Tuesday, January 5, 2016

Four Months Of Orkambi

*This is a long version of my experience on Orkambi*

This is a post I keep starting, but can't seem to get myself to post. To be honest, my Orkambi journey started torturous at best. As much as I wanted to gripe and complain about the sickness that came with this new pill I knew I was unbelievably lucky to have the ability to even try this medicine. I know how many people with CF would love the opportunity to try this medication that was already in my hands and that none of them wanted to hear me complain. At the same time, when I felt as sick as I did, it was hard to feel grateful and lucky for a medicine that I wasn't sure could ever help me. And so I kept quiet and internally kept note of the changes, both good and bad, going on in my body. I am just finishing four months of Orkambi now and feel I have a clearer picture of what Orkambi means for me and my disease.

I started Orkambi feeling cautiously optimistic and well aware that the first month or so would be tough. However, I had no idea what the next several months had in store for me. My symptoms were not that unusual and many people that start the medicine experienced the same side effects. Some of the symptoms that came with this little pink pill were shortness of breath that was so extreme I had trouble walking across the room. In fact, there were times I felt like I was suffocating while sitting still. I had fevers, chills, aches, streaking of blood in my mucus, a cough that never stopped, but didn't feel productive, which meant I hardly slept. I was mentally prepared for these side effects, but I wasn't prepared for how long they would last. I was warned by fellow Orkambi users that the first week is the hardest and the worst of it was usually gone by a month. My symptoms seemed to get worse as time went on and as I passed the first and second month with no relief I started to lose hope that this medicine could work for me.

The physical aspect was tough, but the mentl aspect felt torturous. I dropped from 40% lung function (fev1) to 29% and despite the passing weeks, my numbers wouldn't budge. I worried endlessly as the weeks passed that I was harming myself rather than helping. I was so tight, coughing was not super productive and my exercise consisted of walking in slow motion around the house. I worried that my lack of movement and my tight chest meant that more mucus was pooling in my lungs and that I would end this Orkambi journey worse off than when I began. I felt like I was sicker than I had ever been in my life and I knew I was doing it to myself by taking this medicine. I kept waiting for a sign that things may improve, that my body was still okay, but nothing seemed any better.

And then one evening 2.5 months of worsening symptoms I broke down and cried. I had decided in the morning I would call the doctor and tell him that I just couldn't do it any longer. Physically, I was tired and mentally, I was terrified I was hurting myself and I don't have a lot of lung function to spare (at 29%). Orkambi didn't seem to be the right medication for me.

That night I went to bed and slept through the night for the first time in months. I didn't cough once! The next morning my shortness of breath had miraculously disappeared! It was a complete transformation. It was all I needed to stay on course and continue using Orkambi. My next clinic visit my lung function was 35% and although that was better than the previous 29%, it still wasn't to baseline. Regardless, I felt a huge sense of relief that at least my lungs were moving in the right direction.

During the fourth month I suddenly starting seeing all the changes in my body I had secretly hoped would happen. I started coughing up old putrid plugs from my lungs. Sinus plugs were coming out at an alarming (yet wonderful) rate. I could climb stairs and even run in the yard with my daughter. My mucus lightened in color and was much less copious compared to years past. My mucus came up easily and I started to feel stronger and more sure of my body. my morning cough has completely disappeared. I am starting to see my weight steadily climb despite starting at a healthy weight to begin with.

Clinically, I am not sure my lungs seem much different, but I feel like I just came off a round of IVs. I am in extreme awe of how well I feel without needing a hospital stay and weeks of IVs. I feel amazing and I have done nothing to work for it, no oral meds, no hospital stay, no prednisone, this hardly feels like real life. Orkambi may be considered a marginally beneficial medication and yet I feel stronger, healthier, and my lungs feel clearer. I may not gain lung function or see a huge change clinically, but I am taking something that is making my body work more like it should and that is amazing!

13 comments:

  1. This post brought tears to my eyes. Although you don't know me, I have been following your blog for years. I'm a mom with CF. I have similar lung functions as you and started off-label Kalydeco last November. It has been life changing for me as well. Would it be ok with you if I shared the link to your blog on a forum that I'm on? I really think it would help many people who have started Orkambi and can't see a light at the end of the tunnel. Or if you'd rather, I could copy and paste your post instead of share your link.
    I am so very happy for you. These drugs are changing the face of this disease. I feel like I'm living a dream at times. Although I tried my best to stay optimistic, I never truly believed that I would benefit from such a miraculous discovery. It is my hope and prayer that every last person with CF will soon find relief from this awful disease.

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    1. Yes, I would love for you to share! Thank you for asking :) It is so scary to trust that a medicine will help when initially it makes you feel so sick. I questioned my choice to stay on the medicine constantly. Sometimes knowing what the right decision is can be so hard.

      I am so happy you are able to get Kalydeco off label and that it has changed your life. There is so much real hope in the future of medicine for Cystic Fibrosis.

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    2. Also, thank you for following my blog ❤

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  2. Wonderful news!

    So happy for you, your husband , your daughter.

    So, so happy for you.

    What a twisted nightmare of having to feel sick for so long in order to feel better. I can't imagine what it was like trying to keep faith in the treatment when you felt so bad. Thank goodness you knew about this counter-intuitive route to increased healthiness from reading the reports of other Orkambi users.

    Science is amazing.

    You are amazing.

    Happy start to 2016!

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  3. So glad you are finally seeing some positive results!! Definitely scary to think of how downhill things can go at first. I don't know if I would have had the guts to give it that long to kick in. Hope you keep seeing benefits!

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    1. The only reason I stuck with it was my doctor ran a study for Orkambi and was confident that it would eventually benefit me if I gave it enough time. I am very grateful for him because if he had shown any hesitation at all I would have absolutely quit. I am just glad he was right.

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  4. I'm so impressed that you stuck it out so long, great job! I'm thrilled that you're seeing benefit. It would be great to see an exciting FEV1 number, but I've always felt that quality of life, energy, and a general healthy feeling was way more important than a number on a page. I'm so happy for you! :)

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    1. Thank you! I do agree that FEV1 is only part of the equation, although I would not mind if mine was a little higher ;)

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  5. This is such wonderful news!! I hope you'll provide an update as you keep taking Orkambi. I hope you continue to feel well!! :)

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    1. Thank you. I will keep updating if I notice any other changes. :)

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  6. Hi there,
    lovely to read your story. Very encouraging indeed. Well done for sticking with the Orkambi long enough!
    Would it be ok if I translated your story into German for a German Orkambi group, as the drug has only been here for two months now and stories like yours are rare for us. People would love to hear your story .... and I could link your blog to be completely transparent

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