I should have updated sooner, but as you’ve seen I had a lousy week last week. So here is the official Angel – CF update.

Angel’s lung functions went up – but only by 3%.  This is not a good amount of improvement. We were allowed to go home, but our Pulm wants more improvement.

In case you missed it, last year I posted about our journey to the CF diagnosis (Here: Part 1, Part 2, Part 3). Because of that journey and the low sweat chloride levels, plus her generally clear lungs there has always been a niggling of a doubt about her diagnosis. Not just for us, but for the pulm as well.  After x-rays she’d make statements like “her lungs look more CF now” and other statements like that.  There was always a “maybe-probably” to it.

Well there is no more ‘maybe-probably’.  Our pulm stated that any doubt is fully erased. You do not get Pseudomonas unless you have CF.

For now, we’re home.  For now, we won’t be checked in.

We re-ran the sputum culture. If it comes back positive we start a new lifelong treatment cycle.  The TOBI (antibiotic) will become a permanent fixture. Every other month she’ll be on the antibiotic. We’ll know by the end of the week.

So that’s where we are.  Continuing on our path with our Angel a little the worse for wear.  After this week it may be a regular regimen of medicine, but no hospital stay for now.

All taken w/ Canon Rebel XTi.
For week 7/12-7/18/10

This week was crazy bad (see previous post), but it also had some good things. Today is my birthday and I want it to be good, so I’m trying to remind myself to just breathe and remember the good things. These pictures help.
I did attempt to re-start project 365 this week, but I forgot on Thursday (worst day) and decided to try again next week, so hopefully that will be happening in the next few weeks. I want to get ahead of the game by a week.
I also got a new photo-editing/managing software program this week. I’m wishing I could afford the pro version when it’s time to buy, but I’m afraid it’ll not be happening this year. Either way, I’m loving my new editing software and will tell you more about soon. It’s my new best friend.

Onto the pictures!

Waiting on a train

Spanning the distance

Bridging the Gap

Lines Across the Times

Tying it all Together

Lost along the Way

Pretty Hair for a Special Day

JUST B-R-E-A-T-H-E
(This was the test that determined if she stayed home or was checked in. She just had to breathe well. She didn’t, but she breathed better than last time.  That’s my Angel’s RT in the picture with her. She always makes it fun for Angel!)

It’s a lot this week, I know…but Sunday was a good photography day. I ran out and took a little photo walk, thus the railroad theme for most of it.

Go over and visit Lotus for some more great WW’s!!!

Ever had one of those days? How about one of those weeks? Months? Years?

I’m in the middle of one of those weeks.  Coming about 4 weeks after one of those months…which is turning this into one of those years.

Let’s focus on this week.

My birthday is coming up (this Sunday).  For the past several years my bday has not been anything spectacular, and in some cases it’s flat out stunk. Not sure why, I just haven’t had the birthday Gods on my side or something.  This year is proving to be no exception.

In one week we’ve had a trifecta of appliance crap, been overcharged by WalMart Eye Center, and lost the ability to finish my deck for my bday (all I wanted for it), oh and the plants I purchased for it are pretty much dead (my fault, I shouldn’t have bought them until it was done.).

First, over the weekend the fridge died.  Freezer worked fine, fridge wouldn’t cool.  Appliance repair guy came on Wed.  Assured me w/ the statement, “It’s a $45 part.”  Then proceeded to charge me an hours labor for a 30 minute job (that’s $60, mind you), AND $40 to defrost my fridge with what sounded suspiciously like a hair dryer.

Thursday morning I went to give my two little stinky angels a bath. Oldest was washing dishes.  All of a sudden, there was no hot water. Well, crap.

I pull open the utility closet and the pilot’s out on the water heater.  At least, I think that’s the problem.  If it wasn’t, it sure became the problem when I turned the whole thing off.  Archie came home and re-lit the thing and we have hot water again.  We just aren’t sure how or why the pilot went out and hope it’s not going to crap out again all too soon.

Not four hours later I stepped outside and the Air Conditioner sounded really loud. Thinking it was odd I walked on over to find the fan not spinning.

GREAT.

On our side for that one is our neighbor is an AC guy and he came over to take a look.

But seriously?

Why does Murphy hate my birthday so much?  Or, for that matter, me?  I didn’t do anything to him.

Well, he can go to Hell.  I’m taking my birthday OFF.  I’m not going near an appliance, a vehicle, my computer, nothing that can break. I’m staying in bed and pretending it doesn’t exist.  I’m really tired of this crap storm.

*******************

P.S. For those who haven’t seen me on twitter or FB, here’s the Angel update -

SHE’S HOME!!  No hospital stay for us!  Just as suspected, we are now officially, 100% w/o a doubt a CF family. You do not get psuedomnas if you don’t have CF.  The culture results will NOT send us into the hospital, but if they are positive we will be starting a lifetime regimen of one month on/one month off on the TOBI (inhaled antibiotic through nebulizer – very expensive stuff)…but we won’t know that for a week.  I’ll keep you posted!

