For people taking Orkambi

[saintoffeon]

I get told a lot I should write. Even a "psychic" at my sister's birthday party =P I happen to be writing a book, but it's more for going through the motions, one of those "free weekend release then it sells 17 copies" Amazon CreateSpace kind of deals.

If anyone had a chance to read it, CF or otherwise, I think it could help, if for no other reason than it gives an optimistic yet practical model of the world some voice (which can be drowned out a bit these days).

Thanks for the observation! =)

View attachment 530
I drew this ridiculous graph with my mouse just now to help people visualize my journey thus far. I forked it off at the end because I'm not breathing like I did, but everything else is pretty legit.

I also thought it might be useful to give a sort of profile of my stats for those who are curious:

Age: 31
Weight: Started 155, came down to 140, back up to 145 (throughout taking the pill, that is)
Height: 5' 11 1/2"
Location: Melbourne, Florida
Last known FEV: 43%, end of August 2015
Other prescription medications: Pulmozyme, tramadol (just for the first few days), Xopanex
Over the counter treatments: Melatonin chews for sleep, Tylenol Arthritis for super sore back pain (2 x 650mg every eight hours, but I only do it once a day)

Fat-containing foods, all aiming to be more unsaturated than saturated and 0/no trans:
Avocado (21g)
NUT-rition (14g or 15g depending on assortment)
Cabot Greek yogurt (21g/cup)
Babybel cheese snacks (4g/5g depending on flavor)
Sargento Balanced Breaks cheese/nut mix containers (12g per shallow plastic tub)

Official dosage time: 9:19am/pm
Biggest "miss" in the six hour window: 2 hours (almost always later, sometimes barely an hour before depending on outside circumstances)
How many times I missed: 4

Most important non-drug elements for me: Sleep and water. If I felt bad enough to not get good sleep, I didn't repair, and the next day didn't bring much good....I actually have a giant 72 oz jug of water I carry around with me at home and at work because I'm often too unmotivated to keep refilling a glass 8-12 times.

Latest I've gone to bed: Midnight
Earliest I've gotten up: 6am
Average hours of sleep when not completely robbed of it: 6-7

Am I missing anything I can edit into here?

 

[jamest]

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'

According to the stuff Vertex released, the major benefit is a reduction in exacerbations over time. Lung function improvement was relatively minor.

 

[saintoffeon]

Day 21

I coughed up blood this morning and woke up feeling decently stiff and sore, so there was some small concern I was slipping back a tiny bit today.

That said, a few days ago, I did that short walk with my folks, and the next day I did a longer walk with my mom where I had to stop a few times, and the next night I did that same longer walk (not actually long, just in the context of lately)...without having to stop. I could have, and it would have felt good, but I was almost done when I realized I hadn't and then I had to make sure I didn't =)

I receive invitations to exercise, but I'm already nervous doing just these walks, hoping I'm not overexerting myself. I'm the guy that's falling apart, so he goes into the hospital for IV treatment because he can't take any pills anymore, spends 4 or 5 days feeling like death, then another 4 or 5 climbing out of it slowly, then goes home and moves around like he wasn't just in the hospital for a week and a half.

Every time, and I get knocked on my arse for biting off too much too soon.

I don't want to do that here.

Maybe in a week's time some slow, menial basic exercise can get slipped in, but for now I'm content to move around a bit just walking from place to place, looking for subtle opportunities to test my legs.

I've also been having to carry stuff up and down stairs for work, and in a place where lots of people smoke and leave gross smoke clouds lingering in the air, and that's been getting a tiny, tiny bit easier.

So it all represents progress.

Today, though, the "freedom" feeling was in full effect, stronger than ever. I almost felt this temptation to just use my body for the sake of it, a motivation to move around because I had a chance to, in stark contrast with how I started and certainly the middle time of my journey so far, where I'd call up my body and try to schedule some movement, it would shout out a bunch of expletives and hang up.

Bled out again tonight. After a two hour movie, so it wasn't exacerbating like I thought before. I did have a lot of caffeine earlier in the day, and I think the half life is six hours. Maybe it caught up with me.

I mostly get it in the form of sodas, which I don't drink a ton of, and nowadays drink way less than I used to. More often than not it doesn't even sound appealing to me, but I still did it.

