Thirty years ago, I began the process of leaving my family behind, as acceptance letters to medical schools far away from my home state of Nebraska began to arrive in my mailbox. Those remaining in NE at the time included my parents and my sister, Kathy, who was dying of cystic fibrosis. My five other siblings were scattered far and wide, but my closest friends remained.
Kathy was the center of my life then, although I don’t think she knew it. In her, I saw my future, but somehow was able to keep this fact at a distance and think of it only as her disease and her death. I loved her to pieces, but I was also afraid of her…afraid to get too close and not be able to escape the reality of the horrible disease that I, too, would some day die from.
In the spring of the last year of her life, I visited her in the hospital to discuss the options that were opening up to me. She told me I needed to leave…to go to a medical school where I could do research in CF. To get out of Nebraska and find my own life.
Now I know that she was trying to spare me the sight of her withering body, her rapid decline, the horrible depression that she lived with as her body defied her will at every turn. I decided to go, accepting the offer to Stanford Medical School, and packed to leave for Palo Alto. The lyrics of a popular song back then, Old and Wise, by Alan Parsons Project, rang in my ears as I left in my Buick Skylark with my husband and dog:
As far as my eyes can see
There are shadows approaching me
And to those I left behind
I wanted you to know
You’ve always shared my deepest thoughts
You follow where I go
Shadows were approaching me, but at twenty-three years old, I barely acknowledged them. Yet Kathy’s were closer…much closer, as I abandoned her to manage them on her own. But I held her with me, and after only a few months of getting used to California, before classes even started, I returned to Nebraska to be with Kathy as she died. At thirty-one-years of age, her life was taken. At her funeral, my brother Tom, who also had CF, and I could only hold hands and understand each other in a way that the other siblings couldn’t. Our shadows were approaching.
Inexplicably, I made it past thirty-one…way past. I have lived entire lifetimes, as a picture perfect picket-fence marriage ended, followed by a less than perfect eight-year relationship which resulted in the two best things that have ever occurred, my two sons. Finally, at forty, I found my true life partner, and a crazy life of boys, dogs, and a two home relationship ensued.
And then, when I was forty-eight, Tom died. If Kathy was like a mother to me when I was young (she was), then Tom was like a caring, nurturing father. They looked after me as a kid the way my parents couldn’t. They understood me. We were the sick ones.
Now, I was the last one standing. The shadows were getting closer. The sadness was overpowering.
And oh when I’m old and wise
Bitter words mean little to me
Autumn winds will blow right through me
And someday in the mist of time
When they asked me if I knew you
I’d smile and say you were a friend of mine
And the sadness would be lifted from my eyes
Oh when I’m old and wise
I don’t know when I am considered old and wise. I’m certainly old. In CF years, I’m ancient. I do smile as I think of Kathy and Tom. It is a sad smile, of course. Their lives were stolen. They didn’t get three entire lifetimes with kids and dogs and good drugs to keep infections at bay. They didn’t live long enough to be guinea pig subjects of new medications that fix the protein that malfunctions in CF. It doesn’t seem possible for that sadness to be lifted from my eyes.
As far as my eyes can see
There are shadows surrounding me
And to those I leave behind
I want you all to know
You’ve always shared my darkest hours
I’ll miss you when I go
My body is not quite as functional at age fifty-three as it was thirty years ago when I held Kathy as she took her last breaths. My own shadows are getting closer. Antibiotics don’t work as well. Organs are getting tired of toxins. I have been given so much more than I deserve. When I think of Kathy and Tom, I feel so incredibly grateful. First, to have had them as role models and caretakers. But additionally, I have been able to enjoy years and years of family and friends. From old friends (AKA frieds) from way back in high school to California friends who have been with me from day one of knowing me, caring when I’m sick, helping when they can. I’ve been able to watch my sons morph into young men, something I never dreamed would happen when I was young. And even though two are gone, I have four remaining siblings who call and worry about me. I have a partner who is willing to live with me to the end…not an easy task, let me assure. I am so fortunate. I don’t get it.
And oh, when I’m old and wise
Heavy words that tossed and blew me
Like autumn winds will blow right through me
And someday in the mist of time
When they ask you if you knew me
Remember that you were a friend of mine
As the final curtain falls before my eyes
Oh when I’m old and wise
The final curtain will fall, of course. It has now fallen for both of my parents. I hope that when it happens, in the mist of time, I will finally be able to call myself wise. And I hope you will know that you were all my friends.
