A GIANT LEAP FORWARD?

Welcome back!

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

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

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

CF Mutations 101

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

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

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

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

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

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

Clinical Trials 101

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

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

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

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

Now, back to regular programming:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Running From Depression

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

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

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

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

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

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

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

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

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

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

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

How Important is “Quality of Life” in CF?

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

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

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

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

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

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

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

The Pros and Cons of Working Out at a Gym

I like to start an article off with a bang!

I read an article online a couple of days ago entitled,  “The Four Germiest Places at the Gym,” and it made me a little ill.  You can read it for yourself (I recommend this), but in a nutshell, you want to avoid public yoga mats, dumbbells, bike seats, and shower floors and walls.

Now I don’t know about you, but I actually touch dumbbells when I go to the gym.  Not only that, but I always have to adjust the exercise bike seat height!  Now, I can definitely avoid yoga mats (I bring my own), and I rarely (by rarely, I mean never) use a locker room shower anymore.  Still, the article got me thinking about the good and bad points of public gyms for someone with CF.  So, here’s my take.

THE PROS

1)  The first that come to mind is obvious, and the video above is a perfect example:  PEOPLE WATCHING!  Let’s face it, treadmill running, or elliptical training, or stationary cycling, or (fill-in-the-blank) for 30 minutes at 70% of your estimated maximum heart rate can be BORING.  Watching those around you, especially when they are doing unusual things, can be very entertaining.

2)  Variety of equipment:  My gym is a great example of this.  I could be there all day, and not have enough time to try every machine.  It’s mind blowing.  For an exercise nerd like me, it’s like being a kid in a candy shop!  Chest day, you say?  Well, let’s see…I can use dumbbells, or barbells, or cables, or stack machines, or take a group weight lifting class.  The possibilities are endless.

3) Vicarious experience:  If you are short on inspiration or motivation, the gym can be a perfect remedy.  All you need to do is look around.  There are always people there who can provide inspiration.  You can see yourself in others, and aspire to push yourself a little harder.  When I see a woman who can do 10 pull ups, I am both impressed and motivated to work harder, because, darn it, if she can do it, so can I!

4)  Variety:  Let’s say you get sick of your “usual” aerobic or lifting routines.  Check out the group classes!  Again, if your gym is like mine, you can choose from anything from “Zumba” (I don’t know what that is…it sounds like a soup to me) to kickboxing to group “body bar” classes to yoge to (fill-in-the-blank again).  Never even THINK of a boring exercise session again!

5)  Social connection:  Working out alone, either jogging or lifting weights in your living room, is kind of lonely, isn’t it?  A gym is a much more social experience, even if you don’t know anyone around.  There are people there!  You can talk with them, or not, but you are not alone.  You might even make friends with people there.  Some people have hooked up romantically after gazing from afar for months at the gym…

6)  Guidance:  Let’s say you have no clue what you are doing, for instance.  At a gym, you can a) watch what others are doing on the machine in question, or b) ask for help.  There are people who get paid to answer your questions.  And there are people who aren’t getting paid for it who will answer your questions.  And, of course, there are people who want to answer all of what they perceive to be your unspoken questions (but they go in the other column).  Last, if you have the cash, there are people you can hire (personal trainers) to teach to what to do and set up a program designed specifically for you.

THE CONS

1)  This one is easy.  I alluded to it earlier.  GERMS!!!  Gyms are germ havens.  Ask around, I bet you can’t find any self-respecting microbiologist at your gym.  They know better.  Now, this freaks out many “normal” people (i.e. CFTR-able).  Imagine how it might affect those with CF!  If you have a transplant, forget about it…there’s no way you should go into a gym.  So this is serious business.  I go back and forth on this, and I am addicted to my gym!  The best I can say is if you are like me, and can’t stay away from your  gym, wash your hands…wash your hands…wash your hands!!!  And until you can wash your hand after touching the machines or weights, keep your hands AWAY FROM YOUR FACE.

And avoid the locker rooms…and the yoga mats.

2)  Inconvenience:  Getting dressed, packing your bag and water, finding your keys, driving down the block, returning home because you forgot your membership card, and driving to the gym take time.  For some, this series of events take longer than the entire workout.  This is not efficient use of time, nor is it good for the environment.  The worst part is that often this series of events presents an insurmountable obstacle to the exercise itself.