I've never smoked, I only occasionally dabbled in drinking alcohol for events or for every couple months, tiny airplane bottle amounts when I had company or wanted to try inventing a tiny cocktail. I have not even considered drinking a drop of alcohol since starting Orkambi.

Sodas, though, were at one point two a day/night up to 5 times a week. Now in my best weeks it's one the entire week, the day we go grocery shopping. I'd like to not do it ever again, but I get loose with those kinds of restrictions fairly easily.

If I find out it's messing with me enough to break the camel's back and provoke bleedings, though, that'll make for a way more convincing argument for a lasting ban.

Last year, I had next to no treatment, and my FEV was 9 points lower than now, and every other figure was just terrible....big huge lungs, super tall, almost half of it unusable trapped air, etc. Then a solid week and change where I coughed up so much blood, so often, I had to get a transfusion.

So is it a side effect? Is it that time of year again? Am I just weak enough with the wait loss and quick sprinklings of up-and-down infection-feeling things that something got knocked loose?

Not sure, but it's a party pooper. Fortunately, at least for the time being, the music at the party is loud enough to drown most of it out. But coughing up blood is still annoying and gross and scary. There's not a lot I wouldn't do for it to not be an entity in my life anymore, especially with the uneasy paranoia it creates.

 

[imported_Momto2]

Hey Saint, you doing OK? Ya havent posted yet today and your post from yesterday was a bit concerning.....

 

[saintoffeon]

Days 22 and 23

New theory with the sodas. I really, really don't like coughing up blood. At the risk of sounding spoiled, for as much as I count my blessings (and they are many, if not always obvious to others), I can't help but sometimes feel a little defeated when things go 90% well, and then 10% sh-- on top of it.

So with all these mini victories, all these gains, if I have all that and then have the exploding lung blood hanging over my head...it steals some of the joy from the experience.

With it happening again, I knew my first order of business had to be to try to dial the bleeding down out of control.

I don't know what's doing it, except to say the worst of it was last year when I had terrible lung function and no treatment, as I've mentioned before, but I've had stronger lung function lately which only has the recent weight loss and shortness of breath to combat it.

I mentioned Friday about the caffeine, curious if the increased heart rate might have a similar compounding effect to physical over-exertion. Now, I am dialing back to something I've dealt with before. I only really ever drink colas or so-called "spiced" sodas like Dr. Pepper. If you check the ingredients, many of them have phosphoric acid. They say they're using it as a flavor additive, to give a bite or tartness to the overall flavor profile. Phosphoric acid, though, is also REALLY REALLY good at robbing your body of calcium.

Why could that be important? In my somewhat desperate hunt for things to thicken my blood, trying to prevent more bleedouts (I have thin/not-really-good-at-clotting blood to begin with), I started looking up foods that "thicken" your blood. One article said, Vitamin K is good for thickening, but E can actually help thin things out, so look for high E but low E in the same food, and try eating those.

The same article, though, mentioned calcium. It didn't come right out and say, "Eat calcium-rich foods to get thick blood," but it indicated that not having enough would lead to abnormally thinner blood.

On the off-chance that was true, I tried it.

I'm happy to say, the whole weekend went by, no bleeds. Two on Friday, none through Saturday and Sunday. Now, that's not science. It's a terribly small sample size and one of a jilliion variables at play. But I think I'm going to stick with it. I already don't smoke and already don't drink, and if I ever really want caffeine coffee's a better hyper-upper for me anyways. I don't need the sugar crash, especially as CFRD looms on the horizon, and to be honest, everything else I eat in my life I eat pretty healthy, so it a) doesn't always taste as good as you want it to in your mind to be refreshing, and b) is one less thing I have messing with my tastebuds and cravings, meaning I possibly could be making slightly more healthy eating choices anyway.

You wouldn't think a couple a week would make a huge difference, but I'll be testing anyway to see what it does.

As far as the shortness of breath? Holy smithereens, Saturday was tough. After my slightly compromised "win" at the end of my third week, where minus the bleeding I got to the point of feeling motivated to move, my body overall on Saturday was still slightly sore and beat up, but was dialed down a bit, maybe 10%, from that freeing feeling. And I was so short of breath, so consistently, that it overwhelmed the rest of my body and left me not really moving much at all, or rather, being very judicious about my movement choices and constantly having to sit down and recover, even if I was just leaning on things, getting into "opening up airway" positions, or even if I was just standing in place too long.

