Letter to Emma #1

My Dearest Emma,

You are a beautiful soul. You are the little girl I never thought I'd have...my miracle baby. The first time I looked down at your wrinkly, red little face, I was madly in love. I used to dress you up like a baby doll. I was so proud of you and overjoyed that you were mine!

You have ALWAYS been a Daddy's Girl. I can't say that I always found this to be fair. After all, I'm the one who carried you in my body for nearly 10 months. I gained weight, endured pain and have permanent stretch marks as a reminder. I worried about you from the moment I found out that you existed. Your father, on the other hand, gained a few pounds of sympathy weight...but never endured quite the same experience. Yet, you always preferred Daddy. I've taken enough Psychology courses to know that this is common in little girls. So I never really worried that it meant anything significant...until recently.

Early last week you woke with a start very soon after falling asleep. You ran into me and Daddy's room sobbing, saying that you had a bad dream that Daddy died. You were inconsolable. We tried to find out more details, but you just cried and cried...hanging onto Daddy for dear life as I stroked your hair. You slept with us that night. Afraid that if you left something horrible would happen.

As I thought more about that incident, I tried to make sense of your tears. This was much more than the run of the mill reaction to a bad dream. Why were you afraid that Daddy would die? Might you have been afraid that I was going to die, but unable to express this fear? Might you have already come to terms with the fact that I may die soon, so you were fearful that if Daddy died too you would be left without a parent. Maybe I'm making more out of the situation than need be. I just want you to know that my heart hurt for you that night, if only because I hate the possibility that my disease makes these thoughts even enter your world. I truly hate that my disease complicates your otherwise normal childhood.

As you have grown it has become apparent how very similar you and I are to one another. Not only do we hear how much we look alike all the time, but I think you have picked up many of my traits...both good and bad. You're a smart girl...always preferring books over dolls, you know what you want and how to get it, you can be stubborn, you truly care even if you have difficulty showing it at times and you struggle with anxiety. Sometimes we butt heads. Even as I'm writing this you became upset with me when I told you that you couldn't put a K$sha temporary tattoo on your forehead. I don't care how temporary it is, the answer is still no.

As you've gotten older I can feel that you sometimes keep me at arms length. I often wonder if this is self-preservation. Might you be afraid to get closer, since the reality of losing me is there? When I was sick a lot, sometimes you even seemed a little angry with me. I want you to know that I understand. I honestly think I would do the same thing. I am a master at putting up walls to protect myself from devastation. I get it and it's OK. However, no matter how high or thick that wall gets, I will never stop saying and showing you that I love you to the end of the Earth and back again.

I know that you know that this song reminds me of you. I know that you roll your eyes every time I say that when it's playing on my ipod. It's true, though. Emma, I want you to listen to these words and please, ALWAYS know that I love you more than you may ever realize during my lifetime. You're my little M&M after all...

I love you and I love you and I love you!

Mommy

(Sorry I couldn't get the actual video, as it wouldn't allow me to embed...don't click on the screen if it doesn't play...like it says)

CF Denial Awareness

I got my new "CF Denial Awareness" shirt in the mail today! Look...


I told my husband that I ordered the shirt as I was giggling about the message, which I find amusing. He just looked at me, dumbfounded, and said "I don't get it". Please tell me you get it.

CF denial is sometimes a wonderful thing. Not the type that leads to apathy and non-compliance. Instead, the kind where you unconsciously go through your daily CF routine, while not thinking or obsessing about the harsh realities of this beast we call Cystic Fibrosis. The kind where you're feeling sorta good for a day or two and you 'almost' forget. The kind where you're so busy or preoccupied with life that CF doesn't make it into your thoughts for minutes...sometimes hours. The kind where you sometimes feel like everyone else...all the other 'normal' people. This is my kind of CF denial and let me tell you...it is one comfortable place to be. So this is my effort to raise CF Denial Awareness!

This shirt was designed by Kelly Langs, a graphic designer with CF, for the late Paul Mooney aka "Q". I never got to know Q...he died just before I delved into the world of cyber-CF. From what I've heard, he was a funny guy, who wrote and sang music about CF in a very Bob Dillonesque fashion. I have all of his songs, with my favorite being "Cystic Dreams". Listening is a good time. These shirts were being sold for Q to help pay for his transplant. Following his death, Q's parents started an organization call The Cystic Dreams Fund and all shirt proceeds now go to this fund.

