CF Awareness Post Question Day 27

How does having Lynch Syndrome affect your CF? And vice versa?

For those who are not sure what Lynch (HNPCC – Hereditary Nonpolyposis Colorectal Cancer) is I will tell you.  It is a genetic predisposition to certain types of cancers.  They include colon, anus, pancreas, brain, sebaceous glands (skin), uterus, ovaries, bile duct, urinary tract, stomach and small bowel.  Basically the digestive organs and some reproductive organs. (I have a blog post about it here: http://mycfjourney.blogspot.com/2009/07/my-colon-cancer-screening-visit.html I apologize for the text, I have changed my blog layout since it was written!).  My family carries the hMLH1 mutation, but there are four total.  A strong family history means you should probably get tested for the gene.  This quote from the print out I have will help calm anyone out there that is thinking OMG my great granddaddy had colon cancer, and that was it, in my family! “Persons at risk for HNPCC usually have a family history of two successive generations of colon cancer or at least once generation with cancer and one with polyps.” So if only one person in your family has had colon cancer, passed the age of 50, and no one had has polyps, chances are slim you have the gene. NOT saying you DON’T, just saying chances are slim. And remember I am NOT a genetics counselor nor am I a doctor!  If you are concerned that you may have this in your family, talk to your PCP and find a genetics team to discuss your family history with.  They will tell you if you need to be tested or not.  My family history is every generation has had colon cancer and colon polyps on my dad’s mom’s side.  This includes my dad having colon cancer when I was 26 years old, and only a few months old.  So I grew up with knowledge of both CF and Lynch Syndrome (didn’t know the name of LS just that colon cancer was hereditary).

Lynch affects my CF in a lot of ways.  I need to have yearly testing right now to be sure I am not growing any cancerous cells in my body.  Yearly I get colonoscopies, thyroid ultrasound, upper endoscopy and a skin checkup.  Having CF complicates my colonoscopies and upper endoscopies.  A prep for someone without CF only takes the day before the procedure.  If the procedure is on Thursday they stop eating Tuesday night, clean out Wednesday and are nice and clean for Thursday.  Not for me.  I need to stop eating Saturday night and eat clear liquids/foods on Sunday.  Then Monday, Tuesday and Wednesday I have to clean out.  And even with this I am not always “perfect” but enough to get good results. 

Lynch has also played a significant role in my evaluation for a double lung transplant.  The team at BWH was not too sure what to do with me.  They had many meetings and in the end decided I would be an OK candidate.  Under the strict terms that once I am transplanted, those yearly tests become six month tests.  There is a much higher risk for cancer post-transplant because of the immuno-suppressants so my risk will be even greater than that.  As it stands now with my yearly colonoscopies, there are always 3+ adenomas polyps removed (those are the ones that if left to fester can turn into cancer).  My whipple done last July was done only because the transplant team did not want to take the risk that I had precancerous cysts on my pancreas.  They removed me from the list until it was figured out, and it could only be figured out by doing the whipple.  So I had to have this huge surgery to be sure.  I was lucky and have had no real complications so I am fine with having had it done now and thankful they were only CF cysts.  I also had a full hysterectomy in 2011 because of Lynch.  Many years of reproductive issues coupled with my desire to not pass my genes on to children led me to have all my reproductive organs removed.  

CF Awareness Post Question Day 26

I took a break from CF awareness this weekend to celebrate Memorial Day and remember why we celebrate.  God bless the fallen service men and their families.

I know you like the winter months the most. Having CF is there any seasons that are better for you. Or months.

I love the snow of the winter months but my lungs do not like the cold!  I was hospitalized this past January primarily because of the awful cold weather we had making my lungs constrict so much I was requiring 3L of oxygen while exercising.  Normally I would need none for what I was doing. 

The summer isn’t too bad for my allergies but the excessive heat makes me lose more salt than I normally do and I dehydrate faster.  And the humidity makes it difficult for me to breathe. 

In spring the allergies get to me a lot. 

Fall is probably the least awful for me.  Luckily it is my favorite season.  Allergies are at bay, for me, and the really cold weather hasn’t hit full force yet.  

CF Awareness Post Question Day 21 and 22

Day 21 I asked for more questions and I got them!!!

Day 22:

What was it like for you to travel outside the US having CF??

In all honesty it was not bad at all.  I had my doctor write me some notes for all my medications and in case I needed to be seen at a local hospital.  I had researched hospitals in the cities we were visiting to see if they had CF centers and wrote them all down.  Just in case of course.  I had to pack extra medications in case we were stranded there longer.  I had to be sure I had conversion plugs since the sockets over there are different, though you have to check with the specific hotels since I think some voltage might be different.  It’s been a while lol.  I took fewer medications then too.  I didn’t do my vest so I didn’t have to travel with that which made it easier for me. I had all my medications in one bag in a carry on so that nothing would be lost.  Plus a medication bag does not count as luggage! 

