Imagine ... (Prayer request)

Imagine your favorite food.  Can you see it?  Smell it?  Taste it?  ahhhh.

Now imagine never being able to eat it again.  In fact, you're never going to be able to eat ANYTHING ever again.  Nothing.  Nada.  Not even the stuff you kinda don't like.

You can't even have coffee.  Not even a taste.

You can smell it.  You want it.  But the people around you just will not let you have ANYTHING.

Well ... Welcome to Manny's world.

He was 8 months old, going along just fine drinking baby bottles.  Then one day, he was taken to the hospital and strangers decided he needed a tube in the tummy and to be "NPO" (nothing per oral).  Nothing.  Not one taste.

Since then, he has been NPO.

We have a friend whose daughter Abby is thrilled to be NPO.  For whatever reason, eating was traumatic for her.  It has taken a long adjustment to get the right tube and such but it seems Abby has never missed being able to eat by mouth and is now growing.

We have another friend whose daughter I'll call Edith.  Edith is having major aversions to food.  Her feeding team is working with her and the mother to try to help her swallow but for whatever reason, Edith only wants to swallow very bland, soft foods in small quantity (if anything) and her mother still is trying to feed her sausage and pork chops.  Poor baby!

Most kids who have problems with swallowing (dysphagia) actually have an aversion to food.

Not Manny!  He tries to lick or chomp anything that comes remotely near his mouth!  Doctor's stethoscope, gloves, Mama's arm ... anything.  We are careful about eating in front of him too as he looks so longingly at what we are doing.  His pitiful puppy eyes are staring at you.  (And we have NEVER been able to feed him.)  He used to cry when he would see another baby with a baby bottle.

So what's the prayer request?  Tomorrow, Monday at 10 am EST, Manny is having 2 tests.  First is a clinical feeding test.  The speech pathologist, trained in feeding issues, will give him a series of things to eat and drink.  She will continue as long as he can tolerate it.  She will stop if he has any issues.  We are praying he passes this with flying colors.  If he passes this part of the test, he will go to test 2.  This is where the same foods and drinks are laced with barium and he is put in an xray room where they do a video xray of him swallowing.  THIS is the key test.

Why are they doing this?  To see if he can truly swallow.  For a person with "normal" muscles, if something goes down the wrong way and into the lungs, it's called "aspirate" and this person will choke, cough, sputter, get tears in their eyes, etc.  (You know if you've ever had anything go down "the wrong way".)

But tests showed (before we got him) that Manny was doing "silent aspiration".  Which meant food/liquid was going down the wrong way and he was making no indication that it was.  This is a potentially life threatening condition.  They felt it a strong enough possibility that they ordered Nothing Per Oral.

So that brings us to my final concern.  Since he has had no practice in the past 4 months, his skills might be a bit "rusty".  Also, since he's never been given food, he doesn't know how to do that yet.  Swallowing food is a very different skill than swallowing liquid and most babies take a little practice before they master it.  (He's never been given that chance yet.)

Thanks for the prayers and I can't wait to show pictures of him wolfing down a baby bottle and a picture of him with strained peas all over his face.

Bad news and good news

Bad news:  He's got a cough.  Woke up Monday with one.  Went away.  Another on Tuesday.  Went away with treatments. Wednesday and that night.  Both went away with treatments ... but he coughed through the night off and on.  Today, he's still croupy even with extra treatments. 

As he lays here beside me tonight, he's sleeping but he's still coughing.  He can only have the albuterol/pulmicort every 4 hours and it's only been 2.  He's still saturating nicely and he's not struggling to breathe.  I have a stethoscope and to my untrained ears, it sounds like all the crud is high and nothing low in the lungs.  I'll get up through the night to give breathing treatments through the night every 4 hours. 

Hope and pray it clears.  If not, we'll be at the Doctor tomorrow to see what she hears.  And other than IV fluids, I have EVERYTHING at home that they use for him at the hospital.  (That was their plan.) 

And no, the irony is not lost on me that the cough assist might have arrived just in time to, uh... er ... "assist in his coughing".  (Update on that ... in talking with several moms with similar situations, many use it only when the child is sick, not a a preventative measure.  I think I can live with that.)

