PFT's are for the "special Cystics" apparently...

So another day, another CF clinic appointment. We're on the 3 week schedule. I'd be lyeing if I said that it didn't bother me, but you know what, we're getting by...day by day we're walking thru it all. No more pft's for my sweet angel. He's too out of breath. His lungs are too sick. Kind of pointless now. I think his last pft had an FEV1 of 33% or so. So no need for lower numbers. Wish they were higher. I cringe when I see people complain about their "low" pft's and it's like an FEV1 of 90%...oh how I wish we had that number. I bet Conner had that number when he was like 2 years old. I spent alot of time the other day, pulling out old pictures of him, of all the boys, of my husband and me..just reliving family memories. And my breath was taken away from me when I saw old pictures of Conner. To me he's always been so skinny with all his digestive issues from CF...but I saw all these pictures of him from about 18 months when we got the disease on track and weight gain was quick thru about age 4...chunky cheeks, full, round face...regular colored skin...I never realized how far he's come in these past 2 years. How bad it's really gotten. I've got all his numbers down to a T in my brain but I never caught how far gone he was looking at him physically. I'm sure it's because it's been so gradual and over time, and I see him each day that I didn't notice. but it is just night and day. Unbelievable. A friend that I know whose known Conner from back when he was 4 and had those glorious round cheeks came over the other day after not seeing us in a few years and said to me "WOW Sarah...he looks so much sicker than he used to" (and it's ok for her to say that...no heads were bitten off...) I kinda laughed it off...but then I looked back to all the pictures and she's right. wow. how did we get so far gone? Which springs up a TON of mommy guilt...which I know is ridiculous but that doesn't stop it from coming into my head and torturing me....what could I have done better, differently, remember that day when we missed a treatment...what have I done? on and on and on. Ridiculous I know. But there, nevertheless. Look at how he's changed...this is him 18 months...
Beautiful full chunky cheeks and it wasnt really baby fat because before this he was a very bad case of failure to thrive. So this is him with his feeding tube getting him to where he should be...before all the wonderful PA and MRSA colonizations in his lungs... and the years have not been kind to Conner...this is how he looks today...4 years later
Maybe it's not noticable right away...it took me a few minutes to really see it all. And this really isnt' a good picture to make an example out of, because I've already did some editing adding more color to his face...so take a peek....what do you see? I'll tell you what I see. I see a very skinny face. I see very tired eyes with huge black rings under them from his lack of adequate rest and his sheer exhaustion from just breathing, I see a "V" in the bottom of his throat, his airway....you Cf'ers probably know what I'm talking about for the rest of you, that area at the bottom of your throat, but just above your collar bones in you and I isn't really prominant. It's there...but it's not really noticable. His is. Because when he breaths, he's gasping which makes that little "V" area sink in deeply with each breath signaling the need for additional oxygen supplementation...looking even more at him (but you can't see in this picture) I see just how skinny he really is. If you put your thumb and your pointer finger together to make a zero, it's pretty dang small isnt' it? Guess what, his arms are so skinny you can put your fingers like that all the way around his arms, upper and lower, and if you make it just a tad bigger, his upper thigh will fit in there too! I see his ribs from his back, right thru his skin. Like seeing a walking skeleton. He's had a feeding tube ever since diagnosis at one year old and it used to help us greatly, but now he's on a very high calorie formula that he gets 4 cans of a day (375 cals each), with 45mls of Microlipids (straight fat) with each can, and 4 Boost Plus formulas to drink each day and we STRUGGLE to keep his weight stable. He barely eats anything anymore, its a good day if he eats one thing...honestly. his body can not eat and breathe simultaneously. You think about the energy it takes you and I to breathe...it's not even noticable right....well for him, his body is working so hard just to breathe that it burns thru a ton of calories just to live. thats not even to walk or to run or anything. thats just to sit down and breathe. That is so scary. How could I not have noticed? I've been so wrapped up in the trenches of CF, making the appointments, dispensing all 15 meds 4 times a day, cleaning the nebs between each treatment, administering the bolus feeds, helping him drag his oxygen around, carrying him because he's too tired to walk, helping him in the bathroom because he's having diarrhea, going to the pharmacy to pick up all these wonderful oral antibiotics he's grown so accustomed to, talking on the phone ordering more IV supplies or formula, driving to appointments, fighting with the insurance, arranging his home tutoring, keeping at his side while he's struggeling in the hospital, cathetering him when the IV meds cause his bladder to stop working, hooking up his VEST therapy, or doing hand pounds on his back, or helping encourage him to do PEP, mixing his two inhaled meds that don't come prepared...handeling needles and glass tubes etc, and trying to just let him be a kid. and trying to raise two other children. and to find that same amount of time and attention to give to my other two kids. and to my husband. and to me. quiet time? nah... I can see just how much CF has changed me, as a person, as a wife, a mom, forever. Sometimes I think for the better. Other times I'm not too sure.
I just can't believe how far he's gone. How quickly it all has happened. I thought people with CF were supposed to live longer lives now...with all this wonderful therapies and new drugs and advancements....I was hanging on to maybe the median age of 37....and even that seemed way too young. Hell I'm not even 30 yet and I don't feel like I'm halfway done living my life...theres simply too much to do. Then I think about how much he has taught me these past 7 years. How he's blessed me. How I'm so lucky to be his mom. How I wouldn't trade all those treatments and hours each day dedicated to destroying CF for one second. How many lives he has impacted and changed in his short little fragile life. It is too hard to even fathom life without him in it. I just can't picture it. I'd be bored to tears! Raising two healthy kids would be like doing nothing. only annual well child check ups and the occasional sick visit. No more need to drive an hour to CF clinic. No more hospitilizations for weeks on end. No more home health company. No more feeding tubes and formula, and nebs, no more vest or pep...but more than that....no more grade school until my middle son gets to kindergarten...a huge part of me would be just done. isn't that crazy to even consider? what in the world would i do?
I'm fighting this fight as hard as I can, and while theres not much noticable that I'm doing, if you spent a full 24 hours with us at our house you would see our daily battle, just to make it thru one day getting to be at home w/o being in the hospital. You would see how Conner just does all these treatments most of the time with a smile on his face and no complaints. You will see how his younger brothers run around and play and Conner sits on the couch and watches and laughs because he doesn't have the energy to join them. But sometimes he does. I love when he does. He'll run around the living room and into the kitchen, laughing and screaming with his brothers at the top of his lungs, and his brothers at the top of their lungs til I'm sure someone is going to call CPS because they think somethings horribly wrong at our house....now the old sarah would've put a stop to that like 10 minutes ago...but now...i sit back and watch and smile, or most often I join them and run around and scream with them...then i get Conner his oxygen and sit with him when he needs to take a break to catch his breath. ooh how I love the sound of screaming, healthy lunged children....what a perfectly "boring" and "easy" blessing it is. Not all time consuming...yet no will to fight harder...no waiting on a foundation to find a cure...yet still fighting the fight all my life....
oh who even knows where I was going with this blog...I think I was just going to write about Conner mans CF appt today and look where we've ended up...talking about the ugly reality we're drowning in each and every day...not at ALL for pity...because I'm proud to be in this fight. But oooohhhh how I wish it didn't exist.
how i wish nobody had to suffer thru it.
how i wish beyond wish that it wasn't my son...