As far as the trip itself, I was much healthier then and was able to do a lot of walking which we did.  Now I look back and the thought of doing all that walking makes me want to sleep for days!  We also scheduled some down time.  In Florence we were there for 6 days and we have activities planned for 4.5 of them.  So one day we had nothing planned at all and we lounged at the hotel and relaxed.  It was good for both my mom and I to rest.  We did 5 cities in three weeks which was perfect.  Two of those days were for travelling to and from Europe.  We took the train in Italy and planes from Paris to Rome and Florence (I think we took it from Florence, we may have traveled to get to the airport I cannot remember) to Prague. 

All in all, as long as you know your limitations and you can fly then it is worth the trip.  Of course this was to Europe.  I don’t know how it would be traveling to a third world country the Americas (Latin and South).  

CF Awareness Post Question Day 20

You have had experiences of a volume and magnitude with the health care system that few have. I'm interested in your insights.

As a nurse and a patient, I've experienced both sides of the coin and I'm aware of bad apples, general malaise and the "systemic" characteristics of a system with funding issues.

What I'm most curious about are your experiences. Are there things which healthcare workers unwittingly do or say which aren't helpful? Do you dread your interactions with the medical system? What are you overall impressions?

Being a patient that is in the hospital for extended periods of time on a lot of occasions you learn what you like and dislike.  A lot of the things I don’t like I suck up and deal because it is hospital protocol.  For instance, I use the VEST while inpatient and do not need to see a respiratory therapist for manual chest physiotherapy, though some CF patients do get this while in.  However, they still have to check on me twice a day to be sure I don’t need anything from them.  So each time they come in they listen to my lungs and ask me the same questions.   It’s very monotonous lol.  And annoying!  Especially when some of them come at 8am and you are still in bed.  But they have to do it and it’s what I get for being there for two weeks. 

As far as nursing goes, there are certain nurses that I dread getting and it’s like roulette when I am in.  Will I get her or not?  Some of the day nurses come in at 7:30 and act surprised I am still in bed.  They say things like “oh you’re still sleeping?!?!”  or “I figured you would be up by now!”  Hello it’s only 7:30 am and I have nowhere to go so why would I get up???  Others are very strict about meds etc.  Most nurses give you the day’s supply of enzymes knowing that we are able and willing to give these to ourselves when we eat.  But there are other nurses that insist you page them each time you order food or eat.  It’s annoying for us and I am sure it is annoying for them, though they do ask for it.  Sometimes nurses forget to hook me up to my IVs at the times I am supposed to.  I don’t get mad I just call.  They get busy and forget.  It happens.  I usually only have one or two “bad” nurses each stay, and considering how many I see that is good odds.  Some of the nurses I LOVE and it’s like a holiday when I get them!  A great nurse can really make your stay sooooooo much more “enjoyable.”  And on the flip side, a really shitty one can make you want to go home immediately. 

I don’t dread seeing my doctors at all.  I know that they are they to make me heathier.  The hospital itself I am not a fan of but I am hoping to remedy part of that by switching centers.  Then I can be seen at a different hospital.  My current hospital, I prefer to do a full two weeks in and they prefer me to do it at home (by they I mean the people in charge of the hospital….they tried to move the CF patients to an entirely different hospital because we cost too much money but instead they came to a compromise with the CF clinic where anyone under 35 does inpatient at Children’s and anyone over 35 or over 18 and seen by the transplant team stays in patient at Brigham and women’s).  But financially I cannot do my stays at home and it is a fight each time I go in for me to stay.  That results in my feeling like an unwanted house guest.  So this new hospital is perfectly fine with you being there the two weeks and won’t make me feel like I am unwanted (so I have been told!!!). 

I’ve learned to juggle my life around the healthcare system.  I know that if I have an appointment at 11am I should not schedule anything else before 2pm or later depending on the doctor.  I know that if I am being admitted I will most likely sit in the waiting room for at least an hour and I should bring stuff to occupy my time.  I know that most doctors run late and it isn’t always their fault (sometimes patients ask A LOT of questions and I would rather them answer the questions than make someone feel rushed.  I might be that person one day and would hate it!).  I know there are a lot of faults with the system that need to be changed but they might never get done.  I can’t avoid it so I need to learn to deal with it.

CF Awareness Post Question Day 19

Here's a question: are there any dietary restrictions?

There are no real restrictions, but some individuals can tolerate certain foods better than others, like the healthy population.  People with CF need a higher calorie and higher fat intake than the average person.  We burn more calories so we need to take in more calories.  This is for those who are pancreatic insufficient, meaning the pancreas does not break down food like it is supposed to.  Some people notice that dairy products make them produce more mucus so they avoid milk and ice cream. 

