Wednesday, April 19, 2017

A Rant, An Update, and that time I almost lost my mind

I planned on writing an update the night after we got back from the appointment with Tessie's pulmonologist, but I couldn't.  I was too angry (not at her doctor).  It would have been a rant and besides, I didn't have anything to tell you.

So I held off, hoping that the upset would ebb.  

And it did until I called the office again today and got more news, or not-news.

But now I'm getting ahead of myself.


A week and a half ago I picked up Sadie from school early, along with Patch who was finishing up his day of preschool, and loaded them in the car alongside James and Tessie.  Maggie's BCBA had managed to get staff to watch her until 5 that night, but with Paul at his new job I was bringing the other kids with me, because I knew we couldn't be sure we'd be back in time to pick up the other two, even if they stayed in their school's after care program.  

Just before I got out of the car to pick the kids up, my phone rang.  It was Tessie's respiratory therapist asking if he could stop by the house to drop of the supplies I'd asked him for a week and a half earlier.  I suppressed a wave of annoyance.  No, I was on the way to see her pulmonologist at the children's hospital, I explained.  Would our usual Thursday time work?


It might.  But he had a question.  Had I let the monitor go dead?  Once? I said.  I thought it had gone dead once, when we had been driving a lot on a particular day the week before.  The alarm, for the battery, had sounded so I'd turned it off.  It was hard to remember in the rush of going inside and getting the kids out of school and signing Sadie out.

Well, he said.  When I'd taken the machine in (and picked up the supplies I needed, something that was obviously frowned upon) the memory had been blank.  Because, he explained, if it went dead it would erase all the memory.  

That would have been nice to know before now, I thought, with slightly more colorful language flashing through my mind.  I hung up the phone and took a deep breath.

It didn't make sense, I realized once I was off the phone.  I turned it off the moment it alerted me that it was getting low.  It wasn't actually dead.  The low battery alarm had sounded, but I always immediately plugged it in.  And when I took it in the tech told me that it was 60% full, not blank.  


But what could I do?

We drove an hour and a half to the children's hospital, stopping to eat lunch in the car along the way.  Then I unloaded the double stroller, loaded James and Tessie into it, and navigated our way through the garage and up into the hospital.  

I had my list of questions and things I needed to tell the doctor and after the nurse weighed and measured Tessie she asked if it was okay if she brought the kids three IPads loaded with games to play on and I nearly hugged her as I rushed to say yes.  

The doctor came in and asked me if things were going well.  I was momentarily confused.  Not really?  Honestly I wasn't sure.  I explained the problem.  Sometimes I feel like things are going very well.  We'll go a week with no alarms.  And then the next week we'll have two nights where Tessie's machine says she has low oxygen or is having apneas, every five minutes, for hours.  

But of course I wanted to know what the machine said.  We'd made the appointment for two months in the future so that the doctor would have the data she needed to make recommendations.


That's why we were there.  

That's the thing, Tessie's doctor explained.  They only had two weeks of data.  And that looked pretty good.  She only had three instances of low oxygen in those two weeks.  Admittedly three instances is better than 130 in one night, but also still makes me feel uneasy.

Any instances make me uneasy.

But we didn't have anything else to go on.

The other month and a half of data had not been transmitted.  

That doesn't make sense,  I said.  The respiratory therapist has come out to our house two or three times and I even took the machine in once when it said it was getting full.  I told her that and related what the therapist had said about the last week being erased.

The doctor disappeared and came back after talking to one of the clinic's respiratory therapists.  She said that their therapist said that the machines didn't work that way.  And that they'd put in an order (again) to get the information sent.  

She promised to call me personally when she got it, and we made the hour and a half drive home to pick up Maggie, before rushing to Sadie's ballet class.


On the way I called Paul and asked him to call me when he had a break.  He did and then he called Care Linc, the medical equipment company that was supposed to send in the information.  

First they told him that the information had been sent.  He asked for dates.  

Well, today was one date. 
Not today. Before.  He explained what the problem was.  
There were other dates, they said.  
Could he have them?
Well they didn't really have them.  Someone else did.  Someone who was there.  Oh look!  She left!  Someone was going to send the information last week.  But they were out of the office.  And the week before they almost sent it. But something happened.
Why yes, the person who had left would call him back as soon as possible.

Except she didn't.  The respiratory therapist did.  He called my husband to explain how it was all my fault.  There was no data.  Because I'd let the machine go dead.  It was all erased.  

Bull.  

Paul texted me the next morning and asked me to send him the make and model of the monitor.  I texted him a picture.  A while later he called.  He'd just gotten off the phone with the manufacturer and they said it absolutely would not lose any data because the battery had gone dead.  That was a blatant lie.


So I tracked down another medical supply company that comes out to the small town we live in.  And Paul called the insurance and explained the situation and they said that they absolutely supported switching immediately and not to turn in the old monitor until we had the new one.

And then I called the pulmonologist's office and asked for a new prescription for a new monitor.  And while I was on the phone with their respiratory therapist, and while she had Tessie's chart pulled up, I asked if she could tell me how the information they'd received looked.  I explained that Dr. H had said she would call me, but that I knew she was really busy and that these things took time.

