IAmA 24 year old blogger living with Cystic Fibrosis. Ask me anything - it's Cystic Fibrosis Awareness Month!

I couldn't help but notice that your instrument of choice is the saxophone, and from what I've heard the saxophone (and while we're at it, the clarinet as well) is pretty demanding breath-wise. How has having a respiratory disease affected playing the saxophone? Has cystic fibrosis influenced your choice in instrument/choice to pursue a music-related career?

This is such a good question! I chose saxophone when I was just a kid and still had the lung function of a normal person. The short answer is YES. Choosing this instrument has given me great trial through the years.

The long answer is that I have had to fight and bring awareness to a lot of people I had to deal with. College was the hardest and most trying part of this. My parents had to carefully have the conversation with me that I was never going to be able to be a performance major because I think they knew that I wouldn't have the ability to play forever and musicians don't exactly have very good health insurance. It's also right about the time when I started having enough issues with my CF that it was affecting me on a daily basis. I didn't know how to deal with teachers and other students not understanding that I was sick (because I don't look sick). I actually had to go to my doctors on advice on how to deal with this. There were some encounters that led me to tears out of frustration. I didn't want to whine about my disease or anything, but I just couldn't always practice as much or play as long as my peers. I successfully put on my senior recital. We were clever and had 2 of my fellow saxophonists play in between pieces so that I had a chance to rest between pieces. We also had to cut some pieces down because I couldn't get through the whole thing. I actually have most of the recital up on YouTube if you want to check it out. Just look up The Living, Breathing Wendy Senior Recital. Playing a wind instrument is excellent exercise for your lungs too, which is one of the best treatments you can do to keep your lungs happy and healthy.

This makes me so happy to read! I am a music therapist who pretty regularly works with kids who have CF. One of the primary modalities I use to assist with breath support/lung capacity/moving the phlegm (not the best wording😊) is playing the harmonica. It really helps the kiddos. I can see their blood oxygen saturation level rise pretty immediately, and their lung functioning improves as evidenced by their peak flow meter.

I'm so glad you've not only found the joy in playing an instrument, but also realize its extraordinary health benefits! Keep on keepin' on, fellow musician! I wish you the best!

NO WAY! I almost went to school for music therapy and now that I am done I kind of wish I had actually done that. It's such a cool thing. That's so crazy that their sats rise just form music!!!! You are interesting person. :)

It's never too late! They offer masters equivalency programs! :-) But yes. Music is the great motivator. Why struggle with those shake-vests and peak flow meters when you can sing or play an instrument and accomplish a lot of the same goals?! Not to say that the medical interventions aren't absolutely important and necessary, but if you can do other things, like play a wind instrument or sing, then why not? Right?

Good on you for sticking with your instrument. I'm sure that your lungs are stronger for it, and the emotional benefits of music are innumerable!

I am so sick of school. Haha. I'll have to research it a little, though.

Funnily enough, I also have CF and for a few years in elementary/middle school I played the sax before my lung function deteriorated too much and I had to switch to percussion.

Aw. I'm sorry that you had to switch! I find sax to be fun lung exercise. Maybe pick it up once in a while. Even if you can only play for 5 minutes. It's still fun and beneficial. :) Hope you have fun with the crazy kids in the back. Haha.

I'm so glad that playing a wind instrument has provided you with help and enjoyment! I'm a brass player who had a go on my friend's alto sax and found it nigh on impossible to play, going to look up your recital later :)

Excellent! It's ok. I can't play brass to save my life! Haha. It tickles and I just can't get the buzzing to different notes right. Hahaaha.

Your stubbornness reminds me of how my brother was with CF. Despite knowing that meeting anyone with a cold could kill him he became a documentary photographer and went into slums in India and Africa to give the poorest a voice.

Please, if you could, read more about him here http://www.walesonline.co.uk/news/health/striking-image-captures-toll-cystic-11254622

Wow. Your brother was INCREDIBLE! That's so cool that he devoted his life to helping others. I'm sorry to see that he got diagnosed so late in his life. It is so sad to see that. I'm sure it's really peaceful to know that he led a fulfilling and proud life.

Have you ever considered using an electronic wind instrument and hooking it up to an amp? It probably has a sensitivity setting so as to be less demanding.

I haven't really considered it. It is good to play an actual wind instrument. It provides fun exercise. I just play for fun, so if it gets too difficult to play I just stop.

Not to be insensitive, but how do you cope with the fact that you won't live a long life?

