The Waiting Game

Hello World! It's been a while and I have no excuse, except for feeling a bit boring lately. A couple of readers have requested an update, so here goes...

My health is no better, but not tremendously worse. No huge complaints! I am a bit disappointed that my lung function is back to where it was before I started all of this ridiculous treatment for my Non-Tuberculosis Mycobacterium two and a half years ago. I'm also frustrated that I'm still culturing MAC, but I guess that's the nature of this beast sometimes. I just wish the LOADS of antibiotics that I have been taking for 2 1/2 years straight would have showed some tangible progress. I want clean cultures, Dammit!

In October, I started a clinical trial for the MAC. The trial involves replacing the inhaled Amikacin that I was taking twice daily for once daily inhaled Arikace. The Arikace is the same drug as Amikacin, but it is attached to a lipid that allows it to absorbed deeper and better that Amikacin. The other benefit is that I inhale the Arikace out of a Trio nebulizer, which makes treatment time about 12 minutes. My inhaled antibiotic treatment time decreased by about 48 minutes! I'm really liking the ease of taking the new drug. Another benefit of the clinical trial drug is that it is not supposed to cause the same hearing damage as the Amikacin. The bad news is that even if it's working and I prefer the new drug, once the trial is over in May I have no option to continue. I have to go off of it and wait for FDA approval (years) before I can access it again.

This is the same frustration I'm feeling about Kalydeco. If you are not part of the CF world you may not know about the exciting new drug that has been discovered that basically corrects the malfunctioning CF genes. It was named as the Most Important New Drug of 2012 by Forbes Magazine. For some patients symptoms and progression of the disease have virtually reversed. Lives have changed! The catch...this drug is only FDA approved for 4% of the CF population. Only people with a certain genetic mutation have access at this point. I'm not included in that small group. The good news is that Kalydeco is being trialed in those with my genetic mutation ~ Delta F508 & R117H ~ and the trials have shown impressive improvements in some of those involved! I want this drug and I want it now (said in my best Veruca Salt voice). Unfortunately, I have to wait until this drug is FDA approved, as well. My doctor said this may be another 2-3 years.

So here's the deal...I am very slowly watching my lung function decline, but still holding my own. All while I'm waiting for clinical trials to progress and the government to give me access to these drugs that could rock my world. I asked my insurance company if I could go on Kalydeco off label, but they were not impressed with the $300,000 yearly price tag. I am going to appeal and fight for access to the drug, so we'll see. I feel like I'm desperately trying to save as much lung function I can before my sliver of hope arrives.

Other news in my world... I retired from my career. Whew! This was an extremely difficult and emotional decision for me to make. Trying to juggle a full-time job, 2 (sometimes 3) children, a husband and Cystic Fibrosis has always been chaotic. For many, many years I thrived on the chaos. It made me feel like I was able to do it all. This became more difficult after the NTM diagnosis 2 1/2 years ago when my treatment plan became much more complex. To be honest, I've been faking it for the past couple years. I have been exhausted to the core, sick and struggling. When something had to give, my health got put on the back burner. I knew that this couldn't continue. After a particularly exhausting business trip that left me with another lung exacerbation I decided to go on Short-Term Disability and explore my options. That was in early August. I never went back to my job. I retired...a bit earlier than I expected I would, but I knew it was time. I had the privilege of being with my company for 13 years. I really felt like I had done everything I wanted to do in my career up to this point. I didn't have aspirations to climb the ladder any higher. After 5 1/2 months of trying to determine if I should go back to work or not, I decided that now was the time to devote everything to my family and my health. I am very happy with my decision now, but, jeez, that was a stressful and difficult decision to make.

I guess I had more going on that I thought! I am going to try to post a bit more often. I've missed my readers...

Here's a little song that's been speaking to me lately.

Boiling Over

Warning!!! I may complain a little in this post. It's where my head is right now. If you are looking for happy thoughts you can skip ahead to the very last paragragh. Happy reading!

Do you ever feel overwhelmed to the point where you feel like you can't make good decisions? Do you ever feel like your running in place, and getting nowhere? Do you ever feel like you're trying to do everything for everyone, but you end up doing nothing well and ignoring your own needs?

Unfortunately, these feelings have been crowding my my life as of late... My surgery went well in May, but it's now two months post-surgery and my body feels broken. A few weeks ago I got the news that my MAC (Mycobacterium Avium Complex) is back. What sucks is that I never even got to a point where I could take a break from treatment. At this point I'm considered as having refractory MAC. This is such and evil and tricky (and acary) bug. The good news is that there is a new clinical trial (Arikace)starting within a month for people with Refractory MAC. I am first in line at my CF center to start this clinical trial. I'm hoping that this inhaled drug will beat this bug into submission.

