Enlightening Conversation About Vertex Results

I thought it would be good to share a conversation I had today with a momma in the community that wasn't exactly pleased with my thoughts/comments/commentary on the Vertex announcement yesterday. Many of you may feel the same way...

"I am upset at the comments you made yesterday and the one today."

What specifically upset you? (see paragraph towards the end of this post for her response to this question)

"I am surprised that you would post things that could upset parents and other CF'ERS that have new hope. I understand that you have strong feelings and emotions about this combo and they are rightfully yours to have."

Right. And I'll always be honest, even if that means a few feathers are ruffled or I have some push back. And if posting something accurate and truthful on FB takes away the hope of some, I am sorry. With that said, I also included parts in my statement that would support the hope felt like "There are also people who blew the doors off of old PFT results!!!!!" or "I'm not saying that this is not good news." or "And I've always been consistent in saying that we do not need to see positive numbers for it to be a success. Remaining steady would be a BIG WIN for the CF community."

"However, the post yesterday was used by others to continue to be hateful to many in the community."

I cannot control what others do, and I will never censor myself based upon the possibility of others using my words out of context. That would not be authentic and I certainly won't put on a false facade - with what I say or what I don't.

"It may not be what you intended but it came across as you being mad that you were not able to get them at a time when you were younger."

Honestly, I have no clue how that came across to you in what I said. Do you think I would volunteer 1000's of hours a year in the CF community, if I were mad or bitter in any way at the opportunities afforded to the younger generation? Does that sound like a guy who thinks "life isn't fair"? I thought that was pretty clear when I said, "My biggest goal, and frankly why I got so involved with the CF community, is to make the life experience of the next generation of CFers, and the generation after that, better than my own. I don't want to see the CF patients of the 90's, 00's and 10's experience the same struggles as I did, a patient of the 80's."

"I have always respected you and seen you as such a godly man. (I even spoke of how you were such to my mother yesterday when everyone else was posting hateful comments)."

I'm certainly happy to hear that you think (or thought?) of me in that way. I do my best to show God's love through my approach and interaction with others, and unfortunately, I'm pretty sure I fail everyday. I can assure you if I served a God that wasn't gracious, I'd be screwed.

"To see your post after saying these things was disappointing. I hope that you see that your posts can be hurtful and truly upsetting to some."

Again, I am sorry if anyone was hurt by my commentary yesterday. This was in direct response to seeing statements like "I'll finally be able to breathe again" or "This may be my last hospital stay ever!!" or "We'll basically be cured." or "I can't wait to take those pills and for this to all be over!". We still have a long way to go. As it stands right now, the combo drugs will not support the statements above for the majority of people with CF. Less exacerbations? Probably. Less frequent hospital stays? Probably. Maintain lung function for longer? Probably. Cure? No. No hospital stays ever again? Probably not. No more treatments? Probably not.

"You have been given a wonderful platform to be such a strong witness for the Lord. I am always so thankful for your standing up for what is right and true."

I will continue to do so.

"Please know that this is not intended to be mean, just expressing how this has made one Christian woman feel."

Not a problem. I didn't think it was mean at all. It's hard to articulate feelings through an online medium such as FB. Expressing oneself, and having different feelings towards the same object/result/person is what personal expression is all about.

"I want this combo to give you decades more to work for the Lord and be with your family as much as I want it for my little girl."

Amen. I want the combo to work for your little girl with every fiber of my being. I would gladly give up any potential decades I have left to see that happen.

"On a side note, the lawsuit against Vertex being brought forward by the state of MA makes me question the validity considering their health care system. Just a thought."

The lawsuit is about people purchasing the stock based on "grossly overstated" results by said company and then losing 100's of thousands of dollars when that information was redacted...all the while executives of said company potentially made 100's of thousands of dollars by selling a portion of their shares before the results were corrected.

"I think the most upsetting part was saying I hate to be that guy type of comment. It kind of starts you off with a negative attitude. I did not see the comments from people that you mentioned. I do not think this is the end of treatments or anything for my daughter. But I do think it could keep her where she is until something better comes along which is more than I've had before. I know that most parents feel hope but know this is not a cure. For those of us who have a realistic view of the combo, the post was like someone coming into the first glimmer of hope we've had and screaming "just kidding!". Because we look up to you so much....I don't think you are bitter and that's why I even said something because it seemed out of character. I know you can't control what others do; however, if you had read what some were saying you might have held off on.that comment for a few days. It's ugly out there...and it just gets worse. It's hard for a parent of a child with CF that deals so much to see the negativity we've seen from this announcement. There are so many that have been so hateful about this not helping them that they don't want anyone else to have hope. It seems like such a miserable life for them and it's hard to understand. Your posts may not have upset me on another day but after dealing with all the negativity it was hard to see yours. I do not look at you differently now. I just wanted to let you know how it is from a mommy's view right now. There was such excitement for Kalydeco and we can't have the same joy that's all we want. I know you want that too. Thanks for responding! And I want address the other comments on the lost because I don't feel it's okay to stir the pot. But feel free to share this without my name." 

Totally understand where you are coming from [withheld]. I can see why this may have felt like I was piling on. It was never intended to be that way. I was trying to provide some history and context to a community that has felt let down many times in the past based on announcements like this only to discover it's not what they thought it would be. I'm sure I could have worded it better or, as you suggested, wait for the flames to die down a bit before throwing some gasoline on them.

On a side note: Did you know that I am currently on the drug and I think it helps? Maybe that would have been beneficial for me to say?