I guess hope and laughter. This actually brings out something very positive I have learned about CF. We are positive people. I'm not sure why. We were given such a cruel disease, but we make the most of it. Why let it bring you down when there is nothing you can do about it, but make the best of it? I just take life one day at a time. I make sure that every day I am doing what I WANT to do and not what I think I have to do. I travel a lot. One thing I really want to do is see the beauty of the world, so I just make sure that happens. You get your priorities in order very quickly. I think we live just as fulfilling life as anyone else, just quicker.

My roommate had CF and passed away in 2011 just weeks after his 23rd birthday. He wasn't very public about his illness, I knew he had it, but didn't quite realize what it meant. He always used to say (with a smirk) "It's not what you do in life that counts, it's who you do it with."

I'm sorry to hear about your roommate. He sounds like a classic CFer.. Always wanting to have a good time. :P

Also, isn't the life expectancy so much longer now? I know compared to age 70 or 90 it might seem shorter, but with new treatment developments, hasn't the life expectancy for CF extended to 40 or 50? (Compared to teens or 20's in the past)

Yes, the life expectancy is much better than even 20 years ago. It's incredible. However, there are still so many people that still pass in their 20s from this disease or are waiting for transplants.

How hard on your body and mind are the the treatments and what after effects to you experience? (I could Google but real people are way cooler).

The treatment are pretty easy and I can play video games, read, go on the internet (I am doing a treatment right now). However, they are very time consuming and keep me pretty busy. I do three 45-60 minute treatments per day along with several pills. Then there is the hospitalizations that last for 2 weeks (I got in 2-3 times per year, which isn't all that bad for a CFer). The hospitalizations are the hardest part of CF care. They are lonely and long and the IV antibiotics can make you feel really sick if your body doesn't agree with them at first.

Respiratory Therapist here. Playing Mariokart against CF patients who think I will go easy on them is one of my favorite things. Treatment time is the best time to show them who runs this...

YOU ROCK. That is literally my GO TO video game in the hospital. Everyone always says it's a trap to play with me. I kick butt. :)

Wow. Just read your port section of blog. I may have to get a PICC line for IV abx, I have neurological Lyme.

You are a TOUGH WARRIOR.

I wish you luck with it! PICC lines are pretty easy. Ports are just easier than PICCs (if you have to get them pretty often). :) Plus then your siblings compare you to Iron Man and you feel pretty badass.

That's awesome. From the other comments , Music is great! What kind of sax?

I primary play alto sax, but I did do a piece on soprano sax for my senior recital. It was awesome. I played tenor for a Dixieland jazz band that I was in, but I don't think I could handle that anymore! Haha.

I'll check your recital out. Ever try a MIDI sax? My friend has one, it sounds otherworldly.

I have not tried it! Maybe I will have to check it out. I have really not heard much about it, to be honest.

Extremely versatile, and you can use headphones, full synth access.

https://youtu.be/KJnw-r1e9jQ

THAT IS SO COOL!

What are some common things that you have trouble with that others may not realize?

ENERGY. That is seriously the biggest issue I have. It's an invisible illness. I look like a totally normal person on the outside. It takes me so much energy just to breathe that I often am too tired to do everything I want to. The other thing is the cough. People always think that I'm sick or make fun of me for smoking too much and don't realize that it kind of hurts my feelings because I can't help it. I would LOVE it if I never coughed again.

I know what you mean; coughing in itself is very exhausting. Are you not on oxygen to assist your breathing?

I have never been on oxygen. My oxygen levels have never been low enough for that. They currently sit around 93% and need to be below 87% (I think) to need supplemental oxygen. I actually know a CFer who sits around 10% lung function and doesn't need daily oxygen. Then there are some who have 40% lung function who can't breathe without. CF is a tricky disease!

23 year old Cyster. Naps are my best friend. I seriously try to plan my class schedule around being able to take a nap between class and the days I work.

Ahhh. Yeah! Naps rule! Haha. I actually hate them, but I almost always need one. :P

As a medical student, I wonder how doctors speak and have spoken to you about your disease and its prognosis. Are there any dialogues youd like to share, both "how to do it" and "how not to do it"?

CF is one of the trickiest diseases, I think. There is no "right" or "wrong" way to do it because it is such a different disease for everyone. What works for one person may make another person worse. Along with that, some people have worse lungs while some people have fairly healthy lungs and awful digestive system. It looks so different on everyone. I have seen my doctor be extremely frustrated sometimes because you have to be so creative with treatments. You have to try so many different things before you find out what works. For example, I am on high doses of ibuprofen still because if I don't then I grow nasal polyps the next day and that's not really what ibuprofen is for. So it's just crazy like that.

What does your typical day of treatments look like? I don't think people understand how much time you spend treating CF.

