Q&A From A Newly Diagnosed Parent

As some of you may know, I really love to reach out to new mom’s or dad’s who have a son or daughter who has been newly diagnosed with CF.   When a parent hears the words your son/daughter has cystic fibrosis I can guarantee they have a zillion thoughts, emotions, and questions running through their mind.  Most of these parents have never even heard the words cystic fibrosis.  

I have been trying to offer my guidance/life experiences to these parents.  I always introduce myself with a little background about me and how I have had many dreams come true. At the end of my introduction I always offer the parents the opportunity to ask me any questions about this disease.  What is great about offering them the opportunity to ask questions is they actually do have questions for me.  I love to answer their questions to help shed some light on this disease.  As long as they ask I will keep giving answers!

Here is the latest round of questions from a mom to a baby CFer.  These questions I can see many parents asking themselves! I am glad she decided to ask me for my opinion.  I hope that the answers help.

   

I’m obviously very protective over NAME……is there any advice you can give me in being a mom to a CFer?

Well being a protective mom is not something that would change even if your son/daughter didn’t have CF. Being a protective mom is just a mommy instinct.  Trust me I see it at home with my wife being protective of Alayna.  As for giving you a piece of advice the biggest thing I would stress to any new parent is to treat them like any other child.  You cannot raise your child in a bubble and worry about every single CF bacteria out there. You have to let them experience the world every day just like every other child.  If you start to shelter them they will feel that is how they should live their life in fear of the “outside” world.  I know it has to be scary to a parent about all the “possibilities” out there but you also have to look at the positive possibilities when exposing your child to all the great things in life. 

One thing that I would like to stress is to make your child’s health a priority right away.  It is going to seem like a major challenge to “change” your life around airway treatments, neb treatments, doctor appointments, and all the various other things CF throws at you.  All those things will become a part of your life once you get into a routine almost like getting into a car and fastening your seat belt. It just becomes old habit. 

Lastly, a child will view his/her parents as a mirror.  The way you portray CF and the outlook you have on this disease is how he/she will view their disease. I know not every day is going to be a beautiful picture. But if you try to paint a picture that is positive so will your child.  If you are looking for more advice on this topic read a prior blog I did for a newly diagnosed CF parent here.

http://livingmydreamswithcf.blogspot.com/2013/03/parents-accepting-cf-my-response.html

Was your mom to protective? What did she do that you appreciated?

For those who have not read my CF diagnosis I was not diagnosed until I was 12.  So my mom didn’t really smother me as a child growing up.  Yes, of course she was looking out for my best interests when it came to my health.  In fact both my parents made it my responsibility to take care of my airway treatments. I really appreciated my parents making me accountable for my health  They would remind me occasionally to do my treatments and I was really good about getting both airway treatments in everyday.  

It wasn’t until a few years after my diagnosis the vest was made available for airway treatments.  My vest was located in my room and again it was my responsibility to use it.  I can honestly say by giving me the responsibility and holding me accountable and not nagging me every time to do my airway treatment really worked for me.  I felt like I was in charge of my health and I took a sense of pride in it at an early age. 

I think it's very important to have your child at an early age take on responsibility when it comes to this disease.  The earlier and sooner you get them involved in CF care it just becomes easier and a way of life.  They begin to “accept” that they have to do this medicine, this treatment, or attend this doctor appointment because it is a part of their life.

So how do you get your child involved? You can have them setup their nebulizers, get the vest all hooked up, or even have them make up a treatment schedule calendar. The more accountability and responsibility you give them, will make them feel more in control of their disease. 

One thing I forgot to mention that I appreciate about my parents and not just my parents but my wife as well is that I have my own entourage when it comes to me attending my doctor’s appointments.  I still love the fact that someone I love is willing to attend my doctor’s appointments with me even at my age of 28.  This may not work for all CFers but I still really appreciate them showing an interest in my health.  I hope they continue to attend my appointments for years to come!

What did she do that annoyed you?