"I had no idea. I am thankful you are and are doing well on it! I hope it keeps you healthy and at home with your family more so than not! It would probably put out the fire if you said that ;)"

Haha, maybe I'll mention it then. I didn't see PFT improvement, but I think it gave me the ability to fight off infection quicker and I have remained stable for longer periods of time.

Questions from a Reader: No Silly Questions!

A couple questions sent my way that may help others out there...

Good night, Ronnie, 
I'm really very impressed by your great work about CF community. It's amazing and very inspiring. I wrote that I'm from Russia and now I live in Portugal. Also I've been in German clinic with my child and read a lot on CF forums in England. I spoke with many doctors and physiotherapists everywhere. But I have to say that your community, your actions, your positivity are great and unique.
I'm a little bit shy to ask some questions on the board as the answers seem to be very easy for most of the community here. But still I have some. And I decided to ask you. I'm sorry.
What is PFT? The level of oxigen in the lungs? That the doctors usually measure with a special clip on the finger?What is FEV?When do your doctors prescribe IV? I've read tons of information about that. But still i'm puzzled as all the cases are very individual and it seems that doctors have different point of view. Is there some "rule" or everything depends on the patient/symptoms?Do the doctors prescribe IV as prophylaxis without bacterias and symptoms?Do your doctors prescribe ANY antibiotics as prophylaxis without bacterias or/and symptoms? 

I understand that the questions are too silly and you might not have had any time to answer. I'm sorry.

First, thank you for your kind words. I'm honored to do anything I'm able to do for the community.

I'll answer your questions to the best of my ability...

PFT explained: http://ihavecfsowhat.blogspot.com/2012/03/awesome-pfts.html

The special clip on the finger measures oxygen saturation explained here: http://www.health.harvard.edu/diagnostic-tests/oxygen-saturation-test.htm

FEV1 is covered in the blog I provided above.

There is no rule when prescribing IVs or oral antibiotics. As you get into adulthood, much of it is a team decision. For me, I request IVs when I can no longer sustain my exercise at level consistent with me being healthy. When I was younger, we would base much of it on how I felt and how my PFTs were.

Yes, there are some doctors who are more proactive and will give antibiotics at the first sign of infections. Some will also prescribe with no sign of infection but other symptoms.

I'm on Azithromycin all of the time, it is an oral antibiotic. I also inhale either Cayston or Gentamicin each month and both are antibiotics.

Hope this helps! Let me know if you have any other questions. 

Question from Reader: Air Travel for Little One with CF

I thought I would share in case others out there had similar questions and/or concerns.

Reader:

Hello Ronnie!  I hope all is well!  I was wondering if you could give me some advice / tips for traveling with cystic fibrosis.  I'm about to take my 6 year old daughter to Denver and had a few questions...

1. Do you have to check The Vest?  Or can it be a carry on because it's medical equipment?

2. Do meds have to fit in a quart zip lock bag in the carry on, or ar they exempt from that?

3. Should I have her wear a face mask on the plane?  Cold and flu season and  all.

Ronnie:

1. No, you do not have to check the Vest. It can be carried on and will not count as a carry-on since it is medical equipment.

2. I have never put my meds in a zip lock bag and always carry them on. Just remember that if your meds are on a carry-on that also has non-medical items (clothes, etc), it WILL count as a carry-on.

3. I've never worn a face mask on a plane, but it is certainly dependent on the comfort level of the individual (or their parents).

Hope this helps!! Don't hesitate to ask more questions

Reader:

Have you ever been to Denver or somewhere similar?  We live in Iowa, met you at the CF Family day a couple years ago , and she's never left the state.  Do you have any issues with the thinner air?  I know each case is different, just wondering. Thank you so much!!!

Ronnie:

I didn't have any issues with altitude or thinner air until much later on in life. And when I say issues, they were minimal at most. I would bet that any change she would feel, you would feel also.

If you have a question that you would like my input on, please never hesitate to email me at ronnie@cysticlife.org!

Can You Really Gain Back Lung Function?

Question from reader

Question: 

So I know I've read things in your blog and have heard stories of CFers who had lung functions in their 30s and somehow managed to get it all the way back up to the 50s, 60s or even 70s. I'm pretty sure I even saw that you yourself were down in the 30s at one point. Do you have any idea how people (or yourself) have gained so much lung function back? How many breathing treatments a day does it take? How many hours of exercise? How much of a miracle?

Last year I was training for a marathon with my lung function in the high 60s, doing great. In July, I ended up being rushed into emergency surgery for a bowel obstruction and twisted intestine and a month afterward my lung function plummeted to the 20s and 30s. A year later I still can't recover. My lung function is now 27% and I'm going next month to meet a transplant team. It's super disappointing to have your feet knocked out from under you like that and even more disappointing when I've quadrupled my breathing treatments, tried exercising my butt off, and prayed every night for a miracle to no avail. I know there's a point when you're lungs can't repair themselves, but I also just can't believe that could happen in ONE month after surgery. I'm just wondering if there is something else I could be doing that others have done.

Answer: 

Thank you for taking the time to send me an email. Hopefully I can help you in some way :)

Generally, the people I see bounce back from lower lung function are from 3 camps - 

1) Never took care of themselves, almost died and got scared into changing

2) Took okay care of themselves, got in a routine that didn't change much, slower decline and then finally realized that if they continued doing what they were doing they were going to die 

3) Took great care of themselves, hit a rough patch or a big event, sharp decline and then bounced back after changing a few things up.