It takes HOURS of therapy. I personally spend about 4 hours total a day. Here is a post I wrote last year about my treatment schedule: Keeping Up With Health. However, I do even more than that now because I do my treatments 3 times a day (the midday treatment is the same as the morning, basically) instead of 2 times. The hospital stays are the most time consuming, though. Those (for me) occur 2-3 times per year and last up to 2 weeks. It is lonely and disrupts your life completely. You miss work, social events, and anything else going on in your life on very short notice. There's almost nothing you can do about it too because you don't know it's gonna happen and you are too sick to not go in.

My brother has cystic fibrosis so i was wondering what could i do to be supportive of him?

Just be a brother. The best things my sibling ever did for me was just treat me like a normal human being. Stick up for him when other people don't understand why he's coughing or is out of breathe and can't do anything. Now that I am older, my older sister takes care of me when I am sick and takes me to all of my appointments. She even flushes my port for me so that I dn't have to go to an appointment to get it flushed.

I think it's so cool that you are trying to learn how to be more supportive. I think that's the sweetest thing ever! :)

Thank you for doing this. I lost one of the most important people in my life to this disease, my Uncle David. Unfortunately, cf doesn't get much attention, and most people aren't very educated about it. I honestly didn't know much about it for a long time. I didn't do much research until after my uncle's death. I miss him everyday but I also know that he lived a full life and didn't let his illness define him, which is the main reason I was so uneducated. On a lighter note, what is your go to breakfast cereal?

I'm very sorry to hear about your uncle. It is so hard to lose someone, especially in such a painful way. I certainly don't let CF control my life, but I am a huge advocate for awareness and research because we are so close to finding a cure. I usually like to snack on multigrain cherrios or the Special K cereal with the chocolate chunks in it. Yum. :) Right now I am eating crepes for breakfast, though.

I read through your blog a little, interesting thoughts on Orkambi. My girlfriend has been on it for about 9 months now, and she had a lot of the same symptoms to start with (tiredness, harder coughing, she had to take a hospital stay to get over the transition onto it). A few months later, once a lot of old mucus had shifted out, she actually started feeling a lot better. It's honestly been a life changing thing for her, to the point where it's honestly now more mental than physical factors stopping her doing something. It's a amazing to watch her rediscover that she can do things almost like a normal person, all thanks to four pills a day. Just wanted to share this in a relevant discussion!

I suppose there has to be a question here. Since I'm a Brit, what do you think of the Orkambi situation in the UK? If you haven't heard, it's been provisionally denied on cost grounds, and the UK CF community is in uproar.

I have actually kept up a decent amount on that. There has been a lot of speak of the UK issue on Twitter recently. I think it's always an unfortunate thing to deny people something that could ultimately save lives. That's why awareness is so important. It's amazing what we can accomplish simply through education.

Also, I'm really glad to hear that the Orkambi is working for your girlfriend! It's an amazing thing to be healthy after not being healthy for a while. You feel like a SUPERHUMAN.

ELI5: What exactly is Cystic Fibrosis? I've heard of it but I'm not sure what it is. Also, how does it interrupt daily life for you?

"In people with CF, a defective gene causes a thick, buildup of mucus in the lungs, pancreas and other organs. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients." - Cystic Fibrosis Foundation

It mostly interrupt my daily life by the amount of maintenance it takes to stay healthy. I spent almost 4 hours a day on treatments. Also, as I get older my energy is starting to be used up more and more to breathe so I am able to do less in a day and get tired out easily.

What are the symptoms you have to deal with? How do you acquire this disease/syndrome/condition?

Cystic Fibrosis causes the body to not absorb salt into the body correctly leading to thick, excessive mucus. The symptoms are a chronic cough, which I have a pretty bad one. It causes headaches, horrible sleep, and is very hard on your body. The other biggest symptom is a hard time gaining weight. My body doesn't digest fat or protein, which is what make it so hard to gain weight. The pancreas is blocked off by the excess mucus, so we have to take pancreatic enzymes in the form of a pill (which is what allows your body to digest fat and protein).

The disease is hereditary, meaning that you are born with it. Both of your parents have to be carriers and then you have a 25% chance of getting the disease. I have 3 siblings and I am the only on with CF.

Cystic Fibrosis causes the body to not absorb salt into the body correctly leading to thick, excessive mucus.

Does this mean your diet contains as little salt as possible, as to not having to deal with the mucus?

It is the LACK of salt that causes the mucus. It sweats out because it is not being absorbed into the body. so the diet is actually the opposite. (It's confusing, I know!) We lose a lot of salt quickly and easily causing dehydration. It is highly advised that if we are going to be out in the heat for long periods of time to make sure that we have a salty snack on hand. CF diets also require high protein and high fat as our bodys are deficient because we cannot digest them properly.

