Finally...

Finally, finally I might be able to see it.

It's been a crazy ride around here this past week (month?  year??).

I was realizing that it was five weeks ago on Friday that he went in for his ear surgery.  That went pretty well, aside from his idea that his ear cap was an optional accessory.  Whatever.  I kept him out of school the next week to recover and sent him back on Monday the 3rd.

The cute "little" tank is
actually the full-sized one
he usually uses.  Next to it is
the M tank.

Tuesday the 4th had us back at Primary's for a long 17 day stay. Then it was come home and work and fight to keep him at home.  He came home sick, no doubt about that.  When we came home, the hospital also arranged for us to have a few oxygen M-tanks brought in.  For comparison, the E tanks we usually use (and what you see older people pulling) hold about 625 liters of compressed oxygen.  The M tank is over 3400, so roughly five times as much oxygen.  That's also the size tank the ambulance carries, for whatever that's worth.

And we've used two in the past 5 days.  And we only use them when he's needing more than ten liters of oxygen per minute.  There's been some bagging, pushing 100% oxygen into him.  There's been a lot of vest CPT (chest physiotherapy), a lot of albuterol, a lot of prayers and pleading.  And not so much sleep.

Yesterday I have to admit, I was feeling defeated.  It felt like the movie Groundhog Day.  Each day was slightly different, but I wasn't finding the key to getting out.

But when I stopped and analyzed things, I realized it was getting better.  We hadn't bagged him for a few days.  He was spending less time on the oxygen tanks each day.  We started to space out his CPT and his albuterol.  When he first came home, it was every three hours for both, around the clock.  Thank heavens for night nurses.

Yesterday, he only got his vest three times, and when well, he gets it twice, so very close.  We only gave one extra dose of albuterol.  He still needed 8-10 liters most of the time, but wasn't on the tank at all.

And today?  Well, today he's below five liters of oxygen, and satting just fine, thank you very much.  He's still pretty tired, but you would be too if you thought daytime was for sleeping and nighttime for playing.  I think he might almost be better.  He might even go back to school soon.

Finally.

"And sure enough, even waiting will end ... if you can just wait long enough."

William Faulkner

Minion on the Move

We made it home!

This hospital stay really started to weigh on Aaron especially.  Each day he got less and less interested in interacting.  Fewer smiles, more tuned out.

But oh boy, put him in his bed in his house?  Wild man returned in force!  He couldn't stop smiling and talking and laughing, and setting off his alarms because he was laughing so hard.  (The ventilator thinks laughing is breathing, and it was too fast.)

He's still sick.  In fact, the snot factory is in full force.  And his oxygen needs have been all over the place, like as low as 1 liter (haven't seen that in forever) and as high as 12.  But we're handling things here at home.  Right this very minute, he's singing in there.  And his smiles make it all worthwhile.

"The best journey takes you home."

Author Unknown

The TALK

A touch of whimsy up here.  This giant pumpkin is sitting
outside the main entrance.  It is HUGE (that's a pallet underneath it)
 and just  look at all the detail in the carving. 

 We're still here, still hanging out in ol' room 3058, on the J pod, in the Children's Surgical Unit. And I think we're both getting a little tired of the scenery.

But once again, last night, he had a weird bump.  And given that his cold isn't supposed to peak until tonight, we decided to play it safe and stick around one more night.  Frankly, if he decides that he'd much rather have the PICU ventilator than his old boring one, I want to be a short elevator ride away from it, not a 45 minute ambulance trip.  By tomorrow morning, we should have a change of scenery.  It will either be home (yay!) or the PICU.  Honestly, my money's on home, but you're never quite sure with this kid.

So anyway about the TALK.  Anyone with a medically compromised kid knows about this one.  And well, if your child wasn't supposed to live at all, you know, "incompatible with life," you've probably had it more than once.

Dad and big brothers came for a visit on Monday.

I hate it.  Let's just get that out there.  I know it's necessary.  They wouldn't be doing their job if they didn't make sure we parents understand reality.  It is what it is, and there is no way around the ugliness.  So for those who might be wondering what all it entails in Aaron's case, I'll put it out there for you.  And then, until the time comes that I truly have to face it, I'm shoving it back in the deepest, darkest corner of my mind and nailing the door shut.

