CF Awareness Post 3 - Differences in Treatments

For this one I asked a few of my non-American CF friends to help me.  Since the question deals with treatments in other countries, I could not answer.  Thank you to Julie , Alice  and Tracy for helping!!!
A little background on my helpers.

Julie is from Canada.  She still has her original lungs.  
Alice is 5+ years post transplant living in South Africa.  
Tracy is 5+ years post transplant and living in Australia.

Question: Hey, Amy! I have a CF question... how does CF treatment vary between countries? Or, maybe another perspective would be, how does America differ from other countries in its CF treatment? I know each individual case is unique, but it seems like if I've read a blog based in Canada vs the UK vs America that each demonstrate a different approach to their treatments. Some examples might be: when a person is listed for a transplant, or how aggressively something is treated, or what conclusions are drawn from the same data… is the nation’s healthcare the #1 factor? Is it more dependent on the medical team you’re with? Or is there a general impression that depending on where you live, you can expect a certain response? I’ve read a few blogs where the individual may have gone on ECMO, for example, and it seemed like that was only an option for a few days, while others may remain on ECMO for many weeks and that is okay… though I know there’s no one-size-fits-all approach. So this rambling post is to ask how much does WHERE you are have to do with HOW you’re treated?

Answer:

Tracy: Australia does not have the vest.  

Alice: CF treatment in SA differs quite a bit depending on weather you live in a big city or not and if you've got health insurance or not. Where I grew up (smaller city) treatment depended on what I wanted, if I felt I needed IV's they gave me IV's. If I said I wanted to try oral antibiotics first then they let me. Here in Johannesburg, I live now post transplant, they treat the CF patients WAY more aggressively. Not sure about the ECMO, but I doubt they'll transplant someone on ECMO (but that might also be because they do only about 5-7 lung transplants a year so the odds of someone surviving long enough on ECMO is slim).  

Julie:  Canada has universal healthcare and I have private through my disability plan. CF patients in Ontario have a provincial drug plan that covers almost everything, and what it doesn't cover like Pulmozyme (waiting for the patent to run out on it) we have section 8's that cover the cost. I don't pay anything for any of my drugs, so everything, including Boost, insulin, enzymes everything is covered.

CF exacerbations are treated depending on how I feel.  If I want to try orals we do and if I want to skip than we skip and do IVs, or if I feel I can get over it on my own we do nothing. It depends on how much of a drop I get in FEV1 if I'm just down around 35% from 39% we'll try orals, if I'm down anything below 35% we usually do IVs. And those if we can help it are usually done outpatient because they like to keep us out the best they can. We have isolation rooms but we all know there are so many bugs that can be picked up in the hospital. So as long as we're not terribly sick we can opt for outpatient PICC line and home care. But I have a friend who refuses PICC lines, so she has to go in patient for the full course of IVs.  I find each CF person is treated on a person to person basis, some people are treated more aggressively while others may not be, but they still get good healthcare. If that makes sense. I mean if you want to be treated aggressively they will, if you don't then they trust you know your body. 

We have a CF gentleman in the ICU right now waiting for his second set of lungs and he is on ECMO and has been for a long time, we use it as a bridge to transplant... where you know the situation with Rhi, at her transplant hospital being on ECMO makes you untransplantable while on it. That is more probably varying from center to center, just like some transplant hospitals want you 3 years post cancer and some want 5. 

We have all the same drugs you do in the US as far as nebs. We have access to the Vest but it isn't covered under our healthcare plan nor is it usually covered through most private insurance because it's coming from another country... from what I've heard anyways.  And there are people in Canada with the VEST, I know quite a few but they raised the money or was donated it to them. As for myself, since there's not really any proof that the VEST is that superior to manual CPT I stick with my flutter and hubby beats me when I ask him to. Other people still have percussors, mine crapped out and died years ago and I gave up on them because they're always dying.

Exercise is a big part, we see a physio therapist with every clinic visit and clinic visits around here are mandatory every 3 months for everyone no matter their lung function.  So is nutrition, we see a dietitian every 3 months as well.

My clinic has strict infection control. We have isolation rooms for in patient and if you have certain bugs you are not allowed to leave your room. At clinic they don't let us stay in the waiting room very long, we're shuttled of to a private room to try and keep us all separate. Cystic Fibrosis Canada is trying to adapt the American CF control policy and many people (mainly adults) aren't happy with it.