I am trying to think of something that my mom or dad did to annoy me when it came to CF………..Well I guess it is a good thing that I really cannot think of a big event that annoyed me.  I guess more so nothing that annoyed me but they always felt like I should guard against telling people about my disease.  For the most part if you were not a part of our family or not a very close friend to our family you really wouldn’t know I had CF to begin with.  Now, as you can tell through my blogging I am a lot more open about spreading awareness about CF.  I never introduce myself as John, I have CF.  I tend to reserve that conversation down the road until I know what kind of role you will play in my life. I am a lot more open about CF because I feel the more people who know about this disease the better we are as a community and the closer we will be to cure!

Also, did you get teased at school? I think I would go crazy if someone was mean to NAME!

As I said in a prior answer I wasn’t diagnosed with CF until later on in  my life.  I really cannot ever recall a time where I was teased about having CF.  The only people who knew I had CF were my family and close friends.  Nothing was ever said negatively about this disease around me, that I know of.  When it comes to being teased I can see this happening on different levels.  Many of us have clubbed fingers or odd shaped fingernails.  I have heard where some cysters of mine have been made fun of because of the way their nails look or they have the CF bloated stomach look.  I guess as a male I didn’t have to worry about that.  Or how about for males getting semi undressed in the locker room………..I guess there could definitely be a body image issue there as most of us are very skinny.  I can attest to one story that my wife was making good humor of my skinny legs way back in high school.  She said I had chicken legs! Haha!! Thank you enzymes no more chicken legs for me : )  Once you get past the high school drama  all that potential teasing fades very quickly.  Plus it doesn’t matter because every person is different CF or not!

What age do you think it’s ok to send them to school?  We were thinking when NAME is 4? 

I feel there are more positives than negatives when it comes to having your child around other children. I have read that there are some people who are afraid to have their CF child playing on playgrounds, going to daycare, or even attending school because of the risk or possibility of contracting a bacteria like pseudomonas.  Just remember a lot of the bacteria a CFer can get is found in your home!!  Bacteria lives on surfaces, in water, in soil, even in the air.  So the more you are able to accept that you cannot protect your child from all bacteria in this world the better. Just make sure that you and your child practice excellent hygiene techniques.   

I can relate to this experience as I was not diagnosed until well past birth.  My mom and dad let me play in the dirt, they sent me to school, I swam in public pools, and I played sports.  Never once did my parents think about pseudomonas before I was diagnosed with CF.  In fact, once I was diagnosed I still did all the same things and never once did I hear my parents tell me to stop doing something because of the possibility to contract a bacteria.  I just continued to practice good hygiene techniques.

I suggest sending your child to school when you feel it is right for them.  You will know when that time comes.  The more you treat your child like a “normal” child the better off they will be when it comes to dealing with CF and life, in my opinion. 

 

When it comes to children you can read all kinds of books on how to raise a child but children are all different.  In the CF world, we are all different as well.  So what works for me or works for you may not work for your child.  Sometimes you have to jump with your two feet in the water to test it out. You will learn as you go and find things that work for you and your child.  What really matters is finding what works.   Always remember this........you are a parent to your child not a parent to a disease.  CF will never define who your child is.  Your child will define who he/she is!!! I wish you nothing but the best! If you have any more questions please feel free to ask away and that goes for whoever is reading this as well.  There is never a dumb question either.

Alayna 9 Months Old!

July 17th marked 9 months on this earth for you Alayna.  As I have said every month, where has the time gone? In 3 short months we will be celebrating your first birthday.  I still cannot wrap my head around that statement.

Your mom and I were looking at pictures of you when you were a few weeks old and you have changed so much in that time.  You were this little string bean as you were so thin and super long.  When we look at you know, your still super long but you have also packed on some pounds.  I think you are about half the size of mommy in length now.  So I think its safe to say that you will be taller than mommy but the jury is still out on that! But just remember she is still your mom no matter how tall you are.

This past month you finally learned to crawl.  At about 8.5 months you decided it was time to explore on all fours.  Honestly, now we cannot take our eye off you once we put you on the ground. There is one thing that I do not understand and that is how you love to crawl to things you shouldn't get into or next to.  The other day you decided you were going to crawl all over your bedroom.  You crawled to your bedroom trash can and decided to knock it over on the ground.  I could not get mad because you had the biggest smile on your face once you got to the trash can.  I said to you......."Alayna, what do you think you're doing?" You looked back with a smile and tipped the trash over. I was glad there was nothing that awful in the trash can for daddy to clean up.