Do you think you will ever need a transplant? My older sister had CF and got her double lung transplant at 19. Unfortunately her body rejected the new lungs and she passed soon after. I hope you stay healthy as long as you can and enjoy life.

Thank you. I'm so sorry to hear about your sister. I have no idea what the future holds. All I can say is that I am no where near needing a transplant today. I also hope I stay healthy for a long time and I most certainly do enjoy my life. :)

I'm post transplant.

The day you hit 30% you should begin the process of being put on the list. You will have a low score so chances of getting it then are exceptionally low. I was still out living my life at 18% lung function, but using oxygen, woke up one day in lung failure and didn't come out of the hospital for five months after a new set of lungs. I waited to that last possible moment so instead of being a routine transplant, in and out in two weeks, I was on end of life support living in the ICU only days from being dead when my lungs came.

Yikes, that sounds really intense. I'm really glad that you were able to come back from that! You are very strong!

What medications do you take?

Have you had any serious respiratory infections? B. cepacia, etc?

What do your drs tell you with regard to your life expectancy?

I personally take 5 different inhaled medications: albuterol, hypertonic saline, pulmozyme, Cayston (every other month), and Tobi (opposite months of Cayston). I do 30 minutes of Vest treatment 3x per day. Then I take about 30 pills a day, which include Creon enzymes, high doses of ibuprofen, vitamins, prednisone, and azythromicin.

Yes, I have had several respiratory infections.. Umm.. I don't remember the name. I usually culture the same ones most of the time. I think I had B. cepacia when I was a teenager, but then it magically went away (which is very rare to happen). So I was lucky with that one because it's a nasty one. The one that I've been dealing with lately is AVPA, which is an allergic reaction to a fungus called aspergillos (sp?). It causes a lot of inflammation (more than already exists) in the lungs.

We don't talk about life expectancy. Cystic Fibrosis looks very different in everyone. That's why it is so hard to treat. What works for one person may not work for someone else, symptoms look different, and body reactions vary so much. In my lifetime the life expectancy has risen 20 years and they are doing so much more research. I am just hopeful and thankful for how far I've made it and what I have achieved so far.

How did you get diagnosed?

It is diagnosed by a sweat test. People with Cystic Fibrosis sweat out high amounts of salt. I was lucky enough to be caught in a study that they were doing of infants and got tested for CF. So my parents found out I had CF when I was 6 weeks old and were able to start treatment before I had any major or damaging health issues. Here is a link to the Cystic Fibrosis Foundation about it: Testing for CF

My step-mom and her company have been working tirelessly for the last 10 years on a new and improved sweat test kit working closely with the university of Minnesota and it is in its final stages of approval from the FDA and it will prove to be a game changer in both efficiency and cost and if successful it will be the new standard in hospitals across the country. She may be doing a Ted Talk X about it in the coming year. I'm very proud of her dedication to the CF community.

That's so cool! I would love to hear more about that via email: thelivingbreathingwendy at gmail dot com

You should be proud. She sounds like an incredible lady!

Hi! I'm also a 24 year old with CF. :-) I have a few questions:

1. What do you typically get for your FEV1 test?

2. How often do you have to make doctor's appointments?

3. Do you have any siblings? And if so, do they also have CF?

4. What mutation do you have? (Mine is 2 copies of delta F508).

5. On your website you stated you do 'alternative treatments' to the vest. What are they? I typically do 2 30-min therapy treatments per day + albuterol and pulmozyme. I'm curious what you use to replace the vest occasionally.

I'll probably have a few more questions as I read through your comments and blog. :-)

Haha. Feel free to ask. It may be easier for me to respond via email because I'm worried your questions will get lost in the sea that is this AMA: thelivingbreathingwendy at gmail dot com

1. Right now I am sitting around 60%, I think. It's been a rollercoaster lately so it's hard to tell. I've been as low as 38% and as high as 81%, soo..... :P
2. I believe the general rule is 4 times per year, but I have been going almost every other month due to my inconsistent numbers and my doctor trying to figure out what's going on and that I am still healthy.
3. I have 3 siblings. I am the only one with CF. I THINK 2 of them are carriers and 1 is not.
4. My mutation is the same as yours!
5. I actually just had to reread that post to figure out what you were talking about. It's just different medications to try, not an alternative to the Vest. So, for example, they let me go on Tobipodhaler instead of nebulized Tobi. Make sense?

My 3 y/o daughter is a carrier for CF, any advise on what I should eventually tell her about the disease? I feel it's important that should she have children that she be fully aware of what life is like with cf in case the father is also a carrier.

Definitely get the father tested when the time comes, but CF is a recessive gene. I wouldn't make a big deal about it if she is just a carrier. Just educate her about the disease. You don't want to scare her.

