For people taking Orkambi

[Cubs fan Brian]

Ever so slight increase for me with the FEV1% today. I made it up to 34%, which isn't very good but I'll take it for now. I was at 33% back on July 20 and the doc had me come in for a 11-day "tune-up" session. I am definitely still breathing better today than I had been the past couple of weeks though. Perhaps I'm near the end of the really tough part of this.

Just the thought of something having double the effect that the Orkambi has makes my head hurt. Honestly don't think I could survive that living home alone. As it is, I sort of wish I had been in the hospital for the first couple of weeks of Orkambi.

 

[Aboveallislove]

Brian,
so glad for the improvement even if slight...hoping with the bad patch or worst of it seemed ngly behind you that maybe you'll soon get more improvement...and then in a couple of years ready for the next generation to do even better! Godspeed

 

[liveitup]

Hi Everyone!

I started Orkambi last night. I took my 3rd dose tonight. So far, just a little cough and maybe the tiniest chest tightness. When did the "Hell week" start for you all? I'm scared, and it almost prevented me from starting! I'm 36 and FEV is 87%.
Thanks! 36 F/married w/3 kids/DDF508

 

[imported_Momto2]

Hell weeks for me started with the very first dose, within a hour............

 

[Aboveallislove]

Hi Everyone!

I started Orkambi last night. I took my 3rd dose tonight. So far, just a little cough and maybe the tiniest chest tightness. When did the "Hell week" start for you all? I'm scared, and it almost prevented me from starting! I'm 36 and FEV is 87%.
Thanks! 36 F/married w/3 kids/DDF508

i think many who have really struggled unfortunately face lower fev when starting up...which is consistent with the label recommending doctors monitor closely those under 40%. From those I've seen too...it did all sound like it started real quick...that you'd already have been hit by the Mack truck if it were going to. Hopefully you'll have some good results with the he!! Week. Please do post more.

imported mom...any mmore re updates on overall changes?

 

[liveitup]

Thanks for your responses. The other thing I notice (and still am noticing) my mucus (ewe - sorry!!!) feels like I just got finished my Hypertontic saline neb. it seems "super flowY" (I'm sure that isn't even a real word!) OH YES, and I was so tired yesterday. That could be for many reasons though! I have 3 kids (1, 5, 10) and school started this week. I home school my oldest and the baby is home with me all day.

Imported Mom - How are you feeling now? I hope the Orkambi produces wonderful results for you.

 

[imported_Momto2]

Well, still having fevers, (which my doc said is my immune system going at my lungs due to the Orkambi) GI issues, some insomnia, fatigue. Havent noticed any improvement in the lungs and that's a bit discouraging. I was optimistically thinking I was finally getting back to at least baseline with the lungs until I stated taking some data and writing it down. In the AM when I wake up, with my little handheld FEV1 monitor I usually blow about .95L with 120-140 flow rate. An hour after I take the Orkambi it has fallen to about .80 L and 80 flow rate. By midday, it goes back up a bit to around .9L with a about a 100 mls/min flowrate. By the Orkambi evening dose, its not quite up to the AM numbers, but there's been a lot of "recovery". After the PM dose, the same darn drop happens Again. Now, I dont have a medical license, but to me, these numbers are still going in the WRONG friggin direction......... and my sats follow the FEV1 numbers (92/93 in the AM prior to O, then falling after the dose). Its bloody frustrating. I am withholding any final verdict until I've been on the Orkambi for at least 2.5 months and my FEV1 and FVC are taken at the clinic in October, and then I'll see the official numbers. But right now, all I see is loss in the short term numbers plus side-effects. I am telling myself I am still only just shy of 4 weeks out of initial dose and there is still time for this drug to actually help and the side effects to go away. AARGHGHGHGHGH. I SO want this medication to be helping me........ I NEED for it to be helping me......

 

[liveitup]

That is so frustrating Imported!!! I am going to be praying for YOU and that this DOES WORK for you!!! I am hoping and praying that it already is! Will be waiting for GOOD NEWS!!!

 

[saintoffeon]

No kidding. There are so many horror stories, it makes me really think twice over how it was able to register as an average improvement in trials. I realize some people that feel good and have good results might not be as vocal right now, not everyone has access to the medicine, and the trials lasted a lot longer than the month or two people have been taking this...

...but the perception is that most people are not just not better, not just no effect, but worse on it, and it makes you wonder how something that hurts instead of helps gets approved.

Last known FEV1 for me was 34%, but I am in a bit better exercise shape, and have access to some breathing meds for the first time in a long time, so I feel like it's going to kick my arse pretty bad, but hopefully come up a little rosier over the long term.

 

[saintoffeon]

I was just approved. Sounds like Vertex's insurance verification will have an answer from actual insurance tonight or tomorrow morning...so maybe early next week I'll start and report back in?

 

[imported_Momto2]

saint- good luck!!! I'll be keeping my fingers crossed that you have a totally uneventful starting.....
Just a note of caution, if you dont have oxygen on hand and/or some powerful meds to stop coughing, I would prep prior to that first dose given your FEV1.
At the very least, have another person around......

With regard to it not seeming to help people yet, perhaps enough time hasnt passed. I am trying so darn hard to be optimistic here.
But it also makes me wonder, that lady at the FDA hearing who didnt think it should pass.....maybe she knew something? I dunno.

Still having all the issue I was at my last post. The only change being more asthmatic today.....joy and rupture.

 

[Ratatosk]

DH attended a cff mtg yesterday and most of the individuals reported their family members had shown improvement. There were some who had initial breathing issues. One in particular indicated their teenage son, who was looking at a feeding tube due to weight loss, had grown significantly since he's been on it. DH has noticed ds tends to be breathing better. Used to be when he'd get excited, laughing or talking fast, he'd cough a little bit and clear his throat, have to pause to take a breath. And I've noticed when sleeping he isn't doing any mouth breathing. Much quieter.

