Hellooooo ElectroFlo 5000.

Okay, Okay. So now that I'm done with all of the girly griping, sorry, I digress. Let's move on to more important things....Like airway clearance. Yaaaaay!

I have been hearing about percussors and flutters and acapella since my debut on the scene of the online CF community roughly a year ago. (give or take a week or two).  As time has gone by I have increasingly questioned the efficacy of The Vest. Dont get me wrong, we still use it religiously, but its just one of those things that you wonder about. It never elicits a cough, how much can it really be moving?

So with this in mind and a couple of references to this device by some others, we ordered the ElectroFlo 5000. Its a percussor. Its supposed to be closer to manual CPT than The Vest. Its supposed to be amazing. We shall see.

The ordering process was super easy and the people at MedSystems Inc were very nice. I was on and off of the phone in a matter of 10 minutes, max. This is quite possibly because we are paying for this piece of machinery out of pocket. I think that if our insurance had covered it, it may have been a more involved process. The total cost is $2750 and we have 30 days to decide whether its something that is going to be helpful or useful for Natalie.

Quite frankly, I don't care about the money. I care whether or not this thing is going to help. It supposedly allows for a more focused therapy in the places that you need it. It supposedly penetrates deeper than The Vest vibrations. That would be nice. Natalie has some areas in her mid lobes that are deep, they are only visible in the lateral  view of her CXR....

I'd love to have a crack at them with a 3k handheld vibrating machine.....and it looks like tomorrow I will be getting the opportunity.

In the midst of all of this I realize that I am getting excited over medical equipment. I also realize that I am getting excited over spending close to 3k on a handheld, vibrating piece of metal and plastic.

Now those are the true joys of CF....the moments when you realize you live in an altered state of "normal", and you're completely okay with that.

 Because the truth of the matter is that I would rather my altered state of normal over the alternative any day of the week.

My best to you. ~j

8 am on a Tuesday morning.....what could possibly go wrong?

Let the food wars begin!

I guess if I were 3 and I had people shoving medicine down my throat and up my nose and strapping me into weird shaking contraptions, I would want to control what I could too. The problem is that Natalie chooses to control food. She doesn't realize what she's doing.....

Other times I think its just genetic.....there are some picky people in the family and her eating bears a close resemblance to them. The good news is that they all have outgrown it, the bad news is that it took each and every one of them a minimum of 20 years to accomplish that. I don't have 20 years to wait for her eating habits to change.....

If its green you can forget it, if it has more than one part to it (ie. a hamburger) you can forget it, if it requires being chewed for more than 15 seconds you can forget it....this leaves very little to choose from, but I am ever diligent, ever trying new things.

Sesame Street is blaring on the TV over the sound of the vest between phone calls to the MD office...... Natalie is coughing, but no fever, I think this is worse than being "real" sick. They wont do anything if she isn't running a fever, we just have to up her treatments and hope for the best. If they had to watch her cough and gag and sit on the sideline helpless to do anything but encourage her, maybe they would be more concerned, I'm not sure. Maybe there really isn't anything they can do, maybe we're already doing everything that we can.

Other than the (make you gag along with her) cough that Natalie has....she's fine. No fever, no malaise, no other signs or symptoms which makes for an awkward situation. To go out or not to go.....she needs her exercise, that will help, but I don't want to get her over tired or overheated and potentially do more harm than good. ugh!

Maybe a little further evaluation of the situation is in order, its early. Natalie was up and moving early this morning....that means we're all up and moving early.

Thank You.

When my CF journey started, I was alone. Not just the "oh I'm lonely" type of alone, real alone. My sister helped with babysitting while I went back to work, my family would have done more if I had asked, but as far as moral support, I couldn't find anyone who understood....really understood. Newborns, NICU's, CF specialist, tests, blood draws....It made me want to crawl into a hole....A very deep dark hole. It made me hate myself, after all, I had given this little baby this dreadful death sentence. It frightened me beyond all belief, I think I thought about death, funerals much more then than I do now. If I could put it into one word....despair. Total and utter despair.

As with most of my stories, there is a light, however dim it may be, there is always a light.....

Strange became normal, fear became understanding, and this fragile frightening baby grew into my life lesson, my life's greatest frustration, and one of only two people on the planet that I would give my life for.

Almost a year ago things changed dramatically.....I got a laptop for my birthday and it was over....O-V-E-R, but in a good way.

