Thoreau on Illness

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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?

Inner Strength

If you can start the day without caffeine or pep pills,

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.

Posture For the Sick and Happy

A few days ago, I uploaded the above video to YouTube.  I think posture is an incredibly important thing to think about when living with a pulmonary disease, so I thought it deserved a blog post.

Think about it:  When you have cystic fibrosis, or any other pulmonary disease, every single alveolus is precious (“alveolus” is medical speak for the tiny little air sac that, together with it’s millions of comrades, comprise the lung and allow for oxygen exchange–I like to think like a doctor sometimes).

As we get older, (happily, we all are now, aren’t we) there are two forces working against our lungs–gravity, and CF.  We tend to think that we have little control over either, but we do!  I write all the time about how we can positively influence our health by controlling what we can about CF.  We can do our treatments.  We can eat nutritiously.  We can exercise religiously.  We can get enough sleep.  We can make sure we go to all of our clinic appointments….etc.

Today, my focus is on how to control gravity!  Really.

Now mind you, I like gravity.  It does many very positive things!  It would be quite a chore to sit here and type without the assistance of gravity.  But, gravity can wreak havoc on your body if you don’t learn to use it properly.

Huh?

Our bodies were designed by a genius(es…who knows?).  The bottom line is that our bones, muscles, tendons and ligaments all start out aligned to oppose gravity perfectly…until we screw it up.  As I sit and type right now, my shoulders are rounded, my upper back is hunched over my computer, and my chin is jutted out over my chest.  I know that’s sort of a scary image, but stick with me here.

Look around.  Isn’t just about everyone assuming that position?  It doesn’t just happen when typing or sitting at a computer all day.  We gravitate unconsciously to this position  when we play video games (watch your kids do this for a good shock), when we drive, when we play poker, when we slouch on the couch, you name it.  It happens as we rush from one thing to the next.  Isn’t your chin usually the first thing to enter the room?  There are opportunities for this posture all day long!  Over time–and not that much time– our default position consists of forward rounded shoulders, hunched over upper backs, and forward jutting chins.  Compensating for all of this often comes a sway-back position of the lumbar spine.  Suddenly, gravity is our arch enemy.

When you throw your body into this position, the muscles, ligaments and tendons  of your back and neck HAVE to work overtime to simply keep you upright.  These poor muscles become chronically overworked…and they let you know it.  Slowly, the muscles of your upper back become stretched to a position that is not optimal, and they are thus weakened.  At the same time, the muscles of the front of your shoulders and chest, low back and hip flexors (remember that sway back thing?) becomes tight and shorter than their optimal length, thus weakened.  So, front and back muscles are weak, and working over time to keep you from falling on your face.

Ok, now throw in a chronic cough.  Does your back and chest wall  go into spasm just thinking about this?  Now you understand REASON ONE for establishing good posture when you have CF.

Now for REASON TWO:  Conjure up that image again, the one of the rounded shoulders, and slumped upper back.  Do you think it is possible to take a full breath using all available lung tissue when in this position?  Not a chance.  You  can use most of your upper lungs when you are collapsed that way.

It is estimated that poor posture can rob you of __% of lung tissue.  Now, I don’t know about you, but I need every bit of my lung tissue with every breath I take.  I can’t afford the improper effects of gravity1

So watch the video, and try to incorporate at least one or two of these exercises every day.  They aren’t hard, and they don’t take much time.  They will slowly work to strengthen and shorten those overstretched back muscles, and stretch and strengthen those tight chest and shoulder muscles.  The result will be that you will be able to pull your shoulder blades back and down, thus opening your chest and allowing for full expansion of your lungs.

The next trick will be actually remembering to do this!  I have some tricks for this, too.  Watch for my “mindful breathing intervals” in a blog post coming to you soon!

Five Reasons You Must Start Resistance Training Today!

I love list posts.  They are so easy to write, and even easier to read.  If only adopting the habit they propose were so easy…

But in this case, it is!  Resistance training is not difficult to do.  You don’t need to join a gym.  There is no requirement for fancy equipment or expensive clothing.  While a routine does take a little bit of time, you will begin to see and feel significant results in as little as 20 minutes 2 or (ideally) 3 sessions per week.  You could multitask, and do your routine while watching Scrubs reruns.  How simple is that?

Your own body weight can provide all the resistance you want or need, or if you are so inclined, you can purchase some very reasonably priced resistance tubing to use in your living room.

Here’s the trick.  Don’t fall for the fitness magazine articles that suggest complex moves, or drop sets, or supersets, or unbelievably crazy-sets.  Pick exercises that target multiple muscle groups like squats, lunges, front and side plank, or good old fashioned push-ups, and just start doing them!  Here is why you should start today:

Reason 1) Resistance training is a friend of your metabolism.  Why is this?  As you begin to overload your muscles beyond what they are used to, you injure them slightly (don’t go for major injury…that doesn’t do any good at all).  You cause little tiny microtears in the muscle fibers, and this is why you are sore one or two days later.  But this is good news, because as your muscle fibers heal, they become stronger and bigger.  You add muscle mass, and over time, this increases your metabolic rate.

