosen up my ankles and calves.

So why do we care?  We care because it isn’t just that pain in one area leads to pain in another, but muscular weakness (resulting from those painful knotted tissues) leads to imbalance and weakness elsewhere, which leads to improper movement.  Somebody famous once said, “The body does what it does perfectly.”  So if the body moves imperfectly, it then perfectly continues to move imperfectly.  And if we, for instance, go for a walk or add resistance to imperfect movement, we solidify that pattern in our brains, and become nicely set up for injury…and more pain!

So what’s a (tight and knotted up) body to do? Well, before asking the body to work, it is nice to do whatever you can to lengthen and unknot the muscles that need attention.  This does not have to be a long process, and you don’t need to spend 30 minutes on your foam roller, thus using up your workout time allotment.

I have three favorite tools that I use for this purpose.  First, the aforementioned lacrosse ball is a must for my feet, and occasionally for very tight knots in my back.  If a lacrosse ball is too hard (and you will definitely know this), you can start with a tennis ball and move to a harder ball as your tissue gets more pliable and your nerve endings get used to the process.  For my feet, I simply stand up and put one foot on top of the ball and roll it around.  You will automatically gage how much weight to put on the rolling foot (by how much pain you can tolerate).  When I started this process, I could barely tolerate any pressure—I have very tight feet. But I persisted, 5 minutes every day.  After a couple of weeks, I didn’t mind it at all.  Now this actually feels good.  I don’t usually do this at the gym.  Instead, I have found that the easiest time to do it is when I do my treatment every morning.  It’s like giving myself a foot massage!  I also keep a lacrosse ball in the bathroom.  You figure it out.

The second tool I use is the foam roller.  You’ve probably seen these around.  Most are about six inches in diameter and about three feet long.  They are made of Styrofoam, and have lived in the gym scene for years now as a favorite instrument of torture.  I say this because they are used for “self-myofascial release,” a process that is slightly uncomfortable, especially when it is really needed.  I roll my calves, hamstrings, gluteals, and if I’m really feeling masochistic, my quadriceps over such a roller.  You will know what you need to roll by how painful it is.  The more it hurts, the more you need it.  You simply modify the move to a tolerable level of pain by taking some of the weight off the body part being rolled.  I generally spend five minutes or so rolling as the very first part of my workout.

Tool number three is a very expensive, intricate and complicated instrument.  Kidding.  It’s called a “peanut,” and is comprised of two tennis balls duct taped together to form a peanut-shaped duo.  This handy little tool sets my thoracic spine back to where it’s supposed to live every single day.  The maneuver is simply to place the peanut on the floor under your lower ribcage, and lay back on it so that the groove in the peanut is directly under your spine.  This places each ball of the peanut perfectly under the bundle of muscles that lie adjacent to your spinal column.  You simply roll back and forth on the peanut several times in the position, then move it up the spine about an inch and repeat.  Moving this way all the way up to the level of the big protuberant bone at the bottom of your neck takes only a couple of minutes, and is one of the best things you can do for knots along the spine as well as mobility of the spine (to be discussed in a later post).

That’s it!  Soft tissue rolled out…now it’s time to move on to mobility work.

Related Posts:

• Step Five of the Perfect Workout: Strength Training
• Step Four of Perfect Workout: Movement Preparation
• Step Three of Perfect Workout: Corrective Exercises
• Running From Depression
• How to Structure Your Weight Lifting Routine

Living with an unpredictable illness is not all mirth and laughter, though.  Some aspects of getting older are a bit more frustrating, at least for me.  This is where Tonic, a new iPhone/iPad app, enters my story.  As you probably know, staying healthy with CF requires more and more effort with age.  There are more medications to take, more treatments to do, more effort is required to stay fit, to eat well, to manage CFRD, to get enough sleep, enough water, to do whatever it takes to make the plumbing system work well, remember appointments for the doctors and port flushes, to do…pretty much everything.  Yet, just as the “CF care complication” factor increases, the ability of the mind (mine, at least) to keep track of it all begins its downward descent.  This is poor design, but it is what it is.

I noticed it first with Advair.  I can never remember if I have taken it.  I don’t know… I seem to have a mental block.  The block then spread to enzymes, vitamins, children’s names, books I’ve read, etc.  While it’s much cheaper to be able to read the same book (and be entertained) multiple times, too many shots of ProAir or too many Pancrelipase capsules can be problematic.