I did try a heating blanket for the super annoying back pain. It melts it a bit for some relief, if not some remedy for the larger issues. I also still had that crazy strong Tylenol left (the arthritis kind, which are 650mg -apiece- and they ask you to take two at once), but I wasn't sure if that was a bleed risk too since so many things are layered on top of each other timing wise trying to find magnetic north and escape the icky parts of all of this.

Today was kind of similar. Getting up once in the middle of the night, and getting up early at a fairly decent time, my body didn't feel too bad, meaning some of the weakness and soreness was gone, especially in important parts like my legs, but after a couple hours the pain and shortness of breath built up steadily so that I was back in "picking and choosing" mode. I looked like my three-time cancer survivor 70 year old Grandpa, who does a stumbling "my mother was human but my father was concrete" dance move in slow motion when he tries to do anything resembling walking (when he's at his worst, that is).

My mother invited me to ride bikes with her, which I was definitely on board for. It's REALLY hard to do the stuff, especially when you're not feeling well, and I sucked at it. Everyone else was three times faster than me, and kept circling back to repeat the parts of our neighborhood I was in (cul de sacs and such), but it was only a defeat if I thought about it on their terms. My private goal was simple to keep pedaling, hoping that the whole experience meant I was getting some low-effort mechanical exercise that might strength natural motions of my legs, meaning less work for the rest of me once I heal.

So I was probably going 5 or 6 miles and hour, barely over a brisk walking speed, but I did the nearly mile bike ride. The weather played mostly nice, despite being Florida. Not too humid, decent breeze. I was starting to go into soft, Vader-like breathing rhythms, though, and it took at least a half hour if not a full hour to recover once I got home.

I also got invited to do errands shortly after, and I thought I could do more than I did, but it wasn't the case. As soon as I got in the first store (which wound up being the only store), my body was hurting and vibrating like it was swollen, and my mind was going, "Use all your energy to get to the safe place, by the rugs where she's going to start her shopping. Then we can sit and we'll be safe again!" Which is exactly what I did. After 20-25 minutes of sitting I kind of felt like maybe I could get back up, but I wasn't really truly feeling it, and the only energy I had I used up going to the checkout and getting back in the car.

Now, without a Saturday bleed, I wondered if I could alleviate some of my problems bringing the super Tylenol back into the fray. So I did the heating blanket and the Tylenol, and in an hour? Freedom!

We went shopping to two other stores, and I could stand in place, I could walk, I wasn't nearly as short of breath, and felt like a mostly normal person again (on our relative scale, anyway =)

THAT feeling is the stuff I wanna give away. There's absolutely nothing like it. I'd rather be me now in the low 40s with this feeling than in the low 50s without it. Again, it's not a high, and it's not euphoric, and I've had distance enough from when it started to fully confirm it's not the absence of all the other bad things. And, it occurs to me now, maybe not everyone has the same experiences with inflammation, or feeling weighed down or heavy or sore all over, every second of every day, but I'm inclined to believe they either do or can relate somewhat. That stuff is just GONE. And it's an enormous gift. One that I missed Saturday, telling myself, "Well, you got to visit, and a visit is better than no visit, plus at least you know it's possible....there are people waiting a half a freaking year for any signs of hope and you got signs after 3 weeks so don't be greedy, etc."

So right now I'm just trying to survive, go about my normal life, build up some kind of strength, and leave the poor breathing behind me....try to aim to consistently have this good experience. It's definitely worth fighting for. It's a bit like lunges, if you don't exercise all that much, especially if you're a tall, thin guy like me who sits for his job a lot. They suck to do, always work, always, but they pay off like busted slot machines.

 

[saintoffeon]

Day 24 (Monday)

Wasn't able to get on here last night, apologies.

Little, itsy bit of coughing up blood in the morning. It probably last a full minute, but because it happening at all sucks, it seemed like longer. It only qualifies because it was an "episode" like the others, where you feel the gurgling, and just start bleeding, and -that- makes you cough, versus coughing so hard you bleed (different format).

Shortness of breath was a nominal improvement, but I'll take it. Just enough to notice, but still not freewheeling and dancing and spinning =)

I did my first real exercise since I started (no offense, walking and very-slow-biking, I mean like calisthenics) and while I had to sit out for weird craning-twisting moves, because I figured I would either not be able to breathe or they'd make me bleed out, I did at least 80% of a twenty minute tape.