Growing up I was told to eat eat eat, anything and everything.  My mom would put butter on everything.  We would search the food store looking for high fat foods only ever finding low and no fat.  You would be surprised how hard it is to find good foods that are high in fat and calories (by good I mean healthy and not chips, snacks and junk foods).  To this day it is hard to control my eating because of how I grew up and needed to grow up.  I never had to have self-control with food, because there was no need for it.  So if I felt like eating a container of ice cream I was allowed (I don’t think I ever did lol).  This is an area in the CF world that is slowly changing.  Stressing not only high calorie and high fat foods but also healthy foods.  Especially if growing old is an option now.  If you think you won’t live to see 30 you aren’t so concerned with heart disease etc.  But if you are going to live to be 80 you need to worry about those things! 

Gaining weight is an issue.  We struggle every single day to maintain our weight let alone put weight on.  I have been fortunate enough as I aged (thanks menopause!) to not have to worry so much about my weight.  But up until I was 30 I struggled for every ounce I gained.  By the time I lived on my own, I had a calorie regiment that would blow your mind!  I repeatedly heard the “you must have a hollow leg” comments or “where do you put all that food?”  Breakfast was light as I have never been much of a breakfast person.  But all day long I would snack and eat like it was my job, even while working, because it WAS my job.  I would eat snacks almost every hour if not more.  I brought two bags to work, one for food and one for work stuff.  Yes an actual bag dedicated solely to food and a drawer filled with snacks and cheese dip (I loved cheese dip on pretzels!).  I spent small fortunes on food and supplements.  I tried all types of high calorie milkshakes to eat throughout the day, coupled with snacks and dips and meals.  I would come home and eat more and then eat dinner and then have a snack before bed.  And even with all of this, I was lucky to be 107 pounds.  When I graduated high school I weighed 104 pounds.  By the time I was 25 I had only gained 3 pounds in 8 eight years.  For reference, since weight has stopped being a big concern for me, right before my pancreatic surgery last July I was 132 pounds.  I was a normal weight for someone my size.  Now I am 114-117 pounds depending on the day, time, etc. 

Many times I would stop eating because it was not fun for me.  It was a chore; it was a treatment for me. It was just like all the medications I was taking, except that I had to do it continuously. (this has changed now that I stopped worrying about my weight, I love food again!)  Sometimes I would dip below 100 pounds and I would stop getting my period.  I took many pregnancy tests thinking I must be pregnant, only to realize I was fighting something off and my weight had plummeted.  I was lucky again in the fact that my weight never got dangerously low where I needed to get overnight tube feedings via a g-tube.  But I know many people, as adults and children, who have gotten tube feedings in order to just maintain their weight.  It is a real struggle. 

And it is a struggle that many healthy people think is great.  Hearing “oh you are so skinny you must love it,” or “but at least you are thin,” are NOT helpful.  Twigs as legs and arms, they are not pleasant.  A barreled chest (common in CFers thanks to lung disease – http://www.mayoclinic.org/diseases-conditions/copd/expert-answers/barrel-chest/faq-20058419 ) makes us feel abnormal and weird.  Especially when you have skinny arms and legs, you feel like E.T.!!!  This country, and many others, thinks that weight issues are only a problem on the other side of the spectrum…those who are obese and overweight.  But super thin is a weight problem too.  

CF Awareness Post Question Day 18

I know the Cystic Fibrosis foundation as well as other organizations have funded research and development programs specially aimed at identifying innovative new therapies for CF. As a patient do you believe these programs have added value? Have you been approached to enter a clinical trial for a new therapy?

I definitely believe they do!  They have a drug out right now that “fixes” the genetic issue of CF.  It reopens the channels across the cell membranes leading to almost normal function.  This drug is only for specific mutations but they are working on more for others.  This drug can and has helped people live much more normal lives – healthier, able to keep on weight, less to no lung infections.  For some, this drug could be the “cure” that people want.  But that depends on your definition of cure!!!

I have participated in many trials.  Not so much lately because they usually have a 40% FEV1 cutoff and I miss that mark.  But in the past I have been involved in a bunch.  And actually right now I am in one just where they collect your mucus to examine it once a year, only requirement is culturing Pseudomonas.  Even these little studies help the larger picture.  I feel that it is my duty as a person with CF to participate in trials that I feel comfortable participating in.  There is a small pool of people that can participate so we need all the volunteers we can get to do so.  Foundations like the CFF can do research and studies galore in the lab, but if there is no one to test these on then they become moot.  I don’t do these trials so much for me, I don’t know how much of them will actual help me in the long run (I did an inhaled Cipro study 7-8 years ago and had great results but since then my lungs have decided to not like inhaled antibiotics so that med will never help me out), but more for future generations. 

Even though this is not a CF specific study, I am currently enrolled in a colon cancer research study.  Its 6 months of taking a mediation daily to see if it helps ward off colon cancer.  Anytime I get asked to do a study I participate (unless it’s a procedure that might put my lungs at risk).  The current one I am on I am able to be taken off of if my lung health declines and I need to stop.  I am not allowed to take certain types of antibiotics, and of course those are the ones that I usually try in oral form before doing a different kind of IV antibiotic.  I also like doing studies because I am such a hodgepodge of diseases that they can see the results on someone like me.  I don’t mind being a guinea pig at all!!!

