Random Thoughts on Living with Cystic Fibrosis

I dream of feeling free, to engage myself with all that I see.

I dream of taking steps that lead further than my own front yard.

I dream of laughing without choking, and singing or dancing without coughing.

I dream of waking up each morning and bounding out of bed, and just taking off, somewhere, anywhere.

I still remember how it used to be when I could dance without gasping for air or my heart feeling like it was going to explode… times when my heart raced out of excitement and joy versus out of desperation to support my lungs with the oxygen they need.

It wasn’t too long ago that I had bigger accomplishments each day than simply walking up the stairs or taking a shower.

I no longer sing made-up songs about the cats or the weather or anything else that tickles my fancy, because I can’t spare the oxygen.  

I no longer dance when I hear my favorite songs, because that, too, requires my very precious oxygen. 

Instead, I shake my hands and shoulders with as much energy as I can afford to give to them.
I cannot laugh easily anymore. It robs me of my breath.

I work hard to strengthen my muscles so that they can take on some of the effort when I’m doing something strenuous like folding laundry or walking up the stairs or taking a shower.

I have to monitor my oxygen levels when I eat, because even a partially full stomach can make it hard for me to breathe.

Why has God taken my ability to dance, sing or laugh?

Today I am having a hard time seeing the glass as half-full. We, as humans, have the gift of such pleasures, and I feel robbed.

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Creating Magic

The holidays are supposed to be a time of joy and celebration, if we are to follow the messages bombarding us from television commercials, festive songs, and colorful decorations.

But for many people, the holidays bring out a lot of things that are not right in our lives.

It could be the recent loss of a loved one, or the lack of finances to buy gifts that you want to get for people, or struggles with your personal belief systems, conditions such as depression or anxiety, and in my case, chronic illness.

As I’ve been pretty sick and spend a lot of time doing my respiratory Vest treatments, I end up watching a lot of television because the shaking from my vest machine makes it difficult to read or write or do anything else that requires a steady hand or steady eyes.

And I kept thinking that I still hadn’t found a gift for my husband that would give him that “WOW! this is amazing” moment that we see happen in commercials and movies and television and advertising. It’s a pretty seductive desire, the feeling of making someone so happy by simply opening a brightly wrapped box.

But that almost never happens in real life, does it?
And before allowing myself to feel like a failure, I realized how much commercialism has conditioned me to think that in order to be a good wife or sister or aunt or daughter, I have to create these moments.

But the magic doesn’t come in a box. The magic comes from our hearts’ desires to make someone happy. How many times have you made someone’s day just because they called you or texted you? How something as simple as someone opening a door for you, can make you feel that there are good people in this world who care about others with no expectation of getting anything in return?

As I have been sick constantly since August, I had to tame down any of those conditioned expectations that I was going to make someone’s world with a simple gift that I had wrapped in pretty paper and a colored bow. 

I realized that the magic that I can provide is to be able to participate in our family Christmas celebration. There have been some holidays I spent in the hospital. There’ve been some holidays when I was too sick to travel. And more lately, there are holidays when I have to save up all my energy for a week to just go “out” for two hours.

But in those two hours, I soak up every smile, every laugh, every moment that I can. Because even if I can’t last more than a few hours, the magic is in connecting with those we love. 

So whatever you have to celebrate during this time of year, remember that the biggest gift that you could give someone is a piece of your heart.


These are the people who keep me going. These are the reasons I continue to fight. And these are the pieces of my heart.

Relinquishing Control in the Hospital

I just received a letter in the mail which included a six-page questionnaire about the care I received while in the hospital for 2 weeks in September.

They ask questions about the care I received, the hospital environment, my hospital experiences, tests and treatments, etc.

I am under no delusion that my care is very complex. At home, I manage 15-20 medications, test and monitor my blood sugar for diabetes, and perform airway clearance (lung therapy). It’s not just a matter of taking a bunch of pills each day. Many of them have to be taken at specific times, in relation to waking up, eating, performing certain activities (like driving), avoiding potential interactions, managing treatment side effects, etc.

So when I’m admitted to the hospital, I have to turn in all control to a series of more than a handful of different departments to manage.

Every hospitalization is a challenging experience. I’m awakened early (sometimes 4am) to get blood taken, doctors from 3 or 4 different specialties visit me daily, each asking all of the same questions, poking and prodding me according to their focus. I feel like I have to give up agency over my own body. I succumb to the authority and power of mostly strangers. Continue reading

The Top 5%

I’ve recently realized something profound.

When I was diagnosed in 1972 – a month after my first birthday, the data showed that most patients wouldn’t reach their teen years.

Amazingly, though, as of 2014,  of all CF patients currently living, 50.7% are older than 18. That’s incredible news. However, the median age at death in that same year was only 29.1 years.

