For people taking Orkambi

[Aboveallislove]

Just wanted to say thanks for taking the time to provide all these details..I know it takes time to write it all up for us but it does help so much reading. I do hope you turn the corner soon and push on through.

Day four was kind of impressive. Nothing ancient or hairy or gross came out, but I easily did a month's worth of productive coughing in the past few days, bolstered by my seemingly unending stream of sputum spitting I was engaged in. Big session in the morning, big session in the afternoon (also very weak..once I got home I crashed and slept for three hours... I was COOKED) and decent session tonight.

It sounds weird to say but I am kind of excited because it's just a few days in and I see more change...it's not pleasant-feeling in the moment, but in my mind I'm fast-forwarding to, say, seven days, and wondering about what other bigger scarier gross things might leave me. Let's clear the table! =)

Also, this is the first day I was involved in any non-walking exercise. After my nuclear-level weakness and sleeping through dinnertime, I had enough energy to do some light cardio. Timing-wise I needed to do my vest to be able to take my drug on time, so I cut out early (also, they moved to doing a lot of on your back stuff, and I have trouble breathing and not coughing like that)...but what I got done was more than I thought I could do and hopefully helpful to the cause. Probably a solid 12 minutes of legitimately moving around and working things out.

I have been seeing a lot of bitter/jaded comments about the drug on social media. It's absolutely okay to react that way; in many ways it seems less and less like even the compromised version of what we thought it would be. Just remember not to dwell on that and live in that place for so long it becomes unhealthy.

We've all spent our whole lives fighting things and being bent over, so we're tough enough to live through a false positive with our character intact. We also don't need any extra stress that might cause other health problems.

Aside from trials, though, the most anyone's been on it should only be about two and a half months, and the trial results, while modest, took place over 24 weeks. I really believe patience will be rewarded. I think it's doing its job for everyone, in terms of mechanism of action, I just think there's SO much going on with each individual case that there's no way to tell how it'll play out.

Sometimes when I'm in the hospital they give me MucoMist and I say I don't want to because it dries me out, and they say, "No way, the book says that's impossible," and then they ask me to cough up something to be cultured. I can't, it's all dry in there now. It may be similar to that, where with the fifty thousand meds we have to take, the playing field is changing so much that ever-so-slightly-thinning-mucus has more of an uphill battle to produce any obvious effects.

For what it's worth, I am not backing this medicine or defending it, and I will be the first to support anyone upset by it. I'm literally throwing ideas out, and nothing I'm saying is intended to be professed as opinion. Just openly discussing theories =)

I could be taking this forever and not get any better and my views will remain the same. For as expensive as it is, you'd hope there was a better chance that it would override every other thing wrong with you to be clearly doing its job in an overt fashion, with a much more increased likelihood of overall success, but we also can't get caught up in that, this time, that may not be the case.

That's why I am trying to focus so much on what I can do with what energy I have. I think it not just benefits from the help, but may need it. It's like when you see those diet pills, and people say, "Oh, I just took it and in twenty weeks I lost 140 lbs." So you look up the drug and there's a note that says "Take one a day, and then diet and exercise." Hmm...maybe you can take anything and diet and exercise and it'll help. Maybe that's the first time they dieted and exercised, and that has more to do with it than anything. Credit doesn't go where it's due, but hey, whatever works if the end result actually happens.

Cheers to you guys for staying strong. I hope we all come out as well as possible through this.

EDIT: Almost forgot. I don't know if it's an aberration, so I was waiting a few days to see if it was the case, but I'm a lot more regular, consistently, in these past few days. None of that classic paint-stripping CF grease that can sometimes accompany our bowel movements. Also very easy to pass with little strain, and up until day it wasn't too soft or watery at all. Having just had a bout of fecal impaction prior to starting, since I stay hydrated enough, I'll take that over the alternative any day. I have always been more successful without pancreatic enzymes though (a secondary reason, aside from trying to prove eligibility to get into clinical trials for this medicine​, that we went through with a blood test/genotyping to confirm the baby diagnosis, based primarily on "failure to thrive" and sweat chloride off the charts)...so I'm not sure how that will play out for the tons of other people who need them to live.

 

[uncjenny83]

I wanted to chime in with my experience so far, since reading this thread has been really helpful for me! Background- I am 32 and my baseline fev1 is about 41%. My health has been doing unusually well for the last couple of years. When I started Orkambi on Monday, I'd guess that my fev1 was about 44%.

