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Thread: Kalydeco

  1. #1

    Kalydeco

    I know there isn't many of us out there with the G551D Mutation. But who else on here is actually using this besides me. I am really curious to what effect it has had on you guys/girls. I have been on it since April and my FEV1 went up by 11%. I have had about a 20 pound weight gain. And am generally doing a lot better health wise.

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  2. #2
    Hello,

    I have been on Kalydeco for 5 weeks. I am on a compassionate program in Australia as it is not approved here yet. My FEV1 changed from 41 to 70%, weight from 55-58kg and sweat test from 102 to 54. I did a lot of physio, nebs and exercise so that definitely helped my pft increase. I've also noticed other changes, no cough overnight, little cough during the day, not very productive (light green/yellow instead of dark & much much thinner), no crackles, no chest pain, reflux is better, more energy and there is no salt on my face when it is hot/during exercise. Pretty amazing what these tablets can do! I have written more about the last 5 weeks here: magicbluepill.wordpress.com

    Have your pfts been stable since April and have you needed any antibiotics? I am hoping I will be much more stable now.

    Gen

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  3. #3
    Friend of mine's daughter has GFF1D and another mutation -- not Delta f508 -- and is 7 years old. She started Kaly in June -- she gained a pound, grew an inch. Has more energy. Her sweat test went from 110 as a baby to 14.

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    Parent to a child wcf double delta f508

  4. #4
    Wow 14 is amazing! I just wrote a post about sweat tests under CFTR Modulation- a study found that the normal value for a 5-9 year old is 12.6. So 14 is pretty much normal!

    lprulez- there is a group on facebook called Kalydeco Miracles where people have posted about their improvements on Kalydeco, so you will find more people there: https://www.facebook.com/groups/359954544023650/

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  5. #5
    Quote Originally Posted by GenH View Post
    Hello,

    I have been on Kalydeco for 5 weeks. I am on a compassionate program in Australia as it is not approved here yet. My FEV1 changed from 41 to 70%, weight from 55-58kg and sweat test from 102 to 54. I did a lot of physio, nebs and exercise so that definitely helped my pft increase. I've also noticed other changes, no cough overnight, little cough during the day, not very productive (light green/yellow instead of dark & much much thinner), no crackles, no chest pain, reflux is better, more energy and there is no salt on my face when it is hot/during exercise. Pretty amazing what these tablets can do! I have written more about the last 5 weeks here: magicbluepill.wordpress.com

    Have your pfts been stable since April and have you needed any antibiotics? I am hoping I will be much more stable now.

    Gen
    really hope there is something for f508 soon because that sounds incredible. i am at 41% FEV1 now and going to 70 would be fantastic. need to get in the gym more. i would also love to cough a lot less too.

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  6. #6
    Quote Originally Posted by GenH View Post
    Wow 14 is amazing! I just wrote a post about sweat tests under CFTR Modulation- a study found that the normal value for a 5-9 year old is 12.6. So 14 is pretty much normal!
    GenH, the studies you wrote about identified that the median value for a 5-9 year old is 12.6. Median is not a synonym for "normal": there is a range of values either side of the median which will still be normal. Sweat chloride levels of up to 35mmol/L can be considered "normal" and generally anything of 39 or below contra-indicates a diagnosis of CF; conversely, only levels >60 mmol/L reliably indicate CF. 14 is not merely "pretty much normal" but it is within the normal range.

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  7. #7
    Yes I am aware that they are the median values, I mentioned that in the post about sweat chloride. I was trying not to be too technical when I wrote 'normal', this is easier for most people to understand over median.

    I'm not sure if you have read the article but here are some more figures: The estimated median (95% RI) for sweat chloride were: 5 to 9 years, 13 mmol/L (1-39 mmol/L); 10 to 14 years, 18mmol/L (3-47 mmol/L); 15 to 19 years, 20 mmol/L (3-51mmol/L); and 20+ years 23 mmol/L (5-56mmol/L). The 95% RIs are similar to the values that were found in each of the groups (the article has a dot plot showing this).

    So yes according to that data up to 39 is 'normal' at age 5-9. If I take that further my 54 at age 27 is also 'normal.'

    The numbers are postively skewed, eg with age 5-9 half the people are between approx 1-13 and the other half between 13-39 (75th centile at about 19, so 75% below about 19). There may be some non F508del carriers in the higher range of normal in this study.

    I'm hesitant to write that a level is normal as I am aware that G551D with Kalydeco has about 50% CFTR function. So my 54 is in the grey zone, I know some people who have been diagnosed with CF with a similar sweat chloride, and according to that study, some people also have that sweat chloride and do not have CF. I agree that 14 and 17 seem normal for a 5-9 year old, but for those of us in the grey area I personally dont use the word normal (ie the borderline between normal/not normal is hard to define).

    Whenever I write about scientific studies I am very cautious about what I write (eg use words like may or could instead of definite words) which is why I used pretty much normal.

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  8. #8
    Hi

    Last week they found out that I got the G551D mutation and I'm taking Kalydeco for 5 days now. I put together a homepage with general information about CF, Kalydeco and I plan to share my experience with the rest of the world. Unfortunately it's only available in german so far but I'll try to translate it also in English. So if anyone is interested and understands a bit of German feel free to visit www.the-silverlining.ch. The english audience I ask to be patient - I'll update the page asap.

    Best,
    Marco

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  9. #9
    gods speed to you Marco!

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  10. #10
    I finally agreed to go on it. As long as I'm fairly consistent with the 12 hour window for taking the med, I have a significant reduction in mucus! It's really been amazing.
    If I miss the 12 hours (forget a dose or whatever), then the mucus almost instantaneously increases. Docs say, they haven't seen that before, but we are all different!

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