For people taking Orkambi

Gammaw

Super Moderator
Saintoffeon -Do you have a reasonably close Adult CF Center? And a regular everyday doc, like a family practitioner or Internal Medicine Specialist? They would coordinate referrals, diagnose and treat everyday problems. They can also help run interference with your pulmonologist when you're not getting responses. But really if you have a pulmonologist at a CF Center, I cannot imagine waiting a month for a response to an acute problem. Not good.
 

saintoffeon

New member
I was told about proper ones in Tampa, Gainesville and Jacksonville. I'm in Melbourne, so Orlando's the closest big place, but everyone I try to contact there is either pediatric (like Nemour's) or can't give me a straight answer on whether or not they're a proper clinic, how they identify, what services they provide. Not just in terms of how I'm referred to them, but even if I call them directly.

I have a great primary, a good listener pulmo who's very experienced and also is certified as an internist, and then it sort of fragments for people I don't see regularly (general surgery, GI, blood, ophthalmology, etc.)
 

Gammaw

Super Moderator
I'm glad you have a great pulmonologist. But coordinated care can really step up your medical care standards. Here's a link to the Cystic Fibrosis Foundation website"s locator service for Approved Care Centers.

https://www.cff.org/CareCenters.aspx?id=2397&page=1&state=650

If the link doesn't get you there, go to www.cff.org, then go to Get Support" and then "Locate a CF Center". After you put in your State, or zip, you will get a list of both adults and children centers, as well as Affiliates - which are basically outreach or extension offices so you don't have to travel so far. For your first visit, I would skip the Affiliates until you establish yourself at the Adult Center. You might even fund your pulmonologist on the list, but there should be other services available thru the CF Center that can help you. I've been concerned with several of your statements about what you've been working with. They also generally have people who can help with funding your care if necessary. Please call them.
 

saintoffeon

New member
I've actually burned through that entire list a few times, but it never hurts to try again. A couple hours on the phone is definitely worth sticking around longer.

Thanks for the assist, and the caring and watchful eye =) It's much appreciated and genuinely helpful support; it's symptomatic of what has always been a remarkably compassionate and generous community of individuals.
 

Oboe

New member
Shands at UF in Gainesville's probably the best in the region. I was with them from when I was 4 to when I turned 21. I never got enrolled in their adult program for mostly personal reasons. I go to the one in Jacksonville now, and it's small but pretty good. One of the best RNs and CF coordinators we had went down to Port Charlotte. I'm not sure if she specifically works with CF anymore, I think she does outreach in Sarasota but I couldn't swear to it, but she was seriously amazing when I worked with her.
 

CF in TX

New member
Out of curiosity, how were you able to notify them? If I call and tell my doctor's office my skin is on fire and spider eggs are falling out of my tear ducts, they ask me to make an appointment for the following month.

Personally, I have a very good rapport with my CF team and typically I can call in, speak with a nurse, and get a response within 24-48 hours depending on the Drs schedule, especially if I express urgency. In this particular case, I expressed a great amount of urgency because I was worried I was having another exacerbation. I will say though, my clinic wasn't always that responsive so a lot of relationship building has gone on between both parties to improve our communication and increase response times.

The best piece of advice I can give you is to have a conversation with your pulmonologist and discuss with him and his team ways to improve response times. Surely they will understand that their can be a higher sense of urgency for CF-related patients than say, someone who has asthma and is trying to get a bronchodilator refill.

Glad to hear you think it's progressing but I'm sorry for how long you spent feeling crappy.
 

saintoffeon

New member
Surely they will understand that their can be a higher sense of urgency for CF-related patients than say, someone who has asthma and is trying to get a bronchodilator refill.

The funny part of that is, years ago, he used to say things that basically were equivalent to, "You need to suck it up, man up. I have other CF patients and they look like they're F'ing zombies next to you. Why are you complaining? Fight while you still have strength!"

Then I was just in last month and he was like, "Oh, I hardly have any CF people now...maybe three or four other people in the whole county so I'm not totally up on the new medications. You're definitely the worst off, though, they're actually pretty fine, pretty far ahead of the game. So I'm always open to hearing how you'd like to proceed, what your perspective is on advancing your care."

When I was a teenager I felt silly because they'd always put me in pedatric wards. Now, I'm an adult, and I feel silly because I am a grain of black pepper in a salt shaker, with seventy-to-ninety year old people on special machinery, wheelchair-confined, etc. Sallow, empty skin, no muscle mass.

I'm not especially heavy, 145-155, a hair under six foot, and 31 years old. Decent posture, full control of my limbs. So I always feel guilty, or like I have eyes on me, walking into those places. Young people, generally speaking, ought not to be in a decrepit-lungs place, so there's people who got diseases, or made choices that led to one, or it all caught up with them, etc. And I wonder if they're thinking, "What kind of problem does he have? He looks fine."

