In Between

I haven't posted for nearly a month.  What could possibly have kept me out of the virtual world for so long?  In all honesty, I've just been so busy with life that my computer time has been limited.  These past couple of months I have been living in between.  I am an official, card-carrying member of the sandwich generation.

I have a very small family in town.  My mother was an army child and her family barely lived in the US during her childhood.  They lived in Hawaii (before it was a state), China, the Philippians and she met my father as a teen in Nova Scotia. When it was time to return to the US, her parents, brothers and sister all scattered.  Somehow we ended up in Ohio, which is where my mother and father have been since.  We have no extended family locally.  It's just my parents, step-parents and brother. 

About a year and a half ago we encouraged my grandmother to move from Philly to Cleveland to be nearby.  She agreed and moved here to an assisted living facility with her husband.  At that point, my grandma had Stage 4 kidney disease.  Over the past few months she has progressed to stage 5 disease.  At the same time, my mother, who has dealt with severe back injuries and pain, was preparing for major surgery that would require rods, screws and a long, difficult recovery. 

All of this background just to tell you that I have been wearing my caretaker hat over the past couple months.  My mother hasn't needed much.  I actually wish I could have been around for her more during this time.  I made her some homemade soup and took her tons of DVD's, but what she needed most was sleep and rest...so staying away was actually better.

 My grandmother, on the other hand, needed my help.  I've spent a lot of time this past month taking her to doctors appointments, getting her admitted into the hospital and getting her dialysis started.  It was a difficult transition for her, but she's now managing like a rocks star. 

My kids have also had a lot of activities.  The end of the school year is always full of stuff.  Throw in a little bit of work travel and I was left juggling a lot of balls in the air.  Trying to keep everything afloat.  Just managing most days... 

I haven't spent a lot of time thinking about Cystic Fibrosis.  It's been nice!  I've been feeling great!  I've been as compliant as humanly possible.  The Ethambutol seems to be having no side effects.  I had to have an eye exam prior to taking the first dose.  They also had to take pictures of my optic nerves.  I guess optic nerve damage is a possible side effect of this drug.  I'll go back to the Ophthalmologist every 3 months to ensure that all is going smoothly.  So far, so good!

This past couple months have proven that I am officially part of the sandwich generation.  I spent a great deal of time taking care of my older and younger family members.  I am so thankful that I have been feeling well enough to take on this role.   I love being needed.  I love being able to help...and I love not being the patient myself! 

Mom and grandma are doing great!  This is a picture of my grandma, mom and aunt in Hawaii, where they lived for several years in the 1950's.   My mother is wearing the striped dress.  My grandmothers first name is Avalon.  She is the reason I named my daughter Emma Avalon.  I am more like my grandmother than any other family member. 

I sure do love these women!

Miracles & Such

As a new graduate with my Master's Degree I was working in the outpatient oncology department of Cleveland's largest hospital.  As a large, specialize facility, we would get patients coming to us from all over the world.  I remember her like it was yesterday.  She was bald, skinny and her face could no longer hide her desperation.  She was forty years old, at most.  Her body was riddled with cancer.  After conventional treatments failed to work, she went on a clinical trial in the US somewhere else in the country.  When her cancer failed to respond to that clinical trial, she flew to Germany to try an innovative new treatment offered in that country.  That treatment didn't reduce her cancer burden, which led her to Cleveland.  She looked worn, exhausted, but absolutely determined to fight until the very end.  After speaking with her I found out that she had little children, a husband, she was a hard worker, she had a lot of friends...and she was not willing to go gently into that good night.  Yes, I did just throw a Dylan Thomas reference in here...

I was in my early twenties.  I was naive.  I thought I knew it all.  In my head I was thinking... 'Go, be with your family.  You've tried so hard...you've done so much.  Spend the rest of your days loving and laughing and crying and holding your children close.'  Of course, I never said this to the patient.  This was her choice.   This was her journey.   She ended up dying within weeks of our meeting.  That day I remember promising myself that I would never spend my last days searching for my medical miracle.  If my miracle was not easily accessible, then I would be okay with my fate. 

I guess13 more years of living, a husband and children of my own has changed my perspective.  On Wednesday of last week I broke this promise to myself.  No, these are not my last days...not even close.  No, I have not exhausted every option and I am not searching for my medical miracle at this point.  Yet, I did travel across the country to see an Infectious Disease expert at the National Institute of Health.  Dr. Oh (not his real name) knows more than most physicians about mycobacterium.  Dr. Dazzle has been encouraging me to make this trek to Maryland since last August.  I finally decided to make the trip and I am so, so thankful that I made this choice.   I left with a new treatment plan, a plan if that plan doesn't work, then yet another plan if we're still not seeing progress. 