Hyperion - a CF Film

I was contacted by the writer to get the word out about this film he is trying to get produced.  If you can take a minute to check out the website and watch the short clip.  Its worth it.  Its less than 3 minutes long so won't  tie you up all day.

HYPERION

The writer and director was inspired by Eva Markvoort.  You remember Eva and her 65 Red Roses Documentary.

The director is also working with Emily from Rock CF and the CFF to make sure facts are correct.

Looks like a good film and I look forward to it coming out.

Everything is Vanilla Right Now

Last Tuesday I had my clinic appointment.  I also met with the respiratory therapist to go over my vest settings and airway clearance tactics.  It is a new thing they are doing to try to get people more aware of how to use their devices and improve their health.  Nothing really changed with my settings.  She mentioned I should schedule in breaks between frequency intervals so I can do some huff coughing.  Its hard when I am not all gooey to get stuff up.  I told her I do the vest not because I feel better when I do it, but because if my lungs still crap out I can say well I am doing everything.  Its the truth.  And at least I am doing it!  Faithfully!  Seriously its been like 3 months now and I have missed a few days to being sick and that is it!  Considering it had been months before this is like wicked amazing.

Clinic was clinic.  Nothing changed.  I am more run down and feeling crappy but I also had the flu and she told me to finish the 2 weeks of orals I was given and see how I feel.  I go back in June and if I am crappy still I will see about IVs.  This is the longest stint without IVs since I moved up here.  I attribute it to my exercise regime.  Whether or not that is true I don't know, but I feel pretty good considering its been almost 5 months since my last course.  My lung function was the same 38%.  Weight was down 3lbs thanks to the flu.  O2 normal, HR normal, BP normal.  Booooring lol.  Nothing at all exciting, and quite frankly I will take it.

I am a little disappointed though.  My clinic is participating in the next Vertex trial and I was asked to do it, if I wanted to.  Unfortunately you have to have an FEV1 of 40% or higher, and even with a treatment I don't hit that mark.  So I am still out.  Kind of sad.  I enjoy the studies.  Maybe more exercise will bump me up more and I can do another one....this makes two in two months I was shot down.  le sigh...

CF Awareness Post 2 - What is CF?

My second question comes from a fellow CFer as well.  Her question was meant more as a joke but I decided to take it and answer it!

What is CF?

CF means Cystic Fibrosis.  CF is much easier to say and write especially in long conversations and posts.

But what IS CF?

CF is a genetic disease that affects many aspects of the body.  It varies from person to person but it affects mainly the lungs, the pancreas, the digestive system, and the reproductive organs in both sexes.  Some patients have issues with their kidneys, liver, joints, bone density, and heart.  These are secondary issues caused by medications and low oxygen respectively.

The low down dirty reason for CF is the inability of cells to transfer sodium chloride across its membrane.  The result is an abundance of salt in the body.  We CFers tend to be a very salty bunch.  If you look at my legs on a hot summer day they are dry as a bone but covered in a white film....lick me and drink a Margarita!  

This leads to a build up of thick sticky mucus in the lungs which harbor bacteria and cause lung infections.  These lung infections, over time cause a decrease in lung function because of scarring.  Eventually the lungs don't function properly and can not get enough oxygen to the rest of the body, requiring supplemental O2 to be worn.  The decrease in lung function ultimately causes a CFer to either pass away or require a double lung transplant.  These lung infections can be treated with oral antibiotics, IV antibiotics, and inhaled antibiotics.  As well as the use of the airway clearance device the Vest, accapella, the flutter and manual chest physiotherapy (CPT).  Bronchodialators (sp?) are also used to keep the airways open so the medicines can get in there and do their work.  As the diease progresses though, orals and inhaled meds may not work.  As is the case with me.  Orals work sometimes, and inhaled antibiotics are out of the question because I have reactive airways.  Meaning when I inhale something my lungs don't like, they close up and cause severe bronchospasms.  NOT FUN.  Basically I have a massive asthma attack that only Prednisone can get rid of.

