Tuesday, April 29, 2014

CF Community Interview - Jessica Pettus (Parent)

In this interview, Jessica describes what it was like finding out her son was in limbo CF status.  Crew is her son, and wasn’t diagnosed until a few months after his birth.  In fact Crew is very much like me.  We both have passed the “golden rule” test in the CF community and that is the sweat test.  He was diagnosed with CF after a genetic blood test.  His mutations put him in a CF limbo status.  Both of Crew’s mutations were not the well known mutations that many of us CFers have.  So the doctors were saying Crew has CRMS.  CRMS is basically having a borderline sweat test with one or more CF gene mutations.  The individual may or may not show signs/symptoms of CF.  Isn’t that a lovely definition especially to give parents who are so concerned.  For more information on CRMS click here. 

Here is Jessica telling her story about her son Crew! 

 NAME:     Jessica Pettus

Social Media Site:

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

Where do I even start?? Crew story is a little different then the most...so bare with me.Crew was born a healthy boy and was doing great until our 3 month check up, I had noticed hewasn't looking any bigger but thought I was just being overly worried. But after we weighed himthe doctor cam in and said “Your son hasn't gained any weight and something isn’t right. Please go straight to Children's ” I immediately wanted to bust out in tears, I got to my car and cried.What was wrong with him, why my child. I picked up my husband, Joe, and we went straight to the hospital. Children's admitted us and ran a million test and everything was coming back completely normal. They discharged us with FAILURE TO THRIVE(which basically means we don't know why your child isn't gaining weight) but there was one more test, the sweat test and we took him to get the sweat test and left. The hospital would call us in about 2 hours with the results. The nurse called and said “the test was inconclusive, there probably wasn't enough sweat collected, just go to your follow up” and thats what we did, after weeks of follow ups and probably gallons of blood later, they decided to do a genetics test for Cystic Fibrosis. I waited & waited for a phone call and when they finally did, it changed our lives forever! I will never forget that day the nurse called, sitting with my sister in law, and the nurse said “well we have some odd news for you, your son has 2 mutations on the CFTR gene but don't know what will happen, you are in medical limbo. please come to the pulmonary clinic next week.” I was TERRIFIED..what in the world did this mean. I think I cried for a week straight, So we went to the doctor and they diagnosed him with CRMS. This meant that one of his genes could or could not cause CF no one knows what will happen. So we went to our appointments and never felt satisfied, my husband and I felt like they had missed something, so we got a 2nd opinion at a different clinic, 4 hours away and SO thankful we did, And they did a CT scan and other tests & he is now on full treatment for CF and doing MUCH MUCH better.

Life before diagnosis was a lot less worrisome, as a parent i worry about all my kids but Crew I worry about a lot. Why is he coughing, is he gaining weight? But now we’re “getting in the grove” and CF is becoming a part of our lives. Just another thing we have to do, like getting dressed we just have too. No choice, just have to.

Before your child was diagnosed with cystic fibrosis, have you ever heard of cystic fibrosis?

Nope, Never...sad but true. CF needs more awareness, I hope one day its like the Pink ribbon everyone knows what the pink ribbon stands for Breast cancer. One day everyone will know what the purple ribbon is.

How did you cope with the diagnosis?

Well I still consider Crew newly diagnosed, even though its been almost a year. There is so much to learn. So coping we have our bad days and good. My husband I think is better at coping with it, he is so optimistic and thinks positive about everything! Im more like an open book, at first all I wanted to do was google, google EVERYTHING..and let me tell you, please don't google. It does NO good. I wanted to fix it and I can’t fix it. No matter how much money I raise or how many treatments I give him, I can’t fix CF. I can try to be the best mother to him that really helps me cope now, knowing I’m doing my best and that he will be okay & have a normal life, just like the other kids.

If we can go back to that day you found out your child had cystic fibrosis, what would you have liked to be told by a doctor? Where did you go to find more information about cystic fibrosis?

