Quick September CF Update

I haven’t been online too much this summer. I have been sick so frequently that some exacerbations began only days after the previous one(s) settled down. It was a rollercoaster of antibiotics, mostly to treat my MRSA.

Now, it appears that the common CF bugs – Pseudomonas Aeriginosa – has taken advantage of pushing down the MRSA, and the 3-4 strains of PA (I culture between 1 and 4 strains at any given time) have found their way to the top of the food chain.

My lungs are in rough shape – coughing at night, weird night moaning that signals dropping oxygen levels, extreme fatigue, coughing up mucus and pus, fever, and the feeling that I am absolutely losing my ability to function on a basic level.

I am likely going into the hospital next week for IV antibiotics.

There are no oral antibiotics that can treat my strains of PA, so my only option is a cocktail of 3 different IV antibiotics to try to cover all of the bugs growing in my lungs right now. As you may know, it’s become increasingly difficult for the Interventional Radiology team to find a good vein to place a PICC line, and a year ago, I ended up with a very uncomfortable tunneled PICC line that threaded through my jugular vein – a process that took 4 agonizing hours while my face was covered with a sheet.

It will take a miracle to find access into my veins…thanks to the number of blood clots (we’re at 3) that have formed around PICC lines), a dozen or more traumatic PICC line placements each lasting hours, repeat 2nd and 3rd failed attempts, and failed medications designed to put me in a twilight sedative state (that does not work on me).

The nightmares have come back and I am asking for prayers for my strength and courage to get through what they may have to do. I feel like I should be “a trooper” since I know what to expect, but sometimes, it makes me more fearful to experience again.

I will update when I make a decision or anything changes (like a miraculous turn of events!!).

Thank you for checking in.
Love,

Toni

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.

Let Freedom Ring. Just don’t let it be my cell phone.

I am pretty sure that people think that I am exaggerating when I tell them the number of unique phone numbers that have “Spam called me”.

This list includes – but is by no means exclusive – several hundred phone numbers that have called me with the intent to somehow scam me.


Continue reading

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.

 

Continue reading

Please Support our Fundraiser for Cystic Fibrosis!

It’s that time of year again! We’re walking to raise funds and awareness at the Cystic Fibrosis Foundation Great Strides event!

This is our fourth year participating in Great Strides! Here are our totals for the previous three years:

2013: $2,575
2014: $2,310
2015: $2,935

The past two years, we had held our own additional fundraiser, Darts for CF, that helped raise our totals, but we aren’t able to do so this spring. So our only fundraising efforts through the Great Strides event!

We hope to raise at least $1000 this year, so I’m reaching out to everyone!

CLICK HERE TO DONATE OR JOIN OUR TEAM!

Continue reading