Country Concert for Cystic Fibrosis

CF adults w/ chronic joint & mucle pain

abnormal — Sat, 04 Feb 2012 17:11:01 +0000

Originally posted in Cystic Fibrosis - Adults

Now there are several threads out there that touch on this issue but I felt it would be best to to start new one posing a few simple questions for those to answer.

1. Have you had your joint & muscle pain diagnosed? If so, how?

2. How do you treat your pain?

3. Is there anything that makes your pain worse?

4. How long has this been a problem for you?

5. Do you feel your pain could be related to CF and or a treatment regime in some way?

I ask these questions to see if there is anything in common we as adult CF patients share that point out a correlation to CF and chronic joint and muscle pain. With the lack of research and knowledge on this issue in the CF medical community, I feel it is up to those dealing with this to come together and speak up.

If anyone can relate to this or has something else to share on the issue, PLEASE respond!

Thanks,

Abnormal

36w/CF

FDA drugs

dyza — Sat, 04 Feb 2012 17:11:00 +0000

Originally posted in Cystic Fibrosis - Adults

Has anyone any insight into how long it takes FDA approved drugs to reach the shores of the UK, or other countries for that matter?

Obvoiusly I have the new drug in mind, and subsequently the next one ( 890?), Oh and on that note, fantastic news on the vX770,

its close, its coming

One would assume that the UK would do their own tests on outside drugs before approval, I'm sure other drugs, tobi, whatever, went troo the same scenario.

Only problem I can see in the UK is the cost, only today the NHS (national health service), knocked back the use of a drug for use by men suffering from prostate cancer, their reason being that it only gave an average 4 months extra life, and that it costs £3000 per month.

craig

Doing cayston on the go

mamaScarlett — Sat, 04 Feb 2012 15:59:23 +0000

Originally posted in Cystic Fibrosis - Adults

Currently doing month on/month off Cayston. Figuring out how to handle it when I'm out is tough. There's alot of conflicting info on how to sanitize properly, how long is ok to wait before cleaning the Altera, etc.

The CFF's Altera cleaning video and Cf pharms instructions state washing with clear dish soap and tap water is fine, then sterilizing. That's what I do. When out though, sometimes I can't clean the Altera for hours. Should I be wiping down with alcohol then washing and sterilizing when I get home?

I'm going on a small vaca to Disney and bringing 3 headsets with me. I can wash the AM and PM dose headset right away. But the midday dose headset of course I can't sterilize till I get back to the room in the evening.

Not sure what to do.

Cystic Fibrosis Research Opportunity

Ennio — Sat, 04 Feb 2012 13:16:34 +0000

Originally posted in Cystic Fibrosis - Adults

Cystic Fibrosis Parents or caregiver with children between 6 – 17 and patients between 14+ interesting in taking an online survey, please contact me at ennio@cysticfibrosis.com or PM me.

If you qualify for the study you will be compensated $30.00 for your time.

Any questions, comments or concern fell free to email me ennio@cysticfibrosis.com

Parents of CF child

Beccamom — Sat, 04 Feb 2012 07:44:37 +0000

Originally posted in Cystic Fibrosis - Adults

I am wondering after a child is diagnosed with CF did any of the parents then find out that they too had CF. I think I have read at least one story on the forum like this. I have one child with G542X and one child with a 5T variant. After reading the carriers with symptoms posting recently I thought that could definately explain my symptoms. Then it hit me that a Cf mutation and the 5T variant together causes mild CF and if I passed both of these to my children rather than my husband having one and me having one of these to pass on then that could explain my symptoms too.

So if you were tested who requested the test? Primary care doctor? ENT? Pulmonologist?

Who recommended the testing if you as the parent were tested?

Thanks for any advice. I am trying to get myself healthier so I have less illnesses and thus germs to pass on to my children.

Parents of CF child

Beccamom — Sat, 04 Feb 2012 06:14:07 +0000

Originally posted in Cystic Fibrosis - Adults

So if you were tested who requested the test? Primary care doctor? ENT? Pulmonologist?

Who recommended the testing if you as the parent were tested?

Thanks for any advice. I am trying to get myself healthier so I have less illnesses and thus germs to pass on to my children.

