Kalydeco Week 3

I just wanted to post a quick update, although there is not much to report. The Cipro seems to be helping, but I am still coughing, my lungs are still rattling, and my FEV1 is still down. Greg and Grace are still coughing too. Sigh. Hopefully I will have something to report soon!!!

2.5 Week Update

This coughing virus is a beast! My husband and I are both still coughing. Last week it was so bad I almost threw up a few times, and the same thing happened to my husband. I was talking to a friend who said she and her husband had the same virus - she said she was coughing forever and that her husband threw his back out.

So, with that said, I still can't really say for sure how Kalydeco is helping my lungs. I usually blow about 3.3 L on my FEV1 monitor. At one point last week I was struggling to blow 3.0 L, but I started feeling better, and at one point I got it all the way up to 3.9 L! I didn't believe it the first time, but I managed to blow 3.9 L two more times. H-O-L-Y C-O-W. The highest I have ever blown on that thing is 3.6 L, and that was over two years ago, when I was pregnant with Grace and my FEV1 was 120%. BUT, two days ago my numbers went back down and I started feeling worse. I had horrible night sweats and fell like crap yesterday - coughing a ton and feeling short of breath. So, last night I started Cipro.  I already feel better this morning. I don't remember Cipro working this fast in the past, so I'm not sure if I would have felt better anyway. But, hopefully I am officially on the mend!

Kalydeco Days 8 & 9

Warning! Talk of mucus ahead.

I'm sick! Everyone in the house has some sort-of coughing virus. No other symptoms, just a cough. But, I am the only one with a productive cough...I was hoping Kalydeco would prevent that, but maybe it's too early, or maybe it's because I have some mild bronchiectasis. My mucus does, without a doubt, seem thinner when I cough it up - it feels just like saliva, instead of a blob. So that is exciting.

If it weren't for my husband having a cough too, I would have chalked this up to part of the "clearing out" stage. Last night I was coughing so much I was gagging and I couldn't sleep, so I ended up taking cough syrup, something I never do. Wow, that stuff worked instantly!

My sinuses continue to feel awesome. Hopefully my lungs will too once this cough goes away.

Kalydeco Days 6 & 7

Day 6: No night sweats or night time wheezing, but I woke up feeling wheezy. I was coughing and clearing my throat a lot this day. But I had plenty of energy and felt good overall.

Day 7: No night sweats or night time wheezing, but I again woke up feeling tight and wheezy. My nose was less congested this morning. But, I had slight rattling in my lungs and I was coughing all day. I hope this was just Kalydeco loosening things up.

So far, today seems to be a repeat of yesterday.

It has now been a week since I started Kalydeco. I would say that I think it is helping me, but it is not as obvious as I was hoping for. I haven't had a bad day since starting, which is unusual for me when the cedar pollen is high. Usually I feel very run down, tired, achy, and drowning in mucus. My sinuses feel really good!

Kalydeco Days 4 & 5

I think one detail I need to remember is that it is currently cedar season here in Austin. Many people, including myself, suffer from cedar allergies that make them miserable, CF or no CF. This time of year, I almost always end up needing a round of antibiotics to treat a sinus infection. The week before I started Kalydeco, I had lots of mucus in my sinus rinses. So, some of what I am feeling is most likely influenced by that.

Day 4: More night sweats a bit of tightness in my chest, but I didn't need my inhaler in the middle of the night. Woke up feeling wheezy. Had the usual mid-morning cough & coughed up a big (for me) blob of green gunk. I went for a run and wasn't able to run any faster than normal (my pace is about a 10 minute mile). At the end of my run, some sweat ran down my face and into my mouth, and it tasted salty. But, I wasn't coated in salt, like I sometimes am. Still, I started to worry that Kalydeco wasn't working.

Day 5: More night sweats and nighttime wheezing. But, I didn't wake up with a sore throat, which almost always happens thanks to post-nasal drip, and my mid-morning cough was a bit less. By noon I started to feel really good. All afternoon I kept taking deep breaths, expecting to feel the usual bit of mucus build-up on the back of my throat, but it wasn't there. I started to get excited that maybe the Kalydeco was doing it's thing!

I have also been obsessively using my PiKo FEV1 monitor, and I am holding steady at around 3.2 liters. My peak about a year ago was 3.6 liters. I'm not sure if I am going to see much of an increase because my FEV1 is usually 110-115%.

Last night I didn't have any night sweats or wheezing, but I woke up wheezy and congested. We'll see how today goes!

I am on Kalydeco!

This weekend I got the FedEx delivery of my dreams - my first shipment of Kalydeco!

I thought it would be fun to log my initial experiences on the drug, so here we go :)

Baseline: CF-wise, my baseline usually consists of a cough from 10:00 AM until around lunchtime. And my nose is usually at least partially stuffed up. On top of that, I have good days and bad days, which I think are triggered by allergies.  On the bad days, I have night sweats and wheezing in the middle of the night, and then I wake up feeling tight and wheezy. And I feel tired and gunky all day - clearing my throat, coughing, and congested. Sometimes I also feel achey, and sometimes I have a sinus head ache.

Day 1 - This particular morning I woke up feeling good after a good night, so it was a sign that I was going to have a good day. The FedEx man showed up at 8:30 AM, and I immediately took my first pill with a scoop of peanut butter (you need to take fat with the medication). Then I headed to the gym.

I did start coughing up some gunk about an hour after my first pill, but that is normal for me at that time of the day. The rest of the day I felt good. I took my second pill with dinner, and again I had a little bit of a cough an hour after I took the pill.

Day 2 - I ended up having night sweats and a bit of wheezing in the middle of the night, and I woke up feeling tight and wheezy. I had my normal mid-morning cough, and I was tired enough to need a nap in the afternoon, (but Grace also woke me up in the middle of the night, so that was at least part of it). My nose was half stuffed-up most of the day. My stomach had little pains off and on.

Day 3 - More night sweats and a tight feelings in my chest. I immediately did an albuterol treatment when I woke up, and I notice it didn't help with the tight feeling right away, like it usually would. But, when I did my hypertonic saline 90 minutes later, (and an hour after taking Kalydeco), I noticed that the hypertonic saline was less irritating than it usually is. I still had my mid-morning cough.

I would say, overall, I haven't yet noticed Kalydeco having an affect, but it's hasn't even been 72 hours, so no big surprise. I am so excited to see how this goes!

Big Kalydeco Day!

Today was another big step forward in getting Kalydeco in my hands! My CF mutations are dF508/R117H-5T. Today the FDA had an advisory committee meeting to discuss the results of the clinical trial of Kalydeco in patients with the R117H mutation. The committee voted to approve it, 13-2! Now I have to wait and see if the FDA will decide to go along with the committee's recommendation, which it usually does. The FDA has until the end of the year to decide.

I was almost in tears listening to the testimonies of people with R117H who have been on the drug. They all said they can't imagine going back to the way they were before Kalydeco. They all said they can now plan their retirements. They all said it was a miracle. I can not believe I might be able to feel that difference soon - to not cough anymore, to not be so tired and achy by the end of the day, to be able to fully breath out of my nose and really smell things again! And to have the confidence that I will be here to help raise my grandchildren :) Crossing everything!