If you want a shirt like mine, you can order from here.

Ciao!

No Longer Who I Used To Be

I work full time. My office is in New York and I live in Ohio, so I telecommute. It works for me, because most days I get the kids off to school head to my basement and do my thing for 8 hours. I'm able to do my nebs at my desk in front of my computer. It's really the ideal situation for me right now.

However, I manage/consult with 22 Chapters of my organization...18 in the US and 4 in Canada. That means I must travel. A few years ago I was traveling A LOT. I was reaping the frequent flyer rewards...being upgraded to first class from time to time...getting to pre-board and walk on the "blue carpet" that is reserved for "elite" members only. At the security checkpoint I knew just how far I had to strip down, what had to go in a zip lock bag and how to smile just right at the TSA agent to avoid a pat down. I used to zip through the airport in my suit and heels, checking my Blackberry, walking with purpose...because I had places to get to and people to see. I'd catch my flight, get to my destination, catch a cab and meet with colleagues for many more hours. I'd then meet up with any friends I have in that town/state/province, head back to my hotel late in the evening, get a HORRIBLE nights sleep in a bed that is not my own, then get up and do it all over again the next day. I'd fly home, being sure to buy some little trinket for each of my kids from a random airport gift shop. This always helped with my own guilt for leaving them for a few days. Then I would get up the next day and work a full day...never missing a beat.

I'm pretty certain those days are now over...

I set out for my first trip in many months last week with letters from my hospital explaining my medical devices and wearing sensible shoes. I knew before even leaving for this trip that the heels were going to be a no-go. I got through security with no problems and my flights to Oklahoma City were fine. All was on time and smooth. Besides the fact that my lungs felt like they were shriveling like raisins while on the planes, all was great. I was thinking...this isn't going to be hard after all.

I got a good nights sleep with a little help from my friend, Ambien. My work day went well. I even got to meet with a fellow CFer, Darby, that evening. We compared coping mechanism and micro organisms, listened to the music of a fellow CFer who lost his battle and laughed a bunch...all while abiding by the three foot rule. Good Times!

I had another OK night of assisted sleep, woke bright and early (4am) and made it to the airport by 5am for my flight. We all boarded the plane and pushed off from the gate. All was right with the world. It was going to be another smooth day of travel. NOT! It turns out Houston was not allowing planes in due to fog. Back to the gate we went to wait for our clearance. I bought one of those gynormous bags of trail mix and read for a good while, until it was time to board once again. Three hours later...off we went.

We arrive in Houston and have to exit the plane outside. It's 175 degrees outside, and being a born and raised Ohioan, I'm draped head to toe in North Face gear. I start to sweat. My skin starts to burn from the salt being left behind. I rub my eye and now I'm done for...salt in the eye is the worst!

I get into the airport and find a screen to get information about my next flight. Turns out it takes off in 15 minutes. I spot a cart with one of those holier than thou drivers who gets to decide who gets a prized seat on the vehicle. I contemplate asking...maybe even begging...for a ride. I even thought about pulling out the CF card for this one. Then I decided it wasn't worth the questions or the ridicule. I think I would have cried at that moment if the driver had said, "But you don't look sick".

So I run, only to find out after a few minutes of running that this is going to be a marathon. This is where I thank the Lord that I decided to wear sensible shoes. After more than a mile of running with 2 bags wearing some of the most insulated clothing there is, I finally make it to my gate a few minutes too late. I rebook on to the next flight to Cleveland and set out to find something other than the sunflower seeds and raisins that I have been munching on all morning. This, of course, means more walking...more lugging of bags...more sweating... The people...there were just so many of them. A lot of them were twice my age! They were all wearing their suits, checking their Blackberry's and walking with purpose. I was just sitting there...defeated...coming to terms with the fact that I was no longer part of that club. My body has been beaten up by this damn disease.