There was a pause.  The files weren't there.  They hadn't been sent.  Oh, actually there was one.  12 days.  Of the month and a half they had sent 12 days.

Of me putting the monitor on and off five times a day for car rides and naps, and Tessie's poor little foot getting raw from the tape and bleeding, they'd managed to only successfully transmit about 30% of the data.

Which is when this post turned back into a rant and my week and a half of progress, cooling off, was completely undone.

I just can't even go on.

Deep breaths.



In other news, Tessie saw her neurologist today.  He's very pleased with her progress in her gross motor skills.  He was happy with the MRI results.  In another six months he wants to order a second MRI, just to make sure that the bleeding in her brain hasn't grown.  He doesn't think it will, but he wants to be sure.

And because we've noticed that Tessie tends to get clumsy when she's awake before she has apneas he gave me a lab slip to take her in and have a certain test done looking for metabolic problems (again) the next time she's very clumsy.  It will hopefully eliminate something called "intermittent maple syrup urine disease" which google tells me twenty people in the world have.

I feel like our odds are good.

We're still waiting on the insurance approval for the scary test though (that 1000 people in the world have... still fairly good odds).  Maybe it's a good thing that my frustration with the whole monitor information has kept me distracted from dwelling on that overly much.

Here's hoping the next company can get the data and actually transmit it to the doctor without all of this extra drama.

Because I'm sure I can find the energy to keep fighting all these extra, useless battles, but I'd rather not have to.  

Friday, April 7, 2017

Super Tessie Update

I realized at around 3:30 this afternoon that it was unlikely that we were going to hear from the neurology office today and that we were heading into the weekend without any answers.

And so, sounding truly pathetic, I called and left a message for her pediatrician on the nurses' line, asking them to take a look at the recent MRI results and to please call me back so that they could hopefully tell me that what I was looking at wasn't as terrible as it seemed.

The nurse, who was actually the first person to suggest apnea, three months before she was diagnosed, and a full month before anyone else even considered it, called back immediately and said she could definitely see why I was nervous and that she'd make sure that either she or the doctor called back before the end of the day.

She called back a couple of hours later with great news.



She said that the doctor said that it's a historic bleed.  At some point in the past, deep in her brain, Tessie had a bleed, but they don't believe that it's clinically significant at this point.

So it looks like we haven't uncovered the reason for her central apneas, but it also wasn't the horrible thing it sounded like it was when I read it myself.

And that is your Friday night update!

Thank you for all your prayers!  Hopefully we'll hear that the genetics tests has been approved and we'll be able to answer that question once and for all soon.

Thursday, April 6, 2017

While We Wait

Looking back to that phone call two weeks ago, when the results of Tessie's MRI came back, I realize that the person who called selected their words very carefully.

She said "I was calling to let you know that your daughter doesn't have the brain malformation we were looking for.  She doesn't have the Chiari malformation."

"That's great!" I said, barely feeling relieved as I began to worry about the next bath of tests.

I quickly announced on Facebook that the MRI results were good.



With perfect hindsight, I realize that that wasn't what the caller from the pulmonology clinic had said though.  She never said that the MRI was good.  She said that they didn't find the malformation they were looking for.

Yesterday a thick packet of paper arrived from the geneticist's office.  This is something new that's begun to arrive from our doctor's offices lately since they've switched to a new office operating system.

Most of the packets arrive with a list of immunizations and a few sentences about what the appointment was about.

Well baby check.
Developmental delay.
Central apnea.

stand one on top of the other in a neat line down the page.



This envelope bulged with the weight of the paper it held and when I ripped it open I found pages covered with a tiny font with some of the words in bold, and others in ordinary type.

I made dinner and paused for a moment in the kitchen, the papers catching my eye.  Paul had called at lunch time and announced that he had gotten a call for a job interview that was going to take place in at a restaurant at dinner time, so I knew that he wouldn't be home.  I'd already thumbed through the papers once, but this time I leaned against the counter and read it line by line.

I almost didn't pick it up a second time because I thought that I already knew what it said.  It had information from my conversation with her geneticist in the PICU and then later about her appointment.  When I was nearly done reading through the notes a section in bold caught my eye:

Brain 3/20/17: GRE hypointensity associated with glomus of the right lateral ventricular choroid plexus is probably due to previous choroid plexus hemorrhage.  Other calcifying or hemorrhagic lesions the choroid plexus are less likely.  

I tried not to freak out.  I failed.  I managed not to call Paul and ruin his interview.



I served dinner and got the kids into their pajamas and got them to bed and congratulated Paul when he called and announced that he had got the job and would be starting first thing tomorrow (today now).  And I googled "choroid plexus hemorrhage" and "GRE hypointensity" and then "what causes a choroid plexus hemorrhage?"

And then I watched Tessie sleep and wondered if she had had a stroke before she was born.

By the time Paul got home I had already reminded myself at least a dozen times of how well she's doing now.  She's pulling herself up to stand.  She's saying "dada" and "mama."  Earlier this week after I posted about how she won't eat or put anything in her mouth, she grabbed a piece of pizza and tried to devour it.