I found the CFF on FB, CysticLife on FB, blogs, people, friends. People who however far away they may be, were right beside me, fighting everyday. *sigh* what a relief.

Today, I am so grateful for the people Ive "met", and grateful for those who remain "unknown". I love my online peeps as much as you can possibly love someone you "know" online. You all make my life easier, make me smile a little bit more, and make this CF life bearable.

So this one's for you, many many thanks, you could never know how much you are loved and appreciated.

blessings to you all this weekend, Im out. ~J

The only sure thing in life is that things will change.....

I don't want to make this post a depressing one, Natalie is fine....we're just starting down a new path.

Today was her clinic appointment. She's lost weight. That is not a really huge shocker seeing as how she was in the 70th percentile last visit. Natalie's weight is much like a roller coaster, it has its ups and downs....we just do the best we can. She hasnt fallen far, just into the 60th percentile, so we have 3 months to make it up before they start to intervene. Our clinic is real big on keeping her weight up, so we shall see.

Natalie's O2 sats (oxygen saturation) have been on a steady and progressive decline since January. January. January. I get so sick of saying that month, of reliving that month, but its when all hell started to break loose....a month I'll remember for life, a month when everything started to change. Anyways. Seeing as how there is a noted difference from January to March to June, we're starting on Pulmozyme. Im crushed. Not because I dont want to give her the medication, but because Ive spent the past 3 1/2 years bargaining with myself to make it alright in my head, "well at least she doesnt have to....(fill in the blank)...." Now those days are over. No more denial for me. We are finally, totally, completely CF patients, the vest, the nebulizers, the enzymes, the whole shebang. Oh how I wish that werent the case.

Natalie's doctor can see the difference in me. He looked at me, cocked his head sideways and says, "You look tired". This is simply a nice way of saying, "You look like hell, like you've aged 10 years in the past 3 months". I cant argue with him. I simply respond, "yeah, a little, but Im okay." Okay, fine, great, wonderful, fabulous, I am AWESOME....just ask me.....(that was a tad sarcastic....okay maybe a lot sarcastic) He just smiles, looks at me from the top of his glasses, pats me on the leg and says "Its okay Mom, she's doing just fine". Ive never been a huge fan of anti-anxiety meds, actually have been more of a "heal yourself" kinda girl when it came to such, but today, today I would have taken something if it were prescribed to me.

I must admit, however, there was a different feel to the clinic today. I dont know if they were just trying to make me feel better or if VX-770 and VX-809 are finally making their way into common knowledge or what the deal is but I had two separate people saying "just hold on Juli, it wont be long....." It was awkward to hear from the RT, from the RN. Ive said it to myself tons of times, sat in front of the Vertex page and cried....trying to move that stupid purple line with my mind....but to hear the hope in their voices, it was kinda surreal. I cant say that it has made me feel any better though.....as my Maw-Maw used to say, Im from Missouri, cant tell me anything, have to show me :) (for those who dont know, Missouri is called the "show me"
state) Oh and if you dont know about the Vertex pipeline, there's a link on this side of my blog. ------>>>> It shows their progress and how close to availability they are.

Got a call from my local CF chapter....great, they know I exist :) j/k....I told the girl that my motivation comes in waves, it does. Ive been trying, Im going to get out there again, I am....I just need a minute....or two, or three. Its hard to be a warrior mommy ALL the time, sometimes I just dont want to be seen or heard, sometimes I just want to be normal. I hope that doesnt sound selfish, but sometimes I need it, we all do.

Anyways. Sorry for my absence and lack of photographic "pick me ups" but I am still chained to the Mac, therefore not the happiest blogger on the block. Will try to stay in the loop more, I know Ive been slacking....but it is Summer....that means making the most of my time with Caitlin, with both of them.

I wish all of you peace this evening. lots of love! ~j

laptop was KIA and Ive been MIA

I'm writing to you from a Mac....that's right, my laptop was KIA last week....such a sad, sad time. Ive been struggling to put together a post without erasing it and when the darn thing finally quit on me, it just gave me an excuse to not even try. Justin was kind enough to offer me this Mac, so I feel compelled to update. I don't like the way the keyboard feels, I don't like that it seems to have been made for someone who is left handed, it feels awkward, backward, wrong somehow, like I'm cheating on my laptop, so I'm just going to muddle through :)

We have had a busy week or two. Justin and I decided to pamper ourselves and the kids for the summer and get a membership to Francos, a very nice athletic club in Mandeville. Four pools, two water slides, sauna, spa, full upstairs gym, and children's areas that the kids are happy to get to visit, even if it means getting out of the pool.

