CF Community Interview - Tara Haddix (Cyster)

Cystic fibrosis is a disease that can effects people in many different ways and there are many different outcomes.  Sometimes when I see young children really struggling health wise with CF it really causes a lot of sadness.  Why would a child need a life saving double lung transplant and they are not even a teenager?  

I think many of us have heard about Sarah Murnaghan.  She is the very young cyster who needed a double lung transplant but the family pushed very hard to get her on the adult donor list.  I know that fight has really been beneficial to Sarah and some young children so far.  But I had no idea that there were was someone much like Sarah Murnaghan years and years before.  

In this interview you will see how at 11 years old Tara overcame one of the greatest challenges for someone her age.  Tara is truly an inspiration to all of us even outside the world of CF! 

NAME: Tara (Bell) Haddix 

AGE: 33

Social Media links 

https://www.facebook.com/Tara2lungtx

http://www.caringbridge.org/visit/tarahaddix

Please share your cystic fibrosis diagnosis (Include age, how you felt at the time of diagnosis, how was life before and after diagnosis)?

I was 6 months old when I was diagnosed.  I started wheezing pretty soon after my mom took me home from the hospital.  She kept telling the doctor my baby is wheezing.  He would not believe her and told her she was just a new mother and nothing was wrong with me.  Finally at my 6 month check up I began wheezing in the office.  The doctor had me rushed to the local ER because he thought I had water on my lungs.  Being born in 1980 many doctors had never heard of CF.  Finally after several tests one doctor suggested a sweat test.  And I was diagnosed with CF.  I was then transferred to RB&C where my parents began learning what cf was and how to do all my treatments.

What has been the biggest challenge for you dealing with cystic fibrosis?

Never being “normal”.  I hated that trying to run and keep up with my friends made me cough and short of breath.  I truly hate when I am sick and can’t keep up with life.

Please share with us any triumphs you have accomplished because of cystic fibrosis?

I would say it is all the CF friends I have.  Without CF in my life I would never have met all the amazing people I know with CF.  Although I have lost some of those friends I feel it has been a privilege to have known those people and my life is truly better off because they were in my life even if only for a short time.

Now that we know you have had a lung transplant, how did you view the world before transplant and after transplant?

Before transplant I felt that one day I would just wake up and feel better.   While waiting on the list I kept thinking I wouldn’t need new lungs because I would get better (the mind of an 11 year old) but after transplant I realized what I thought was “feeling better” is how I now feel when I am sick.  So a normal sick day for me now was a very good day before new lungs.  I remember being scared to death to take that first breath without oxygen.  The ICU nurses actually had to trick me off my O2. But that first breath was amazing!!

Tara at her organ donor's grave site! Paying her respects! 

 What advice would you give anyone who is on the lung transplant list?

It isn’t easy.  It takes a lot of fight and a lot of determination.  And then things can still go wrong.  I was in the hospital for 8 weeks after receiving my new lungs.  My chest had to heal open in the center due to a staph infection that spilled out of my old lungs.  I had many issues with scar tissue and because of that I had more bronchs, along with laser surgery to open my airways up, than I can count in the first 9 months post transplant.  I crashed on a CT table and looked down at them trying to bring me back.  I fought for life every step of the way and it was worth every step.   It is not for the faint of heart and if you aren’t willing to do a lot of work yourself it really isn’t something you should look into.

As you mentioned above you not only had a double lung transplant but also a kidney transplant.  Was the kidney transplant process any easier since you went through a lung transplant already?

In my opinion yes.  Does that mean a kidney transplant is easy.  By no means.  But compared to a lung transplant yes.  I actually told someone that after a lung transplant a kidney is a walk in the park.  So she was expecting her kidney transplant to be easy with no issues at all (she also had already had a lung transplant).  She actually posted that the people who told me that getting a kidney is a walk in the park lied to me.  I commented back and said no that is not what I said.  I never said the surgery or recover was easy.  I said compared to a lung transplant it is.  I would take a kidney over another set of lungs any day lol.

You seem to have overcome a lot of odds in your life! What is something that helps get you through the really challenging times?

My faith.  God has done miracles in my life.  And I know He will never give me more than I can handle.  Sometimes I ask Him ok God how strong do you think I am?!  But He has always provided and always helped me overcome the odds.  Plus my family is a big help.  They believe in me. 

Were there things you wished your parents did differently as you grew up with cystic fibrosis  (Example: more freedom to be in charge of your treatment, speaking at doctor visits,  life pre diagnosis/post diagnosis, not allowing you to do certain things because of CF)?

My parents never held me back. And I have always been very vocal with all my doctors.  I went to school after getting my lungs, and was in dance classes and on the high school dance team.  Sometimes I think that is why some people do so poorly, they live in a bubble.  You need to live in the world.  Now I am not saying find a bunch of people with the flu and go hang out with them but don’t live inside all winter because there are too many “germs” out there. I work in the public at a store all year round.  I have only had the flu once in my life and it was in 2012 the year they were giving the shot for the wrong one.  And I actually caught it from a family member not at work.  My parents really did good and I am so thankful for how they did raise me.  They also didn’t keep secrets about my health.  I knew up front that I might die going through the transplant surgery.  They didn’t sugar coat it and I am glad.

Tara with her oxygen tube.  Her sister is in the basket.  She passed away at 9 months old from CF! 

You have seen a lot of things as I call you a CF veteran.  .  Can you describe what it was like in the early days of CF and where we are now and where you think CF is going?

I only had CF lungs for 11 years of my life.  And in that 11 years I was pretty much always sick.  I know most people don’t get so sick so young.  Up until a few years ago I felt so disconnected from the CF world.  All the new drugs won’t help me.  I have never even used a vest because they came out after I got my lungs.   And although I know a lot of advances have happened I still see CF people dying young.  Kids dying, young adults and so on.  Do I think a cure for everyone with CF is right around the corner?  No.  I don’t think we will ever have a full cure that will help everyone.  There are great medications out there that will help the younger generation live longer.  And I pray I am wrong.  But I am still seeing a lot of people with CF dying way too young.  CF is too complicated to have a cure for everyone like so many people want.  I guess that is just what I feel in my gut.