I have officially dubbed this past winter, “the winter from hell.”  For some reason, I had three episodes of pneumonia with resulting rounds of home IV antibiotics over the span of eight months.  If this wasn’t enough, I cultured MRSA recently, so each round of IV’s included three different medications, all through IV access.  IV meds must be refrigerated of course, and when they come in Entermates (as two of mine did), they need to be removed from the refrigerator a few hours before being used.  When you forget to do this, 1) brrrr, and, 2) the infusion takes forever.  Not to mention that during exacerbations, I do three treatments per day, try to sleep, try to remember to eat (when I have no appetite), and take the usual oral meds, supplements, and vitamins.  I also have follow up appointments with doctors and need to get to the lab for blood draws three times each week.  Did I mention the port dressing changes? I literally needed a flow chart to traverse through my day, or I would forget an essential item.  Of course, most people get admitted to the hospital for this rather intensive treatment time, where, at least in theory, they keep track of these things.  Wink.  But I hate hospitals, and only go in if it is absolutely mandatory.

Enter Tonic.  As luck would have it, I was able to beta test Tonic this “winter from hell.” Tonic is an app that helps you to remember and track whatever you need to track.  Setting up Tonic is a simple task.  You simply make a list of all that you want to remember and track.  It took me about 30 minutes because my list of “tonics” was enormous.  When it was done, though, my trusty little iPad would bleep whenever I had to do something.  Take out med (bleep).  Infuse this (bleep).  Take this (bleep).  Eat (bleep).  Weigh yourself (bleep).  Eat more (bleep).  Trust me, the thing was bleeping all day, every day of each exacerbation.  But I was so completely on track!  I missed nothing.  Nor did I do or take anything more than I was supposed to, because you check off each “tonic” as you do or take it.  This is a very satisfying property of Tonic for anal compulsive people who like checking things off lists (like me).

Second, remember that the idea is to make your life easier, not to add another chore to your day.  When my life is so complicated that I really need help, I use Tonic.  But other times (like now) when things are pretty good, I let Tonic fade somewhat into the background.  It still reminds me, but I turn off the “bleep” alarm and don’t always check off all the activities or enter a lot of data.  You will find your own balance.

In summary, Tonic is just what I needed at just the right time.  I would recommend it to anyone, whether you have a complicated regimen like mine, or just want to track a few things.  If you decide to give Tonic a try, let me give you two hints.  First, use it for all of the things you do for your health.  Everything. Really.  Meds, sleep, exercise, nutrition, water intake, everything you can think of that you know you want to do to be at the top of your game.  For instance, Tonic even reminds me to journal, to stretch my hamstring muscles, and to meditate.

You can find Tonic on the web at http://www.tonicselfcare.com.

Related Posts:

• Step Four of Perfect Workout: Movement Preparation
• Step Three of Perfect Workout: Corrective Exercises
• Step Two of The Perfect Workout: Just Move It
• Step One of Perfect Workout: Rolling
• Why I Hate CF

After living 50 years as a fitness nerd, I have a few ideas about what constitutes a perfect workout for me.  Of course, this all depends on which “me” shows up to exercise that day.  Is it the “feeling great” me, the “getting back up on my horse after a round of IV’s” me, or the “not exactly sick, but not feeling at my peak” me?  The right workout for each situation will be vastly different, but the basic constituents of the hour or so at the gym are the same.

Is there a “perfect workout” for you?  Yes, but I guarantee it is unique to you, and unique to you on this particular day.  From the 10,000 ft point of view, the “perfect workout” is the one that you will do, consistently, and if not enjoy, at least not abhor. It should leave you feeling tired in a good way, so that you know you did some work, but not so exhausted that you dread the next encounter with your inner athlete.  And if you live with CF or some other chronic illness that waxes and wanes, the perfect workout is a moving target. Some days, 20 minutes on the elliptical is the right amount, while other days, 5 minutes on the stationary bike is what your body needs.  On really awesome days, a 5-mile hike in the woods fits the bill perfectly.

But whatever state your body is in, the components of each workout should be the same, modified to suit your body, with its particular issues.  Each component is important, and the order that you complete each component matters.  I’m going to run briefly through each, and offer some suggestions for specific exercises that work for me and might also benefit you.

The order is as follows:

soft tissue work

mobility exercises

corrective exercises or “pre-hab”

movement preparation or active stretching

strength training

cardio or “metabolic conditioning”

stretching (passive)

nutrition

Yes, I put nutrition in there at the end, because at least for me, it is vitally important to feed my muscles nutritious food including both carbs and protein very soon after exercise.  Experts say a ratio of 4:1 carbohydrate to protein is what you should shoot for.  You can do this very easily by drinking some chocolate milk, or eating a peanut butter sandwich.  It doesn’t have to be complicated, but it does have to happen.

So, let’s go through the actual workout.