Natalie is well for now, watching her like crazy....she's never been in a pool that wasn't our personal pool and exposed to this many people. It worries me, but the truth is that I want her to live as normally as humanly possible. I'll stop taking her if there are any signs that she may be getting sick from it, but she is just so happy in the water, I just cant see taking it away from her unless I absolutely have to. There will be no school this fall, she falls short of the October 1st cut off, so she has another year at home with mommy. That's not a bad thing for her, I think she needs the extra year, not because she is not ridiculously intelligent, but because she is just so spoiled, she doesn't listen very well, and this will give us a little extra time to work on that :)

Caitlin is enjoying her summer so far....she keeps asking me what we are doing this summer. I'm assuming that she means a beach trip or something along those lines. Ive tried to explain to her that its just not safe, I don't think she is grasping just how far reaching this oil spill is. Tar balls have been washing up in Alabama for the past two days and the oil sheen was supposed to reach the FL panhandle yesterday. Caitlin and I lost most everything we had in Katrina.....I know it sounds silly and its not even close to the same thing, but I just hate to explain to her that, once again, life as we knew it is gone, a trip to the beach will have to be planned far in advance, not the day before. How I hate to break that child's heart. She is so proud of the fact that she was born in Ft. Campbell, Ky. So proud that she was born somewhere other than here, she roots for the Titans and cheers for Ky in the college realm as well, even in spite of the fact that they are in the same conference as LSU.....but she is a Louisiana girl. Loves her seafood and her time under the live oaks at the park, and she finds beauty in our off kilter ways. I just don't want to tell her that its another change, not that she couldn't handle it, like i said before, maybe I'm just chicken :)

My accomplishments are limited to housework and work outs. I actually used the upstairs portion of Francos and worked out for a whole hour. Gonna try and do it again today. Another staff meeting, picking up Caitlin , squeezing in a workout ugh! guess I better get moving huh? ~J

Always and adventure, Always a story

I never realized how amusing my life is until I decided to start sharing it with you guys, not that I feel like my life is exciting, but Im hoping that maybe someone can relate, just maybe someone has been there, felt it, wondered it, seen it, hoping that somehow in the solitude that a mom can feel dealing with this disease day in and day out, I can find a connection, conjure a laugh, or make someone feel not so alone in their quest. Our day started out with grocery shopping. I am a nurse and I only work a "weekend special" for those of you who dont know, thats 2 16 hour shifts, one saturday and one sunday, so im pretty much out of pocket until monday, the good thing is that I am home 5 days a week with my little ones :) anyhoo, being gone that long requires groceries to be stocked so that there is an endless list of choices on the food end, i try to make it as easy as possible on her daddy, and in the end that means that its easier on me. On the way there, i did it, i admit it, i bribed a 3 year old, i told her that if she drank ALL of her scandishake, i would buy her a toy, and she did (YAY!) so mommy bought a toy :) it only took her 30 mins to pick it out too, i couldnt rush her, after all it was my own doing, right? i sat and watched her as she stood in the toy aisle, gazing in wonder at the what must seem monstrously tall and shelves of toy after toy, for a moment i envied her, how wonderful the small things are, toys, christmas lights, rainbows, sprinklers, all the things that seem common place and ordinary by the time we've grown "older and wiser" by the time we got to the checkout, the ice cream was melted all over the broccoli, but hey, we got an extra 600 calories in! woooohooo! :)Tonight we went to see "Princess and the Frog", it was Natalie's first trip to a movie theater. She kept asking over and over on the way there, "mommy, where is the DVD theater?" I suppose it was a cross between the fact that she knows that movies come from DVD's and us telling her we were going to the theater. anyways, i think i enjoyed it as much as she did, not that the movies was all that great, but I enjoyed watching her see everything for the first time, it must have seemed so big, so amazing, and for the second time today, i appreciated the little things just a little bit more because of her, because she is part of my life. My little girl is amazing, and she just happens to have CF.