This question floats around a lot.  If you could be born without cystic fibrosis would you? But what has CF taught you?

No I wouldn’t.  I truly believe CF has made me the person I am today.  And I would rather it be me than someone else.  Like most of us.  CF has taught me to not take life for granted.  I am not promised tomorrow, but I absolutely HATE the saying that anyone could walk outside tomorrow and get hit by a bus.  I heard someone else say this once as a response to that statement and I love it!  Their response was, Yeah but when you have CF the bus is chasing you and trying to kill you. I really wished I remembered who so I could quote them.

I have found that there are a good amount of CFers who are worried to tell their boyfriend/girlfriend they have cystic fibrosis.  As a  CFer  who is married, what advice would you give to a CFer who is afraid to disclose they have cystic fibrosis?

I have never kept my health problems a secret.  I mean I don’t tell every person on the street I meet but I also don’t try to hide it from people.  I also don’t start by meeting a new person and saying by the way I have CF, I had a double lung transplant and a kidney transplant and I have diabetes and so on.  I just talk about it.  And sometimes I just start with I have a lot of health problems and as it comes up people learn what they are. I don’t remember how Kevin, my husband, found out.  I don’t remember sitting down one day and giving him a speech but we were friends before we dated and he knew about me when we started dating.  He just learned through our friendship because I talk about it very causally. It doesn’t bother me at all.

Tara and her husband!! 

I talk with a lot of newly diagnosed parents to CFers. Many of these parents feel the world is crashing down on them when they hear the two letters CF.  I want to know what you would tell a newly diagnosed parent to a CFer.

I can understand being scared.  It is scary.  But they also need to realize their kid is still a kid.  People with CF are normal people we just can’t breathe lol.  We aren’t as fragile as some people make us out to be.  I am not saying we can do everything without our health getting in the way. But we can still do a lot.  Just be cautious when your child is sick and make sure they take their meds and do their treatments.  You can do everything right and the kid can still get sick.  Or you can do everything wrong and the kid still be healthy.  Because that is what CF is.

What advice would you give anyone who is battling cystic fibrosis today?

Don’t give up.  Fight till the end.  Which is hard.  But all of us CFers are a lot stronger than many of us give ourselves credit for.  As long as we fight to the end CF doesn’t win.  And in my book that is all that matters.  Not letting CF win.

Is there anything else you would like to share with us when it comes to your battles with CF?

Sometimes I feel really guilty for doing so well.  I have lost more friends to CF than I can count and I have also lost a sister to CF.  I have no clue why I have been so blessed with my CF and I have had to watch friends die.  Then I think, maybe I am here for those who are ready to give up.  They hear what all I have been through and realize if I can do it so can they.  Or for those who have no hope and they think their lungs won’t come in time. Or that they will get lungs and only live a couple years.  Then they hear my story and realize there is hope of living twenty plus years post transplant.  My hope for every person with CF that I meet will live longer than me and do better than I ever have.

John's Notes:

I met Tara on facebook and when she started talking about her transplant with me I almost fell out of my chair.  She said she was 22 years post transplant!!! I believed it but was still in shock.  I also didn't realize that she had a life saving kidney transplant from her mother.  Talk about real love for your daughter.  

I think Tara is destined and was put on this planet to share with us her life story.  I can tell you I learned more in this interview with Tara than I think I could have in any 5 minute conversation with her.  

I can tell CF doesn't define Tara's life.  Her life and how she views things is truly shaped from getting that second chance.  All I can say is Tara you are more than an INSPIRATION to all of us.  I wish you much more success in your future endeavors!!

Alayna 18 Month Post

Well here we are...yesterday made it 18 months in the book for you Alayna.  It’s crazy to start to think that 18 months ago you gave us the best gift when you took your first ever breath.  Life from then on has been really rewarding and also very different.  Just the other day while watching you play mommy said to me isn’t it just amazing to have you in our life.  I said it is beyond amazing!!  One day you will hear about the true miracle you are and how hard we worked to have you in our life.

So what has happened this past month in your life?  Well let’s start off with daddy’s blunder and something I will probably hear the rest of my life.  We took you to the store and I thought you made a dirty diaper.  I carried you to the car and on the way out of the store you started crying because mommy was still in the store.  As we made our way to the doorway you stuck out your arm and grabbed a hold of the side of door.  I didn’t realize this and just kept carrying you on out. Then you really started to cry.  You let go of the door and we made it to the car.  I checked your diaper and low and behold the diaper was completely clean.  You didn’t stop crying until we got back into the store. 

On our way back home you slept in the car seat and then continued your nap in our bed.   You awoke from your nap and you still were not yourself.  Eating and even playing with a tv remote or cell phone was something that seemed awful to you.  We started to notice you didn’t want to use your arm.  I then told mommy what happened in the door jam.  I was in big trouble for not telling what happened to your arm.  I didn’t think twice about why you were crying.  I just thought you were upset because mommy was in the store.  I told mommy and she said oh great maybe it’s broken.  Well that did it and off you went to the emergency room. 

The doctor came into the room and asked what happened and felt your arm.  He popped your elbow back in place.  Yes, your elbow came out of place and it’s very common.  I do not recommend picking any child up by their arms as the doctor said this is how many elbows and shoulders are displaced!  I guess this is something that you can throw in my face one day! : )

So in other news, we took you a little early to your 18 month checkup.  You continue to amaze us with how big you are.  Sometimes when we look at you we think you are well over the age of 2.  We just cannot believe how tall you are.  You now are 34 inches tall which puts you in the 97^th percentile.  Your weight is 24 pounds and 7 ounces which puts you in the 50^th percentile.   I guess we still have a giant on our hands.  You are well over half the height of your mommy.  Just remember though no matter how tall you get we are still the boss!!

