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Today the CFTrust launched its CFTruths campaign once more concentrating on the pulmonary effects of CF. While the effects of thick mucous in the lungs is the most serious issue in most with CF limiting the discussion to just that seriously short changes most. Bowell and pancreatic problems are well recognised and attributed to the viscous mucous but is that all. Many pwCf have a plethora of Co-morbidities and renal and hepatic failure are all too common. Obstruction of airways and ducts does not explain failure of the endocrine glands such as the Islets of Langerhans in the Pancreas (causing Diabetes), nor Adrenal dysfunction causing Hypokalaemia and Addisons Disease. Hyperparathyroidism too occurs despite the Parathyroid Gland having no goblet cells secreting mucous. Heart failure was reported as long ago as 1980.
Many of these so called Co-morbidities cause considerable distress and disability in pwCF, are often overlooked by the superb Respiratory Physicians leading CF Teams. Unfortunately too, specialists in other fields rarely understand the role of the faulty CF gene in their cause.
The thick mucous is well ascribed to the failure of the Chloride transport from the cell caused by the faulty CFTR channel and gene. If the Chloride (an anion) is not being pumped out then the levels within the cells must be high and must be balanced with cations esp Potassium (K) and Calcium (Ca). High K within the Adrenal cells will surely make the cells ‘think’ K is too high and increase Aldosterone secretion, causing hypokalaemia. Similarly high Ca within the Parathyroid cells will affect PTH and the Ca metabolism balance. K & Ca are also involved in the function of muscle cells (incl Heart Muscle), neurons and to some extent every cell of the body. No wonder pwCF are so complex!
We do need the CFF to lead research into these co-morbidities. Cardiologists, Endocrinologists and other Specialists need to understand the effect of the faulty CFTR channel and gene is having in the organ they specialize in. To date, they usually don’t. Cardiologists do not understand why pwCF and heart failure often have severe problems with many heart drugs. ACI’s, ARB’s & Ca Channel blockers often increase blood pressure rather than decrease it.
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Very true indeed Tim. CF is working all over the body in many systems in ways many of which are not yet known.CF care is currently organised in little islands of expertise, that are scattered and incomplete. Often they do not work together very well either. There needs to be an interdisciplinary approach both in treatment and in research. The focus on only the lungs and digestion, however understandable from an historic point of view, must vanish. Perhaps we need a real super cf specialist, who is proficient in all the systems of the body that can be affected by cf. This could be a specialisation within internal medicine, the cf-internist. And under her suoervision, the normal specialists would work together in a team to optimise treatmemt for each individual pwcf in the clinic.
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On Wed had a consult with Reva’s cardiologist. Once more he showed that he has no understanding of CF – no understanding that in pwCF heart disease does occur. With all Reva’s co-morbidities I find it impossible to believe that they are all unrelated in causality including the Heart. My theory is about intracellular electrolyte inbalance (high Cl, K & Ca in particular) is causing intracellular fluid retention and cellular dysfunction. But he is unwilling to listen and consider possibilities beyond the textbook protocols. He is a good technician, less so a good doctor. We have not found a cardiologist that is willing to “think beyond the box”.
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Tim, this can be so frustrating! As you mentioned, there is real cause to believe these things are related. I’m sorry you’ve yet to find a cardiologist who is willing to entertain and investigate your theory. My life has been quite literally been saved by doctors who’ve been willing to listen and act on my concerns and ideas. I hope that Reva will be able to find a physician willing to do the same.
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That is exactly the problem.
Tim, are there any specific tests or examinations or treatments or strategy you can think of yourself that you would like your cardiologist to look into or to perform? Your theory sounds almost self evident to me, but perhaps is too much for your doctor to accept. When you present it as small practical steps he might be more inclined to look into it. Often when doctors feel helpless, they hide behind textbook knowledge. What would you have been willing to do as a vet in a situation like this, when a dog owner presents his pet to you with strange comorbidities and a theory of his own, that is not in your textbook or education? How would he have been able to make you overcome your position and go along to assess whether or not his theory holds ground? Try to find out this for yourself and then apply it on your cardiologist, try to break through his passivity, try to evoke his curiosity, give him something to look into in a way he is familiar with. I gather you will not be satisfied with him to only acknowledge your theory, you want him to do things. So bypass the convincing, the theory, and think of something to make him do things you deem useful anyway.-
Thanks Paul for your encouragement. Last Monday week our cardiologist threw a spacko because our Endocrinologist had not put Reva on a SGLT2 inhibitor. The result is the Endo has acted and Reva has now started on dapagliflozin with promising results. Dapagliflozin is has been in use in diabetes for some years and has been shown to be beneficial in heart failure especially in HFrEF. Recently it has been registered for such and also for weight loss.