P.P.S. I’ve been trying to get back into 365 by getting a week in advance done.  I’m afraid I missed yesterday w/ all the stuff going on…so it’ll be another week or two before I get those started again – but I will be participating in Weekly Winners again this week. Have a few pictures to put in once they’re edited. I have a trial version of a new photo manager/editor that I LOVE and plan to buy once we have the money. I’ll tell you about it later.

P.P.P.S. I actually have a bit to post about…but have been trying to skip days until I got more to write.  I may just blow off that idea and do a random post of stuff. Who knows….

Today is the day.

Angel goes in for her checkup after her rounds of antibiotics.  It’s time to pay the piper.  There are three possible scenarios after today.

1. Her lung functions have not gone up – hospital admittance, do not pass go, do not collect $200.
2. Her lung functions are up – go home. Get a call in a week that her sputum culture results are bad and we need to check into the hospital.
3. Her lung functions are up – go home. No further issues.

Now, obviously I want #3 to happen.  I want her lung functions up and I don’t want a call in a week ordering us back in. I want her to be clear.

I fear the worst.

Stress for Archie and I is high today.

So I turn to a show I never watch, to find a pair of beautiful young women to inspire me and give me hope.  Two sisters, Ali and Christina, both with CF (in a family of 4 children, all of whom have CF) – SINGING on America’s Got Talent. They give me joy, they give me hope, and they put me in tears every time.

*Otherwise titled “The post in which I piss people off.”

From the time our troubles started, from the time we first realized there was just something ‘off’ about Riley.  From the day We realized Angel’s torticollis was more than just a lazy neck and a heavy head.  From the day we realized Angel’s cough was going for a month and showed no signs of letting up. From the first time Angel intentionally injured herself. From the first toys Riley lined up.  Every day, every night, every in between we are aware.  We notice when Angel runs out of steam before she’s played for ten minutes.  We notice when Riley is starting to retreat into her shell. When we’re in danger of a meltdown of epic proportions.  In danger of a night of a screaming, pouting, angry Riley.

We notice it all.

We see it in the every day.

We see it in the little things.

Every action, every reaction.

It’s a part of our lives.  It is our every day.

Then you come along and say “But she looks just fine to me.”  Or “She doesn’t seem autistic.”  And of course the “She doesn’t act sick.”

I know you mean well.  Really, I do.

But I hate it when you say that.

It’s like you’re belittling our every day.

The hours at doctors and specialists.  The hours calming and refocusing an over-stimulated child (longest meltdown stands at 36 hours).  The hours a day hooked up to machines to live every day.  The way I lay awake at night listening to labored breathing. Check temperatures every time we feel a little warm.

I know you don’t mean it this way.  I know you just mean to say that despite their troubles they look healthy and happy.  That unlike other children with special needs it’s not a visible/noticeable difference.

But I hate it.

Just say they’re beautiful.  Say they have a great smile. They look like they’re having a blast.

Don’t contradict what we KNOW.  Riley does have autism (no matter how mild). Angel does have CF, and right now she is sick (and we spend every day wondering just how badly it’s holding on).

It is our every day.

We try to live life beyond our labels – and we only succeed by accepting them and integrating what they mean into our lives.

Gimme head with hair
Long beautiful hair
Shining, gleaming,
Streaming, flaxen, waxen

[Show as slideshow]

[View with PicLens]

The time has come, the girls are at the age for “pretty hair”!  The whole reason I wanted girls…to be able to do their hair and play with it and style it.

They still don’t sit perfectly still.

There are occasional struggles.

But we all love “pretty hair”.

Three years ago we got the diagnosis, Cystic Fibrosis. The pulmonologist assured us that over the course of the next few years we were likely to have more difficulty from Angel’s “other issues” than we were from the CF.

She was right.

For three years we’ve avoided any serious medical ailments from her CF.  One bout of croup is the worst we have suffered.

For three years it’s been a diagnosis. Nothing more.

Now it’s a reality.

On my last day in Buffalo the real world invaded.  The pulmonologist called my cell phone. We had been in the week before for Angel’s yearly testing.  Blood work was drawn, lung functions done, and a sputum culture.

The results of the sputum culture had come back. For the first time ever, Angel tested positive for Pseudomonas.  We were already on Cipro (her lung functions have been on a steady decline & she’s been having some labored breathing)…we had TOBI added on (a $3000 drug, pre-insurance! *faints*), which is an actual inhaled antibiotic.

My baby is sick.

She runs and plays.  She laughs and sings.  But her body is slowing her down.

At night she rasps and wheezes.  She’s now chained to her machines for an hour each morning and evening. Trying to fight this off so that she can run and play without getting so worn down so fast.

We let her run.

We let her play.

She needs it. WE need it.

Our baby is sick.

It’s no longer something that’s in the background.  It’s staring us in the face and gnashing it’s teeth.

I’d come out fighting, but I’m worn down too.  So instead I fight quietly. I pray to find my strength again so that I can be strong for her, for my family.  Life has tossed me about lately, the phone call from the pulm was the last in a string of crapstorms swirling about me.

