Hypertonic Saline

Read The Study

A study on how using 5% Hypertonic Saline can reduce hospital visits and acute wheezing in children

Birthday Post

Another year alive. And like my family always say every 20th of May - "We're winning".
Having a long term illness is funny. It doesn't really dawn on me that I could die from CF, or that I should have died at 10 if it wasn't for my parents. It sounds weird, but it's easy to forget. So on every birthday, it's remind you that your still beating CF, and will continue to do so. In case it's hasn't registered with you....it's my birthday today, and for some reason, every other CF'er out there always asks "and what age are you", so to put everyone's mind at ease, I'm 26. This question probably stems from the opening sentence of the paragraph. I'm on the other side of a 1/4 of a century now!!!
I got a new Neb the other day. I always get disappointed with new nebs. Apart from the Eflow Neb. I always think its going to be better looking or do something fancier, but they never do. They still always make a "HMMMMMMMM" noise, and still vibrate. Apart from the Eflow. But I needed a new one, and it arrived in about 3 days!!!!
I'm very excited about the new drug coming out which treats Delta508, which is the gene I have. I'd say it will be at least another year to be brought out on the market. At least.
Iv read that it costs about €280,000 a year for one person to be on it. That seems like a lot, and you will probably think that they won't pay it. So here's what I came up with. I'm not on that much medication, and the last time I calculated it up, my medication was over €100,000 a year. So if I'm not on that much meds, then a person with CF who is sicker then me will probably be on more then €280k a year. This new medication is two tablets a day. It targets the cause of CF and not the symptoms, meaning the symptoms will get better, lung function will go up, and potentially fewer hospital emissions, resulting in the health service paying less per person with CF then they would if they were not on the new drug. So in the long run, €280,000 is probably cheaper then the current average CF patient.

I ran a race yesterday. I was 2nd, and made a valuable mistake. I'm still not confident enough to realise I could run at a certain pace, and that will come with more races. It was a 10k race. I could probably count this race as my first proper race in 18 months. I ran one on Paddys Day, but it wasn't really a race. I wasn't fit and I was just arsing around really. 

The course was very hilly. A two lap race. From 2k until 4.5k was uphill, and not an easy uphill  (Is there such thing as going uphill easy?). On the second lap, the hill was from 6k until 9.5km. The leader went out at some mad pace, and I didn't go with him. I should have. My 5k split was faster then my Paddys day 5k race, and felt easy. I had to throw in a 4:43 mile to catch him. I didn't expect to do a time like that during it. It was a good race and I will take coming second as its a comeback. 

We also won the county team. Im from Meath but run for Dublin. It breaks my heart every time!! So a gold amd a silver medal!!!



Onwards and upwards from here. Not sure when the next race is so more raining to be done!!!!

I'm off for a long Sunday run, and my legs are bloody killing me. I wouldn't have it any other way on my birthday!!!!

Adios

Exercise Is Medicine In CF

Exercise is Medicine in CF

Like iv always said.

Viagra For CF

Viagra Usefull For CF?

As I was looking for more stuff on the VX 809, I found this study. Viagra corrects DeltaF508-CFTR location in nasal epithelial cells from patients with cystic fibrosis.
I also found a site where to buy the VX809. Obviously this is ridiculous and a fraud site, so don't even think of buying it. Unless you want very expensive rat poison!!!

New Drug For Delta F508

A piece from CCF.org site

Phase 2 Study of Kalydeco and VX-809 in Combination Shows Promising Interim Results
May 7, 2012

Vertex Pharmaceuticals Inc. today announced promising interim results from a Phase 2 clinical trial of its cystic fibrosis drug Kalydeco™ and VX-809, a CF drug in development.
The results showed a significant improvement in lung function in people with two copies of the most common CF mutation who received the two drugs in combination.

Both Kalydeco and VX-809 are designed to treat the underlying cause of CF. Complete results from the Phase 2 trial are expected this summer.

The ongoing Phase 2 study enrolled 108 people, ages 18 and older, who have one or two copies of the Delta F508 mutation. Today’s results are based on data from about half of the study participants after they had completed 56 days of treatment.