Oh the guilt that comes with brushing her hair and telling her "No"

I love waking up in the morning to her morning breath and her saying "mommy, the sun is awake!". There is rarely a time when she doesnt start my day off in a really great way, I love this age and all of the curiosity and imagination that comes with it. Natalie is 3, but she is soooo smart....i know, i know, all moms think theyre child is the most beautiful and the most intelligent, we're supposed to :) After the initial morning routine has been completed, c'mon you know the drill......the fight to brush our teeth, whats for breakfast, what kind of "food mood" is she in today, enzymes, periactin, vitamins, 15-20 mins of CPT *whew* goes by quickly when you're typing it, but its at least an hour and a half process, she's a slow eater.....Anyways, after all of that comes the day's regular activities. I had to run to work this morning for a minute and so, of course, Natalie needed her hair brushed.....and this, of all fights, seems to be one of the most heartbreaking. I have detangler but she cries anytime she even sees a hairbrush. Yes, she has to have her hair brushed, yes, it must be put back, otherwise it goes in her mouth. But with all of the fights that i MUST have with her....the CPT fight, the blood draw fight, the chest xray fight, the nebulizer fight, i feel the most guilty about these, the dumb ones, I mean, in the grand scheme of things, what does her hair really matter, must she cry over this, must i be so insistant on the hair being brushed??? Then its off to the store to pick up some applesauce, the portable kind, that has the lids and are "single serving" so we can go out and about for a while without worrying about getting enzymes in her, and of course now there's the toy fight. Christmas just passed and i find it absurd that the child could possibly want anything more. We went way overboard for Christmas (we usually do) but somehow, even though there are still presents she hasnt fully appreciated yet, there is still the "I see, I want". Must she cry over a $2 toy? must i say "NO"? is the principle so important that I would have her cry over something so small when she has so many other, more important things to cry over? Day in and day out she tolerates things that other children have no idea about. Shes been poked and prodded and xrayed and tubed and monitored......doesnt this deserve a little wiggle room? Doesnt this deserve a break from "mommy the nag monster"? Or is it simply that I am so overwhelmed with guilt, i feel guilty that she has to endure this, why not me? Ive prayed and prayed, "God just give it to me, let me have her pain, let me have to take the meds, let me be the one to do the treatments, just let her be healthy and give it all to me" In the end, this is what we have been chosen for, in the end she must learn the lessons that all children must learn: hair and teeth have to be brushed, you dont always get the toys that you want, when you want them, everyone is different, friends are special people, sharing is a good thing, do unto others as you would have them do unto you, and Mommys are there to make sure you know how things are supposed to go, even when they themselves do not understand why they are this way.

The good days, the bad days, and the ones that make you want to cry......

I am a nurse by profession, a mother by the grace of God, one of my daughters just happens to have CF. I am a fiance, a daughter, an Aunt......I wear many many labels and all of them is a caretaker in one form or another, this is my lot in life, i enjoy it, i thrive in it, but when I percieve failure, I can make life very hard for me :) Especially when I am thriving on the professional end but feel like I am struggling at home, when my patients are all doing well, and I cant even get Natalie to take a bite of food, that day, that moment is a hard pill to swallow.......




Today was a good day........one of those, the ones that make you sit back and say to yourself, "Im okay, she's okay, we can do this" . Natalie has eaten well all day, 4 pieces of bacon and a piece of sausage with grits for breakfast, some apples and peanut butter with a pediasure for a snack, chicken and fries for lunch, and the list goes on and on, shes been a human vaccum cleaner today and that, my friends, makes for one happy mommy...........The problem, it seems, that there arent that many days that are just okay....maybe its just being in the midst of it makes your memory short, im not sure either way. I know that finding high calorie foods that are have real nutritional value has been the new mission in my life (aside from raising my two little girls). It seems like everyone is so calorie conscious, i have to do two rounds of grocery shopping, one round so Natalie has her high calorie, high fat, high protien diet, and another round so the rest of us dont blow up like balloons......the attempt at balancing is never ending, but what a ride, huh?




However, everyday is not so blissful. There are days when she would rather do ANYTHING other than eat.......days that im lucky to get 500-600 calories in her, days that i want to sit and cry, days that it seems that a feeding tube is coming and would be a blessing, days that i just dont think that God chose the right mother for this little girl because Im OBVIOUSLY not doing something right........then she crawls in my lap and says "mommy, i so glad youre here" in that sweet three year old voice, and i know im here for a reason and there's nowhere else Id rather be.