Compliance, Judgement & Progression

A fellow blogger, Piper, over at A Matter of Life and Breath wrote an amazing blog post yesterday and extended a challenge to others to write about their thoughts on CF control and progression.  So here goes...

 I am an old CFer, who has by luck or biology or by the grace of God, had an easier road than most with this disease.  It has limited me little up to this point and for that I am extremely fortunate.   However, I don't ever feel like I have "control" over my disease.  Instead, I feel like I do what I can to "manage" my disease(s) on a daily basis.  It takes A LOT of work.  It take enormous amounts of patience.

Sometimes, it even takes giving yourself a break.  Allowing yourself to not be perfect.  Treatments are crucial...compliance is so very important...  Yet, sometimes, life gets in the way.  My personal definition of compliance involves working hard and doing your best.  Everybody's best is different.  I think people in the CF community like to compare each other.  If this one is running a marathon, while that one walks around the block for exercise, the one who walked is not doing "good enough".  My wish is that people would stop making these comparisons.  There will always be someone doing more than you.  There will always be someone doing less.  I think each of us should only judge ourselves when it comes to self-care. 

I also think that sometimes a person can do everything by the book, yet still decline.  This is the nature of the disease.  I think judgement coming from others regarding this decline is harsh.  That person who is declining is often judging themselves harshly enough already, even if they have done everything in their power to stave off the progression. 

This is going to sound odd to some people, but I honestly feel judged for being "too healthy" within the CF community (no, I'm not complaining about being a too healthy CFer...I'm fortunate!).  A lifetime of doctors, hospitals, pills and daily treatments doesn't make me a card-carrying CFer in some people's eyes.  Yes, I have been told this directly.  My lung function is not low enough to qualify.  I haven't needed IV's enough times.  I have been told that I shouldn't have feelings regarding my CF, because my road has been easier. 

Here's the deal.  This is MY experience.  This is MY journey.  These are MY feelings.  I admit that my road has been easier, but I am entitled to my feelings, fears, worries.  Just as I am entitled to celebrate successes without being judged.  I wish more people were accepting of that fact that EVERYONE has a unique experience...all of them valid.  Yet, I know that the judgement will continue to exist.  Honestly, it's human nature.  There are people who feel better about themselves when they put down others.  I consider that the judges problem, not the problem of the judgee.
So, I have been feeling excellent lately!  So much so, that I have had very little health-related news to blog about.  Yet, the MAC infection persists.  I have an appointment in a week in a half, where I will be cultured to see if the MAC has been suppressed at all.  If not, we may need to switch things up again.  I struggle with the unpredictability of this disease.  I think I keep motivated to exercise regularly and be compliant with my treatments to give myself a sense of control.  It makes me feel like even if nothing is going to change, at least I'm doing something.

These are my jumbled ramblings about compliance, judgement and progression.  If you wish to take the challenge, read below...
1. Write a blog explaining your personal thoughts and experiences in dealing with CF control and progression. This could include your views on whether CF is in fact a "controllable" disease, your personal definition of compliance, your thoughts on whether (or how) someone with CF should be judged in terms of "good enough" self-care (what makes you feel judged? do you think those fears are justified? is judgment ever useful in this context?), your own struggles with control vs. unpredictability, and how you keep motivated in the face of so many questions. Or, you know, whatever you want to write about really. It's your blog.
2. Comment below with a link to your blog so that all of us can read your response. YOU DO NOT NEED TO LINK TO MY BLOG IN YOUR ANSWER. If you'd like to do so, please feel free, but this is about starting a discussion, not publicity.
3. Encourage your own readers to get in on the conversation by posting the same instructions on your blog. Remember, the more responses, the better the conversation. Let's see if we can get this one going as much as with past challenges.
4. If you don't have a personal blog (or just don't feel like going through steps 1-3), feel free to still make yourself heard by simply leaving a comment with your thoughts below.
5. Non-CFers are 100% welcome to participate, either by pulling from their own experiences or simply by offering their perspective as people, friends, and loved one's.

Fun Surgery Pictures

I have my skin excised today.  Besides some excess bleeding, all went very well!  It was relatively pain-free.  I did find out that what I have is pre-melanoma.  This made me feel thrilled that I caught it and took care of it before it progressed.  Now I just need to be more diligent about getting screened annually. 

So here's how things went...

 This is a picture of the eye-shaped cut that he made, prior to removing the actual skin.  They remove down to the fat.

This is after they removed the skin.  They remove all of the skin down to the fat.  I have to wait to hear from pathology to ensure that they got clean margins.  If not, I go back for them to take more.

This is after I was all stitched up.  It required 8 internal stitches.  They used steri-strips on to to avoid external stitches.

In all honestly, it really wasn't bad at all!  It was WAY easier than a PICC placement.  The best part is that I've got friends who have told me that they are now going to get their skin checked.  You just never know with this stuff.  I was certain that the spot was just a benign little freckle.  Who knew?  I hope this concludes all posts about skin issues.  

In other news, we have been having a FABULOUS summer.  We have had a few out of town visitors, we have been swimming quite often, we  have been busy, busy, busy!  We are really looking forward to a 4 day little trip to Washington DC with the kiddos.  I loved my first trip to DC as a kid!  A couple weeks later the hubby and I will be getting away to Boston ALONE for 5 days.  I can't wait to eat my way through the city!