3)  Being “Noticed:” This one is only sometimes the case for people with observable health issues:  When I look or sound sick (you know…”the cough”) it can be embarrassing to exercise in public.  Once I was at the end of a course of IV antibiotics, and I went to the gym with my PICC, infusing Tobramycin.  I was on the treadmill, jogging I think, and this guy came up and asked what was wrong with my arm.  “Nothing,” I responded, “I’m just getting antibiotics for a lung infection.”  I wish I had words for the look on his face.  He truly thought I was a nutcase that should perhaps be carted off in a straitjacket.

At least I wasn’t dancing.

Rant

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

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

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

Ok, enough.  I’m done ranting.

First Sick and Happy Video!

More to follow with real exercises…

Can I Exercise When I’m Sick?

Not that I’m an expert on this… The last time I had a PICC line I ended up with a DVT (blood clot) in my arm and had to be on blood thinners for 3 months.   Why?  Well, I’m not exactly sure, but it could be because I didn’t want to atrophy away, so I was doing push ups as well as my daily walk.  Dumb.

So maybe this does make me an expert because I definitely know what NOT to do.

Here’s my take:

If you have a PICC, NO UPPER BODY RESISTANCE TRAINING NOT EVEN PUSH UPS WHAT ARE YOU THINKING?!  But when you start feeling better after the first few days of “happy juice,” as I now call it, you can definitely go out for a nice walk every day.  Or get on an stationary exercise bicycle.  Basically, the bottom line is don’t push it too hard, but it is good to move! If you don’t move for three weeks, it will be very difficult to get motivated again, and you will lose muscle mass, a very bad thing indeed.  Be gentle.  Be mindful.  But, move.

If you have a fever, no exercise.  Rest.

If you are just coughing more than usual, but don’t feel too bad…well, this is a tricky one.  Are you losing weight?  Are you eating well?  Do you have energy?  This is probably a good time to call the clinic, let them know what’s going on, and ask for your doctor’s opinion on the exercise question.  Do I do that?  No (well, I ask myself and I usually tell myself to quit being a wimp and do some push ups). But I’m learning to not listen to that inner little sergeant.

Last week (Week One), was the “walk every day” week.  My dogs loved it.  I also did daily Qigong (gentle stretching).

This week, I’m adding some lower body exercises (squats) and curl ups to the above.  Feeling good!  Zero cough.  I love this happy juice.

Next week, who knows…but it won’t include push ups.

What is the Right Career?

How is a career choice related to wellness?  First, when I write about wellness with CF, I am not simply talking about physical health and wellness, but also emotional well being…a sense of contentment and fulfillment.  Many things are related to this sense of wellbeing, and fortunately, most have little to do with physical health.

I’ve been reading and thinking a lot about happiness lately.  If you’ve read my column before, you probably know that I find the field of positive psychology fascinating.  Essentially, it is the study of what causes people to be happy and to live rich and fulfilling lives.  Happiness is a popular topic these days.  You see happiness “secrets” revealed on book and magazine covers, on PBS specials, on happiness blogs, websites…you name it.

My purpose is to mine the field of positive psychology and happiness research to come up with scientifically validated ways to improve the subjective wellbeing of people with chronic illness, and of course, cystic fibrosis is a perfect example.

So what does this have to do with career choice?

The research tells us that one of the most important elements of living a good, fulfilling life, is the ability to use your strengths in a manner that serves a purpose that is larger than yourself…one that you believe in deeply and that aligns with your core values.  Those people for whom work is a calling feel the most fulfilled.  And there is a strong positive correlation between happiness and using your strengths every day.  Wouldn’t it be awesome if you could do that and get paid?  You can…and you should strive to do just that.

We all have strengths, and I’m sure you have a very good idea of what your particular strengths are.  It has only been a recent discovery that people who are the happiest immerse themselves in using their strengths rather than using their finite amount of time and energy to “shore up” their weaknesses.  I believe that the very first thing to take into account when deciding a career path is “What are you good at?”  Notice, I didn’t say, “What do you think you can handle, given CF?”

If you want to try a fun and often revealing exercise, take the VIA Signature Strengths Survey at http://www.authentichappiness.org.  This is a series of 240 multiple-choice questions (it takes 45 minutes or so), and when you are done, you will immediately see which are your top five (or Signature) Strengths.   I thought I knew what my results would be, and I was close, but there were some that completely blew me away.  You will also get an interesting perspective on your own strengths by asking those that know you well what qualities they most admire in you.  Finally, make your own list of things you love to do and that you know you do well.  Try to narrow this down into five or six things in which you take great pride and satisfaction.  Combining all of these methods together will give you a very accurate map of the kind of career you will find most fulfilling.   It will be the one(s) where you see the need and the opportunity for these strengths at every turn.

