Not My Miracle

“A vacation!” I squealed.

“To where?” my husband asked.

“Anywhere! After Orkambi, I’ll probably be able to go anywhere. I read that someone traveled to Europe after years of being too sick to do much of anything…

…Ohh!! We can also go see Billy Joel at Fenway next summer! Remember how I was in the hospital when we had 7th row tickets 2 years ago and we had to sell them?

Oooohh…We can also visit your parents next Christmas!”

On a random day this past November, we were hanging out in the living room, watching a movie. My husband was sitting on the love seat, and I was laying on the couch, clutching the area of my chest where my lungs felt so tight.

I was taking a new medication, developed specifically for people with my gene mutation of Cystic Fibrosis. The medication with the funny name – Orkambi – held so much promise, but was known to cause chest tightness and shortness of breath in the initial stages of treatment.

My husband and I were okay with that – what’s a week or two, or maybe a couple of months, of discomfort, if it meant it would change my – our – entire lives? Continue reading

Hitting a High C

I needed help blowing out the candles, but I did it!

December 31, 2015 It’s the last day of this year, and this is my official New Year’s Eve post. It’s also the eve before I begin month 3 of taking Orkambi, the new Cystic Fibrosis drug. I originally planned to chronicle the major ups and … 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

ToniV 2.0: “Works as Designed”

Words Matter

A few people well acquainted with medication in general, and patients who have taken Orkambi since the studies began, have mentioned the symptoms I am experiencing are not “side effects” in the true sense of the word.

Let’s simplify this.

  • The Gene Mutation has caused a specific action within each cell of my body to not act correctly for 43 years.
  • I started to take the Medication, Orkambi.
  • The thick mucus is no longer being made.
  • Now, the mucus must get out somehow.

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

The Day of Reckoning

Tomorrow morning, I have my Pulmonary Function Test and some blood tests. A lot is riding on the results of these tests.

Providing my lung function is at least better than it was two weeks ago, and not going in the opposite direction, and my Liver function blood tests don’t show any irregular numbers, I am planning to finally begin taking the new Cystic Fibrosis drug, Orkambi. Continue reading

How Cystic Fibrosis Laughs at My Attempt to Plan Anything

In a way, it seems so long ago that I was last in the hospital, but it’s really only been four weeks.

Home! But…Healthy?

I completed my two-week course of my antibiotics exactly one month ago.

Normally after getting out of the hospital, I go through a period of recovery – still fighting the bacterial infections that landed me in there in the first place, and then trying to regain lost strength and energy from being laid out for so long. My body also has to recover from the effects of the strong antibiotics that, while killing off the bad bacteria in my lungs, also causes problems to other organs and systems.

I would experience a time when I felt GREAT (comparatively). This time, however, I missed out. Continue reading