There is of course other situations out there, but these are the three main ones that I see. There are also some who experience one or more of these situation during the course of their lifetime (If your curious, I was mainly #2). 

To answer your direct questions, it's generally increased breathing treatments and a regular, intense and consistent exercise regiment that gives people the best chance to bounce back. For me, it took 18 months of running 6 days a week and doing 4 treatment sets a day (4 to 6 hours devoted to CF care) for me to go from a 50% to a 75% (I went into the hospital in the 20's and after a week in ICU I blew a 31%). I've been able to maintain my 75% by doing 3 to 4 treatment sets a day and exercising 6 days a week for the last 3 years. I devote 1 to 2 hours a day to some type of exercise and/or physical activity.

It's funny that you mention the bowel obstruction as the catalyst for you to see a sharp decrease in lung function. One of my largest declines in lung function also came after a bowel obstruction and removal of part of my small intestine. I've been able to get most of that lung function back, but it's been over a decade (obviously a lot happened in the last ten years, and much of it was me not taking great care of myself, or as some people call it, college.)

With all of that said, here's my advice: Put yourself in the best position to succeed in and every day. Treat each day as a new day to be your absolute best and better than the person you were the day before. Don't get discouraged by the numbers. It's much more important how you feel. If you don't think you can push anymore, push harder. A lot of gain can be made during the time of resistance. Sleep is just as important as activity. This doesn't mean that you sleep the day away, but it does mean that you may not be able to have any late nights for a while. I always found that my best mucus clearing workouts came first thing in the morning as the sun was rising! If your aren't seeing the results you want, either by the way you feel or by the numbers, change up your routine (medications, exercises, treatments, breathing techniques, etc). And probably most importantly, never accept a place you don't want to be and never stop pushing until you hit your ceiling. How do you know you have hit your ceiling? You never will, so that means you're in this for the long haul. The only way I'll know that I maxed out is after I'm dead; know what I'm saying?

If you do what I outlined above, and you still don't see the results you'd like, that's okay! You'll still have your head held high because you know you gave it your best shot. Remember, failure only occurs when you don't try. If you try, no matter the result, you're a winner.   

Please let me know if you have anymore questions!!

.............

If you have any questions that you'd like to send my way, please email me at ronnie@cysticlife.org!

Are You Honest With How You Feel??

Question from reader

I love when I get questions from the community and I always hope that my answer can help more than just the person asking the question. We all seem to undergo similar experiences in this life and I figure it can never hurt to share my own personal life experiences. 

Question:

I am meeting lots of new people who don't know anything about CF, or what that means in my daily life. I am very comfortable talking about what CF is and how it effects me, but I don't know how honest to be, and how best to explain it to people who don't know me well. To look at me you would never know (unless you saw me sick) that there is something "wrong" with me, so I find when I try to explain it, it's almost as if people don't quite believe me, or think I'm being overly dramatic about the time I put into breathing treatments, or how sick I can get. I don't know how to explain to my jogging buddy that I ran 2 miles with at 6:00am (feeling junky but not too bad) that by 9:00pm that same day, I was coughing up blood and had a fever of 104. I don't know what to say to a new mommy friend that I canceled a playdate with on Monday because I wasn't feeling well, that by Wednesday I had a PICC in and am now doing a full course of IV antibiotics. She, being a kind person, texted today (Friday) to see if I'm feeling all better, and the truthful answer is no. No I don't feel better, I actually feel worse. I can barely carry my baby up the stairs because it's that hard to breath. I'm exhausted because I've spent 2 whole days in and out of the hospital and hours in waiting rooms. But saying things like that freaks people out, and then they don't know what to say. However, pretending things are fine when they aren't isn't exactly a good option either for any kind of lasting friendship.

So how do you handle it? Where is the sweet spot between complete honesty, and pat answers like, "I'm getting better!"?

Answer:

The answer to your question is a tough one (as if you didn't already know that).

For me, I've rarely been totally flat out about how I feel with my friends and family. If I were to say "not great" or "pretty bad" I would always follow it up with "but I'll be alright" or "I just have to keep pushing". I've never wanted to put an unneeded burden on someone who really can't do anything to make me feel better. Sure they care, but they can't actually heal my lungs, do my medication for me or take my place in the hospital. I'm the only one that can do those things, so I've always internalized a lot of those different "I feel like crap" feelings.

In times that I feel helpless, I don't want to cause those around me to feel helpless as well.

Even with my now wife, I was very honest about what CF was, how it affects my body and what it could all potentially look like in the future, but I always quickly followed that up by, "but I'll work hard to make sure I'm always living the dream!".

Think about this too, will anybody around you ever really "get it" no matter what you say or how you say it? Probably not. The only people that could get it are others with CF or maybe other chronic illnesses. For me, I'd rather others in my life see me for my perseverance, positive attitude and faith in Jesus - than for something they will never be able to truly grasp.

Response: 

Thanks so much for getting back to me so quickly! That does help, and I think you are absolutely right. Thank you for affirming that for me, and for the encouragement to be positive. I am generally a very positive person, but this go 'round has been a little rough. I know you are right and so I will keep on, keeping on!

If, as a reader, you ever have a question for me, please never hesitate to send it along. You can send it via CysticLife, Facebook or my email address (ronnie@cysticlife.org)!

Increase from Exercise?

A question that I received lately that I know must be a common thought or question on the minds of others:

Ronnie, I wanted to ask what your pft range is or more specifically how much has it changed with exercise and how long did it (or has it taken) for you to see improvement?