I finally drag myself to my new gate and board my new plane. When I got to my seat I pretended to struggle with getting my bag in the overhead bin, until a kind business man lifted it for me. When I was a "real" traveling business woman, I would have NEVER let anyone help me with my bag. That would have been a sign of weakness. This time I just didn't care.

I make my way to my window seat and put my headphones on to attempt to avoid any and all interaction with other human beings. The guy next to me doesn't get the hint. He bumps my arm and signals for me to remove my headphones. I humor him, only to hear him tell me how very glad he is that he's sitting next to me on this flight. He continues to tell me how his last flight was hell, because he was sitting next to a guy with the most disgusting cough. He was certain that the guy had Tuberculosis. It took every ounce of restraint I had to not tell him to F$#& Off. I should have told him that I have something similar to TB...I should have coughed on him. Instead, I just put on my headphones and pretended he didn't exist.

I got home in one piece. Even though every muscle, bone and join ached. I also brought a nice little sinus infection home with me. While I used this opportunity to bitch about how crappy business travel can sometimes be, I am happy to still feel well enough to work. I have just come the the realization that I am no spring chicken anymore, especially in CF years!

Getting My Grove Back

Just a wee little update...

So, the news from last week knocked the wind out of my sails for a couple of days, but I picked myself back up and got my groove back. I can't live my life getting distraught about what a test result says...especially when I'm feeling so fine. AND, I must say, that I am feeling so, so fine!

After going off of the Zyvox I noticed a huge difference in my energy level. I actually have some energy now. Having been sick for so long, then being on a gajillion medications, I hadn't felt my typical manic levels of energy in eons. It's back and I am cherishing every little ounce of it. I know it may be fleeting...so I am making every effort to take full advantage.

I have to say, I feel a little guilty posting about feeling well for a change. So many of my fellow CF warriors are struggling right now. I have a friend in New Zealand who is picking up the pieces of her life after a devastating earthquake earlier this week. I have lots of friends right now who's lungs are bleeding, belly's are unbearably nauseous or are just recovering from hospital stays. All of this suffering, while I feel, dare I say it...healthy. There's a little bit of that survivors guilt creeping in again :-/

In other news, I started the Spireva phase 3 clinical trial yesterday. I did the phase 2 a little over a year ago, but didn't see much change in my health since I was in the midst of fighting off all of these bugs that I didn't even know I had yet. Yesterday I was at clinic for 6 hours. We did a pre-dosing PFT, then 4 post-dose PFT's. Once every hour for four hours. Here's the cool thing...my pulmonary function increased by 5% from my first PFT to the last!

Spireva is currently FDA approved for patients with COPD. Some CF patients currently take it off label. This study is intended to allow it to be used more liberally with CF patients. So far so good! I've had no side effect and my lungs feel fabulous! The plan is that I will be on the drug in the trial for 12 weeks, then I will go on it off label for up to 9 months longer. After that point, if I want to stay on it I will have to be prescribed it as part of my treatment plan at my expense.

We'll see where this takes us...

I want to thank all of my followers for your kind words and messages last week. Your support means more than you could ever imagine. I am convinced I have the best readers in the universe!

Guess I'm Not One of "Those" People

When Dr. Dazzle and I first began our discussions about eradicating the mycobacterium abscessus, he told me of these people who have been diagnosed with this nasty bacteria...who received treatment...and who never cultured it again. They were able to basically render the abscessus dormant. I knew that this particular bug is difficult to treat. I knew that this bug can cause irreparable lung damage. I knew that the treatment itself was very risky. I decided to take the risk, because I KNEW that I was going to be one of "those" people that Dr. Dazzle told me about in our discussions. One of "those" people who would have nothing but positive results.

Today I found out that I am, in fact, not one of "those" people. The mycobacterium is back. I was able to suppress it the entire 4 months of my initial IV therapy. I finished my IV's in December. It waited less than 2 months to return. It looks like this is going to be one of those things I am just going to wrestle with for the rest of my life. Dr. Dazzle and I had a chat about it. He said that while we were hoping that it would not return, we knew it would be a possibility. For now, we are not going to change my treatment plan. We are now going to focus on treating based on symptoms. My PFT's are very stable, I'm not having fevers and I'm not having an increased cough. For now, we will just go with the flow.
After the phone call with Dr. Dazzle I joined my family in the kitchen to help my husband with dinner. I told him the treatment plan, saying that I will now be on Azithromycin and nebulized Amikacin for life. My daughter overheard this conversation and said, "You mean you'll even be on these medications when you're 96 years old?". *SILENCE* When I finally caught my breath sufficiently to respond I said, "Oh sweet pea, yes, hopefully I'll still be doing these medications when I'm 96 years old". Moments like these are the worst part of this damn disease...