CF Awareness Post Question Day 16

Do people with CF always get diabetes?

Not always.  Because our pancreas is a mess, many CFers develop CF related diabetes (CFRD) which is different than Type 1 or Type 2 diabetes.  I don’t know the ins and outs of any of the diabetes so I can’t really explain them.  Similarly, many, if not all, post-transplant patients develop diabetes because of the need to take prednisone constantly to avoid rejecting the new lungs. 

In my case, a couple of years ago I was given an OGTT (oral glucose tolerance test) which came back as positive for CFRD.  So I received my fun little test kit to test my sugars.  Since then I have remained in the normal range when doing finger sticks for the most part.  I only ever require insulin shots when I am on a prednisone burst/taper.  I actually tend to have more lows than highs and insulin could make this worse.  Ironically, and I have no actual data to back this up just me, the prevalence of lows has decreased significantly since I had that large cyst removed from my pancreas in July.  My A1C, which is the marker for your sugar levels over the past couple of months, is only slightly higher than normal at 6.3, normal being 4.5-6.  But my A1C has gone up to 6.8.  It fluctuates.  At some point I will need to use insulin, whether it is before or after I am transplanted, I won’t know. 

CF Awareness Post Question Day 15

What's the one biggest misconception you get when people hear you have CF?

A lot of people in the non-medical world have never heard of CF before so they have no real misconceptions.  Most of the time they are a clean slate.  Which is of course good and bad! 

Occasionally someone will have a friend whose friend’s child has it so they have heard of it.  Then the misconception is that I am an adult and alive.  Sounds crazy, but CF is portrayed as a childhood disease so people assume it’s a childhood disease and there are no adults with it.  But in 2013 49.7% of people with CF were over the age of 18 (the CF annual report comes out the following year, so we don’t have 2014’s yet).  CF is NOT a childhood disease anymore.  The stats are almost 50/50 thanks to advancements in treatments.  Even in the medical field I have run into nurses who are surprised I am alive and still have my original lungs!  I always give the benefit of the doubt, especially if they are in a field where they may not see CF at all or all that often, and take the chance to educate them on how adults are alive and well living with CF.  But this is something that adults are working with the CFF on.  We want to be portrayed too.  But kids bring in donations and donations are needed to make those kids in to adults. 

Another is that I must be really healthy if I am still alive.  Not true.  The body can endure a lot, CF is a good example of this.  Just because I am 34 and have my original lungs does not mean I am healthy.  Remember, 35% lung function is not good.  It’s not horrible, but it’s not good lol.  For where I am in my disease progression I am healthy, but compared to the average Joe, I am not :)

CF Awareness Post Question Day 14

How old were you when you were diagnosed and what indication was there that lead to being tested?

I was 5/6 when I was diagnosed.  I was small for my age and I had what the doctors thought were bad allergies.  I also ate like my 200lb father and gained no weight.  And thanks to malnutrition, I had that bloated belly you see on the help feed the world commercials.  I did not digest anything so my bowels were a mess.  I coughed a lot, a junky cough.  My mom can tell you more about all of that since I can only relate what I remember being told.  My local pediatrician told my mom to get me tested since he thought I had CF.  This was after all the good hospitals in Philly told my mom I had allergies.  So I was given a sweat test which is the gold standard for CF.  Anything over 60 is positive for CF and mine was 162!  What a sweat test does is determine how much salt is in your sweat.  CFers sweat salt.  In the summer I am a horses dream, a salt lick.  This has to do with the CFTR mutation and the passing of sodium and chloride across the cell channel – too much science that I only vaguely understand lol.  Cff.org has all of the scientific details if you want to read up on them.  Now luckily, most states have newborn screening which catches almost all new cases right at birth.  Getting ahead of the game is very important and now parents don’t have to wait until there is something wrong to begin treating; they can start immediately and have a lot more hope of a normal life for their CFer.

CF Awareness Post Question Day 13

How come you didn't tell us about your CF while we were in school?

This is a great question and one I am sure many young kids will relate to.

In elementary school I was not so shy about it.  I went to the nurse daily for pills, I got out of gym glass when I got my flu shots (I hated gym lol), and over all I was not quiet about it, but I was not shouting it from the roof tops either.  Some of my friends in elementary school knew about it when they slept over because I had weird medical equipment in my bedroom.  But other than that I was pretty whatever with it.  That all changed in junior high school.

Anyone who knew me in school, whether it was junior or high school, knows I was a shy person.  I am still very shy to this day.  But now we have the internet.  And much like the bullies who can hide behind a computer screen, those of us that are shy in person can come out of our shells online.  In junior high and beyond I did not want people to know I had CF.  Being unique is not something most kids in school look on with admiration, and while many can ignore that, I was not one of those kids.  I wanted to fit in. I did not have very many friends since I started public school in 7^th grade and did not move over with many I knew from Catholic school, just some close ones I started to make in my new school.  I was far from popular.  I was far from athletic.  I was artsy and followed that path, but even so I made next to no artsy friends until college.  I had enough for the kids to make fun of more for; I did not want to give them any more ammo.  Of course hindsight is 20/20 and all those things I thought I could be made fun of for, were probably all in my head.  But some were not.