First day day was not bad for me, I was slightly more SOB than usual and a little bit more tired. I had a fairly productive cough that afternoon, my mucus slightly thinner. I'm on day 3 now, and I'd say that while I'm definitely noticing some increased SOB and fatigue, overall it hasn't been too bad for me. I certainly don't feel anywhere near as bad as I do with an exacerbation. I've had some waves of nausea, but they are slight and pass quickly.

The kind of weird thing is I really haven't coughed much up at all since day 1. I have a little bit of a cough (not much at all), but it's fairly dry. I'm very grateful for the mild side effects, but I did think I'd be getting more junk out already! Maybe I'm just being impatient .

Quick question- how strictly are you adhering to the every 12 hours? Is it a big deal if it's 10 hours instead of 12? I'm having a little trouble doing the 20 grams of fat thing on a 12 hr schedule.

 

[Ratatosk]

For the most part we've tried to have about 10-12 hours between doses. DS has his breakfast around 7:30 and then has supper around 6-6:30. Weekends if he sleeps in 8 or 9, I try have a late supper. If he's been snacking all day and isn't all that interested in supper, then I'll try to give it to him with some popcorn or Hershey bar and a glass of milk.

I thought the product literature said something about if waited longer than 6 hours from the 12 -- so 18 hours between, to skip the dose...

 

[saintoffeon]

I thought the product literature said something about if waited longer than 6 hours from the 12 -- so 18 hours between, to skip the dose...

I have it handy in the other room:

"If a patient misses a dose and remembers the missed dose within 6 hours, the patient should take the dose with fat-containing food. If more than 6 hours elapsed after the usual dosing time, the patient should skip that dose and resume the normal schedule for the following dose. A double dose should not be taken to make up for the forgotten dose."

 

[saintoffeon]

Day 5 was scary! I woke up last night around 2 in the morning, and in a matter of about 10 seconds I went from just being tired and uncomfortable to feeling soreness then a popping sensation and then doubled over in pain. I couldn't make it better or worse changing my position and it became really, really hard to breathe.

I have a test I did when my lung was collapsing, which is to breathe in and hold it, and then breathe out and hold it. When my collapse was happening, that was impossible; there was too little breath and I needed to "manually," consciously, control each breath in and out instead of it being involuntary.

Fortunately, that wasn't the case this time, but the other thing it felt like, more, almost exactly like, was the pulmonary embolism I had in 2009. Back then, I went to my primary care, and he sent me to get a cat scan, and the radiologist walked out and stopped us from leaving and told us to go to the ER. So I wasn't taking any chances and we went to the hospital this morning.

After being brought to an ER room immediately (thanks!) and seen fairly quickly, they got me to a scan in a decent amount of time and then we waited approximately five thousand years to find out their imaging machine broke and the doctor hadn't gotten around to receiving a report from radiology.

Again, fortunately, no PE. But it was crazy painful, very localized, just south of midway down on my back on my left side. Almost like a tiny ball of almond roca, with, like, a pulse or something, was slowly rolling around in me. My pulse ox dropped from 96 to 92 in a few minutes, so they put me on oxygen just to buffer it. It went back up to 96, then 92 while on oxygen.

They told me it was most likely a pleurisy and to have a nice day =P They said my productive coughing day yesterday and the frequent muscle activity involved may have been the source, directly or indirectly, for my lung lining to get inflamed.

I was floored. I always feel horrible and then they take pictures and nothing is wrong, and it's embarrasing, but I always still go because better safe than sorry (I can't believe how bad even a simple infection can hurt!)

But I'm obviously thrilled it's not a PE. If not for the not dying than for the not going on blood thinners for a half year again, and the constant valve-collapsing pokes multiple times a week to check my PT/INRs.

I wasn't productive cough-wise the first half of the day, but after I escaped I did pretty well again, and some thicker, really dark, little flecks of blood stuff came out. Nothing dry or scaly or old still (my ENT calls them "crusts"...ew) but still very glad to be rid of anything gross. Go live somewhere else! =)

Not stopping taking this stuff. I'm earning my stripes. I obviously don't want to go to the hospital every day thinking it's trying to kill me, but I also think this is probably a one-time deal and I can be more judicious about what's worth going over there.

 

[saintoffeon]

Day 6:

"O, is for the 'Ow' I yelled, twelve times,
R, is for the way I ralphed, up slimes
K, is very, very, Kalydecordinary,
A, is even more than I thought I had signed up for

M! is all I say when I, can't breathe
B is biting on fat, I teethe,
I am almost done with my first week, and
Skepticism please don't deepen

ORKAMB-I was made, for me and yoooooouuuu..."