I am currently at 30-35 percent and on 1-2L O2 24/7 I have lost about 10-15 percent over the past 7 years. In that time period the highest I was able to reach was 49.something, well almost 50%. I was hospitalized in June of 2009, my first in an 8 year period somewhere at 45%, however, can't give exact because records are stored since our move. After that hospitalization I cultured b cepacia, treated aggressively in Oct09. It took until August 2011 before cultures were clear. Anyhow, I've just started back at bicycling. I haven't participated in sports for 20 years (last softball season 1993). My main reason for having to quit was major hemoptysis. My husband and I have been in St. Augustine for a year and I truly believe that the salt-air is what keeps my lungs from episodes. 

So...after my last hospitalization in Oct. 2012,I was able to gain the 12% back I had lost in Sept., but it took 3 months. Since my last clinic, I've been riding every other day 30-40 minutes. A total of 9 weeks. So...the big test will be this coming Wed. clinic to see any change. Hoping and praying so...I want to get back to as close to 50% as possible. Lord willing with His help, I will.  Sorry so long and not sure how much you were familiar with my history, so I apologize if I had mentioned any of the details previously.  
Thanks, In Him, Laurie 

ps-if you'd rather not share I understand, just find it hard to find other Cfers that still have their original lungs! And if you did get a transplant, then I didn't catch it in your posts.

Thanks for sharing some background Laurie. (As a side note, Mandi's grammy lives in St. Augustine.)

As far as my PFT history, you can see much of it here: http://runsickboyrun.blogspot.com/2009/03/my-pft-history.html

I have not had a transplant.

It took about 18 months of working out everyday and doing 4 treatment sets a day no matter what for me to see "real" improvement. And by that I mean setting a new baseline and bringing my range closer together instead of further apart. That's continued to improve over the last 4 years.

I also have bouts of hemoptysis and it's one of those things that's always in the back of my mind. However, I'd rather "bleed out" trying to take care of myself, than slowly "commit suicide" by doing nothing (just how I think about it).

Your next PFTs may not show all of the hard work you've put in. The numbers will come, but they usually trail the effort. How are you feeling? Right now, that's what's most important. We always want to be in a position in which we can give our best. If we can't give our best, then we need to do what we need to do to get back into that position (for me, it's often a hospital stay).

Just keep your nose to the grindstone no matter the numbers. Our numbers can't dictate our effort, only we can, and should, do that.

Thanks, Ronnie for your insight and getting back to me. I am with you on not focusing on the numbers. I feel sooooo much better and I am just so grateful that I am able to get back into riding. I've had more junk, as of late, but it makes me wonder if the cycling is finally causing it to "rise". Which then I say...Good riddance!! Just to also add that the years from 2001 to 2008, I opted for "natural remedies" that kept my lungs clear or helped me through a particular illness. 

I also need to start in again with chiropractic care, it has always helped me to not be so tight from coughing and such. I've felt Cayston has helped me the most since my last hospitalization, but I am finding a slight ringing in my ears once I've done my neb this time around. Which is a bummer and something I will discuss with the Drs. this week at clinic.

I'm so happy to hear that your feeling soooooo good. That's what it's all about!! If you keep working hard, that won't change and your numbers will most likely catch up.

Turning Back the Clock

Great question from a reader:

I have a few questions for you, I remember reading on your blog about how you raised your PFTs. I was recently told I can't raise mine and nothing motivates me more than someone saying I can't. I obviously went through the stage every CF patients goes through and stops or halfway does their medicine and treatments and stops working out. So my question is what all did you do to raise yours? What kind of exercise? And did you eat differently?

I was told in 2009 that I should get comfortable with blowing a 50%. As with you, that motivated the heck out of me. It took two years of crazy hard work and dedication, but I was able to blow a 73%. Since then, I've remained steady and haven't had too many drastic dips. I've even been able to inch them up a little more.

For two years, this was my schedule -

Albuterol/Atrovent 4 times daily
Pulmozyme 2 times daily
7% 2 times daily
Inhaled antibiotic 2-3 times daily
Vest 4 times daily for AT LEAST 30 minutes at a time
Ran for 20 to 45 minutes 5 days a week
Took daily walks and bike rides
Lifted weights 3 to 4 times a week

I didn't really eat all that differently.

As you see though, it was a big commitment and obviously one that has paid off for me. When I blew that 73% in 2011, it was my highest number since 2003. So you can't tell me that we can't "turn back the clock"!!

Anyway, hope this helps and please let me know if you have any other questions 

Tough Answer

This is definitely a common concern with many moms and dads in the CF community. I actually wanted to post this exchange, because as you'll see, I don't have a great answer. Maybe you do? Please share your experience and maybe a helpful tip or two to this mama. I'll make sure she reads it!!

Hope you won’t find this question too personal to answer. 

We have a toddler with CF. The one thing that still bothers me about his diagnosis is the likelihood that he is infertile/won’t be able to have children without assistance. Given all the other stuff CF does to one’s body it just seems so unfair! And kind of random too actually. 

Just wondering how old you were when you realized that possibility and how you dealt with it? Was there something your parents said to you then that you appreciate now? I know we have a LONG time before this conversation comes up in our house but it's been on my mind for the past two years. Thank you! 

There is nothing ever too personal with me mama.

To be honest, I can't really remember an exact conversation about this topic as a kid/young adult. I have a feeling that how my mom handled ever situation that came our way is how she taught me to handle the subject of infertility - We'll figure something out. She had faith that things work out as they should and ultimately, we're not in control. Through it all, she has believed the "God is good". He would have been good whether or not I had children.