You can't play the peeing contest over who has it worse, but anyone with CF knows (as it's all they know) that it's not a fun game to play. So you feel like you've earned your sick-people stripes enough.

But wow, if that's a preview of what's to come, on my worst day some of those people help give me perspective.
 

saintoffeon

New member
Day 19

I find this amusing, because I started on 9/19, taking my first dose at 9:19. And this happens to be the first real day of note, Day 19.

I am getting better.

Not necessarily better than I was, but WILDLY better than I've been. The progress day to day in the past half week has been at least 10-20%.


It is hard to fathom how today would even be possible last Tuesday, let alone that it would come in just over a week's time.


My first dose was so harsh in terms of shortness of breath, that while I am still short of breath now, I think it's actually better than it was after the first dose.

The other day, while getting ready for work, I was fixing myself some cereal, and every time I went to grab something, like the box or the milk, I had to sit down in the dining room and recover. I was looking up poses and postures to accommodate shortness of breath, open up the airways, because I was psyching myself out.

Later that night, despite not being able to cross the kitchen in the morning, I told my family I was going for a walk. They were surprised, because it was later in the day, I was tired and had already moved around a ton. "It's just a short one. A very, very, very short one," I said.

"So, like, to the stop sign and back?" We live on a cul de sac, so I nodded; it's about three hundred feet away.

They told me they'd go with me if I could wait for them to finish their respective activities, so I did. I like my family and they're good company and always supportive.

I didn't expect to make it halfway, one way, -to- the stop sign. But I did. It was hard. I don't mean, I did eight pushups and the guy on the tape says two more, but it burns. I mean, I can't forge a mental connection where it makes sense how I'll get any further. It's not just fatigue, it's almost like there's a psychological component. Like you can't send a strong enough signal to convince your body that moving forward is a good idea. I think I mentioned before, I literally tell my body to kick out....if I were normal and healthy, it would result in a goofy march, but with CF on Orkambi, it was just enough to produce baby steps.

The ONLY way I have been able to deal with this is not my old habit of fighting in the sense of toughing out being abused and victimized by my ailments....but fighting in actively summoning willpower to overcome how overwhelmingly sucky it is. In other words, when you're kicked and your down and you can't muster the energy to push yourself back up....I just kept trying. Most of the time I stayed down =) but other times I got partially back up, or more...and collectively, I think it's been adding up, because I haven't left anything on the table, and it's all I COULD do (this is just my personal experience, by the way...nobody knows what any of us is truly going through but us, so this is no reflection on anyone else's experience...I am just trying to be candid about my mental state)...

So tonight, I made it farther. I had to stop a few times, where as pre-Orkambi me could have done the same distance twice over in about the same time. I'm not "there" there...but it's a cosmic leap from the middle of the days accrued so far...

Restorative sleep has been a huge thing for me (thanks, melatonin chews!) and today to fight the aggravating back pain I picked up that special arthritis Tylenol (yellow band, 650mg apiece) and it made me sweat but gave me a couple hours of relief...

But there are changes taking place right now that are notably different....they aren't meeting an expectation, because I don't have one, because I don't know what one to have...but, even compared to less damaged younger me...these are NEW feelings...I'm not even saying it's good (though it's not bad), but it's a wholly unique sensation.....a soft, comfortable feeling but still very physically "connected" in the sense of muscle to bone, mechanics, etc.

And it's really narrowed down to back and chest. With more exercise I think my outside bits are really close to home base. Today is the first time in two weeks I've felt well enough for exercise (minus the walking). Granted, it was literally just standing in place and doing different flexes watching a DVD a family member had on, and doing it until I ran out of breath and had to sit...but it was something, and it was more than I was doing.

The best way to explain how I'm feeling now is that it's as if I just got out of the hospital. In other words, I don't feel AMAZING, but I've got lots of bad things flushed out, I'm relatively stable, and my primary pain and discomfort are mostly from the weakness of not getting to move around or sleep.

I can tell you that I am far enough along that I'm starting to plan what I'm going to do to celebrate what it will be like to see the other side....like, even if I just get back to where I was before I started....singing, jogging, whatever. I am able to contemplate those things as realistic and plan for specifics...That's an amazing sign...an amazing possibility...an amazing turnaround.

Maybe it all goes away tomorrow, but since I don't know, I'm going to wear my naively optimistic cap and just push forward like a mofo like this is my one window of opportunity...
 