I am 44 years old. In the general population, most people would consider that middle-aged. But in the CF population, I’m an anomaly.

The fact is, only 5 percent of Cystic Fibrosis patients are older than 40.*

*According to info published in the most recent Cystic Fibrosis Patient Registry, information collected from 28,676 patients seen at accredited CF care centers. Most, but not all, CF patients attend such centers.


I have so many questions, but I know that I will not find any real answers.

So I will share the three things that I DO know.

1. I am beating the odds.

  • Whether you believe in luck, fate, chance, karma, punishment, or a higher being’s plan for you…
  • Whether you believe that those who live longer are more compliant with their medications and treatments than those who don’t live as long (note that I do NOT subscribe to this theory)…
  • Whether it’s due to my environment and lifestyle, such as the way or what I eat, how I manage certain issues, my BMI, the type or amount of exercise I do, my airway clearance regimen, how much sleep I get each night, or some concoction of supplements that help me recovery from flare-ups or that slow overall progression…
  • Whether it’s because, in addition to my mutated genes that cause CF, I have some crazy fabulous genes that compete with the bad ones, and many times win the battle….heck, my two grandmothers did not pass until they were in their 90s, so I have some good genes in me.

2. CF is taking its cumulative toll on my body

As I age, my body is battling the effects of decades of malfunctioning, infections and organ disease, and toxic medications to help me to the most basic things such as breathing and eating.

My lungs, digestive system, and other organs – in fact, every cell in my body – have been fighting the imbalance of salt channels and the thick mucus that causes disease in our bodies for more than 4 decades. As my organs deal with the dysfunction and disease, they become inflamed with every exacerbation. The frequency of exacerbations and the shorter recovery times between them mean that my body is often in a state of constant inflammation. And unfortunately, the number one medication that treats inflammation is also really, really bad for people with CF. It weakens our immune system and also negatively affects those of us with CF related Diabetes.

3. Having CF doesn’t preclude me from what’s considered normal symptoms of aging. Name a body part and it changes with age. CF doesn’t stop those things from happening.

My body has been fighting – since before I was born – to perform normally. Now, in what is considered middle age for healthy people, I’m starting to experience regular “growing old” stuff. The kind of stuff that make you think, “I’m not as young as I used to be”. The things that slaps you into coherence that you’re really NOT 23 anymore. Or even 33.

At least I can laugh and commiserate with my sisters when we notice one another’s white hair spreading across our foreheads, or deal with puffy eyes or a quicker-to-twinge back muscle.

Back to the white hair thing – I recently came across an online group for people with CF who have gray hair. As much as I have been fighting this sign of aging, others have reminded me that our predecessors didn’t get the chance to have their first gray or white hair!! What a privilege is it to actually have reached a point in life that indicates you’ve survived into adulthood! While I still haven’t given up the color battle, I do feel like it’s my own little secret, my own little victory that I get to wear every day (there’s always a new stray white hair popping out, somewhere on my head!).

Next-to-last final thought

All of this also reminds me that I need to give myself a break sometimes. I have high expectations of what I should be able to do and so many things that I want to do. And the reality is, that my body has been fighting for so long just to be alive, that sometimes I think I expect too much from it.

If, on any given day, my biggest accomplishment is taking a shower, I need to be OK with that. I always talk about celebrating the small victories, and I think it’s important to view all aspects of my life that way. I am proud of what I have accomplished – not just physically, or professionally, but spiritually, mentally, in the way that I view and live my life.

But the real point I am making…

I am 44 years old, I have CF, and I am still here.

And I feel damn lucky.

Growing Stronger

Activity. Our bodies are made to move. Even when I’m in the hospital with lung infections/pneumonia, my doctors expect me to exercise regularly (as tolerated, of course). At my hospital, CF patients now usually stay on the Pulmonary floor, have visits from Physical therapy to walk in the hall or up and down stairs, and a stationary bike moved into the hospital room. This is in addition to Respiratory Therapy six times a day.

I am not a fan of the stationary bike, as it’s really uncomfortable to sit on, so I walk the halls several times a day, and I do Pilates right there in my bed, when it’s safe to do so (as in I don’t have low blood pressure, low oxygen levels, or low blood sugar, all of which can happen during these infections).

Last fall, during and post hospitalization, I had lost a lot of my muscle tone. Pilates has brought me back from a state of weak muscles and as a result, even poorer levels of endurance.

 

What is Pilates?

It’s a series of movements, designed to stretch, lengthen, and strengthen your muscles, especially your core – the center of your body, including the abs and your back. It helps you strengthen and sculpt your muscles and gain flexibility.

Some people use pilates machines at the gym, resistance bands, or simply, their own bodies, to create resistance. This is what makes Pilates so simple – you can do it anywhere, even in bed!