I was 19 when I got tested to see if I had sperm present. I of course had none.

I also don't think it's all that random of a "side effect" of CF. The only sure way to stop a genetic illness from "spreading" is to not bear/produce children.

Sorry I don't have a great answer for you. It was just never made a big deal because God was in control.

A Common Frustration

I wanted to post this email and my response as I'm sure plenty of you out there can relate.

I read your blog every time you post and I just love all you do for the CF community. I am not sure what exactly my point is in the email except that I guess I am a bit discouraged. I read your blog and see stories like today with your guest blogger and I am so happy for her but discouraged at the same time. I guess I don't understand how _____ can be so compliant, do all his meds, exercise, treatments, and yet we are still sitting at high 50's/low 60's on pfts with IV's and hospital now almost every 2-3 months! It just doesn't make sense to me. 

We are doing everything they say and pfts just go down. I know that colonized pseudo, apergillus, and yeast are all to blame in his lungs along with the worse sinus disease our doc has ever seen in a kid all that sinus disease is with many surgeries and he does daily nose sprays, ointments, and sinus rinses. But I see others say when their pfts go down its because of lack of compliance and exercise and then when they do those things they see improvement. But he is compliant!  

I guess I am just frustrated that we are literally doing EVERYTHING we can including now adding a nose nebulizer to dispense antibiotics straight up his nose and I am still watching my kids pfts go down. :( Again, not sure why I emailed except I am a bummed mom that just doesn't get CF ya know? Makes no sense to me. 

Thanks for your blog and all you do.

Your frustration and feeling of being discouraged is totally understandable. I'm sorry to hear that ____ is struggling so much at this time. Not cool for a kid his age to be held back!!

My response to you would be nothing earth shattering, but maybe something I say will help.

 - First, even though it feels like this has been going on forever, it could also just be a season in his life. We never know how long the season will last, but we do know that we have to keep our head up so we can recognize the next season when it comes.

 - When I was his age, I also had PFT numbers that dipped into the 50's. 

 - My best guess, which I'm sure is the best guess of the docs, is that's _____ sinuses are the big issue. Sounds like they are the driving force behind his uphill battle with his lungs. I'm happy to hear you added a sinus neb.

 - If what you're doing now isn't working, do something else. There is something out there that will help. At least, that's always my attitude. Could be a med. Could be a treatment. Could be exercise. Could be more hours. Could be prayer. Could be a combination of a bunch of things. Continue to tweak until you find the right combo. There will be road blocks and speed bumps along the way, but you have to keep driving.

 - All we are called to do is the possible. If we take care of the possible each and every day, there is no doubt that God will take care of the impossible.

 - Continue to be the strong mom that you are. CF is unpredictable. For _____, you must be that rock that is always predictable. He's always watching and takes his cues from you.

Again, nothing I said there is something you haven't heard before. Unfortunately, there isn't a magic pill. All we have is what we do each and every day that will determine our outcome. We can do everything right and still be disappointed in the outcome, but we must remember that our expectations aren't always in line with the Will of the Father. We all have our ceiling when it comes to our health, our only job is to make sure we strive to do everything to find out where that ceiling is.

I'll continue to pray for ____ and your family.

Precautions for MRSA

I had a clinic appointment yesterday in which I found out that in my last sputum sample, I cultured MRSA. This was a HUGE blow as MRSA in one of my biggest fears. I've also gone almost a year without a hospitalization (normally I'm doing at least twice a year visits) because I've been extremely compliant and my PFTs had been steadily increasing, so it was kind of a shock to hear that I got it, but not from the hospital. I did a quick CL search on MRSA and came across a question you responded to, and noticed that you have cultured MRSA yourself. Reading that was very calming, as it tells me that you can be healthy even if you have it.

I was hoping that you could answer a few questions based on your experience. You've mentioned before that your doctors said you didn't have to worry about avoiding babies or older individuals- is that true for brand newborns? Did you have to (or choose to) take any extra precautions when Mckenna was born? This is a major worry of mine because my cousins are all having babies that I would like to be able to hold and play with. I also hope to be able to try having children in the somewhat near future, and, although the situation is a tad different considering I would be the one carrying the pregnancy, it would be comforting to hear from a CF parent with MRSA about their experience. Another major concern that I have is that my brother Brian, who also has CF, doesn't have MRSA. I asked my doctor about precautions, as I will be seeing him at Christmas, and he said that we should avoid hugging, but that mask wearing isn't as crucial. Do you have any suggestions there? As much as it's difficult for me to accept that I can't hug my brother the few times I get to see him, I plan to do follow his suggestion, and I have considered wearing a mask anyway, just to be safe.

_____

Culturing anything new is never what we want to hear, but as you've seen me say already - not the end of the world. Of course everyone reacts differently to various bacterias, but I've been told that many US doctors don't believe that MRSA is a huge deal nor has a major impact on the lungs. They feel differently in Europe, but that's neither here nor there.

My lung function started to decline before I contracted MRSA due to "being responsible for my own health" and making poor decisions. I've had MRSA for about 10 years now, but through better adherence and an increase in treatments and exercise, I've been able to increase my lung function back to where it was 10 years ago. It's hard to know if MRSA has had any impact whether it be on lung function or "recovery time".

