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 :) 

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