After I made it through being searched, having my belongings searched and having my car searched (this is a HUGE government facility and security was heightened) I made it to my appointment at my scheduled 6:30am time.  I had a schedule jam packed with tests and consultations that would take until 5pm to complete.  NIH is not a place that people go for every day treatment or health care.  You have to go through a screening process and be accepted to be seen at NIH.  They typically provide consultation and you almost always have to take part in a clinical trial to be seen.  I signed two clinical trial consents while there. 

This made for some very interesting people-watching.   Every patient at this facility was likely looking for their medical miracle.  Most of them have likely had treatments fail.  While waiting for my CT scan I sat in my little paper gown next to a man who was obviously battling cancer and a woman who was both deaf and blind.  I felt strangely fortunate...overwhelmingly fortunate.  Sure, I have to worry about my health and do things to take care of myself every day that my healthy, real-world friends don't.   Yet, it could be worse...it could always be worse. 

I made a new best friend during this appointment.  Dr. Cha Ching (not his real name either) is the Infectious Disease Fellow who spent a good two hours chatting with me about my history and present illness prior to my appointment with Dr. Oh.  Dr. Cha Ching made understanding more about mycobacterium fun!   When reviewing my CT scan from earlier in the afternoon he made a little game out of locating the areas of "schmootz" hanging around in my lungs.  Apparently, he likes saying schmootz better than infiltrates.  Dr. Cha Ching gave me even more reason to adore him when he called me before 12 noon the day after the appointment with my AFB (acid fast bacilli) smear results.  When the smears came back positive, meaning that my mycobacterium is still being resistant, he told me how sorry he was...and I could hear in his voice that he meant it.  I was really, really impressed.

The very last thing on my schedule for the day was a one hour appointment with Dr. Oh.  We basically reviewed the entire day and came up with a plan.  My PFT's dropped by 14%, but this didn't bum me out.  It was different equipment and I had done hypertonic saline about 2 hours earlier, so I'm thinking that may have irritated my airways.  I have an appointment at my own clinic in a couple weeks, so then I will be able to really gauge where my lungs stand.  My CT scan showed that I have some new nodules/infiltrations in my lungs, compared to my pre-treatment CT, but no cavities.  I can deal with a few little nodules.  This bacteria eats the lungs and can create huge holes/cavities in the lungs if unchecked.  Sometimes, there is no change in PFT's when these cavities are present.  The only way to determine the damage caused by this bug is through CT scans. 

The plan is that I will be continuing my oral Azithromycin and Doxycycline, while doing the Amikacin every day now...instead of doing a month on and a month off.  I am also adding a forth antibiotic called Ethanbutol.  This is an old antibiotic used to treat Tuberculosis.  If I still can't get clean cultures after a few months on this protocol, we may move on to IV Merepenum.  Or there is a clinical trial starting at NIH for people like me, with refractory NTM (non-tuberculosis mycobacterium), so that may be yet another option. 

While the lower PFT's and new nodules didn't bum me out, a couple of things were frustrating.  The first was Dr. Oh's use of the word "refractory".  When he said it my ears perked up and it's been bouncing around in my brain ever since.  This word is used in the cancer world that I work in when someone's cancer won't go into or stay in remission. It usually doesn't end well.   The woman that I spoke of at the beginning of this post had refractory disease. I never thought of my NTM as being refractory, but then I realized that I have never had a clean culture since this was first discovered last year, despite gobs of antibiotics.  I guess that makes it refractory. 

The other thing that I wasn't expecting was hearing that my treatment is nowhere near being complete.  I must be on this drug combination for at least a year following my first clean culture, which I haven't had yet.  I was really hoping my belly would get a rest.  I was hoping that Dr. Oh would say that my NTM wasn't so bad after all.  I was hoping that I could take a little break starting in September.  Instead, Dr. Oh said that he thinks I need to make my treatments a bit more aggressive, that I need to start them now and continue them longer.   As frustrating as these things were for me, the fact that we had multiple plans in place put me at ease.

