Still getting reports such as “Trikafta cuts need long-term for other CF treatments in real world” which are reporting only on the effects of the modulators on the lungs being the only benefits. Other changes are reported, when they are reported, as side effects generally in negative tones. There seems an ongoing ‘Ostrich’ effect where the…

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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…

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My wife was diagnosed aged 67. Is now 82. We suspected CT for nearly 10 years before that as the cause of her episodes of ‘Asthma and shortness of breath. Also severe bowell disease. But when asked Drs would universally reply ‘not possible! You would be dead by now.” Even after diagnosis many dismissed CF as a mis-diagnosis. Recent…

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Great to hear your story, Robert-lane. What else has changed/improved with Trikafta?

What has been reported/documented? Sadly Vertex and many CF Physicians appear to think pwCF are just lungs. No other organs in the body are important. You had year of allergies and infertility. Many have a range of ‘co-morbidities’ – most of which, if not…

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The Alfred Hospital is a large hospital in Melbourne Australia which hosts one of the 2 Adult CF Centres in Victoria, Australia. The other is at Monash Hospital. Children are seen at Monash and also The Children’s Hospital in Flemington. Ali 4 are excellent centres. All do have a psychiatrist on staff.

Jeanne asked: “what does compound heterozygous mean?” It refers to there being different mutations on each chromosome, in my wife’s case G542X on one and R75Q on the other. G542X is a class 1 nonsense mutation that results in no effective CFTR protein. R75Q does produce some CFTR but it is less effective than normal CFTR. For that reason…

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The study, “Assessing the impact of elexacaftor/tezacaftor/ivacaftor on anxiety & depression symptom scores in adults with Cystic Fibrosis,” was published in the Journal of Cystic Fibrosis. This paper is interesting and supports the need for further research. It does support the thesis that in CF the Faulty Chloride channel may be affecting…

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Hi William. I would like to see more research into the co-morbidities seen in pwCF. Into why pwCF get so many and why. Research into what the faulty CFTR is doing to the electrolytes within the body’s cells as I suspect abnormal electrolyte levels within cells may be the cause of many co-morbidities. I suspect Tricafta (TK) may be normalizing…

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My wife was diagnosed in 2009 aged 67 after years of bowel and respiratory problems. As a child & teenager was diagnosed as having bronchitis and constipation and learnt that riding her bike vigorously enabled her to expel mucous that was causing her cough. In her 20’s and 30’s had 2 episodes where she did not tolerate living at altitudes of…

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My wife definitely has – from some hospitalizations that did not go well. And COVID did not help. She was helped revently by a good talk with the Psychologist on the CF team at the Alfred.

Multiple diagnoses are common in CF. Asthma is a diagnosis that simply means ‘difficult breathing’ and has many causes of which CF is one. Every pwCF has difficult breathing to some degree. But pwCF do have a plethora of co-morbidities – oft related to the thick mucous in the lungs, resp tract, bowel, bile ducts, uterus, and other organs…

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Hi JP, Yes TK is a gamechanger and we don’t know all we need about it. A lower dose is adequate in some people and reduces side effects. Side effects are not well understood – many seem that it is doing its job too well. Damage in the lungs has been progressing in most pwCF for years and some anti-inflams may be warranted. The new Phage…

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Hi Kay,

my wife attends the CF Clinic in the Alfred Hospital in Melbourne. She was found to have G542X and R75Q when her gene was sequenced by the Canterbury, NZ lab in 2009. Her sweat test was equivocal but her list of symptoms were 20 of 25 for CF. I would reply to your Dr who said it is not worth finding your other mutation “Bulldust! It…

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Unfortunately, it seems that still far to many of our CF Experts are experts in lungs only, appearing to be in denial that any other organs than mucous membranes are affected by the faulty CFTR protein and Chloride channel. Except they do recognize that sweat glands are affected. Even the registries fail to record the extent of CF…

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Hi Kay. No I am not in a CF Clinic just a humble husband of a wife who does have CF and has done so for nearly 82 years but only diagnosed 15 years ago. She has one of the 100 or so mutations, R75Q; of which just 9 are recorded in the Australian CF Registry and is probably the only person in Australia with that variant and not F508del on…

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Are they going to try you on a lower dose of Trikafta, Orkambi or on Lumacaftor (an earlier drug)? That often reduces the liver damage. It appears your sarcoidosis is an inflammatory response to past infections.

The CSIRO, Australia’s government owned and funded research organization, has developed a diet that is recommended for Diabetes and obesity – indeed most persons. It is published in a number of their books, one of which I have attached.

This seems common with CFRD (probably CFRD is called Type 3C by some). My wife’s blood sugar (BG) swings widely sometimes even when she has eaten nothing. Usually dips overnight and may be blow 3mmol/L about 3-6am. At times has been difficult to get up. We have found adding glucose to an electrolyte drink seems to work best. It seems…

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Aspirin (Acetyl Salicylic Acid) is an anti-inflammatory and reasonable pain killer, but not usually classed amongst the NSAIDs and is not a steroid such as Prednisolone and Cortisol are. It is an extract from the bark of the willow tree. It has many benefits but also like all drugs some issues. it is irritating to the stomach and can…

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Hi J-P,

Lyrica (Pregabalin) is a rather strong pain killer that works best in neurogenic pain. Was originally developed for epilepsy. Needs reasonable supervision with a particular check for suicidal ideation. Otherwise is very good but can be addictive.