I wish the doctor would have told me more about the CRMS that Crew was first diagnosed with. There was hardly any information about it, even online. It was all the same like “your child could get CF or couldn't” but no one told me statics or how or why it happened...It left me in a state of trying to find out on my own & caused a lot of useless frustration. If i would have known a simple CT scan could have told us I would have pushed that a long time ago. His CT scan showed valves in his lungs where the openings are suppose to smaller but on a Cystic Fibrosis patient they are larger and Crew’s were larger. Also, I REALLY wish the doctor talked to my husband and I in English and by that I mean not is medical speaking over our head talk! Just explain it in day to day talk. I remember getting out of the doctors office once with Crew and immediately writing you, John, to ask a question and at that moment I realized that there was some lack of commutation. I got more info about CF from the Cystic Fibrosis Foundation but I also joined different social media groups and I downloaded and app that helped also. But to be honest the most information was from PEOPLE, people who have CF and/or a parent of a CFer! When you first get the diagnosis I think the biggest thing you are really needing or looking for is support!

 Were there things you wished you heard on that to either reassure you about your child and his diagnosis that were not said?

Well I wished I would have heard of all the NEW drugs and all the new accomplishment the CFF is making! Im stunned when I read about it online! Not TOO long ago, CF patients didn't live to be out of Elementary school, now the life expectancy is late 30s and BEYOND! The number keeps going up and up and up... Another helpful thing would have been care packet, at our new clinic they actually did give us one with TONS of info. It might should be minatory, if you ask me:)! For the record, I HATE THE WORDS LIFE EXPECTANCY, no one knows what their own life expectancy is.

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

The unknown. It’s hard for us to not know everything thats going on. like for example his Pancreas enzyme levels are always low like 290 but thats still normal(anything >200 is
abnormal) but a person without CF have levels of 500+...so why is Crews so low? Its frustrating,but in the same breathe im so thankful its normal. I could give you plenty of explains or unknown. Why do some people need constantly stay sick and others go there whole life without a hospital stay. We just keep doing treatments and medications to keep him as healthy as he can be.

Please share with us any triumphs you or your child has accomplished because of cystic fibrosis?

Well its the little victories that mean the most to me. Like Crew being able to sit still with a mask on his face and shaking for 30 minutes 2X a day and more without crying or   making a fuss. If you have children you can imagine how impossible it is for them to sit still, but he does. He  amazes me every time. I can even do dishes or clean the kitchen and he will sit there quietly, but I do have to give some credit to The Wiggles. :) Our pediatrician even mentioned today about how good he is even though he is sick Crew will let him listen to his lungs and look in the ears and do an xray without a struggle...I couldn’t be more proud. As for me I am in the middle of trying to accomplish a Big event in our town for a Cystic Fibrosis fundraiser. I want people to know about Cystic Fibrosis and awareness brings money and money helps find a CURE! I am very passionate, not just for Crew but for everyone with CF. These people are some of the strongest, helpful people I have ever met. They deserve a cure!

Can you describe what it is like being a parent to a CFer?

Being a parent is the most wonderful, hardest, funest job in the world, we have three children Crew being the youngest & only boy (yes, he is spoiled rotten;). But being a parent to a CFer is a bit different, you wonder how long you can hold that child for, that is too many unknowns for us with Crew and that’s what so scary. We go to countless doctor’s appointments and we wait for cultures to come back constantly and we never had that with our other children. If one of the girls were sick, we could wait and try something over the counter without even thinking but Crew keeps us on our feet. Our children have taught us more in life then we ever could teach them. And I mean that. Crew is so resilient, he can handle way more then I ever thought a little 24lb person could!

What has CF taught you?

CF has taught me to be thankful, thankful for everything. As humans we take a lot for granted, our healthy lungs, being able to go places without a worry we might get sick, or a possible hospital stay. Its taught us to be more responsible as parents and not as care free, we can’t skip any medication or treatment because it’s late or we are tired, there is no excuse. Crew solely relies on us!

 How will you approach the subject of cystic fibrosis to him/her?

We haven't gotten to this point with him yet, he is only 17 months old. But his older sister is very curious about Cystic Fibrosis, why he has to do these things and she doesn’t. We have tried to explain to her that Crew’s body works a little differently then most kids, and his body needs a little more help to work right. I plan on buying some children’s books about CF to read to them and one day read to Crew. I also hope and pray that he embraces Cystic Fibrosis and never feels ashamed by it. I want Crew to know that CYSTIC FIBROSIS DOES NOT DEFINE HIM! It’s just a part of him.