It seems to be a trend with you that you crawl right in front of your dresser or the crib or the floor vent because you know WE get nervous of you getting hurt.  You will crawl to these mentioned places and decide you would like to sit up.  Once you sit up you love to just flop your entire body backwards which really makes mommy and daddy nervous.  I guess you have no fear.......yet. 

Well just speaking of how you love to flop backwards, you did just that the other week.  Mommy put you in your crib and you decided that you were going to be daring and pull yourself up and stand up while holding on to the crib.  Once you got yourself up, you decided that you should flop backwards and hit your head on the back of the crib.  So after that experience mommy and daddy dropped your crib to the lowest level.  If you keep growing this fast you will be in a big girl bed very soon!!!

Alayna, you continue to love eating baby food and I guess that shows in how big you have gotten.  But you also think that it is play time when you eat.  There have been numerous times WE have been feeding you and all of a sudden you decide to do what I call "raspberries".  You start spitting your food out all over me.  It is funny because you get this little grin when you do it.  One time, I was feeding you and you sneezed and the food shot straight out all over my face and clothes.  I didn't mind it because you had a big smile. It's just a memory between you and I!

This past month you learned to clap your hands. In fact, you LOVE to do this.  Sometimes all of a sudden WE will look at you and you just start clapping.  Not only do you love to clap but you will clap your hands when we ask you to clap. You started doing all this clapping when your Grandma Judy taught you the song patty cake. It is so adorable to see you clap through the song.  I guess we can say you are one smart baby.

In other developments, you top two teeth have finally broke in so that makes a set of 4 teeth in that little mouth of yours.  Now that you have these 4 teeth you think it is funny to try and bite our face which includes our nose and even our cheeks!! As for the vocabulary you still have not said "da" for daddy yet.  I know that day will be coming soon though.  You continue to grow out of clothes left and right.  You are now in 18 months which is pretty unreal if you ask me.  You are all legs Alayna!

Today, at your 9 month appointment you weighed 18 pounds and 13 ounces which is 50th percentile. As far as your length.......you are SUPER TALL 29 and 5/8 inches which is 97th percentile. You also have 5 teeth. Two on the bottom and three on the top.  Next month will mark 10 months.  Really??? You will be double digits!! I am sure you will be even bigger in 4 weeks from now. You are growing up way to fast in front of our eyes. 

CF From The Other Side........"What's it like in their shoes?"

When we think of the CF community we tend to think of it as a relatively small community, and it is.  In the US there is approximately 30,000 CFers and a total worldwide of 70,000 of us.  To me, that doesn’t seem like that many people but there are thousands more who are “indirectly”affected by CF. These people are the spouses/fiancés, boyfriends/girlfriends, parents, brothers/sisters, extended family members, children to CF patients (years ago I may not have put that in there but its FANTASTIC WE can have families), and lets not forget the countless doctors, nurses, and researchers. When I look at that brief list you could easily add thousands more to our CF community.  

Even though, there seems to be more individuals who are impacted/indirectly impacted by this disease the feeling of being alone or isolated is still there for MANY people.  So what happens when people feel isolated or alone?  They turn to others who are going through the same things or who they can relate to. Who else do you know on a personal level who is either impacted by CF or has a loved one with CF?  I assume the answer to that question is NOT many people you know who are “in your shoes”.

So what brought this writing topic to my mind?  Honestly, I was having a conversation with the wife this weekend about people not understanding what it’s like to be “in your shoes”.  The fact of the matter is that no one truly understands the battles anyone goes through unless we can connect in some way.  I do not know how it truly feels to be in a lot of situations.  For one I will never know how it feels to be married to a person who has a life threatening disease such as CF.  I can guarantee you that my wife has thought about losing the man she loves more than anything in this world before.  I know we can say well CF or not no one has a crystal ball and nothing is guaranteed in life and that is so true.  But in all reality, maybe that magic 8 ball that has my life in it may have a few more dice sides with a negative scenario.  I can tell my wife and reassure her that I am doing everything in my power to make sure those negative scenarios do not come up when it comes to this disease.  But I know losing the man she loves will always be in the back of her mind whether she expresses these thoughts to me or not.  

