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...

I've been asked by the OR CFF to be the speaker!!!

Wow!
I am humbled and excited, nervous and thrilled all at the same time. I've been asked to speak at this years annual "Chef's Dinner & Wine Extraordinaire" for the Oregon CF chapter! It is their largest fundraiser. It is blacktie...I am so excited to be even a small part in getting the much needed funding to advance research and therapies to try to stop that dang monster CF from destroying the lives of too many families...I had some much needed quiet time this evening and really wrote out whats on my heart. Our journey. our struggles. our passion. our drive. our goals. our ambitions. i am pleased with the outcome. i believe my speech is complete! although, being the type A that I am (NO! not ME!) I will read and reread it and maybe change things around a bit or what have you. but the nuts and bolts of it is just how I would want it to stay. An amazing opportunity.
I have to say that sweet Conner man is doing so well these days! He's really perked up since making it home from his wish trip to Hawaii!!!! His oxygen needs have lessened, his smile is back, his zest for life is back, and yes even the fighting with his brothers is back too! that is a sound that i used to dread!!! but let me tell you, after weeks of seeing him just slumped over, not even enough energy to talk or fight...i WELCOME the shift! Bring on the fighting! I am just so glad that he can breathe again! I took him to clinic on Monday and they didn't even have us do pft's or check his o2...that was just a relief. Not sure about you, but i HATE numbers. Especially when they're bad numbers. I don't want them floating around in my brain causing me more greif then Im already dealing with. Phew. Conner will now go to CF clinic AT LEAST every 2-3 weeks they want to keep a very close eye on him. Unless he gets sick, in which case we take him straight in to be admitted. We filled out the forms to extend his home tutoring thru the school district. that was a hard decision to make. first and foremost I want my child to be healthy and safe. but at a very VERY close second i want my child to have an AMAZING quality of life. so to make that choice to not risk his health by sending him into germville was a very tough one. but i really feel it was the best decision given his current health status. we will make arrangements for his friends to come to our house, or maybe he can go to school when they have special activities like library day or something...it's all about quality.
today was also a very bittersweet day for me. i got his paperwork turned in to the local DMV office and got his disabled parking placards. the handicapped parking. ugh. thats a tough bullet to bite. who would've thought that would be so difficult. I was fine talking about it before they actually put them in my hand. i wasn't prepared for that today. i thought i was just dropping off paperwork...but she gave them to me right there...and said "oh gosh honey, this little guy is so young. bless his heart. best of luck to you" and that was IT! done! my heart sunk. my eyes stung. reality sank in.
cf...i hate you!!!

Aloha!

Halleluyah i thought this day would never come. Conner has been fever free for 29 hours and his rash has gotten much better. So looks like either tomorrow or Thursday we are going home, YA NO MORE HOSPITAL! (well for awhile anyway!) And since the fever went away after they stopped the IV Zosyn, we are assuming that was the culprit. Doc says that can happen at anytime, even a drug you use frequently. I shouldn't say with any drug, with specific drugs, like zosyn it's in the pipercillin family which i guess is famous for these drug fevers. So no more zosyn or any of it's cousin meds either! ;)
We've got Conner's make a wish trip scheduled for Saturday thru thursday then daddy will have the rest of the weekend to be home with us, for some much needed family time!
I am well aware that Conner is not at 100% for this trip. We are taking along our FAA approved oxygen concentrater with lots of spare batteries fully charged, and if you've been following me a bit, or have known us awhile you'll know that we do realize that Conner may be readdmitted soon after his wish trip. It seems anymore he can't make it past 4 or 5 days w/o iv's before he gets another infection, which is so frustrating.
I've learned to respect CF greatly. I've also learned that having mrsa and pa is really not a good combination. it can make your body weak and with no reserves to battle lung infections because your body is constantly fighting anyway. But I also learned that i am hopeful. And hopefully optimistic. This time about a week ago, or maybe the week prior who knows anymore, I was feeling pretty beaten down and even "raised my white flag in defeat" but no more. I've learned that those feelings will come and go, you can't be 100% positive, 100% of the time. Just as long as you realize that you can't stay in that negative, frustrated space for too long! So game on CF, it's GO TIME! I had my moment of doubt, but no longer. Meet the new and improved Sarah, and get ready to be TAKEN DOWN! (oh and by the way, PA and your bff MRSA, you're up next!)