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.

The Anatomy of an Interruption

There I was, getting better from my recent lung infection, when the other part of my body that Cystic Fibrosis likes to mess up – my belly – decided to stir up some trouble.

I was seriously at a point in my life where I was beginning an “overhaul” of me – ToniV. It sounds silly, but a few days prior, I had actually taken a poster-sized foam board and outlined my plan for overhauling 7 areas of my life. 

Some of the Gastro Intestinal problems that patients with CF can face, that I have, include Pancreatic Insufficiency (the mucus blocks digestive enzymes from leaving the pancreas), CF related Diabetes, GERD, chronic Distal Intestinal Obstructive Syndrome (called DIOS, which is frequent bowel blockages caused by mucus and poor digestion), Chronic Pancreatitis, and Gastroparesis (slow emptying gut). Continue reading

Hope is an Amazing Thing

Tomorrow marks the start of my second month on the new CF drug, Orkambi.

Just arrived!

Just arrived!

It’s been one crazy month. Just as I started the Orkambi, I ended up having a flareup of my bugs. It came on kind of slowly.

Prior to starting Orkambi, my doctors warned me that I could experience side effects, so for the first week, I chalked up most of the symptoms to the new medication. But my husband, in his usual crazily-accurate assessment of my symptoms, tried to convince me to contact my doctors. I finally did, when my fever spiked to 103.5, which definitely wasn’t listed on my list of symptoms to monitor. Continue reading

My Cystic Fibrosis Journey: 1987-1988

This was a big year.

At the end of the summer before my Junior Year of high school, I came across a newspaper ad for a new professional theater’s Young Professionals Program. Admission was by audition only. I nervously applied and auditioned. It was  a huge commitment. Surely, the plays and musicals I had done up until then had required major commitment, but this was a year-long program. I had to maintain not only my grades, but most importantly, my health.

I had enjoyed several years of stable health. I still had frequent respiratory infections, and constant tummy problems, but they were part of my norm. As the year progressed, however, I would learn that uncertainty would become my new norm, as it had been for my parents so many years earlier when I was first diagnosed with Cystic Fibrosis. In addition, my older twin sisters had left for college, leaving a huge hole in the house and in my life. I was now the “oldest” at home, with my younger brother starting high school that same year. Lots of changes ahead! Continue reading

My Cystic Fibrosis Journey: 1984-1985

Eighth Grade. What an accomplishment! Not only were we headed to high school soon, but I, Toni Marie, the girl who everyone feared would never live long enough to reach this milestone, was still here. And she showed no signs of going anywhere soon, except forward.

This was the year that everything that could happen, did happen. I purposely didn’t expound upon serious bullying that had gone on throughout my childhood, because I didn’t want it to steal focus from sharing my successes during the earlier days of my journey with Cystic Fibrosis. Continue reading

My Cystic Fibrosis Journey: 1981-1984

When you spend your entire childhood with the same kids, you go through everything together. I attended a Catholic elementary school that taught Kindergarten through the eighth grade. For most students, it’s a breeding ground for strong bonds and a lifetime of shared experiences. For me, however, life happened a bit differently.

I was still sick frequently.  I was still chosen last for everything in recess and gym class. I was still “teacher’s pet” and mocked for my love of school, among other things. I was still the smallest in the class, so much that when I made my Confirmation in the seventh grade, the other kids made fun of my dress, asking me if I bought it in the “First Communion Department”.

Confirmation Day with the "favorite" priest, Father O.

My Confirmation Day with one of the school’s “favorite” priest, Father O.

Continue reading