But here's what this visit has brought:

There are only a few different pulmonary hypertensive medications.  Oxygen is first line of defense, and he's been on that since birth.  Then there's sildenafil (liquid Viagra).  He started that at about four months old.  Three years ago they added a drug called Bosenten.  That's an interesting one.  There is no actual clinical data for pediatric patients, meaning no dosing recommendations whatsoever for people under the age of 12.  And it is known to sometimes cause liver issues, really bad ones. But when your option is the drug or death, well....  'nough said.  He takes it.

Biggest grins I've seen in a while.

This time we've started something called Ventavis (iloprost).  That was the subject of most of the recent posts, like this one, this one, and this one.  But here's the issue.  This is the last one.  There are no more.  We are out of options.  All the others are some sort of variation on these.

Pulmonary hypertension is considered progressive and non-reversable.  And he's already quite compromised because of his trach and ventilator.  This is the ugly truth.  What does it mean for Aaron?  I'm not sure exactly.  Each of his other meds have bought him about three years time.  There are studies which show really, really good results with the combination of bosenten and iloprost.  But of course, there are no studies, none at all, with pediatric patients, let alone peds with Trisomy 18.

And he has full Trisomy 18.  And he is trached and ventilator and oxygen dependent.  And he is a boy.  Yes, that does make a big difference.  The time is coming when we will have to make really hard decisions, decisions no parent should ever have to make.  But that time is not yet here.  And I refuse to face it until it is.

What can't  be measured is the songs he sings, the smiles and giggles, his mischievous nature when he teases us.  You can't size up the joy that he brings and the lessons he teaches us.  You can't calibrate how much love we've learned from him.

So we will bury him.  It will be awful, beyond description, more than anything we've had to bear.  But to avoid that pain, we would have missed out on all the beauty.  There's already enough anticipatory grief.  And maybe not enough anticipatory hope. Today, I chose hope.

"God puts rainbows in the clouds so that each of us — in the dreariest and most dreaded moments — can see a possibility of hope." 

~Maya Angelou

Practicing Patience

After the "excitement" on Friday, Aaron managed to stay on the floor.  This was almost entirely due to everyone involved knowing him well, and knowing that we had the ability to bag him easily if problems developed.  Had anyone involved, from the doctor, RT and nurse on the floor to the PICU staff not been very familiar with him, we would have taken a field trip downstairs to the PICU.

We thought (and hoped!) that it would be a one-time thing, and the next several hours seemed to suggest it might be, with the exception that he was on higher oxygen needs again.  Not crazy high, but higher.  Then his 8 a.m. treatments came on Saturday.  Same thing, although not quite as dramatic.

With that, I requested a second viral panel.  That's the test to see if he's got nasties brewing, and since his panel when he was admitted was negative for everything, it had the potential of being very useful.

Remember how I wanted to get out of here before he could catch anything?  Remember how much "fun" we have with rhinovirus?  Guess whaaaaat....

Yep.  He's got it.

So we won't be going home for a few more days.  Right now, his symptoms are mostly increased oxygen needs, but not to the point where we need to be in the PICU.  In fact, where he currently is, I could support at home.  However, it hasn't hit its peak yet.  And this is the single biggest reason that we know our local paramedics so well.

Often when he's struggling, like he was when we came up, I can hyper-oxgenate him by bagging and giving him 100% oxygen and that will buy me some time, usually an hour or more.  But with rhino, once I put him back on the ventilator after bagging, he almost always drops again within a few minutes.

So we're hanging out, making sure it doesn't get worse.  We might make it those ten-plus days after all...

“Patience is the ability to idle your motor when you feel like stripping your gears.”

Barbara Johnson

Sometimes It's Really Hard

When you've worked all week, making dozens of calls a day all over the state and country, trying to get a medication your child needs authorized and delivered...

When you sleep(?) night after night on a hospital couch/bed...

When you finally get that medication, and it feels like forever, even though the nurse manager says you've moved mountains and gotten things done in less than half the time it usually takes...

When you plead with your child to just hold on, keep breathing...

When your planned day to go home comes, and goes, and you're still in the hospital because now he's not processing his oxygen well enough, but almost...

Sometimes you take a short break, leave him with people who know him well, and step aside to grab a bite to eat.

And the phone rings.

It's the nurse, he's on 15 liters (you left him on 6, or 8, you can't quite remember).  The respiratory therapist is with him.  You race out, rushing back, wishing you'd thought to tell the nurse to start bagging, but also knowing that the RT knows him well and really doesn't need to be told.

The small room is full:  doctor, nurse, respiratory therapist, IV team, x-ray waiting right behind them, PICU fellow and charge nurse, too.