For example, and I remember this like it was yesterday and I still remember the girls who were involved, though I will not say their names (I know their faces not really their names anymore!).  In 8^th grade social studies I sat near a small group of girls who were friends, and of the popular crowd.  One day as I was sitting there doing work (must have been a free period type thing because they were all facing each other) I overheard the girls talking about me.  One of the girls was sick with a cold and was complaining how annoying it was.  I heard another of the girls respond with “it must have been Amy who got you sick, she is always coughing…it’s gross.”  From that day forward I vowed I would never tell anyone about “being sick.” 

And I held that promise, pretty much to this day.  I am not vocal in person about CF.  I will talk about it after people know but I will not come out and say “I have CF” to new people I meet, face to face.  If they see it online then the ice is broken and I can talk about it.  And I am more than willing to get involved and throw benefits and do walks and educate.  It’s very hypocritical I guess you could say.  I also don’t like to say the words “Cystic Fibrosis,” I don’t even like writing them.  I pretty much use CF for everything.

Similarly, I do not like when people tell people about me having CF.  I don’t mind if we are talking as a group and it comes up, but I don’t like when people tell people it to get something.  Again hypocritical I know.  If a friend/family member/co-worker is soliciting donations for the CFF walk and they say I have CF, I don’t mind it.  But if a friend/family member/co-worker uses my having CF as an excuse to get out of doing something or to try to gain something for themselves I don’t like it.  Even if it is to try to gain something for me and my family I don’t like it. 

So in short, I did not tell people in school because kids can be mean lol.  

FDA Advisory Panel Recommends Approving Vertex Drug for DDF508!!!

HERE

What does this mean for me?  This means that if on July 5th the FDA approves this new drug I could be turned around.  My life could go back to normal.  I could get a job, buy a nice house, stop doing so many treatments, grow old with my husband.

CRAZY!!!

CF Awareness Post Question Day 12

Can you have children and have CF?

Yes and no.  Women can have a harder time getting pregnant because of extra mucus around their cervix.  Most CF men are missing their vas deferens which is what delivers the sperm.  They can have children but usually not the natural way (I know a couple of CF men who fathered children naturally so some can).  I have many CF women friends on here who have had successful births via natural and IVF.  I also know many who have adopted instead.  Having children when you yourself have CF can lead to the child having CF as well.  Your partner needs to be screened for CF to be sure s/he is not a carrier.  Even then, there is a small chance that the baby will have CF.  The panel covers the most common types of CF and sometimes a partner may have a rare mutation and pass it on to the baby, along with yours, resulting in a CF baby.  If a CFer has a baby using their sperm or egg, their baby WILL BE a carrier, meaning they will have one mutated CF gene.  If the partner supplying the other half is a carrier there is a 50% chance the baby will have CF.  CF is a recessive genetic disease meaning that there needs to be TWO mutated CF genes in order to have CF.  

CF Awareness Post Question Day 11

My brother asked this question, hence the reason for parts of the answer!

I don't usually read things that are this long, but it was good!

And cool idea. My ? Is where did you come from and how did you turn out so well?

Great question!!!  See I have these two cool parents who decided to have kids, well kind of….They wanted kids but maybe not so soon lol.  But after they had me they decided they needed another one, and they got this weird little boy.  But that was OK, I am glad I have a weird brother.  Though I have been told I asked if we could send him back a few times!!! 

Turning out well may still be out with the jury lol.  But having a kick ass support system helps and being allowed to live life.  I see so many parents wanting to place a bubble around their child to protect them, CF or not.  But that is not fair.  Let your children go to sleep overs, have friends over, go to school, socialize etc.  Because growing up isolated is hard.  I grew up thinking I was the only one who felt how I did with CF.  Not until I went online to find a support group for CF in my mid 20s did I realize I was not alone and was able to make friends with people who GOT me.  If children are treated like they have a disease then they will live like they have a disease.  This doesn’t mean that you should say screw it and miss a week’s worth of treatments, but if your child wants to go to a sleep over and will miss one treatment isn’t that worth it so that they can have a great life, not just a long life?   (this is where CFers and CF moms butt heads ha!)

Happy Mother's Day!!!

Today's post is dedicated to the mother's of "sick" kids.  This does not mean Cystic Fibrosis only.  I mean any mother who is raising a child with any type of "issue" whether it is physical, mental or emotional.

Parenting is tough.  From my few years as a step mother I have learned how difficult it can be, and I didn't even start off with a baby!  I was lucky enough to take on an 8 year old (though the older they are the more issues they come with too since you can't "mold" to your liking lol) and skip the diapers, potty training and terrible twos.  There is a lot of give (you) and take (them) and not much reward.  It truly is a thankless job.  And now that we have a teenager in the house, it is even harder.  You can see the young child in them struggling to override those awful hormones, but hormones win 90% of the time!  At the end of the day, it is still a great job.

