For people taking Orkambi

[saintoffeon]

there is a lot less mucus in my lungs now. I am questioning whether clearance is useful or not anymore, because not much comes up.

First of all, I am fabulously happy you found a solution that works for you. But this particular comment interests me, because the way I feel right now, I'd just die to see an X-ray...it feels like it would be many many shades darker than anything that I've done in a while.

I think I mentioned before that I haven't generally been mucus-productive in a long time, years really, and that my first two weeks on Biomark...er..Orkambi were more productive than the previous two years.

It's now come down to days where I cough up a couple things, seemingly nothing if I'm not paying enough attention, or then a day or two when I'll bring a few good things up.

I've started using old duoneb because of my doc office/pharmacy problems, to open airways, but even if I can get the Pulmozyme back...well, I'll still feel it out and try it...but it does feel super weird, like, what am I breaking up/cleaving?

The vest makes me feel good either way and I like how it's helped me get into a more structured habit with my health, and presumably there could be even more I'd be freeing by having more methods of attack (in other words, the faucet has only appeared to stop dripping, because I've taken just these meds as far as they'll go in terms of clearance)...so I'm going to chance it unless it seems like it could harm me, but I can't imagine it will. Pulmozyme seems really inert. Most documentation I've found says you can even drink while taking it (I don't, but hey...some people =)

 

[jamest]

jamest, Thanks so much. That is good to know. When you talk about the clearance, do you mean the vest etc? The reason I ask is that I'd say even if you aren't brining up anything keep doing. With young CFers there's nothing to bring up yet but it helps anyway to keep them healthy.
Re the tapering: Did you go back to 2 a day all the time or did you do 1x a day, then 2x a day every few days as you built up to steady state of 2x a day???Thanks!

I do vest and flutter. The amount of effort required (huffing, etc) no longer seems commensurate with the amount of mucus coming up, as compared to before.

Tapering: I went down to 1 a day for two or three weeks, until there were no perceptible side effects. Then went up to 2x/day for a week or two, and didn't see any side effects. After that I did a full dose, no side effects, but didn't see anything like a result until several weeks later. IIRC I felt the same way on the 661 trial, but could tell immediately when I went off the drug.

 

[DjFunkyFife]

I'm on my 11th day of orkambi, and so far, nothing extreme. I did note a few days ago that i think it's giving me slightly lower blood sugars in the morning. Averaging 96, which is down from the average of 108 i had before orkambi. I also think it has SLIGHTLY lowered my appetite, but i am not losing weight... in 11 days i'm up a couple pounds. So that seems to make me think i'm digesting food slightly better. (i've also had better bowel movements (the stomach issues i had the first few days died down, i notice no bad issues now)) Also, i believe it has helped somewhat in my hydration. I have hardly gotten any headaches in the last 11 days, and the ones i have gotten, i know are from sinus, because they've been accompanied by sinus pressure.

 

[Gammaw]

DJ - it sounds like you're doing really well! That's so good to hear!

 

[saintoffeon]

Just wanted to chime in and say, as of Day 46, I am getting even better.

Before it was like, whoa, this is so super different from the nightmare crap when I first started, and even a bit better than before I began...

I have to keep doing all my treatments for it to persist, but when I'm staying on top of all of them, it is, by comparison, like I'm not ill anymore. In other words, I can't know or imagine what the difference would be between me if I never had this, and me on these things.

Granted, I still can't do what others can as ably, but I don't need to, and I don't often try a lot of them...plus I'm still improving, nudge by nudge.

 

[Gammaw]

You had such a rough start SaintOffeon. Its is wonderful to hear you're doing so well!
Blessings.....

 

[saintoffeon]

Thanks! You know my favorite part? No more freakin' bleeding =P

Probably helps that I've worked out enough to build up better lung function...someone told me once that the blood rushes in to keep them from collapsing sometimes if you overwork them, even if you don't have my exploding-artery problems...

 

[Gammaw]

Whoa! How wonderful!

 

[saintoffeon]

Day 54...

Just chiming in to report I have had my first 98 on pulse ox for the first time in...well, since I can remember. Probably a couple years.

Also, finally back to my pre-Orkambi weight. All fifteen pounds are back, and they look better than ever.

 

[Gammaw]

Wonderful! Any bleeding?

 

[saintoffeon]

Not a drop. Not even in the mucus as streaks or anything. This is a whole different planet of existence on this other side. I feel like everything before was kind of sleepwalking. My other non-health problems haven't even changed all that much, and I'm just able to be better at life because there's not this dragging weight hurting and bearing down and stealing energy.

It also makes a huge difference having the capacity to exercise consistently. Unless they repeat with another miracle drug (like the triple-combo therapies or an alternate means of doing the same thing), this is as complete and authentic and 100% me as I'm probably ever going to be, and I'm loving it.

I mentioned something similar before, but it's SO interesting seeing what was me this whole time and what was an affectation living through the filter or medium of a less-healthy life. Similar to chronic pain sufferers. If they find reasonable treatment, it's like getting 20% of your brain back. They have focus and clarity that seems supernatural, but really they just are able to do their normal natural processing unobstructed.

I'm literally still in shock that this was on the table somehow as a possible experience. I pray constantly that Orkambi continues to work magic for people, and that for those folks who've yet to mesh with it (or stopped) that they get some similar experience. We're all putting in our version of the same kind of fight, and thinking that the rewards are coming in is just super exciting.