The bed is pushed catty corner, to allow more people and equipment in.  You squeeze in, hold his shaking little body as the team works quickly, trying to find a vein.

He's not being bagged, but you don't like the way he looks, the way he sounds, and when the RT calls for a therapy bag to be brought in because it's more sensitive than the typical bag, you reach around and grab yours to hand to her.

All the time, you're silently pleading, "keep breathing, breathe deep, Father in Heaven, help my son, my child, my baby."

And his lungs begin to respond, the IV lasix starts working quickly, the x-ray isn't great, but isn't any different than the one the night before.

And he smiles, and starts batting at toys, and everyone steps back and watches for a few minutes.

The PICU doc makes sure that transfer paperwork is filled out as much as possible in case he turns south again.

He starts sucking on his tongue, a sure sign that he's getting ready to sleep.

And while you're relieved, so relieved, a small part of your heart breaks just a little more as you realize you have to tell the family at home that once again, you're not coming home tonight, and you don't know exactly when you will.

But he's breathing, he's comfortable, and the couch/bed, extended stay, missing family is worth it.  Because he's still here.

But sometimes, sometimes it's hard, really hard.

“The bravest sight in the world is to see a great man [or child] struggling against adversity.”

– Seneca

The Saga Continues...

Well, we finally have medication coming.  It should land on the doorstep tomorrow before 10:30, for reals this time.  (Thought it was coming a couple days ago, but that turned out not to be the case.)

The nurse came and trained me this morning on how to break open the medicine (it comes in little glass vials) and how to draw it up, give it, and hopefully not stab myself in the process.

Now we're waiting on the nebulizer 'cause just any ol' nebulizer won't work.  I've got two at home already, and neither will do the job. But reimbursement being what it is, the DME doesn't want to just order it in without making sure they can get as much back as possible.  I get that, really.  Except that, every day we sit in the hospital waiting is costing them more than it would be to just buy it outright and not get anything back.  And since the insurance is the other side of the same company that runs the hospital and DME, you'd think they could move sooner rather than later.  Instead we're saving pennies and burning dollars.

We don't even have to have the machine in our possession.  As long as it's ordered, we're fairly confident that the hospital will loan us one until it comes in.  They just need to know I'm not going to disappear into the night and never show up again.  Like that could ever happen anyway. I mean, this kid doesn't exactly fly under the radar.

Anyway, even though it's a drug that's often given in the hospital, (like really often) it's fairly rare for a kiddo to go home on it.  And even more rare for that kid to have a trach and go home on it.  As in, they tell me we're the first.  So what was challenging and time consuming initially has become even more difficult.

So we sit and we wait.  I do take some comfort in knowing that when the next kid comes along (and they will) it will be a much easier process.  Sometimes being a pioneer isn't exciting.  Sometimes it's tedious and downright boring. I wonder if my ancestors sometimes felt the same way.

"In a word I was a pioneer, and therefore had to blaze my own trail." 

Major Taylor

Waiting, Waiting, Waiting...

Well, he looks great, and we're still here.

See, apparently this kid does nothing that's simple.  But I guess we already knew that.

Before we can go home, we need his new medication.  Except you can't just go down to the corner drugstore and pick it up.  Nope, it has to be ordered from a specialty pharmacy.  One headquartered in Tennessee.  And before that happens, it has to have a prior authorization from his insurance company.

If you've been following him for a while, you might remember this post from about three years ago.  Yep, same situation, except this time it's not just a pill, and I actually have to have equipment and training.  

Yesterday his doc came in and said, "it's a good thing you like us, 'cause this is going to take a least ten days."

What??  Oh, no, it's not.  (At least I hope not.)  I started working the phones and by last night, we had insurance approval.  Less than a day, not too shabby.

Now we're trying to get the medication and the training.  I'm really, really hoping to be home tomorrow.  That is, if all the little ducks line up in a row.  So far, they seem to be more interested in waddling off.

Every day we sit here increases the chances of him picking up a bug, which means a week in the PICU to the tune of about $10,000 a day.  Yeah, that's four zeros per day.  Plus further recovery time on the floor.

And besides, I really miss my own bed.

"You can't stay in your corner of the forest waiting for others to come to you. You have to go to them sometimes." 

A. A. Milne

A New Weapon for our Arsenal

We think we've found the culprit.  And it's what I figured but didn't want to admit. It's his pulmonary hypertension.