Good news:  He has yet another new skill.  You know the game "uh-oh"?  Where a typically 1 year old takes and object and throws it on the ground.  He says, "Uh-oh" and you pick it up.  Then he does it over and over and over?  Well ... he started that today!  Complete with the sound "Uh-oh" and the giggle at the end. 

Of course we have to modify it for him due to muscle weakness.  Usually it's a set of car keys or some other noisy object.  But for him, it was the corner of a piece of crinkly paper.  Nevertheless, he understood the game and played it for a long time with me.  Tooo fun!  

And as I recall, I've played this with many a 1 year old.  So he's right on track!

For the record, the pics are from his first month home.  Just wanted to show how much he's changed in 3 1/2 months!

Torture Machine

Torture Machine is the affectionate name I call the machine that arrived in my house today.  It's technical name is "Cough assist" and I have mentioned it before on previous blogs. 

Before I start my rant, let me say that I am very grateful to have been given this machine with no insurance headaches.  (It's a very expensive machine.)  And not everyone qualifies for it.  I saw a youtube video about it just the other day.  A 9 year old girl with SMA was talking about how she is still alive but many of her fellow SMA friends who didn't have the machine are now dead.  She thinks it's a life saver.  And perhaps some day we will feel the same about it. 

But for now ... my heart is in my throat. 

Today, I got a call from our Durable Medical Equiment company saying our cough assist had arrived.  I must have sounded like an idiot as I had no clue one had even been ordered for him.  And I'm sure I sounded a little less than grateful.  I should have been jumping up and down with, "Wow!  Yippee!!  We got one!!!"  But instead, I basically said, "Well, if the doctor ordered it, I guess he wants us to have one.  I had no clue it had even been ordered." 

For a refresher ... the idea of this machine is simple.  Pushes air in then pulls air out of the lungs.  The goal is to produce a cough.  This is especially helpful for kids with weakened muscles like Manny has.  (And in case I haven't been clear about this, he has been labeled as "Severely hypotonic" and several doctors have described him as the worst they have seen.) 

So this all sounds great.  Right?  What's the problem? 

4 things.  1)  When this machine is on Manny, he looks like he is going to die.  He has these eyes that beg me to save him.  (And that's when the Respiratory therapists were doing the treatment not me.)  He seriously looks like he is being tortured!

2)  *I* am the one who is supposed to be administering this torture from now on.  It's not for very long ... but several times a day. 

3)  Several of the respiratory therapists mentioned that they felt this machine was actually unsafe.  One went a far as to say she wished someone would push it down the stairs.   One actually "refused" to administer the treatment since he felt the doctor had ordered to high of a pressure setting.  (He said that the level was the same as those used by 120 pound teens and he is a 19 pound baby.) 

4) There's a question of effectiveness.   In ALL the times they did this treatment for him ... he NEVER ONCE produced even the smallest of coughs.  So is this torture worth it?

So as I sit here, just feet from this machine ... I'm really not sure what I'm going to do yet about it.  I'm not one to go against medical advice ... and especially with Manny since he is so complex.  But I also can't imagine myself turning on this machine, walking up to him and starting the torture ... I mean "treatment". 

I'll let you know what I decide.

A catch-up post

Not sure how it's been over a week since I last posted.  Can that be right?? 
So let's see how well I can summarize. 

The afternoon of the last post, the baby started this new thing.  He was doing the same choking like episodes but instead of falling asleep, he was screaming in pain!  I mean screaming.  It came in waves and then it would end.  Then again and again.  I noticed that the longer it had been since food, the less his pain.  So I started messing with his feed rate.  It got so bad that I basically stopped feeding him. 

I got advice from lots of docs and moms.  We decided on a time line of when to take him in (since it was the holiday weekend, ALL my usual docs were out!).  Then Saturday about 5 pm (26 hours later), he just stopped. 

Looking back, it might have just been a flu like thing but it's so hard to know since he can't tell us and he is very atypical in almost every area. 

July 4th - Church came over to our house for the "traditional" meal.  Nice to have a semi-normal time!

That week, I took the kids again to Busch Gardens.  This time we decided to stay until close - 9pm.  But then there was a bonus show.  So by the time we got home, it was almost 11pm!  My 6 babies were all very tired and slept very well. 

As you notice in the picture, he has discovered his toes!!  We have to prop his hiney up to get it there but he LOVES them!