I really felt the weakness. Not in the lethargic fatigue way, just...wow, haven't used these pieces in a while, definitely don't feel like I'm coming from a place of any strength, that sort of thing.

I started out the day with a Tylenol, having some distance from the bleeds, acting as a kind of preventative measure since I could feel the achiness coming up pretty early on.

The other feeling is getting better. I actually darted up a couple steps on my way to my office today. I paid for it =) but it was fun and a good sign...I have this....I don't know what to call it..."physical optimism" thing going on. Where when I can move, I want to, and I do, -just because I can-.

Part of it is to embrace the blessing, and part of it is, I don't want to slide back into what happens if I don't move. So I'm biting off everything I can chew so I don't get stuck in a weak or shallow-feeling place.

I overexerted this morning (Day 25) and bled out, similar length, maybe a bit longer, but I am not totally sure I would have bled had I not overexerted.

With more self-control, I think I'm just going to keep getting the couple-percent-more progress in getting my breath back, and in perhaps keeping this great goodbye-BS feeling in my overall body and limbs.

Even though I know I could breathe better, I'm officially calling it that I'm better than when I started. Part of the point is to breathe better, but I have faith in that coming back -eventually- and it's pretty close. But the other stuff is just so overwhelmingly like what I imagine it's like to not feel like crap like I always did day in and day out...for me, right now, in my life and how I use my body? It counts for more.

Just this past May I had a pneumonia so bad everyone thought I was going to die, even my folks, and I was all but immobile and in excruciating pain. The breathing part gets worse, for sure. I don't even think I'd be that upset if the shortness stopped here, even if it would register as a net loss. That's how much better the rest of me feels. Again, it's trippy...minor FEV was supposed to be on the table...maybe that happens, maybe it doesn't....reduced exacerbations/hospital visits....too soon to tell, but I feel like there will be more of an uphill fight to infect me now.....but I never thought I'd get this Kalydeco-esque thing where existing...living...feeling....just got better....the way they describe it, their best cases, still sounds better than where I'm at, but proportionately given the differences between the drugs I think I'm still close. If I'm 1/4 of that, it doesn't honestly matter to me. I wouldn't keep track, I'd just hope it wouldn't go away and that I'd never lose sight of what a gift it is.

Makes me wonder if anyone working on these drugs actually got to take it, like they had CF...so they could know firsthand what -good- it could do, -when- it does good.

 

[DjFunkyFife]

I just got the call from Vertex that ive been approved for Orkambi. Tho i'll probably receive it later this week, i wont be starting the medicine until Saturday the 23rd, because of plans that i dont feel like preemptively canceling. I'm 33, male, about 140lbs, last good FEV1 is about 78, so i'm interested to see how i react.

I appreciate Saint's updates on his journey, but with such a FEV1 difference, i dont know how my experience will compare. i havent found anyone with an FEV1 around mine that is posting regular updates like Saint is.

I'll post updates if you all would like me to.

 

[Aboveallislove]

Yes, please do post. And thank you for all who have provided updates. It helps a lot in so many ways!!

 

[saintoffeon]

I started on a Saturday as well; it gave a clean break that didn't screw with my work schedule, and it helped me make sure I was watched in case anything went screwy.

I'm glad you're still deciding to take it; you might not have the contrast in feelings I had, but I feel like what I'm going through is a demonstration that it's at least possible for the mechanisms in place to do their job, follow their instructions, and counteract that process.

In other words, I'm a 43 (once as low as 35), so I have some exposure to waking up where your body feels miserable even when your mind doesn't, like you're starting the game with 1/3 of your health bar, and on the same difficulty as everyone else...so all that BS lifting will be really damn noticeable...

Since you're above where I feel to -start-, it might not be a magical journey, but if it means it takes longer than before for you to ever have to feel like I have, so much the better!

I only signed up for the fewer exacerbations/infections, and the off chance it'd take longer for me to die. I didn't sign up to feel great, though I imagined it would have to feel -somewhat- better.

The level that this is at for me is a huge surprise, and an unexpected but much appreciated gift. And I'll fight harder to keep taking it knowing what's at stake.

BUT if it all goes away tomorrow, and I'm just left with the idea that I'll be harder to kill, and not feeling much different, I'm here to stay.

The biggest thing, though, and I'm happy to see people encourage it even if it doesn't work out for them right out of the gate...is to see how it works for -you-...you're the only one you taking it really matters for in your day to day life.