You seem to be doing a lot better with eating solid foods and drinking some liquids.  If we do give you meat like steak or chicken you really do not care to eat it.  You will put it in your mouth then spit it out once you realize the texture is not to your liking.  Yes, you still eat a lot of baby food and it has been a true chore lately as you do not want to sit in your highchair.  You would rather sit in our adult chairs or stand I should say.  We still are feeding you but you do eat with your hands and pretend to drink from a cup or water bottle.  Speaking of water you still have trouble swallowing water.  I think you are just still trying to figure out the whole swallowing technique!

Alayna you have a personality that is like no other.  I don’t know where you get this from!  You will now copy some things you see on t.v.  Your vocabulary is really strong.  You repeat words we say.  In fact, you can sing along to the entire Barney I LOVE YOU song.  Twinkle twinkle little star you can complete a few of the actual words before they are even sung.  But by far your favorite things to say is Hi Daddy Hi Mommy!!! You say those one liners 50 times a day. That truly is the greatest thing I have ever heard you say.  There is nothing like hearing you say HI Daddy when I am at work or bye bye.   Anyway,  I think we are going to have our hands full.  Oh wait we already do!

The next time I say 18 you will be 18 years old.  Just to think that is crazy!! Well its almost time for Easter this coming Sunday.  I am sure you will be spoiled beyond belief : )

WE LOVE YOU Alayna! 

Liver Enzymes, CF Clinic, & The Secret to Lung Function Improvement

 Today, we received the results of Maria’s latest liver enzymes.   Some of you may recall that Maria has been battling with elevated liver enzymes ever since the birth of Alayna.   The doctors really do not know what why.  They did basically all the liver tests they could and have no real reason why.  So she continues to get blood work done every 3 months to see where the levels are at.  Her last quarterly blood work showed the liver enzymes were slightly above the normal range which was actually good because months and months ago they were highly elevated.

 

Maria had her blood test late last week and today the office called and said the numbers were all in the normal range.  This is some fantastic news as we have been seeing her liver enzymes returning to normal.  In my opinion, I think they are returning to normal because Maria has been eating more.  Yes, eating more.  I should say more often.  I can tell you from first hand experience raising a child is beyond tough.  So there have been times were Maria wasn’t able to get those 3 meals in a day.  Over the past 6 months she has been able to eat more often which I think we both agree has improved the liver enzymes.  She will go back in 3 months for another blood test to make sure the liver enzymes have remained in the normal level.  All I can say is WE are all very happy with these results!! It has eased the stress on Maria as she was worried why we never had answers why the enzymes were elevated.   I LOVE my wife!!

Today, was also my quarterly CF clinic.  I was actually full of emotions today.  I was excited, anxious, nervous all at the same time.  Why is that?  Well from my last clinic in Jamuary my PFT took a dip.  I blew a FEV1 of a 66 back in January.  I knew something wasn’t right then.  I felt tired and was coughing a little more.  So I pushed my doctor to try some antibiotics to see if that would help.  I cannot emphasize this enough but it is so important to have a doctor who listens to your concerns and is willing to come up with a game plan that all parties can agree upon.  Remember “You are your own voice!!” 

So I did a month of inhaled meropenum and doxcycline.  Yes, you read that right inhaled mero.  I know it is used IV but my dr. said it will work if we inhale it.  I did it before a year ago and it killed my Psuedo.  I finished the meds up middle of March.  I felt really good after the meds. 

When I was doing  these meds, a very good cyster friend of mine Tammy who could actually be my mom.  I call her my CF mom.  Tammy when you read this be proud you are old enough to be my mom and kicking CF butt.  Sorry for the sidetrack, but I will tell you Tammy is truly a wonderful lady and a rockstar CFer and I am so proud to call my friend.   We were exchanging messages and she asked me about hypertonic saline and how many vials I use etc.   As many of you all know I LOVE hypertonic saline more than I probably could express.  I know I am nuts but I call it my Kalydeco.  HTS is my Kalydeco until I get Kalydeco one day if that makes any sense.  I responded to Tammy’s question with I use one vial of 7% HTS in the morning and one vial 3% HTS in the evening.  I used the lower dose at night because previously when I tried to use 7% I coughed through the night.  Tammy told me she uses two vials in morning and evening of 7% HTS.  I thought to myself hmm maybe I should try that to see if I get more production in my airway clearance and more mucus out. 

I trust Tammy, but I wanted to do a little research and I was honestly shocked at what I found.  It looks like Tammy is on to something as there is research that points out the optimial benefit from HTS is achieved with 84 minutes of HTS 7% with an administration of 10 mls.  Here is the proof.

Administration of 10 mls of 7% hypertonic saline twice daily increased FEV1 by 12% with a delivery time up to 84 minutes daily (Ballmann & von der Hardt, 2002; Wark et al, 2005). Administration of 4-5 mls twice daily increased FEV1 by 3% with a delivery time of about 40 minutes daily (Suri et al, 2002; Elkins et al, 2006). Treatment with hypertonic saline is a significant additional burden for the patient. Faster delivery with the new nebuliser systems is being studied (Elkins & Bye, 2006).

You can read more here:

http://www.cfmedicine.com/htmldocs/CFText/hypertonicsaline.htm

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2083400/

So with this information I said why not give it a try.  I also notice that one vial is not enough to make my now 25 minutes of vesting.  I usually run out at 15 minutes.  So all this adding a second vial made sense to me.   If I coughed through the night I would stop doing two vials of 7% HTS in the evening. 

To my surprise, I was able to handle two vials which is a total of 8 mls of 7% HTS in the morning and evening.  What did I notice after I started doing this?  I felt like I was moving mountains of mucus in my lungs.  I was coughing junk out left and right.  By the time my 25 min vest session was over my lungs felt so clean.  Honestly, it was the cleanest they have ever felt.  I never once coughed through the night or coughed up any blood. 

I kept up with my compliance and adding an extra vial of HTS every treatment.  It was finally time to see if all this extra salt would impact my PFT.   That day was yesterday.  I got a clean bill of health from my doctor.  The lungs sound good.  I don’t have any real complaints about my health.  It was then off to the PFT room.  I mentally prepared myself to just hit the ball when I got in the PFT chamber.  Whatever happened after I hit the ball was going to happen.   I thought about my FEV1 66 last time and I knew that it would be better this visit because my lungs felt so good. 