 I talk with a lot of newly diagnosed parents to CFers. I want to know what you would tell a newly diagnosed parent to a CFer.

I would tell them that your child will be perfect and OKAY!! He/she will get sick, just like every other kid in the world. Please don’t keep them in a bubble, I tried to in the beginning...i got a bag vacuum cleaner, soy candles, no mud, but wow, how can you keep up with it all. We CANT KEEP THEM FROM GETTING SICK! Bacteria is everywhere, probably on the keyboard I'm typing on. Just enjoy your child give them extra loving, you know what’s right for your child.

Mothers have an instinct in us, seriously. Also, be up front with the doctors, always. If you don't feel comfortable with your current doctor talk with others. See what the best fit is for your family. There HAS to be open commutation!!

Is there anything else you would like to share with us about yourself or your child?

I also want to add that Crew can still PASS a sweat test, and that he has 2 rare mutations and Alabama newborn screen test only test for 32 most common so that’s why he also passed the NBS test! SO NEVER GIVE UP! If you feel like something isn’t right, do something about it!

What do you have to lose? Even if you were crazy and everything was normal, well then you would be 200% sure, right?? I knew something was going on with Crew and I wasn’t going to stop until I got answers!

Crew is an AMAZING boy who will grow up to do great things and be loved and live a happy, long normal life!

John's Notes: 

Well I can tell you that the Pettus family has come a long way already in this short year and a half.  I recall reading Jessica's forum post on a CF social media site about Crew passing his sweat test but the doctor's believing he had CF.  I am so thankful that I reached out to this family to show them that Crew can live a normal life with CF.  I am just glad that the Pettus family didn't have to wait 12 years like my family did to find out I have CF.  The lovely gold standard sweat test really needs to be reevaluated to see if we should be relying on this test even after the new born screening suspects CF.  If one person falls through the cracks it's not worth to use the sweat test. If any doctor believes a child could have CF do the sweat test and the genetic test.  I know it's about the cost but we are talking about people's lives.  Ok I will get off my soap box.  
Jessica provides some great advice for newly diagnosed parents as she understands you cannot prevent your child from all bacteria. No parent and no matter how often you clean it's virtually impossible to prevent everything. You have to see that your child is "normal" even though they have CF. I can see that where Jessica states "Cystic Fibrosis doesn't define him". That is how I view my life with CF and how I hope many in the CF community view their life.

I couldn't agree more with Jessica about hating the phrase "life expectancy" I see way to many people get caught up on the life expectancy of a CFer. In all reality not a single person with CF or not has a clue on how long they will live. You have to live in the moment and squeeze as much good out of every day. No one ever will get a single day back in life.

This is a great interview from a newly diagnosed parent's perspective. I hope you find something in it that you connect with.

I am proud to call Jessica and the Pettus family a true friend!! Thank you for participating in the interview series!

To read more interviews from the CF interview series click here.

Also, if you are interested in participating in the interview series click here.

Thursday, April 24, 2014

CF Community Interview - Beth (Cyster)

Pregnancy and CF is a new wave of the future for CF.  I know years ago many cysters and fibros were told to not mother or father a family especially cysters.  The idea was and still is today, that carrying a baby full term is not the best for any cyster's health.  I think that is changing and we some of us have Kalydeco to thank because of this.  There is no scientific proof yet that Kalydeco thins the vaginal mucus secretions but some have hypothesized that it does.  I think the biggest benefit that Kalydeco has for cysters who are finding themselves pregnant is the fact that they actually "feel better".  They have more energy, lungs feel better, more weight gain, and a big one maybe less stress focusing on how sick they used to feel.  These are my own assumptions so I am not sure how accurate they are.  

In this interview, you will read from Beth what it was like trying to conceive and her thoughts on staying on Kalydeco while pregnant, having CFRD, and battling CF illnesses while pregnant.  She has some great advice for all cysters and even fibros on what to consider before even having a child!!   

Name: Beth

Age:  25

Social Media Site:

Please share your cystic fibrosis diagnosis (Include age, how you felt once you realized cystic fibrosis was)?