To answer your questions....
1. I asked as many doctors as I could and they all said the same thing - I did not have to worry about being around my newborn daughter. They said the only people that I would have to take precaution around would be those with no immune system (ie, chemo patients).
2. We did not take any special precautions with Mckenna.
3. A mask would only be useful if you are actively coughing. Otherwise, no contact and not coughing/talking in his direction should be enough.

I wouldn't worry a whole lot about it. Just continue kicking butt by taking care of yourself and discuss with your brother what would make him the most comfortable.

Making a change isn't easy.

I had a pretty general question for ya, and i saw how much u like questions. I am 19 years old and never been really compliant with my meds. i was never able to motivate myself long enough to keep doing them. i recently admitted myself into the hospital because ive felt worse than i ever had. I was told to watch your talk about cf and bag of tricks and i found you very insirational. in fact your the only person that has really gotten through to me and i realize i have to take my meds so i dont feel this crappy but im scared i may slip back into a depressive state about it.. I wanted to know how you kept doing your meds after your major scare, because you must have created a habit of not doing your meds consistantly n thats very hard to break out of. I to remember just beingon albuterol and chest pts when i was younger. times sure have changed just wish i could do it as easily.

............

I'm happy to hear that you're open to change your habits concerning your health and I hope that you start to feel better. With that said, yes, I've been in your shoes.

The biggest thing for me, was to stop being selfish. All of the choices I was making that in turn had my health declining was all about what I wanted to do and what made me happy. Yet, everyone around me, who cared for me, was very sad about how sick I was getting. They were sad to see me become the shell of the man I once was. They didn't like to see me struggle to breathe. They hated seeing CF "hold me back".

After I had my "come to Jesus" moment I realized that it wasn't CF holding me back, but that it was me. It was a culmination of all of the bad choices that I had been making. Simply put, I wasn't putting my health first. And as you can probably guess, life isn't as awesome with bad health.

When I made a change, I decided to make a schedule and stick to it no matter what. I also committed to doing "everything right" for as long as I was doing "everything wrong" which in my case was 8 years. I see too many people that commit to doing their treatments for a month or two, don't see the change they had hoped for, and then quit. I didn't think it was realistic to erase 8 years of bad decisions in two months, or even two years; I committed for the long haul.

This commitment meant exercising regularly, increasing my daily treatments and being pro-active about hospital stays.

All I can say, is that it's worth it. I feel great. I spend less time in the hospital. I look better. I breathe better. My mind is better.

Hope that helps a little bit. I'm always here for a chat or any question that you may have.

Ronnie

A Tough Choice

Hey Ronnie, I know we've never met, but I have a lot of respect for you and no one better to turn to who could give me some advice. My son [Name Withheld] is 18... the magic age where I no longer have control. His PFTs have been really low for a couple months now, 65% today. Docs think we've mucked around enough and really want to admit him for a clean out. He will not give his consent because it will mean missing the first game of basketball season. Basketball is [Name Withheld] love and this is his last season with this group of boys that have played together since 2nd grade. I know this is very important to him, but waiting to treat can mean perminent lung damage.... I know you know the routine. I was hoping you had some words of wisdom that would help me help him. He has always been a very compliant kid, and knows his disease quite well. Sometimes there is resistance, but he usually makes the best decision in the end. This time Im not so sure. What is it like to be an 18yr old boy with CF? As that boy once yourself, what would you have wanted from your mother... should I push, or let him makes a dicision that could impact his health for years to come? Thanks for listening

......

That's a tough one for sure...

I can only think to give you advice through a story. My freshman year of high school, I earned the spot of a starting running back during my pre-season workouts. I worked hard all summer and leading up to the season all I cared about was football. I then had a check-up about 8 days before the opening game - my lung function had dropped. I was given a "choice" by my docs and by my mom - go in now for a short stay, work my butt off and be ready to go for the second game of the season, or delay the stay, possibly get sicker and end up being forced to go in and miss 2, 3, 4 games or more. I say "choice" because my mom had a good way of getting me to choose what she ultimately wanted most of the time. I think it was a healthy balance of love and fear that I had for her that was the trick 

I chose to go into the hospital and I ended up missing the first game of the year. The coach stood behind the decision of of putting my health first and held my starting spot for me upon my return. I worked my tail off in the hospital and ended up making it a very short stay. My lung function shot up and I felt great. The dangling carrot of that second game was out there, and for me, it worked! I went on to play in every other game that year.

Looking back on my regrets from when I was that age, I never once think about that decision. What I do think about are the times that I didn't put my health first. I have the "what-ifs" and "if only I would haves" that will pop up from time to time.

On the continuum of time, the start of a basketball season is but a speck of dust. Admittedly, it looks like a mountain range to your son, and that is completely understandable. I couldn't fault him for either decision that he would make as it's much easier to be a Monday morning quarterback sitting here today 

I can tell you this, the people in my life now are VERY thankful for the times that I put my health first. When I compare that first football game of the season to my wife's kiss or my daughter's snuggle, it really puts into perspective how insignificant the game, the sport and that time really was.

At 18, you're not only making decisions that will affect your future, but the future and happiness of others.

So, to finally answer your question...As a son, I always expected my mom to be loving, respectful and honest. Kids are always more open and responsive when their parents show empathy and not "I'm the boss" in this situation. (With that said, may I remind you that you are in fact the boss if he still lives under your roof and you're paying the bills).

Hope that little story helped prepare you for your conversation with him and please let me know how it turns out. If there is any other question I can answer or anything else I can do, please let me know!!