As far as my eyes can see
It’s been a weird day. I had planned to write about meditation and why having a “lung disease” doesn’t mean you can’t meditate. This was something I used to actually believe, since beginning meditators are often instructed to “follow the breath.” I tried this…I really did! It only made me completely anxious and sure that I was suffocating!
I was going to wax prolific on how it is actually easy to use other objects to “anchor” the mind, and how wonderful it is to start each day with a relaxing (usually) and centering meditation practice, without even thinking about the breath.
Then, as I always (sadly) do before I started to write, I checked my email. There I saw the following press release , and I literally lost my breath.
Vertex Announces Results from Phase 2a Trial of VX-809 Targeting the Defective Protein
Responsible for Cystic Fibrosis
-VX-809 was well-tolerated at all dose levels when dosed once daily for 28 days-
-Statistically significant changes observed in measurement of sweat chloride suggest increased CFTR
-Data support planned combination trial of VX-809 and VX-770 in second half of 2010 for CF patients with
the F508del mutation-
CAMBRIDGE, Mass., Feb 03, 2010 (BUSINESS WIRE) — Vertex Pharmaceuticals Incorporated (Nasdaq: VRTX) today
announced results from a preliminary analysis of data from a 28-day Phase 2a clinical trial of VX-809 in patients with cystic
fibrosis (CF) who are homozygous for the F508del mutation. VX-809, an oral investigational Cystic Fibrosis Transmembrane
Conductance Regulator protein (CFTR) corrector, was well-tolerated across all four dose groups studied. In the trial, VX-809
showed a statistically significant decline in sweat chloride at both the 100 mg and 200 mg once-daily doses, suggesting that the
activity of the CFTR protein was increased in patients during dosing. Additionally, VX-809 demonstrated a dose response in
change in sweat chloride across the four dose groups. On the basis of these results, Vertex plans to initiate a combination trial
of VX-809 and VX-770, an investigational CFTR potentiator, in the second half of 2010. VX-809 and VX-770 were developed
with support from Cystic Fibrosis Foundation Therapeutics, Inc., the nonprofit affiliate of the Cystic Fibrosis Foundation.
“This Phase 2a trial evaluated the potential effect of an oral compound to improve trafficking of the defective CFTR protein,
and its results represent an encouraging step forward in the development of new therapies to treat the underlying cause of CF
in patients with the most common CFTR mutation, known as F508del,” said J.P. Clancy, M.D., Director of the Pediatric
Pulmonary Center at the University of Alabama at Birmingham and Principal Investigator for the VX-809 Phase 2a trial. “In the
trial, VX-809 was well-tolerated across the dose groups, and statistically significant changes in sweat chloride, an important
biomarker of CFTR activity, were observed at certain dose levels. There is high interest in the CF community in new
approaches to CF therapy, and we look forward to the future exploration of VX-809 and VX-770 as part of a novel combination
regimen aimed at treating the majority of CF patients.”
Ok, first if you need a review of what this all means, read an earlier post here. The big news here is that Vertex 809 somehow “corrects” the trafficking problem of the dF508 defective protein…at least enough to cause a change in sweat chloride concentration. This is huge! We know that another Vertex drug, Vx 770, potentiates (enhances) the effectiveness of the chloride channels present at the apical membrane in the G551D mutation, and is now being tested on people with the dF508 (most common) mutations as we speak (read, write, whatever we are doing). So, if Vx 809 gets the protein up there, and Vx 770 opens it….
Is this the beginning of the end of CF as we know it?
I am 47 and have garden variety, homozygous delta F508 CF. While it is true that I probably benefit from a helpful modifier gene or two, I am convinced that the reason I am so healthy today is that I have exercised regularly and vigorously my entire adult life. As I write this, I have rivulets of sweat dripping down my shins making tiny little puddles on the floor. My schnauzer loves it. The reason: I just completed the “Plyometrics” DVD from the P90X Home Fitness Program (have you seen the infomercials?). I am on round two of P90X now. I don’t know what the “P” stands for, but “90” is the number of days the program lasts, and “X” is for Extreme.
I’m not really endorsing P90X here. I like it because it is HARD, it is different from my usual routine, and because it is a home workout program…no germ-infested expensive gym is needed. With a few dumbbells and a chin up bar, anyone in pretty good shape can do it. For example, the DVD I just finished included about 45 minutes of jumping up and down in various ways. Imagine wearing the Vest while riding a racehorse and being the horse, all at the same time. It’s hard! But this program has changed the way I look at and do my exercise, and that is what I want to share.
I was sicker than I have ever been this winter. When my energy started drifting down, we discovered that my lung function was doing the same thing, so I did a three-week home IV treatment. Not surprisingly, except for drug allergy issues and a DVT in my arm from the PICC, I felt GREAT when it was over…for two days.