How does that work?  Body fat doesn’t do much.  It just sits there and looks back at you in the
mirror.  It doesn’t use up much energy.  Heck, it doesn’t even need much of a blood supply since it requires so little maintenance.  As a result, it burns very few calories.

On the other hand, muscle is very active.  It requires food (glucose and amino acids) and burns tons of calories by just being there.  Clearly, if you want to be a lean, mean, calorie burning machine, you want as much muscle as you can get.

Reason 2) Muscle, because it requires glucose and amino acids, is very sensitive to insulin.  Insulin opens the doorway to  to the little muscle cells, so glucose and amino acids can get in.  If you are insulin resistant, as in Type II diabetes  (and possibly CFRD), lifting weights will increase your insulin sensitivity as you build muscle mass.  A finely tuned insulin sensitivity mechanism is required for a stable blood glucose level, which leads to good health.

Reason 3) This is a big one for me, and maybe you can relate.  Building muscle and feeling and being strong physically is one area of my life where having cystic fibrosis doesn’t even matter!  My lungs may not be the best in the gym, but I will take on any woman my age in a push up or pull up contest!  This is a very empowering feeling…I have at least a modicum of control over my body which is otherwise at the mercy of my lung status.  Now, some days my lungs even interfere with my time at the gym, and that is OK.  I know that when I recover, I will be back, strutting around the gym with the big boys, knowing that my muscle fibers are no different than theirs:-)

If you have an illness other than CF, lifting may just provide the same benefit.  Lifting weights is a very black or white thing to do.  You do it and you see and feel results in as little as two or three weeks.  You have control of this.  It may not feel like you have control of much else, sometimes.  But you do have control over this.
Reason 4) More and more studies are showing that well-designed resistance training programs in post-treatment management of cancer patients and survivors are beneficial in improving health status and quality of life.   This is true in other chronic diseases as well.  Weight training is anabolic, meaning it builds up the body.  Often, treatment for illness is catabolic, or breaks down the body (think steroids or chemotherapy).  While these treatments are necessary, we can counter their bad side effect of breaking down tissue by weight training.

Reason 5) Weight training is fun!  Ok, maybe I’m in the minority thinking this, but stand by this statement.  When you get over the initial “I have no clue what I’m doing,” and move through the “Oh my God this huts,” you begin to see improvement!  And this is fun!

Are you ready to begin?  I’m starting a YouTube channel where I will teach easy, and very modifiable exercises that anyone can start doing today.  Check it out, and subscribe today!

The Gift of Giving

I have recently taken on a new challenge.  I love challenges!  THis morning I was sent an email link to the most fantastic site, called the 29 Day Giving Challenge.  It was started by a young woman with multiple sclerosis, who found happiness (check out her video on the home page) and abundance through the simple act of giving a small gift to someone else every day for 29 days in a row.  This has erupted into a huge movement of giving by thousands of people across the world!  Imagine.

So today is Day 1.  I just joined the Global Giving Village, as one of the 29 Day Giving Challenge member suggested.  I think I’ll update my giving here, to keep me honest.

You know, having CF is no picnic.  But when I check out these organizations and what they do, it reminds me first, that I really have so much to be grateful for, and second, that the quickest way to happiness is to give to others.  Of this, I am certain.

Now….off to the pharmacy for the third time this week!

THE TRYING, I MEAN ADOLESCENT, YEARS

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

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

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

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

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

More on positive reinforcement later.

Make it Social

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

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

Weight Training

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

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

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

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

Make Use of Multitasking

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

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

Defer to the Coach

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

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

Bribery

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

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

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

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!