(this is one day of antibiotics)

The bad news is that I have been feeling lousy. To say that I have been fatigued is an understatement. I had a clinic visit last week. I got put on another antibiotic. That means that I now take 5 antibiotics a day. For 2 years I have been taking 4 antibiotics a day, yet the infection is still brewing. How is that possible? I am now taking inhaled Amikacin, Azithromycin, Ethambutol, Doxycycline, and Moxifloxacin. I have tried the power of "positive thinking" to no avail. Will this infection ever succumb? Why am I so, so unbeleivably tired? Will my belly ever get used to the insult of all of these medications?

After feeling really, really lousy for several months, I finally decided that I had to take some time off of work to get my health in check. I need to focus on my treatments (all 4 hours a day of them) and this clinical trial. I have pushed and pushed and pushed through...for the past 2 years of this treatment. I have told myself that I can do anything I put my mind to. Lately, my body has decided to revolt. I have finally decided to listen to my body, instead of trying to convince myself that all is well. Growing older with a progressive disease can be really, really hard...

I turn 37 later this month! That's life expectancy for CF, isn't it? I'm looking very forward to blasting past "life expectancy"!

Hollow Inside

Hello out there!  Hello?  Is anybody out there?  It's certainly been a while.  I was asked twice today for an update, so I figured it was a fine time to dust off the keyboard and write a little.

Life has been busy.  Life is always busy, so this isn't really new.  I've been traveling a lot for work, with a fun family vacation to Florida thrown into the mix.  We went to the Gulf Coast of Florida again this year.  It was a great time!

The week I got back I traveled to Wichita, Kansas for work and got to meet up with Justine K., a recent (1.5 year) double-lung transplant recipient.  I adore her!

On the health front my lungs have been pretty stable.  I continue to take enough antibiotics to cure a village, but the side effects are manageable.  To be honest, I have been dealing with more non-CF health issues lately.  I am preparing for surgery in 3 weeks.  I am preparing to have my 3rd organ removed.  I will soon be saying Good Bye to this:

Out of respect of the one or two boy readers I have, I will spare you the gory details.  Half of me is looking forward to being rid of this nuisance of an organ, while part of me is a tiny bit sad...feeling as if I'm losing my womanhood.  Another part of me is wondering how many non-essential organs a girl can do without.  This would be the 3rd organ I will be ridding myself of.  My uterus is preceded in death by my gall bladder and appendix.  I'm tempted to ask if they can take my spleen and tonsils while I'm under, if only to avoid future organ removal surgeries.  I really hate surgery.  The night after laprascopic surgery can be a bitch.

I was starting to feel a bit bummed about these circumstances.  I was feeling old...something I typically feel privileged to have achieved.  The newly discovered gray hairs that I found last month didn't help the situation.   I was feeling like I've gotten to a point in life where normal things are breaking down.  Then I was feeling a huge amount of guilt for feeling this way, knowing that every year has been a gift.  I was having such a complex mixture of emotions.

My funk didn't last long at all, though.  Justine helped tremendously in reversing this attitude.  Justine had the procedure I'm about to have one week prior to our meeting.  She is 10 years younger than me.  About six months ago she had feet of both her small and large intestines removed.  A year and a half ago she had both lungs removed and replaced.  She has also had her gall bladder out.  Justine  has definitely got me beat when it comes to organ loss.  Just meeting her gave me that dose of perspective I needed. Have I told you yet that I love Justine!

I returned home from Kansas, dyed my hair and changed my attitude.  The surgery is not such a big deal in the scheme of things.  My uterus has served me well, but it's time that we part ways...and I have now come to terms with it.  I'll take all the good juju you can spare!  I'll keep you posted...

I Won...I Won!

I was so excited when I learned that my dear friend, Jenny, author of My Life as a Livingston recently chose me for a Liebster Blog Award.  She chose ME! I adore Jenny's writing style, her sense of humor and the honesty that pours off of the page with every post.  She is absolutely one of my faves, so I felt honored to be selected as one of her faves, too!

Liebster is a German word that literally translates to "favorite", "dearest" or "beloved". By one blogger giving it to another, they are essentially saying, your blog is a favorite of mine.