For those mothers out there who deal with all of these things and then they raise a child with an illness, well you are saints.  So here is a little thank you from me to you (not all encompassing since I know I will miss a few!).

Thank you for:

taking us to all those doctor appointments
not letting us dwell on our illness
letting us live our lives
not keeping us bubbled up
feeding, clothing, bathing etc us when we were sick
telling us it would be ok
visiting us in the hospital
holding our hands when we got bad news (and good news!)
raising awareness and money for us
fighting for us in schools for what we needed to succeed
watching us grow into the people we are today because of you
helping us figure ourselves out
not letting us give up
being the best support system we could ask for
hugs
kisses
love
kindness
the kick in the ass we needed when we rebelled against the illness
knowing the illness didn't define us and letting us figure out what did
watching as our dreams came true
watching as our dreams crashed down (and holding our hands)
lifting us up
bringing us back to reality
nagging us to do our meds
nagging us to eat
nagging us to play outside
nagging us to be responsible
and well, just EVERYTHING


Please post any thank yous in the comments.

And thank you MOMS!!! (and single dads who have to do both!!!)

CF Awareness Post Question Day 9

Today I am taking a break from a question and posting a fact. Remember everything I post pertains to me mostly. Each person with CF is different, just like each person is different.

Today I am going to explain what lung function is. Thanks to a fellow CF friend for posting this and letting me borrow it from Facebook.

First a lung function, or PFT (pulmonary function test) is defined as: Pulmonary function tests are a group of tests that measure how well the lungs take in and release air and how well they move gases such as oxygen from the atmosphere into the body's circulation.

This test gives the Dr and Respiratory Therapists a reading of several different numbers. There is one number that gives the "over all" percentage of how the lung is functioning.

Now for me personally my "baseline" (what I aim to be at my clinics) is mid to upper 30s. I have hit as high at 37% in the past year, but I hover closer to 35%. This may seem very low compared to 100%, and it is. But there is a lot of factors that account for how you feel when your lungs are at this point. For me, I don't always feel like I have 35% FEV1. Some days I feel like I have 25% and others like 50%. My energy levels can be great really helping me feel good, or I could be exhausted all the time and feeling crappy.

Right now, as I write this I feel great. But I have a secret weapon for a week - Prednisone burst. This little steroid is a godsend for some people. After transplant is helps ward off rejection. In people like me, it helps open the airways and gives me energy. I was helping a friend yesterday and she asked a great question about it. She wanted to know if the week I am on prednisone is a tease - like I feel great for a week and then back to normal. It is and it isn't. Lungwise it is a tease in a sense. Sometimes I get a huge boost from it and feel phenomenal, and other times I get a small reprieve and it doesn't faze me when I stop. Instead I take full advantage of how I feel that week. I do more, sleep less (drug induced insomnia lol), and overall get so much accomplished that after I stop the meds I am happy to sleep for a few days to rest up. But I also forget I am on prednisone and think "wow I feel so good maybe I am turning a corner." And this can be the hard part. Because then you realize you are on drugs to help you that you have to stop in a few days, and then you stop and go back to normal and know that it really was the drugs.

For those that may be wondering, no I cannot be on prednisone all the time. After transplant you need it, and it can causes issues with long term use. Most doctors don't like to use it as a maintenance medication pre-transplant unless absolutely required. And I don't require it all the time, though sometimes I wish I did lol.

If YOU have any questions feel free to leave a comment asking. Each day I will post the answer to one question.

CF Awareness Post Question Day 8

I have a question to add -- how are the treatments helping you? What exactly is being done when you do these treatments and how many times a day/week do you do them?

Without all the medications I take I would not be able to function in society.  At all.  I would probably be dead from the infections that would have grown out of control in my lungs.  And I would have been severely malnourished.  These medications and treatments keep my lungs open and relatively infection free.  They allow me to gain weight and fight anything that may arise in my body. 

I am positive I would have survived until adulthood though.  I was very lax with my regimen until I was in college.  I took my enzymes and sometimes I did my nebulizer (it was different way back when!!!).  I was on oral antibiotics a lot I remember in college but not much before that.  But I do know I would not be alive to write this today had I not shaped up and started to take care of myself!

The remainder of your question was answered already :)

You can also watch my youtube video on my treatments.  Though some have changed in the few years since I made this video (it was done before I was married).  I may need to make an updated one!  (Ibuprofen I am no longer taking thanks to my kidneys not liking it ha!)

https://www.youtube.com/watch?v=nm5ubMkdEOQ

CF Awareness Post Questions Day 7

What type of treatments are available for CF?

There are so many out there right now and many in the pipeline.  For a complete list you can visit

http://www.cff.org/treatments/

I will list what I take and explain why I take them.

DuoNeb – this is a bronchodialator which opens my airways up.  I take it at least 4 times a day when I am feeling good and 5 even 6 if I am feeling particularly crappy.  It is taken via a nebulizer.