Also, even though you might not feel much better if it works well in the short term, it may feel a lot worse in the short term...stay tough, and try to set a goal you feel comfortable with as far as what you're willing to endure before making a decision to stick with it or not. I've seen some folks on Twitter feel no different, and maybe a little worse, and then -six months- later make a decision. I'm not sure if I have that patience. But trust me when I say listening to your body has never been more important.

 

[saintoffeon]

Day 25

Another day with an early dose of Tylenol. Weaning off...at 2/day (they're extended release), my box only has enough for twelve days, and it says on the package you ought not to be taking for 10+ days without talking to your doctor about it (I've skipped, and I think I've only gone through half). It makes such a huge difference to not ache. I've also read that pain relievers have some capacity to address shortness of breath.

I did get my new tramadol prescription. Like before I started Orkambi, though, it's only supposed to be for as-needed, and I intend to keep it that way. I only got it originally to bridge me until I started Orkambi, thinking that most of my pain was CF-related (directly or indirectly, through things like inflammation). When it took longer than I anticipated to get Orkambi, I called it in again. Now, after three weeks and change, I know there are some lingering "break glass in case of emergency" style deals I wouldn't mind having a supply on hand for (although emergency is overstating things).

But even when I did have bad pain, I was seeking to take the edge off. If it mostly sucked but wasn't unbearable, I didn't take any, and I tried to avoid weekends. If something can really help you out, you don't want to build a tolerance to it.

I'm feeling so good right now, part of me thought of not refilling at all, but I think as an alternative to the Tylenol (since acetaminophen isn't exactly child's play in large doses) it's nice to have a safety net (for example, when I've coughed up so much blood it feels like there's an open sucking wound inside). If not for the pain itself, than the pain plus how it makes you anxious which leads to shallow breathing on top of the shortness of breath, or makes you feel like you have to restrict your breathing to avoid hurting more, etc.

On the whole, though, progress today in overall feelings and in the shortness of breath. My routine doesn't change much, walking, driving, lifting, opening, sitting, etc., so it makes it easier to identify noticeable changes, like things getting easier to lift, less pressure when carrying something on your shoulder, how much BS a task like bringing something to the dumpster feels like, etc.

So it's a slow taper, but no setbacks, really, on the broad front.

I did bleed out again tonight. Not sure why. Up to 145lbs. It wasn't drastic, relatively short-lived, and I was actually able to swallow it as it was happening (not to sound gross -and- stupid, but I have one TV show I watch each week and I'm pretty busy, so I didn't want to miss a third of it potentially and have to go hunt for it on demand).

No exercise like last night, but did get another loop around the neighborhood bike ride in. Amazing weather, and a little less work to pedal and keep up, plus less recovery necessary after the fact.

 

[thebiglug]

My Orkambi Adventure

I am a 40 year male CF’er with double DF508 mutation, my most recent PFT of 96%. My typical base range is 94-101% over the past few years. Overall I have been extremely blessed with good health and have very little history with regards to infections or hospital stays.

My most underlying concern with my health is that I culture Mycobacterium ongoing (abscessus or chelonae depending on the day). It has only become "active" once in the past 5 years which sparked a rapid reduction in my PFT down to 48% and an 11 day hospital stay to find the correct treatment plan. At that time I started treatment for CFRD and inhaled Amikacin that slowly not only got me back to my baseline at the time of 88-90% PFT. As I continued this new treatment regimen my baseline PFT went all the way up to 101%. To this day I continue with the insulin treatment for CFRD but discontinued use of the inhaled Akikacin 3 years ago and I have been antibiotic treatment free for the same period of time. Yes, I am humbled by how healthy I am and attribute it to a little bit of luck along with being very disciplined in my CF preventive maintenance routine (HTS, Vest, Pancreaze, Insulin, Exercise, etc.).

A few months back I started to research Orkambi's clinical trial, the CF patient blogs and decided once it became available to speak with my CF doctor about adding it to my treatment plan. My current doctor is the only CF doctor I have ever trusted as he is very strategic, analytical, sticks with facts and I would describe his approach to CF as an ongoing conservative yet can be assertive and think out of the box when and if needed. My doctor was definitely not sold on the results of Orkambi and was concerned with the side effects. He stated he would support my decision but the clinic would closely monitor my health during the process of adding it to my treatment plan. After an hour long conversation, I decided that I would give the medication a try.