I left feeling very encouraged.  I left feeling confident that we are moving in the right direction.  I left feeling completely smitten with this new medical team, who will be working hand in hand with my current medical team.  I left and headed to an adorable little sushi place in Bethesda to meet with Josh and Melissa Sams.  Josh is 35 and has CF and Melissa is his wife, who also has a child from a previous marriage who has CF.  I can't tell you how much I adore these two amazing human beings!  We laughed and had a grand time.  It was a fantabulous ending to a reassuring day!

The Diagnosis

May is Cystic Fibrosis Awareness Month.  Several of my blogger friends, like Jen and Tara, have posted the story of their diagnosis or that of their child.  I figured I would take this opportunity to share mine. 

It was 1978 and I was 2 1/2 years old.  I had been in and out of the hospital with pneumonia for more than a year.  I would improve, go home, and decline.  Each time my pneumonia was treated, but the cause of the repeated infection was never investigated.  After being in the hospital more than not that entire second year of my life, my mother refused to take me home until someone found out what was causing my repeated illnesses.  The doctors were reluctant to do a sweat test, because I was not a "failure to thrive" baby.  I did not show signs of the digestive symptoms that CF often causes.  At my mother's insistence they did a sweat test, which came back at a 95.  They repeated it several times and got this very high number each and every time.  My parents were told that I had Cystic Fibrosis.  They knew what this meant.  Our neighbor 2 doors down, Kim, was 12 years old and also lived with CF.  She actually babysat me many times.  My parents knew how complicated life was for Kim and her family with CF as an additional burden.  The doctors told my parents that the life expectancy was 12 and that they would have to work hard to keep me alive.  This was the knowledge that medical experts had about CF in the 70's. 

My parents worked their butts off for me.  I did nebulizer treatments and they did manual postural drainage on me every morning and night...never fail.  Both of my parents worked full time, but they never skipped my treatments.  They always made sure I took my pills and they missed a lot of work ensuring that I got to all of my well appointments and all of those unplanned appointments when I was ill.  I know that my parents grieved.  They both grieved differently, but they both grieved.  When you have a child you have expectations for that child and their future.  When a parent is told that the future of their child may be compromised it is a loss.  I think that's one reason I worry so much about my future...not for my sake...but for the sake of my beautiful babies.

This story gets to the heart of what I think my parents may have felt:

WELCOME TO HOLLAND
by

Emily Perl Kingsley.

c1987 by Emily Perl Kingsley. All rights reserved

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.

After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.

The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."

And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

While I think my parents got more than they bargained for, they made my life full of wonderful things.  They helped feed my determination.  Caring for myself was necessary, but not limiting.  They made sure I stayed active, putting me in dance and sports.  I think that's part of the reason I continue to work out 4-6 times per week.  When the going got tough, when they were tired and overworked, when their marriage failed...they did not give up on my health.  They worked their asses off for me and taught me how to be an aggressive advocate for my care.  I give them, along with medical advances, a lot of credit for getting me to where I am today. 

I love you Mom  Dad!  I'm so sorry that my health made your world more complicated.  I am forever thankful for all of your hard work and care over the past 33 years!

My Nemisis

I had a little set back a couple weeks ago. Nothing too major, but enough to land me on more antibiotics.

As my disease has progressed I have come to despise stairs. All stairs frustrate me, but none more than the stairs that lead from the first floor to the second story of my home. In the olden days, I used to whip up the stairs with no problem. Now, my ability (or inability) to master the stairs has become an indicator how well my lungs are functioning.

Prior to the 4+ months of IV's that I had in 2010 I needed to take a good 2 minute break to catch my breath after navigating the 13 steps to the second floor of my house. If I was on the phone I had to discontinue my conversation and call the person back after I caught my breath enough to speak again. It was as if I had just finished a 100 meter dash. I would avoid unnecessary trips upstairs. My steps got piled high with things that needed to be taken upstairs, but had to wait until I was going up to stay. Once I was up, forget about asking to me to get something from downstairs...it wasn't happening.

A couple weeks ago I was feeling off. I barely got through my work day, then collapsed in bed each night. My belly felt horrible, my body felt horrible, my lungs felt horrible...I plain and simple felt horrible. The last straw was when I didn't have the energy to walk my son upstairs for a time-out after he misbehaved. While my son was enjoying this version of mommy, I was feeling rough.