But I also remember the feeling in the hospital when I was nursing her, or watching her lay silently in her bassinet, and I felt like something was just off.  She isn't a normal baby, I kept thinking and then I asked myself what I really knew about "normal babies" anyways.


She was just so quiet and "good," always sleeping and never fussing or crying.

This morning I left a message on her neurologist's nurses line, saying that I didn't think they'd probably seen the MRI results from the other hospital yet (it always seems like it takes a while before they get the results) but that I'd seen them and I had a question.  Then I read the sentence that I'd read a few dozen times since I first spotted it and said that I was hoping they could call me and tell me that it wasn't as terrifying as it sounded on the report.

I'm still waiting for a call back.  I'm guessing her doctor wasn't in today and I'm praying that he's in tomorrow and that they call me back before the weekend.

And that they tell me that it sounds way scarier than it actually is.



In the meantime I'll keep looking at her, because, I mean, look at her.


She couldn't be any more perfect and lively and lovely.

As always, we appreciate your prayers and thank you for bearing with me while I write out all the thoughts bouncing around inside my head, trying to barrel their way out.

Monday, April 3, 2017

The Tessie Update: A Jumble of Thoughts, Relief, and Worries

I'm not sure why I've been struggling to sit down and post lately.  Things have been busy, as they tend to be around here, but I have had time.

It's just that every time I sit down to write the Tessie post that I've been meaning to update all of the feelings and nervousness that I have about whatever it is that's going on with her get too big and I stop.  And I've composed a dozen other posts in my head that I haven't written down here, because I needed to post this update first.


So here goes.

Let's start with the obvious.  Tessie is growing. She's happy.  And if we take the whole breathing thing out of the equation she's healthy.

Gloriously healthy:


I can't even remember what I've already written on this so bear with me if I repeat myself.

So far we now know for certain that Tessie has central apnea.  We know that it's on the more severe side of things.  But while that's scary, it's the least scary part of all of this.

Let me go back.

Tessie's doctors have explained that the causes of central apnea are generally a heart problem, a brain malformation, mitochondrial disease, prematurity, or a certain gene called PHOX2B that with a certain mutation leads to something called Congenital Central Hypoventilation Syndrome (CCHS).

In the hospital we eliminated the possibility of a heart problem.  She has a little hole in her heart, patent foramen ovale, but apparently 25% of the population has it and it's not a big deal and if it weren't for the apnea we likely never would have known about it.


Next was the MRI that I mentioned on the Facebook page in a prayer request.  It was nerve wracking to have her going in for sedation, because in the PICU they had said that they don't like to send babies with central apnea for MRIs because they're likely to just stop breathing and not start again when they're sedated.

Unfortunately the MRI that was taken of Tessie when she was sleeping didn't clearly show her brainstem, so it needed to be redone.

We drove to a children's hospital an hour and a half away, arriving at 6 am with an angry, hungry, fasting Tessie.

I told Tessie's nurse that I was nervous because of what they'd said at the other hospital and her response reassured me.  She said: "They were absolutely right not to do the MRI there since she has central apnea.  Because that means that now she's here.  We are the number one sedation center in the country.  We sedate six thousand children a year."

She went on to explain all the different things that they do in the sedation center, and that after I said goodbye to Tessie they would give her a gas that would put her to sleep and then she would be intubated.

They weren't going to allow for the possibility of her having an episode in the MRI machine, so she was put on a ventilator that breathed for her while she was sedated.


The MRI was done, and she woke up voraciously hungry and immediately was able to nurse, which was a very good thing.

We headed home and received the results only a few days later.

The MRI was normal.

I will admit to somewhat mixed feelings about this.

I wanted to MRI to be normal.  But I will say the MRI coming back as normal increased my anxiety exponentially.  Because if we go back to the causes of central apnea that leaves three.  Prematurity, mitochondrial disease, and the PHOX2B gene.

We were extremely confident of Tessie's due date, it was confirmed to the day multiple times by early ultrasounds (the first was at five weeks) because it was a high risk pregnancy, and when she was born at 39 and a half weeks she was 8 lbs 12 ounces.  In other words, she was not premature by any stretch of the imagination.


That leaves the PHOX2B gene and mitochondrial disease as possible common (or should I say uncommon) causes.

Tessie's geneticist said that based on the second round of test results (because the first were slightly abnormal) that she does not believe Tessie has mitochondrial disease.

Which is why my feelings upon getting the MRI results were mixed.  Honestly a mild chiari malformation sounded not all the bad compared with what I'd read about the syndrome associated with PHOX2B gene.

I was incredibly grateful the results didn't show something horrible.  And still terrified about the possibilities that were left.

So the relief that followed the positive results was immediately replaced with anxiety about Tessie's upcoming appointment with her geneticist.


The thing I had been clinging to, through all the other appointments, was how far Tessie has come developmentally since December.  Her geneticist, however, after evaluating her, had some concerns.