The rules of the Liebster Blog Award are:

1) Acknowledge the blogger who gave the award by linking back to them

2) Give this award to 5 other bloggers (who have fewer than 200 readers), and let them know through a comment on their blog

3) Post the award on your blog

4) Best of all - bask in the glory, have fun and share the love!

The process of choosing just 5 favorites was very difficult for me.  I enjoy all of the blogs I read or I wouldn't be reading them.  However, these are 5 blogs that really float my boat.

1)  Welcome to Joshland ~ Josh is a great friend, a fabulous guy and a tremendous advocate within the CF community.  I have great respect for Josh and his puppet-friend, Moganko.  Josh's posts are usually heartwarming, often hilarious and always brutally honest.  I like it...I like it a lot!

2) Unknown Cystic ~ Who the hell is this guy?  I dunno?  Whoever he is...I adore him!  Unknown, has decided to keep his identity a secret, which likely allows him to speak his mind and share details about his life that are sometimes jaw-dropping.  Unknown's posts make me laugh...ALWAYS...even when talking about the most horrific situations.  There's just something about that mystery man!

3) Lizi's Lifeline's ~ Beautiful...it's just beautiful.  Lizi's posts read like a poem or an amazing piece of literature.  Lizi is a writer and she has CF.  She has the ability to put many of the feelings that we share into words that make me never want to stop reading.  I devour her posts.  Lizi is taking a bit of a blog break, but I will be right here waiting when she returns. 

4) Life in the Pickle Jar ~ Kelly is the mother of three children, two of which have CF.  Kelly is HILARIOUS!  She does not mince words.  Kelly lays it all out there...the good, the bad and the ugly.  Kelly recently moved her family from Iowa to Cordoba, Spain.  I have loved following the adventures of her and her clan!

5) Dollars and Sense Decor ~ Nope, it's not a CF blog.  Tracy is the mother of one of my daughter's classmates.  She lives nearby and I like to live vicariously through her.  She is crafty (which I am not), she is a decorating queen (which I am not) and she's got amazing style (which I do not).  Her blog is tons of fun, which I look forward to, since most of the other blogs I read deal with heavy stuff.  Tracy rocks!

 Check these blogs out!  You won't be disappointed!

Loss

What a week...full of loss and mistakes on many different levels.  This week has left me with a heavy heart and a questioning soul. 

First, a quick health update.  I am feeling really well.  Last year I participated in a clinical trial for the COPD drug, Spireva.  I was on the drug for 10 months.  In December I was called by my research coordinator asking if I could come back to participate in another little study related to the first one.  There were two arms of the initial Spireva study...one for people with CF and one for people with COPD.  It turns out that several participants in the COPD arm of the study died of heart-related issues.  Therefore, they wanted to bring several people back to go back on the drug for one more month, while monitoring the heart with EKG's at several intervals during the month.  I spent part of my Monday at my CF center having repeated EKG's.  I have a really low heart rate...always have and always will.  However, it was even lower than normal.  We didn't get a reading above 42 beats per minute on Monday.  In an effort to not hurt my hearts feelings I choose to call it "highly efficient" instead of "slow". 

I'm still trucking along with my 4 antibiotics.  I'm still not seeing a great deal of side effects.  I still HATE doing my nebulizer treatments twice a day.  I'm still thankful beyond words that my health is stable!

On to the biggest mistake of the week.  After years of begging, I finally gave in and got my kids a dog.  If you read my previous post, you know that my kids have horrible lungs, asthma and allergies.  However, I did my research.  I looked for dogs that were the most hypoallergenic, we visited the puppy twice before we took her home to ensure that the allergies wouldn't be an issue and we took the dog with the caveat that this was a trial to see if our lungs agreed with this pup.  We had the puppy, the most adorable yorkipoo ever, for a total of 24 hours.  My eyes burned the entire time we had her, but more worrisome was that my son...whose health we just got stabilized a few weeks ago...started having asthma symptoms again within hours of bringing the dog home.  I made the decision to give the dog back swiftly and she was back where we got her within hours.  BUT not without tears.  My son didn't seem to care much, but giving up the dog was equivalent to ripping my daughters heart out.  She cried for hours on end.  ***SOBBED***  This made my heart break.  All I wanted to do was make my kiddos happy and I ended up making one sick and the other sad.  This was a HUGE mommy-fail on my part.  I should have never even gone through with the doggy trial.  I'm left kicking myself and second-guessing my parenting choice in this instance.  Emma is better and seems to understand why we couldn't keep the puppy, but my son is still not well.  I had to start another prednisone burst.  Why, Stacey, Why?!?!