A call to my doctor resulted in prescriptions for Flagyl and Levaquin. Dr. Dazzle thinks that this was an exacerbation of the H flu that I cultured in February, but we didn't treat. I took two doses of the Flagyl and experienced the worst peripheral neuropathy I've ever had. It was much worse than the neuropathy I got from the Zyvox I was taking a couple months ago. There goes another drug that I can no longer tolerate. The list of options decreases once again. Unfortunately, I am still experiencing neuropathy at night. This condition can become permanent. It's really annoying, so I hope this isn't the case for me.

The Levaquin, however, has done the trick. My lungs feel great! The stairs have become slightly less daunting. I have to admit that I have had THE conversation with my husband recently. The same conversation my parents had several years back. The conversation most people have when nearing 60 years of age. I had the "I think we should look into buying a ranch-style house soon" conversation. I've been dreaming about whisking freely about the house, punishing my children without a second thought, and feeling like less of a prisoner on whatever floor I happen to be on at any given moment. Easy access is my goal...

Breathe

Four years ago today, Matt Scales, a musician from the UK with CF, died. He wrote an amazing song that was reproduced last year. This time it was sung by 3 amazing singers living with CF themselves. One of the singers, Josh, is a very dear friend of mine.

Click the link below to listen to this beautiful song and read about Matt...

Breathe Song Event

The Story of Us ~ 20 Years in the Making

The year was 1991. It all started at a table in the back of the cafeteria at a very small private high school. The table in the back corner was where I sat, along with all of the other kids who were "rough around the edges". I remember our lunches together, but only vaguely. We bonded over french fries with ranch dressing. Ketchup was too ordinary for us. We threw caution to the wind and dipped our fries in a shared container of ranch dressing. That was our common bond at that time. I would like to say that it was love at first sight or that we knew we would always be together, but I can't and we didn't. M had steady girlfriends all through high school and I had the same boyfriend throughout. We were just two people who enjoyed our fries slathered in sauce.

Fast forward to 1997. I had just finished undergrad and was preparing to enter grad school at Case Western Reserve to pursue my dream of being a health care Social Worker. I worked hard at my studies, but I knew how to have a good time. Sometimes I was guilty of having too good of a time. I was always surrounded by friends and was the healthiest I had ever been in my life. Life was grand. Sorry, Jen, I couldn't resist posting this oldie but goodie... M was a full time punk musician and a part time college student. His band was busy touring the US and Europe. I've heard the stories and it's pretty obvious that a lot of oats were sewn during this time. It was in 1997 that I caught one of his shows when the band made it back to Cleveland. I have to admit that this wasn't my scene. I was never part of the punk culture...no purple hair and safety pin earrings for me. I did have fun at the show, though. Even though the music wasn't up my alley, I bought the bands latest CD, which M autographed for me after the show. It wasn't until I got home that I read what he wrote.... "French Fries with Ranch Dressing" followed by his stage name. Very clever, indeed.

I'd like to now write about how he had me at "french fries", however, it was not to be. I had a different serious boyfriend at the time, while M also had a long-term girlfriend. We were just two people passing in the night.

The year was 1999 when we would meet again. My best friend since 1st grade worked at the bank where M was a police officer. That's right, folks...my man morphed from a punk rocker to a police officer in a matter of a couple years. This time were were both single so we decided to actually go out on a sorta date kinda thing. It was pretty lame. From there we hung out when it was convenient, but we each were dating others along the way. We would go months without seeing one another, then hang out again on a whim.

What mattered at the time was that we were friends and it our relationship was easy. The love grew day by day. It wasn't until 2001 when I was offered a job in Baltimore at Johns Hopkins and I was preparing to move away that we both finally felt that what we had was right. I am not a big fan of the term "soul mates". Honestly, I think finding the one person for you has more to do with timing and circumstances than anything else. I know, this isn't a very romantic notion, but it works for us. Our timing and our circumstances finally allowed a real relationship to blossom.

M surprised me with a beautiful proposal in October of 2001. It was 9 years ago today, April 6th, 2002, that I wed M and became a bonus mom to then two year old, Sarah. The day was beautiful, maybe even perfect! Marriage is not easy. Marriage with a child is difficult. Marriage with three kids, two full time jobs and Cystic Fibrosis is tremendously challenging. I'm not going to lie to you, my esteemed readers, and tell you that we've had a fairy tale life. I won't tell you that we've always been supportive and appreciative of one another. That sometimes, maybe even often, gets lost in the chaos of this thing we call life. However, I will tell you that we are friends and our love has deep roots. Roots that are 20 years in the making. Lately I feel like M has gotten the short end of the stick when it comes to the in sickness and in health part of the vows that he spoke 9 years ago on this day. Yet, he has stepped up to the plate and kept this family functioning when I wasn't able to do my part. I am forever thankful for him for taking on the challenge known as "Stacey". Many others have tried, but failed miserably.