Then, as luck would have it, I caught a nasty virus, which resulted in a week in bed, followed by a week in the hospital with pneumonia, followed by ANOTHER three weeks of IV antibiotics.
Not surprisingly (to me, anyway), as soon as the line was out, I was searching for a workout program to get me back to the shape I had been in before all of this began. P90X was the way I did it, but there is a myriad of ways to get moving.
So every day, I followed the program. I exercised to the DVD’s six days a week in my garage. These were short sessions, about an hour long each
Now, here is the part that is hard to believe. Between the antibiotics and the exercise program (nothing else changed), I seem to have grown a new lobe of lung tissue! Kidding. Not really, of course. However, I am a bit of a nerd about my pulmonary function tests. I have them dating back over 20 years. When I blew for the first time this spring after finishing the exercise program (and six weeks of antibiotics, don’t forget). The volume of air I blew out in one second (FEV1) increased by 39% since the previous, and the small airway number (the FEF25-75%) improved by 70%!
I was a bit shocked by the “percent predicted” figures, having not heard those numbers in decades, so I decided it was a mistake. Maybe the norms being used were different. Maybe the machine was wrong. Then, I blew again. Same numbers. I drove home wondering how in the world scar tissue could turn into lung tissue (I’m sorry, but I still have a Pathologist sense of humor, despite retirement).
So I did what a nerd would do, and I dug out all the previous reports and compared volumes. Volumes don’t lie. Sure enough, the last time I saw numbers even close to these were in my 20’s. Did I mention that I’m 47?
Yes, this is an anecdotal story (but a true one). I did not do a randomized, double blinded, placebo controlled study demonstrating unequivocally that exercise caused my lung function to improve (although several well designed studies HAVE shown that exercise slows down decline in lung function in CF). I know that the antibiotics helped immensely. But it isn’t like I haven’t had antibiotics before…
The bottom line: You must move! It helps to push yourself a bit. Breathe hard! Cough! Repeat! If you tend to desaturate, use oxygen! It gets easier. Even though we have CF, we do show a training effect, just like everyone else. It is hard work to stay alive and well with CF. This is undeniable. But the reward is worth it.
It’s a hot, summer day last August. The “world’s champion” (not really, but we thought so) Frisbee dog, my 13 year old Border Collie, Cisco, is out in the back yard chasing squirrels with his two schnauzer brothers-from-another-mother, Wiley and Jaxon. This is their favorite game, and I am watching them from my glass- enclosed office, where I am sucking on some hypertonic saline. Cisco has been the most athletic dog I have ever known…literally winning regional Frisbee catching competitions, running with me wherever and whenever I go, defying any and all attempts to contain him his entire life by either jumping fences or opening gates. I kid you not; he could star in his own television show with episode after episode of tales of his pure determination and ingenuity in fulfilling his life’s work, never letting me out of his sight.
But this day, he knows I am safe, and his focus is on the squirrel above him, taunting him from the telephone wire. Another squirrel joins the game from the edge of the yard, and the schnauzers are off like lightening, tearing across the grass at a speed I didn’t know was possible. Cisco starts to follow, and then I hear the most heart-breaking and terrifying scream emit from his mouth as he flops on his back and writhes in agony.
It takes me two nanoseconds to get to him, and less time than that to realize that he is in serious trouble. He clearly can’t move his back legs, and I can literally smell his terror in the air.
Fortunately, the boys are with me, and we get him in the car and to the vet as fast as we can. One X-ray later, we are rushing to another veterinary office, where they specialize in back surgery. I get the news there: he has ruptured a disk in his vertebral column, and he needs immediate surgery to decompress his spine. He will “likely” be able to walk again, but every second that passes decreases the chance of significant recovery.
There I am with two young and very upset kids and a dog that is like another child to me, barely looking at the price estimate. You can’t place a price on my relationship with this dog. He’s been through thick and thin with me. For thirteen years, he has always been there for me, and now I needed to be there for him, big time. I call my partner, and we decide…we’ll figure out how to pay for it later.
Days later, we take him home. The few weeks that follow are a bit of a blur. I nurse that dog like a baby. I learn how to “express” his bladder because those nerves don’t work at first. I put diapers on him, and “chucks” under him, often to no avail. I get peed on and pooped on and even nipped when he was hurting, just like old times with the boys! I even put my pillow down on the ground next to him and sleep with him at night, holding his paw and stroking him so he knows I’m there…otherwise he cries and neither of us gets any sleep. I carry him everywhere because if I leave the room for a minute, he starts yelping, “Mommy…where did you go???” in dog. Forty-three pounds of limp Border Collie is a LOT of weight to move ten times a day, and I am very appreciative of all the dead lifts I’ve done at the gym.
