Small update...

About 3 weeks ago I started feeling awful. It came in quickly and I'm pretty sure it is the flu. I'm dismayed that I can't kick this thing. I'm extremely bothered by the fact that everyone around me seems to be paniced over this...I mean most people get a cold or a virus and it is really not a huge deal. Me? I get it and it stays around for a while. I suppose what adds to the worry is that I haven't been this sick since before I was actually diagnosed with CF at 21. So everyone is looking at this as a huge back slide for me.

I've lost my voice, still have a cough, am wheezing like a 40 year smoker and still have a fever! I have no health insurance, but I think I'm going to see my primary on Friday, although I am honestly not sure what will happen after that. I can't be admitted at our local hospital, I don't have health insurance. Then next week I have my CF clinic; where I hope they admit me. But that rarely ever turns out the way I want, so who knows what will happen. My husband is positive my PFT's have gone down, and I have a feeling he's right. My muscles are just so sore from coughing, moving even hurts. But I've been trying to maintain a level of activity and doing some breathing exercises to try to help my lung function. I'm hoping that my PFT's won't have taken a serious hit because of this, a small one I can handle (I think).

Clinging to CF

So after a discussion with my husband a few nights ago and some serious soul searching...I have come to a realization.

I've been in denial about this whole mitochondrial disease issue. I've been so focused on CF, and raising money and awareness that I have not acknowledged the other serious disease in my life. I've talked about it, sure; but I've done little to fund raise or spread awareness. (At least compared to the stuff I've done for CF)

It occurred to me with VX-770, that a pill for me might be around the corner and I got excited. I've spent a lot of time this week going over stuff I'd like to do, going back to work is one of the things on that list. But then I realized...I'm not working because I get frequent infections, I don't have the energy and my muscles cramp and ache. According to the mito information; those things can all be caused by mitochondrial disease. I knew my muscles were a result of the mito but I kept telling myself everything else could be CF related.

To clarify; I didn't delude my self because CF is less serious in anyway shape or form. I did this because I can explain cystic fibrosis. Because when I tell people I have CF and they ask, "What's that?" I can actually answer. When I tell people I have a mitochondrial disease 95% have no idea what I'm talking about. And when they as, "What is that"; I explain as best as I can a disease that my doctors don't even fully understand. People look at me like I use this as an excuse to be lazy...so I only discuss it with people who already know me and understand mito. When I meet people I don't say "Hi I'm Chrissy and I have two genetic diseases". When health comes up I usually just say I have CF and another genetic disease that is not well known. If people pry by asking what it is, I tell them it is a muscle disease. It sounds better than saying, "It is a disease in which my body doesn't produce enough energy on a day to day basis. So the symptoms can vary from person to person."

I mean typing it, it reads as a straight forward answer...I just wish it was so straight forward that people would not judge me.

Conserving energy

I haven't blogged in a while, mostly because I haven't had the energy. Yeah...that's right. I said "energy". It takes a lot for me to sit down and organize my thoughts here. I have found myself; lately, having to pick and choose my activities a lot more carefully. Here is a list of some of those things that I have to choose between most days:

Things that take energy
1. Getting out of bed
2. Getting a shower
3. Getting dressed
4. Coming down the stairs
5. Getting my medicine together and prepping treatments
6. Going into the kitchen to get something to eat
7. Eating breakfast
8. Cleaning up after breakfast
9. Going to the bathroom
10. Making phone calls
11.Logging in to school and participating
12.Walking back from the kitchen to the sofa.

I think you get the idea (a few of these I have to do more than once). Some days I wake up and can get 5 or 6 things done while others (less often) I can maybe even help out by starting a load of laundry or *gasp* I might have enough energy to spend some time with friends.

I do not consider myself a lazy person, but I'm not sure how many of my friends and family actually get how much energy it takes me to do the smallest task. Yesterday I had to take a shower with my husband, not for anything kinky mind you...but because I couldn't wash my hair. I'm not kidding either. Most people don't really know how bad it is because I honestly don't talk about it much.

I just feel bad about it. Embarrassed. Frustrated. Angry. I would rather not get the looks filled with pity, or they say "What about trying this or that?". My condition isn't something my doctors seem to know much about so, I don't like trying to explain something that even the "experts" have problems understanding.

These things are why when I have the energy I'll take my daughter shopping (even if I have to keep her out of school). This is why when friends or family call and ask for help or say they want to spend time with me--I'll move heaven and earth if I have the energy.

Matter of Life and Breath Blogger Challenge: Personal Disease Perspectives Edition

So yesterday I read the blog of a friend:
http://amatteroflifeandbreath.blogspot.com/2011/07/blogger-challenge-personal-disease.html
She posed a challenge, so I will do my best to answer her questions in hopes to reveal my outlook on cystic fibrosis to any who are curious to know what I think.

"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."