Jacob turned 12 on July 7.  Can't believe how time flies until you look at a growing child.  Jacob is our oldest.  We got him and his biological sister when he was 19 months old.  They said he was "retarded" (their word, not mine) and possibly deaf.  Turned out, he just needed some love and attention!  I still remember the Helen Keller type moment with him where he realized I was trying to talk to him!!  I still wonder sometimes too what might have happened to him had we gotten him earlier in life. I know it's young, but so much damage had already been done.  He's high functioning autistic (some say Asperger's, depends on the neurologist) ... and I wonder. 

Anyway ... he's now 5'8", 135 pounds and wears a men's size 11 shoe!  On his birthday he decided he wanted to see if he could pick me up.  He could! 

Thursday was his 1 year old check up.  They're still not doing vaccines on him.  Feel the risk is too high (and this is a VERY pro vaccine doctor).  He was 19 pounds 2 ounces and was 28 inches long. 

Friday was a trip to the GI.  After much talk to him, I feel like Manny's issues are GI related since food often triggers them.  But he feels this is not the case.  He said there was this one thing we could do and we'd know right away.  The next time he's in an episode, I can immediately vent him.  (For those not familiar with gube, this is where I take a tube and connect it to his button.  I then take a syringe and pull out the gas/stomach contents.)  If it is an issue with his nissen fundoplication, this will immediately take away all his pain and he'll stop.  Good to know.  But he truly felt these were some type of abnormal seizures too.  (sigh)  And afterwards, it was "Cow Appreciation Day" at Chick-fil-a.  Basically if you dress up like a cow, you get a free combo. Can't pass that up as a family of 8!  Not many pics came out. 

Saturday was another HORRIBLE day with the baby.  We were out and he started on his episodes.  We've never been out before during this and whew!  Dan had Jacob and I had the other kids.  I texted him what was going on and where we were and they came and met us in the parking lot.  He just had episode after episode.  Thinking about what the GI said, I vented him.  And to my shock, not much gas came out, but almost ALL of his formula did!  The reason this is significant?  I put in about 6 ounces and pulled out 5 ounces...  over 2 hours after the feeding had stopped.  That should have been LONG GONE!  Ah-haaa!  I think we discovered the issue.  I've found gastroparesis might explain some of it.  Even the fact that sometimes this happens, sometimes it doesn't ... very unpredictable. 

Sunday - clean, decorate for the party, church, decorate the cake.  Jacob's party. 

Monday - Visit to WIC ... he's on ready to feed infant formula.  They give me enough to last about 15 days and the rest is up to me.  VERY expensive!  The GI wants him switched to Pediasure.  So I take in the form the GI signed with the amounts he wants.  Of course, they won't cover all of this either.  (But it's closer, thankfully.) 

Today - Busch Gardens with friends Bruce and Snooks.  It was great having them with us since there are rides the kids want to do but can't since I can't go with them (who would watch the baby).  So Snooks kindly watched him as we rode various rides.  Thanks guys!! You're awesome. 

OK ... so that's the catch-up post!  I'll try to do better with this blogging thing. 

Finally, I wanted to let you all know about the adoption fund ... the chip in (middle right column).  I know it only reflects $200 ... but several people have sent checks or given me cash.  The actual total is $580 towards the adoption fund.  Thanks!!

It's amazing what you can put up with

Ever heard of the proverbial frog in a pot? He'll stay in if you heat it up slowly. I find it truly amazing what we can put up with and what becomes the "New Normal". 

Some background:  For weeks now, Manny has started doing more and more of these "episodes".  Basically he looks like he's choking, can't catch his breath, his oxygen levels drop. This happens for give or take an hour and then he passes out.  THIS is what the docs all thought were seizures.  (Mostly because of the way he is after the episode.)

They are terrifying!  Your mind races with - could this be it?  We have no clue what to do during these episodes and no way to help him.  So we just sit there and pray for him.  I rub his face and hold his hand and tell him I love him as calmly as I can.

The on Friday ... it seemed like they changed.  For the next 26 hours, he would do these episodes, but instead of passing out, he SCREAMED!!  About every 15 minutes or so, he would be in such pain.  (He never cries, never screams!) 