With all drugs we should read the Product Information sheets. In Australia we have 2 forms,…

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The drugs you mentioned are quite varied: some are anti-inflammatories and some anti-allergy . Both have their place – we have found low doses of Ibuprofen do improve breathing. Usual dose is 2x200mg 4x a day but we find just 1 capsule once of twice a day is adequate. It is a Nonsteroidal Anti-inflamatory (NSAID). Need to watch kidneys &…

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One may question the cause of your sarcoidosis. little is known about it in ‘normal’ people though like Sjogren’s it is thought to be autoimmune. In CF both these conditions appear to be inflammatory reactions in the damaged lungs. Could be a sign of the presence of Mycobacteria, a bacteria not uncommonly seen in pwCF.

The latest news from Aus:

1. Ivacaftor has been recommended by the PBAC (The Pharmaceutical Benefits Advisory Committee – a Gov Committee) that it should be available to any pwCF who has a mutation where there is evidence it should help. This will make it available for about 10 persons in Aus. that can’t get it now, incl Reva. Just have to…

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One may question the cause of your sarcoidosis. little is known about it in ‘normal’ people though like Sjogren’s it is thought to be autoimmune. In CF both these conditions appear to be inflammatory reactions in the damaged lungs. Could be a sign of the presence of Mycobacteria, a bacteria not uncommonly seen in pwCF.

“More vigorous exercise boosts life quality, lung health with CF” was posted today. It is well known the benefits of exercise is great for pwCF as with all people but the conclusions of an article such as this are flawed by the question ‘which is the chicken and the egg?’ The conclusion made was that those who exercised better had better… Read more

High fat diet is proving to be unnecessary even harmful in persons who have responded to one of these modulators or Trikafta. And the drugs seem to be having benefits in the function of many other organs, Adrenal and other endocrine glands, heart, mental illness and others.

Reporting of these effects are needed not just as side effects but…

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Kay, Sorry your experience has been so poor. You should be asking for further genetic testing as the unknown mutant may be one that responds to a modulator (eg Trikafta). If it does then that would improve your treatment immensely.

I am looking forward to:

1: Trikafta being available to those who have mutations other than F508del that Vertex has shown to respond.

2: Greater awareness of the effect of the faulty CFTR as the cause of the so many ‘co-morbidities’ seen in pwCF. It appears that these are caused by abnormal electrolytes within cells caused by the Chloride ion…

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As William said – Talk to your Doctor.

Your diet as a person with CF has been designed to cope with your gut not absorbing food well. With starting TK that changed though some damage may persist – take time to heal or even be permanent. Fibre is important in the functioning of a normal gut but how normal is yours at this time. Other drugs can…

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Yes the benefits to the lungs is amazing and MUST not be sidelined. But in assessing Lumacaftor, Trikafta & other therapies for pwCF the co-morbidities also should not be ignored. Too often the effects on other body systems are reported as adverse and undesirable side effects without any attempts of trying to understand why they are…

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Don’t!

Unless your Dr prescribes and closely supervises them.

Premature death and heart failure are all too common .

The last section of my post yesterday was was rendered somewhat incomprehensible by formatting instructions. I commented on the report received yesterday:

“”Trikafta leads to clinical gains for 2 adults with rare M1101K mutation”

I commented that while wonderful, it was an example reporting on the lungs but nothing as to whether improvements…

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No. Not much snow in Melbourne – we do get the occasional flurry on the Dandenongs – hills on our eastern border but very rare in the city. Do have snow on the Australian Alps.

As I have said before:

Beautiful one day – perfect the next!

Though some say we have all four seasons in one day – can have. Stops us getting bored.

An update:

3 Months ago Reva was admitted ’cause she had splenic infarcts causing severe pain and caused by clots been thrown off from the left atrium. Was in hospital for 7 weeks. She now has a device blocking off the left Atrial Appendage which should reduce clots being formed by 90%. The Alfred Hospital which has the CF Unit just wanted to…

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I prefer not to fall. falling in hospital causes too much paperwork! We have a beautiful spring day here in Melbourne. Lovely day – Beautiful one day – perfect the next!

Hi All,

The last 2 months have been just horrendous. First mid June my wife had a viral infection that put her in hospital for 4 days, then 2 weeks later her chronic discomfort became severe pain so sought out an emergency GP to get better pain relief. During the triage exam the nurse called in the GP who on examining her immediately referred… Read more

I agree; the medicines taken will almost certainly have a profoundeffect. My thesis of ‘Intracellyte Electrolytes’ affecting organs and systems that are not just mucous membranes open an understanding of CF and the multiple co-morbidities seen in pwCF to greater understanding. It explains that Hypokalaemia may well be the result of abnormal… Read more

Persons with CF (pwCF) have a long history of depression and anxiety. There are so many reasons why this is so -anxiety about what tomorrow may bring, deteriorating health, being a burden being just a few.

The faulty CFTR Chloride channel may be expected to lead to higher Chloride in the intracellular environment which may be expected to… Read more

‘Tis logical that we see a reduction in inflammation in persons taking these modulators. More normal mucous  which can be more easily swept from the airways may be expected to reduce the bacterial in the lungs. Similarly, more normal mucous on other mucous membranes may be expected to reduce the bacterial load on those surfaces.

But what are… Read more

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… Read more

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… Read more

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… Read more

My father said: “If work interferes with play, give up work!”

He did and then he died (some 20 years later at 86).

It still appears that these issues with Trikafta (TK) and electrolytes are still not being addressed by Vertex nor by most if not all researchers and CF Physicians.  CF Physicians need to be not just specialists in Respiratory Medicine but good General Physicians.

The discussion on this needs to progress. Side effects of TK need… Read more

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.

We appreciated 5 Feet apart. Yes it was not perfect and it dealt with one particular couple. Others with CF may have different experiences. No two relationships are the same nor are experiences with CF the same. There is much that is common but we are all unique. So no one film or documentary will cover all experiences. But 5′ Apart did deal… Read more

Hi All, Any more reports of side effects? Please keep them coming. Any heart issues? Endocrine? Muscle strength and Neurological?