Does it baffle you that people with perfectly good lungs ruin them with tobacco?

Yes and no. While I think it's gross, I think people have the right to choose to do what they will with their bodies. It is their own home to treat how they wish. It's the same thing as people putting all those processed foods in their bodies. It's unhealthy, just in a different kind of way. Unfortunately, that's just the way society and the world is right now. I hope I was clear enough.. I think some of my thoughts got jumbled together in my head.

Whats your way of dealing with it?

I make sure that I surround myself with positive people and laugh A LOT. Mostly, the way I've found best to deal with it is just do the best I can do with my treatments and live my life as close as possible to a "normal", healthy person. Blogging has really helped me work through a lot of fears and issues too.

What is the most difficult part of having CF for you?

Thanks for doing this, mad respect to you btw

The most difficult part is fitting my treatments together with my life. I love to do stuff. So I am constantly learning how much one day can consist of.

Thank you!

I thought there were gene therapies for CF now. Any hope you could participate in a trial?

I actually was in the Orkambi drug trial for 2 years and am in the middle of looking into doing another trial. That one did nothing for me. You can read about my experience in the trial here: Vertex: The Journey to Orkambi. Unfortunately, that drug did almost nothing for me. There is a drug called Kalydeco that has been a miracle drug, but it is only for a rare gene mutation. I have the most common mutation, which is 2 copied of deltaf508.

Thank you for doing this. I lost my best friend to CF at age 39. He underwent a double-lung transplant at age 34 knowing it would only prolong the inevitable- and wound up losing his legs as a result. Have you thought about the lung transplant option should it arise?

I'm so sorry to hear about your friend. I can't believe how long he lived! That is so incredible. I'm sure you must be so proud of the fight he must've given I have not thought about it. I am kind of the type of person that takes things as they come. It keeps me from stressing and worrying about the future, you know?

Hey Wendy. Another CFer here. How helpful have you found hypertonic saline? How much do you use it? What concentration? It's great to see that you play the sax.

I actually LOVE hypertonic. I know a lot of people that HATE it. Haha. Yeah, it makes me cough, but it makes my lungs feel so loose and like I can actually move junk out and feel less congested. I use 1 vial of 7% three times a day. Thanks! I love music! I play any instrument I can get my hands on, really.

That's interesting. I like to see what other Cfers think of hypertonic. I personally use a lot of it as well. I think it needs to be more of a primary treatment rather than just the add-on that most people see it as. I make my own 20% solution and use lots of it. Sounds a little crazy, but it works really well for me. I'm glad you're a fan of it!

Woah! That is crazy. I can't imagine using anything stronger than 7%. Haha. It's the perfect concentration for me!

As a soon to be registered nurse, what can I do to make those with CF have a better hospital stay? :)

Nurses are literally my favorite people. Some of my closest friends are the nurses that take/took care of me. It's crazy. Just be our friends. Haha. I know it sounds dumb and easy, but that's all. The worst part about being in the hospital is being away from your friends and family and missing out on that part of life. So it's really cool when you are at peace with being admitted because you just get to hang out with your "hospital friends".

I saw the movie Alex: The Life of a Child when I was in early high school and was so incredibly affected by it. Have you seen it and if so, how accurate is it as far as the symptoms go? I remember one part in particular where her father is firmly patting her chest as she is upside down. Daily treatment has to be brutal.

Well done on staying so healthy despite your disease. I'll be checking out your blog.

I can't remember if I saw the movie, but I definitely read the book. It was very accurate. Unfortunately, Alex lived before there was much treatment and people died very young from CF. My mom used to do that patting when I was a kid before the Vest was invented.

23andme.com let me know that I am a carrier for cystic fibrosis. So, there is a chance of my offspring coming down with the disease. How would you rate your quality of life through the years and years ahead against your peers?

I think my quality of life is pretty good. I think a lot of it has to do with perspective. I may not be able to do EVERYTHING I want to and some things are hard, but I make the most of what I have and that's what's most important

Hi, Thanks for hosting this AMA, it's great to see people raise awareness of CF. My mother was a CF sufferer passed away in October while waiting for a lung transplant. What keeps you so optimistic while battling such a cruel disease?

I'm so sorry to hear about your mom. That has got to be so hard. She must've been an incredible lady to be a parent and battle CF. That always impresses me greatly.

I think just reminding myself that what is the point of life if you aren't happy and enjoying it? I'm not really totally sure why those of us with CF are born so optimistic. Maybe it's just a side-effect. Definitely a necessary one, though. Haha. I am just thankful for the things that I have in life. I have wonderful friends and family, my pets make me smile every day, and maybe we don't have the same fears that others have because we have different things to fear.