I called and talked to tons of people about what to do, what this could be, how to help him.  But the truth is, there is nothing that can be done during the long holiday weekend. 

I noticed food triggered it ... even the smallest amount of anything would set him off. 

But then, all of the sudden ... it ended.  5pm Saturday it was all over.  We know tons of people were praying!  And God was listening. 

All over? 

No.  Now we're back to the original episodes.  And somehow ... they don't seem so terrifying any more comparitively. 

And that bugged me when I realized it just now.  This is the "new normal".

I'm a frog. 

Home again

Love, LOVE going home after a trip to the hospital.  I dress Manny out of his hospital gown and put him in regular clothes and he starts to look like a real baby.  Then I stick Manny in the stroller and he does this very happy face.  By the time I put him in the car seat, he does a little jig he's so happy to be going home.

This trip was a lot less hectic than usual for a lot of reasons.  We knew it was coming 24 hours ahead so I had time to pack, plan, get the kids squared away (versus the usual of "You're staying" and then the mad scramble).  It was also easier because he isn't truly "sick", it was for a test so they acted differently to us.  Finally (and this is huge), because he was on "Long Term Monitoring" ... they weren't allowed to disturb us in the night!   (Versus the usual endless trail of people every hour or so.)

I also felt more "relaxed" once he had his episode and I knew they were watching and recording.  That we were closer to answers.  And I just had to wait for the diagnosis.

At 8am, the Neurologist came in with the news, "No seizures".  I never thought it was, but many, many specialists had seen his episode and thought it was.  Why does it matter?  They're getting closer together, longer in duration and more intense.  AND, seizures are the "end stage" of Leukodystrophy.

11 times we rang the little bell that he was having an episode and none were seizures.  He also had none in the night.  Nothing!  None!  Nada!  This is very, very good news.

So what IS it?  We need to find out and soon as these episodes are bad enough to cut off his oxygen and he desaturates and he could die during one.  So we need to find out what is causing them.  GI?  Respiratory? Other?

As I drove home, I thought to call the Hospice staff.  The Hospice Doc saw him after the episode yesterday and the Hospice nurse saw him during one a few days ago.  Maybe we can figure out our next steps.  Which specialists to see next?  Tests?  So that will consume our next few weeks I'm guessing.

Later I'll have to blog about the people we ran into while at the hospital ... little "clues" of why we're on this journey.  But for now, Manny just woke up and I'd rather play with him.  Time well spent.

Back in the hospital

We're back in the hospital ... this time for tests. 

Lately, Manny has been having more and more of these "episodes".  They're coming closer together, lasting longer and are much harder on him/us. 

It's not quite retching, not quite choking.  One nurse called it "retracting".  And every medical professional who has seen it, calls it's a seizure.

Tuesday we had a 20 minute EEG and it seemed to be normal with no seizure activity ... which is GREAT news.  We then went to the Neurologist and he said that 2 genetic tests came back ... he doesn't have Canavan's or Alexander's.  But he doesn't have a clue what Manny DOES have then.  He is not acting the expected way.

For example, kids with advanced Leukodystrophy tend to have big heads ... Manny's is small.  They lose skills ... Manny is gaining them (like he says Mama, Daddy, Hi, Bye-Bye and he couldn't do that a month ago.)  By the time it's spread as far as Manny's supposedly has (per the MRI), they lose cognitive functions and the ability to interact with people ... Manny is extremely engaging still.

So we left the Neuro scratching his head.  (Usually a fingerprint that God is up to something in my experience.) 

But then Tuesday night he had a bad episode.  We were told to take him to the local ER and we did.  They saw it weren't able to EEG him quickly enough.  The ER doc called our Neuro and they agreed these must be seizures based on the way he behaves after.  So they set up the 24 hour video EEG. 

Wednesday, he had 2 episodes.  (They're so scary and you just sit there completely helpless.)  Kaley (age 10) says he looks like, "He is locked inside his own mind."  I think that's a pretty apt description of the situation. 

I sit in the hospital again and for the first time, I'm actually HOPING he has an episode.  Seems strange to wish for.  But if he's going to have them, we need to know what they are.  We need to know if there are possible treatment options. 

But you know how you go to the mechanic and your car stops making the noise?  I'm hoping that doesn't happen today, that it's not a complete waste of time.