In a good portion of CFers, but not all, the pancreas does not work properly.  The pancreas produces enzymes which break down food put in the body to get nutrients.  In some CF patients this does not happen so we must take enzymes WITH EVERYTHING.  If we want the nutrition from the food we must take the pills.  There are a few different enzymes on the market and it takes time to figure out which works for you.  Me personally, I don't take pills with every meal because I have a tendency to get backed up fast.  I take them with my big meals but my 2 small meals a day I skip.  I also have to take a ducolax and Miralax every day.  If not, I risk getting a blockage from my slow moving digestive system.  We learn that the digestive system works in about 6 hours or so.  For me, and many of my CF friends, we might not "see" that meal on Monday until Wednesday.  Bloating and the CF belly are quite common with CF patients.

CFRD is also caused by a reduction in the function of the pancreas.  CF related diabetes.  Kind of like regular diabetes, but not quite.  Sugars need to be monitored, insulin may have to be taken.  I am new to the whole CFRD thing and only monitor with diet so I am not as up to speed on this aspect as I am the rest.  Many CFers don't reach CFRD until after transplant because of the use of Prednisone to reduce rejection, but many do get it prior.

The reproductive organs are also affected.  For many CF men the vas deferens is missing so sperm is not able to make it out when ejaculated.  I do have a few CF male friends who have fathered children the natural way.  But this is rare.  Most need to see a fertility specialist to have the sperm taken out and artificially inseminate the woman.  For women, getting pregnant can be easier than the man getting a woman pregnant.  Though it varies from person to person.  Some women just never get pregnant without help and others find they have "opps'" easily.  There is a extra amount of mucus lining the cervix which can block the sperm from getting through.  This is normal, to see mucus there, but because of the excess mucus in a CFer's body, there is way more here than is normal.  Once pregnant there are risks to carrying the baby and all that, but it is done, and has been done by many many CF women.  Please talk to your CF doctor about it.

The secondary issues such as the kidneys, liver and heart are caused by medications and lack of O2.  All of the medications taken on a daily basis are processed through the kidneys or liver and over time they can wear out causing the patient to need a transplant.  This may happen before the lungs give out or after.  And does not always happen.  Heart issues can also occur because of its need to make up for the lungs declining. Tachycardia is a common issue among CFers.  This is a fast heart rate, I believe above 110 BPM resting.

Check out this chart for more issues we CFers can encounter.

(source)

For more information visit the Cystic Fibrosis Foundation's website.

CF Awareness Post 1

My wonderful CF friend Cindy asked why are people with cf so much BETTER than everyone else? I mean, they are clearly superior to the rest of the human race. What causes that?

My answer on FB was:

Well Cindy, we CFers are so rad because someone or something gave us such shitty genes they decided we need the AWESOME genes to make up for it. So we are some bad-ass, sexy, down to earth, mo-fo's who take shit from no one. We rock.

The longer version is this:

CFers are amazing and awesome because we deal with a ton of shit on a daily basis and the only way to get through it is to be awesome.  We need to have kick ass personalities to make up for the short life span and the coughing uncontrollably at the most awkward moments.  Our sense of humor needs to be such that we can laugh at ourselves over the silliest and most disgusting things.  We need to live life to the fullest in only a short time so we don't have time to waste on the stupid shit.  

Frankly, we are who we are because of CF.  And no one can take that away from us ;)  Whether you think your CFer is awesome or not, they are fighting a hard battle that only gets worse as the years go on.  Remember that the next time they are too tired to do something, or just want to hang out at home.  We are stubborn as all hell too and won't tell you when we feel like shit, unless we really feel shitty.  Sometimes saying, "I'm a little tired let's stay in," means I feel like shit and a need a rest.  

Treat your CFer with kindness and respect and love.  We all want and need it, CF or not.

May is CF Awareness Month!!!

How are YOU spreading awareness?

Me?  Well I have decided that each day I will take a friend / or stranger's question and post the answer as a reply on here and on Facebook.  FB will be the short version and the longer on on here.   Some days I may have 2 posts if I need to update about my health etc.  But there will be a post everyday.  That is my blogger challenge!