As I child my sister and I were always sick. My sister developed pulmonary infection after infection, while I was labeled ‘failure to thrive’ and wanted to do nothing but drink my bottles all day. My parents continually brought us to doctors and were continually turned away with the same old story of “kids get sick, it’s just another cold.” Finally, when my sister was 6 years old, and I was 4, my family met with a pulmonary doctor who took one look at my sister and I and said “Have they ever been tested for Cystic Fibrosis?” We got a sweat test that day. My sisters’ diagnosis came back positive, and the pulmonologist broke the news to my parents that I should be tested as well. My test also came back positive.

Being 4 I didn’t really understand the full impact of CF. I knew I had to learn how to swallow pills and for the longest time I refused to take enzymes without sprinkling them on applesauce first.

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

Being diagnosed decently early helped in the fact that I don’t remember much changing. I was still allowed to go outside and play until dinner, I had friends over, I ate things off the floor, etc.  Being a teen with CF was much harder. When you’re fully aware of just how different you are- when the whole teen dilemma is to ‘just fit in’ you become painfully aware of your limits.

I never wanted to go to college. I wanted to do hair and makeup but every single person who knew of my CF said it was a terrible idea and I couldn’t do it.  So, I didn’t.  I went to college instead where I struggled for 3 years before finally quitting.

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

Acceptance. I still, at age 25, have yet to accept the fact that I have cystic fibrosis, and not in a “I won’t let CF define me” sort of way, but rather when I daydream about the future my CF doesn’t play a role. I can’t imagine being 50… with CF. I go through bouts of denial, depression, anxiety and anger. 

CF isn’t an easy disease and everyone’s experience with it is different. I’ve watch my sister almost die on a few occasions and I watched her be “re-born” when she finally got her lung transplant.  I’ve seen the slow, steady, painful decline and I can’t help but know that perhaps that’s in my future.  While some people would use that as motivation to do everything in their power to “Kick CF’s butt” for a long time I did the opposite. It made me scared and I shut down.

Now that I’m pregnant, and I’ve been dealing with on and off again infections from catching the flu… a lot of these emotions come up again. I’ve been feeling lots of anxiety about the future with a little one – someone dependent upon me.

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

After leaving college I moved to a different state and started seeing a new CF team. When I first started seeing the new CF team my lung function was hovering around the 50’s. I had been misdiagnosed as bi-polar and was battling being on some very high doses of medicines I didn’t need.

I worked very hard with this team to get myself better, in all aspects of my life. Not just physically but mentally as well. It was a very rewarding two years. I left with my lung function in the low 70’s.

I continued to improve after moving to the Johns Hopkins’ clinic with their adult team. I boosted my numbers up to the mid-70’s. Later that year Kalydeco became available for my mutation and it helped boost my numbers up even more.

There have been many times in my life that I did things despite CF: cheerleading, gymnastics, singing & acting, etc. but I think my most triumphant accomplishment was allowing myself to be helped and wanting to be helped. Change is a very scary thing, for anybody, so finding the power to ask for help knowing I needed to change something was very hard and something I’m very proud of.

Since we know you are expecting your miracle child please share with us your feelings on being pregnant and having cystic fibrosis?

First off, I’d like to just go on a quick tangent and say that the phrase “miracle child” pisses me off. It just does.  A miracle, according to wikkapedia is “a surprising and welcome event that is not explicable by natural or scientific laws and is therefore considered to be the work of a divine agency.”

While being pregnant is certainly a welcome event, my child is fully explainable by natural and scientific law: I was ovulating and my husband and I had unprotected sex. BOOM, I got pregnant, that’s how these things happen people.

By calling her ‘miracle child’ means that there was absolutely NO way in all of nature, and more importantly, science that I could have fallen pregnant. That my dear friends, is just bullshit. CF may cause complications and may make the journey a bit harder for some, but it is no way a miracle. Now, if I didn’t have a uterus and still managed to conceive well then I’d be singing a different tune…

Anyway. Apologizes for my ranting. Onto my feelings: anxious, nervous, delighted, excited, scared/terrified, tired, grateful and a few others thrown in there.