Maria came back for the PFT test and she screamed!! My doctor could even hear Maria from another room and we had the door closed.  I got credit for a FEV1 of 74!! What? ?????? I havent’ seen a FEV1 of 74 since August of 2010.  That is 4 years since I have been that high!  So going from FEV1 66 to FEV1 74 was incredible.  A big 8 point change and a 12% increase from last visit is hitting a HOMERUN in my book.   My doctor asked what is all the screaming about.  I told him that I hit numbers I haven’t seen in 4 years.  He couldn’t believe me! Yes 74 baby!  He said well John looks like what you are doing is really working for YOU!!  I told him maybe he needs to prescribe an extra vial of 7% HTS for all treatments.  He said he would definitely think about it.  He echoed that this saline is like my Kalydeco as well!

I knew my numbers improved because of my saline.  It is really awesome seeing improvements from things that you are changing.  I also think that my dedication to my compliance is really helping my lungs feel great.  So that is the secret that works for ME adding an extra vial of 7% saline to my morning and evening treatment.  It may not work for YOU but is it worth a try?   You have to make that determination.  The only way you will know is if you try it. 

***BEFORE YOU MAKE ANY CHANGES TO YOUR MEDICAL CARE ASK YOUR DOCTOR!!! THE OPINIONS THAT ARE USED HERE COME FROM ME AND I AM NOT A DOCTOR.  PLEASE CONSULT YOUR DOCTOR!!!!*** 

CF Community Interview - Megan Lee Mobley (Cyster)

In this interview,  you will read how Megan still would choose to have CF even after going through her double lung transplant.  Just by reading Megan's answers I can tell you that CF does NOT define who this woman is!!  She has some great advice for both sides of a relationship when you start dating a CFer.  I love the perspective that she puts forth in that answer. 

I truly wish there were more people like Megan who reach out to other newly diagnosed parents.   Megan states in her interview she has made a true friend in a parent to a CFer.  I LOVE this.  As many of you know I really enjoy talking with parents to CFers.  I guarantee that when you talk with a parent to a CFer they are so rewarded from that conversation.  I can bet that my parents probably wish they had a sounding board to go to minus the doctors who actually has been through this disease.  The time is NOW to reach out to parents with CFers!!! 

Lastly, I think Megan's advice for all of us who are battling CF are words we should all make a part of our daily life. One thing that jumps out at me is where she makes a statement that MENTALITY is key in fighting this disease! I couldn't agree more.   

Here is Megan's interview and story! 



Name: Megan Lee Mobley (cyster & double lung transplant warrior)

Age: 26

Please share your cystic fibrosis diagnosis (Incude age, how you felt at the time of diagnosis, how was life before and after diagnosis)?

When I was diagnosed I was 5 years old, and what did CF mean to a 5 year old? All I remember was seeing my mother and father cry and the drs running a lot of sweat tests on me.  My life before diagnosis I was always at urgent care, after being diagnosed it was breathing treatments, hospital stays and home IVs.  I didn’t really feel any different, just that I didn’t understand why I was the one out of all my friends that had to go through it.

What has been the biggest challenge for you dealing with cystic fibrosis?

The biggest challenge was staying on top of  my treatments when I became older, because becoming older that meant getting a job, juggling medications, and hospital stays. I lost a job over it.  Also breathing became a challenge around the age of 19, which now I am almost 27.

Please share with us any triumphs you have accomplished because of cystic fibrosis?

I always barrel raced even as a young child, I had to stop for a while until after my transplant but im right back at it.  Getting a lung transplant is a huge triumph, I was also awarded hero of hope through genetic J

Now that we know you have had a lung transplant, how did you view the world before transplant and after transplant?

My quality of life was not good 4 years before my transplant with a lung function of only 28%  I viewed the world the same as I do now, I just have more faith, and more faith in the doctors that treat me, I always said I never wanted a lung transplant, but when you are knocking on deaths door, you re think a lot of things, you do it for the people you love more so than yourself.

What advice would you give anyone who is on the lung transplant list?

Keep your faith, and stay strong, your body can be so weak and they look at you and think you will not make it but its your will, it has to be strong, you have to fight and never give up…no matter how hard you think it is, there are people put there that may have a rougher time. Work out, stay on your oxygen, don’t be afraid, take and do all of your meds!

Were there things you wished your parents did differently as you grew up with cystic fibrosis  (Example: more freedom to be in charge of your treatment, speaking at doctor visits,  life pre diagnosis/post diagnosis, not allowing you to do certain things because of CF)?

My parents were great! I lived a normal childhood when I wasn't sick, and learning from my parents is what made me so strong, they would always reassure me no matter how I was feeling…they treated me normal not like I had a life threatening disease, but they always made sure I did all of my treatments and dressed according to the weather outside J

Can you describe what it was like in the early days of CF and where we are now and where you think CF is going?

In my early days of CF they hospitalized more, but always gotten to come home with IVs and my mother always ran .There were no chest vests, no flutter valves, no inhalers just breathing treatments, and manual PT which I think is better anyway.  Where we are now I think is great! It gives hope to all the younger generation that may not ever have to have lung transplants because of scarred lungs.  They are making medical advancements every day that we do not even know about J

This question floats around a lot.  If you could be born without cystic fibrosis would you?.  But what has CF taught you?

NO.  I wouldn’t be who I am today without CF I would have the knowledge of a disease that a few people only know about surfacely, they don’t know what CF really is.  I aim to let that be known!  CF has taught me to grow up faster, not take any bull from anyone choose my friends and boyfriends wisely, keep up on my health so I don’t leave the family and friends I love so dearly to soon  I love life, even though its been hard at times, I still wouldn’t trade it, I wouldn’t be me.

I have found that there are a good amount of CFers who are worried to tell their boyfriend/girlfriend they have cystic fibrosis.  As a  CFer  in a committed relationship, what advice would you give to a CFer who is afraid to disclose they have cystic fibrosis?

I ALWAYS told a guy I was talking to about my CF before dating, before I would get hurt, 1) Its not right for them to fall for you and something happens and they had no clue you have CF, and they cant handle it, so they are put in a hard position  2) its not right to fall for them not knowing how they would react the first time you got sick around them.