She still has some social delays.  She still has fine motor delays.  And she has a huge oral aversion and will not put anything, other than her thumb and fingers, into her mouth.  She has entirely refused every type of nutrient that doesn't come from nursing (which we're working on with two therapists).

Normally I'd be all "we do baby led weaning" and let her do her own thing but with the delays she's already had we can't really just wait around to see if it will all be okay.

Developmental delays, along with central apnea, mean that CCHS is still on the table.

I asked her how likely it was at this point (because she was going to say "oh not likely at all," right?), and she replied that because of the developmental delays and central apnea we really needed to order the test.

And so we wait for the insurance to approve her request (because it's an extremely rare condition and an uncommon test), and then we wait for the test packet to be sent to us, which we then take to a lab, and then send via FedEx to the lab that processes these particular types of tests.


I've been praying constantly that the tests show that the gene is normal.  I've also been learning as much as I can and trying to mentally prepare for the possibility that it might not be.

I'm trying to have hope and balance that hope with the desire not to be blindsided if the results are bad.

It's been explained to me by her doctors that the treatment for a positive result on this test would be a tracheostomy and ventilation, at least at night.  CCHS is considered a fatal disease, although when managed with extreme care people survive with it.  The biggest concern is that someone with it can never, ever fall asleep off of their ventilator because they might not wake up.

This video basically sums up what we'll be facing if the test comes back indicating this gene mutation:


A Week at a Special CAMP from Aaron Cahan on Vimeo.

Right now Tessie's monitor tells us when she isn't breathing, or when her oxygen levels are too low, and an alarm goes off until either she starts breathing again on her own or until I rub her back hard to make her start breathing again.  It's nerve wracking.

But I also know that 1 in 7.5 million have this mutation.  At least that's what I keep telling myself.  I imagine the sleep study results somewhat skew those percentages but still.  Only a thousand people on earth have been diagnosed with this syndrome.

What are the odds?

They must still be heavily in our favor, right?


I've asked Paul this question so many times, looking for reassurance, that I've just about driven him to distraction.

So now I guess I finally understand some of what I didn't understand during our first meeting with her pulmonologist.  You see, during those first few couple of months after the sleep study I was focused on the central apneas.

"She stopped breathing forty five times!" I said at our first appointment at the sleep center.

"That's not the bad part."  Her doctor replied.  "I mean it is bad.  But what I'm concerned with is this number here.  The 130 hypopneas."

At the time it seemed so odd.  Shallow breathing is still breathing.  Why would you worry about that when she stopped breathing altogether forty five times.

There were so many other questions that I asked that I didn't ask that one.

As I've been reading though it came back to me and I finally understood.  Because the hypopneas were when her oxygen went down to 79%.  Because the hypopneas point to CCHS.

Which is still what's happening now.


Tessie's alarm goes off for one of two reasons.  It will sound when the little sensor on her chest says that she hasn't taken a breath for twenty seconds.  Or it will go off if her oxygen levels fall below 89%.  It frequently sounds for either reason.

But never at the same time.  Either she is having an apnea and her oxygen levels are still high or she's breathing too shallowly and her oxygen levels are abysmal.

Now we wait.  And pray that this time it doesn't take the insurance two months to approve the test and that they approve it the first go around (it took two months to approve Maggie's genetic testing).

Prayers that she doesn't have this mutation are hugely appreciated.

She will still have central apnea.  She will still be on a monitor.  But if we could just eliminate these last few causes then we could go on hoping that for some reason her brain is still immature and that she might outgrow it.

For anyone who's made it this far through my rambling, that's the latest.  We appreciate your prayers so much.  Hopefully we know more, and receive good news soon.  One more normal test and I will be enormously relieved.


Saturday, March 11, 2017

Baby Doll Tessie

When she isn't zipping around the house following the big kids around (and sometimes when she is) she kind of reminds me of a doll.  It's got to be all that smiling she does!  








Saturday, March 4, 2017

A Diagnosis

Where to start?

The last couple of weeks have been intense, between the appointments that we already have, and Tessie's appointments, the calendar has been packed.

On the Monday after I wrote my last post, Tessie, Sadie and I made the hour and a half drive north to the children's hospital that she had been referred to, to see another pulmonologist at the hospital's pulmonology and sleep clinic.

The day off from school meant I had a helper volunteer to come with me.
The doctor was not happy that Tessie had not been put on a monitor for the month leading up to the appointment, since that was the first recommendation that the previous sleep center doctor had made.

She said that if she had seen Tessie when she was three months or five months old, when she was struggling to make eye contact or track with her eyes, she would have been afraid that she had the gene PHOX2B, and that yes, the treatment for that gene generally requires a tracheotomy and a ventilator, but that now, with all the progress that Tessie had made she was nearly certain that wasn't what we were looking at.

She immediately put in an order for a monitor for Tessie to wear while she slept and she ordered a sleep study, coding Tessie's chart as red, which meant that the call from the sleep center came as soon as I arrived at home, and they squeezed her in for an appointment the next night.