Then, physical therapy starts (think “ching”). I learn to “walk” him using a sling for his rear end. He gets massages, and TENS, and learns to walk on an under-water treadmill. The therapists and I actually re-teach him how to walk again by moving his back legs over and over again in a bicycle motion and flexing and extending his bad leg over and over. Then, one day at therapy, he struggles to his feet by himself and sort of pulls himself by his front paws across the room toward me as his back legs stagger and slide. He moves about 7 steps before he falls down. It definitely wasn’t pretty, but this is the first time he is able to move on his own accord and we cheer and scream for joy. I swear he’s smiling!
Winter comes, and I have probably my worst cold season ever. I’ve got a PICC line in for many, many weeks and am having other health issues recovering from a DVT in my arm. Still, Cisco and I make our daily treks around the neighborhood. There we are… I’m coughing and infusing, and my faithful boy is limping and sliding along at about the speed of a slug. Sometimes it’s not clear who is taking whom out to walk, but nothing keeps us from our daily treks.
As I write this, it is summer again, and I am happy to report that Cisco is still with us, as a much older (functionally) and much lighter and greyer version of his old self. His back hips and legs have atrophied down to the bone. He’s recovered some of his strength, but still needs assistance with his “morning constitutional” (which, by the way, is a total misnomer… It happens at all times of the day). Every morning, afternoon, and evening, Cisco and I go for our stroll around the neighborhood, him hobbling slowly but with as much dignity as he can muster with me holding up his butt with a sling. When he sees other dogs, he still growls with the best of them, and, oddly, seems to forget that he is disabled as he tries to chase them down, dragging his left leg (and me) behind him as I desperately try to keep holding the sling.
This is similar to me doing push-ups and yoga with the PICC line in (Did I mention I developed a DVT?). Anyway, the point is, you can’t keep us down. We are there for each other, and we are quite a pair! End of story.
Why am I telling this story? What in the world does it have to do with cool equipment and technology that makes life easy? Well, Cisco and I were chatting just the other day. It turns out that we are both getting rather tired of going around the same old area, the same houses, the same trees and plants. We both want to go new places, see new things, growl at new dogs. But he just doesn’t have the stamina to go too far from home.
So I made him an appointment to get fitted with a K9-cart. These are so cool! They are “wheelchairs” for dogs. You put the dog’s hindquarters in a type of sling, and they pull themselves around on wheels! He’s going to be so happy. I can’t wait to get it.
Finally, we are back to why I wanted to tell this story. I once told my partner, who was trying to convince me that long distance running may not be my best exercise option, that I had to run….well, jog. I used to run. I ran until I couldn’t anymore. Then, I started to jog. Then, the continuous jog became a jog/walk. This is where I’m at now. When I can’t do that anymore, I will walk. When that becomes too hard, I will walk very slowly. When that becomes too difficult, I guess I’ll roll (but I’ll pull myself along).
Cisco is the dog model of exactly the way I plan to do it. He used to run like the wind. He could jump tall buildings. He could open any door. He could do crossword puzzles. He was Cisco, the Wonder-Dog. Now, he drags himself around, limping after me, wherever I go. He uses mostly his front paws and stumbles and falls quite a bit, but unless there is a need to squat (as I referred to above), he gets around on his own. Not only that, but he still tries to chase the schnauzers around the yard, squeaking his ball like crazy while they wrestle. It’s becoming hard for him though. He’s decided it’s time for some assistance, and I agreed. Now we’ll roll. Well, he’ll roll. I’m still jog/walking for a while longer.
So maybe you can’t run. I bet you can walk. I even bet that some of you, who think you can’t run, can build up to a jog. Or maybe you’d be happier on a bike…or roller blades…or swimming in a pool. If you are still breathing, you can do something! Even if your breathing is labored, or you have problems with desaturation with exercise, they make some awesome portable O2 tanks these days. Not only that, but there are portable O2 saturation meters that you wear on your finger so you know how much oxygen you need! There are heart rate monitors, portable blood glucose meters, GPS devices…you name it! Check out Jerry Cahill on You Tube as he takes his oxygen tank for a run (http://www.youtube.com/watch?v=dT6aEuZKpC4). Take a cue from Cisco, and from me, and from Jerry: Keep moving, no matter how it looks, and keep squeaking your ball.