Vertex plans to begin a pivotal trial of Kalydeco and VX-809 in people with two copies of the Delta F508 mutation, pending final study results. Pivotal trials are typically designed to gather data that could be used by the U.S. Food and Drug Administration (FDA) to decide whether or not to approve a potential drug.
"We are eagerly awaiting the full results, and are pleased that Vertex is accelerating its plans for a pivotal study of the combination treatment in those with two copies of Delta F508," said Robert J. Beall, Ph.D., president and CEO of the CF Foundation.

The CF Foundation played a key role in the development of Kalydeco and VX-809, providing significant scientific, clinical and financial support.

About 50 percent of people with CF in the United States have two copies of the Delta F508 mutation. About 40 percent of people with CF in the United States have one copy.

Earlier this year, the FDA approved Kalydeco for people with the G551D mutation ages 6 and older. Kalydeco is the first drug that treats the underlying cause of CF — a defective gene and its protein product, known as CFTR.

Feeling very excited about this. Although it is still in stage 2, it's still very positive, and something to look forward to.
I'm not sure what to do at the moment, as I don't want to get too excited, but I can't help but feel over the moon.

Wedding Bells

No not mine!!! My brothers.

My brother got married yesterday. Typical Irish weather, it stared to lash. However, the weather held off for the pictures, amd what not.
The church service was funny. First of all, my phone went off. I thought I had it on silent. I'm always in demand!! Turns out it was a client who needed treatment done on their leg. There was a song being played, and my brother whispered "all I can picture is horses on a carousel", and it was a perfect image to go with the song. So the giggle set in. It came to the priest saying "Lamb Of God.....". Low and behold, there was a farmer sitting beside me. My sister boyfriend, James. Curiosity got the better of me, so I whispered "is the Lamb Of God on your farm", which set him off. Fun was had at the service!!! 
I'm not leaving out my cock up with the prayer. I was the last to talk, gave my prayer, and stood there like a fool, thinking, why is everyone just looking at me, and the priest not walking back up to the alter. At the end of the prayer, I was supposed to say "lord hear us". I left it out until a person shouted it up. Bloody heck!!!

I found it weird that my brother is now married, and we have another person in the family. I still do find it weird. We are all growing up so fast. Huh!!! I suppose it's my turn next, going on what everyone keeps asking/expecting. Huh!!!
The hotel was absolutely amazing. Dunboyne Castle was the venue. Go check it out. We got all the photos taken, and a bit of snacks etc. I don't think iv ever drank as much tea as I did that day. It didn't help, because i was in bed for 1 o clock. Knackered.

I always love spending the night in a hotel. One of the things I always do is check the showers out and give them a test. Either that or the bath. When I woke up, Yaz had already left as she had college, so I made myself a cup of tea, sat in bed and watched crap, then had a lovely shower. By the way, it was a lovely shower!!!
Darren and Rocio seemed to have enjoyed it, and are now in Croatia on their honeymoon.

Running is going good, I suppose. It takes so long to get back to where I want to be, or where I was running at an ok standard. Doing speed sessions, and getting into the swing of things isn't easy, especially when I'm training with middle distance athletes, and I'm a long distance athlete. It's fun though, which is the main thing. When it becomes boring, it means it's work, and that won't get you anywhere.
Iv stopped taking my Ventolin Neb before a hard run, and just take a few puffs of the inhaler. I find no difference and I don't get those horrible shakes from the nebs. The Singulair is also working nicely. I can't complain about that, amd it has eased my chest down a huge amount. Aww Yeah!!!

Enjoy the photos

Place Names

Mammy, the Bride (Rocio), The Brides Mammy and Daddy

Myself and Yaz

Myself and Yaz again. Aww

myself, James (Caoifes boyfriend), and my brother Ian

Myself and my sister Caoife

The Boys. Me, James, Darren (the groom)and Ian

Singulair

So my 4th day on Singulair. Might just be my mind, but I'm not as chesty, or inflamed after a run, and throughout the day. It feels like i can take deeper breaths.
I was told that it would take a long time to take effect but im feeling effects so far. Maybe a placebo, and of it is. I don't care because it's still working.
I just did a 20 minute tempo run which means going at around 70-80% effort. Normally I take a Ventolin nebuliser before any hard run. Iv stopped that and I'm just taking 2 puffs of the inhaler instead. I don't miss those bloody Ventolin nebuliser shakes after it.