We arrived at the children's hospital and crossed the sky bridge and sat and watched a rainbow bubble wall bubble, and a giant projection screen explode with fireworks as the other kids who had come from further than we had waited for their names to be called.

I have to say if you have to get a sleep study done on a baby, and there is anyway to go to a pediatric facility, I would recommend it.  It was so much easier than it had been at the other sleep center or at the PICU.  The sleep study tech quickly hooked her up, and when the nasal canula was too big for her tiny nose he actually pieced a custom canula together and twisted the wire around it that would feel her breath before carefully taping it in place.


It was a long night.  Tessie was furious about the wire that needed to hang down in front of her mouth, because every single time she tried to suck her thumb it would touch her hand.  Inevitably screaming would follow.  But she finally slept for a few hours, waking every hour from midnight until four, before drifting off for two more hours until it was time for us to wake up.

When it was time to wake up she repeatedly rolled over and tried to go back to sleep by burying her little face in the mattress.  But once she was unhooked she was fully awake and we were ready to be on our way.


We drove back to meet Maggie at her early morning speech and OT and then take her to "mermaid school."  And then we settled in to wait.

The sleep monitor arrived.  The first night her oxygen dipped a few times, but was otherwise normal. The second night the alarm didn't sound once.  The third night she stopped breathing around fifteen times, although I can't be totally certain because I lost count after the twelfth alarm.

Fourteen of those times the alarm woke her and she started breathing on her own.  Once it didn't.  I was laying in my bed, staring at monitor, which had gone off twice in the previous hour.  I saw the respirations per minute slowly tick down.  8, 7, 6, 5, 4.... "Tessie!  Tessie!" I said as I started to sit up.  3, 2, 1, 0.  The alarm sounded.  It kept going off as I scrambled over to her and rubbed her still little back hard until she gasped and started to breath again.

That was a long night.  But it was followed by night after night without incident.


By the time that the call came, a week after the sleep study, on Ash Wednesday, I was almost convinced that she didn't have central apnea.  Had I put the sensors on wrong that night?  I'd taken them off twice and reapplied them, but with a few good nights in between I was beginning to doubt what I'd seen with my own eyes.

Which really is the theme of the last month.

Doubt.

I knew I had seen her stop breathing a half dozen times.  But had she really stopped breathing or was her breathing just extremely shallow.  Half of the doctors that we saw believed that something was going on.

Half told me it was probably shallow breathing and that the first sleep study, which showed 45 central apneas, 3 obstructive apneas, and over 130 hypopneas, was just a fluke. One doctor told me that the "story I was telling" didn't match the baby he was seeing.  A nurse repeatedly said "I don't know why we have a healthy baby like you here...", later asking me if our time in the PICU had felt like a vacation because I have all those kids.

The answer was that it absolutely wasn't a vacation.  It was terrifying.  And I was even more afraid to take her home without a monitor, which I repeatedly asked for and was denied.


I really, really liked just about every person who worked with us and helped her, and by the end so many people had so confidently told me that nothing was wrong, that I doubted that instinct that kept telling me to push on until we were absolutely certain that she wasn't going to stop breathing again.

Even the doctors who had seen her before believed that it was over and that I had nothing else to worry about.  I knew that they were more of less humoring me when they sent her for a second opinion.  She had been fine in the PICU.

Except in the PICU there were alarms.  I tried to point our the flaw in our two nights of tests.  She had had a band around her chest monitoring her respirations.  Every time it dipped below ten an alarm sounded.  It sounded until she started breathing again.  It sounded every few minutes all night long.

A vacation, I thought?  On your vacations does a piercing siren wake you up every five minutes all night long?

I asked if the alarms could possibly have been the reason that she didn't stop breathing.  I was told that she hadn't woken up all the way, so that couldn't be it.

After watching how the monitor rouses her just enough to breath again without completely waking her, I believe that that was a huge error.


A few days ago I got the call from the sleep center.  I hadn't expected it.  They would only call with abnormal results and the doubt had been creeping in.  I was beginning to doubt what I'd seen with my own eyes again.

She had had 38 central apneas and 7 hypopneas this time.  Her oxygen level only dipped into the 80s this time, not the seventies.  But she has central apnea.  She had needed that monitor and what I'd been witnessing really was her not breathing for more than twenty seconds at a time.

She now wears a monitor when she naps and at night and when we drive in the car.  Yesterday the alarm went off in the car and continued to sound until she started breathing again.  In two weeks we go back to the same children's hospital for an MRI.  Usually central apnea indicates a problem with the brain stem, so her neurologist would really like to take a look at it to see if that's the root of the problem.


And I am a jumble of emotions.  Initially I was relieved. I was afraid that if the sleep study didn't show anything they would take the monitor away and I would never sleep again.  But at the same time central apnea is a frightening diagnosis, as I was repeatedly told in the PICU, and I hadn't realized how much I was relying on that doubt, and the hope that maybe she didn't have it, to get through the days without a monitor.

"The thing is," one nurse told me after they were certain the first study wasn't accurate, "babies with central apnea will eventually have an 'event' where they stop breathing and don't start again."

Those words keep going through my mind.