Vx-770 and hemoptysis

jmiller — Sat, 04 Feb 2012 04:59:29 +0000

Originally posted in Cystic Fibrosis - Adults

Something I've always wondered is if drugs like vx-770 that address the underlying cause of cf will decrease hemoptysis episodes… Anyone on here have frequent hemoptysis and participate in the vx770 trials? Did you see a decrease in bleeds?

Late Diagnosis of atypical or "variant" CF questions-late onset PI?

Ringer — Sat, 04 Feb 2012 03:21:43 +0000

Originally posted in Cystic Fibrosis - Adults

Hello,

I am looking for help. I just received my results from a standard mutation screen (50 mutations?) and they were negative. I would think this would be good, but I'm still without answers. I am feeling a bit lost

History:

Asthma diagnosed in infancy. No family history of asthma but cousin on father's side has "mild" CF (Sorry, I know the term is controversial, but this is what she calls it). I am not able to find out her mutation. Never responded to traditonal asthma medications, mother was told to perform CPT. As an adult I now use Ventolin, HyperSal7% , exercise, and CPT for airway clearance (but hopefully will be getting a flutter soon).

Pneumonia, lung infections, mycobacterium, etc. throughout life (not working and not being in school reduced the incidence). I am currently not working (which I absolutely hate) but my health is always better when I don't.

I consider my lung disease (whatever it is) to be "mild" as my lungs are still overall in good shape. Can't say how or why!

Chronic sinus infections. This current one has been a real doozy complete with my eyes swelling and a fever. Mucus is always thick, yada yada…

Diagnosed with "silent" chronic pancreatitis (indication was panceatic insufficiency and malnutrition/low fat soluable vitamin deficiency) in 20s, around the same time my asthma became more "symptomatic" (I now have a productive cough at least once or twice most days-there'd better be a sink nearby when I'm laughing . I was hospitalized for IV nutrition. I am now completely pancreatic insufficient at almost 30. I have been on a high calorie diet for as long as I can remember. I look somewhat thin (especially if I slack on my diet) but still "average" weight. My problem is just keeping weight on, not so much having to gain weight.

My exhaled nitric oxide test was slightly below normal, which I was told makes asthma unlikely. I was told to book a sweat test through my GP (my mother thinks I had one as a child, but it was borderline). I am calling my doctor Monday to book it.

My sinus and lung symptoms are "mild" (but seem to be getting more symptomatic), but my pancreas is pretty much kaput since my mid 20. I need Creon to eat everything but fruit and some candies.

I tend to have low electrolyte levels, so I take extra salt and potassium.

For any of those with an adult late diagnosis (particularily with complete PI), could you please tell me your mutations? How were you diagnosed? What were your sweat test values? Did you always have mild or atypical symptoms? Or did your symptoms suddenly appear later in life?

Right now I think the genetics clinic doesn't know what to do with me and securing a diagnosis (if it IS in fact variant CF) would really help because then my vitamins, flutter, HyperSal, etc. would be paid for (right now all I get covered is Creon. If someone can help me that would be great. Sorry for the long first post! This is my first time ever posting on a CF forum, and I'm feeling really lost and confused.

New drug–Kalydeco–Approved

SIcklyhatED — Sat, 04 Feb 2012 03:21:43 +0000

Originally posted in Cystic Fibrosis - Adults

My lovely pharmacist sister, who's always researching and keeping us up to date on CF med stuff, just sent me this email about the new drug Kalydeco, which targets the G551D CF mutation. It was just approved by the FDA a few hours ago I believe, and this could lead to the approval/release of a drug to target F508

Here's a link to the article:

http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm289633.htm

They're making progress!

Sleep Issues

icefisherman — Sat, 04 Feb 2012 03:21:43 +0000

Originally posted in Cystic Fibrosis - Adults

I've been thinking about some of my sleep issues lately and was wondering if anyone else had any input or wanted to share thier experiences with sleep.

First off I noticed a few years ago that i felt better lung wise when i didn't sleep much and since the wife left i can sleep what ever hours without distractions. Also i work nights and my work schedule changes a lot, but has been stable lately, so the last few months has been the first time in a long time i have had a sleep schedule.