CF and Time

I am researching for a talk I will give to a CF Education Day in a couple of weeks, and found this article that I wrote three years ago.  The talk is going to be about exercise, of course, and how to find both time and motivation to move when you live with CF.  But re-reading this made me slow down a bit (hard to do on Prednisone), and remember what this time thing is all about.  I hope you enjoy: CF and Time As a fellow anatomic pathologist, I fully respect Dr. Dorothy Anderson’s description and naming of the constellation of signs and symptoms that we now call Cystic Fibrosis back in 1938.  Her observations marked the beginning of several decades of medical research, the outcome of which allows me  to sit at my computer at age 45 and write this. But for now, I would like to think way, way outside the box, and ask a question posed by one of my favorite spiritual teachers, Eckhart Tolle.  Does chronic disease really exist?  Does CF really exist? In “The Power of Now”, Tolle differentiates your “life” from your “life situation”.  He speaks of your “life” as this very ”moment”, as opposed to your “life situation”, which is comprised of the circumstances or conditions of your life.  As such, your life situation is your “story”, which has a beginning, middle, and eventually, an end.  It exists on the horizontal axis of time.  However, there is a part of each of us that is unchanged over time and exists outside of time.  Think back to that day you learned to ride a bicycle.  Or think of throwing that high school graduation hat into the air with a big cheer.  Or perhaps, think to the moment when you said, “I do”….  In each of these situations, there was an awareness of what was happening, and that same awareness, unchanged, is aware that you are reading this page now.  That awareness is what Tolle would call “life”, and it does not exist in time.  It is NOW.  It does not change, because it lies on a “vertical” axis, with no past and no future. Try not be offended by this (many of us are very attached to our CF), but imagine for a moment the possibility that CF is part of your “story”, existing on the horizontal axis of time.  At some point you were diagnosed.  In other words, a combination of sounds emitted from your doctor’s mouth (copied from the sounds Dr. Anderson decided best described this disease) was suddenly ascribed to YOU.  Those sounds became part of “who you were”.  The beginning of your CF story may have been when you were a baby, or later, but part of your identity was now as a “sufferer of” CF. The story then continued, with a different trajectory for each of us.  For some of us, the story has already ended. Your story isn’t just CF, of course.  There is the story of what you do for a living, how your body has grown and changed, what you have learned over time, who you know, who is in your family, how many dogs you have loved and lost….If you think about it honestly, whenever you ask yourself the question “Who am I?”, the answer is usually just more of the story.  “I am a lawyer”, you say.  Then who were you before you got your JD?  “I am a father and husband”.  Who were you before you were married?  “I am sick” really means the physical part of “you” is not completely healthy at this point on the horizontal axis of time.  Yes, your body has weird and unusual chloride channels lining its epithelium.  But is your brain “sick”?  Is your heart “sick”?  Are your bones intact?  Can you see?  Are you breathing? Can you love? “I” (the author) am a 45-year-old female “sufferer” of CF, retired physician, mother of two, partner, wellness coach, friend, daughter, sister…  Yeeeees, but other than being “female”, all of that has been variable throughout my life (and as I understand it, even being female could be changed, if I wished…).   Who was I when I was 9 months old, and had no language yet? But, you argue, my brain is the same…!  No it isn’t.  Neuronal pathways are always changing; cells are dying; plaques and tangles are forming (at least, in our “old survivor” brains).  I’m sure you’ve heard that all of your cells are dying off and regenerating constantly. Nothing is constant on the horizontal axis of time.  The “life story” is ongoing, and ever changing, just as the body is. Do you see the point of this tirade?  A part of you, and I would argue, the ”real” you, is the observer of your story, or as Tolle would say, “the awareness” within which your story unfolds.  Does that “awareness” have CF?  NO!  The body in which the “awareness” resides has CF!  So does CF really exist?  Only in time!  Only in the content of your life, the content that always changes and that describes you, but is NOT you.  The content is your life story, but is not your LIFE.  The real you is life itself, and that life is now, this moment.  “CF,” then, in this moment, is really reduced to what you are actually experiencing now.  Maybe that is a cough.  Maybe it is rapid breathing.  Perhaps it is pain in your joints as you walk.  Or, possibly, in THIS MOMENT, it is nothing at all. “Wow,” you say, “Julie has completely lost it!”  But if it sounds kind of interesting to explore the idea of “this moment” where CF possibly doesn’t even exist, maybe you are asking, “How do I get there?” I’m glad you asked.  This is the cool part.  Tolle describes “portals” into the NOW.  Entering these portals is sort of like a meditation, yet it is not meditation in the way we usually think of it.  I don’t know about you, but when I focus on my breath, I do NOT enter a state of bliss!  These portals are much easier to use. One portal, my personal favorite, is to become aware of the “inner body”.  Another way to describe the inner body is your “life force”, or “life energy”.  To do this, you simple sit or lie in a comfortable place, close your eyes, and ask yourself without moving it or looking at it, “Is there life in my left hand?”  This may take a few moments.  You probably will be tempted to move your hand, but don’t.  Just sit or lie in stillness and find out, “How do I know that my left hand is there…without looking at it or moving it?”  After awhile you become aware of the energy of your hand.  That is your “inner body”.  When you become aware of it in one hand, you then move your attention to the other hand.  Then, when you can feel it there, you try to feel it in both hands at once.  After that, you get adventurous, and move to your feet, and up your legs, to your torso, then arms, then neck and head.  With some practice, you can soon, at will, become aware of the “inner body” within your entire “outer body” (the one you can see and move). Here’s the catch:  the only way to feel this “inner body” is in the NOW.  You can’t do it if you are remembering the past, or worrying about or anticipating something in the future.  You can only feel your inner body in THIS MOMENT.  This technique essentially forces you into the “NOW,” the vertical, timeless dimension called awareness.  In this space, I would argue that there IS NO CF.   There may be a cough (see if you can maintain awareness of this life energy while you cough), but the energy is separate from the cough… from the body… from your thinking mind.  It’s wild. If you get really into it, you can practice this awareness throughout the day.  You can do it during a treatment.  You can practice while folding clothes, or while walking, or while in a particularly unpleasant conversation with an ex-partner.  The more you practice, of course, the easier it becomes. There are other portals, of course.  But this is getting way too long.  I would be happy to share my experiences with them (all very legal!).  Just give me a call or email.  Until next time: BE FIT…BE STRONG…BE WELL!