I asked the respiratory therapist who called with the results if the improvement in this study compared to the last showed an improvement in her condition.  She said that it might, or that it might just mean she had a good night.  I'm thankful that if she was going to have a night with apneas that at least it was a night while they were doing the study.

So now we wait for the next test and keep lugging her little monitor around with us.  We also wait for another retest on the organic acid test, which for a second time was deemed inconclusive.


Now for a change in direction.

The best moment in the last few days came on Thursday.  James' physical therapist hadn't seen Tessie since before Christmas.  She'd been the therapist that first suggested testing for Tessie, and she had been one of two therapists that evaluated her.

When she arrived yesterday Tessie was sitting next to the fire place but quickly raced across the room to greet her.  After watching her she said that she could not believe the improvement.  She said that in her entire career she had never seen a baby go from the condition Tessie had been in at her evaluation, to the condition Tessie was in as she raced around the room interacting with everyone and smiling widely.


We've appreciated the prayers so much.  And we appreciate them going forward as we try to figure out as much as we can about the reason she stops breathing in her sleep.  At the very least we have that breathing monitor now so that if she stops breathing it will wake us up so that we can help her.

And hopefully I'll have some good news soon to match our smiley, sweet girl.

Saturday, February 18, 2017

A New Sleep Study

I originally wrote this as a Facebook status update, but realized as I finished it and posted it on my personal page that it is basically a blog post in length. And it's an update as to what has been going on these last few weeks while I've been waiting and stressing and silent here. Here it is in all it's Facebook glory:



Finally! Tessie is making the drive to the children's hospital two hours away on Monday to be seen by their sleep specialist/pulmonolgist.

 It wasn't easy getting here. A month ago her pediatrician told me she wanted the doctors at the children's hospital to see Tessie and that she was going to call a friend who worked there and see what he thought and then make the referral. She said she would do it by Thursday.

 Thursday passed, and when I still hadn't heard anything a week after that Thursday (giving referrals a time to go through) I called back. She had talked to the doctor and they wanted to see Tessie, but had forgotten to send the referral through. If I didn't hear back in a week from the hospital I needed to call back.

 Another week passed without hearing anything. I called back. The referral had been sent to the wrong hospital. Whoever sent it through didn't look closely at the destination and sent it to the hospital that did the last one. I managed to convince that hospital that we didn't need to schedule when they called and was told by Tessie's doctor's office to wait another week for the referral to go to the right hospital.



 Wednesday was a week from the last time I had heard from the office. On Thursday I called back. I found out the referral person had been sick for a week and the referrals hadn't been sent through. 

They would put it through as soon as possible.

 I explained Tessie's whole story. I told them the first sleep study showed that she had 45 central apneas and over 100 hypoexemias (I think it was 130, but who's counting at that point) and that while she'd had no events in the hospital (possibly because alarms literally went off every ten minutes the entire time she was there) I'd gotten a home monitor and that the home monitor informs me 2-4 times an hour that it senses "low movement" with a cute little picture of lungs framed in red. I have no idea how accurate the monitor is, because it isn't a medical grade monitor, because if our doctor prescribed a medical grade monitor she would have to formally diagnose her with central apnea and if she diagnosed her with central apnea the insurance would refuse to cover a second sleep study right now. 

We got the monitor after I saw her stop breathing for at least 18 seconds. Which is not quite long enough to count as an apnea but is enough to make it pretty hard to sleep. Ever.

I said that I was really, really worried.

Yesterday the children's hospital received the referral and called. They will see her on Monday at 12:30. However the scheduling person told me that right now they're scheduling into the end of March for sleep studies.



Say a prayer that they find a way to get her in earlier than that. And that the studies in the hospital were correct.

The last month has been a roller coaster and I'm ready for answers (good ones!).

She seems so much better. She's hitting all her developmental milestones now, even the ones that were way, way behind before. She laughs and smiles and looks at our eyes. She says mama and dada and loves playing with the older kids.

I just really, really want to know for sure that she's through this and that I don't need to jump up four times an hour when my phone tells me that low movement has been detected.


Sunday, January 29, 2017

What Must I Do?

Luke 10: 25-37

There was a scholar of the law who stood up to test him and said, “Teacher, what must I do to inherit eternal life?”

 Jesus said to him, “What is written in the law? How do you read it?” 

 He said in reply, “You shall love the Lord, your God, with all your heart, with all your being, with all your strength, and with all your mind, and your neighbor as yourself.”

He replied to him, “You have answered correctly; do this and you will live.” 

But because he wished to justify himself, he said to Jesus, “And who is my neighbor?”

Jesus replied, “A man fell victim to robbers as he went down from Jerusalem to Jericho. They stripped and beat him and went off leaving him half-dead. 

 A priest happened to be going down that road, but when he saw him, he passed by on the opposite side. Likewise a Levite came to the place, and when he saw him, he passed by on the opposite side. 

 But a Samaritan traveler who came upon him was moved with compassion at the sight. 

 He approached the victim, poured oil and wine over his wounds and bandaged them. Then he lifted him up on his own animal, took him to an inn and cared for him. 

