January 4, 2021 at 2:11 pm #16060Jenny LivingstonKeymaster
I hope you all had a lovely (and healthy) winter/holiday break!
My family and I had a quiet Christmas and New Year at home. I’ve felt well enough to spend time outside (even in the snow) doing some sledding and hiking in the hills near my home, which has never happened in all my adult years. I generally hunker down indoors for the duration of the winter months. It’s been a delightful and beautiful surprise for me to be enjoying Mother Nature during this season.
As we begin this new year together, I want to ask what YOU hope to discuss as we move forward. Are there particular topics you’ve found interesting and would like to delve into more? Do you enjoy community member highlights? Do you hope to talk about something specific that we haven’t gotten around to? I’d love to hear any and all thoughts you have on this, or suggestions that might improve your experience here.
And on a final note, please know how much we appreciate you being here. Your comments, thoughts, and heartfelt responses have meant so much to me. From my heart, truly, thank you.
Happy New Year!
January 5, 2021 at 2:18 pm #16069John F SchroederParticipant
And HAPPY NEW YEAR! So glad to hear you had a good holiday and were able to get outside and enjoy yourself.
Boy , what to discuss this year! So many things. I am very interested in the CFF’s initiatives to get reliable therapies for people with Rare and Nonsense mutatuons of their CFTR gene. Being a geek, I am enthralled by the science and can see forward, with the success of the mRNA vaccines, what an mRNA therapy could do for CF’ers with the Rare and Nonsene mutations. And, if theese therapies can even coome close to the success of Trikafta, it brings us all much closer to our shared “Until It’s Done” ideal!
Beyond mRNA therapies, I can see the day – which likely is several years in the future – when CRISPR or other gene editing tools can actually cure CF. That dream has been so far away for so long – my kids were diagnosed in 1974, 15 years before the CF gene was isolated! – and hard to belive in. But now I see a better road forward. It will take a lot of work and a lot of luck, but the path is becoming clearer.
On a final note, I strongly recommend the book “Breath from Salt” by Bijal P. Trivedi. The subtitle of the book,”A Deadly Genetic Disease, a New Era in Science, and the Patients and Families Who Changed Medicine Forever” says it all. It’s a wonderful, inspiring, read for everyone in the CF world.
That’s all for now! On to 2021 and, hopefully, a COVID-Free world!
January 5, 2021 at 8:45 pm #16073Tim BlowfieldParticipant
Happy new year Jenny and John.
We had a very busy and wonderful Christmas with our whole family able to get together on the Sunday. By the Monday my wife was exhausted so we delayed a ‘day out’ with our younger daughter till Tuesday. It was great news to hear that the US FDA and Vertex have expanded the list of mutations for Ivokafter etc to include one of Reva’s mutations (R75Q) – so we will now push for it. The expanded list is not yet approved in Australia.
January 6, 2021 at 8:11 am #16075Jenny LivingstonKeymaster
@johnfschroeder I am very interested in CRISPR research! It’s so intriguing and, as you said, there is so much hope on the horizon. I’ve now heard from several people that Breath of Salt is a worthwhile read; I may need to get my hands on it!
@timb The news about Trikafta is truly exciting! I am so happy it’s been expanded to include Reva’s mutation! I hope that the expanded list will be approved soon and she’ll be able to start taking it. My experience with Trikafta has been so positive;, it makes me want everyone to have a chance to try it (if they so choose).
January 6, 2021 at 9:24 am #16076Paul met DebbieParticipant
Happy new year to everyone!
We had a very peaceful and quiet holiday together. The Netherlands are in a pretty firm lockdown at the moment so most of us stayed at home.
New subjects for the forum will pop up spontaneously, but for now I can mention two:
1. the covid vaccination. What are the specifics for the cf community to focus on? I can think of several questions to ask our doctors:
a. is there a preferred type of vaccine for cf patients, meaning: if we have a choice, should we opt for an mRNA vaccine, a vector-vaccine, a spike-vaccine? If there is a preference to be mentioned, are we going to have a choice?
b. same question for patients that had a transplantation
c. are there any precautions we could or should take prior to getting the vaccine, for instance should we take (extra) antibiotics to prevent an exacerbation caused by the vaccine
d. should we stop taking certain medication prior to vaccination, for instance oral prednisone or other medication that inhibits our natural immune system (medication that ends on -mab)
e. are there any special concerns for patients with a bad lung function?
g. is vaccination possible or advisable during an exacerbation? If not, what to do if the second (follow-up) shot is due during an exacerbation? How long could the second shot be postponed until one would also need to have a new first shot again?
Has your cf team contacted you with guidelines regarding any of these questions? Have you asked them any of these question? Does your national cf organisation take inventory of questions like these, and if so, what are the aswers given so far? Do you feel you are sufficiently guided, informed and coached by your team or the official organisations?
2. There seems to be a policy to prescribe extra salt intake for cf patients. However, in different countries/continents the guidelines (if any) differ enormously. And scientific research seems to be absent. Questions
a. What is our own experience in this? Do we think we need extra salt? Do we take extra salt?
b. If so, who prescribed this and when (age) and why?
c. How much do we take?
d. What do we take to get extra salt (table salt, special salt, special drinks, special tablets, salty food/snacks)
e. Do we take standard doses regularly or only if needed when feeling bad?
f. Do we take only in special circumstances like when we are sweating more than normal?
g. Have we ever experienced dehydration caused by low-salt levels?
h. Is our extra salt intake monitored regularly in the yearly tests, or was it ever checked in the lab if we needed it at all and how much?
i. Do we experience kidney problems, high blood pressure or other side effects due to high salt intake?
January 6, 2021 at 3:55 pm #16084Tim BlowfieldParticipant
Thanks Paul. Well said.
I guess most of us will be just given the choice of whether to have the one offered or not. You raise very relevent questions.
Re Salt: Yes there are few if any well controlled studies but lots of experience and opinions. There is so much variation in the needs of one CF person to another. My wife’s Cardiologist (says ‘reduce salt and water’) has been at loggerheads with her CF Physician (‘Take more’). I think we have a happy mean now! Reducing salt and water intake has been long recommended by cardiologists to reduce hypertension, leg swelling and fluid overload in persons with Congestive Heart Failure. But reducing salt has been shown to be of no avail in most people who do not have CHF & hypertension, can also be harmful if it leads to hyponatraemia (may cause disorientation, weakness, light headedness even fits. And too rapid treatment of hyponatraemia can cause swelling of the brain with serious symptoms. So taking little and often as needed and constantly is appropriate. Most CF’ers do feel when they need it (craving) and most CF Physicians advise such.
January 7, 2021 at 12:41 pm #16085Paul met DebbieParticipant
Adding to my previous post:
In the Netherlands, the national cf patients organisation is taking inventory of all questions cf patients might have regarding the vaccination. I expect them to report about the results and answers soon.
I asked my cf team. Only answers they could give right now are:
1. they advise not to take the vaccine during an exacerbation;
2. patients that had a transplantation should get an mRNA vaccine (like Pfizer or Moderna). Not a virus-vector vaccine (like the Oxford vaccine).
3. other patients could go for any of the presently available vaccines.
4. oral prednisone only could hinder the immune reaction of the vaccine when taken in high dosis.
Other questions/anwers are still pending.
I will report back when anything new comes up.
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