I think with any pregnancy you feel some level of anxiety- especially first time parents. Are we going to be able to provide everything this little human needs? Did that non-decaf coffee I had yesterday affect my growing baby? Will our son hate us if we circumcise him and all his friends aren’t circumcised? Am I bad mother if I bottle feed? Etc.

Having CF may amplify some of these feelings because you have more to consider, more weighing in the balance. Will this drug potentially harm my baby more than if I didn’t take it and got very sick?

I know you have decided to continue on Kalydeco since finding out you are expecting your child.  As Kalydeco is so new to the CF world we do not know how this impacts a fetus.  How hard was it to decide to continue taking Kalydeco?

There was almost no question in my mind whether or not to stay on it. My husband and I, after scouring the available literature, decided before we even started trying to conceive that I wanted to stay on it.

I had some insurance issues when I first started out on Kalydeco, and I ended up having to stop taking it for about a month and a half after taking it for 3 months. Within a week I was full on lung infection sick. I felt like I was drowning. I lost weight, and I just was overall miserable.

Knowing how my body reacts when I stop Kalydeco there was no way I felt comfortable coming off Kalydeco, especially knowing there were other drugs in my regiment that needed to be stopped due to knowingly cause defects in a fetus.

My husband and I both felt that the animal research that was done looked promising enough that any unknown risks were greatly outweighed by the benefits Kalydeco provides for me.

What have you noticed from being on the miracle blue lightning pill, Kalydeco (feel free to share any health changes)?

There’s that word again- miracle. ;-)
Anywhoo, Kalydeco has been quite an amazing little pill. I started the pill around 74% lung function (as measure by FEV1- around 1.2 L for me). Within 24 hours of taking my first dose I noticed a difference. My first dose was taken at night, and then the second dose was taken the next morning when I woke up. (8pm and 8 am) by noon I was coughing a TON up. But my coughing was different; it took almost no effort to move up massive amount of mucus. It was so noticeable that even my husband commented on it!

Within the first month my lung function had shot up to about 80%. I felt less out of breath, I had more energy, and I gained about a kilo (2lbs).
Since then I’d say the change has leveled off. While my overall lung function numbers are higher – I now have a baseline 10% higher than pre-kalydeco, my general feeling of health is the same. Whereas I would feel great after IV’s at my baseline of 74 – I now feel great at my baseline of 84.  Previously, I would continue to drop until about 67 or so and need some oral antibiotics, whereas now I drop until about 70-75ish and need oral antibiotics.

I feel that I get sick just as frequently as I did before. General colds may not last as long, and there have been a few that I’ve just ‘gotten over’ like a normal human being, but all in all after being on Kalydeco for over a year I’m a little disappointed.

Do not mistake my disappointment for ungratefulness. I am still every single day, grateful for the extra gain of lung function. I’m just disappointed that I don’t actually FEEL continually different. I still struggle with gaining weight, and I’m still getting IV’s just as frequently as I was before.  So while my numbers say I’m healthier, I just don’t feel it.

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, not allowing you to do certain things because of CF)?

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

This question, while it floats around a lot, I honestly feel only has one answer: YES!  I wouldn’t wish CF upon my worst enemy, if I had the chance to go back and be born without it? ARE YOU KIDDING ME? How is that even a question, there’s no other answer besides yes.

Now, I know, there are a few individuals out there who answer all high and mighty “No, CF has taught me so much and has shaped who I am so much that I would choose to do it all over again” --- you freakin’ kidding me? Everyone has a battle in life, if it wasn’t CF it would be something else, something just as prominent in shaping who you are as a person.

Now, these people maybe healthy, these people may be determined and may have a system that’s working for them… for now. But I have seen first hand just how devastating this disease is and can be. I’ve watched my sister wither away for years, and I’ve watched her best friend (also with CF) wither away as well. They both went through the toughest battle of their lives getting transplants, and while my sister has recovered very well and is still going strong 3 years out, her best friend only lasted a few good months before rejection and infection set in and took her life.

I’ve seen where the path leads and it’s not something I would ever EVER EVER choose.

What has CF taught you?

Life just isn’t always fair. Be kind and nonjudgmental of everyone since you never know what battle or demons they may be fighting.