So if I understand correctly, prolonged exposure to other CF patients could be damaging to both of your health. What is it like to not be able to meet/make lasting friendships/relationships with others with the condition?

Right. I think with all of the technology that is around, it is easy to have a lasting friendship from a distance. The hardest part about having friends with CF is when they pass.

My dad works in the pharmaceutical industry and his company is solely focused on developing drugs to combat cystic fibrosis. One of the biggest comments he has regarding his work with cystic fibrosis is how welcoming the organizations around CF are to new drugs and medication. He works in clinical trials, so it's one of the most important parts in determining how effective a drug can be.

My question for you is what is your take on an increased pace or effort to pass drugs on the part of cystic fibrosis organizations? Do you feel that the mortality of CF makes drugs more credible to have lots of (overwhelmingly so) support from CF organizations? Thanks.

I think that it's great and so supportive that the CF organizations are trying to make more drugs available. It's kind of why they exist, right?

I hope I understood your question right... I had read it a few times before I made sense of it. My brain is getting tired from reading and typing for the last 5 hours. SOrry.

Are your parents well off?

My parents were able to provide proper care for me, yes.

Have you met the Esiason family and what do you think of Boomer's work combating CF?

I have not heard of that family. It is also not advised for CFers to be in close contact. The bugs and infections that we grow in our lungs are highly contagious between CF patients.

What kind of work do they do for CF?

Ok, so this was on an episode of some medical show (greys anatomy? House?) where the doctors got angry with a couple when they realized that BOTH of them, not just one as previously thought, had CF. They were reprimanding the two of them pretty severely. Can you elaborate on why this is a big no-no? When I searched Google to try to understand the dynamic, I didn't really get much of a detailed answer. You can catch each other's lung infections?

Yes. Bugs and infections that we grow in our lungs are highly contagious between CF patients. The rule is to be no closer than an arm's length.

Make sense?

Yes, sorry I guess that was super straightforward!

Haha. It's ok. I feel like it's kind of a weird thing that wouldn't make sense unless you knew.

What research / advancements / new treatments are you most looking forward to that will help people with CF?

Of course I would most be looking forward to a cure, but at the moment I don't have anything specific that I am looking forward to. I was in the Orkambi drug trial, which was unsuccessful for me. I am hopeful, but try not to get my hopes too high.

During grade school where you allowed to have your inhaler on you?

I honestly don't remember. I remember my teachers holding my enzymes (a pill that I have to take whenever I eat fat/protein). I am almost positive that I had an inhaler in the nurse's office for emergencies. I did nebulized treatments before and after school, so I didn't really need an inhaler at school very often.

Thx for the response. My neighbor has CF and she needed her inhaler from time to time. We went to middle school together. The fact that she wasn't allowed to keep one on her was fucked up.

I think it is more of a protection thing. Think if they lost it and someone picked it up and used it who wasn't supposed to. Obviously an inhaler probably wouldn't hurt anyone, but some medications can be dangerous. She should've been able to keep it in the nurse's office so that she could still have access to it.

I'm trying to think back and I think by middle school I was allowed to keep my medications on me.. I'm sure every school is different.

What are the things you like to do when you're not getting treated or working?

I LOVE to travel, play music, be social, and play with my dog. :)

I work at a hospital that has a lot of adult CF patients. How did you find the transition from child to adult services? We're sometimes worried about the patient being ready to leave a pediatric CF service that they have relied upon for years.

It took me so long to transition. I am 24 and I only FULLY transitioned over within the last year. I know someone who is 30 who still goes to peds sometimes. I think it's just hard because it's only just recently becoming an adult disease. I was lucky that my CF doctor was both the adult and pediatric CF doctor and is now only an adult doctor. So she just transitioned with me. It was pretty easy though, once you have the freedom of the adult hospital you realize that you have been ready for it before you knew it.

My little guy (6) has CF (two Delta F508 mutations). It sucks but he fights through it so well. Thank you so much for doing this AMA and helping spread awareness of this shitty disease.

My question: given what you know now and the effects this has had on you growing up, is there anything you would have changed or done differently knowing what you know now?

I will be reading all the important things that I see in here that I think will benefit my little guy to him tonight. Thanks again. You rock!

I don't think so. My parents let me follow all the interests that I had when I was a kid. I was super active until I couldn't be anymore. I mean, I'm still active just not in sports and that kind of stuff. Now I walk. haha. Just make sure he has fun! :)

You can also subscribe to my blog if you want to continue to learn about living with CF: The Living, Breathing Wendy

As someone that doesn't have CF, what do you wish that non CF people were more educated/understanding about?