I get home around 8 a.m. and usually get some food in me play some guitar then its a cup of sleepytime tea and i go to bed. I am always up before noon and hardly ever stay asleep for more than 2 hours. Then i get up, play some more guitar (wife took the tv so my guitar is my entertainment now lol), and get my house work done. Then i lay down a few hours before i have to leave. i either leave at 6pm or 10pm. But i don't use the tea because i don't want to be tired when i get up. So all in all i average about 4 hours a day. some days just the first 2 hours other days i may get 6 hours all together in two seperate naps. This keeps my lungs feeling good, and when i do get to sleep for 5-6 hours strait my lungs are full of crap and it effects the rest of my day.

Ok, so there is that which works for me but leads to my other issue that i really haven't talked to anyone about. I have very, very, vivid dreams. I have had for probably 8-10 years but its everynight now. I learned early to be carefull what i watch on tv and other things because I have had a few very graphic dreams that really upset me for days. That isn't so much an issue now but It has gotten to the point where I get confused about what was a dream and what was real. Yep i'm nuts lol. Fortunatly my dreams are just everyday life and work now and really don't have many nightmares, but i'll dream about buying something or putting something somewhere and a few days later go look for it before i realize it was in a dream that i did it. its never anything big just like buying egss, and feeling like making an omlet thinking you bought eggs and then you open the fridge, no eggs. On the other hand i remember where i put things that i lose during the day in my dreams, and figure out guitar riffs in my sleep that i work all day trying to figure out. So yeah thats not normal, strait jacket here i come lol. So any input? I was wondering if oxygen levels change much in your sleep and if that could be part of it.

Ben

New drug–Kalydeco–Approved

SIcklyhatED — Sat, 04 Feb 2012 01:29:51 +0000

Originally posted in Cystic Fibrosis - Adults

Here's a link to the article:

http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm289633.htm

They're making progress!

Sleep Issues

icefisherman — Sat, 04 Feb 2012 01:29:51 +0000

Originally posted in Cystic Fibrosis - Adults

I've been thinking about some of my sleep issues lately and was wondering if anyone else had any input or wanted to share thier experiences with sleep.

Ben

CFRD and hypoglycemia

Sralidar — Fri, 03 Feb 2012 22:05:50 +0000

Originally posted in Cystic Fibrosis - Adults

Hey everyone, So I'm 23 with CF and have CF related diabetes. I was diagnosed with CFRD at 18 and get pretty crazy spikes and drops in bloodsugar every day. I wanted to put a question out there in case anyone has this problem and might be able to help. I've been getting worsening episodes of hypoglycemia every day and am practically attached to glucose tablets, I'm on hardly any insulin 2.5 u novolog in the morning for breakfast and that's it. I crash several times throughout the day and have to take loads of sugar just to come back up to normal, on the other side I hit 200 pretty consistently as well but never stay there very long. I'm concerned any more insulin would send me into a hypoglycemic coma, but even trying to eat a variety of foods crash all the time. I keep being told that the spiking and crashing is just part of the CF diabetes, but it's gotten to the point where I can't go 2 hours without sugar and food of some sort. Anyone have this problem who might have figured a way around it? Mostly the lows over the highs. (side note, from 18-21 I was on low dose of Lantus, which I was taken off of because I was starting to crash low all the time).

~Brandon

AquaDEK

2DIE4Corey — Fri, 03 Feb 2012 18:38:26 +0000

Originally posted in Cystic Fibrosis - Adults

..I am just wondering, how many ppl use it here. I got it the first time in my life last week.

Sounds like a good vitamin-medication. I used to take ELEVIT before.

greez, D.

Cystic Fibrosis/Great Strides 2012 Video!