Let's move on to Thursday.  I got a call from my mother that the end was near for my grandfather.  This is the only grandfather I have ever know.  My maternal and paternal grandfather's both passed away before I was born.  My grandmother got remarried to Merv at least 25 years ago.  My memories of my Grandmother all include memories of Merv.   Merv has had a long life of over 91 years.  He has been deteriorating over the past few months.  On Thursday he was rushed to the ER due to his inability to keep his blood-pressure up.  I took off an afternoon of work and went to sit with him.  I held his hand and told him that it was okay to let go.  I reassured him that we would always take good care of Grandma.  I listened to his stories...some of which made sense.  Apparently, "they" are writing a story on the ceiling that he has been reading.  It's kept him entertained the last several days.  I honestly think this is lovely...that when his mind is deteriorating it's causing his to see stories on the ceiling.  Merv is an author.  He has written a few books, including "We Had a Shore Fast Line".  Reading and writing have always been his thing.  I am so glad they can still be his thing as his life winds down. 

So I said my good-byes and Merv was moved to the ICU.  It was determined in the ER that his white blood cell count was very high, so he had an infection of unknown etiology.  He was started on IV Zosyn before I left.  However, the prognosis isn't good.  Both he and we want no extraordinary measures.  It was explained to us that antibiotics are not considered an extraordinary measure.  They are treating him, but there is still not much hope for recovery.  It is very sad to see a person get to this point in life...where there is nothing left.  I get peace in the fact that he is feeling no pain.  So basically we are preparing for a loss to come, probably within the next few days.

I got a call from my mother last night telling me not to visit Merv in the hospital anymore.  Apparently, the raging infection is c diff.  CRAP!  If you know my story you know that I have been hospitalized for c diff.  That particular infection is not my friend.  It's evil!  Once you've had it, you are much more susceptible to getting it again.  They are now requiring visitors to mask, gown and glove before entering Merv's room.  CRAP!  I kissed him when I arrived and I held his hand with my ungloved hand for an hour.   I don't regret doing these things...I would do it all again, but I should have not been so lax in protecting myself.  I should have masked, gloved or maybe even scrubbed.  I did none of these things.  So, we will wait and see what happens.  Last time I got c diff the symptoms came on about a week after exposure.  Now, we wait...  CRAP!

Finally, just today one of my CF friends took her final breaths.  Kristi was a beautiful friend.  She frequently checked in on me, even though her health was so much worse off than mine.  She was a kind soul.  She was friendly to everyone.  She was waiting for a new set of lungs.  She didn't make it long enough to receive those lungs.  Kristi and I talked about meeting one day when she was in Cleveland, since she was going to get her transplant at the Cleveland Clinic.  That never happened.  Kristi was 29 years old...  My heart is heavy after hearing of this loss.

This week has been full of praying for guidance, praying for comfort, praying for healing...praying...praying...and praying some more.

What Would You Do?

I've never really shared information about the health of my kids on here before, but I feel like I'm at a point where I need to make some decisions and I'm looking for some advice.

Both of my kids...Emma (age 9) and Jacob (age 5)...have had troublesome lungs their entire lives.  Prior to having children my husband was genetically tested to see if he was a carrier of CF, which he was determines not to be.  At that time they only had the test that identified 83 of the most common genes.  Now, they have a test that can identify more than 120 genes.  I had always been fine with the 83 gene test, especially after both of my kids were sweat tested and came back with relatively low scores.  They are both carriers of CF, but they do not have CF.  This is what I have always been told, so this is what I have been going with for years. 

However, lately we have been less and less able to control their Asthma.  Since October 1st my son has had 3 prednisone bursts, 2 ER visits and 4 rounds of antibiotics.  We can't find the antibiotic that will clear his lungs. We're currently on Biaxin.  He honestly sounds like a full-fledged CFer.  My daughter has had one round of steroids and one round of antibiotics, but she has been needing to go to the nurses office during the school day almost every day for albuterol and needs her rescue inhaler from time to time at home.

I had been taking them to our general pediatrician who has some experience in Asthma for several weeks.  More recently I was able to get them in with their pulminologist.  I adore their pulmonologist.  For many years he was a CF doctor at the CF clinic I go to...  Unfortunately, my children can't go to my clinic, since they have insurance through my husband and he works for (and has insurance that only covers) the competing hospital system.  I feel comfortable taking them to this pulmonologist, though, since he has a CF background.  I feel like he would be able to see if there is ever a connection between their lung issues and my own. 

