Improvements on kalydeco with advanced lung disease

ladybird

New member
I have advanced, severe lung disease aka bronchietasis. My mutations are R117C and DeltaF508. Since starting Kalydeco off-label, I have had some significant changes. I started almost 2 months ago.

1. Sweat Chloride is now normal.

They tested my sweat before and after kalydeco. It went from 77 to less than 10, i.e. is now normal. The chloride was so low they could not detect a specific number.

2. Bronchial wall thickening has improved.

According to my latest CT report:

FINDINGS:

There is no change in extensive bilateral bronchiectasis which is most mass in the left upper and right lower lobes. No change in cavitary nodule in the superior segment of the right lower lobe which measures 2.9 cm. Improvement in bronchial wall thickening in the right lower lobe, with additional mild improvement in bronchial wall thickening in the left upper lobe. No change in numerous centrilobular pulmonary nodules or mosaic perfusion from the previous examination. No new consolidation or pulmonary nodule. No pleural effusion.

No lymphadenopathy.

No change in heart or mediastinum.

No suspicious bone lesion.

Visualized upper abdomen is unremarkable.

IMPRESSION:

Features of cystic fibrosis and chronic airways infection with improved bronchial wall thickening from the previous examination. No new nodules or consolidation. No change in cavitary right lower lobe nodule.

END OF IMPRESSION:

3. While my last lung function, taken 6 weeks after starting K was the same, my breathing feels deeper and I am sleeping less. I believe that due to the extent of lung damage, it may take years to fully heal.

My hope and prayer is that with intensive exercise - Pilates, yoga and walk-jogging, antioxidants - NAC and Curcumin, essential oils - eucalyptus, oregano, cinnamon, peppermint, thyme, and an organic diet rich in fruit and veggies will bring further healing. I really want to recommend Pilates to all CF patients with low lung function as it is one of the few exercises that I can manage to do and that also works up a sweat. It also tones the whole body.

I am also considering trying serrapeptase. http://www.naturalnews.com/026360_serrapeptase_natural_health.html. Has anyone had any experience with that? I am still researching as I am not sure if this enzyme would be safe.

I praise God for this amazing progress. I have never before had a chest image with reduced bronchial wall thickening. I also wanted to thank Stephen on this forum, whose posts about Kalydeco off-label first alerted me to the possibility that this breakthrough drug could help me too. Words cannot express my gratitude, Stephen. Thank you so much.:):)

NOTE: If you have residual function mutations and need help with scientific articles to support your insurance appeal to get Kalydeco off-label, I can help.


 

Aboveallislove

Super Moderator
Oh Ladybird, I have tears in my eyes as I always do when I read of the miracle these drugs are to people. That your SC went to "normal" is so amazing. I pray that the improvements continue.
 
S

stephen

Guest
Ladybird,

That’s really GREAT news!

Since your post indicating that you were finally successful in getting Kalydeco I’ve been wondering how you were doing. Not seeing any updates, I was afraid that your experiences with it weren’t so great. Thankfully that’s not the case.

It’s also nice to see your offer to provide others with information to help them appeal insurance rejections. When I first got Kalydeco, both my doctor and I were quite surprised that there were no insurance problems, so I had no real advice to give.

My doctor had prescribed it right after it got FDA approval for the additional eight mutations in February of 2014. He thought there might be some confusion between my mutation and those that were just approved. I’m not sure if that was really the case, but there have been no problems getting refills.

My doctor did indicate that the CF center team has expended considerable efforts in appealing insurance rejections for others to get Kalydeco off label. Some appeals were successful, some were not. One patient who was getting Kalydeco subsequently had it denied. Submitting data showing it was effective for him got the rejection reversed. Only recently have my concerns subsided every time I order a refill.

It sounds like despite the success Kalydeco is providing, you’re not going to stop all other types of treatment. That’s probably smart. I was doing so well this past 15 months that on a recent 25 day trip to Israel I did stop all other treatments. That was probably a mistake. I began to have increased difficulties breathing when walking the very hilly streets of Jerusalem. Upon returning home I resumed nebulizing hypertonic saline once a day and Pulmozyme several times a week and thankfully my breathing difficulties promptly subsided.

Let me add my wishes that your success with Kalydeco continues. Hopefully, in a short time many others will have access to Kalydeco/Orkambi and experience the improvements we have.
 
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