Remember the race I ran on Paddys Day. Well here is the closest picture I can find of me!!! If you look at the centre of the picture and see the green Nike runners with the black swoosh?? That's me. Hahahaha.

Patient Voices: Cystic Fibrosis

I rob and steel from other people who post link up on CF. So thanks O.T

NY Times Patient Voices:Cystic Fibrosis

Exercised Induced Pulmonary Hemorrhage

So here is something I found when looking at some Acupuncture treatments on Lung Bleeds. Well, I did find how I can tat lung bleed with acupuncture. However, here is something I came across and I found it interesting. The study was done on race horses. It's very common on race horses, and no, they don't get put down!!!

"A wide variety of treatments have been used or suggested for treatment of EIPH, including resting, anti-inflammatories (e.g. corticosteroids), bronchodilators, anti-hypertensive agents (including nitric oxide donors and phosphodiesterase inhibitors), conjugated estrogens (e.g. Premarin), antifibrinolytics (e.g. aminocaproic acid and tranexamic acid), snake venom, aspirin, vitamin K, bioflavinoids, diuretics (e.g. furosemide, known as Lasix or Salix), nasal strips (e.g. FLAIR™ Nasal Strips), concentrated equine serum (CES) and omega-3 fatty acids. Both the FLAIR Strips and the diuretic furosemide have been shown to have similar efficacy in reducing EIPH under laboratory and field conditions. A single study has shown that concentrated equine serum also reduced EIPH. There is currently no evidence to suggest that rest, bronchodilators, anti-inflammatory drugs, bioflavinoids, oestrogens, antifibrinolytics, aspirin, phosphodieterase inhibitors or surgical correction of upper airway obstructions reduce EIPH. In contrast, nitric oxide and aminocaproic acid have been shown to worsen EIPH.
Furosemide (Lasix) has been used to prevent the condition, although it does not work in 50% of cases,[1] and it is banned in some countries. Other treatments that have been used include bronchodilators, coagulants, and oestrogen"


I find it interesting that they used SNAKE VENOM to treat it!!!!!!!

CF Check-up

As promised. Here is how my tri-monthly check up went.

Lost 4kg (which I'm happy about)
Iron (16.4)and bloods is at a steady state.
Sputum came back with only traces of pseudomonas and staph in it. Nothing has really changed there.
My FEV1 went up by 1% (82%) but that's without Ventolin. Im normally 81% with Ventolin. So that's brilliant. My other test, where you take a deep breath in and they pump some crap (hydrogen is what they told me)and then you blow out...what ever that is, it went up by 6%.
The bleeding issue. Well that's still unresolved. Iv to get my Vit K levels checked. It's not that, as I explained yesterday, I would bleed more if I cut myself if I had a deficiency in it. Time will tell.
I am now on Singulair. He is treating more like an Asthmatic rather then a CF, which is good. The level of activity I'm at means im not productive. I find it hard to get anything up (CF impedency). What I do find is that i have a dry cough which is irritated by running hard, and certain weathers. Like really cold weather. This is my exercised enduced asthma reacting, and not my CF. So it was a clever move on his behalf.

NOW....for the all important question. I asked Prof this question.
Q. Do I have a mild or severe case of CF
A. "ohhh you have the most severe case of CF there is"
I know it seems like I have a mild case, but you don't understand how much I train,and keep on top of every fine detail, and if you do, then you will know what I'm talking about. You just see me "going on the odd trot" to stay well.
I had a conversation with Prof about this. By this I mean doing things like exercise and physio, at an early age, and obsessively (I say obsessive in a light term, so don't start with your crap!!!). Heh totally agrees with me that exercise is a huge part of a CF early life, and needs to be done, everyday.

He gave a good formula;

If you have a BMI over 20, are over the age of 24, a FEV1 of 52 or greater, and more then a certain amount of exsasserbations in a year (I think he said 2-3), then you will be ok for the next 4 years.
Likewise, if you have everything opposite, then you most certainly will either be dead, or need a transplant within 4 years.

I will leave you with this quote "you don't want to be ok, you want to be the best and win" - Prof, speaking about my CF.

Adios