I guess what I am trying to convey is that I will never know how this “truly” feels.  I am just so thankful that my wife has accepted me for who I am. But it does hurt to think that the love of my life would may be one day have to raise our daughter on her own and take care of all the other duties ofraising a family on her own.  The thought of all this motivates me to be so compliant with my health because I want to avoid that scenario if I can control it in anyway possible.  

In fact, during this conversation my wife said to me “I don’t know how you do it every day knowing that you have the possibility of not being here one day.  But I am so proud of you John how you view your life so positively!” I responded with this, “That is the only way I know how to be with this disease is to try to be as positive as I can.” As I have said on my blog before nothing ever comes from negative thinking.  When we think negative thoughts we just create a hole that we keep digging and it’s so hard to get out of it.  

The world of CF may seem small and it truly may be that way. But in all honesty, it doesn’t have to be a place where you feel alone and cannot find someone going through something very similar to you.  You can be a CFer, a spouse to a CFer, boyfriend/girlfriend, a parent to a CFer,the list goes on and on but you should never feel like you are going to battle alone. I suggest that you find an outlet to express yourself and connect with others who are battling the same war. If you cannot find someone in person I encourage you to use all these social media outlets we have at our fingertips.  So the next time you are feeling alone and would like to express to others who are "in your shoes" look into the world of social media. I suggest blogging, to forums such asCystic Life, CF Connect, CF Forums, and other groups on Facebook (that include the CF wives club, CF mommies, IVF groups, and the list goes on and on).  I know I have found my place where I feel connected to this community in my blog, forums, and Facebook. 

 

 Those two girls are the reason I FIGHT every day!

 

 

My Thoughts On The Lung Transplant Policy Change

****DISCLAIMER – I am not a doctor and I am just expressing my opinion on the recent news of the lung transplant policy change regarding Sarah Murnaghan**** 

I was debating to blog about all this lung transplant policy change for children and decided I will give my OPINION.  As I am not a medical professional, this post is coming from what I have read online (I know not the best source) and also my very own opinion on this policy change.  

If you are not familiar with this recent lung transplant story in the media here is my summary.  Sarah Murnaghan a CF sufferer is 10 years old and is in need of a lung transplant as her CF had progressed to end stage life.  Sarah was listed on the PEDIATRIC transplant list.  In order, to be on the adult transplant list you have to be 12 years or older.  Sarah and her family were waiting for a pediatric set of lungs to become available. The issue about being on the pediatric transplant list is that there are very few pediatric donors and the chance at receiving a second shot at life is not as high as being on the adult list.  

Sarah’s parents took it upon themselves to do whatever they could to get Sarah listed on the adult list as they were told Sarah wouldn’t make it much longer.  In order for Sarah to be listed on the adult transplant list the family filed suit against the 11 and under rule.  A federal judge granted the policy to be overturned in favor of adding children to the adult transplant list on a case by case basis.  On June 11^th the OPTN/UNOS executive committee approved a revision to the national lung allocation policy affecting transplant candidates age 11 or younger.  The policy basically allows a lung transplant program to submit a request to a national lung review board for a candidate 11 or younger to have an additional listing, at the same hospital for adult/adolescent lungs based upon the candidates Lung Allocation Score.  The Lung Allocation Score (LAS) is calculated based upon a series of formulas that take into account statistical probabilities of a patients survival post transplant as well as current health status.  Basically the sicker you are the higher your LAS score will be.  

The decision by the OPTN does not mean that children 12 and under will automatically go to the front of the transplant line.  They will also not receive special treatment just because they are children.  This simply means they will now be placed on the same list as everyone else based upon the severity of their illness just like people 12 and over are listed.  Sarah’s LAS finally mattered and she was now able to get that chance of a second shot at life.  Her LAS in her region was close to 100 (the scoring system is 0-100) which placed her as the sickest in her region.