Soft Tissue:

This consists of a:bout 10 minutes of foam rolling or using a tennis or lacrosse ball in areas of muscle and connective tissue that is excessively tight.  You know an area needs to be rolled when it is uncomfortable to do so.  If you don’t feel discomfort, you are good to go on to the next spot.  I generally start with a ball to my feet (ouch), and then go to the foam roller for calves, hamstrings and glutes.   I then flip over and do my quadriceps.  Then I take out the magic “peanut,” my extremely complicated and expensive device that I now absolutely cannot live without.  With this, all my tension dissipates from my back, as it remembers how to extend after my day of sitting, coughing, and typing. That’s it!

As I said, I’m fifty.  I have accumulated a lot of tight areas.  You may not need this much, or you may need more.  Only you will know, by trial and error.

Mobility Exercises:

This is also not complicated and we are talking about joints here. Having good mobility simply means that you are able to take each joint through its natural range of motion.  Each joint is different, of course.  The knee joint shouldn’t be able to traverse a circle, while the ankle joint should (ha…tell that to my ankles!).  Take a survey of your body.  You will be able to tell which joints are tight.  Work on those.  Also, do some range of motion in the joints that you intend to use in your workout.

Corrective Exercises:

These are also referred to as “pre-hab” exercises, presumably because if you do them, you won’t ultimately require “rehab” exercises.  Simply put, the idea is to strengthen weak areas that contribute to unhealthy movement patterns or poor posture.  In my case, and likely in the case of anyone with CF, this is primarily my thoracic spine.  Lung disease and chronic coughing cause the biomechanics of the chest wall to get messed up (to use a technical term).  The result is the “hunched” back and rounded forward shoulders we commonly see in each other.  Corrective work for this focuses on opening the anterior chest and shoulders with active stretching, and strengthening the muscles of the back that pull the shoulder blades back and down.

Movement Preparation (AKA active stretching):

This is the “warm up” part of the workout.  The goal for this portion of time is to actively work the areas of your body that you are about to engage.  You slowly start asking more of the heart and lungs as you begin using large muscle groups in a similar way to what you are about to ask of them.  For example, if this is a leg workout day, you might begin with some lunges, or body squats and add in some walking hamstring stretches.  If you are going to focus on bench pressing, simply pressing a very light weight for a few sets of 5-8 would be a great warm up.  If you are going for a walk or jog, beginning to do that exercise at a slow rate for a few minutes is the way to go.  It all depends on what you plan to focus on that day.

Strength Training or Cardio (metabolic conditioning):

I put these in the same category because I would suggest focusing on one or the other during a workout.  You can do this by alternating lifting days with cardio days. Alternately, you can do both at the same time by doing weight training in a circuit fashion, with little rest between exercises.  My favorite way of doing this these days is with kettlebells, which I will discuss in another article.

The main thing to remember here is to start small and slowly progress as your body adapts to the challenge.  If you want to be able to run a 10K, that is awesome, and you can do it!  But start with walking/jogging intervals which feel like work, but also feel good!  There is no better way to sabotage yourself than to rush your body faster than it can go.  How will you know if you are?  You won’t want to keep doing it.  When you start dreading your daily jog, you know you are pushing too hard.  As you very slowly start adding time to your workout, or lesson the periods of rest (if you are doing intervals), you get stronger and stronger.

Stretching (Passive):

Ah…this is what you’ve been waiting for.  The end of the workout!  You’ve done your last set, or run your last interval.  You want to grab your stuff and go fall on your couch.  But wait!  There’s more…

This is the time to do just a little bit of flexibility training.  Your muscles are warm and pliable…a perfect set up for some passive stretching.  Passive stretching just means holding a muscle in the stretched state for about 20-30 seconds.  This is what we normally think of as stretching. Spend just 5 minutes stretching those areas on your body that tend to be tight.  You don’t need to go through a whole yoga series here.  You know what you need.  I almost always need low back and hamstring stretches at this point.  I also find that this is the perfect time to lay across the foam roller lengthwise (so it is under my spine from my head to my butt) and open my arms to the side and let gravity open my chest.  Some deep, meditative breathing in this position is the perfect way to conclude the workout.

And before you hit the couch, don’t forget to eat!

Related Posts:

• Control Trumps Fear When it Comes to Adherence to Exercise in Cystic Fibrosis
• Step Five of the Perfect Workout: Strength Training
• Step Four of Perfect Workout: Movement Preparation
• Step Three of Perfect Workout: Corrective Exercises
• Step Two of The Perfect Workout: Just Move It

Well, it has been a very long time, hasn’t it?  You may wonder where I’ve been…why no words of wisdom from the now 50-YEAR-OLD Julie?