This is what I posted to the FB world:

Yesterday was the start of Cystic Fibrosis Awareness Month! Taking a cue (and some of her words) from Anne, I am doing this. Each day I will pick a question and write about it. I can give the short answer here and the long answer on my blog. Ask whatever you want to know about me and CF, CF, lung transplant etc. If I don't know the answer I have a stash of some dam good friends that can probably answer the question, especially if it pertains to the post-transplant aspect of it. Seriously ask anything!!! If you have a question you want to ask but not others to know about you can send me a message. Otherwise, happy thinking, and asking!!! ♥ ♥ ♥

***Just a reminder, any answers I give are from my own personal experience with CF and the information I've gained on my journey living with this disease. Please don't take any of what I say as advice or the only answer. Each CF'er has their our own story and their own routine as prescribed by their care team.***

Start thinking and posting those questions to me!!!

Bitten by the Flu Bug!!!

Yup, it got me, a little late in the season but it got me.  It started with Peter last weekend and I picked it up this weekend.  My CF doctor told me to come to the ER if my fever spiked again and since it was 103 this morning, we decided to take a trip.  Some fluids, a nose swab and a chest x-ray later and it was determined I have Flu B.

They are keeping me over night to monitor me and make sure my breathing doesn't get any worse.  Its very hard to cough since it feels like I have glass shards in my chest, but hopefully the TamiFlu and fluids will help with that.  Looking forward to being back to normal and getting my paper done this week.  I finished one yesterday and printed it out so I am going to make use of my time in here and get the other one done as well as the assistantship stuff and practice for my presentation on Thursday.  I have so much to do this week it really sucks the flu had to hit NOW!!!

I have an appointment with my CF doctor next Tuesday, the 7th, so we will see if this flu did anything to my numbers.  I was expecting to be put on IV antibiotics next week, but who knows now.  Right now I am just getting IV fluids, no antibiotics.  But that could change tomorrow depending on what my culture comes back with.

I might as well update on everything while I am here right....Thursday I started working out with my personal trainer.  LOVE IT!  I was so sore on Friday but a good sore.  I am looking forward to being in shape and toned up.  I cannot wait.  Thursday I also had transplant clinic and I got another clean bill of health and a "see you in 6 months" as I walked out the door.  Love that too!  I go back in 6 months and that will be right when I am finished with the PT so maybe my numbers will have improved some.  She was excited for me when I told her I want to look into going back to work.  She thought that was a great idea, as long as I figured all the SSDI and disability issues out.  I've got a year to do it...I had seen them right before the honeymoon from hell so I was telling her all about that and she was so shocked my lungs crapped out as much as they did on the plane.  She said they would have had to make an emergency landing had I not been wearing my O2 for sure.  She said some people just respond totally different to flying than others....yeah ya think lol.

So that is all.  Hopefully I am out of here tomorrow and back home curled up in my bed.  It took me forever to get out of the house today because I didn't want to move.  103* fevers will do that to you...

Sorry for the Slacking

I just realized that its been almost 2 weeks since I posted!!!  I am slacking and I apologize for that.  Life has been hectic around here.

Last Monday, as most of you know, the Boston Marathon was bombed, killing 3 and wounding over 200 people.  Friday one suspect was killed and the other was taken into custody at a Boston hospital where he remains.  Living in the suburbs of Boston this was a scary event.  When they shut Watertown down (I have a friend that lives there) I was nervous for her and nervous that the guy got out.  Friday was filled with ups and downs as the country watched the events unfold.  We were thrilled when the guy was captured.  Now we can rest easier in this great city.

Besides the tragic events that unfolded last week, I have been swamped with school work.  I have two papers due May 2nd, one of which is written, just needs to be proofread.  The other I have started to write, but I also have another paper due this Thursday I am concentrating on.  Plus my assistanship work needs to be finished by May 1st.  I don't think I went to bed before 2am all last week.  And up again early to get going on the work.  I have not slacked on exercising which is usually the first thing I cut out.  I have forced myself to go every day no matter what.  I read on the treadmill so the readings for class at least get done.