So, we get to the appointment and the kids have their initial Pulmonary Function Tests (PFT's).  The goal is that they reach 100% of the predicted value that was determined by their age and weight.  My daughter went first and blew 70%.  My son did worse, only blowing a 58%.  I was SHOCKED.  The little guy is only 5 years old...how can he only have 58% lung function. 

The doctor explained that with Asthma they focus less on the numbers and more on the change that happens after albuterol is given and the tests are repeated.  The change for my daughter was significant.  She went from 70% to 100%!  This pattern showed standard Asthma.  We switched around some of her daily medications and increased the doses of others.  I felt very comfortable with this plan. 

My sons PFT following albuterol only increased a bit...he went from 58% to 62%.  This frustrates me.  The doctor explained that it is likely because he is going through an acute illness and when we can get that figured out with the right antibiotic he should improve.  I don't know...

The doctor was limited in his time, but I asked him if my kids...Jake in particular...might have something I have read a little about call "atypical cystic fibrosis".  This is a condition where someone displays CF symptoms when all tests reveal that they are only carriers of the disease or they have non-typical symptoms.  My children are both carriers, this we are sure of.  The doctor said that the lower sweat test results indicate that atypical CF is likely not the case.  He said we could genotype them when I come back in a few months to determine the type of gene they got from me. 

The other thing I have thought about is whether my kids (particularly my son) may be dealing with the mycobacterium that I've had for a while.  I know non-CFer's can contract this infection.  My kids have been exposed to the same environment as I have, so they likely have come in contact with myco.  I'm kicking myself for not asking for a simple culture.  My son certainly has enough of a "sample" to provide.  My own CF doctor told me to get this done during my kids appointment and I completely forgot. 

Since the appointment I haven't felt satisfied.  I have been wondering if sweat tests are ever false negative.  I remember the nurse during my sons sweat test commenting on how she was unsure if she got enough sweat for his sample...the cotton was pretty dry.  Could this make the reading lower, or would it just turn out to be unreadable?  Does it matter if they label what they have as atypical CF?  Would their treatment plan be any different?  Would they benefit from being treated more like a CFer?  I'm just not sure... 

I'm looking for advice on what you would do in this situation.  I don't want to be one of those "difficult" mom's by continually bringing up this issue, if I've already been told it's not likely.  However, my gut tells me there might be more to the story with Emma and Jakes' health.  Johns Hopkins is doing a lot of atypical CF research.  Do I even go that far to get another opinion? 

What would you do???

Why I Fight...Blogger Challenge

Why I Fight for a Cure for Cystic Fibrosis...

When I was born in 1975 the prognosis for Cystic Fibrosis was very bleak.  I was always painfully aware of the current life expectancy and I always had a hard time envisioning life beyond that age. 

Yet, I know that I have shared here before that I feel like one of the "lucky one's".  I have worked hard at staving off CF and for the most part it  has steered clear enough for me to reach milestones that my parents once thought would have been impossible. The current life expectancy is 37.4 years.  I turn 37 next year.  I no longer have trouble imagining a life beyond the life expectancy.  I feel pretty certain that CF won't take me anytime soon. 

So why do I fight for a cure?  I fight for a cure so the next generation of kids with CF can suffer less and live longer.  I fight so new parents of babies with CF don't have to worry about out-living their children.  I fight so this nasty disease can be beaten into submission.  I also fight for all of the amazing men and women I have met with CF who work so very hard every day to stay alive.  I am hopeful that someday in the neat future we will all be able to live without having to work so hard at it...

Please take a look at this video that was put together by my friend, Emily's, family.

Emily's Entourage Video

Then take the challenge below!

Here are your official rules for participation:
1) View Emily's amazing video here, then consider making a donation of your own or "liking" her page on FB (neither is required, but both are encouraged!).

2) Create your own blog post, FB status update, or other form of social networking tool (letter, email, whatever). In the body of the message, place a small paragraph of why YOU fight for a cure for cystic fibrosis and why this cause matters to YOU. This can be your CF story, your wish for the holidays, your version of community -- whatever.

3) Link to Emily's Entourage Website and encourage your own readers to take up the challenge.

4) Comment on Piper's blog here with a link to your blog a message about how you shared this vision for an automatic entry into a drawing for a very special CF/transplant-awareness prize package, including gifts from iheartguts.com, apparel, and other fun goodies!

5) Re-post these rules on your own page.