Looking back to my decision to go to medical school to ultimately “cure cystic fibrosis,” I realize that I could have used this advice.  When one thinks of a good researcher, strengths like the capacity to love and be loved, humor, zest, curiosity and love of learning, and hope/optimism/future mindedness (my top five) are not the ones that first come to mind.   A great researcher would show strengths like industry, diligence, critical thinking, caution, judgment, ingenuity, and leadership (not even close to my top five).  While my passion was in the right place (curing CF), my strengths were not suited well to this career decision.

Now, this didn’t turn out all bad.  I loved going to medical school.  My love of learning and curiosity strengths were force-fed every day for 10 years of training.  I got to tell great pathology jokes.  But let’s just say that sitting around diagnosing cancer (after the intellectual thrill of figuring it out) did nothing for my zest, my hope and my optimism.  And who loves their pathologist?  Was I happy?  Not so much.  When it came time to retire to take care of my children and myself, I went through a slight existential crisis (well if I’m not a doctor, then who am I?), but then settled into post-physician existence quite happily.

Now I am entrenched in career number two, coaching and training wellness to people, who, like myself, live in less than perfect bodies that often require care and attention above and beyond the norm.  I use my strengths in a much more effective an ongoing way, and I am appreciated for them more than I ever was sitting at my microscope.  And, I care deeply about the meaning and usefulness of my work.  I feel that I am doing what I “should” be doing.  Given that I have always had a passion for fitness, nutrition and stress management, I get a kick out of sharing this with other people, and love learning even more about these topics.  This leads to a sense of fulfillment and contentment that I didn’t feel as a surgical pathologist.

So what can you learn from this story?  First, it pays to learn your strengths and give them serious consideration when choosing your career.  The same goes for following your passions, and figuring out a way to merge your core values with your daily job.  But finally, what you can learn from this story is that sometimes, despite your best intention, your “dream” job takes awhile to manifest.

You may decide on one path, and find out later that it doesn’t work out as well as you had hoped.  Or, you may love what you do for a time, and then physical challenges may force you to be more attentive to your own health needs than that particular job allows.  All of this happens…to everyone, really.  When you are first deciding on a career, in your early twenties, it may seem like you only get one chance, and you can’t afford to mess it up.

You might be making yourself crazy by thinking, I can do this now, but what if I get sick?  Sure, be practical.  You probably shouldn’t become a firefighter!  But why paralyze yourself by imagining what may happen in the future?

Barack Obama said something in his inaugural address that stuck me (actually, most of what he said struck me…but this I remember).  He said he rejected the notion that as a nation, we couldn’t both follow our values and be safe.  To paraphrase him, I reject the notion that as people with cystic fibrosis, we can’t both follow our passions and be well.

Your career is obviously a very personal choice…one that you will live with day in and day out.  Most people you talk to will give you practical advice:  Think about your health.  How stressed will you be?  Will you be able to care for yourself appropriately?  How healthy are you now?  What can you do now?

These are obviously important to consider.  But remember also to consider the following:  What are your strengths?  What are your values?  Is it more important to you to work your tail off doing what you love, or to work at a less stressful job so that you can place more energy and attention on your own health and family?  These aren’t right or wrong questions.  They are just questions…to which only you know the answers.

Exercise for Preschoolers and Elementary School Children With CF

FIRST, THE FACTS

First of all, if there is ever an easier time to get kids to move…this is it. Just as before, encourage the notion that exercise is fun.  Being active is fun.  Playing with your family and friends outdoors (or indoors)  vigorously is fun!  The neural pathways you help your child establish now will be with them into adulthood.  They can be health promoting pathways, making an active lifestyle easy to maintain…or , they can be sedentary living pathways, which will be difficult to overcome.  Your power to promote long lasting behaviors at this stage is enormous.