 The next day he took out two silver coins and gave them to the innkeeper with the instruction, ‘Take care of him. If you spend more than what I have given you, I shall repay you on my way back.’ 

Which of these three, in your opinion, was neighbor to the robbers’ victim?” He answered, “The one who treated him with mercy.” Jesus said to him, “Go and do likewise.”


Thursday, January 26, 2017

The CD of the MRI

While we were in the hospital the doctors really wanted to be able to see Tessie's brain stem.  That region of the brain controls breathing and since no breathing was the biggest problem that she was facing, making sure that part of her brain was normal was a big deal.  

Thankfully she had recently had an MRI, without sedation,on our trip to Missouri.  

I called and spoke with the contact person for the study and she explained that the doctor who could release the CD was out of the country, but they had an urgent call in to him and were waiting to hear back.

Many times the necessity of having another MRI done was discussed.  It would start with various doctors who'd come to examine Tessie explaining to me that it was likely that the images from a research study wouldn't be as well done as the ones they would need and so we would have to do it over again.  I would nod.  Then they would say, "but putting a baby with central apnea under general anesthesia can be very dangerous.  Because of the breathing."  

Because she might stop breathing, my brain would fill in.  


"So we can wait for the CD and see what it says." was the conclusion reached over and over again.  As long as she continued to do well, we could wait.

Yesterday it arrived.


I'd gotten a phone call from her neurologist's office asking me to bring it in the moment I got it and ask for K-.  K would take the CD and make a copy and then I could keep the original in case any of her other doctors wanted to see it.

The minute we got it I loaded the kids in the car and headed to the neurology office, stopping to pick up Paul on the way.

I'm not sure why what happened next was so amusing to me, because on another day I think I would have been frustrated.  Maybe because it feels like we've been through a lot and so it seemed fitting that dropping off the CD wouldn't be as simple as it probably should have been.

Here's what happened next.

Me: "Hi.  Is K- here?"
Receptionist: "K?"
Me:  "Um... K, Dr. D's... person?  She called and said to bring this in and ask for her so that she can make a copy of it?"
Receptionist:  "Oh!  K.  I'm not sure we can make a copy.  Let's see. She was supposed to go home early today but let me see if she's still here."  (disappears and then reappears a few minutes later)  "Okay, so here's what we'll have you do.  I'm going to point you in the direction of radiology.  You take that down to them and they are going to scan it and upload it to the system so that he can see it right away."

Five minutes later having traveled down to radiology and waited in line.

Me:  "Hi, neurology just sent me down here.  We have this MRI for my daughter and they said that you guys would scan it in so that the doctor could see it."
Receptionist (makes phone call and repeats what I just said, says "okay, thanks." and hangs up the phone:  "Actually we can't do that.  It's actually in violation of HIPAA law. If she'd already had a scan here we would be able to do it.  But she hasn't.  Even though she's in the computer.  So we can't do it."
Me:  "Yeah... she's in the computer because she was in the PICU last week.  They couldn't do an MRI because she might have central apnea and they were afraid she would stop breathing under sedation.  But we have this one which was done as part of a study not under sedation. It took two nights to get..."
Receptionist: "Sorry."
Me:  "Thanks anyways."

Trudging back to the elevator then back to the neurology office I run into someone from radiology who is certain I work for the neurology office and it takes several tries to convince her that I'm not kidding when I say that I don't.

I'm just here a lot.  

Back in neurology...

Me:  "So.... radiology says scanning this is a HIPAA violation."
Receptionist:  "I was just on the phone with them before I sent you down!  They told me they would do it!"
Me:  "I think maybe I didn't talk to the same person you did.  So how about I leave this here with you?"
Receptionist:  "And I'll make sure the doctor sees it and we'll mail it back to you once we get it scanned."

And there you have it.  Getting it wasn't easy and getting it to the doctor was more difficult than I imagined but it kind of fits with the way things have gone (twists and turns and then ultimately working out) and I am relieved to have put that thing in their hands.  And I'm relieved that I did not have all five kids with me while I tried to figure out where it went.

And I'm really thankful to everyone from the doctors to the receptionist at her neurologist's office who made it work so that they hopefully will be able to use this CD since it's what everyone thinks is best for her if there's any way that it can work out.

Now I'll be over here praying that that CD looks entirely normal and that I get that call soon!

Wednesday, January 25, 2017

My Wish for You

Yesterday was a punch in the gut.

I want to write happy.

You have no idea how badly I want to write happy.

But right now I'm afraid.

As the mother of a child with rather intensive special needs I'm actually terrified.


I've been told I shouldn't be.  I was told after the election that there was nothing to worry about. That my fears were baseless.  The conservatives love life and wouldn't hurt the disabled.

People said I was liberal.

As in "look at these stupid liberals who get all their news from MSNBC and wouldn't know the truth if it hit them upside the head."

Which made me laugh.  Sadly.

I had actually voted a straight Republican ticket for years. I was on the board of our Republican Women's group in the county I lived in before we moved out of California.  I've posed for pictures with Republican congressmen.  I've been vocal about being against abortion. And MSNBC?  I don't think I've watched that in over a decade.  We don't actually have cable.