Yeah, that beast. The one that is progressive and has no cure.  That (so far) is not reversible.  And it's continuing to get worse. Can you see why I didn't want that to be the answer?

Anyway, over the past few years, we've visited the idea of iloprost, an inhaled medication that will help relax the blood vessels in his lungs.  For various reasons, we've discarded it each time.  But now, we're putting it on board.  We're starting with (only) four times a day.  It can be increased up to 10-12 times a day.  Yeah, every two hours, 'round the clock.   Even with all his other meds that we do, this one is sounding kinda daunting to me.

But then I have to remember, when we first started this journey, his feeding tube scared me speechless, which is a hard thing to do.  Now?  If I needed to, I could instruct someone on how to place one while talking on the phone driving down the freeway, no problem.

So anyway, I'm told that it will take a little time (don't know how long "a little time" is) for it to take a full effect.  BUT after one dose at 2:00 this morning, we started seeing changes, big changes.  Like, I got after the nurses because he was satting 99% and they were just letting him sit there. They didn't know he wasn't supposed to be that high and he was only on 3.5 liters.  It dawned on me that this has never been a problem in the hospital before.  As soon as he's maintaining low to mid-90s, we bust out of here.

And he played and played and played all night long.  He's snoring now, but you would be, too, if you went to bed about 4 a.m.

So now we watch him through the weekend and make sure things go well, make sure he tolerates the medication okay, make sure his blood pressures don't end up dropping.  Then on Monday they'll train me on administering it, and hopefully, we'll be good to go.

Gotta get our supplies in order and all, but the end of the tunnel is in sight, and I don't think it's an oncoming train.

"Carve a tunnel of HOPE through the dark mountain of disappointment." 

 Martin Luther King, Jr.

Friday in the Big House

There's still not a lot to tell.  I've put off posting updates, because I just don't know what to say.  

Nothing's growing out from the sample from his lungs.  He still has no fevers and his lungs sound pretty good.  Albuterol doesn't seem to be doing a lot for his oxygen needs, but it's also not raising his heart rate, so it's probably needed.

We pulled a BNP yesterday.  It's a blood test which measures how much stress the heart is experiencing.  Anything under 100 is normal.  Normal for everyone, not just Aaron.  His was 34.  34!  He's never been that low before, and no, there isn't a "too low" number for that one.  So while that doesn't give us a new direction, at least it's comforting. 

But he's tired, really tired.  When he's awake, he's happy.  But he's sleeping a lot.  And still needing 5-10 liters of oxygen, depending on whether he's awake or asleep.  

Watching "Inside Out"

I'm really hoping to see cardiology today.  I don't know that they've got anything to offer, but because of the holes in his heart, his pulmonary hypertension could still be causing issues.  So we'll see what they have to say.  We've also given him some steroids, in case we're missing inflammation in his lungs.  

Because we don't have any idea when he'll get sprung, tomorrow afternoon I have to go home and make more food for him.  Thank goodness for good friends who are willing to come in while I'm gone.

Basically, it kinda feels like we're shooting in the dark. 

Oh, and don't mind the lovely bandage on his nose.  He's got a small scab that he keeps scratching off.  And in spite of his platelets finally being up at least close to normal, when he bleeds, he likes to bleed.  So this is our latest effort to keep his bed from looking like a massacre site.

“Only in the darkness can you see the stars.” 

― Martin Luther King Jr.

No Answers

Not sure where to begin with this one.

Monday, Aaron went back to school.  He was doing well, and seemed excited to get out of the house.  He did seem to be experiencing a little discomfort, but I wasn't sure. He did well at school and then went to cub scouts with Michael and I, where he saw some of his friends, the Lone Peak paramedics.

Monday night he struggled a little more.  More oxygen, less smiles.  But nothing else.  I considered keeping him home.  Through the night, he still was restless so I sent out texts to his nurse and bus driver that I was just going to watch him.  I guess he thought he needed more attention than that.

Watching TV upside down in his bed. Silly boy.

I ended up bagging him five or six times over the course of the morning, and about 10 o'clock, loaded him up for a trip to Primary's.

What's going on?  We just don't know.  We really don't.  He hasn't acted like he was in pain since sometime early Tuesday morning.  He has no secretions, none.  He has no fevers.  His x-ray looks really good (okay, good for him).  His labs are just about perfect.  Heart rate, breathing rate, totally normal.  Nothing.

Nothing except he's requiring a minimum of eight liters of oxygen while asleep and ten when awake.  But I guess we'll be grateful for the ten.  Above that, and we transfer to the PICU.  Great people down there, love having lunch with them, don't really want to need them.

