New CF modulator

[cfmox]

My wife was diagnosed with CF at 55yrs- no symptoms before that but they are pretty classical now. She’s had a difficult time with trikafta- it radically improved her symptoms but it seems to be triggering substantial depression/anxiety and cognitive effects to the point we’ve had to stop it twice and are back on a half dose to see if she can stand that. She had a very bad reaction to Alyftrek- severe cognitive effects the day after the 1st dose and its taken weeks for her to get back to normal… scary stuff; she couldn’t recall her doctor’s name or any of her passwords, I had to hack into her accounts to get a response moving.

Not sure if she’ll be able to go back to work.. she’s on short term disability for another month or so. Her lung function is pretty bad but stable- she needs supplemental oxy at rest. Not complaining.. there is much to be thankful for- but CF is a long game for everyone involved.

So please, if you’re taking Trikafta or Alyftrek, be careful with those side effects!

Both times she went off the deep end with trikafta there was a “warning period” of about a week when her voice was affected- becoming very breathy and weaker than normal. That period passed and a week or two later and depression/anxiety started to go out of control. I’m on a hair-trigger now- if that voice stuff starts up again I’ll be advocating stopping the meds right away.

[William]

It’s good that you are aware of the warning signs and want to advocate for her to stop the medicine as you see how much it is negatively impacting her. Ultimately, it’s her decision and a choice that should be made with the help of a medical professional.

[Tim Blowfield]

Such is Trikafta. Sorry to hear of such a bad reaction. But it does show that TK and CF affects NOT Just the lungs nor just the organs with mucous membranes. The depression in this case is obviously the result of the electrolyte changes within the brain cells. These will have reduced as the CFTR Chloride channel improved leading to lower intracellular Chloride and lower balancing cations, Na, K etc. Has she been followed up by a Psychiatrist? What of other organs? heart and muscles? Adrenals? Hypokalemia?

[William]

To piggy back on top of this, she should maybe getting routine bloodwork done, whether it’s every week, two weeks, or month.

[cfmox]

Trikafta sure is a fancy complicated drug so complicated side effects aren’t surprising. In consultation with her docs we’re easing back into it, at first a half dose, and tapering up to full, while observing the reaction <very> closely. So far she’s not shown other physiological issues- docs are monitoring it.

Her pulmonary function is pretty bad but at least has been stable over the last few months. Her next PFT is on the 31st, the practical goal being to ease her need for supplemental o2 so trikafta and alyftrek are definitely still on the table. Its also true that the appearance of the impairment and drama could well be coincidental.. probably hasty to start talking causality.

This time around she is engaging a lot more with her CF buddies and making an effort to stay engaged- she is very prone to anxiety and depression. The biggest physiological symptom she showed before the CF diagnosis was a chronic cough and constant sinus drainage. When she first started trikafta that stuff cleared up in about 2 weeks, after several years of it.

Since I’m an engineer by trade, my job is to manage the supplemental o2 at home- so I’ve been learning a lot about the used medical device market as related to concentrators and pumps. She’s relatively mobile now, we have “car tanks” and a mobile tank in a roller laptop bag so its possible for her to visit her buddies and so on. Next hurdle is for her to get back to work; she has to go in to get her badge and laptop straightened out- so having a good supplemental o2 system is helpful. So far her o2 need is larger than what the portable battery concentrators can produce.