Lower Body Time!!!

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).

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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Hot flashes, Hand-Me-Downs, and “Honey, did you see me take my ___________ today?”

I don’t know about you, but I’m getting older.  Last I checked, I was well into living my 50th year.  Now, nobody has actually ever told me, “Julie, you are not likely to live to be 50,” but having not lived in a cave all of my life, I have received this message loud and clear.  So what am I doing here?

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:-)

Thoreau on Illness

So I’m walking my dogs tonight, as I often do, while listening to a podcast.

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:-)

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?

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