That it is an invisible illness. My cough is not contagious. If you are sick please stay away from me. Getting sick for me usually means a 2 week hospital stay and missing out on 2 weeks of work, social events, and other things I had planned. I guess I just want people to have knowledge of how close we are to a cure and that even though I don't look sick, I am.

Do you like pancakes?

Yes, I love pancakes! Especially blueberry ones. :)

Thanks for doing this! How has CF affected your friendships/relationships?

I actually VERY recently wrote a blog post about this EXACT subject! Chronic Illness & Relationships.

Let me know if that answers your question. :)

Have you had genetic testing? What's your mutation? Do you have siblings or other family members that have subsequently had testing to asses whether they're carriers?

My mutation is 2 copies of deltaF508, which is the most common mutation. I have 3 siblings, I am the only one with CF. I believe that 2 of my siblings are carriers and one is not.

Is it possible to eradicate Cystic Fibrosis by preventing carriers from having children?

Umm... I don't know.

Hate to be this guy but what is Cystic Fibrosis? Care to explain?

It's ok! That's what this AMA is for! Here is the link to the official definition on cff.org: What is Cystic Fibrosis?.

For me it means hours of treatment a day and difficulty breathing. I cough A LOT too. You can check out my blog too because all I write about it what it's like to live with CF: [The Living, Breathing Wendy](www.thelivingbreathingwendy.com)

A good friend of mine passed away at age 24 about a year ago due to cystic fibrosis complications. She had other underlying conditions as well that didn't help the prognosis, and was unable to find a donor.

I don't want this to seem morbid, but how "serious" is your case, and have the doctors given you a good prognosis?

Bonus: You remember that Katy Perry video at the Children's Hospital? The "star" (community appointed) of the show has Cystic Fibrosis, and people were wondering why she was in a children's hospital--she actually responded that not all illnesses are easily seen on the outside.

I'm really sorry to hear about your friend. CF can be such a tricky and complex disease. I had a pen pal with CF that died pretty suddenly from complications too. She never even got a chance to be listed for transplant.

I actually have never heard of that video. I'll have to check it out. Props to her!

Hi, thank you so much for doing this AMA! So I have Aquagenic Wrinkling of the Palms (AWP), which is associated with the genes for Cystic Fibrosis. Since I'm most likely a carrier for the disease because of this, do you recommend I get genetic testing for both me and my partner before having children? Would it be able to make a difference at all? Also, do you experience AWP? Thanks!

I would get tested (it's really easy). Then you'll know whether to get your kid tested. Your partner too. If your partner isn't a carrier, then you have nothing to worry about because CF is a recessive gene. I don't think that I experience that at all, but I have seen a YouTuber who mentioned that she gets it. Her AWP is not diagnosed, though.. just an observation.

Wendy do you often find that the Respiratory Therapists are an effective part of your care?

Since I got my Vest when I was 10 years old I have not actually had manual chest PT. I do know people that need both to effectively clear mucus from their lungs. Thankfully that is not me! When I am hospitalized I use respiratory therapists to play video games with me while I am doing my treatment since they have to be there anyways. It's so fun!

You've never needed hands-on PT? Jesus, the advancements are huge. I remember learning how to give my aunt basic PT when I was 9 to give my grandmother breaks; that Vest would have been a godsend.

It's nice to read that things have gotten better, day to day, then they were.

Yes. Advancements are INCREDIBLE. I had hands-on PT when I was a kid, but not since then.

Do you have to wear a vest, and if so for how long?

Yes, I do my Vest for 30 minutes 3 times a day.

How go the PFTs?

They can be pretty rough. They definitely make me cough a lot!

Hey there! I worked at a CF facility for a couple of years. We also dealt with infectious control. We had TB, mrsa, and the like. The one that affected a lot of the CF patients was MDRP. Have you ever had to deal with that? And have you had a lung transplant yet?

I feel like I've heard of that, but don't think that I have dealt with it.. There is so much lingo in the medical world that I feel like I forget a lot of it.

What does a blogger do exactly? I thought that was a "fad" of the early 2010's which was slowly disappearing. Serious question.

I just write about my thoughts and life. I try to help others by sharing my experiences. It also helps me sort through my trials and feelings.

I recently found out that I'm a CF carrier and my wife will be getting tested soon.

Did your parents know they were both carriers before you were diagnosed?

I saw in another post that you have siblings. Are you youngest or did you parents continue to have kids after knowing they were carriers?

I really doubt they did. We found out I had CF because I got caught it a screening study. It was just luck. I am the second oldest, I have 2 younger siblings. I think the youngest was an oopsie, but all 3 of my siblings are healthy. I think 2 of them are carriers and 1 is not.