CystikOne — Fri, 03 Feb 2012 18:38:26 +0000

Originally posted in Cystic Fibrosis - Adults

So, this is a little piece I put together the other day for my Great Strides 2012 campaign. If you could watch it, pass it on and possibly donate, that would be AWESOME.

http://youtu.be/4K-OGMj2DRs

G551d kalydeco/vx770

robert321 — Fri, 03 Feb 2012 18:38:25 +0000

Originally posted in Cystic Fibrosis - Adults

With all the frenzy of the 770 getting passed through the FDA rumors are rampant. I have read in blogs and forums everything from people claiming to feel "normal" and come off of other treatments (it is to my understanding that this is strongly discouraged) to a NY Times article that says patients "have been able to shovel snow for the first time" to people claiming that it is more of a research and development victory than it is a miracle pill and is just "another pill".
I'm not sure if the study subjects are allowed to talk about the results yet or not and that may be why I'm finding mostly second hand information and rumors. So my questions are;

Can the study subjects discuss the results without risking tainting results?

What are the benefits patients are actually seeing? Any GI improvements? Quality of life? Weight gain? etc. Is it the magical pill the press releases are talking it up to be?

Cystic Fibrosis/Great Strides 2012 Video!

CystikOne — Fri, 03 Feb 2012 17:05:50 +0000

Originally posted in Cystic Fibrosis - Adults

So, this is a little piece I put together the other day for my Great Strides 2012 campaign. If you could watch it, pass it on and possibly donate, that would be AWESOME.

http://youtu.be/4K-OGMj2DRs

AquaDEK

2DIE4Corey — Fri, 03 Feb 2012 17:05:49 +0000

Originally posted in Cystic Fibrosis - Adults

..I am just wondering, how many ppl use it here. I got it the first time in my life last week.

Sounds like a good vitamin-medication. I used to take ELEVIT before.

greez, D.

Drug trials…NOT at your clinic

MCGrad2006 — Fri, 03 Feb 2012 17:05:49 +0000

Originally posted in Cystic Fibrosis - Adults

So Since 770 got approved I have been researching a bit. I had read on here that they are doing a trial for those patients with one copy of deltaF508…which I have…and N1303K as my other one. On CFF.org it lists the sites that are doing the 770/809, but my clinic is not listed. Childrens Boston is listed, but not MGH. Has anyone ever done a trial at another clinic? Did your doctor allow that? How did it work with your records? Did you have be evaluated at the new clinic? I am VERY interested in more information. Please let me know if you know anything. I should mention that…it did say that not all clinics doing the trial are listed, so MGH may be doing it. I have a clinic visit next week and will certainly be bringing this up, but would like to be armed with info! And if anyone specifically knows about Childrens version of the trial feel free to chime in or PM me.

Thanks!

UPDATE: GOT IN TOUCH WITH CHILDRENS. THEY UNFORTUNATELY FOR ME ARE NOT TAKING ANYMORE NAMES. THEY HAVE 60 PEOPLE ON THE WAITLIST, SO YES IT IS GOOD NEWS FOR CF RESEARCH…AT LEAST THEY HAVE PEOPLE WHO WANT TO DO THIS FOR THE REST OF US! EITHER WAY IT WILL BE HELPING. I AM SLIGHTLY DISAPPOINTED, BUT GRATEFUL FOR MY FELLOW CFers THAT VOLUNTEERED THE TIME AND EFFORT. THANK YOU TO THOSE INDIVIDUALS. <3

Drug trials…NOT at your clinic

MCGrad2006 — Fri, 03 Feb 2012 15:38:43 +0000

Originally posted in Cystic Fibrosis - Adults

Thanks!

What song do you relate your CF fight to

live2breath — Fri, 03 Feb 2012 14:19:05 +0000

Originally posted in Cystic Fibrosis - Adults

Hey all I have always wonderd of there are other people out there who relate a song to there battle with CF for me its Down with the sickness by Disturbed and one else Do this

Training for a 5k!

alavoie2 — Fri, 03 Feb 2012 13:09:10 +0000

Originally posted in Cystic Fibrosis - Adults

Hello, I am not really someone who does any kind of exercising. This year for our cf walk they are also doing a 5k run. I have been training to try to do it, the walk is in May & I just started last week, but reallllly started this week. I want to prove to everyone, including myself, that I can do it! Anyone who has any ideas on a good way to do this feel free to let me know! I WILL do this & I will do great!

Andrea 26 yrs old married for 4 years!

