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.

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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.

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.

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!!

(Part 1 is here)(Part 2 is here)

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“Cystic Fibrosis.”

The pulmonologist – one of the top in her field – had said it.  In relation to our tiny little baby girl.  I’d never known what it was like to nearly faint – until that moment.  I managed to hold it together, even though Archie was watching me with fear and concern.

I clutched the phone tightly as Dr. A explained that she had left instructions to get us in the following week so that she could explain to us better what CF meant. She reiterated again to not panic and emphatically said “Do NOT research online. Many sites will give you the worst case stories in an effort to raise money.”  She explained that Angel’s numbers were in the mid range for a very low amount of sweat – so we couldn’t be 100% sure yet. 

We would rerun the sweat test. We would have a blood draw for genetic screening. I didn’t want to listen anymore, but I had to so I could repeat it all for Archie.

We went back to the hospital a few days later, still numb and confused.  We met with the pulmonologist.

She said that Angel’s first test had had very little sweat on it.  But that “safe” numbers for CF were 0-40. Mid line (in the CF range but very low) were 40-60, and surefire was 60-100.  That Angel’s numbers had been 62.  That 100 was usually the results she saw with a positive result. 

She explained with reassuring tones that CF wasn’t the death sentence it once was – that many people today live long lives with CF.  That every case is different – that she just started treating a 45 year old woman who only learned she had it because her daughter was diagnosed.

All the reassurances couldn’t stop the sickening beat my heart had pulsed since she’d first said Cystic Fibrosis.  She answered all of our questions through my numb haze I can’t remember any of it…but told us to call any time we had any questions at all.

We went for the tests and were told that it didn’t seem that she’d sweat enough for the test to work, but they’d try to run it anyway.

It didn’t work – Angel does not sweat well.

It took two months and five more sweat tests to make the child sweat enough to run a second test. 

In the mean time the x-rays came back clean.  The genetic screen (which only screens the 80 most common genetic indicators of CF – there’s like 190+) came back clean.  Her weight continued to be low, her cough faded away into the night.  But still we waited and wrung our hands.

Archie moved back in and we worried together after we put her in bed each night.  Our family repaired itself, but our baby still hovered in the unknown.

Finally after 7 sweat tests we got enough sweat to test again.  Again the numbers came back elevated – but still on the low side. Dr. A consulted with another doctor and then said that they had to call it…it was Cystic Fibrosis.

In June 2 years ago we went into the hospital for a day long marathon of tests.  More x-rays and blood work.  A lung function test (which is FUN for a baby that reacts poorly – or not at all – to knock out drugs). A sputum culture – a day in the CF clinic.

Since then we’ve been blessed with the good fortune of Angel being relatively healthy.  After a year of CF we were told that her lungs were ‘starting to look more CF’.  But we’ve still had a peaceful time.

At the time of her diagnosis, the pulmonologist said “At her age you are more likely to have problems from her other issues like the hypotonia than you are from the CF.”  So far she’s been correct.  We only hope that it remains that way for a LONG time.

(Part 1 is here)

(Sorry for the delay…my host has been really cruddy the past couple of days and posting this second part has been difficult because it has been SO SLOW.)

So after two months of barky coughing, we finally got Angel into the pulmonologist.  She did a thorough exam, listened to our story and ordered x-rays and a bronchoscopy. 

The following Friday we reported to the PICU at 5:00AM.  Angel was prepped for her ‘minor surgery’ and her nose was sprayed with a numbing agent.  We were then shooed from the room and into the PICU waiting room where families slept and silence reigned. 

Hushed undertones of inane conversation crossed the table between us, our hands clenched together belying our internal fears.  The lights started flickering on, the sequestered families started waking to decide who would go steal a shower first.

Still we sat quiet, our forced conversation disappeared as we watched the faces that coule be ours depending on what happened face a new day.  The strained lines, the forced normality of conversation that had been ours.

The nurse arrived finally with a smile and told us we could go back and see her – that she was awake and feeling ornery.  We leapt to our feets and rushed back to the room, scooping our precious Angel into our arms and holding her close and tight. 

The pulmonologist had her wonderfully calm and warm smile, letting us hold her for a few minutes before she started in on the results.

She showed us images from the camera and told us that Angel’s Adenoids, Aretnoids and larynx were all swollen.  Then she showed us this barriers of mucous that lined the path to her lungs.  She said that this could mean many things, including Cystic Fibrosis – but she believed it was still the mucous of baby asthma.

She was so reassuring as she sent us off with a schedule card for blood tests and “just as a precaution” a sweat test. 

We went into the Outpatient Center of the hospital, and the blood work was horrifyingly terrible as it always is when they stick needles into a child’s arm…and then it was time for the sweat test.  The tech was so wonderfully sweet and kind as she cleaned our tiny babie’s arms and put on the medicine – followed closely by two electrodes.

We sat for five minutes as the machine buzzed, sending waves of electricity through Angel’s skinny little arm.  Then her arm was wrapped up in layers of wrap and we were sent out to play with a little timer set for 20 minutes.  By the time we got back to the room, our nerves were raw.  Even though we’d been assured that the pulm truly thought it was baby asthma…the thought of the words ‘Cystic Fibrosis’ sent chills down our spine.

The super-nice tech frowned as she peeled off the wrap to reveal the litmus paper on the arm, “Oh my, this little one doesn’t sweat much, does she?”  She pondered whether there would be enough sweat to even test…and carried it out of the room.  We sat silently, looking up in surprise when she returned and handed us a call-back card.  Apparently we had to wait until 4:30 for the results.

I headed home, and Archie to work.  Our nerves were wrapped up tight, but we suffered silently until 4:30.  I called the number with shaky hands, a deep seated dread filling me.  I punched all the numbers and the computers automated voice said the words I’d been longing to hear “Your test results are normal.” 

I heard nothing else, hanging up and calling Archie in relief.  We cheered our escape from such a fate, cried happy tears and went back to our days with lots of planned hugs for Angel.  We’d lived through our short journey to answers…our relief was immense….

And short lived.

An hour later Archie had come by and was playing with the kids – and the phone rang.  I was surprised to hear the pulmonologist on the other end of the line, “I know the automated system told you to call in, but I didn’t want you to have to wait.”

My heart stopped beating for a full ten seconds as I stammered, “Uh…no…the…the computer said the results were fine.”

“I’m so sorry, but it was wrong.  We’ll have to look into why it told you that.”

I met Archie’s eyes across the room, I knew tears were starting to fall, and that I wasn’t breathing.

“I wanted to call you personally because I didn’t want you to panic.”

Too late.

“But the test results showed elevated sweat chloride levels…”  The rest of her comments were a blur until she said, “Cystic Fibrosis.”

What had been a short journey into answers was about to get a lot longer – and impossibly more frightening.