Symbicort 160/4.5 – inhaled medication that helps open my airways and keep them open.  I take one puff twice a day.  I have to be sure to rinse my mouth out after using it so I do not develop a yeast infection in my mouth (thrush).

Spiriva – a new medication I just started to also help keep my airways open.  It is taken via a handihaler.  What that means is that there is a small capsule filled with the powdery med that the handihaler pierces and I am then able to inhale.  I take this once a day.

Pulmozyme – helps to thin the mucus in my lungs so that I can cough it up and out and reduce infections in my lungs.  I take this once a day in my nebulizer while wearing my VEST.

Sodium Chloride 3% - an inhaled medication via my nebulizer that I take twice a day while using my VEST.  I use this before I use the Pulmozyme in the evenings.  It also helps thin the mucus.  There is also 5%, 7% and 10% but I cannot tolerate those.

Airway Clearance VEST – a machine that vibrates my lungs and helps expel the mucus in them.  I use it twice a day.  In the morning I use it for 20 minutes with the sodium chloride.  And in the evenings I use it for 28 minutes with the sodium chloride and Pulmozyme.

Accapella – a device that shakes you from the inside.  Helps clear the mucus out.  I use this mainly when I have a plug in my lungs I need to get out and not as a daily therapy.

Prevacid 30mg – Many CF patients have acid reflux from the weakening of their esophagus.  I take these pills once a day.   

Azithromycin 500mg – Oral antibiotic that helps with inflammation.  Also known as a Z-pack in the regular world, though I take it 52 weeks a year.  But I only take it on Mondays, Wednesday and Fridays.

Zyrtec 10mgs – allergy med.  Spring and summer are bad on my allergies which make it much more difficult for me to breath.  When there is visible grass I take these pills.

Singulair 10mg – asthma medication to help with inflammation as well.  This is a pill I take once a day.

Supplemental oxygen at night and with vigorous exercise – my breathing gets very shallow when I sleep, which lowers my O2 levels and increases my heart rate.  Peter will tell you I sound like I am running a marathon when I sleep without O2, which to my heart and lungs I am.  Because my O2 does not get too low, 1L is plenty for me right now.  Same with exercise.  What I do now for exercise I do not need to wear O2, but if I run, I do.  My heart rate skyrockets and my oxygen levels plummet.  Not a good combination.

Creon 24 – These are enzyme pills I take before I eat so I can digest food properly.  I take 3 with meals and 2-3 with most snacks.  My body does not digest fats properly so I must take these pills in order to get the nutrients from them.  It has taken a few years to find the right amounts to take.  I took different enzymes before, but they were not FDA approved and were pulled from the market.  If I don’t take enzymes, well let’s just say no one wants to follow me in the bathroom ;)

Diflucan 150 mgs – With all the antibiotics I am on I get yeast infections pretty regularly.  My Gynecologist has me on a pill a month with the option to get one every week if needed. 

Vitamins – CFers are deficient in the fat soluble vitamins A, D, E, and K.  We also have to take extra calcium.  I take D, E and calcium along with a daily multi-vitamin which has A and K in in too.  In the winter I have to up my D intake to 50,000 units a week.  Normally I take 2,000 units a day (14,000 a week). 

Miralax and a daily Stool softener – Despite the digestive issues when you don’t take your pills, I have lots of issues with constipation as well.  I must take these daily in order to have daily bowel movements.  If I miss more than one or two Miralax doses, I need to do a clean out of my system to get it back on track.  When I say cleanout, think of a colonoscopy prep! 

Probiotic 30 billion – Because I am killing off so many good bacteria, I supplement some each day to help regulate all my inner workings.  Most of the time it works, but not always.

IV Antibiotics – a few times a year I need to combat the pesky infections that are always present in my lungs but which rise up to irritate me on occasion.  I spend 2 weeks in the hospital receiving these meds along with getting lots of rest (and crocheting done).  Many people chose to do these at home and I used to, until my insurance switched and now it is too expensive to do them at home.  But I do not mind.  While in I also get daily exercise, and manual chest PT if I want on top of my vest treatments.  These antibiotics kills off the bacteria wreaking havoc on my lungs and help bring my lung functions back up. 

Oral antibiotics – many patients use oral antibiotics to combat their lung infections.  Unfortunately, my bugs no longer response to oral antibiotics.  This means I have to use the big guns when fighting an infection.

Inhaled antibiotics – there are a few inhaled antibiotics on the market.  These are prime in the CF world because they deliver the drugs directly to the infection via a nebulizer.  TOBI, Cayston, Colistin and TOBI podhaler are the ones currently on the market.  My bugs do respond to these meds, but so do my lungs, and not in a good way.  About 6 years ago I had to stop using inhaled antibiotics because of the severe reactions I get when I use them.  Basically my lungs get really inflamed and I cannot breath and my oxygen levels drop.  So now we don’t even attempt them.  Some CFers inhale IV antibiotics as well.  It is what it sounds like – inhaling the meds that would be given via the vein.  Many swear by it too.  Whatever works to keep those infections at bay!

