CF Therapies Symdeko and Orkambi Seen to Also Ease Inflammation

[Paul met Debbie]

And Trikafta/Kaftrio seems to do this as well. At least in my experience, since taking Kaftrio inflammation is almost absent. How about you?

[William]

I personally look forward to the day that I can experience one of these medicines. Thankfully my lungs have been okay, otherwise.

[Tim Blowfield]

‘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 we seeing in other organs? Are we seeing improved Adrenal function?  Improvement in osteoporosis from both an improvement in digestion and in Parathyroid function? And muscle (incl cardiac) function? (improvement due to improvement in the intracellular electrolyte environment?)

So much we need to learn! as these drugs may be expected to affect the function of every cell in the body. Let us look beyond just the lungs and see just what these drugs are doing.

The old high calorie, high fat diet previously seen as suitable for those with CF may now be inappropriate for persons on modulators as digestion improves and weight increases.

 

[William]

I think the high fat, high calorie diet is slowly being moved away from the CF Community. Now, yes, some people still have to abide by it (like myself), but my understanding is that it’s not the necessity it once was as patients find themselves going through digestion problems due to that diet. In regards to the other stuff you mentioned, I’m not sure we’ll understand the full negative and positive results of what the modulators have caused.

[Tim Blowfield]

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 there needs be discussion about them. Are we seeing improvements in Osteoporisis?

Headaches are reported as a common adverse side effect. But what is really happenning here? In most they seem to wane with time. Are they evidence that the brain is adjusting to changed intracellular electrolyte levels?

Many pwCF with CFRD have reported improvements. This suggests that the Islet cells are responding.

Abnormal intracellular electrolytes appear to be the cause of many of the so called co-morbidities seen in pwCF. Logical? It certainly appears that the faulty CFTR is causing high chloride within cells with corresponding high cations (K, Ca, etc). Some cells may be little affected but in others the effect may be profound: Adrenal and Parathyroid Glands, Muscles incl Heart muscles, etc.

The study of CF needs much lateral thinking about this. While the lung issues will long be most important, Intracellular electrolyte abnormalities may be causing a host of ignored issues.