But then I see her face. Those huge brown eyes that have melted my heart from day 1.

That smile that manipulates my anger into a grin, even when I don’t want it to. The goofy playfulness that can turn the grumpiest grump into a grinning fool.

I know that she’s too little to know what her tiny body is trying to fight off. I know that she’d rather run and play then sit hooked up to machines and fed medicines.  I know that it all makes her sad.

And that’s when I know.  I’d give anything to keep that smile.  That goofy grin.  The annoying repeating chatter that fills my days (and sometimes my nights).  The songs she makes up.  The piercing shriek of a scream she emits that makes my ears bleed, but show that her fire is not gone out.

I will find my strength to fight this thing.  The Mama Bear will emerge, and we will fight the now present danger of CF.

Diagnosis has become reality.

Now we brace ourselves for the fight.  We will fight for every tomorrow we can get. Angel may be tiny – but she’s got a strength and joy inside unlike anything you’ve seen.

I believe that if anyone can win the battle against this disease it’s her.

And if my littlest, my tiny Angel-girl is strong enough – I sure as hell can be too.

In two weeks it’s time for my Irish Twins birthdays.  They’ll be five and four. 

Riley is trying very hard to read, and Angel is doing her best to follow right behind.

Riley can write her own name (forward and backward, literally), and is figuring out how to write the numbers 1-10.

Angel is curious…about EVERYTHING. We cannot keep her out of anywhere from touching anything.  She is the child that as you run through the store runs her fingers along the shelves and tries to touch everything she sees.

Riley is cautiously curious outside of the home and her comfort zones – but around people she knows is talkative and friendly.

They are each others best friends and worst enemies. 

Technically I should be signing Riley up for Kindergarten based on her age. That doesn’t seem real. It seems like just yesterday that I was in the hospital watching them decide the new pope on TV while wondering when the pitocin was going to make labor actually hurt (didn’t take more than a few hours).  Or just this morning that I walked the four miles to and from Dairy Queen to try to spark labor for Angel, and lamenting the fact that it failed (it didn’t – just took two hours to hit me). 

The days of diapers are finally gone (almost – we still wear pullups at night).

The days of learning and growing and wonder at the world around them have arrived.  They are curious and thirsting for knowledge – and I can’t wait to keep watching them learn. 

I miss my babies.  More than I imagined…

But I’m loving the excitement of my big girls and their big personalities.

Angel had her go-round with the Developmental Pediatrician on Monday. Avoiding discussion of the horror of a day surrounding said appointment, I’ll get right into the actual facts and (lack of) diagnosis!

The short of it – we may never know what causes our little one to struggle.  It may be that she was born so early, or just a quirk in her brain that makes her crooked physically and mentally. 

However, that won’t stop us from a) looking for a cause and b) getting her all the help she needs.

To that end, the Ped did see her unevenness in her gait (we walked and/or ran down a long hall to display), and tone.  She again listened and heard all I had to say on everything we’ve been through the past couple of years with her.  She gave advice and took a good look over her, just as she had with Riley.

Her solutions here were a little more complex, but not by much.

1. We are doing an MRI on her brain.  Taking a good hard look to be sure that there is nothing specific or noteworthy that could have caused this.

2. We are doing a full-run genetic test. AFter a pre-approval by her dual insurance plans, of course. 

Why are we doing these?  Because the ped recognized that almost as much as we want to learn the HOW’s of Angel’s issues…we also would like to know WHY’s.  She said that while it is likely just her early birth date, we should exhaust the remaining bit of question.

And onto the HOW’s…

1. Restarting Speech Therapy
2. Restarting Occupational Therapy
3. Having a psychological exam done to determine need for Behavioral Therapy
4. Returning to the Feeding specialist for  monthly visits
5. Restarting Physical Therapy

All of this will be done at Riley Hospital, and all should be covered by insurance (if not primary, then secondary) as part of her diagnosed issues.  Thank goodness for Indiana’s wonderful Special Needs insurance (now if only we could get Riley qualified for it *sigh*)…it has saved us more than I can ever say.

And that is where we stand now.  Therapy, therapy…and more therapy.  I’ll be doing a bit more traveling once it all gets started…but if it helps, it’s all worth it.

I spent the better part of the weekend (from Thursday to Sunday) sleeping.  I was sick, sick, sick…and have just now started feeling better.  The worst part of being sick was having two sickies with me (*thing 1 and thing 2*) that were just as miserable as I was. 

Riley recovered fast, but it clung to my Angel like  glue. The cough lingered and she was put on antibiotics (drugs…woohoo).  She recovered a bit, but continued to cough.  And cough. And cough – waking up at nights.  It wasn’t continuous – just lingering. So, as luck would have it we had our regularly scheduled visit to the CF clinic yesterday. Her lung functions have gone down, so the pulm has extended and increased her antibiotics….and added steroids. 

So after all of this, I’m finally back.  Still keeping an eye on my baby – but I’m here!  Sorry I was so MIA.  Whateer this summer cold was made of, it kicked my ass!

And the Rock and Roll?

Well, it’s RIGHT HERE!!