"Why is gramma so sick?"

Those are the words Conner asked me tonight at the dinner table. Brads gramma Shirley in Idaho has been hanging onto life by a slim thread this past week in the ICU on a ventilator. She's had moments where we thought she wasn't going to make it, then miraculously she'd wake up a bit to acknowledge the visitors, so it's been quite a roller coaster of a week. Well tonight, she gave up her fight. She passed on about 2 hours ago now...which brings about a very wierd mix of emotions, worries and feelings. Brad is very distraught, naturally. And I miss her terribly already. I feel a bit better knowing that now she's another angel watching out for sweet Conner along with both my grandpa's, my great aunt, and brads other grandma among others. Because we could really use all the divine intervention we could get! but now...


Now we have to figure out how to bring up gramma GG dyeing to our sons. We were planning on talking to the kids about illness and dyeing with everything that is going on with Conner, and now it's going to be very real. a very intense talk. Because now it's not taboo...it won't be a "someday" you will die type talk...it'll now be "today" your great grandma died...etc and im so very worried about that. Talking about death never was something that I felt would be easy to do, or for Conner as well, but now it's real and now it's going to be even more difficult.



He asked at dinner why is GG (great gramma) so sick and in the hospital...and Brad has real emotion right now having just lost his grandma...so now i'm very concerned about this discussion. and I'm just not sure what to do

Lordy, if anyone has any advice or ideas I'm all up to listening. Because I feel so confused just thinking about it.

Please pray for strength, pray for me, for brad, for conner and hunter and please pray for the entire Erikson family for their great loss tonight. And may GG Shirley forever rest in peace.
(GG surrounded by Michelle, me, Tammy and Jenny)

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...

Conner's famous again!!!

Well I guess I shouldn't be too surprised...but our home away from home, Long Beach, WA did a remarkable article in this weeks paper (yep...only one paper a week!!) Conner's had articles in papers before, but this one is truly exceptional. The writer, Amanda Frink did an AMAZING job capturing CF and our lives!!! You can read it HERE.

In other news, last night was Brad and I's counseling appt where we discussed the when and how to talk to Conner and Hunter about Conners declining health, CF, and dyeing. ICKY! Thats not a good talk to have! I feel we've made a solid plan, and I have complete faith that God will help to give us the words to speak, and the strength to do so. Part of me is still in denial of it all, quite honestly. I mean here's the best way I can explain it. So back in October when Dr Link sat me down and told me how serious Conners condition was, I went thru a good 2-3 week mourning period, where I just felt broken and was easy to cry...but as time went on, even though we kept going back to the hospital week after week up thru January it kinda wore off. I got numb to it. and life went on. Then when the second peds pulmonary cf doc sat me down in mid January before our trip to Hawaii and said the same things, i was completely taken back! why? because i was so numb. i refused to waste time FEELING the grief, and honestly, I just didn't want it to be true. So when he said that to me I again went back to that intense feeling of despair, but as we got home from Hawaii and his health is stable (not great, but it never has been) I'm back to my numbness. I don't believe it. It's easy to see and believe when he's in the hospital, horribly sick, but when he's home and doing ok I just can't believe it. So I pray that our plan will fall into place, that it'll open up a dialogue between us and Conner, to let him vent because we just know he knows whats going on but has been too afraid to say it, and to let us all just love each other and hug and support each other. Our plan with Hunter is very similiar, only the conversation will be different. He's only 4. It will be very devestating and quite confusing. It is a grown up conversation being given to a 4 year old child, with child like feelings and understandings. It won't be easy. But I pray that it'll be effective, and it will just continue to help us grow closer in this time that we have together.
Nothing else is really new, I think that's enough for one day, don't you?! Continue to love and support those with CF and their families. We all need strength and love and support to navigate thru life with his disease. But I still say we're the lucky ones, who learn in a very short amount of time the true meaning of life. and love. and living each day to the fullest. and that tomorrow is never a guarentee!
Lots of Love!

