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Liver Problems
I’d been on Trikafta 3 months, was just starting month 4 at the end of Nov. I got bloodwork done and my liver values were off, with enzymes pretty high. Results got in at the end of a Friday, & I got sent to the ER (a waiting room full of people with flu, covid and respiratory virus!). My liver was painful and swollen – “inlflamed” per the ultrasound. I had been having GI problems already and thought that was why I was feeling pretty lousy. But I was feeling lousy for multiple reasons. It turned out I also had a pretty significant UTI.
I got taken off the Trikafta immediately, of course. I continued to feel lousy for about another month. Took about two weeks for the UTI antibiotics to knock the UTI down. Dr’s have had me getting a weekly Hepatic Panel – liver enzyme values started coming down each week. It took another month to start feeling gradually better. I do now feel better than I’ve felt in months! My liver enzymes were still a bit high in my last hepatic panel, though. But now under 100, rather than over 300!
Sadly to me, after only 2 or 2.5 weeks after stopping the Trikafta, I felt the beneficial parts of what Trikafta was doing for me reversing. For me this was in my sinuses, earache came back (it’s intermittent), joint stiffness came back, and dry eyes came back with a vengeance. My gut had already been on a rampage for months and so I have no idea about if Trikafta was helping my gut, or not.
As for my lungs, on Nov. 2nd I’d had a bronchoscopy which showed that in fact I don’t have any bacteria in my lung airways, but I do have pulmonary sarcoidosis (outside the airways and in my lymph nodes). Next week I will finally get to see a pulmonologist who specializes in sarcoidosis (which is now what is thought to be causing my shortness of breath). Anyway, I felt no change in my lungs regarding the Tritakfta… or at least nothing I could figure out.
There was talk from a NP in the CF Clinic that if the hepatologist clears me, I might go on a reduced dose of Trikafta. All as yet to be determined.
Throughout this my already low weight dropped 10 (US) pounds. In the past 3 weeks I have gained back 4 1/2 pounds, which really is helping my energy level. I’ve really been working at it. It is more work than most people understand.
Tomorrow I have a CF Clinic check in. Then the next day, the hepatologist appointment. Then next week the “sarcoid pulmonologist”. I’ll try to remember to come here and report back.
Happy 2023 everyone! -jeanne-
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11 Replies
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Jeanne, I’m so sorry to hear about your issues this past November! I am, however, happy to hear you’re back on the mend. I know from personal experience how hard it is to gain weight (I struggle constantly), but four pounds isn’t anything to slouch at. Keep up the good work!
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Thank you, William! Besides you, only my closest friends help me celebrate with no jokes such as “I’d be glad give you some of my extra weight.”.
Found out today at my CF Clinic appointment, that I will not be put on Trikafta again. I guess it’s good news in a way – So if my lungs didn’t need the Trikafta – as the bronchoscopy revealed – then it’s not worth the risk of what Trikafta did to my liver for the benefit I got for my sinus issues and other unexpected benefits. But I was hoping that Trikafta would help me gain weight. So now I just have to do that on my own.
Sprouted brown rice protein powder in smoothies is a new food thing I’m working with. I have “concocted” some pretty tasty 24g protein smoothies. -jeanne-
-jeanne-
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Have your CF Drs explained why the liver was affected? No it does not suit everyone. But why? We can expect that the liver cells will be affected by changed electrolyte transport. Maybe that caused the bile output to increase into an already congested/partly blocked gall system. Have you gall stones? Many pwCF do.
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Tim, so sorry that i did not reply! I have been so busy lately – and in some ways enjoying the boost of being on a high dose of prednisone for the sarcoidosis.
Liver enzyme issues are a known/sometimes side effect of Trikafta. And they usually resolve, and mine finally did! Last week I had a CF Clinic check up and a hepatic panel showed my AST and ALT enzymes finally back down within normal limits. Yay!
Have never had gall stones. A CT with contrast scan about 3 or 4 months ago showed my liver looking fine, even when the enzymes were still up. Glad to have those enzymes down. Now I know what it feels like to have one’s liver not working so well, and it was not a good feeling.
I started the prednisone treatment (for “sarcoid”) the 2nd week in March. I am in week 3 now. It is a 4 month course, decreasing by 5mg each month, then off. I’m on a sulfa drug just 3x a week to guard off bacterial infections. Both the CF and ILD (interstitial lung disease) clinics consulted together and gave the ‘OK’ to try the prednisone.
I’m having some of the usual prednisone side effects (shakiness, sleep issues), but none of the really bad/scary ones. Also having a lot more energy… and a lot more air! It’s odd, a spirometry test result meant little to me 14 months ago… but last week I was quite excited to have gotten my best score “ever”.
I haven’t gained significant weight – 1 US pound – , but expecting I still might – which would be good (if it sticks). The hope is that this prednisone treatment kicks my sarcoidosis down… and then fingers crossed that that lasts for several years. Meanwhile, it has reduced my sinus swelling, as I knew, from childhood treatments with cortisone, that it would.
-jeanne-
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Good luck with prednisone! When it’s good, it’s great. When it’s bad, well, you know…
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Yes prednisolone is a wonderful drug BUT with many problems. While on it your Adrenal Glands will be suppressed as it replaces the Adrenals normal production of Cortisol. I’m not quite sure how your Sarcoidosis fits in with CF but I doubt many even the experts do. Little is known as to what causes it – is probably like Sjogren’s Syndrome a result of chronic inflamation affecting the immune system. You may need to remain on a low dose of Pred. to maintain control – time will tell. If you need to stay on a low dose then you may have to increase it for a time when you are under stress (infections, lung exacerbations, etc). So be it!