--Frank Sin-snot-ra

Low fever last night in the middle of the night, no recurring horrible pleurisy on the order of my hospital visit, but a real pain in the butt headache (you know, when you wanna grab a drill to release the pressure? =) Not as antagonized during the day, but a couple hour sleep chunk "recovering" on my way to get actually awake and productive, then slowly coughing and fighting for each breath through my regular day. Felt very "scooped out."

I firmly believe it's still all part of the process...it's just, wow, if this stuff ever comes out, assuming it's working its way out, it might have a face and children and car keys. I will not be surprised.

On the plus side, even more dark and color goopy disgusting crap came out, fairly regularly. Not multiples times as much as the days before, no orders of magnitude greater, but I'll take consistent, at least! Clean that house!

 

[Ratatosk]

Just wondering with your headache and feeling of pressure if it might help to do sinus rinses to help get the crud out. DS rarely coughs, but does have more sinus issues and I know he said he got some nuggets from his nose after starting orkambi, which he was thrilled about (grossness factor). I think he was disappointed that nothing all that interesting came up and out

 

[aslong]

I had the headache on day 2... I also get the pressure when I am coughing and it does not let up when I am done! I was doing that before Orkambi at times as well... I thought I must be a good cougher, I have all the muscles working!

I just took the last dose for the 6th day...dose 12. I have had chest pain like crazy... I can feel stuff in there. I cannot do enough Physio to get it out. For the soreness I use Sambra, I got it off of Amazon and my physical therapist recommended it over biofreeze. I love it, I guess it is the same ingredients as pepper spray. It really relieves the pain from the back and i dabb some on the base of my skull it it helps with the pressure headache. Might be worth a try. It lasts a long time, I put it on last night about 10pm and it is now 12 hours later and I can still feel it.

Best of luck- Maybe our lung people can hang out somewhere else together when they finally get out

 

[saintoffeon]

Starting Day 7 early and will come back to edit....because...

I feel GOOD.

Don't get me wrong, if I tried running I wouldn't get very far, and I'd feel tight and congested.

But, like, my limbs...I haven't been doing much exercise lately because I've felt so bad and yet I still feel nimble and flexible, and less inflamed...

My back is sore and I'm nauseous, but I can hold a note...not for any longer than before I started, I don't think, but maybe things are starting to clear out? Heck, maybe it'll all be bad tomorrow again but I'll take the break =)

But yeah...I just feel cleaner and simpler and more basic...even though I'm tired and hurt it just "counts for less."

Hope this sticks....

 

[aslong]

Saint, I think we are on the same pattern! I take the 1st of day 7 tonight... I also feel pretty good right now. I have more energy and can take a deep breath. MMMMM....

 

[Callista15]

I've been on Orkambi since Monday... the first day I didn't notice much but the second day I was immensely tired... I usually have no problem getting thru the work day and a workout afterwards but could barely muster the strength to get through... I was asleep by 8 oclock the next night and i usually suffer from insomnia. My husband thinks this is the best thing ever for me! Its been a bit better today...however...I'm itchy as all hell. I haven't read anything about this as a side effect. It could be the dry desert air but it's definitely been worse since Monday... no rash (saw that as a side effect) but my skin is beyond itchy and it takes awhile for it to calm down. Is anyone else experiencing this/??

 

[mander31880]

I started taking Orkambi on 9-24. The only symptom I've had is some moderate shortness of breath and slight leg pain. That was only after the second dose. I'm 35 with a FEV of 42%. I was expecting some at least moderate symptoms. Is it possible they will come? And my fear is, if I don't experience these changes or symptoms, does that mean the drug isn't working as well on me? Lol. I'm worried that I'm NOT miserable starting out.

 

[imported_Momto2]

There is a subset of people with ddf508 who have a protein that blocks the effectiveness of Lumacaftor. (published in Science Signaling, Doutor Ricardo Jorge)

 

[saintoffeon]

Days 8 and 9

Sorry to disappear...I'm back! Was across the state seeing my cousin in a play and trying to stay awake ....because I am sick, not because she's boring. She did a good job! =)


Not much to report. Still tired, still beat up, still draggin' butt, still coughing things up (thankfully)...still looking forward =)

Right now the biggest thing is adapting to the changes and needs of regular life while feeling half as good as the half-good I normally do....regular life isn't easy to begin with, but it's definitely its own experience at quarter-power =)

Hoping and feeling like the breaking point is within a week or so...or at least to feel what I did before I started...real life doesn't care if you need rest, because at least in my real life there's no opportunity, because even when you're awake and feeling well you can work phenomenally hard and be a good person and still struggle to make ends meet or live a life.