Thank you for doing this and it has certainly given me an insight into how you have coped with CF. Do you think that your early diagnoses at 6 mnths has had any impact on how you have been able to manage the condition?

Yes, I think it has had a HUGE impact on my health. I think that if I hadn't been diagnosed at such a young age that I would be much less healthy. Most kids find out by getting really sick, which can do permanent damage to your body.

What mutations do you have in the CFTR protein? Just curious. Are you eligible for or participating in any clinical trials?

I have 2 copies of deltaF508. I was in the Orkambi drug trial for 2 years. I'm not sure what's next for trials, but I am talking the coordinator at my center.

Hi there! My boyfriend actually has CF as well.

If you don't mind my asking, what does your daily medicine regiment look like? How is your lung function?

I also noticed you have a Great Strides page! So do I! I'll be walking June 9th in my area to support CF research. The CF Foundation has been absolutely beneficial to all of the research.

I don't have many questions but I just wanted to say, I hope you're doing well!

Thank you! Your walk is so late! Woah!

Here is a link to my blog post about my daily regiment: Keeping Up With Health. It's a little out dated, but that's the basics. I now do my treatments 3 times a day, so just double the morning therapy.

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I'm so sorry to hear about that. I've done some pretty stupid and irresponsible things trying to be a "normal" person. Not that bad, but still not great. I think that it can just be hard to tell people because you don't want them to think of them any differently. When I was freshly 21 I had a really hard time telling people because I would often go out with friends and skip treatments. I didn't want people to think badly of me because I was kind of neglecting my health. Don't be hard on yourself. She was going to do what she was going to do regardless. I'm really glad that my friends stayed with me through my dumb actions. The hardest thing is worrying about being judged.

Now I have no problem telling people and am a huge advocate. I have a great support system. I find it really great to connect with other CFers over the internet so that we can talk about the "hard stuff". No one else can understand.

I meant to include this book that I read that I think you would greatly benefit from reading. It's called Dear Nobody: The True Diary of Mary Rose by Legs McNeil and Gillian McCain.

Is it difficult to be isolated from other CF patients? I know that people with CF are supposed to stay away from each other so as not to pass lung bacteria and infections back and forth.

I know with type 1 diabetes, being able to attend diabetes events and talk face-to-face with other diabetics provides so much emotional support. Is it hard to go without that? Or do you find alternate ways to find support from other CFers?

That is literally the coolest thing about this day and age. It is SO easy to connect with others over the internet. I had a really good CF pen pal when I was younger. She was the only one that I knew with CF for the longest time. She passed away a couple of years ago, so I began to meet others on the internet because all of a sudden I had no one to talk to. now i have a wonderful friend and we Skype. We met once, but were careful.

How much money do you make from blogging? Any tips?

I don't really make money from blogging. I mostly just do it for fun and an outlet for my struggles and joys of life. It would be nice to, though. I actually got an email about an ambassador opportunity, so we'll see where that leads and such.

Just make sure you truly enjoy doing it. I don't really know much about doing it as a profession.

Hey! I am myself a starting blogger, so I want to ask a qouple questions related with it.  

1.What was your inspiration?  

2.How did you started blogging?  

Thanks! And good luck!

That's so cool! What do you blog about?

1. I don't really remember what inspired me to start blogging or what gave me the idea. I'm sure it was something I read while out and about on the internet.

2. I started blogging just so that I could write about the trials that I was going through. I used it as kind of a coping outlet. Then I realized that I could actually help other people and that gave me such joy. That's what keeps me inspired to keep writing.

You're so kind! Thanks for an answer!  

I tried to begin with an inspirational stories that I've created, but later on I've added quotes, pictures. So basically a website with a lot of motivating stuff, I guess. Some people seem to like it, so I make more. Recently started using Photoshop, so I include my works too. And made an option to submit your own inspirational real or fake stories to have more work with a website, too bad none of the people have submitted it. Anyway, if you want to you can check it. https://everydayexplorerblog.com/

I'll check it out later tonight when I am done with this AMA! Thank you for sharing. :)

This is actually a serious question, does your disability limit your ability to "jerk off"?

My ability, no. The comfort of it, sometimes. It is a physical activity so it can throw me into an unpleasant cough attack.

Why do you think you deserve your own month? There are over 12 forms of cancer and only 12 months in the year.

May is not strictly Cystic Fibrosis Awareness month. Just like purple is not strictly the color for Cystic Fibrosis Awareness. It just happens to be the disease that I am battling and raising awareness for. You are right, there are only 12 months in a year. So we just all have to share. That's all. :)

Also, as a side note. Cystic Fibrosis is a disease, not a cancer.