Microcyn

petersymons — Fri, 03 Feb 2012 03:28:30 +0000

Originally posted in Cystic Fibrosis - Adults

Just wondering if anyone here had inhaled microcyn to try and kill bactreia in the lungs?

regards peter

Reactions to Vancomycin?

TreasureGoddess — Fri, 03 Feb 2012 03:28:30 +0000

Originally posted in Cystic Fibrosis - Adults

My son just got home from a 10 day stay in the hospital for iv vancomycin. He had the usual mrsa/staff infections causing pft's to decline, felt like crap, hurt all over, low energy, no appetite, the usual. Every couple of years my son would go in, have 10-14 days of vancomycin and be happy, healthy and bouncing off the walls. Last vanc. was 2 yrs ago. Never any issues.

This time Joe developed "red man syndrome" where he threw up, turned red in splotches and was extremely itchy. I was told it was very common, they treated with benadryl along with slowing the rate of the iv down to a 3 hour drip plus flushes to being hooked up for 4 hours on and 2 hours off meds along with increasing benadryl to the max.

My son got severe stomach pains and headaches after 7 days, had to pull the meds because he was just moaning in pain the entire time he was hooked up. I asked for meds to stop and could we do anything else? DR said red man syndrome was common, but the level of stomach & head pain my kiddo got was rare. I asked what to do next time as this go-round didn't restore his health very well. He said the team would have to "think about it." Told there are other iv antibiotics for treating mrsa & cf lung infections, but they're not used much and they've all got their own side effects to deal with.

We're going in for a 2nd opinon next month and iv meds will be a big question for me. Has anyone else had bad reactions to vancomycin? What did you do instead? Are there other common iv meds that are used for lung infections for those with CF?

I'm afraid we've crossed the bridge from a kid with general good health and has cf issues once in a while to dealing with pain, feeling crappy, coughing and poor health daily. He's 12 yrs old, in his first year of middle school and he's hardly been to school. I was hoping for the vanc iv stay at the hospital to do its usual magic and he'd be uber healthy for a few years more, but it looks like I need to create a new version of our baseline/norm health levels. He's not much better than when he went in to the hospital 2 weeks ago. cf sucks.

medic alert

rubyroselee — Fri, 03 Feb 2012 03:28:29 +0000

Originally posted in Cystic Fibrosis - Adults

Hi all,

Just wondering…do you wear a medic alert tag? I have been considering getting one, but I'm just not sure. I feel that having CF isn't enough reason to wear one for me because I wouldn't suspect any emergency situation to arise because of my CF at this point. However, I got to thinking about it after I had hemoptysis because I was worried about it happening when I was by myself – and what if I was by myself and had a large bleed. I also am a little worried too because of my frequent low blood sugars. I have not been diagnosed with CFRD yet, but I get lows quite often.

So what are your thoughts on having a medic alert tag?

Anyone have one? If so, what does yours say exactly?

What made you decide to get it?

Have there been instances when it was helpful and/or saved your life?

Thanks!

Please help www.dafcf.org keep its doors open in 2012

julie — Fri, 03 Feb 2012 03:28:29 +0000

Originally posted in Cystic Fibrosis - Adults

As some of you might know, DAFCF is struggling to keep their doors open for 2012.

DAFCF is a nonprofit 501(c)(3) that provides a number of legal advocacy services for persons/families with CF, who need assistance in navigating a number of services including insurance, state medical, food stamps, GHPP, social security disability benefits and other related issues.

Last month DAFCF was nominated by friends and family and made it into the finalist list for December voting.

All you have to do is click on the link, select the name Julie Raysbrook-Delgado, and hit submit. There is NO requirement to registser or give out your email address. This process takes LESS than a minute

http://www.komonews.com/about/events/137892803.html

I would also be so grateful if you would pass this information for your friends and family to vote!

Thank you!!!!!
(you can see the write up about DAFCF here http://www.komonews.com/younews/136550428.html)

Just for fun but CF related – Real things doctors have said to me in the hospital

CFkitty — Fri, 03 Feb 2012 03:28:29 +0000

Originally posted in Cystic Fibrosis - Adults

There is a cute site where you can make videos with animated characters. I am not advertising the site, but wanted to share the video I made.

http://www.xtranormal.com/watch/12953949/are-you-sure-that-you-have-cystic-fibrosis

Implications of bodybuilding and Pseudomonas!