I can't believe how well this stuff works at getting stuff out of me. It's annoying, and gross, but each one makes me so happy. It's like unwrapping a gift, except it's more like you're creating and giving the gift, to nobody, because who would want it, but the giving makes you feel as good as a regular gift would either way! =)

Cheers...

 

[benthyr123]

I have been taking Orkambi for about a year. I was in the Traffic study, then the extended study. I started the commercial version in July. I'm sad to say that I stopped taking it on Sept 18.My numbers had been declining and my headaches were getting worse. The day after I stopped, no headache. Hmmm. I'm not saying I won't ever take it again, but it did little or nothing for me. I'm currently at 38%. When I started this drug I was baseline 48%.

 

[saintoffeon]

I have been taking Orkambi for about a year. I was in the Traffic study, then the extended study. I started the commercial version in July. I'm sad to say that I stopped taking it on Sept 18.My numbers had been declining and my headaches were getting worse. The day after I stopped, no headache. Hmmm. I'm not saying I won't ever take it again, but it did little or nothing for me. I'm currently at 38%. When I started this drug I was baseline 48%.

Would you say you might have been worse off if it hadn't been around? In other words, what was your decline like before versus after? Is your current baseline different than this last reading?

Would you mind sharing any other broad aspects of your regimen so we know what goes into the mix, like Pulmozyme, vest, etc.?

I'm not advocating, by the way. I couldn't; I just started myself, and reactions seem very mixed, and I'm not a scientist to know better either way.

 

[benthyr123]

I should have elaborated more...sorry. I'm 51, my baseline has been in the upper 40's for the last 7 years..even reaching 51% shortly after taking kalydeco (the first Vertex study) I'm not new to Vertex...I believe in them totally! I just don't think this combination is for me. I was in the hospital for the first time in 4 years this May...while taking the extended study drug. My FEV1 dropped to 36%. With two weeks of Ceftaz and Tobi I got back to 45%. During my hospital stay I continued on the drug. I got the commercial version in July and immediately noticed and increase in my headaches, fatigue and my BP went up. I started taking medication for that also.
I've always been pretty healthy, so I'm disappointed in the general decline over about a year. I have always taken Pulmozyme, Zythromax and Symbacort for daily meds. This year I started using the Vibralung.
This is only my experience and I'm thrilled to see that other people are benefitting from this drug. I do believe they will continue to improve on this and VX 661 holds more promising data.
For right now, I'm going to try getting my lung function up without Orkambi...I always have the choice to restart.

 

[saintoffeon]

Thanks for the detail! Really helps flesh things out.

It does seem like bad news if you stayed in one place for a really long time and then plummeted.

Are you saying you were able to get access to meds for both genotypes, then? I think there was a group of folks with Kalydeco, right, where they wanted to see if it helped even if you didn't have the rare mutation? And that's when they started focusing on a double-delta specific one?

 

[ksy8384]

Hi, I am a 48yo ddF508 and I started this drug on Aug 18th of this year. I actually took my first dose in the car on the way to Disney World. After reading about all the sIVthat was different than normal. I didn't get the pop, tickle, then cough, tickle, cough etc. Basically I didn't realize till I was coughing up clots. My cough has decreased and I actually feel pretty good. I had some shoulder pain and thought it may be related but it isn't. I do get SOB but thought that was the drug. Imagine my horror then when I went to clinic Tues and my pft's are down from 41% to 33%. Baseline is about 45%. So I am to start IV's if they ever get here and see if the numbers come up. If they don't I stop the drug to see if that helps. Now I see why I was so SOB as 33% usually does that for me. I too have issues sleeping where as not normally even when I take Ambien.

Kalen

 

[saintoffeon]

Day 14...

Sorry for yet another absence.

I have had a pulmonary embolism before, I've had a lung collapse before, and I've been completely fecally impacted twice.

Those were very painful. The collapse was itself especially scary.

That said, it was this one big focus and then you dealt with it.......stay focused, hope and pray you get better, conquer the fear, etc...

But in terms of feeling used up and beat to sh-- this takes the cake, at least for anything in recent memory...

Running out of breath sitting in place, having to consciously control them....no position feels good, no sleep, LOTS of blood coughed out...I had to sound crass but I do feel a bit like a used condom....and for, metaphorically, very similar reasons.

I feel like I don't even belong among fully living people right now.

I still feel like it's going to go in a positive direction...but hot pickled scratch, this is tougher than I anticipated.