Post Your Orkambi Updates

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DjFunkyFife

New member
Wow, interesting stuff. It seems like it doesn't do much for people whose lung functions are over like...70. Mines 75-80. I really don't care about it increasing my lung function, I was hoping for other improvements. The heat related issue that someone else mentioned that it fixed, that's what I need for me. I get the same thing, constantly drinking extra salt Gatorade in the summer, and still going home feeling like crap. (I spend a lot of time in the heat for work) and nothing helps me endure the heat well. Summers almost kill me.

I guess I'll just wait and see...
 
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imported_Momto2

New member
DJfunky, I assume you've tried OTC thermotabs (sal pills for Na and Cl) and K pills? I used to be a professional rider (out in the heat all day in VA) and gatorade did nothing for me, but hydration plus the pills helped a lot. But everyone is different of course. :)
 
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TreasureGoddess

Member
We FINALLY have the medicine scheduled to deliver! My son gets to start on TUESDAY (crossing fingers it actually works out). Too many hoops to jump through, and things seemed to fall through the cracks a bit working with Vertex's group, but it did give us a VERY VERY LOW copay ($15/month) so I guess it's all worth it! Hoping this just helps overall. So exciting to actually have a medicine available that could help my kid feel better on a daily basis and potentially help him spend less time in the hospital. Will give an update after we've made it through the first 1.5 to 2 weeks of heavy coughing we've heard about.
 
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rubyroselee

New member
Started my Orkambi today! The only things I've noticed today are that my chest is just a teeny bit tight, coughing a lot more and bringing stuff up easier, sinuses are draining a lot as post-nasal drip, and I've been sweating a lot. But overall, I would say the first day went good :)
 
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imported_Momto2

New member
Rubyroselee, out of curiosity, what's your FEV1?

I'm on day 5 now and last nite was still horrid. 5 hours of nonstop coughing followed by 3 hours of insomnia and 1 hour of sleep. UGH.
 
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Aboveallislove

Super Moderator
imported_Momto2 said:
Rubyroselee, out of curiosity, what's your FEV1?

I'm on day 5 now and last nite was still horrid. 5 hours of nonstop coughing followed by 3 hours of insomnia and 1 hour of sleep. UGH.
Click to expand...

imported mom...in reading label yesterday isaw it said those under 40 fevery should have extra monitoring with doctor. Fwiw.
 
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rubyroselee

New member
ImportedMom - I am around 80%. I slept well for about 6 hrs and then was wide awake and coughing a ton. I even coughed up some nasty old plug. Very 'crackly' as well, but seems like when I cough I get something up most of the time. This is very unusual for me because I rarely cough anything up. Even during the last two months when I was on IVs (and now on Cipro again), I was very sick but couldn't cough anything up.

Hope things get better for you soon, and hopefully you're just dealing with the first week issues and they will subside soon. I've heard of many other people having problems sleeping in the first week.
 
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imported_Momto2

New member
aboveallislove- thanks for your concern. :) I am in touch with my doc on a daily basis with regard to how its going. He knows that I monitor myself better than any hospital ever would in this situation with hourly inspirations monitoring, constant sats, temp, pulse, BP, oxygen need, you name it. In a former life I was an EMT and my husband has had some training too. :D If I thought the situation was going to get out of control I would head to the ER in a heartbeat.

Leah-yeah, got the fingers crossed for improvement. Going to go and try to take a nap while the kiddos are at gymnastics....
 
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Aboveallislove

Super Moderator
oh, thank you so much for sharing. I was worried. It does sound like you are having a horrible time, but I pray that it means it is working...really really working for you. And ditto re Leah.
 
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DjFunkyFife

New member
imported_Momto2 said:
DJfunky, I assume you've tried OTC thermotabs (sal pills for Na and Cl) and K pills? I used to be a professional rider (out in the heat all day in VA) and gatorade did nothing for me, but hydration plus the pills helped a lot. But everyone is different of course. :)
Click to expand...