Open and Honest

Lets be honest here for a minute in time. I hear Cf'ers and family members all the time say that they don't let CF get to them, or control them, or affect them. But I'm sorry, is that really true or just something you say? It gets to me. I have my moments where CF gets to me. Those moments like today when I had to park in the handicapped parking for the first time because my 6 yr old son with can't make it long distances. Ya, that got to me. Then we get in the store and he immidiatly asks to sit in the cart! He can't walk around much. You bet that got to me! Today my middle child threw another all out tantrum at this store out of nowhere, his entire personality has changed since his brothers been in the hospital so much these last few months. Uh huh... that got to me too! I read on a fellow CF'ers facebook today that one of their friends with CF passed away today. My son has been using his oxygen more in the last two days again, so then my brain goes into overdrive. Damn it, people it GETS to me. How can it not get to you?! No way do I let it control my life, but I feel I give it the respect it deserves as a horrific opponent. I give myself the ok to have my times of mourning, then I wipe the tears from my eyes and put on my all too famous "strong mommy" face and dive into another day of it.
Today I paid very close attention to Conner's treatments and how long they take, and how long cleaning it all and preparing it all takes, the bolus feeds too...it is ridiculous! My child did 24 different treatments today, some nebs, inhalers, antibiotics, enzymes, etc etc...He also did PEP 4 times today, he did 2 bolus feeds today....i cleaned all the am nebs with the boiling water got it laid out to try and you blink and it's lunch time...so it's bolus time, it's enzymes, it's albuterol. it's hypertonic saline time. don't forget PEP. then he has a school teacher come to spend an hour with him. Then it's dinner time. it's enzymes it's bolus. Then bedtime meds...flovent, inhaled gent, hypertonic saline, flovent, cipro, zyvox and more enzymes for his night feed. Look I'm just being honest here. His entire day is about cf. his body is so worn out that he sleeps in, so we squeeze all of this in, in the 10 hours he's awake. How can it not get to me? He never complains, but it's not ok that he's sitting on the couch doing yet another treatment and watching his younger brothers running around playing. His free time is almost non existant it seems. There is always something. You clean up little bit of cf...then it's time for the next meds....
And since we're being so open here let me tell you why else I'm upset about. That damn Vertex clinical trial announcement yesterday by the CF Foundation. in a nutshell..."we're excited to announce a successful phase 2 clinical trial of this vertex compound, (that literally goes in and fixes the underlying cause of cf) We will be looking to move ahead to the next phase for patients to test out in the later part of 2010" . This stuff is amazing. it's specifc to Conners CF mutation (DF508)...which means his body makes the proteins its supposed to in the CFTR but can't release it as it needs to, to make it work properly. This magic new compound (a pill i believe) goes in to the CFTR, gets that protein out and where it's supposed to. When people did this clinical trial it corrected their sodium chloride so that when they were sweat tested for CF, they were NO LONGER POSITIVE!!!!!!!!! Look it's not a cure. It's something HUGE to be able to use until the cure is found. it's going to save thousands of lives. keep thousands of families together. but woot woot...guess who doesn't qualify.
yep you guessed it. I'm upset today to see this wonderful new miracle drug, probably our only chance to keep our son alive is going to be in patient trials but his lungs are too sick to qualify. WTF??!!! Let me tell you something, you wanna see if something REALLY works? Give it to someone seriously sick from CF. I bet the results would show thru faster. and guess what?! you probably just saved a life from being lost. I'm just so upset...to know we're so close to that miracle (prob wont be fda approved for about 2 years or more) and that it's probably going to be too late for us. so please excuse ME if CF gets to me. I'm not afraid to admit it. It does.
My husband and I have our couples counseling this next week and we're going to be making a plan to talk about Conners poor health and dyeing to the kids. We simply don't know where to start. Probably can't even choke the words out. But our kids are scared. They know whats going on, but don't understand it. It gets me. Big time. I can't go thru one day, hell one hour without thinking about something related to Conner dyeing. ONE HOUR! I tell you people this disease ENRAGES ME! It fuels me to fight harder. It's what gets me out of bed in the am and line up all the meds and start preparing them, administering them then cleaning it up..it fuels me to raise even more money this year then I have ever for CF...because my life will be dedicated to killing this disease! Whether or not my Conner will be alive to get to that miracle cure I don't know. But I know I will be. It's gonna happen in our lifetime. It's going to save thousands of lives. I will simply not rest until all the CF docs are out of jobs....
because we no longer need them...