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!