I have also been stressing a bit too.  Next Thursday, the day both final papers are due, I am also presenting at my school's Graduate Research Day seminar.  I have to get up in front of students and teachers and read my paper from last semester, then answer questions people may have.  I am SCARED SHITLESS.  Luckily I have an Ativan left over from my MRI a few weeks ago.  I plan on taking that bad boy shortly before I get up there.  My professor said to plan for 20 minutes in front of them.  Fabulous....Family and friends are welcome so P is going to see about coming with E.  I am honored and I refuse to turn away such a great opportunity.  This will look amazing on my academic resume as well as just my work one.  My paper is on Queen Elizabeth I and her involvement with the Puritans during her reign.  Who knew that working my ass off last semester while trying to plan a wedding, get married, have an extended honeymoon, and lose 2 friends would make me write a dam good paper.  I knew it was good, I loved it when I wrote it and re-read it.  But I had no idea it was THAT good lol.  I should really take more credit for the hard work I put in to school huh?

I have transplant clinic on Thursday.  We shall see how that goes...

Kicking Ass and Taking Names!

Yup that's right.  I am kicking some serious health ass and taking names along the way.  OK maybe not actually taking names, but I could if there were people to take names from....

Anywho...

I have been at the gym 6 days a week for the past few weeks and I am LOVING IT.  I take Pilates and Yoga on the weekends and tonight I did a Body Pumps class with barbells and weights and all that fun jazz.  I took it two weeks ago too.  There is one on Monday nights which I might start going to.  Its a dam good workout!  And considering right now I have less than one full lung working, I am kicking some SERIOUS ASS!  However, I wanted to attempt the Couch to 5k again, so I decided to try 30 seconds of jogging.  Yeah serious fail.  My lungs laughed at me, then died.  My O2 plummeted, but weirdly enough, my HR stayed about the same.  Went up a few points but I expected it to sky rocket.  So looks like I won't be running any marathons in the next few months.  But that is OK.

I have also been doing my vest every single night!  That makes almost six whole weeks of doing it, even when I went to visit family!  And today I did it TWICE!  Say whaaaaaaaaaaaaaaaat.  I know!

In other news...I dropped one of my summer classes.  So now I will be taking just the two.  I decided that three was way too many and bound to land me in the slammer.  Two I can handle.  And I can switch up my program some so I take one class this Fall and then an independent study next Spring and be done.  The IS is a portfolio class, and it entails just revising papers you want to include in your portfolio.

That is all for now.  I have a clinic appointment in May again so I will see if this exercising ass kicking is doing anything for my numbers.

Study Disqualification and More

Clinic went well today.  For the first time in a few years I am not trying an oral antibiotic on the first appointment after a round of IVs.  I feel OK, not fabulous, but not bad, and since my numbers are roughly where they were last time (post bronchodilator) I can wait and see her again in 2 months.  If I make it that long I will be ecstatic.

Since I had to meet with my professor this morning at 10 am and clinic was at 12:30, I knew I would be in need of a treatment.  My numbers reflected this.  I blew at my highest at 1.01L, 35%.  Last clinic I was 1.16L, 40%.  But I was trying to get into a Pulmozyme study so I asked if I could have an albuterol neb and try again.  We did!  I hit 1.15L, 40%....but it was not good enough.  The calculations they use for the study had me at 37.5%.  Sad :(  This is the first time I have ever been disqualified from a study based on FEV1 being too low. I am not happy about that.  What can you do though right?

Holding steady I am.  I registered for classes yesterday and my summer will be BUSY.  3 classes, one is a 12 week class, one is a six week class, overlapping the last six weeks of the first and the third class is a full week institute that will take place simultaneously with the other two classes.  So the third week of July I will have 3 classes!  My week is going to be hectic!  Monday I will be at school from 8:30-4:30, then Tuesday, Wednesday and Thursday I will be there from 8:30am-9:30pm and Friday again from 8:30-4:30.  Can we say fevers and sleep that weekend?  I don't care though.  I need to finish by December and to do so I need to take three classes this summer and two this fall.  Plus this will test me out on something I am thinking about...

Going back to work.

I have been working out at the gym five days a week doing cardio, but will be adding strength training in shortly.  Sunday's I take a Yoga class too.  My goal is to work my butt off (literally as well as figuratively), and see if I can get my heath more stable and get it up.  If I can get to 45% and stay off of IVs for 6 months, then I will seriously consider working again.  P is not excited about it, but he said he will support me....but only if it won't make me sicker faster.  I agree with him.  I don't want to go back to work and in three months have to stop again and be listed for transplant.  But I have nine months to think about it, weigh my options and decide if it could work.