The reason is that it has been a winter from Hell, and I will just leave it at that because my mother (RIP) always told me that if I didn’t have anything nice to say, I should just say nothing.

But, here I am, ready to begin this strange past-time of blogging again, wondering if anything will fall out of my brain.  I decided while on my walk today that I will begin by doing what is easy: posting what I have already written.  Lame, I know, but let me explain.

Two years ago, I decided I knew exactly what would be helpful to other adults with CF, and I set out to write it up.  I fondly titled this project my “CF Wellness Boot Camp.”  The idea stemmed from the fact that most people with CF, and certainly all adults with CF, are increasingly thrust into what I like to term “exacerbation exasperation.”  Say that five times as fast as you can.  You know the game:  you go about, living your life, doing what you do, feeling as good as you feel, and then WHAM, you are sick, need IV antibiotics, and essentially life must go on hold.  Your body-your master- revolts, and you are its slave.

Three weeks later (and can I just get a hand here for Western medicine?) you are better.  Your lungs are clear-or as clear as they get.  You now have enough energy to shower.  You look at your desk, your kids, your spouse/parter, your dog(s), your list of everything you were supposed to do back on the day before the aforementioned body revolt, the scale now reports that you are five lbs lighter…  You take this all in, and the only thing you want to do is crawl back under the covers.  Does this happen to you?  It’s all so overwhelming, this re-immersion into your life.  Whatever fitness progress you made before your illness is gone.  The stress of being completely knocked down is replaced with the stress of getting up.  At least, this has been my experience.

So, the plan for the Boot Camp was to outline a three-week plan (everything seems to come in blocks of three weeks) to begin anew and re-enter the world with some new, healthy habits to accompany those pristine (?) lungs.  So I put on my wellness coach hat and began to write.

This was quite a project for me.  I wrote for a couple of months until I was happy with the content.  I then began to research how to make it into an e-book, put it on the website, and, generally, do all of the technical stuff that one must do in such a project.  Roadblock.  Big time.  Julie is not “tech-y.”

Thank God for David Mahoney, though, because he really tried to help me.  I was just not able to keep the ball rolling, and the project sat for two years, lost but not forgotten, on my hard drive.

So that brings me to my walk this morning.  I want to blog again, so why not start by posting my 21-day plan?  Maybe when it’s all up, I’ll figure out how to bundle it into a pdf and send it out instead of the fizzled out newsletter promise in the opt in box?  Who knows?

So, as my favorite email come-on’s say, watch your inbox (for those who have opted in)!  Tomorrow we begin the CF WELLNESS BOOT CAMP!

To your health….

Related Posts:

• Step Three of Perfect Workout: Corrective Exercises
• CF Wellness Boot Camp – Introduction
• Step Four of Perfect Workout: Movement Preparation
• Step Two of The Perfect Workout: Just Move It
• Step One of Perfect Workout: Rolling

When you are addicted to weights as I am, those weeks with a PICC line can be a pain the the butt, and psyche.  In order to not get completely depressed (i.e. to stay sick-but-getting-less-sick and happy), I always at least walk every day, and then as the magic juice starts working and I feel better, I’ll add in resistance work for my lower body. So I’m starting to add in some video blogs because a) they are easy to do, and b) it is much easier to demonstrate exercises than to describe them in words.

Here is the first of this weeks focus videos–LOWER BODY TIME.  Today is the squat, a very basic exercise that targets multiple muscle groups.  Go for it.  Add weight if you can (hold dumbbells at your sides).

Related Posts:

• Another Great PICC-time Exercise: The Lunge
• Step Four of Perfect Workout: Movement Preparation
• Step Three of Perfect Workout: Corrective Exercises
• Step Two of The Perfect Workout: Just Move It
• Step One of Perfect Workout: Rolling

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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Related Posts:

• Mindfulness-Based Stress Reduction for People With Cystic Fibrosis
• Inner Strength
• Engage Fully in Life
• Step Four of Perfect Workout: Movement Preparation
• Step Two of The Perfect Workout: Just Move It

Here, for example, are a few random things I hadn’t planned on:

1) Hot flashes and menopause:  Isn’t it weird that every time I put on my therapy Vest, I have a hot flash?  I don’t think they were designed with this in mind.

2) Wearing hand me down jeans that used to belong to my son:  It’s true.  My 12 year old son is now giving me his outgrown jeans…and they are too big.  I’m trying to grow into them.

3) Forgetting whether or not I have actually done pretty important things:  Did I take that pill?  Did I inhale Advair?  ”Honey, did you see me inhale this?”  This is truly frightening.