I hope all is right in your world... 

Abnormal

Don't you hate when you get a message from a doctor, that you have TONS of questions about, after hours?  I do...  About an hour ago I listened to my voicemail and had a message from my Derm.   It turns out that my biopsy showed abnormal cells in my skin lesion.  They said it's not necessarily skin cancer, but that I will need to schedule a 30 minute surgery to have the area excised and I will need stitches.  They will then do a more complete biopsy of the excised flesh.

So after I first listened to the message I thought...no big deal.  It's just a little slicing and digging in my arm.  It's only going to take 30 minutes.  I've had 2 organs removed...this is NOTHING.  Then I googled pictures of skin excisions.  Icky!  It looks like they take a big area and that that go pretty deep.  Sometimes they have to put in an inner and outer row of stitches.  What a pain in the ass!

So I've decided to shut my computer down for the night after posting to my blog so I don't freak myself out more than necessary.  I tend to do that quite often when I start researching my own medical conditions.  I refuse to think of this as anything but a harmless little freckle.  I'm sure they are just being proactive in removing the area.

I do wish that my abnormal cells would have been found near one of the horrible tattoos that I got the day I turned 18...just because I could...   Better yet, it would be nice if the cells were found on my belly and the excision could double as a nice little tummy tuck.  No such luck!

On the lung front, I feel AMAZING!  I really feel like this combination of antibiotics, along with the Spireva are working wonders.  After my appointment in May, Dr. Dazzle let me go back on a 3 month clinic schedule.  I was going monthly for about 6 months, then every two months.   I've progressed to the point where quarterly will do :-)

I'll keep you all posted about this new skin stuff. 

Have a fantabulous 4th of July weekend!  Please wear your sunscreen!

I'll Take a Poker Face Please!

I had an interesting Dermatologist appointment the other day.  I've never seen a Dermatologist before, but after watching this video I decided it was time.  Please, please, please take a few minutes to watch.

Dear 16 Year Old Me

So I very rarely go out without my SPF 100 sunscreen.  Living in Ohio helps too, as we rarely get sun.  However, I grew up getting burned quite often.  As a teenager I sat in the sun with vegetable oil slathered on my skin.  It was the thing to do in the late '80's and early 90's.  In my early 20's I actually went tanning now and then.  It was only after I started working with cancer patients that I started caring about my skin. 

This video was enlightening.  I never really made the connection that those of us with CF might be more prone to skin cancer.  We are immuno-compromised by nature.  That's why we pick up so many "bugs" that normal/healthy people don't...  Dr. Dazzle said that this is how/why I picked up this nasty mycobacterium.  Those who are immuno-compromised are at a greater risk of getting skin cancer too.  That mean us guys!   We need to be more careful.

So I had this freckle on the back of my arm that was very dark compared to my alabaster skin.  I have lots of freckles, but this didn't look like the rest.  I'd love to take a picture and show you, but it no longer belongs to me.  It's in a lab somewhere awaiting analysis.  The appointment started off with the standard conversation about my my CF...the normal stuff I get when I list my meds and tell any doctor other than my Pulm that I have CF...the "Wow, shouldn't you be dead! or You're REALLY old for having CF! or Are you sure you have CF?"  I am still alive and kicking.  I know I'm old.   Yes, I'm sure I've got CF.  We then moved on to my skin.  She looked over every inch of my skin, starting at my feet.  At which point I told her that my real concern was this freckle.  I lifted my arm to show her and I kid you not, she said "Oh man, that shouldn't look like that at all!  That's way too dark!  That's a grade 5 (something or other)".  She then had her assistant take a picture of it, the needles came out to numb my arm, a blade sliced the lowly little freckle off and the biopsy was complete.  I had no time to prepare.  The entire process took about 45 seconds.  I was bandaged  up and told that my results would be back in about two to three weeks. 

I left not really registering what happened.  I left wishing the doctor had more of a poker face.  She looks at weird skin all day long.  I'm pretty surprised about how much she reacted to my harmless little freckle.  I kinda wish she would have said something reassuring, like that we're just going to do a little biopsy as a precaution, but it will likely come back normal.

I REALLY think this is going to be benign.  Even if it is cancerous, it would VERY likely be basal cell, which is so easily dealt with.  My father has basal cell carcinoma and it's no big deal.  He has to get his skin checked and parts cut off here and there, but it's really not so life-threatening.   I'm glad I finally went, so I could stop wondering. 

So here's my plea for all of my CF friends out there...   PLEASE protect your skin!  Not only to prevent the potential of getting cancer, but also because we're living much long and you don't want to have to deal with too many wrinkles in old age!

My Dad is Rad

I adore my father.  He is the nicest, most sincere man I've ever met.  My dad is a hard worker, staying at the same job for 35 years.  He's a golfer.  He is kind.  He is simple.  He's a worrier.  When I travel I still have to call him when my plane lands AND when I arrive at my destination.   There is no doubt who I got my "worry" traits from...  He adores my children.  He adores his only daughter. 

This is one of my favorite wedding pictures.  My hands were freezing and my father was attempting to warm them up.  He was taking care of me. 

Happy Father's Day, Dad!!!  I love you for now and always!

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!