Hemoptysis Episode and Children

Question:

I posted some questions on your blog a few weeks ago about how you manage having a baby with all the CF stuff. Thanks again for your response. : )

Here's another question: are you ever concerned about Mckenna's safety or your safety if you have a hemoptysis episode? For instance, if you were alone with her and you had a bad bleed? I have had periodic hemoptysis for nearly 20 years and had an embolization last year, but I still have random bleeds. For instance, last night I coughed up 1/4 cup, but I have no other symptoms of being sick right now. My husband and I have been discussing adopting a baby, but that does seem like a pretty big concern to me.

In addition, here's one more question. Do you feel safe traveling internationally if you are prone to bleeding? I have a lot of anxiety about it and don't really go anywhere too exotic for fear of an emergency. I saw that you went to Singapore, so I was wondering how you made that work.

If you could shed some light on how you deal with this scary and frustrating CF problem, I would really appreciate it. : )

.....

Answer:

I hope your Christmas season was a good one and that you are enjoying the new year thus far :)

To be honest, I've never been very concerned about having a massive hemoptysis and being alone with Mckenna. If it happens, it happens. There is always a neighbor or 911.

Bleeding on a flight is always a slight concern, but I do my best to avoid sudden movements as to control the already increased pressure in my lungs and I sit in the aisle. I used to prefer the window, but now I like access to the bathroom if and when I have a bleed. 

Most places have decent enough healthcare if you were to have an emergency. Singapore has top notch healthcare. I've also however spent a lot of time in China and they have no clue what CF is :)

Here's probably the best way to answer your question - I don't let my health dictate what I do or do not do. If I want to do something, I put myself in the best position to succeed, and I do it. I'm not going to let the chance of having a massive hemoptysis stop me from spending time alone with my daughter, or pursuing a family in the first place. I won't let the possibility of a bleed prevent me from seeing some of the most beautiful places in the world.

As long as I'm putting myself in the best position to succeed and working my butt off every day to be healthy, I'll reward myself with life!! :)

Hope that helps.

Vest too big and ineffective?

Question: Yet another question. This one is about the vest. They ordered mine while I was pregnant. But now I'm not sure if the fit is right & if it's working as well because of that. It poofs up quite a bit even with the straps pulled as tight as possible. I personally don't feel as if it's doing too much good. Just wondering if it truly does make a difference. 

Response: Yeah, it may be too big. But, I've found that I have to mess with it quite a bit for it to feel "just right". Be sure that you're sitting straight up and that nothing is putting pressure on the vest from any angle. Many people sit back in a chair or on the couch and that lessens the effectiveness. Mess with the shoulder straps, the clip straps, everything.

Be sure to mess with the pressure as well as the frequency. It should always be going up, or down, but never stay the same. You can find some good recommendations on CysticLife.org as far as exact settings go. And finally, actively do you're vest. Don't sit there mindlessly on the computer with a neb in your mouth taking shallow breaths repeatedly at the same rate (which I can be very guilty of). Breathe in deep, exhale fast. Breathe in slow, exhale slow. Huff cough. Take time to really cough your brains out.

There is certainly nothing magical about a vest. If we treat it as a piece of clothing that happens to shake, we'll get nothing out of it. We need to treat it as a workout that requires participation.

Question from Reader: Relationship Roles and Boundaries

Question: 

Hi Ronnie! I didn't know where to ask this question and thought it would be one to hear your (and other CFer's) thoughts on! 

As we all know, having CF is not just an illness or an excuse to eat whatever we want, but also a responsibility. This responsibility is ours and our families burden and requires a lot of support. I'm having trouble with how much this responsibility spills onto other. Like friends, and let's be honest, mostly BOYfriends. I don't feel it's okay to place such a burden on someone you've just met, so I normally wait a while. But since being at college it's hard to find the balance between what I tell them (him) and what I don't. When it comes down to it, if you're spending a lot of time with me you're spending a lot of time with me at the doctor, doing my therapies and reminding me to take my enzymes. I'm wondering how you find the balance between what they should know/do for you/ worry about and how to stop it if you think they're over stepping? 

Answer:

Here's my rule-of-thumb: I only hide stuff I am embarrassed about. Since I've never been embarrassed about CF, I was always very open about it with anyone I thought I would spend more than a few days with.

As far as other people, "loving" or accepting this life, I've been there. What I always said to myself was "If I really loved them, I wouldn't bring them into this life with me". I mean, I love and embrace CF, but that doesn't mean they have to.

That changed when I met me wife. She told me that she fell in-love with me BECAUSE of CF not in spite of it. She's convinced it played a huge role in shaping me into the man that she fell in-love with. Frankly, I can't disagree with her on that.

To answer your questions...

1.  What should they know? If it's a serious relationship, everything. Not telling someone we care about everything about something that's such an integral part of our lives would be akin to lying. Just think about it this way, if the shoe were on the other foot, would you want him to leave anything out?

2.  What do I want someone who I'm in a relationship to do for me? Well, the answer might depend on wether you're a guy or a girl, but I know for me, I don't mind being "taken care of". Maybe my mom did too good of a job with me growing up or something, because I don't mind at all when my wife steps up to the plate and does "health stuff" for me. Mandi is great about putting my health #1 and reminding me that they need me to be healthy in order to best serve my family.

3.  We will never be in a place in which we're able to control what anybody else worries about. Mandi may say she's not worried about my health or about the future, but how do I really know? Here's what I do know however, the only way I can control any amount of worry is by showing her that I'm doing anything and everything to put myself in the best position to succeed with my health.