4) Wondering with fear and fascination what will happen if I actually outlive my disability payment:  I don’t think the insurance company was expecting this either.

5) Not being able to see whether the needle is actually going to hit the tip of the  Colistin vial:  Are they making that bulls-eye smaller, or is it just me?

6) Getting so used to the ringing in my ears, that is seems like part of the radio background:  Oh, the years and years of tobramycin….

7) Routinely wondering if it is possible to lose one’s colon down the toilet:  Ok, this is a bit graphic.  I don’t know what the magic number of hours logged will be, but at some point, don’t you think gravity is going to win?

8)  Getting too “old” to run (read: low back and knee pains):  I thought the lungs were supposed to go first.

9) Making more cracking and moaning sounds getting out of bed in the am than my 16 yr old border collie as we hobble to the kitchen to make coffee.

10) Wondering if I might outlive yet another dog:  I don’t know which to wish for.

11) Living long enough that those foolish years of laying out in the sun on aluminum foil  lathered in baby oil has resulted in my wrinkles having wrinkles:  Who knew that shins could get wrinkled?

12) Needing a screening colonoscopy:  Of course, if we wait long enough (see 7 above), we can probably just examine it directly:-)

Related Posts:

• Step Four of Perfect Workout: Movement Preparation
• Step Two of The Perfect Workout: Just Move It
• The Perfect Workout
• CF Wellness Boot Camp – Introduction
• Almost Six Months Later….

This one was by Joseph Goldstein, who is a Buddhist mindfulness meditation teacher. I do this a lot these days.

Mr. Goldstein must have been reading my mind…that’s all I can say.  I was inwardly lamenting the fact that this walk was the first I had moved my butt in four days, as I had come down with some weird virus which seemed to have settled smack in the middle of my left lung.  Not only did it hurt to breathe still, but my scheduled Day 1 this week of a research study where I would take an exciting new drug was definitely looking unlikely.  Poor me… And the Packers lost, to boot.

Then, I heard a story about Henry David Thoreau.  Why was a Buddhist teacher talking about Thoreau?  Well, that is a long story, but in short, the podcast was about contemplating things that would “turn the mind toward the Dharma.”  Basically, it was a very good talk on impermanence.  But I digress…back to Thoreau.

It turns out that Thoreau died at 44, of tuberculosis.  I’m thinking he probably had a bit of chest pain, among other things.  In the podcast, Goldstein quoted Thoreau as saying something so cool that I came home and googled it immediately.  Sure enough, it looks like the statement ascribed to Thoreau was written  by his sister in a letter to a good friend,  telling of Henry’s life, illness, and death.  Thoreau was apparently a very vivacious man, as alive in illness as he was in health.  As his sister writes, “he remarked to me that there was as much comfort in perfect disease as in perfect health, the mind always conforming to the condition of the body.”

Perfect disease…what a concept.

Later in the letter, Thoreau’s sister, in talking of her brother’s attitude about his illness, she says that in response to a friend who said as a way of consolation, “Well, Mr. Thoreau, we all must go!” Henry replied, “When I was a very little boy I learned that I must die, and I set that down, so of course, I am not disappointed now.  Death is as near to you as it is to me.”

Now you know how this made it into a talk on impermanence.

But still I come back to idea of there being comfort in perfect disease… the secret being in the mind conforming to the condition of the body.  I think that means acceptance of what is.  Pretty simple…if not necessarily easy.  So now I’m going to try to quit feeling so sorry for myself:-)

Related Posts:

• Mindfulness-Based Stress Reduction for People With Cystic Fibrosis
• Mindful Shaking
• Engage Fully in Life
• TOP FIVE STRATEGIES FOR LIVING WELL WITH A CHRONIC ILLNESS

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?

Related Posts:

• Step Three of Perfect Workout: Corrective Exercises
• Step One of Perfect Workout: Rolling
• CF Wellness Boot Camp – Introduction
• Almost Six Months Later….
• The Beginning of the End?

If you can be cheerful, ignoring aches and pains,

If you can resist complaining and boring people with your troubles,

If you can eat the same food everyday and be grateful for it,

If you can understand when loved ones are too busy to give you time,

If you can overlook when people take things out on you when,
through no fault of yours, something goes wrong,

If you can take criticism and blame without resentment,

If you can face the worlds without lies and deceit,

If you can conquer tension without medical help,

If you can relax without liquor, if you can sleep without the aid of drugs,

If you can do of all these things,

Then you are probably the family dog.

Related Posts:

• Step Four of Perfect Workout: Movement Preparation
• Step Two of The Perfect Workout: Just Move It
• The Perfect Workout
• CF Wellness Boot Camp – Introduction