I wasn't diagnosed until I was 21, so I grew up sick. Although I wasn't just a sick kid; I was the sick kid that no one believed. I would complain and get accused of being lazy; trying to get out of school or some other obligation; or selfish. My parents say that they always knew something was wrong with me, but I think with no answers from the doctors that they started to doubt themselves. When I was 16 and needed my gall bladder out; I think everyone finally realized that I was sick and when they diagnosed my with pancreatitis they knew some thing had to be wrong. But it still took 5 years for them to figure out what it was. This was, for the most part, because as a child we did not have health insurance and honestly the information out there about CF was (at that time) minimal. Everyone thought that you had to fit into specific symptoms...boy were they wrong.

When I was diagnosed I was so happy to have a name, and I foolishly thought that meant we could start to fix it. After some research and a meeting with my CF doctor, I was terrified. I mean; all of the sudden I wasn't just sick I was dying. At least that was all I could focus on.

Life expectancy...

It took years for me to accept that I was as sick as they said, to want to fund raise, and to look beyond the dreaded "life expectancy." I had to figure out on my own, through research and joining a few CF support groups online that CF is different for everyone.
Sure it is progressive, but we all have different rates of progression. So I feel that it is inaccurate for doctors to tell patients (or caregivers) that they have a "mild" CF. There is no such thing, there can be mild symptoms but not mild CF. At least that is the conclusion I came to after meeting others with CF online and talking with my doctor.

Do I think CF is controllable? Not really. I mean certain aspects could be, like taking medication and using your vest; but the other aspects that cannot be controlled are genetic factors and in some cases environmental factors. You could pick up a bacteria at the beach or a friends pool, but also by using a public restroom or water fountain. So unless you put yourself in a bubble there is no way to protect yourself from every factor. Additionally even if you are extra careful and your best friend comes over to visit they could be caring a bacteria they picked up (at a pool, hot tub, or by sharing a drink with someone else). Not to mention those friends and family members who still visit with a runny nose or a cough. (even if allergies is the cause you are still spreading germs and bugs; you do still have germs in your snot!)
So I think we all have to do the best that we can, but not expect an easy road just because we are compliant and/or we exercise and take really good care of ourselves. Kinda like you can't win the lottery if you don't buy a ticket; you can't avoid getting a CF bacteria unless you don't have CF. (There are just too many factors, in my opinion) But I should say that I do think we need to work on what we can, influence the factors we might had sway over (environmental) to improve the health we do have.

I think it is crazy to lay blame on another CFer for getting sicker; because it is impossible to know all of the factors. The CFer themselves may not even be aware of all of the other factors. I don't think laying blame or judging helps in any situation; but especially not when dealing with an illness.

How do I stay positive?
I still find myself in a bit of denial every now and then; I am very self conscious about being sick. That, I'm sure, is because of my childhood. So I tend to try to do as much as possible to be "normal"; but in the end that is just a fancy way of saying I do too much to attempt to make up for the extra efforts of everyone else. I hate it when people have to do more, because I can only do 50% or less. (This is one of my biggest flaws.) To stay positive I:

1. Blog. I have this blog and another one (On the bright side); this one helps me vent, and the other I use to list my many blessings in life.
2. I take pictures and I cook. They are little things I do that help me be creative.
3. I cry. I used to think it made me weak. But I find that a good cry helps me "get it off my chest."
Last but not least, I am a very spiritual person. I'm not saying that is the only way to cope, just that it is helps me to cope. I don't go to church every Sunday, but I do not think God worries about physical attendance. The thing that matters is how we treat each other, not what church we go to. But again that is only my opinion.

So that's my input! I'll pass this on to my friends who read this CF or not. Here are the "rules."

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 ones.

Another week come and gone...

So this week has been a busy one, but we all managed to get through it fine! Kate got sponsored for a cooking class this past week, and she got one for a class in August too. I’m so excited for her, although I think my legs might need a vacation afterwards! But seriously, Kate has this opportunity to work with an amazing chef; so I’ll do what I have to do to make sure it happens. I know moms brag about their kids but I really think I have a wonderful, talented, and awesome daughter.
Other than that I had NIH this week, and things went ok. Frank has started taking me and when he can’t my brother does. I can’t drive that far anymore. Anyhow he isn’t used to getting the run around or the, “we don’t know what to do with you speech”; so if you ask him how the appointment went it was disappointing. I guess I should feel that way too but somehow I just can’t find it in me anymore to get upset. I mean don’t get me wrong I do spend many a night crying over this whole mess that is my health, but that is out of frustration, I think. Maybe it’s disappointment too…???


So my CF doc tells me about an undiagnosed disease clinic that is at NIH, and she wants to try to get me in. I’m all for that, it seems like NIH discovered these “novel” mutations and then a few years later decided it was too much work to keep up with. At times I feel like a bug in a jar, but I must be an uninteresting bug because they don’t even seem to be watching me anymore. So while I do have hope that NIH might come through, I will not be surprised if they don’t.
I suppose I should get used to the idea that no one will ever really understand me medically speaking. It’s difficult for me to explain to people what I have because while it is a mitochondrial disease, they cannot tell me anything more specific. The only thing more frustrating; to me, than having an invisible disease is having to tell people that you have an unknown (invisible) disease.
So tonight I’m sitting online and relaxing typing this blog post and playing on face book. My sleep schedule has been thrown off. I didn’t take pain meds all week, as I was driving, so know that I’ve taken one it’s keeping me awake. Frank is up stairs drawing, but I think I’m going to sneak in and go to bed!