 

[Aboveallislove]

DH attended a cff mtg yesterday and most of the individuals reported their family members had shown improvement. There were some who had initial breathing issues. One in particular indicated their teenage son, who was looking at a feeding tube due to weight loss, had grown significantly since he's been on it. DH has noticed ds tends to be breathing better. Used to be when he'd get excited, laughing or talking fast, he'd cough a little bit and clear his throat, have to pause to take a breath. And I've noticed when sleeping he isn't doing any mouth breathing. Much quieter.

Thanks so much for sharing. I hope those struggling see some improvement soon, but your note was much needed today.

 

[imported_Momto2]

Just wanted to let folks know that I have discontinued the Orkambi. I made it 5 weeks. It is not worth it for me personally. These have been the most miserable 5 weeks of my life. The FDA transcript was very educational and helped convince me to stop. Thank you all for your support. I hope this drug helps you or your loved one. I'll let you know if my FEV1 goes back up now that I have stopped.

 

[Aboveallislove]

I am so sorry. It must be crushing that this hope has ended up a millstone. Please do post how things improve for you. Hugs and prayers,
Love

Just wanted to let folks know that I have discontinued the Orkambi. I made it 5 weeks. It is not worth it for me personally. These have been the most miserable 5 weeks of my life. The FDA transcript was very educational and helped convince me to stop. Thank you all for your support. I hope this drug helps you or your loved one. I'll let you know if my FEV1 goes back up now that I have stopped.

 

[regina65]

I started orkambi 9/3 been on it for 11 days. I went into the hospital for iv antibiotics because I knew it would be rough if I didnt. I am doing ok so far. I am tired, but i get that way on iv's so can't say that's orkambi. I do have acid reflux. I'll have to get my dr to up my meds. I have had some SOB in the afternoon mostly, but as soon as I do my treatment it's gone. I did that before orkambi with treatments. The mucus is easier to cough up. Hoping for more improvements when I am on it a little longer. I have 3 days worth of ivs left we'll see how I feel after that. I have also noticed that i have a lot more anxiety the past couple of days. I may have to get the paxil dosage increased also. I am going to call Vertex tomorrow and see if it affects Paxil.

 

[liveitup]

I'm sorry to hear that the Orkambi has been rough for many of you. I started taking Orkambi on the 3rd of September. So far, I feel great! I'm definitely not as salty, and I don't even have a cough at all. I was very tired the first couple days and had some very minimal chest tightness. It is weird for me to seek out fatty foods to take it with. I wonder if that is the weight gain? Please continue to post how things are going for everyone!

 

[imported_Momto2]

After stopped the orkambi yesterday AM, the reversal has been startling over the past 24 hours. Wheezing and SOB gone, rhinitis gone, slept through night, no nausea, stomach pain GONE. Still have a slightly elevated temp, but its lower than it was, and I suppose it will take my immune system some time to get the msg. I actually went for a walk this morning and had the oomph to get up with my daughter at 6 am to see her off to high school and do a little gardening. Frankly, I am really shocked. I thought it would take me days, if not a week to bounce back. I am sure there is some psychological effect going on here as well, but since I didnt expect a quick recovery, its probably minimal.

It does make me wonder, since I initially had such a strong lung-flooding reaction to this drug, if it might be useful to take it once a week as a cleanout mechanism. Anyone tried this or have informed ideas?

 

[Aboveallislove]

Thinking through this, given your initial "clearing out" but your extended problems which went away rather quickly, I wonder if you are having an allergic reaction to Orcambi? I have no idea if it is possible and if so, possible to "test" for an allegy and then destenstizize you to Orcambi? Here's my thinking: Others have allegies to antibiotics that are needed and are desensitized and able to use. You definitely had the "appropriate" reaction to orcambi from a cf standpoint, but the continued problems weren't the "start up" kind of problems. ...so what would explain both? I'm thinking allegery. And if they can desensitive people to an antibiotic, couldn't they also do that for Orcambi? OR, looking down the road with 661, if it is an allegic reaction to 809, then a combo with 661 might not...so it might be beneficial to have testing done to see if either 809/Kalydeco is causing it, even if you aren't thinking of desensiting for the "positive" clearing effect? (Note: I'm not trying to push the drug on you....obviously it is causing extreme problems for you...I'm just wondering if there is a way that you can get that benefit and maybe a 2-4 day on and then 2 week off or something might work, in thinking through that I wondered why would you be having the reaction. that's what made me think allergy. FWIW. thoughts?

After stopped the orkambi yesterday AM, the reversal has been startling over the past 24 hours. Wheezing and SOB gone, rhinitis gone, slept through night, no nausea, stomach pain GONE. Still have a slightly elevated temp, but its lower than it was, and I suppose it will take my immune system some time to get the msg. I actually went for a walk this morning and had the oomph to get up with my daughter at 6 am to see her off to high school and do a little gardening. Frankly, I am really shocked. I thought it would take me days, if not a week to bounce back. I am sure there is some psychological effect going on here as well, but since I didnt expect a quick recovery, its probably minimal.

It does make me wonder, since I initially had such a strong lung-flooding reaction to this drug, if it might be useful to take it once a week as a cleanout mechanism. Anyone tried this or have informed ideas?

 

[Oboe]

Still haven't gotten mine. My FEV's too low, ~24-25%, and Florida medicaid doesn't want to cover it. I think my docs are working with Vertex about efficacy data and contraindications about it for medicaid, but I don't expect anything. Though with all the trouble people with low function are having, maybe that's not such a bad thing.