 

[scanboyd]

This is day five for me doing 1/2 dose of Orkambi because my Fev1 is 25% with FVC 100%. Day one and two were very productive cough much more than normal. Within two hours of taking first pill I could tell there was a significant difference in the viscosity of my mucous with increased productive cough. My chest has been tight and EXTREMELY TIGHT at times. This started about 12 hrs. after first pill. O2 stats running about normal 96-97-98 at rest. Last night went to bed, chest was very tight, colder than normal, wore PJ's which normally I do not. I had night sweats. Woke up this A.M feeling good and chest tightness is GONE. If all goes well I will start a normal dose next wk. I have noticed slight increase in productive cough through the wk, nothing like the first two days. I have never ever had the tightness in my chest as experienced with Orkambi, even thought about stopping Orkambi . I was told by the Dr. it would get better and thank God it did over night.

 

[saintoffeon]

Scanboyd, while the number of vocal people online skews heavier for those the drug doesn't work for (or that they stopped out of concern), there are definitely some home runs. Out of all that I've seen, dozens and dozens, maybe two people got along okay without any real side effects. Everyone else (and keep in mind I'm referring to those it turned out well for), had some horrible time span of indeterminate length where the sh-- hit the fan.

Granted, that's not scientific, since it's just the sample of people I've come across, but I think at this point we can safely assume that being beat up by this pill is par for the course. I am, again, not a scientist, but I have to imagine it comes from making drastic changes in short time spans to damaged areas.

Personally, I didn't know how bad I was beat up by the ravages of CF time until I took my rusty old lungs out for a drag race on this stuff. Grocery bags of mucus, pleurisy, coughing up blood like I was being tapped for maple syrup, chills and fevers, pretty much being knocked out for several hours at a time early on because I was so weak and tired.

........but........

I am so far gone from where I started pre-pill that I feel like I've had a life-changing event. I almost cannot relate to my former self of just a few months ago!

That was just after Day 20 where it started creeping out of the woodwork. I'm only on Day 55 today.

It may be three weeks or a month. Maybe six, maybe a year. Go as far as you can and feel comfortable with. Listen to your body and manage your health and safety as you always have. In other words, "don't be a hero" and get yourself hurt.

But, apart from that, endure as much as you can, even if it sucks, because midway through, on day 10 or so, I thought "What have I done!? I traded out feeling bad for the possibility of feeling well, but if I stay stuck here and it doesn't lift...I'll have trapped myself in a worse than bad purgatory forever!"

I didn't contemplate hurting myself, but my already decreased appetite tanked out completely when I got situationally depressed, thinking of my quality of life staying in that phantom zone.

Now, I'm building muscle, knocked out 3 5Ks, and feel happier and more pain free than I have in my whole life (which is nuts, because my FEV is 43...). This is a life worth living, where each day brings enjoyment for the sake of the day's events. Not limited to what the past has demonstrated for me, not trapped in my thoughts about an uncertain future.

Just...normal =)

 

[scanboyd]

Thanks for your words of encouragement saintoffeon. I have no intentions of quitting Orkambi, just wanted to describe my experience. As stated I woke up this A.M with NO TIGHTNESS in chest unlike the previous four days. Good luck and hope you continue to do well. I did not want to post until I had something positive to say.

 

[scanboyd]

Good Sunday morning!!
This is day 7 on 1/2 doses of Orkambi. Spit up some dark mucus, looked old with bright blood last night, none today with neb Rx.. I have not spit blood up in many yrs. like 25.
Still having some night sweats.
NO TGHTNESS OF CHEST. Still some what winded as compared to pre Orkambi. Going to gym tomorrow only went one day last wk. due to extreme tightness in chest. I am thinking about going to 3 Orkambi a day starting tonight depending on how I do today. Have a great Sunday!

 

[saintoffeon]

Good luck scanboyd.

I hit my pre-Orkambi weight again, which makes it feel like I'm on the rise overall (like they say about the stock market--viewed at an instant it looks turbulent, but even the ups and downs collectively shift up over time)...

plus after grocery shopping on Saturday morning, I ran across half the parking lot...and didn't have any recovery time! A few catchup breaths, but not winded, didn't have to sit down.

Tried again last night during an evening walk, shorter distance and more of a jog. I think I'm getting strong enough that my body doesn't see that kind of thing as as much work, yet, so it's likely a combination, but a so-very-cool one =)

 

[scanboyd]

This is day 9 for me on 1/2 dose Orkambi. Feel great with no issues with Orkambi, plan on starting 2 Orkambi tonight. Chest seems clear at this time. Mucous is definitely thinner than normal. I get great results when doing neb Rx with mucous being thinner.
Lets see what the next few days bring my way !!
I have to say the 1'st week was tough.
Good luck to all of you folks!!

 

[Printer]

scanboyd

I'm really glad to hear that you are doing so well.

Bill

 

[scanboyd]

Thanks Bill, just trying this Orkambi. Or maybe the Orkambi is trying me? Hope you are doing well. Bundle up and stay warm winter is almost here. Take care.

 

[scanboyd]

Day 10. Took 2 Orkambi last night, had lite night sweats with SOB which I normally do not have when in bed lying flat. Still had SOB when I woke up this am but that has gone away about a hour. I took one Orkambi this AM with breakfast so I am now up to 3 a day. The symptoms that I am experiencing with the 2 pills of Orkambi is Just like what I encountered with the one in am and one in pm. all of which subsided before I started the 2 pills last night. My mucous is definitely thinner. As a side note when I have the SOB when lying down I use 2 L. of O2 which makes it tolerable to sleep. I will start the normal dose of Orkambi as soon as my body adjust to the 3 a day regiment. Me having a FEV1 of 25, this is dose pattern that I chose after seeing other folk experience.
Headed to gym this AM for 90 min. work out after done with Neb. RX.