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CF Patients Improve Lung Function with Elexacaftor Combo, Data Show

Jan 02, 2030 04:44 am | Cystic Fibrosis News Moderator

Replies: 0

Adding elexacaftor to tezacaftor and ivacaftor leads to marked improvements in lung function and quality of life in people with cystic fibrosis, data show. Read more about it here.
What do you think of this news?

The Basics: CF not just a Lung Disease

Aug 12, 2020 11:12 pm | Tim Blowfield

Replies: 1
CF was first defined in 1937 as a cystic disease of the mucous membranes of the lungs, intestines and pancreas. Before that there was recognised a ‘Salty Baby Syndrome’ in which babies with salty sweat failed to thrive. Over the years understanding has improved and in 1989 the genetic cause was shown to be a faulty CFTR gene. This provided further understanding of the lung and bowel disease due to poor hydration of mucous. The CFTR gene is enormous with by now over 2000 variants or mutations known of which some cause severe disease but others mild or even no illness.
Concentration has been on the organs most severely affected the lungs and intestines and on how the absence or dysfuncfion of the CFTR on the passage of Chloride accross the epithelial cell wall leaves mucous dehydrated and thickened. Thickened mucous blocks small airways, pancreatic ducts, and even the ducts transporting sperm. It affects the function and absorption of and in the intestines.
But is that all?
CF affected persons present with a multitude of issues on top of lung disease. Endocrinopathies and heart disease are well reported. It is apparent that epithelial cells secreting mucous are not the only cells affected. Vasculitis is commonly reported in CF affected persons as are cardiomyopathies and hypokalaemia (Low blood Potassium).
It is reasonable to postulate that the faulty CFTR affects Chloride transport not just in the epithelial cells of lungs and skin but such failure may be expected to leave high Chloride (an anion) within the cells that is balanced by increased levels of cations (Potassium and Calcium being the most important). These cations are important in cardiac and other muscle function, Adrenal function, the parathyroids (affect calcium metabolism and the bones (Ostepaenia/perosis)).
While not neglecting the need for further progress in treating and preventing lung disease there needs to be much research in these ‘other’ problems that Cf affected persons face.

What to do when we are feeling overwhelmed?

Aug 12, 2020 03:43 pm | Tré LaRosa

Replies: 3

It’s of utmost importance that we’re cautious about our mental health as too much stress can actually begin to affect our physical health. Whether it’s a job, being active, or any number of responsibilities, there are times when I feel extremely overwhelmed and need to take a step back.
1. What usually causes you to feel overwhelmed?
2. What are some ways that you go about mitigating that feeling of being overwhelmed?

CF: A whole body disease

Aug 12, 2020 03:19 pm | Jenny Livingston

Replies: 1
I frequently hear CF referred to as a lung disease. While I understand that for those who aren’t informed about CF, calling it this is a quick and easy way to describe it. But also, calling it this can be incredibly misleading. As we know, CF is a whole-body disease!
I have friends within the community who have never been hospitalized due to lung issues, but regularly have GI-related hospitalizations. Personally, my sinuses have been far more problematic in recent years than my lungs have been. CF is such an individualized disease and the ways in which it manifests itself are as unique as each of us living with it.
In what ways does CF affect your body? Do you have other organs that are affected more than your lungs? What symptoms do you have that differ from the “classic” explanation of CF?

Your Disease Process: Which Organ Hurts Most

Aug 12, 2020 02:35 pm | Bailey Vincent

Replies: 3
If you had pick one organ that’s impacted the most by your disease process… Which is it?
And, in doing so, which doctors feel the most important members of your team?
Mine has undoubtedly been my stomach (as I wrote about recently on Instagram with some less-than-flattering photo memories), or- perhaps more specifically- my whole digestive system. I’ve had more intestinal surgeries than I can count, and am down a colon, large intestine, gallbladder, and beyond.
Because of this, I was extremely close with my colo-rectal surgeon and my GI team… until the former retired, and the latter moved to another specialty. I was crushed. And, though I’ve been working to find suitable replacements and carry on, it doesn’t feel “the same” just yet because I truly trusted them so much.
My new CF clinic has been amazing in terms of lung care, medicine maintenance, and amazing holistic mind-body treatment as a whole (I truly feel so lucky to be with them), but any time biliary backfires or major GI malfunctions happen… I’m still supposed to see those specialists.
I have more thoughts on this tomorrow, but for now, my question is: Which organ hits you hardest, and how many specialists do you see?

Should CF Clinics Change Forever?

Aug 12, 2020 02:23 pm | Bailey Vincent

Replies: 2
Yesterday I posted about which organ is impacted most by your disease process, but today I wanted to ask something that I’ve seen discussed a lot on the forums lately…
Should CF clinics include ALL specialists?
For myself personally, I have to attend my GI and colo-rectal doctors frequently, as well as my liver guy, my gastric pacemaker person, my bone specialist, and onward… ALL outside of my clinic visits. Although my sinus surgeon works closely with clinic, he too is a completely different visit.
I’ve been wondering for awhile: Why don’t CF clinics have their own specialists in these specific departments, since CF is so much more than just lungs and bugs?
What is your opinion: Would you prefer this or do you think it should be adapted some other way?

What do you know about Patient/Family Advisory Councils?

Aug 12, 2020 11:15 am | Jenny Livingston

Replies: 0
This post shared by Anna McVey-Tyson on the CF Foundation’s Community Blog in 2016 details how she was able to start a Patient Advisory Council at her CF center. She says “if patients and family members want to make a change, the best way to ensure that change can happen is by speaking up and working in harmony with their CF care team. That’s the starting place.”
The purpose of a Patient and Family Advisory Council is to create an environment where patients, family members, and clinic staff collaborate as partners to improve the patient experience and quality of care. As a member of my center’s foundational advisory board, I can relate to some of the struggles and triumphs Anna writes about.
Does your center have an advisory board/council? Have you ever served on an advisory board? Do you have any tips for someone looking to start a Patient/Family Advisory Council?