Long story short, I started Orkambi about two weeks ago and lasted a total of 8 days on the treatment. My initial side effects over the first few days were diarrhea, tightness in the chest and some minor fatigue. On the first couple of days I did cough up a limited amount of "nasty junk" (I do not normally cough anything up and am very dry with limited cough etc.). Day after day my symptoms progressively started to become worse. Intermittent diarrhea, tightness of chest, SOB, back soreness and extreme fatigue started between days 3-6. I seriously could barely complete my day to day routine and would crash as soon as I got home by the end of the week. On day 7 and 8 I felt like I was the walking dead. I was extremely nauseous, literally could not stand without holding onto something due to the dizziness, and had flu like soreness throughout my body and my diarrhea worsened (lifetime worse).

On day 8 I could not take the side effects anymore and made my decision to immediately stop Orkambi. My current health status and quality of life the Orkambi side effects did not outweigh the reward for me at this time. For the record, I have a high tolerance for medication and pain (at one point while trying to treat my NTM I went back on Zyvox when the doctor told me to quit it and ultimately it made the majority of my hair fall out....I am a fighter nota sensitive whiner). I called my CF doctor and explained the side effects and that I had made the decision to stop the treatment. He was not surprised by the news and more than supported my decision.

With the FDA announcement of Orkambi came hope. Hope for CF patients and loved ones. Hope for a longer future. Hope for healthier future. Hope for the next step in a cure. With hope comes disappointment. Disappointment in the CF foundation. Disappointment in Vertex. Disappointment in the FDA. However, I am a true believe in hope and this quote sums it up…."We must accept finite disappointment, but never lose infinite hope." MLK

To be honest, hope is probably the biggest deciding factor (along with the thought of never knowing and living a life of what ifs) of why I made the decision to start the Orkambi treatment. The lackluster results, significant side effect warnings and even my doctor's opinion were all there and very evident in hindsight. All of the facts were simply overshadowed by one single thing...hope.

For those that continue to battle CF that are on or are debating to start the Orkambi treatment this post is not meant to deter or undermine those efforts (this post would not of stopped me from starting the drug either). This story is simply my personal journey and decision at this time in my own life to stop the treatment. I truly respect and admire all of you and your fight against this unforgiving disease and wish you all the very best.

In closing, it is important that we never lose the courage to fight and relentless hope for what the future will bring.

 

[shay]

Hi Everybody,
My daughter (19 years old) was a part of the Transport study and the following open label Progress study. She did very well on study drug (Ivacaftor/Lumacaftor combination) throughout both studies. She had very minimal (almost negligible) side effects. Her FEV1 is around 80%. We did not see a huge increase in FEV1, but we were thrilled with the fact that exacerbations requiring IV antibiotics were MUCH diminished. Before the study, she was requiring IV antibiotics every 6-8 months or so. And the antibiotics were becoming less and less effective. Since she has been on the study drug, she has increased the time between IVs to a year and a half!!! And the one time during this study when we did go to IVs it was for a shorter time and brought us back up to baseline. So we feel that not only are we avoiding the very harsh effects of those antibiotics, they are also more effective since we are not using them as often. We also feel that although we didn't get a huge bump in her FEV1, we felt that her health in general was more stable throughout this period. No steady decreases in FEV1 that we were experiencing before. She also seemed to have more energy and gained a few pounds. Now she will be starting the retail preparation, Orkambi. We are not sure what dosage she was on in study, so we will be curious to see if things remain constant.

 

[saintoffeon]

Before the study, she was requiring IV antibiotics every 6-8 months or so. And the antibiotics were becoming less and less effective. Since she has been on the study drug, she has increased the time between IVs to a year and a half!!! (...) we felt that her health in general was more stable throughout this period. No steady decreases in FEV1 that we were experiencing before. She also seemed to have more energy and gained a few pounds

This is absolutely amazing, and I'm so happy for you! I remember a few years back when it took a really long time between hospital visits. It makes a huge difference. I think this might be one of the first times I've seen where a relatively high FEV to start has documented overtly positive experiences.

 

[saintoffeon]

Day 26 (Wednesday)

Walked through a smoke cloud going into work today because of the neighboring telemarketers, and that seemed to be the straw that broke the camels back. Started bleeding in front of my door, fumbling for the keys, hoping not to run the carpet once I got inside. Made it free of the carpet, a couple big splatters on the floor, then demolished the bowl with a crime scene photo blood spray for about five minutes.