It does have some issues that must be watched for: 1. can cause Ketosis but that is rare and 2: can predispose to thrush (quite common).
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Wow, that is indeed important Tim. Since 2019 (DAPA-HF study by McMurray et al.) it is known that added SGLT2 inhibitors (10 mg dapaglifonzine daily) have good results in case of heartfailure with HFrEF (LVEF <40%) indeed. Recently this is also deemed useful in case HFmEF (LVEF between 40 and 50%). The DAPA study was a large one, 4744 patients were included of which half were on placebo and half on dapaglifozine and the results had a medium GRADE value and a relatively low NNT of 20. It seems a good thing to try and watch, indeed there are some unwanted effects to be monitored, especially in patients with diabetes.
We sincerely hope for the both of you that Reva’s health and well being will improve from this!
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Hi all,
The good news from Australia this week is that TRIKAFTA IS TO BE AVAILABLE FOR ALL over 12 years with one copy of F508del. on the PBS from Friday
The sad news is that it is not available for those who have one of the rare mutations (eg R75Q) but not F508. These may be few such that I call them the “Forgotten 10”. Most with these rare mutations (there are about 100 in Aussie) will be compound heterozygous with F508 on their other chromosome but about 10 in Australia do not. They will miss out despite VERTEX having shown that pwCF with about 120 mutations should benefit. They will miss out as a result of the bureaucracy and red tape of the Australian registration system.
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To Throw a Spacko! Thanks for this expression Tim, I didn’t know it and couldn’t even find it on the net. Does it have something to do with being “spastic” from anger/amazement? aka to throw a fit, flip one’s lid, blow up, hit the roof, hit the ceiling, have kittens (! nice one for you), combust, blow one’s stack, fly of the handle, flip one’s wig, blow a fuse, go ballistic?
Anyway, I would think both the cardiologist and the endocrinologist are equally at fault here and they should throw a stone at themselves.
This is typical for what happens because of the fragmentation of specialist healthcare in general, and in CF care in particular. A medicine that is commonly used for diabetes (and hence prescribed by the endo) turns out to be effective for heart failure (and hence is on the field of the cardio). And neither of them picks it up for three years, perhaps thinking that the collegue will do it and/or afraid to ask about it and overstep some imaginary borders of competence. This stresses my appeal for a CF super specialist who coordinates the care and medication. The current system of CF teams does not work sufficiently at all.
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Yes. To throw a spacko is precisely as you suspected. Australian jargon. We have some wonderful terms. Australians are the only people in the world that can call a dark horse a fair cow and get away with it! ‘fair cow’ = a very badly behaved animal.
We still have much work to get our CF teams headed by Drs with broad understanding of CF – indeed, as you say, a CF super specialist .
Keep peddling!
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Maybe that shouldn’t thrown stones at each other – just learn & learn to work together.
While in Kenya some 50 years ago there were 2 waring tribes. One invaded the other and carried off the Chief’s throne. The victors installed it in their Chief’s hut (It was much more grand than their own). Then they had to find somewhere to store the old throne – could not throw it out as after all it was their Chief’s. They stored it in the rafters of the Chief’s hut. After a while, after the white ants took one too many bites, there was a loud crack and the old throne came crashing down on the Chief, killing him stone dead!
The moral is: People who live in grass houses shouldn’t stow thrones!
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Then there was the American Indian Chief who had 3 wives. His favourite was huge. He went to Africa on a hunting trip and shot an hippopotamus and 2 gazelles, the hides of which he took back home, one for each wife.
The squaw on the hippopotamus was equal to the sum of the squaws on the other two hides.
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