In fact, physician Joseph Pilates created the exercise regimen for bedridden patients,rigging springs to the hospital bed to create resistance . Its effectiveness was tested during a flu epidemic that struck England in 1918, when thousands of patients died, but not a single patient trained in Pilates. Interestingly, Dr. Pilates’ movements influenced the curriculum of modern dance training and techniques.

The Basics

Many people simply use an exercise mat on the floor, and use their own body for resistance. It looks easy, but it takes practice to not only learn the movements, but to learn what muscles you’re supposed to be feeling, where they are, and how everything’s connected.

I learned Pilates through watching DVDs created by instructor Mari Winsor. I wore out my first set of DVDs after a few years and bought a second set. After another few years, I pretty much knew the movements and routines by heart, so I didn’t have to watch them to complete a workout.

Although it’s not an aerobic exercise routine, prepare to sweat! Imagine holding your arms out straight in front of you, gently reaching them as far as they can go, and seeing how long you can hold them out. That’s what Pilates feels like.

Getting Started

The first step is to learn the movements. They look simple, and they are, logistically. It’s a series of stretching, reaching, and performing gentle movements in repetition.

Once you learn the movements – some might take a few weeks before you can actually perform them all – you can move on to performing repetitions. Some of the movements are done in a set of 3, others in a set of 10. Many are done forwards and backwards, up and down, left and right.

After gaining enough strength to complete all of the reps, you can start working on increasing your resistance – whether it’s on a machine, using an exercise band, or your body’s own weight. I prefer using my own body for resistance, but just recently started to experiment with exercise bands.

A Lifetime of Growth

When I watched those DVDs the very first time in 2003, I remember thinking that I must be crazy. Although I had studied dance for several years, had been through ACL reconstruction and rehabilitation, and knew a lot about stretching and leg exercises, the movements were excruciating to master. I thought that I was pretty good at exercising – I was a gym rat during college and my 20s, and could do 50 sit ups, but this was like sit ups on crack, in every part of my body.

What was great, though, was that this particular program – Winsor Pilates – had 5 training DVDs, beginning with the “Basic” 20-minute learning workout, and specific workouts for the Abs, Upper Body, “Buns & Thighs” and Advanced Slimming. I later purchased an Aerobic version which was intense, non-stop movement, and it wiped me out.

It took a few weeks just to learn the movements and develop muscle memory. It took another month or two to work up to getting through the entire beginner 20-minute workout.

I tend to go through phases when I do it 3-6 days a week for several months, only to have to stop for a while due to a setback like a bad infection, belly problems, etc.

A ToniV Tradition

Regardless of where I am health-wise, however, I always come back to Pilates when I need to regain lost strength and/or endurance. Since restarting several months ago and focusing on my core and legs, I have gained enough strength to carry me up the stairs without putting all of the onus on my lungs and heart. The gains I have made have inspired me to start working on my arms this past week. A pair of 10-pound weights and determination are all I need to start building some lean muscle in my upper body.

I look forward to continuing to grow stronger, which will provide me with more endurance and support when I am not well.

What do you do to stay or get stronger during or after a flareup?

First, Waiting to get Better, Now Waiting for Fun!

I just wanted to write a quick update because things have been a little busy around here and you know how that gets..

After my belly issues at the beginning of March, I’ve been dealing with infection on and off and try to stay off antibiotics as long as I could. That was until about two weeks ago.

I had my regular CF clinic visit, ended my PFT tests, and my numbers were pretty good. They were pretty much exactly the same as they were last time… Not improved all the way back from everything I lost after my experiment of taking the new drug for CF, but it hadn’t gone down.

So when I came home a couple of hours later and I had a fever of 100.1, thought that said my streak is over.For seven weeks, I had experienced several episodes where my infections are flaring up and I had several days of strong symptoms, but you’re doing extra vest treatments and lots of rest and vitamins, and lots of fluids, I actually made it through without loading antibiotics. My immune system *sometimes* does work!

 

This machine measures how much air I can blow out in 1 second…and a few other variables…that help define my current acute and chronic lung disease.

 

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College and Cystic Fibrosis: Who/ What/ When/ How/ Why to Tell People about your CF

I came across some amazing news recently – nearly half of all CF patients are older than 18.

Courtesy of the Cystic Fibrosis Foundation https://www.cff.org/What-is-CF/About-Cystic-Fibrosis/

Courtesy of the Cystic Fibrosis Foundation

That means the patient population is growing up!! How amazing is that? It means so many things, and for some of us, it means:

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No matter where you are in your self-identity journey, it’s never easy to know when to “come out” with your CF. And now that you’re going to college, you wonder, who do I tell? Who should I tell? Who do I need to tell? Who needs to know, really? It’s nobody’s business, right?

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