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.

My Gifts to my Self

Birthdays. Holidays. They seem to highlight both what is great in our lives and what isn’t so great.

My birthday is always right around Thanksgiving. This year, it was 2 days later. Continue reading

TheraPink Series: Kids with Cystic Fibrosis: Kids, Cystic Fibrosis, and Food

When the words “Cystic Fibrosis” come up, the first thing that many people think about is a thin child laying in a hospital bed.

Indeed, I was very thin, always the shortest kid in my class, and despite a voracious appetite in between infections, just never fell anywhere within “normal” on those growth charts.

Research has proven that having a healthy BMI (body mass index) helps our lungs stay healthier overall. However, when we get sick, we can lose weight very quickly, which shoves us into a vicious cycle. Continue reading

Creating My Tomorrow

A New Beginning?

You know the optimism you feel at New Year’s? The renewed hope for the future? The same enthusiasm you feel when a new baby is born, the anticipation of starting a new job or school?

I tend to view life as black or white, up or down, stop or go. I’ve never been content anywhere in the middle.

A few weeks ago, I stopped taking the new CF drug due to side effects and the interactions with several of my maintenance medications. With the drug also went the hopes from both the medical and CF communities that it would change my life. Continue reading

ToniV 2.0: The Orkambi Odyssey

Yesterday, I began taking the highly anticipated Cystic Fibrosis drug, Orkambi.

My box of hopes!

My box of hopes!

The medication, a combination of two drugs, aims to correct the defect that causes the characteristic thick mucus in CF patients with two copies of the most common gene mutation. The mutation, Delta f508, causes both lung and digestive involvement, in addition the sinuses, liver, and other organs. Continue reading

Approved for New Cystic Fibrosis Medication!

I just wanted to share that I just got approved for the new Cystic Fibrosis drug that treats the root cause of the genetic defect of my mutation!!!

While taken, Orkambi corrects the defect that prevents the flow of salt and water from passing through cells properly, which creates the thick, sticky mucus in all of my organs, especially the lungs and digestive system. It is for CF patients with the mutation Delta f508, of which I carry two copies. It is also the most common mutation, and responsible for disease in most of my major organs.

While not a cure, this drug can potentially decrease the frequency and severity of complications, thus leading to a better quality of life. There are no guarantees, and there are side effects, but this is a HUGE chance for me – for us. My husband and I are excited and nervous that Orkambi will provide at least some benefits to increase quality of life.

I hope to share the process here. I see my doctors on Monday and hope to begin the medication in next week or so. If you’re interested in following my story, please subscribe for updates and you’ll be notified whenever I publish a new post.

Chasing a Moving Target

It’s been a crazy summer. I spent most of it either getting sick, being in the hospital, or recovering. One thing that many people don’t understand about CF is that even after we come home from the hospital, or finish a course of medication, we’re not necessarily “all better”. The term “all better” is known as “Baseline”.

Baseline is the place where your “numbers” or symptoms go to when you’ve recovered from an acute (temporary) flare-up or episode.

What is Baseline?

For example, let’s say that you walk for an hour each day, averaging 4 miles an hour. You twist your ankle, and you can’t go on your regular walk for a few days. When you start getting back on your schedule, you might walk a bit slower, or not be able to walk for the usual hour. As your ankle heals, you eventually get back to where you were before the injury – walking 4 miles in an hour, every day. That would be your baseline. Continue reading