The truth about batteries

Since my last blog I have been trying to be more honest, and while it is getting easier - I still find myself sugar coating things for people. Its not that they don't understand but sometimes - they don't understand the depth of it (does that make sense) Not that it something everyone understands, hell even the NIH in Bethesda doesn't understand my mitochondrial disease.

The mitochondria are the little batteries/power houses of the human cells. They can be found in every cell except the red blood cell. The number in each cell varies depending on how much energy demands. (Think of it like having a pack of M & M's. All the M & M's in the pack are energy, but because I have two mutations the brown and the orange are defective. Each cell depending on energy needs is like a pack of M & M's, so some times the bag/cell has 25% defective mitochondria while the next bag/cell could have 60%.)

Most energy production happens inside the mitochondria, so you can imagine that people with mutated mitochondria get fatigued easy. And how easy varies on a day to day basis. One day getting out of bed is a chore while the next I can go grocery shopping. Additionally one day my digestive system has enough energy to work correctly and the next day - it doesn't. This link explains it much better than I could:


So even though it sounds far fetched that I'm not feeling well two hours after you saw me doing fine; it happens. I'm not lying to get out of hanging out, create drama, to cause problems, or to get things my way. Seriously. I have enough drama from the cells in my body - I don't need or want more.

There are several different mitochondrial diseases, with mine the doctors won't give me any specific name. The reason? My two mutations are rare, so rare that one of them is completely unique to me. So they don't know what to expect and neither do I. I have had symptoms since I was little, but no one realized it. I was clumsy and had poor balance. At 16 I started having noticeable muscle spasms. At 21 NIH figured out that it was not 1 but 2 mitochondrial mutations causing all the problems (IN addition of course to my cystic fibrosis). Now at 34 I have muscle aches, cramps, pain, fatigue and spasms; I don't absorb nutrients the way I'm supposed to. While People with CF have problems absorbing fat and fat soluble vitamins; I got a double whammy and have problems absorbing lipids, fat soluble vitamins, water soluble vitamins, and minerals needed to help my body function. (This is why sometimes I get confused and/or forget things) And they don't know whats next. Honest. I don't want to be sick, and I'm not being dramatic, but the doctors really have no idea what symptoms could be next. They do know that it is progressive, having a genetic progressive disease stinks but I find myself more afraid of the progressive genetic disease that keeps me and the doctors guessing. When I was diagnosed with CF they told me that with the proper care there is no reason I couldn't live to be 40. When I was told about my mitochondrial disease I was told that I may be in a wheel chair by 40.

Why am I putting this in my blog? So that people can read it and understand, hopefully; that I don't want your manufactured drama, your rumors and lies, and I don't want your pity. I want to live life, help my friends and family with real problems. I want to love (as sappy as that sounds) and not get hung up on the little things, and I want that for everyone else too. And I'm also putting this in my blog to get it off my chest, as it has been bothering me for the past few days.

honestly sugar coating...

Today I got to go out painting with the gals and had a good time. I have been in sort of a funk these past few days, but today cheered me up!

May is cystic fibrosis awareness month, so I decided to post random CF information on my face book page in hopes to increase awareness. I even thought that if I was honest on how I feel everyday (for the month) that maybe my family and friends who did not quite understand might understand a little better. But that task was a difficult one. I don’t usually like to post things about my health (specifically relating to me that is) because I’m usually not feeling energetic , often times my sinus’s are really causing me problems and well, most of the week my muscles hurt. Whenever I’m honest, it feels almost like I’m complaining…
So my dilemma is should I be honest or sugar coated?
Being honest ensures that there will not ever be a doubt about my health or how I feel. There will be no chance of denial from my friends or family. No chance for them to make comments about my CF being different from regular CF and no chance at any them being able to say things like, “I didn’t know she was sick” or “I had no idea she had that problem.”
Sugar coating allows me to be able to appear “normal” or “healthy” at times when I’m not. Since I have an invisible disease people can’t always tell when I feel less than 100%. I would probably still not get the support in my endeavors to cure CF or mitochondrial disease that I would like and I doubt any of them would rank it as a top 5 priority in life because after all, Chrissy doesn’t suffer too bad.
So I am thinking maybe I can mix the two?
Be honest but not blunt, be optimistic but not delusional, and be upfront be not completely open. I am usually pretty open about my health when it comes to my husband or the cysters and fibros I have met on line but for some reason I’m not able to be that open with family and friends.

So for all of my friends and family (and other people who read this blog)…
If you don’t want to really know how I feel… don’t ask. I don’t like feeling like I’m complaining, I like to be positive. But some days I just can’t be. I can’t smile as often as I would like to, I can’t do the things I used too and I’m too tired to always put up a facade. We’ll use a code; ask me how the weather is…

For those of you who do want to know I will do my best to paint an accurate picture when you ask (although it will not be a vivid picture, lol, just accurate).