Five Steps to Re-Energize

Sometimes it is easy to get bogged down on a project.  You let it “sit,” so you can think about it awhile, and before you know it, three other things have come up that need your attention, and your “big idea” starts gathering dust.

At least, that is how it often works for me.

This blog is a great example, but there have been others.  It has been a challenge to post lately.  Tom died.  Christmas happened.  I got sick.  I got busy.  Life happened.  Writing took a back seat.   In addition to writing, half-marathon training programs, book ideas, and piano lessons are also residing in the back seat.  Now don’t get me wrong…my motto for life in general––I get knocked down…but I get up again––applies to projects as well as it does to my health.  Usually I come back.  Like now, for example.

So I thought a good article to write might be one about just this:  How do you pick up where you left off, before life got in the way?  I’ve come up with a 5-step “Get Up Again” action plan to use when approaching that stack that is growing on your desk.

STEP ONE:  This is the most important one.   Get off your back already!  Unless you live alone, have no friends, have no other responsibilities, have only one interest, and generally have no life, things come up!  Life happens, and you get knocked off course now and then.  For most people I know, this is when the nasty little nagging voice speaks up.  “You are such a loser…!  Why aren’t you working on this?  You had such grand plans…such great ideas…Right.  What a lazy (%&#*!

First off, this is a true waste of energy and time.  It is, of course, much more efficient to use that energy in getting back up on the horse, to mix metaphors.  Everyone gets pulled off course, now and then.

STEP TWO:  Find your motivation!  If you are spinning your wheels, you need to get a grip on something, right?  The traction is found within something called motivation.  What lights your fire?  As much as possible, you need to recreate the energy you had when you began the project.  That’s a tall order, I know.  If I had the secret to that, I would be a bazillionairre.

Why did you want to do this project in the first place???  There must have been a really good reason.  The trick is to remember it. And get back into it!  Read about it again.  Read about how others have done or are doing what you want to do.  Talk to people about your idea.  Enlist their ideas…their help.

STEP THREE:  Set one goal.  This is obvious, but it is so overlooked.  You need a finish line.  It doesn’t have to be far away, but it needs to be a bit of a stretch for you.  It needs to be time-based and measurable.  You also need to really want it!  You need to be excited.  It helps to read the goal several times a day, imagining the feeling you will have when it is accomplished.  I know what you are thinking..”One goal?  But I have at least twenty to get back to!”  This may be true, but just pick one for now.  Just a little bit of traction goes a long way.

The most important aspect of setting a goal (to me) is setting a reward.  Seriously.  You need a carrot AND a stick.  If you are like me, the stick is taken care of.  It’s that voice in your head yelling all of the time.  The carrot is, of course, the reward you pick to give yourself when you’ve crossed that finish line.  Make the reward appropriate to the effort you need to put in to accomplishing the goal.  If you’re going to train for three months to run a 5K, give yourself something worth three months of hard training!

So let’s say, for instance, you had initiated a great workout program.  You were committed.  You had worked out all the details…and then…poof.  What program?

There are two ways to deal with this.  The usual way (for many) is to tell yourself you “don’t have it in you” to stick to a program, and then give up until the next time something wakes up your motivation again.

The second (better) way, is to get off your own back, remember your motivation, set a new and smaller goal (perhaps to just start to walk for 20 minutes a day)…add a carrot…and take STEP FOUR.

STEP FOUR:  Take a small step…every day.  Small is the important element here, especially at first.  The reason for this is that you will build on small successes.  If you do what you set out to do every day, then even if those action items are small, your confidence in yourself grows bigger and bigger.  Soon, you’ll start challenging yourself with larger daily action items without feeling overwhelmed.

STEP FIVE:  Stick to it until you can celebrate your achievement!  Your motivation may wax and wane a bit (have you noticed this?).  That’s ok…that’s just what it does.  If you have a day where you feel completely unmotivated, then make your daily action be to read about your goal.  Google it.  Find success stories.  Get your mojo back!  Tomorrow is a new day, and likely, you will feel more like playing.

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