That said, aside from it being pretty humid at night, everything was pretty stable, if not nominally improved. Shortness of breath was less of a factor and I got to keep that good "calmed down" feeling everywhere else (I really need a name for it). I did have some of my pain medicine handy, but it didn't interfere with that particular feeling, just masked some chest pain from the coughing/bleeding, and I think made me duller/tired...Haven't taking it or Tylenol today so far (Thursday) and don't intend to. Nothing screams for even OTC, let alone an opioid, and I really wanna make sure I'm not taking anything just because it's there. I wanna earn this as much as I can in the free and clear, plus I wanna be acutely aware of changes that I get through things like legitimate exercise.

I did wake up with some congested feelings a bit, but I missed a vest session, and felt better afterwards. It's really nice waking up and not losing anything, really, not having it all be a dream, and just having a body that feels like I can actually use it. I was taking on a lot with my busted up body before, but now it really feels like I could ably take it on with less effort.

 

[Ratatosk]

Okay, so this is relatively minor, but thought I'd share. DS was sounding a little stuffy the last couple nights, as if he was coming down with a head cold. So I asked him if he'd like to do a sinus rinse. Now normally his nasal mucus is so thick it's difficult for him to blow his nose and get anything out. And his nose doesn't run. So when he does a sinus rinse it's this nasty horrible thick greenish/yellow crud.

Did his sinus rinse and it was normal looking cloudy snot -- not green, not yellow. And it washed right down the drain, didn't have to scrub.

 

[Aboveallislove]

Thank you for sharing! And ditto shay! Even the minor things are so wonderful to hear! And I bet it didn't feel minor to you, did it!?!?

 

[leecee]

Ok Saintoffeon, I am concerned about all this blood you are coughing up. You seem very cavalier about it but it scares me to read about it. Have you talked to anyone (meaning a doctor) about it? My daughter coughed up a dime sized amount of blood once and her doctor hospitalized her. She also was not responding to TOBI so had to go in for antibiotics as well but in my humble opinion I would suggest you talk to a doctor about it. I know you are an adult, but dude, coughing up so much blood is bad!

 

[Gammaw]

Wow SaintOffeon. I'm with leecee. What happened to your efforts to get in with an approved CF Clinic. I know you did the search and found two good possibilities. Have you made an appointment with either of them? PLEASE!

 

[CF in TX]

Saintoffeon, not to belabor the point people above have made... a little hemoptysis can be ok but the amount you seem to acknowledge coughing up regularly is concerning to hear. If you have a ruptured vessel in your lung that isn't healing, that blood will continue to cause problems. I really think if you aren't noticing any decline that you should try to get in somewhere quickly

 

[saintoffeon]

It is not my intention to appear cavalier about it. Like everything else I've been experiencing, I am just documenting for the sake of completeness. I've also opined that it is both scary and annoying and frustrating.

I have absolutely talked to a doctor about it, because, well, who wouldn't? It's freaky and potentially life-threatening. It is one of my biggest fears and causes of paranoia and psychological stress.

Here's some background information for perspective:

I didn't use to cough up blood at all. I'm 31 now, and back in 2006(ish), a year before my lung collapsed, I had one really bad episode, at least five minutes, just...gushing out. I didn't die, it just stopped, and even though my stepdad was just standing there watching, no one did anything about it. I got seen ASAP and they told me that with the CF and bronchiectasis layered on top, it was sort of par for the course, and the best I could do for myself was to keep on antibiotics and bronchodilators and the like.

Didn't happen again for a few years, then it happened maybe twice in the same year, then a year or so after that it happened three times.

Last year, I had a horrible week, around this same time (I wrote about it before but it was pages and pages back). I coughed up a lot of blood at school, at my job, tutoring, just sitting in place waiting for kids to arrive. It was the third time in a week, which didn't seem like the sporadic "flukes" the other ones were. So I went to the ER, and the exact conversation was:

"Well, are you still bleeding?"

"No, it stopped. I brought some in a bag I coughed into in case you needed to test it, but once it's done it tends to not happen again for a while."

"Well, if you're not actively bleeding, I don't know what you think we can do for you. It's really not emergency if it's over."

Then they discharged me.