4.  How do we tell loved ones they're overstepping their role? As honestly, lovingly and straight-forward as possible :) I've found that with any sort of communication in a relationship that with those three things guiding your words, you can't go wrong!

Hope this helps a bit.

Question from a Reader: What can I say?

Another great question and I'm sure far too often occurring situation in the CF community. I can understand his frustration and confusion during these events and with the "CF Life" in general:

My problem is my son, who is now 10, and has had a lot of horrible experiences with lab draws but of course needs to get them at least once a year if not more. Beyond being a hard stick he gets anxious so the more time drawn out looking for veins the more he fights. Our regular lab flat out refused to draw his blood work today because 4 technicians couldn't find a vein and when one went in to attempt to do a draw on what he though was a vein [Child's name] refused to hold still. The day pretty much left both of us in frustrating tears. 

He argues the point of "Why do I need to have all these sticks if I don't feel sick?" I don't know whether to say "CF is doing more damage than you realize and watching it now may prevent major problems later" or "While we are responsible for you , you'll get the tests we feel will benefit your health". Both sound harsh but I don't know where else to go with it.

"While we are responsible for you , you'll get the tests we feel will benefit your health". That was pretty much my mom's response while growing up. My roof, my rules and under my roof, your health is number one. We can choose to be proactive, reactive or non active. And under my roof, we're proactive. Also, there is nothing wrong with rewarding good behavior and not rewarding behavior that isn't acceptable.

[Child's name], when you hold still and are brave for the blood draws today, you can pick the restaurant that we go to afterwards.

With that said, next time 3 technicians can't find a vein, you can feel free to "protect" your son and step in and say "I'm sorry, but 3 different techs not being successful is good for today. We'll reschedule and hopefully have better luck next time"

Be sure he is drinking a TON of water on the days he's going to get a stick to really pump up those veins.

And last, nobody walking the earth with CF was perfect as a teen/pre-teen. We'll all make mistakes. Make stupid decisions, etc. What we love though, without saying it, is when our parents put our health number one and love up on us 24 hours a day.

There is no greater love in my opinion than a CF mom holding the line and doing what's best for her CF child and other children of course!!

Do you cough in public?

I was so excited to answer this question as I know plenty of other cysters and fibros struggle with this issue - coughing in public. I hope this answer gives you the confidence to cough like there is no tomorrow...even if you makes others uncomfortable.

I have a question for the CF "expert"  

So i've got that LOVELY CF cough goin' on right now. Luckily right now it's mostly dry so I don't have too much mucus coming up. But you know the drill, a coughing spell every few minutes or so. How should i handle going to dance? Should I skip out until my cough hopefully calms down a little bit? Because I don't want CF to hold me back any, but i also know the realistics of it. I mean I wouldn't want to dance with someone who kept coughing, even if I was told it wasn't contagious. Because a few of the classes we rotate with all the partners which means gettin' all up and close and personal with everybody multiple times through. I can exercise here at the house for the time being, but i also love dance and miss the people (i've been gone doing a CF study for the past 2 weeks so I haven't been able to see them or go dancing. Sadness!!) . What's the smart thing to do? I want to do what i love, but I also want to be smart and considerate of others.

Alright, first things first, I'm humbled you'd even care what I think about this. Second, I'm no expert, and I actually recommend doing the exact opposite of anything I suggests to be on the safe side :) Third, I'd love to share my thoughts with you!

Let's establish this - coughing is a good thing. Yeah it's annoying (both to us and others), but it's playing a vital role in keeping our lungs clear. I myself am a cougher whether I'm at 30% lung function or 100% lung function. In fact, I give much credit to my cough for keeping my lungs relatively clear my entire life. Sometimes I bring up junk and sometimes I don't. In fact, I've coughed about 10 times since writing this response and have only brought up the goobery goo on two of those coughs.

Coughing can indicate that you are experiencing an exacerbation or outside sickness, but it can also be an indication of asthma, allergies or nothing.

So now that you know coughing is completely okay (at least in my eyes), what do you do about it in public?

I think you know my answer - I cough, and I don't care where I am. I'm at the gym 6 days a week. 3 of the days I do partner work in a fitness class and three of those days I'm in a small room with others doing a core class. Needless to say, I'm coughing near, on and around all of them.

Apparently I don't have a lot of pictures of me coughing.
This was the only one I could find off-hand and it's very old!

If I end up with a new partner, and I've already started coughing before the workout even began, I usually go with one of three phrases that all start with "Don't worry, I'm not going to get you sick....

a) I have Cystic Fibrosis and part of it is that I cough a lot, especially during workouts.

b) I have asthma and my lungs are extra tight today.

c) I'm just a cougher.

One of those three answers always seems to put my partner at ease and we can move on with the class.

When it comes to coughing in public, frankly, I'm not very considerate - especially if I'm doing something beneficial for my health. The truth of the matter is that clearing out my lungs and doing something that in the long run is beneficial to me and my family is WAY more important than the thoughts, stares and feelings of others.

It reminds me of a blog I wrote a while back about being selfish. There are times in this CF life that you HAVE TO be selfish. The funny thing is that often, by being selfish, we are doing one of the most selfless things that we could ever do. The blog is here if you're interested: http://runsickboyrun.blogspot.com/2012/07/are-you-selfish-i-hope-so.html

So what do I think the smart thing to do is? Dance and cough your little booty off!!

Sometimes life calls for us being comfortable with making others uncomfortable.

Hope this helps :)