I have always been upfront even when talking to guys I let it be known, because they should get a fair chance to, but you need to explain your own true definition to them because not all CF is standard text book, so you

must give them the chance to want to be a part of your cf life, its only fair, and if they don’t then, there is someone out there better that will! I know all of this for a fact! J

I talk with a lot of newly diagnosed parents to CFers. Many of these parents feel the world is crashing down on them when they hear the two letters CF.  I want to know what you would tell a newly diagnosed parent to a CFer.

I have been talking to now I call a friend since the day her daughter was diagnosed her daughter is 4 now. The earlier the diagnosis and compliance the better!

I have talked to many CF parents and yes they do feel like their world is crashing down  CF does not have to be terminal, even though the text definition says it is…no it doesn’t have to be…it’s a  hard bumpy road, but you get through those roads, and the CF patient worries about their parents more than the parents, because as a CF patient you know your own body and you have a wonderful support system (hopefully) but you still worry more, or well at least I always did and do, but a lot of children will ask their parents why am I sick and not my sister? That was one of my questions and  another one was why did you and daddy make me sick, because I was explained the two dysfunctional genes that both of my parents had to have to have a CF Child.

What advice would you give anyone who is battling cystic fibrosis today?

I would tell them I know it may not seem fair and it isn't, but it makes you the person you are today, just like cancer its not fair either but there is treatment and you could have a way worse disease than CF.  Stay strong, mentality is KEY in fighting this disease, work out, eat healthy, drink tons of water, exercise, take your medication religiously…sometimes that might not be enough and transplant is involved, but that’s done when only to save your life, give you a better life, if your life with CF has gotten that bad, you still have to exercise, eat properly, drink tons of water take your medications… BE COMPLAINT with whatever the drs tell you, and if you don’t feel like you are getting good enough medical care find a diff CF center.

Thanks,

Megan Lee Mobley, CF&DLTX Patient

John's Notes:

Megan is one courageous cyster that is for sure.  I love how her mindset focused on living not just for herself but for the people who love her when it came time for transplant.  As Megan states that CF has made her grow up faster than most people, I truly believe that statement.  Not very many people understand what that is like unless you live out this disease.  But this makes us all embrace our life that much more.  

I really like Megan's advice when it comes to relationships.  Sometimes with an illness we forget about the other side of the relationship meaning that other person.  We don't know how they will react once we tell them we have a life threatening disease.  As Megan states it's only fair to let them know because we do not want to hurt our self as well as the other person.  It is also good to point out how every CF person is different and we need to share with that person OUR CF story..  

I think Megan nailed it on the head about how a newly diagnosed parent feels and what advice she would give them.  For all the parents out there just remember we worry about our parents and how CF affects you as well.  We were given CF because we are able to fight this and be strong.  

Lastly, I share this same praise as Megan and many others who are a part of the CF community.........THANK YOU to all the organ donors.  You give the gift of a second chance!!! 

Well done Megan! 

CF Community Interview - Brad Johns (Fibro)

In this interview you will read what it's like to be a male CFer.  Brad, truly is a pioneer in our disease.  He was the first male CFer to have a child via IVF in his home state, Mississippi.  When you get to read what he has accomplished in the face of CF it's pretty impressive to me.  Even people without CF would be envious to accomplish as much as he has in his career and family life.  I can tell you that from a few conversations I have had with Brad and after reading his responses to CF he truly doesn't let a single thing stand in the way of his life.  Thanks for being an inspiration to us all Brad.  Enjoy the interview!

Name : Brad Johns   Fibro

Age:  42

Please share your cystic fibrosis diagnosis (Include age, how you felt at the time of diagnosis, how was life before and after diagnosis)?

I was diagnosed at 3 months so I have never known what it was like to be normal.

Once you realized you had cystic fibrosis did you change anything (did your goals change, views on life change, did you avoid certain things)? 

I don’t think I really understood until I was in high school. I just thought that it was part of my life. I wasn’t really sick while I was growing up until I got into high school. I missed over 180 days of school over the 4 years of high school. My senior year I was told I had contracted cepacia pseudomonas. I was the 2^nd person in the state of Mississippi to contract cepacia.  I still have it. Currently, I can’t get rid of it.  But it doesn’t cause me many problems fortunately.  At that time though I remember I was very scared. I had a bunch of doctors come in and talked to me and my family. That had never happened before. They told me that I could not visit any of the other patients and that I would have to wear gown, mask, and gloves if I were to leave my room at all times. It was at that point that my view of life changed forever. Once other CFers found out that I had this stuff they avoided me like the plague. So my life for the most part has been lived in isolation from others with CF. It wasn’t until I started trying to find a way to pay for a doctorate degree that I started seeking out others with CF.

What has been the biggest challenge for you dealing with cystic fibrosis?

I think it is the mind games that this illness can play on you. Knowing that you are eventually going to die from this stuff is a hard pill to swallow. That is why I try to stay on top of things all the time. I have a wife and 3 children. I want to live as long as I can so that I can experience as much as possible with them.

Please share with us any triumphs you have accomplished in the face of cystic fibrosis?

My 3 children (Josh, Justin, and Lyzzie).

Hero of Hope (Genentech Pharmaceuticals)

Star teacher

Teacher of the Year

McLaurin High School Teacher of the Month

Channel 16 (local TV station) Teacher of the Week

Doctorate Degree in Educational Leadership

Appeared on TV and in the newspaper multiple times for CF and non CF related events

Mississippi College Distinguished Alumnus of the Year (2013)

Writing Team of the 2007 Mississippi Mathematics Framework

Mississippi Professional Educators (MPE) board of directors

Mississippi Council of Teachers of Mathematics (MCTM) board of directors

Children’s Minister

Appeared through video at the Mississippi CF education day and spoke about having children through IVF

Outstanding teacher award!!! 

Since you have shared with me that you were able to have children what struggles did you face on this path? 