Yeah i did try those supplements. Once i figured out that it was electrolytes, and not just dehydration that was my problem, i started experimenting. I tried a bunch of things, including adding different electrolytes. Nothing made much of any difference for me except salt. In my post i referred to "extra-salt gatorade." After years i settled on adding salt to my gatorade. I'm talking a lot... a tablespoon or more to a 32 oz gatorade.. times at least a few of those each day, depending on the temperature. It helps quite a bit. Before i figured out the problem, i was going home every day feeling like death. Migraines, cramping, lethargy, all caused by the electrolyte imbalance that i didnt know i had. ( i knew that not enough salt was a problem in a CF person, but i never knew just how bad it was, for me, in hot weather) Adding the salt has stopped the migraines (i havent had one that was caused by electrolytes in years) it has stopped the cramps as well. (and i was getting constant cramps everyhwere... legs, feet, arms, neck.) It's still very tough to handle hot weather becauses i still get worn out.

So thats what i'm hoping orkambi does for me. Many people have said that it has helped them like this, so thats what i'm hoping for.

(also it seems from other peoples posts, that, the higher the FEV1 is, the lower the 'horrible-first-few-days-side-effects' are... unless i'm missing something. I'm apprehensive about that. (this is all under the assumption that GHPP says they'll pay for the drug for me, if not, then, i may not be on it.))
 
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jricci

Super Moderator
For anyone starting Orkambi, it’s a good idea to fill out a CFQ-R (cystic fibrosis quality of life questionnaire) prior to starting. Make sure it is documented in your official healthcare record.
Someone told me that United Healthcare has agreed to cover Orkambi for 6 months and then they will re-evaluate for effectiveness and reauthorize for 24 months if there is evidence of clinical improvement. Patient must show clinical improvement in ONE of the following areas:
· FEV1 or
· BMI or
· Pulmonary exacerbations or
· Quality of life as demonstrated by CFQ-R score
I haven’t heard this as a condition of any other insurance company besides United Healthcare; but it makes sense to fill it out in case other insurance companies have stipulations that require evidence of clinical improvement for re-authorization. I’m guessing most clinics are already doing this, but I thought I’d pass on just in case.
Here’s the link for united healthcare guidelines:
https://www.unitedhealthcareonline....esources/PA_Notification_Orkambi_052015PT.pdf
 
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triples15

Super Moderator
jricci said:
For anyone starting Orkambi, it’s a good idea to fill out a CFQ-R (cystic fibrosis quality of life questionnaire) prior to starting. Make sure it is documented in your official healthcare record.
Someone told me that United Healthcare has agreed to cover Orkambi for 6 months and then they will re-evaluate for effectiveness and reauthorize for 24 months if there is evidence of clinical improvement. Patient must show clinical improvement in ONE of the following areas:
· FEV1 or
· BMI or
· Pulmonary exacerbations or
· Quality of life as demonstrated by CFQ-R score
I haven’t heard this as a condition of any other insurance company besides United Healthcare; but it makes sense to fill it out in case other insurance companies have stipulations that require evidence of clinical improvement for re-authorization. I’m guessing most clinics are already doing this, but I thought I’d pass on just in case.
Here’s the link for united healthcare guidelines:
https://www.unitedhealthcareonline....esources/PA_Notification_Orkambi_052015PT.pdf
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Great info as usual J!

Thanks!

Autumn
 
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Brandon Justice

New member
I'm delta F508 homozygous, I'm 20 years old with an FEV1 of 89% and it's all thanks to Orkambi. I've been on the drug for 2 years through its clinical trial phase. Trust me everyone, it's a miracle. Diet and exercise and certain health and wellness products along with Orkambi is key to your success. Take a second to run past my blog brandoncysticfibrosis.wordpress.com I started it yesterday so folks who are interested can track my journey and know that Orkambi not only works, it blew away all of my expectations.
 