So we wait and we watch, we pray and we hope.

Outside my window has been a bit dreary today.  It's been cloudy and rainy (and even a little snow!).  There's a covered bridge to the next building, and beyond that, downtown Salt Lake.  Just over the top of the bridge, I can see the spires of the Salt Lake Temple.  What comfort that brings to me.  A reminder of the temporary nature of this life, but of much better things to come.  When the dark comes, the lights shine bright.  I wish my camera could really capture it.  I'll have to take a picture with my heart.

"All the darkness you may be experiencing cannot dim the light of a single candle. Have hope."

Author unknown

Refocusing on Joy

Well, we're a week post-op, and he's being a kid.  He finally got rid of his ear cup, and while the site continues to ooze a little, he (mostly) leaves it alone.

But it's been a long week.  Aaron being home from school to recover meant that he went everywhere with me, including grocery shopping.  Let me tell you, that was a sight to behold I'm sure.  Pushing him in his wheelchair and pulling a cart.  We were not inconspicuous.  I also took work off so I could be by him and keep his sites clean, and while it was nice to have some time off, it also allowed for a lot of thinking time.  Not always a good thing.

See, when the anesthesiologist takes extra time to make sure you understand the risks of your son's surgery, when he tells you that his odds of dying in the OR are exponentially higher than a typical kid's, when you have just the day before attended another little one's funeral, well, you can see where things are going.

Another mom put all this into words for me last week when she wrote about her child.  To paraphrase, the hard part of having a medically challenged child is not your day-to-day.  It's not the breathing treatments, the equipment, the extra people around.  It's not the tube feeds, diapers or time it takes to move around.  It's not the crazy learning curve where you feel like your brain has been blasted into outer space.  It's not even the lack of sleep.

The hard part of having a medically fragile child is knowing that some day, and you don't know when that day will come,  you will have to hand him back to God, and stay behind.  The hard part is the work that has to go on between your own ears, in your own head, as you try to wrap your mind around what will happen, but still live in the moment and love the child you have. It doesn't matter if it's this week, or ten years or more down the road, having to let go will happen, and it will be excruciating.

Add all this together, plus exhaustion and a million things you "have to do" ('cause somehow the world will fall apart if you don't do everything, right?), and yep, I was struggling.

But every six months, our church has a worldwide conference.  We gather in meeting houses and homes to listen to counsel from the Prophet and other General Authorities.  The promise is that if we will prepare for Conference, there will be personal guidance, directly for us as individuals, in our own lives.

I'll be honest, on Saturday I didn't get much.  Saturday morning I missed because of a soccer game.  And Saturday afternoon, well, I was grumpy.  In the words of one of my favorite authors, I was having a Jonah Day.  Everything was conspiring against me, too much to do, no desire to do it, and so forth.  And frankly, I didn't want to be inspired, not yet.  I just wanted someone to put their arms around me and say, "it's okay, you're okay."

But Sunday, oh, Sunday.  It was everything I could have asked for and more.  It was as if the speakers had sat down and said, "Okay, here's Rebekah.  What is it she needs most? How can we get through to her?"  Never mind the millions of others who were watching as well.  I was taking notes, but couldn't even come close to writing fast enough.  The first one that really hit was one on Joy.  On having joy regardless of our circumstances. Elder Nelson related the story of Eliza R. Snow and some 80-plus women and children crowded into a small cabin in the winter after being driven from their homes.  Physically, it must have been awful.  But she described it as a "very merry night."  I immediately felt chagrin for my own attitude.  Plenty to eat, warm comfy house, no danger, yeah, I was in much better circumstances, and not nearly as grateful.

And that opened the door of my heart for the further messages. Messages about love, forgiveness, repentance, trials building character so we can reach our divine potential.  Faith, parenting and teaching, counting blessings, and so on.  Like I said, as if they knew exactly what it was I needed to hear.  And while I know the men and women speaking didn't know me, I also knew that my Father in Heaven does know me, and He loves me and knew I needed to hear what was said.

So now, I need to repent of my attitude, cultivate a lot more joy and gratitude, and watch Saturday's sessions again, this time in with a proper frame of mind, a willingness to be taught, a desire to learn.  I'm so grateful for today's technology that makes this possible.  My focus is being refocused.

The joy we feel has nothing to do with the circumstances of our lives, and everything to do with the focus of our lives.

Elder Russell M. Nelson