First the cost is overwhelming. We immediately started trying to have children once we were married through IVF. It was hard to pay for all that this entailed and also pay for my CF needs as well. Second, each time it did not work, the emotions that went along with finding out were really hard. Finally, I don’t think we knew what we were getting ourselves into when we found out we were having twins the second time that it worked. It was and still is hard at times. The first 2 years were the worst. Between taking care of kids and taking care of myself it was exhausting.

How has becoming a father shaped your life? 

I think many people do not understand that being a father and having CF is very tough.  Please share any thoughts you have on this topic. (How is treatment time before and after becoming a father? How did you let your children know about CF? If you had to give advice to any CFer on becoming a parent what would that be?)  It’s still hard at times. Making sure that everybody is taken care of and providing the finances to make sure that they have their needs provided for is hard. CF costs for my family have skyrocketed in the last couple of years. Normal people don’t understand how much our disease cost. The cost of our disease never goes away. We never catch a break from the cost. People with other diseases such as Cancer are never sick until they are diagnosed. My CF costs have always been there and will always be there until I die.

My treatments are very different now as compared to when we did not have children. I have to get up much earlier to start treatments. Treatments start each day for me around 4:15 a.m. each day. I use to be able to do them as late as 5:30 each day before we had kids. That extra hour of lost sleep takes its toll on the CF body. Now that my children are in school we have homework each day which makes the evenings more complicated as well. By the time we do homework, get everyone fed and bathed, its time for bed so there is not much time for anything else.

My oldest has known about my disease for several years. My twins who are 7 know it only in name. When they get to an age where we feel that they are able to understand we will tell them about it just as we did Josh.

If I had to give any advice to those seeking to be parents it would be to find out as much information as you can before starting IVF. When we started trying through IVF 15 years ago now, there was not much information out there. We were pioneers back then. I was the first male in the state of Mississippi with CF to have a child through IVF.

Brad's three children! 

Were there things you wished your parents did differently as you grew up with cystic fibrosis  (Example: more freedom to be in charge of your treatment, speaking at doctor visits,  life pre diagnosis/post diagnosis, not allowing you to do certain things because of CF)? 

No my parents let me lead a normal life. I played football, baseball, and was in the band in High School.

You have seen a lot of things as I call you a CF veteran.  I assume you have seen a lot of changes in the early days of CF and where we are at now.  Can you describe what it was like in the early days of CF and where we are now and where you think CF is going?

I remember my parents putting me on a table laying upside down and pounding on me for what seemed like hours everyday when I was little. I remember my parents putting my pills in a spoonful of applesauce so that I could swallow them.

These days there are so many advances in medications. It takes less time to take these drugs and they are more effective than ever before. I am hoping that during my lifetime we will see a cure for our disease.

This question floats around a lot.  If you could be born without cystic fibrosis would you? What has CF taught you? 

At this time I would have to say no. Having CF has taught me to rely on my Lord and Savior Jesus Christ for everything. He provides everything we need. There is not a day that goes by where I don’t constantly have to say o.k. God how are you going to work this out this time? You have to take care of this. I am putting my trust in you. I am totally dependent on God for everything. He provides for us!

I talk with a lot of newly diagnosed parents to CFers. Many of these parents feel the world is crashing down on them when they hear the two letters CF.  What would you tell  a parent who has a newly diagnosed CFer? 

I would tell them that it is not the death sentence that it use to be. We are able to lead fairly normal lives and are able to accomplish much during our lifetime.

I have found that there are a good amount of CFers who are worried to tell their boyfriend/girlfriend they have cystic fibrosis.  As a CFer who is married what advice would you give to a CFer who is afraid to disclose they have cystic fibrosis in a future relationship?  I think that it is important to be upfront with people. I remember telling my girlfriend (later became my wife) very early in our relationship that I had CF. I thought that if we were going to get married then she needed to know. I didn’t want her to find out and then leave because she could not deal with the situation.

What advice would you give anyone who is battling cystic fibrosis today? 

Never give up and never stop taking care of yourself properly. Always work hard to stay as well as you possibly can.

John's Notes:

I am so glad Brad took the time to give us his perspective when it comes to having CF and also being a parent.  I can tell you from my own experience it is VERY TOUGH!  The biggest part like Brad said is fitting in the time for you and your CF.  Treatment times are all different for me especially on the weekends and evenings.  There is NO schedule when you are a parent for your CF.  You no longer are selfish on you it's on the family now.   That is tough and I know from first hand experience its beyond challenging.   As you read, Brad wakes up extra early not to go fishing or hunting but to make sure he is getting in those needed airway treatments.  That to me is saying a lot about his commitment to his health and family!!! For all those out there that do not have to wake up an extra hour earlier to start their day just imagine this.  This is something that Brad does every day and many of us CFers out there as well.  We do this for more tomorrows with our loved ones.  

When I read Brad's accomplishments I just cannot even think of the right words to say but CONGRATULATIONS!!! Even people without CF will be jealous of you Brad.  I can think of one word that describes you and that is determined.  I bet the students you teach are really benefiting from a wonderful teacher and a spirited person.  Not only are your students benefiting from your determination but I could imagine that your wife and children are witnessing a wonderful human being day in and day out.  

Thanks for sharing your life with us all Brad!! You are a true inspiration to many of us out there battling cystic fibrosis.  I know that if you had to run through a wall for CF you would do that.  I wish you much more success throughout your life Fibro!! 

CF Community Interview - Anne (Cyster) Part 2

All I can say is that the first part of this interview with my CF MENTOR Anne was wonderful. If you missed it click here for PART 1.  You can really see how Anne embraces her second chance at life in part 1.

In part 2, you will read Anne's take on offering advice to CFers who are afraid to discuss CF with their partner, what advice she has for parents to newly diagnosed CFers, and her advice for all of us CFers who are battling this disease.  It's kind of funny reading this as Anne and I see eye to eye on a lot of these topics. In fact, some of you who I interact with will see we even offer some of the same advice!

Here is Anne with part 2 of her AWESOME interview!

***If you could be born without CF--would you?

In a perfect world CF, cancer and so many other diseases wouldn't exist. I recently said to my Mom--"It's good I got CF and not Sue or Kathy." They are my sisters. It's much easier having CF than seeing loved ones suffer.