Adrenal function can be expected to be affected by CF as the gland not only produces Cortisol but also regulates Sodium and Potassium by the secretion of Aldosterone hence pwCF often have hypokalaemia (Low blood Potassium).
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Definitely don’t want your potassium clotting post blood test. It ruins the results and makes you think your potassium is worse than it is. (I’m speaking from experience.)
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Hi, Tim & William. Yeah, when I first heard that prednisone is the usual first line of treatment for sarcoidosis, I thought I hope I don’t need it I hope I don’t need to have it. But my lung CT scan between last March and October worsened considerably, as did my shortness of breath and fatigue. I think the weight loss was caused by a combination of the sarcoid, CF, liver problem, and joining that was a bad UTI. I felt so crummy in December… it is hard to believe how much better I now feel.
Prednisone drops 1/4 dose this next Thursday. I am already wondering how much energy I’ll feel like I lose. Odd – I was so scared of this prednisone but now I’m really enjoying that part of the medication.
The ILD Clinic seeing me said nothing about any continuing low-dose of prednisone. I’m not going to dwell on it, just have to go with it, stay positive, and weigh decisions.
It does turn out that having both CF and sarcoidosis is quite rare. My CF however is a bizarre edge case, with my one “oddball” gene for which little is known. Seems to impact my gut and sinuses mostly.
Overall, I still feel somewhat guilty about what I face CF-wise: comparatively lucky next to those who have CF in one of its aggressively infamous forms. Now I have the sarcoidosis to deal with also. At least the word “granuloma” has a fun feel when it rolls off the tongue!
Tim, you’re a fount of info – thank you for sharing!
William, I read your piece about the lung transplant and it really hit me in the gut. I cannot even imagine. Thanks for sharing your time and talents to be the Moderator.
-jeanne-
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In your post of 23 Jan you said “Sadly to me, after only 2 or 2.5 weeks after stopping the Trikafta, I felt the beneficial parts of what Trikafta was doing for me reversing. For me this was in my sinuses, earache came back (it’s intermittent), joint stiffness came back, and dry eyes came back with a vengeance. My gut had already been on a rampage for months and so I have no idea about if Trikafta was helping my gut, or not.”
Such description does give a glimpse about what CF is doing in the body of a pwCF. That it is not just about thick mucous and mucous membranes. Yes the sinuses, ear ache and dry eyes may well be so but joints? – no mucous there! But electrolyte abnormalities caused by the faulty Chloride transport is probable. Was your Adrenal function tested? Abnormal? Muscle strength changed?
This is a glimpse of how the co-morbidities in pwCf are most probably caused by the faulty CFTR gene and Chloride transport.
Sarcoidosis, like Sjogrens Syndrome, is considerred an”Autoimmune Disease” – caused by an overreactive immune system – in pwCF is probable a response to chronic inflamation caused by CF. Inflamation that has being occurring in pwCf from the day they got their first infection.
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Hi, Tim. WRT “joint stiffness”, what I discussed with both my hand specialist and also CF lead doctor was about it being the synovial fluid. The CF doctor said he hadn’t heard of positive impact to synovial fluid before, but that he would not doubt it could be so.
Some backstory: when I was 50, 17 years ago, I had a knee surgery on my right knee. They had to do a little exploring to find the problem as the MRI was unclear. Found a torn flap of cartilage which apparently had been getting stuck in the wrong position but then straightening out, causing 2 years of bad intermittent flare-ups countered by mysterious phases of being OK. After my surgery, my surgeon told me that he had never seen such “lousy” synovial fluid in a person as “young” as 50. He said I didn’t have enough of it, and it was too thick, and that I would likely continue to have knee problems (that’s been true).
Currently, my hand joints are behaving quite a bit better again. Probably partly the prednisone. Though it seemed like my body really rebelled when the Trikafta was taken away, but settled down a bit after 6-8 weeks. Could that joint stiffness possibly also relate to my liver problem? It took nearly 4 months before my liver enzymes dropped back into the normal range. Now I’m on the prednisone, and my hand joints are feeling pretty good. And the earache is gone again, too, even though it’s high allergy season here. (As a kid, I was sometimes given cortisone injections – not local but “whole body” cortisone, to control allergies and ward off sinus infections).
So far, no one seems to think that my sarcoidosis relates to being a pwCF. It is just “very rare”, is all I’ve heard. It’s convenient that my 2 lung clinics are right next to one another.
Oh, my prednisone dosage dropped last week to 3/4 of what it was. And I can feel the drop in energy, but lung function is still feeling quite good. I’m happy to be walking some 3 mile, no elevation changes, walks… 3 miles in an hour. Very good to have that back and I’ll continue to push my walking, in hope I get to hold onto the improved lung function after I finish this course of prednisone.
Still and always feeling a bit guilty for being a pwCF yet so generally lucky, symptom-wise, compared to most pwCF. The lung clinics I go to are in a teaching hospital, and I’m part of several studies. I hope they end up learning some things from my unusual combination of genes and symptoms. -jeanne-
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Never be afraid to ask the awkward questions and keep pushing to get better results!
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Hi Jeanne, you said “I hope they end up learning some things from my unusual combination of genes and symptoms”. I am sure they will. You haven’t lived for 67 years without it telling you something. Re Joint fluid; interesting that it was ‘so bad’ – in what way? Thick and ropey? Could be that it is affected by the same type of mechanism that causes mucous to be thick. PwCF are known for their CF Arthropathy. Would be interesting to analyze the electrolytes (esp salt) in it. The sliminess in it is a property of Hyaluronic Acid in it. Vets have been injecting that into the joints of dogs and horses for decades to treat arthritis and make horses run better.
Keep up asking the awkward questions!
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