When Tessie was in the hospital I found that CSPAN was the only channel I could stomach, and then only barely.


I watched as the future Secretary of Education said that she thought that choosing to educate children with special needs should be left to the states.  When she was corrected and told that it was a federal law that made sure these kids aren't locked out of schools like they were in the past she said she must have been confused.

Our future attorney general has referred to children with disabilities as the greatest annoyance teachers face.

And yesterday we learned that there is a planned trillion dollars in cuts to Medicaid in the next ten years.  Block grants, that is to say limited amounts of money, will be provided for people with disabilities.

This is expected to be devastating to those with disabilities.

Kids like Maggie.

Kids who have preexisting conditions, will no longer be protected under the law.  Kids who used to go to institutions but who now are in regular classrooms interacting with peers and learning life skills will lose the protections that make that possible.


 
Since having a child with a disability I can tell you that there is one thing that has stood out to me over these last years.  People are often afraid of people with disabilities.  People get uncomfortable around them.  Many don't want to see or hear those who are differently abled.

I think a large part of that is because in the past these kids were locked away and not seen by previous generations.  I absolutely believe that the benefit of having them in the classroom is enormous.  It teaches compassion and wipes away fear of the unknown. And I think it's one of the prolife things that we can do.

Knowing other human beings that are differently abled takes away that anxiety I so often see when people hear Maggie's speech and joyful squeals and realize she's different.

People who know her, or who've even just read about her, begin to see a person not a stereotype.

People who know Maggie, when they hear the word autism, can think of her awesome sense of humor and amazing smile.  It's not just the terrifying diagnosis that some people believe is a fate worse than death (it's not).

A lot of my prolife friends have pointed out that many of the people I was marching with this last week are okay with killing babies in utero that have disabilities.  And obviously I disagree with that.  I've spoken out against abortion many times and for anyone who wonders I'll say it now:  Abortion is wrong.  Always.  I am absolutely against it.

But if you want to stop babies with disabilities from being aborted, stripping them of their healthcare and their chance to go to school, is not the way to do it.

Early intervention services are key to helping kids with disabilities before the gap between their development and their peers development becomes too large to be overcome.  These have been slashed, and my heart has ached to see that it is nearly always done by conservatives.

And now this attack on education and disability benefits?



If those disappear let me tell you, parents of babies with disabilities aren't going to be falling all over themselves to have them.  And those that do?  They won't have a future to offer these children.

It isn't realistic, someone told me a few weeks ago.  Helping all these kids.  Letting them all go to therapy and school.  There are too many of them and it costs too much.

I'm not sure I've ever heard a less prolife argument in my entire life come from the mouth of someone who claims to be for life.  We can't tell women to have babies, all the babies but then tell them that those children are just too much when they get here.

I never believed it when I heard pro-abortion people say that prolifers only care about babies when they're in the womb.  I've known personally too many people who embodied the opposite sort of generosity to believe it.  But I have seen it creep into the reality that I'm facing in the near future, here and there, and I have to say, I am terrified.

I'm sure someone will tell me not to worry. That whatever is coming will be better.  And I sure hope you're right.  Because the safety net has been cut down and striped away and we're about to be shoved over the edge as those therapies and education options are hacked out of existence (after all they can't exist if they aren't being paid for and with preexisting conditions back these kids aren't going to qualify for insurance).



Maybe some people will feel better if these kids are shoved away.  It's easier not to see these differences.  I know I have friends who wish I would stop talking about it.  I feel it keenly when close friends like every cute baby picture but are conspicuously silent every time I post about our worries for the future, or worse, tell me I have nothing to worry about.

Silly me.  Worried that the promises that have been made might come to fruition.

Because it's out of sight is out of mind.  At least if you don't love someone who has faced these challenges.

But I have a prayer.  I know many of you.  You're brave.  And maybe you voted for President Trump.  Maybe you're thrilled with him.  Praise him for the good he does.  I'll be right there beside you.  Pray that he is the president this country needs.  I know I will be on my own knees

But if you see this, these safety nets being cut that hold the hopes of our kids who need extra help please, please, don't stay silent.  You can like him and support him and still ask him, and your representatives to protect the most vulnerable of our population who are outside the womb.

No one else is going to if we don't.  So please I beg of you don't stay silent if you see the small gains of the last decades stripped away.



You don't have to chose between babies or kids with disabilities.  This isn't saving one or the other.  You can do both.  But you need to speak out to protect them all.  Advocating for kids with disabilities and babies in the womb aren't mutually exclusive.  In fact, they should go hand in hand.

I want to write happy.  And I will.

I am resolving to start sharing the glimpses of life that I've loved to share with you in the past, the happy moments that are here each and every day, even when the worries of an uncertain future weigh upon me.  But I will also be writing about the difficulties that families who love people with disabilities are going to be facing in the near future if we really end up plunging off this cliff.

And I hope that you'll be beside me in this, speaking out for the voiceless both inside and outside the womb.