Gammaw

Super Moderator
Heaven Bless, Saintoffeon. Thank your pulmonologist for years of ....... whatever he's done. Tell him - or not - that you're following his other CF patients to an approved CF Center so you can make it to the age of his other patients. Get thee to an adult CF Center now. Call them tomorrow and make an appointment. We'll be looking for your post tomorrow night.
Blessings!
 

cam

New member
I started it bout 3 weeks ago and last few days cant breathe and on my incentive I can only get half of what I used to and since startrit my back is totally covered in acne I hope this isnt another hospital stay I just got out 2 months ago my back is hurting to when breathing in but anyway has anyone got major break outs from orkambi thanks
 

saintoffeon

New member
Gammaw — It's down to two... PCCA in Jacksonville, which was well regarded by the Mayo Clinic when I was up there, and Dr. Layish's office around Orlando. I've got recommendations from both (some of which are corroborated here and in other threads) and they're being vetted for insurance coverage. My policy is really, really nice, but they're a bit sensitive to the different stages of referrals and such.

More broadly, today I feel even better than yesterday. In some ways I am experience things I've never felt before, not even when I was better / had higher FEV when I was younger.

My body feels "calmed down," like more at peace. More just like a costume my skeleton wears, instead of some giant meat armature, the sensations of which I am hyper-aware. Even in my weak, unexercised state, and even with shortness of breath a factor (though less OF one)....I feel good!

Tomorrow night might be my last journal unless things get ridiculously better or go back to being horrifying. Where I'm at right now is in some ways superior to where I started, because while I wasn't as weak and didn't have the shortness of breath, I didn't have this new thing going on that makes me feel more "normal"...I bet if I blew right now I could only scratch the 43 I started at...so it's probably not much of a numbers game, certainly not with -less- exercise between my PFTs and now, and with feeling sicker than normal, and such...

...but for me that gold standard is the quality of life, and how much clarity you have in your physical feelings and your mind just trying to survive and eke out an existence...and I keep slowly gliding to a great place. It's like a bell curve. The last bit is a tiny difference, stretched out over really long.

Maybe I'll switch to covering what things I can do to preserve it while I have the shot.
 

leecee

New member
Saintoffeon you must post at least once a day of your progress. Two reasons, the first is because you have piqued the concerns of many on here. "Gee, I hope he is ok today, can he make it to the stop sign?" "Did he find a good doctor?" etc.. Second, you are outright funny and sarcastic and made me laugh out loud.
 

Gammaw

Super Moderator
Yes.....SaintOffeon. And I'd love to see more posters who are doing well on Orkambi tell is all about what they went through in the beginning as SaintOffeon has done. It can make such a difference.....
 

Oboe

New member
PCCA in Jacksonville, which was well regarded by the Mayo Clinic when I was up there

That's where I go. It's pretty good, if you don't mind me adding my two cents. It seems like it's been in a bit of a transition this year, though. Our CF coordinator left in January and they have a woman covering what she did, but I'm not sure if she's actually the CF coordinator now or if she's a nurse who kinda got stuck with the work. It kind of feels like the latter. Not that I'm disparaging her work. She's been working really close and hard with me trying to get Orkambi and my other needs throughout the past year, and always returns calls within a day or two, but it doesn't feel the same as when we had a proper CF coordinator. Her name is Debbie Kent, if you end up calling the clinic you'll probably end up talking to her. Dr. Kate Walsh is who sees the CF patients for the most part, she's very knowledgeable and has been top-notch to me. Dr. Rothstein runs the program, he's not really a CF doc but he's also pretty cool.

The only problem I have with the place is that it's a small clinic. It's also a general pulmonology clinic and a sleep clinic. In the later months of the year (like now) it can be a little hard to squeeze in an appointment sometimes.
 
J

jamest

Guest
And I'd love to see more posters who are doing well on Orkambi tell is all about what they went through in the beginning as SaintOffeon has done. It can make such a difference.....
For those of you having problems with side effects, dropping the dose and tapering up seems to have worked for me.

I started 4ish weeks ago. High temperature, inflammation, SOB, and crazy zombie fatigue. After a week I dropped down to 1 pill a day. Symptoms minimized and then slowly disappeared after that. Stepped up to 2 pills a day a week ago, no side effects. Going to try for the full 4 pills a day next week.

So, the side effects seem to be gone, but I'm not sure I can detect any positive effects yet.
 

saintoffeon

New member
I did not have access tonight to a computer so I don't have a full report to give since I'm on my phone.

Oboe, thanks for all the incredibly thorough insight.

As for the checking in issue, if you guys want me to, and it is genuinely helping even one person, absolutely I'll still write. It's the least I could do. I'll post anything I do here in the way of videos as well whether it's running or a song.

Orkambi has been a stream of bullsh__, the every detail of which I feel I can recall vividly as if it were tattooed on my skin. But I can put up with a lot if I know there's an end at all, more so if I know when that end is. It doesn't make the hard parts less hard to get through, feelings wise, but it helps from a emotional preparation standpoint as well as a mental stability standpoint (from my experience).