In fact, Sarah got the call for her second shot at life and received her new adult lungs on June 13^th.  Unfortunately, the lungs failed a few hours after the transplant as the lungs were of marginal use according to some internet sources.  People can debate if that was due to the fact that the doctors had to trim the adult lungs to fit Sarah.  Sarah was then put on life support until June 15^th  when another set of adult lungs became available.  The issue with the second set of lungs was that the donor lungs had active pneumonia. The lungs needed to be trimmed as well to remove the pneumonia and fit Sarah’s chest cavity.     

Both sets of lungs were high risk especially the set that had active pneumonia.  In my opinion, the transplant team would not have used either set of these lungs unless it was a critical situation.  In this case, Sarah was in a critical state and needed to have any method available to save her life.  The Children’s Hospital of Philadelphia is ranked nationally as one of the best children’s hospitals in this country and are very well known around the world in transplant success.  These are some of the best doctors when it comes to transplants and why would we not trust their medical judgement.   

Currently, Sarah is in the fight of her life trying to get off the ventilators and preparing for extubation.  She will have surgery tomorrow to help with this extubation process.  Sarah has what is called a partially paralyzed diaphragm due to the transplant surgeries. All I can say is that this child has been through way to much for a 10 year old.  She is supposed to be enjoying her summer break and running around with her friends.   

So that is the groundwork for this post but I also want to talk about what I have read about people commenting on news articles relating to Sarah and her transplant.  I was shocked actually to read that many of the comments were negative. I understand that everyone is entitled to an opinion but some of these comments are over the top. They range from anywhere stating that Sarah has CF, even if the operation is completely successful…..well even if all future operations are successful……..she won’t live past 20. It is like using a bucket to remove water from a sinking boat. You’re buying time…..that’s all.  Or the comment of a lung transplant will only buy you 5 years of life.  When I read these comment I couldn’t believe that there are actually people out there who believe that giving the gift of life is not worthy if you do not make it past so and so age.  That is why people donate organs so they can give tomorrows to people.  No one should count the number of tomorrows because not a single tomorrow is ever guaranteed.  That is what Sarah’s parents want, the opportunity for tomorrows for their daughter.  Not only do Sarah’s parents want tomorrows WE all want tomorrows in this CF community.  

As far as Sarah cutting in line on the transplant list or taking the lives of two other adults potentially life saving lung transplants I am not buying that.  Sarah was put at the top of the transplant list because of her LAS score not because she was in the media. She was the sickest person on the list that is all that needs to be said.  No one knows the outcomes of any medical procedure until its performed in life. You have to put your faith and trust in the professionals who make these medical decisions that they are the right decisions for all parties involved.

So how do we make sure everyone on the list gets a second shot at life? Well if anyone invents a way to grow lung tissue in a lab that can be transplanted with no side effects they would solve this problem.  But until that day happens, WE all need to find ways to make this list as short as possible.  If you are not an organ donor you need to become one.  You don’t need to bring your organs with you when your gone. Plus why wouldn’t you want to give people a shot at more tomorrows?

Lastly, in my opinion the number one way to eliminate this CF lung transplant list is to make CF stand for CURE FOUND.  We are very close to seeing the light at the end of the tunnel with the exciting news from Vertex and the magical pill Kalydeco.  But the Kalydeco pill is NOT a cure.  We still need to keep the drug pipeline funded through the generous donations from individuals who want to see a cure for this disease.  Just remember the more people who are aware about CF the better off our community will be.


*** I hope I represented the transplant community well on this post.  I would love to feature a guest blogger who is either going through the evaluation for transplant/ is currently on the transplant list/or has had a lung transplant. Also, if anyone would like to share their thoughts either agreeing with me or disagreeing with me I invite you to be a guest blogger. I would love to feature you as well. Leave me a comment and I will let you know how to get a hold of me. ***


Shame on me! I didn't document the sites properly throughout this post. But I put the sites below that I did use:

http://abcnews.go.com/Health/girl-breathing-lung-transplant/story?id=19521453

http://www.nbcphiladelphia.com/news/local/Sarah-Murnaghan-Gets-Second-Lung-Transplant-First-Failed-Family-213556431.html

http://www.organdonor.gov/whydonate/index.html

http://optn.transplant.hrsa.gov/news/newsDetail.asp?id=1598

http://www.chop.edu/service/lung-transplant-program/home.html

http://en.wikipedia.org/wiki/Lung_allocation_score