Orkambi, preview.


New member
Well the time has finally arrived for me to start taking Orkambi. (warning, long post coming, and you'll get some boring details as well)

I'll start this post off by saying that i took my first dose 50 minutes ago... (at 10:30am pacific) with plenty of fat. So far no reaction. (yes i know, only 50 minutes in)

So i've heard many many things about this drug. And the reactions, opinions, as well as the effect that it has on people is all over the spectrum. This is no news to anyone probably, but it amazes me. I dont have experience like this with any other drug, but i'm shocked that one drug has so many reactions and effects. The variables inside our bodies must be so many that it's impossible to predict how one will fare on the drug. So because of that, i'm not having any expectations at all with this drug. I'm not EXPECTING for it to help me, but it would be great if it did.

So i'll start off with a snapshot of myself to give you all an overview of who you're dealing with. I was going to post a literal snapshot, a picture i took with my phone of the FEV chart that my doctor creates with all the different FEV measurements, but i cant find it. (also, i was going to post a picture in the description of my blog, but it wouldn't work. I linked a photo from google photos, didn't work, and i linked the same photo from dropbox, didnt work!! does anybody know what i'm doing wrong? i went to each site, generated a link, then pasted it in the box here where it asks for the url)

I'm a 33 year old male, living in southern California.

So on my last doctor visit, my fev was about 78%. That's about normal for me. I think the highest I've ever had it was 81%. My lungs don't give me a whole lot of grief, yes i have to cough up crap daily, yes i go through ups and downs where i need to do more treatments, or have to take oral antibiotics, but overall, i'm fortunate in that area. they aren't constantly affecting my life every minute of the day. (granted, if i tried to run a marathon, i'd fall flat on my face from exhaustion and chest discomfort in about 30 seconds, but i don't spend my days trying to run marathons) That said, the other areas of CF torment me!! The GI issues irritate me the most. I've spent years trying to fine-tune my digestion, learning how different foods, liquids and drugs interact with my body. And I've come to the conclusion that sometimes it's just gonna hafta be a crapshoot (no pun intended... well actually.... ) what happens down there. I've learned more about my digestion from reading random things on the internet than i have learned form any doctor or dietitian. And by trial and error, I've benefited from putting some of those things into practice. But still, i wish that i could more closely regulate my digestion. Unless i only ever ate one thing, that'll never happen. (i forgot where i was going with all this... oh yeah!!..) My sinuses also give me hell. It's gotten to the point where almost daily i have some sort of sinus issue, and it isn't drainage, or something that sinus rinses help. (I've tried) A scan revealed polyps, and something else, i forgot.. a swollen sinus something or other i think. So i get sinus pressure and headaches multiple times each week. And FINALLY, Dehydration! Dehydration, I've learned, in addition to being it's own problem worsens all of the above problems (sinus, GI...) Dehydration is a HUGE problem for me. I never knew how much i was dehydrated until roughly a year ago. I was having headaches a LOT, that's what led me to look into it. Well, i learned that the list of possible symptoms of dehydration is longer than the contract that Toyota wrote up the last time i bought a truck! It's Ridiculous! Also, many of the symptoms can have multiple causes. Some stomach issues can be related to dehydration (i read that dehydration causes a change in stomach acid levels.. too little OR too much can cause GI issues) BUT, i was showing some of those symptoms, including stomach ones. to make a long story slightly shorter, i learned that i was dehydrated much more than i thought, and keeping myself hydrated and correctly electrolyted (i made that word up, i think. Chrome tried to suggest Electrocuted! lol) has proved to be a very challenging task! to the point that it gives me almost as much grief as the GI issues sometimes.

So my point is, if Orkambi does nothing at all for my lungs, but could manage to give me some help in any of those other areas, i will be grateful, and will think it's worth taking. If it makes anything worse, yet i see improvements in other areas, i'll have to weigh everything to consider whether it's worth continuing to take.

Finally, on to keeping track of changes/improvements/declines.

I'm a numbers kind of guy. I love numbers, math is like a second language to me. I'm NOT the type of person who looks for meaning in numbers, or think they run my life or anything, i just like math... apparently . (yes, i'm weird) So in looking for changes that i've experienced since starting orkambi, i'm taking a numbers approach. What the heck does that mean? it means, besides the obvious numbers doctors are looking at with respect to lungs, i want to quantify other changes numerically. Examples: I walk my dog almost every day. If i start the walk off by running, i can literally only run about 30 seconds. So, i want to keep that routine, and time my running ability, and document any changes. If i see that there's a change... 33 seconds....39.... 50... 2 minutes... etc. (or conversely, if it goes down) That will give me numeric proof of changes. That's one example. (I actually would like any suggestions anyone might have in this regard. Hopefully someone out there thinks like me) At my doctors request, i'm already tracking my peak flows. And i'm going to track my weight. I already kind of track my blood sugar. I have elevated blood sugar, i'm ALMOST at the point of needing insulin, but not quite, so obviously any lowering of blood sugar i'm going to take as a sign of orkambi improvement. For me the purpose of all that is so that in 2 months, 3 months, 6 months, instead of saying "i don't feel any better, this stuff isn't worth it" i can use numbers to look back and see definite changes.

Well i'll stop writing finally! It's taken me an hour and ten minutes to write this! So two hours in, and nothing noticeable to write about yet. But i'm sure i'll be back, possibly today.