Incomudrox — Fri, 03 Feb 2012 03:28:28 +0000

Originally posted in Cystic Fibrosis - Adults

So as always I've been digging deep through the NIH and putting links together elsewhere. Generally speaking those with CF who bodybuild fairly well and some even come back from situations with the odds fairly against them. None the less I started to ask WHY is this?

I believe I may have found ONE of the reasons why, be it there are most likely others that I will uncover over time. Shall we begin? Let's talk for a minute about our friend PA. Scientists have discovered that mucoid PA, has one weakness – an akilees heel if you will. 87% of mucoid PA has a defect in its genetic code, which it either does not produce enough enzymes to get rid of acidic nitrite or doesn't have any of these enzymes at all. Ironically this defect in it's code is in the same gene that allows non-mucoid PA to BECOME mucoid PA. They have shown the Acidic Nitrite can totally wipe out mucoid PA because it has no ability to get rid of the acid once inside of the PA that lives with in our mucus.

Never the less you maybe wondering what this has to do with CF and why this even matters. Well people with CF have low levels of NO or eNO (exhaled Nitric Oxide) in thier bodies. Nitric Oxide is a derivitive of Acidic Nitrite. So naturally this doesn't happen so well for us. However a group of wonderful scientists discovered that if CF'ers take a dose of L-Arginine orally we can resustain levels of eNO and NO in our bodies. L-Ariginine is an essential Amino Acid as well as one of the building blocks of NO in the human body.

Researchs have done further studies to show that inhaled L-Arginine actually showed an increase in FEV1 in patients that participated in the study vs the plecebo.

So what does this have to do with body building? If you don't dabble in bodybuilding let me explain. Most bodybuilder take pre-workout boosters as well as BCAA's (sometimes these two are combined even).

Most of the commercially avalbile pre-workout suppliments suppliment NO (this helps the work out and blood flow for bodybuilders) as well as oral L-Arginine. In the studies L-Arginine was given to the patients at a dose of 200mg per kg of body weight. Which happens to be right on par (for my weight) with the amount of L-Arginine that is in most body building suppliments.

So in conclusion this would be one of the things that gives bodybuilders with CF an edge to fighting their mucoid PA and generally being in better health.

Further studies show that when CF'ers were given creatine (not to be confused with creatinin) they generally had a better quality of life and gained weight easier as well as having more energy. Most if not all pre-work out drinks and massbuilding protein shakes in the body building world have one form or another of creatine. However I'm not going to go further into this unless someone wants me to.

I am not saying that if everyone goes out and buys bodybuilding suppliments or L-Arginine (which you can buy seperately at CVS/Walgreens etc) you are going to get rid of PA or have a marcaulous recovery or any of that. What I am saying is I know a lot of CFers (mainly the men) have always wanted to get into the gym and start pushing those weights. I know I have for a long time and was embarrassed of my weight and what I was able to lift so I just didn't go. So maybe this can bring some motivation to them for other reasons to get in the gym and hit it.

I would recommend anyone who is going to take pre-work out suppliments to check with the doctors most doctors who are not morons will be okay with it. Also please conduct your own research as well.

CF and…

albino15 — Fri, 03 Feb 2012 03:28:28 +0000

Originally posted in Cystic Fibrosis - Adults

You may have guessed by my username that I have albinism in addition to CF. Anyone else have a condition that's unrelated to your CF?

tiny arms

falloutboygurl16 — Fri, 03 Feb 2012 03:28:28 +0000

Originally posted in Cystic Fibrosis - Adults

hey guys…soo i know im not the only on out there with this SUPER thin arms and a bigger chest and body…well i also have pretty tiny legs too. haha! so my question is how do i get my body more proportionate…i know this is because of malnourishment because of enyzym pancreatic problems etc..but what can i do to make my arms and legs bigger..just exercise? maybe mess around with enzymes? im not too certain what to do honestly besides build muscle. i have absolutely NO fat either of those places either…help anybody?