I just went for snacks earlier tonight. No real reason, just a chance to move my legs again and see how they work in the real world, and also escape the screaming matches at my house (not involving me :) )...I actually smiled as I got out of my car and had an almost mischievous grin twenty minutes later as I was leaving.

I also almost teared up. It's not euphoria, it's not relaxation, it's not "whoops I took my next pain pill too soon" mildly high feelings...It's just...crap free...maybe diminished oxidative stress, as I think I briefly mentioned.

I remember waiting for this medicine and reading about Kalydeco and how it seemed to work across the board and how crazy high the numbers were, then Orkambi moved in and I thought, well, different numbers, but it's better than the not having it that I am now. I don't need to necessarily feel better if I know I'm getting better. Cayston doesn't make a dent in how I feel but it basically turns my mucus clear so I know it's fighting and winning important battles.

So I don't know how this early success compares on a relative scale with Kalydeco, but I can tell you where I'm at now is farther "quality feelings" wise than I ever expected to be when the smaller numbers came out.

If I say being reborn it overstates it. I'm still a sick person with a sickness. Neither can I say it's a clean slate. I've never not had CF so I don't know what that would look like.

Maybe defragmented. Optimized! :) I feel more like myself now in the same way I mourn feeling less like myself when I'm really really sick. It's like Me Plus. Maybe just sixteen ounces at the fourteen ounce price, but free is free!
 

CF in TX

New member
For those of you having problems with side effects, dropping the dose and tapering up seems to have worked for me.

I started 4ish weeks ago. High temperature, inflammation, SOB, and crazy zombie fatigue. After a week I dropped down to 1 pill a day. Symptoms minimized and then slowly disappeared after that. Stepped up to 2 pills a day a week ago, no side effects. Going to try for the full 4 pills a day next week.

So, the side effects seem to be gone, but I'm not sure I can detect any positive effects yet.

This is great! And along the same lines I'm trying to use as my ramp up plan as well.



Given everything I've read on here, what are some of the things people are considering "positive effects?" There seems like so much negativity with the drug and it's after effect that it's hard to tell what are actual positive results and what are just 'no longer feeling crappy side effects'
 

saintoffeon

New member
There seems like so much negativity with the drug and it's after effect that it's hard to tell what are actual positive results and what are just 'no longer feeling crappy side effects'

I think part of it is, like going to McDonald's. If they take your order, smile, the food's cheap, they ring it up right, it comes out in 3-5 minutes, you scarf it down, wave goodbye...that's fine, that's good, that's what's supposed to happen. Nobody wants to give out awards for meeting expectations.

And judging by the numbers alone (like in the giant treasure map that comes with the box, with all the fine print and facts and figures), nobody really had home run success, so you don't really get the flip-side passion, of "My goodness, this is a miracle!"

Best case scenario for most is, life sucks a little less to get through, physically, and your decline slows enough that you live a little while longer.

The only thing that leaves is freaking out because an expectation was set up to not feel like a demon relieving himself down your throat.

I have tried very hard to quantify, and qualify, what I'm feeling right now, to no real success (it's very hard for me to find the words!), simply because I've identified a notable difference between me before I started and now. Some parts, important parts, like the shortness of breath, weakness (and to less of an extent having trouble waking up and coughing up blood) aren't quite done making their mark, but they seem to be lessening.

Everything else, overall, is just...better...it's like a lower decibel level...Normally on my "good" days even, my body feels beat up, used up, things that don't make sense to hurt hurt (like joints), I can move a bit but I don't feel terribly motivated to...I call it inflammation but I don't know what it is. Exercise and pain medicine are all that take the edge off of it.

I just feel more...tangible now...more "in the world"....a more direct connection.....my mental state hasn't changed, really...my mental state IS, as you said, just the BS parts being gone....

But physically I feel lighter, freer...I wish I could slice the feeling up and hand out samples, not only for people to feel better but to get a sense for what it's like.

Sometimes when I'm feeling bad I will jokingly say things like, "Stick me in a hyperbaric chamber, and wake me when it's over"....that's what it feels like....restorative....like the morning after you slept the best sleep ever, without having had the good sleep and without any grog. And it lasts most of the day unaided.

Granted, this is like, a few days total this has been going on. And I left a nightmare behind, and just hope I don't slip back.

But it's definitely enough of a change that I'm like, "How is this a thing? What's going on? I didn't have this beforehand."
 

Gammaw

Super Moderator
Saint, your descriptions are so expressive. I hope you write for a living or consider it! It's like the other day I read a passage out of a good novel to my 10 year old. Of course he is used to the lame books written for 10 year olds. When he heard a good piece of literature he said "Wow. I can see and feel everything! It's like I'm there!" YES Saint. Keep telling us.
 
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