CF, as most chronic illnesses--has the ability to teach one what really matters in life. Probably much of my growth as an individual has come at the feet of CF. But I'd love to see it eradicated. There are plenty of other ways to learn Life's Lessons.

***As a CF'er in a committed relationship--what can I tell others afraid to discuss CF with a partner?

This is sort of a funny question since at any time of the day or night, if one came to our home, they could hear what sounded like a horse galloping. This was the sound of treatments being done--my Mom's cupped hands striking different positions on the ribcages of my brothers' to help remove mucus from the lungs.

Still, I went through teenage years wanting to fit in, but all things weren't equal! I did aerosols and treatments for an hour before school, and I had to be ready for my treatment when my Mom was! She had 3 people to do before school every day. So as my girlfriends were layering on their bright blue eyeliner and eye shadow and finding the perfect outfit to wear, I was doing treatments. It was totally normal in our house.

My oldest brother, Dave, passed away my freshman year of high school, and they announced his death on the announcements at my high school. From that time on, it was known I shared CF with him and my other brother, Dan. Many people that were my friends knew I had CF before Dave's death, but after his passing, the whole school knew. It removed the pressure of trying to be like everyone else.

Anne and her life partner Joe!

My advice to someone who is afraid to disclose their illness to someone they're dating:

I think we all possess an innate feeling of wanting to be accepted and loved. On your first date when you don't know the person, there's no pressing reason to bring up CF--unless you want to. Possibly it's important to remember, you just have CF. It's a part of your life, and there's so much more that makes you--you.

If one hasn't had to work hard or struggle--whether it's school, or grades, staying healthy or getting a good job, it's possible they can lack depth. Some people aren't cut out for dealing with CF, and that's ok. If you open up to someone and they can't handle it, it's best to know before you're going through a really stressful time with your health. A relationship should enhance your life, not make it more stressful. We all kiss some toads before Prince or Princess Charming appears. Those toads tell you what qualities you value in a relationship.

Many of the fellows I dated helped with my treatments and visited me in the hospital. I can't imagine having CF and trying to hide it--along with my cough. It could only be passed off as a "chest cold," for so long!

***What would I tell a parent of newly diagnosed child?

How you handle your child's illness is how THEY will learn to handle their illness. Though it feels like it's the end of the world, it isn't.

I guess the only proof I have is my parents and the other CF families we've known who have navigated CF and remained sane!

I also understand there are plenty of single-parent households. Obviously that makes it much more difficult raising a child--let alone a child with a serious illness. There's a Social Worker on the CF Team that can help you. You deserve having a safe and unbiased person to vent to. On-line there are also good support groups to connect with other parents going through the same things. Don't close down. It's important to connect with others. You are not alone!

Being a patient in a Pediatric hospital through adulthood--I saw and heard a bit. There are parents who coddle their kids and don't want to "be mean," by making them do their treatments. On what planet is it a loving act to not make your child with CF do their treatments?! It won't give your child the strength of character they'll need later in their life, nor will it ensure the healthiest possible future for them.

Also, some parents keep their child out of school due to germs. Well, germs are everywhere. The air recirculating around your home all winter long is full of germs--no matter how great the filtration system on your furnace. I have heard many explanations from many parents over the years.

They ask me how I've kept a good attitude about CF and life in general. Well, for starters, I hit the Parental Lottery. My parents did not cut us slack on important things--doing our treatments, our schoolwork and helping others. Also, grade school, high school and college played an important role in my life. I still have many friends from 1st grade!

If my child had CF, I'd secure a great CF doctor. One who communicates freely and openly with you and your child--one whom you and your child feel very comfortable talking with. They will be your partner in navigating CF. They have to have time for you and be able to communicate with you in clear and concise terms.

Also, teach your child to speak up and advocate for their needs. Advocating for oneself is a very important element in being a good and well-informed patient. Also, follow your doctor's orders. He or she has M.D. after their name for a reason. That doesn't mean you can't question and challenge them. Nobody is perfect!

Kids take their cues from their parents. If you are overwhelmed and depressed, odds are your child is going is going to handle CF the same way. If you are positive, your child will be, also. If you don't believe me, just spend some time on a Pediatric floor! It's clear that kids take their cues from their parents. Dramatic parents = Dramatic child. Level-headed parents = secure, well-adapted child.

Anne and her nephew!

***How do you protect your child from getting sick?

This is a Catch 22. If a child isn't exposed to germs, he or she cannot build the antibodies needed to fend off those same germs when they come knocking again. Living as a Bubble Boy or Girl isn't the answer to avoiding germs.

Side note--my transplant doctor feels if people could stop touching their faces--we wouldn't get sick! Most germs and viruses are transported from our hands by touching our nose, mouth or eyes.

Take precautions--wash your hands, sanitize them after touching high-bacteria items--doorknobs, car keys, money, an elevator button or high-traffic objects.

Being a post-transplant patient, I wear a mask in what are termed, "Construction Zones." Construction Zones are defined as any place one cannot control the distance from another person. A crowd, an elevator, a basketball game in a full gymnasium, etc. Ironically, people tend to believe a person wearing a mask has a horrible communicable disease! Some ask and I explain the mask is actually to protect me.

Once a person is transplanted, they are immune suppressed for life in order for their body to not to reject the new organs. All those precautions one learns while having CF come in handy post-transplant, also. If a parent is overprotective before transplant, they may go off of the deep end after. Everything in life has a balance.

***Advice for people battling CF today?

1.) Be compliant with your treatments--they are money in the bank later in life.

Listen to your body. If you think something is "off," it very well may be. Call your doctor and explain your symptoms.

2.) LIVE YOUR LIFE. Normalcy is one of the greatest gifts one can have. When you are able to enjoy normal an Indians game, some 4th of July fireworks or even a walk around the block on a sunny day. Maintain as much normalcy as possible, and stockpile these times in your memory. Bring them our when getting through tough times.

3.) Possibly the most important element is exercise. What? You don't have the time?