Trouble is, it wasn't a fluke. It was only beginning. I woke up to one so bad, that the blood started -pouring- out of my mouth and nose at the same time, and I couldn't breathe, and as I was running out of breath I involuntarily gasped for air, and sucked up blood into my lungs instead, and started to pass out.

Feeling like that was a little too close to dying, I went to ER again and as nicely as possible demanded they look into it. I was admitted once they're heard that it was repeat, and in such a high amount (I took pictures...I was filling the bottoms of grocery bags).

Even then, they didn't take me seriously till it happened when I was there in one of their beds. They freaked out, and after measuring it at, I think, 220ccs of no mucus and just straight plasma and clots, put me in ICU. I was there for a day when they decided to do a CT scan to identify a possible source of a bleed, and started coughing up blood while lying down for the scan, which had to be canceled.

They brought in an ENT, a surgeon, two pulmonologists, a blood doctor, and an interventional radiologist.

For a week, they examined me from every angle, poked and prodded, trying to find out what was wrong.

Finally, they decided they would do an angiogram. At this hospital, the IR team won't do anything like blocking off blood vessels unless the whole team signs off on it, and they're extremely hesistant to do it because complications are relatively frequent (as many as 1 in 33 patients), and the complications are bad, like losing feeling/paralysis, permanent weakness, tissue death, etc.

Still, they wanted to take action. In six days, I had eight significant bleeds, and lost a liter and a half of blood, necessitating a transfusion as I wasn't able to regenerate it fast enough.

So the lead IR guy does the angiogram anyway, and he tells the team, the top of the chest isn't my specialty, so he won't do it anyway since he'd want it done right and considers himself out of practice. He's more of a skull guy, he says. So they poke a hole in my groin, go up to my face, looking for sources of a bleed (I have a giant cyst behind my eye with extra blood vessels that grew to try to heal it naturally, so it was a good first place to look). They didn't find anything, but near my pituitary there was a kind of weird looking area that they blocked off just in case. It had no effect.

They looked at my lungs, at the top of my chest, and some of the main delivery vessels had some weird shapes to them. They theorized that these were younger vessels, and before they got old enough to get solidified and in place, they were weak enough to not be able to "take the heat" of stress and coughing and all these health problems, and ruptured.

That's where they thought it was all happening, but the IR team said no. They said, sorry, but at this point, you haven't coughed up blood for almost a week. If it starts happening bad again, call us up, we'll re-discuss it, otherwise, we won't touch you.

Three months later, it happened bad. I called them, they said, isolated incident. A couple months later, I had a chain of them, so I showed up in ER. They refused to see me. I went to another hospital, they said they didn't have the facilities to do that kind of procedure. I had to go out of the county to find anyone else who would look at it, and they said they'd go along with it if I could get a pulmonologist to approve.

I talked to my two main pulmos I saw (and eventually more) and they all said the same thing: This is common, and as scary as it is, if they block things off, it'll just speed up your decline, because the pressure on the system won't change, they'll just be even less real estate set up to handle it.

At this point, they seem to be spaced out well, and tapering off a bit in terms of duration/intensity. I was also told to expect it a bit in the beginning of taking this medicine.

If it scares me, and I feel it's out of control, I will go to ER for the sake of it, but there's not a lot of motivation to do anything about it when everyone who CAN help you refuses to. What is there to be done, when the doers won't do?

I requested an appointment with both Layish's office and the group in Jacksonville. At this point I'm waiting to hear back. I schedule follow-up calls to check in on a weekly basis to keep me fresh in their mind. I'm not sure if it's a waiting list situation for new patients, but I also have to get direct referrals through my primary either way since I have an HMO, so as soon as they say yes I have to set up an appointment with him as well to get that done. I'm on top of it.

Right now, I am fighting my pulmo's office to get my other medicines back...Literally waiting two months now for simple bronchodilator and an antibiotic...they even said they'd research what was going on and get back to me and that was last Friday! I might start going somewhere else or get the press involved. I can't leave my life in the hands of an overworked or understaffed office. They got the Orkambi but they don't even have consistent notes from person to person depending on who you talk to. That, I'm sure, will help with the bleeds. Otherwise I am just trying to stay as healthy as possible, work out, and eat high-K and calcium-rich foods trying to 'thicken' my blood as much as possible.

If anyone else has any specific recommendations to combat it, I'd love to hear it. It's just tough when your hands are tied.