While the target goal is to have your child accumulate at least 30 minutes of moderate to vigorous activity each day, remember that this does not have to happen in one session.  In fact, kids are wired to be active in short bursts, so go with that, and break up activity sessions into 5-10 minute chunks.  Go out and play hide and seek with them…or tag…or whatever makes their (and your) hearts pump and lungs work hard.  The “active time” can include swimming, soccer, basketball, biking, jumping rope, rollerblading, running/walking, jumping on a pogo stick (one of my favorites), or rebounder, or safely enclosed trampoline.  Really, you are limited only by your and your child’s imaginations.

Preschool and elementary school aged children can also build strength.  They aren’t ready to pump iron at the gym with you, but with a little creativity, you can help them strengthen their back extensors and other core musculature.  In a soon to be revealed video, I will demonstrate some easy ways to do this.

Habits are being formed now, as I said before, and this also applies to posture.   Why is posture so important to think about in cystic fibrosis?  Several reasons come to mind.  One, optimizing posture optimizes usable lung tissue.  Think about what ends up happening as we age (unless we work hard to avoid it).  Our upper backs tend to bend forward, as our shoulders roll forward from years of typing, driving, working at a bench, or whatever.  At the same time, our lower backs tend to become more “swaybacked” as the pelvis rolls  anteriorly from years of sitting and subsequent tightening and shortening of hip flexors.  The result is that the upper areas of our lungs get, to use a technical term, scrunched, and we get chronic low back pain.  Both of these are bad.  Compressed lung tissue is bad in CF, because compressed  tissue collapses and, 1) isn’t used to exchange gases and, 2) is a set up for mucus plugging and infection.  Back pain is bad in CF for the same reasons it is bad in anyone…it hurts and leads to inactivity.   It is also bad because with low back pain and weakness comes abdominal muscle weakness, and inefficient and weak cough mechanics.

So you are probably wondering how to avoid all of this.  Start strengthing back extensor muscles early, as well as all of the core muscles that surround the trunk!  I have come up with several examples of these in the aforementioned video (coming to your computer screen soon).  Many of these are exercises that adults do, but have been slightly modified and given cute names.

HOW DO I CONVINCE THE LITTLE BUGGER

Finally, a word about motivation during these fun, but sometimes difficult years.  Let’s be clear here…sometimes it’s more about motivating yourself to be consistent and unwavering about establishing exercise as a  “family value” more than it is about motivating your child to do it.  Parenting is hard (tell me about it) and can be draining.  Convincing a young child to do what you want can be like herding cats.  Sometimes “motivating” them really means “bribing” them.  Clearly, you have to pick your battles, and exercise may not be the most important thing to focus on every time.  The point is, at this age, you are aiming a moving target.  Just because a trick works one day doesn’t mean it will work the next.  You need to have a Plan B and C ready.

COMMON TRICKS, I MEAN STATEGIES

Enticement is a wonderful tool. Giving a  “reward” for doing their exercise can be extremely effective.  Pre-school kids will do the most amazing things for a sticker or a small bouncy ball.  A 5, 6 or 7 year old can be told to get 5000 steps on their pedometer (that you gave them as a reward for exercising) before they get to watch their favorite cartoon.  (I discovered the hard way that after this age, the kids quickly learn to just shake the pedometer when you aren’t looking).  An older 4th or 5th grader may gladly do their exercise if they are told that there will be no screen time until they do.

Calling exercise by a different name is sometimes effective.  Personally, I think it is a lovely word, but many people hate exercise and like to say so.  Kids overhear this and can easily associate “exercise” with pain and discomfort.  So, “Let’s go play on the trampoline!” sounds better than, “Go exercise on your trampoline.”  “Let’s pretend you are a wheelbarrow!” sounds fun!  “Let’s make your muscles stronger!” sounds like work.  “Let’s make an obstacle course!” sounds  much more exciting than “Let’s do interval training!”

Competition works well for some kids and completely turns off others.  One of my kids hates it and refuses to play.  The other will do anything if I tell him I’m going to time him.  You know your own child and if this will work.  If it does, you can have races or see who can jump the farthest or the most without missing (jumping rope).

Finally, video games that encourage movement are available and fun!  The Wii has several active games,  both the games that come with the Wii as well as Wii Fit and others that really get you moving!  Dance, Dance Revolution is another great example of this and more are being created every day.  Video games are here to stay.  I think of this with the Aikido mentality of “using the opponent’s force to get what you want.”  If you can, get a Wii or other console system, and allow the games that encourage movement.  Play them with your kid!  Not as a substitute for other exercise, but as a complement.  After all, they are fun!

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