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?

Your feelings are valid. The world you know is crashing down on you. But, from wreckage you can rebuild greatness.

I think one of the most important things you can do when your child is first diagnosed is to talk to someone – professionally. It’s also equally as important to not let this diagnosis rule your life and your family’s life.

And lastly, don’t forget about yourself and your significant other. I watched my parents relationship deteriorate because of our diagnosis but I think its because they put us and our disease first and never took the time to work on themselves as a couple.

As I speak with many parents to CFers they sometimes fear that their child will be viewed differently from other kids at school.  Do you have any personal experience with either being made fun of or bullied because you have CF?

I loved having CF in school! I got to eat snacks when no one else could, if I didn’t feel like running laps in gym I didn’t have to. I was always honest and open and couldn’t care less about who know I had CF. In my opinion if someone sees me taking 8 pills everytime I eat and they ask about it, I just told them “I have CF so I have to take pills to digest my food.”

There are only two times that left ‘scarring’ memories for me: 7th grade- I was coughing (a lot) and while I always tried to cover my mouth, there were sometimes where I just wasn’t aware that I was coughing! The whole gym class was sitting on the bleachers listening to our gym teacher go over something when he stopped and said to me “can you stop coughing, the poor guy (pointing to the boy next to me) doesn’t want your germs, and probably can’t hear a word of what I’m saying” …. I immediately got up and ran crying to the changing room. As I was running I heard one of my friends pipe up and say “You know she has Cystic Fibrosis right?? It makes her cough and she CANT HELP IT”
He claimed he was joking and while he was known for being a ‘ball buster’ and a jokester I ended up going home early completely embarrassed by what had happened. I mean from a kid I could probably understand, but all my teachers knew about my CF, and to call me out like that in front of a whole class – of mixed grades- was unacceptable.

The second time was in highschool. A time where you just want to fit in. I had a best friend who I LOVED. I just didn’t love her friends so much, but since I loved her so much, her friends became my friends. There was one random time where some of us guys and gals were all hanging out in a basement watching football or something when farting as a topic came up. One of the boys continued to say “well you know who I hear has the worst farts ever? BETH!” and everyone laughed while my best friend (really the only one I would ever rip ass in front of) did the whole ‘smack him while you say his name exasperatedly.’ While I wasn’t going to run out to the locker room to cry like I did in 7th grade and instead I responded with a “yea, well, you try not being able to DIGEST FOOD and see how lovely your gas smells ya douche…” 

While people laughed and I had made it a joke I still remember feeling embarrassed and hurt and mad at CF (and my best friend) for putting me in that situation.

But besides those two times, I can’t remember a certain time where I was called out or made feel different because of my CF.
Every now and then being tossed a mint from a teacher, or asking if I needed water – got annoying, but people just get concerned that’s all.

My philosophy was ‘the more people know’ the less of a big deal it will be.

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?
My philosophy was the more people know the less of a big deal it was. If you bring it up casually in a “I have brown hair and CF” kind of way, I don’t see why it would be a big deal?

Most of the time you’re met with a “what’s that?” type of question and that’s your opportunity to present it in anyway you see fit. My go to was usually just summary of things that usually are obvious “oh, just this genetic thing that makes me cough -- a ton –“ (along with eye roll) and other things like, “I need to take pills to digest food, and I need extra salt and calories.”

*I tried to answer your ‘advice for others battling CF’ but I fell short. I just feel that I’m still figuring it out myself. It’s not called a battle for nothing. =)

John's Notes:

Just another wonderful interview in this blogger interview series.  There was a lot discussed here and I know a lot of people will benefit from reading your story Beth.  I am really happy to hear that it only took one try to conceive.  There are many in the CF world who have had so many battles with fertility.  

Thank you for sharing the challenges you have faced carrying your baby and battling CFRD, the flu, and CF illnesses.  Many people outside of the CF world have no idea what it's like to battle our disease and then throw in carrying a baby on top of that! That truly is a feat in itself.  

Thanks Beth for doing this interview and CONGRATULATIONS on your pregnancy.  Welcome to the new club!! That is kicking CF butt and being a parent :) 

Tuesday, April 22, 2014

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 

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!!