Transplant – I have been evaluated for a double lung transplant but am not there yet.  Many of my friends are listed, transplanted or passed away before they could be listed or after they received the gift of life.  This is the final step in saving your life.  End stage CF is not pretty and many do not make it to this point.  But those that do and get this chance tell me it is fabulous.  You still have CF everywhere else but your lungs are OK.  However, you are not cured by any means.  You trade one disease for another and a post-transplant patient must take daily pills in order to not reject their lungs.   Lungs are not the only organs transplanted in CFers.  Some need livers and hearts and many need kidneys because of all the pre and post-transplant medications.  

Emily McDowell Studio - Check it out!!!

I was introduced to this site today.  And I already love it!!!

They have empathy cards that say things like "when life gives you lemons, I won't tell you a story about my cousin's friend who died from lemons," or ""I'm so sorry you're sick.  I want you to know that I will never try to sell you on some random treatment I read about on the internet."

TRUTH!!!

There are also great mugs, tote bags and all types of cards.

I need to buy one of everything!  Best part is they are made in the US so supporting some home grown business right here!

Check out Emily McDowell Studio.  You won't be disappointed at all.

CF Awareness Post Questions Day 5

Ok, I have a question .. Even though I am a CFer .. Since I am experiencing this at this moment I thought maybe you could tell me what you do when you are having bad pain in your lung? I think it is muscle related, although it could be a mucus plug... So painful.  What is your best advice to alleviate the pain?

If I think it is a plug I do huff coughing and my accapella to try to get it out.  If it is muscle related I would take Motrin.  Depending on how severe the pain is, it might be worth a call to the docs too.  I have had some bad pain that after a couple of days I called in to be sure it wasn't anything serious.

CF Awareness post questions Day 4

What is one thing that you thought you could never do because of CF that you ended up doing?

Getting married.  I never thought someone would love me with CF and want to spend the rest of my life with me.  There were many many many boys I dated that just couldn’t deal with me being sick and my future being uncertain.  Which has made me appreciate the one man who could even more!!!  And I never thought I would live long enough to get married.  Sure when I was small I dreamed of getting married and having babies and planned on being well on my way with that by the time I was 25, but I never really thought it would happen.  It was kind of a dream, like living on a ranch in Montana lol.  

CF Awareness post questions, Days 2-3

On Facebook I asked my friends and family to post questions to me about CF or my life with CF.  I have gotten some great questions!!!  So each day I will post the question and answers here too.  I forgot yesterday so I am posting two day!!!

What is the hardest part of your daily regiment living with CF?

Hardest part is trying to exercise faithfully.  Exercise is so vital in staying healthy, but unless you are a lover of exercise it is hard to fit it in.  I try to go at least once a week, which is nothing, but it is still something I tell myself.  Both normal and CF people need exercise, and like normal people, not all CFers like to exercise.  But I try to fit it in as much as I can.  I have my moments.  There are times when I can go 5 days a week to the gym for 6 months and then not go for 4 months.  Being active is vital in helping to clear the lungs and keep infections at bay.  Plus it strengthens your body for when you may need a double lung transplant.  

I would like to know: What are your thoughts when you see people who don't have CF and they are harming their healthy bodies. Also what are some of the most simplest to exotic things you would do if you didn't have CF.

It doesn't faze me too much when I see people harming their bodies.  Each person harms their bodies in their own way.  I drink soda and eat candy like it’s my life source.  Someone who is full blown diabetic may think I am crazy (I say full blown since technically I am diabetic I just don’t need insulin yet).  Smoking is probably the closest I come to getting “annoyed” at, but even so, I married a smoker, my mom and dad smoke, some of my friends smoke.  And even with that it is more for the end result, that their lungs will not be useful for someone when they die.  They can’t donate them.  But each person was given what they have and makes do with what they were given.  Every one’s life is theirs and they can live as they choose.  As long as no one else gets hurts in the process then c’est la vie!!!

If I didn't have CF I would probably have a biological baby or two.  I like to think I would still be an Interior Designer but working for my dream company designing hotels and casinos.  Riding horses all the time.  Maybe living on a ranch in Montana like I always wanted.  Maybe I would travel more.  Most of the things I would want to do I have done, or have come close to doing.  Or they are things I had to stop because my health declined.  But nothing really changes in me with or without CF.  I am still me, just a sick me.  It shaped who I became, but the basic loves and hates are imbedded in me no matter what.  

Brushstrokes for Breath CF Fundraiser

REGISTRATION IS OPEN!!!

Brushstrokes for Breath Paint night at the Artist Cellar in Yardley, PA.

Thursday September 17, 7-9:30pm

Pizza, chips, cheese, crackers, soda and water provided. BYOB and any other snacks you want.

$50 a person. 36 people max so sign up fast!!!!!!!!!

Register HERE