There was CF doctor who ran the MICU at University Hospitals, taught classes at Case Western, was a husband and a father of 3 girls, he was also an Internist and a Critical Care doctor--so he had all those patients too--on top of his CF patients. Guess what? He trained and ran marathons! Thanks to him, it's impossible for me to say I don't have time to work out!

When one is on the transplant list--they send you to Pulmonary Rehab! So there you are on oxygen and you're exercising. Sure it's difficult, but it helps shorten the recovery time post-transplant--not to mention send you into an intense transplant surgery as strong as you can possibly be. There's a method to their madness!

When I was in college, a friend of our family sent me my first treadmill! I began walking on it...and kept going...until eventually I walked 6-8 miles a day! My health was better, and I recovered much quicker when I had an exacerbation. Exercise works--and you don't have to kill yourself. Try to walk for 15 minutes a day, then slowly build-up.

After transplant, one is required to complete 36 sessions of Pulmonary Rehab. It was grueling! My legs were toothpicks post-transplant, but I had the lung power to breathe further than my little toothpick legs could take me! It was difficult but awesome building myself back up to enjoy the capacity of the new lungs!

Post-transplant, exercise remains an integral part of my life. I still walk on my treadmill and ride an exercise bike. I do push-ups and yoga positions and whatever I feel like on any given day. I stretch out really well, it helps protect me from injuries. Sure there are days I don't feel on top of my game, and I don't beat myself up. I try to take a good walk outdoors with our puppy or even run and play with her inside.

With exercise even small changes help. Take the stairs instead of the elevator if you're able, park farther out when you going to the store--it's liberating not dueling with all the other cars for the closest parking spots!

Also, hydration is important. I won't say how much water I drink per day as it'd cause people to vomit! When I had CF lungs it helped thin out my secretions--long before Pulmozyme came along!

The other side of this is nutrition. Your body is working hard to breathe and fight CF, and you're burning major calories doing so. Fuel your body! Now this is somewhat hypocritical because it wasn't until post-transplant that I started to divorce myself from my junk food diet. I was eating for pure calories and fat pre-transplant--I needed every bit I could get! I was 80 pounds heading into transplant at 5' 5".

Keep in mind, sugar feeds bacteria. With CF and the chronic infections in the lungs, those infections are gaining power from all of the sugar one is consuming. Also, the processed food contain many additives that cause inflammation---also not something a CF'er's body needs.

We all have our own way of dealing with CF, and it's important not to compare yourself to anyone! Medicine works differently for everyone too, as do treatments and even exercise. The key is finding what works for you and following through with it. Surround yourself with positive people, they will lift you up when you feel down, and they will carry you when you can't "walk" any more. If you don't feel better after seeing someone, chances are they're not the most positive force in your life.

Some people are what I call, "Suckers." Not because they're sweet, but because if you allow them, they'll suck the life out of you. You know the type--they always have self-created drama happening to them. These people will exhaust you and steal your energy by engulfing you in their soap-opera life. You're living with CF, that brings enough stress. For the best interest in your health, avoid inheriting more!

Stress causes the body to release a stress hormone called Cortisol. It also causes inflammation and suppresses your immune system. Have you ever caught a cold when you were really stressed? Cortisol can put your body in a weakened state so you can't fight anything off.

Also, get out and help others! It doesn't matter if it's helping an older person load groceries in their car, do something. Not only are you helping people, you are helping yourself.

LIVE and enjoy your life! Laugh often and hard--until tears are flowing down your face! Love openly. Accept defeat and learn as early as possible it's really pretty great being you ;) 

Anne, Joe, and a MASSIVE pizza!

John's Notes:

I don't know where to begin.........Just some really in depth words here and wonderful writing.  You can see that Anne got accustomed to her CF life early on.  I think she hits the nail on the head when she explains that a child will view CF as you the parent view CF.  Hmm, this sounds familiar as I say this to many of you newly diagnosed parents out there.  I love this from Anne, Dramatic parents = Dramatic child. Level-headed parents = secure, well-adapted child.  I couldn't have said it any better Anne.  

There are many people out there who fear germs and Anne really goes into some depth on that topic.  GERMS are everywhere and we all know that.  This is a wonderful quote from Anne,  Side note--my transplant doctor feels if people could stop touching their faces--we wouldn't get sick! Most germs and viruses are transported from our hands by touching our nose, mouth or eyes. I think though at times parents and CFers drive themselves up a pole worried about all kinds of possible bacteria. It's plain and simple, its IMPOSSIBLE to eradicate all bacteria. If we are exposed to germs we build an immune system!   In Anne's words we need to treat CFers just as we treat any other person.  It's part of life and we all need to embrace LIFE! 

1.) Be compliant with your treatments--they are money in the bank later in life.    This is a fantastic quote that I hear every day when I do treatment.  I am putting in the time for more time in the future with my family.  Just remember you are all putting in the time for more tomorrow by being compliant!

2.) LIVE YOUR LIFE.  I like to say CF is along for the ride when it comes to my life.  If we can get to this attitude and thinking we will all be a step ahead in the right direction.  I like to think every time I get out of bed is another gift for me to put another mark on this planet :) 

3.) Possibly the most important element is exercise.  We all know how important it is to exercise CF or not.  But even more so for us CFers.  This is something I am challenged on every day.  I do try to find small ways to put exercise in my hectic life but I am full of excuses which needs to change. 

4.)LIVE and enjoy your life!  Nothing really needs to be said here.  Just go out every day and make it a great day.  No one has a crystal ball.  But at the end of the day, when you put your head on the pillow be thankful that you have had the chance to live out another day :)


I hope that everyone who has read part 1 and part 2 has come away with something from Anne's interview. I wanted all of you to "meet" this special person in my life and I thought that this would be the best way to do that.  We all need to find a CF MENTOR in our life and mine is Anne.  I am so thankful that every week I get to exchange emails and talk about life with my MENTOR.  

Thanks for doing this interview cuz you truly are a class act.  I LOVE ya like a sister or I should say cyster! 

If you are interested in joining me as we bring much needed CF awareness to the public please volunteer for the interview series here.