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imported_Momto2

New member
I'm glad it was a miracle for you. Did you have any initial negative reactions and how quickly did you notice a positive change in your health?
 
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scanboyd

Member
Orkambi sounds like a great drug for us delta F508 folks but the cost from what I understand is some where around $259,000.00 a year. This is crazy. This will cause our insurance premiums to go out the roof. I thought Cayston was exp. but this is robbing folks financially without a gun. There is no way insurance companies can afford the cost. It will be passed along to the consumer. Insurance companies will stop insuring CF folks or employers will not hire you or family member that has a CF family member that they will be insuring, if they can? Got to better cost structure, realizing R&D is exp. for drugs but this is insane!
 
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Printer

Active member
scanboyd:

You have got that right. The number that was quoted in the Boston Globe was $310,000.00 per patient per year.

Bill
 
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TreasureGoddess

Member
Day 5 was kind of a whopper for my son. He felt GREAT for the first time and probably did too much running about outdoors with his friends. About 8pm came inside, said he felt bad and just laid around not feeling well. LOTS of coughing last night, it had slowed down since the first couple of days. He's home from school today, just resting up. Our docs said at the 1.5 week point if things aren't resolved and feeling better they want to see him. It sounds like we're right on track with everyone else's experiences of feeling chest tightness & major coughing for the first days and then progressively better. Lots of sweating and hard time sleeping, but he feels really positive that this medicine will help him feel better overall.
 
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Allansarmy

Guest
Printer said:
scanboyd:

You have got that right. The number that was quoted in the Boston Globe was $310,000.00 per patient per year.

Bill
Click to expand...

Hey Bill, good to see you on here.

Yep Bill has it right. I had heard about 27k a month is average. I am currently paying a $100 copay a month and I am not complaining hearing all of this. However, Vertex has reached out their hand to us as my wife and I do not make a lot of money annually. The copay is worth it for what it will do for my son, but added into his other pharmacy bills, its starting to get on up there in price. I wil let everyone know how Vertex handles things. So far on the phone they have been very nice telling the steps. There is a form on the site that the CF clinic must fill out and my son is 19 now, he must sign the HIPPA release and then they will speak to us about possibility of lowering that $100 to maybe $40 if we are lucky and I will love it!

But.. correct me if I am wrong here @scanboyd I am not sure the insurance companies can stop insuring CF folks or employers can't just not hire you based on this. Completely against the law and as a matter of fact, at least here in Texas, they are not permitted to press the issue either of evaluating your health before they hire you. Now do companies fire people that are costing them money with insurance? Ya I bet it happens a lot actually and its beyond sad. I heard it at my last job all the time, how the premiums are going up and they would always say it in my ear shot too. Couldn't stand it, so I dropped my family off my insurance and luckily my wife has a lot better insurance, so we all went onto hers as she works for a big corporation.
 
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TheSaltyOne

Guest
Hey everyone!
I've been on Orkambi for about 6 months now (I was in the drug study). I couldn't say anything about it at first but now that its out I'm able to, just not my exact numbers yet.
As far as symptoms go, in the beginning for about a month I was tired a lot, coughing up a bunch of stuff, flu like symptoms, and short of breath just resting and walking around. I complained to my clinic a lot about the shortness of breath and they reassured me it would go away after a few weeks. It didn't. So since I started I've also been put on Spiriva and Cayston (I previously wasn't doing any inhaled antibiotics). I didn't notice any change at all in my health, overall feeling, or lung function until this past week (starting the 6th month). My lung function has finally gone up some. Apparently there's another person in my study that is now experience late benefits as well, so I guess those who are on it and not seeing any benefits yet.. hang in there! Of course we don't know what will happen long term yet but hoping that I keep gaining ground. Hope this helps some!

I also made a video about my experience on my CF channel.
Here's my video link: https://youtu.be/94I1CENqks0
 
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