So I'm not going to lie...I'm bummed. I feel like I put a lot of blood, sweat and tears into this treatment plan for the past 6 months, and we're right back where we started. Dr. Dazzle assures me that just because it's present, doesn't mean we didn't suppress it. This must be what it feels like to relapse from cancer. I not a big fan of this type of disappointment.

All that being said, I feel pretty good physically. That's a HUGE plus! I also have a lot to distract me from thinking too much about this crappy news. I think I'll go get myself a mani/pedi sometime this week...then all will be right with the world :-)

Clinic Update # Gajillion

I had clinic again today. No surprises meant I left a happy girl. My PFT's were stable and now that I'm on Doxy I feel pretty good. Just a little fatigued here and there, along with intermittent belly pain, but that's to be expected when on 4 antibiotics. The only thing left is waiting the few days for the culture results to come back...

One thing that I have been experiencing lately is peripheral neuropathy. This can be a side effect of long term Zyvox use. Basically, neuropathy is nerve damage that can be permanent, that is sometimes caused by medication toxicity. This is manifesting itself in me by causing intense tingling in my hands. It feels like they're asleep, but no amount of moving them around takes the sensation away. Each episode that I've had has lasted from a couple minutes to an hour, and it most often occurs in me at night. Honestly, at this point it's just annoying. Not painful at all. However, neuropathy can get very severe. It can cause debilitating pain or lead to the loss of all feeling in the hands or feet. I was supposed to have 7 months left on the Zyvox. It is a very important component in abscessus treatment. However, Dr. Dazzle wants me to discontinue the drug immediately. He thinks the danger of having to deal with permanent nerve damage outweighs the benefits that I am getting from the drug right now. Some studies in South Korea show that abscessus can be treated sucessfully with Doxycycine, Amikacin and Azithromycin, so this will be my new plan. I tolerate the Doxy well, even though it makes it torturous to be in the sun. Guess I'll have to try to be a hermit again this summer :-/

I also signed my consents for a new study I will be starting on Monday. What's adding one more drug to the mix after all? I will be doing a double-blind Spireva study. I did a Spireva study a little over a year ago, but it was just testing the reactions of CF patients to the medication. This time they are looking for evidence that the drug can improve lung function. I have 2/3 chance of getting the drug versus the placebo. This is probably the 40th study I have participated in since childhood...no joke... I am the study queen!

So all in all it was a great appointment! Now, I'm just keeping my tingly fingers crossed that my cultures show that I have no new lung inhabitants...

Is it all about the attitude?

I struggle with the notion that the only way to get through a devastating illness is through "Positive Attitude". I hear it all of the time from people, both those with illness and those who have never been sick a day in their lives. I have come to the conclusion that people often say this to protect themselves from having to deal with my emotions. Once I hear that "I've got to be positive" from someone, I will never be honest about my feelings with this person. They would see it as a failure. This person truly thinks that having emotions such as fear, anger and sadness are weaknesses. I have talked to so many cancer patients who have felt guilt for having feelings other than happiness/thankfulness and pressure to maintain a facade of positivity.

I sometimes feel this struggle when in public. Why share my burdens with others? However, within myself I have come to the conclusion that all of my feelings about my journey are OK. I am doing the best I can each and every day. I get sad...I get pissed off...I worry more than I should...I feel deep joy...I am appreciative... I feel such a range of emotions and all of them are acceptable. Maybe not acceptable to the eternal optimist, but acceptable to me.

I do, however, feel that being happy and enjoying life important. I think I am a generally positive person, if only because it's more fun to live life this way. Yet, I make room for other feelings too. I also try to never tell anyone else how they should feel.

The link below expresses how I feel about this topic... please take the time to read!

Positive Thinking vs. Realistic Thinking