9 Years later...or 20 years in the making...and this is what we have become. An entire family! We even helped create entire other little human beings that didn't exist before. I am blessed beyond measure!

Happy 9 Years, M! I love you for always!

Distractions

We just returned from a fabulous vacation to Florida! We spent 9 days away from home, in a different environment with fewer worries. Except for the fact that my son ran a fever on and off for 4 days and my step-daughter had to be seen at an Urgent Care to get antibiotics for a very painful inner ear infection. I guess no matter where we go, how many miles we travel, our worries are never really far away. We made the best of the healthy moments and had a blast!

I was excited to get away. The next couple months are going to be so busy with business travel, my mother's back surgery, my grandmother's new dialysis regimen, and medical appointments galore. This was the last bit of rest and relaxation that I will likely get for a while. I was looking forward to spending time with the family, the sun, the surf and the sand. However, when I walked into our rental unit I became as giddy as a little girl when I noticed this:

A fully stocked book case! I brought my Kindle, which is currently loaded with 57 books...but these were bonus books. More than half were titles I have not yet read. I have noticed over the past couple of years...the years when my health has become more complicated...I have been devouring books. I am a fiction girl. I have no interest in reading about real life experiences. I feel like I get my fix of real life from Facebook and blogs. I want to be swept away in a story. Something completely imagined...a fantasy. I have always been a reader, but never quite to this extent. I find that I have a hunger for books now.

The first book I read on my vacation was called "Home Safe", which was a little story about a mother/daughter relationship and loss. It was good, but it was one of those books that you move on from quickly. No lasting impression.

The next book was not an easy selection. I kept skimming the titles. Maybe I should read something fluffy like The Wedding by Nicholas Sparks. What about something silly, like one of the Shopaholic books? However, my eyes continually focused back on The Kite Runner by Kaled Hosseini. I know, I know...this book came out eons ago...2003 to be exact. I have wanted to read it and wanted to see the movie, but just never got around to it. I knew that the topic was heavy. Did I want to invest in a "heavy" book on my vacation? Of course, I did! I am a glutton for punishment. I have no regrets. The Kite Runner was by far one of the most amazing books I've read. I learned, I felt, I got angry, I cared and I learned some more... This book will stay with me for a long time to come.

Amir, the narrator of the book, was a young Afghan boy who often lost himself in the written word. He read and he wrote, which often helped him cope. In no way does my life parallel that of Amir, but this aspect of the book got me thinking about the role of reading in my life the past few years. Reading helps me fill all the quiet spaces...the times when negative thoughts and worries have the opportunity to creep into my mind. I find that I obsess less about my health when I'm engaged in a good story. When I'm reading a good book in a doctor's office waiting room I find that I obsess less about the germs invading my space. So here lies the correlation...as my health becomes more worrisome I rely more on my distraction...or shall we call it a coping mechanism. So when the going gets tough, you know you can find me somewhere in a corner with my nose deep in a book...er...make that my Kindle.

I decided to add a page to my blog with a list of all of the books I remember having read over the past couple years. I know I'm missing quite a few. This will be a record of all of the hours that I have spent coping...getting lost in a reality that doesn't include Cystic Fibrosis.

On the health front, I am pleased that I have been able to avoid getting any of the illnesses that all three of my kids have had over the past few weeks. I am still on three antibiotics, so maybe that's helping...who knows? I was finally able to schedule my appointment to see Dr. O at the National Institute of Health. I will be heading to Bethesda, Maryland for a couple days in early May for my work-up. Since I re-cultured the mycobacterium, I am really looking forward to hearing if my treatment protocol will change. The coordinator I spoke with today said that they typically treat their patients much more aggressively than other centers. I'm wondering if that may mean more months of IV's are in my future. I am hoping that we can find something to eradicate this beast... I have been having a difficult time following Facebook lately. I have met several people with CF on Facebook who also culture MAC and/or m. abscessus, and the majority of them are doing very poorly right now. I really hope for the best for them. At the same time, it's really hard not to compare and imagine that I may soon in a similar way.

My job over the next month is to not obsess over the possibility of a decline or more IV's. I guess more reading is in my future? Next up "Sarah's Key" followed by "A Thousand Splendid Suns".