"Rejoice in the Lord always. I shall say it again: rejoice!  Your kindness should be known to all. The Lord is near.  Have no anxiety at all, but in everything, by prayer and petition, with thanksgiving, make your requests known to God.  Then the peace of God that surpasses all understanding will guard your hearts and minds in Christ Jesus."  Phillippians 4:4-7

Last night I felt peaceful.  It was a familiarly strange peace.  Months ago, when we had just gotten a diagnosis, I asked for your prayers and I found peace.  Then, as the early months progressed, at times very frightening, I asked for prayers and I found peace.  For the past two weeks, I have been wanting that.  I have been scared.  I was having trouble sleeping and sometimes trouble eating.  Last night I asked for prayers, and I found peace.  Peace that "surpasses all understanding."  Sarah was scheduled for a third extubation attempt this afternoon, and I was at peace.  Prayer works.  

I kissed my baby goodnight and I went to sleep comfortably.  

I was awakened at two in the morning by a strange but familiar sound.  I could not identify it through my sleepy daze.  A gurgling sort of sound.  The sound inspired very strong and positive emotions before I figured out what it was.  I opened my sleepy eyes to see a very nervous nurse standing over my baby.  And it dawned on me: those noises were crying!  Sarah was crying and I could hear it!  You cannot hear a baby's cry when they are intubated.  Something was wrong.  The reason it inspired positive emotions was because the first time I heard the sound was when she was just a few months old and she had just been successfully extubated.  That sound then meant she did not need a tracheotomy.  That sound was Sarah's voice.  This time, the sound only meant one thing: Sarah's breathing tube was out.  

The nurse called for help, and soon the room was filled with very concerned doctors and nurses and respiratory techs.  Sarah was crying.  They checked a number of things (was it possible that she was just able to get sound around the tube?) but quickly determined that the tube was out.  It did not look like it was out, but it was.  Sarah had managed to get the tube out on her own.  They quickly got the tube all the way out.  Some quickly prepared to re-intubate.  Some quickly set up the high-flow nasal cannula.  She was not happy, but she was not in obvious distress either.  They were prepared to re-intubate in a big hurry, but they decided to give her a chance to breathe.  And, amazingly, she did!  She was angry.  She was in pain.  They could not give her sedation medications to calm her down or blunt the pain, because it might depress her respiratory drive.  But she was breathing on her own.  The nervous crowd disappeared.  After half an hour, they ordered a chest x-ray and a blood gas to make sure everything was as positive as it seemed.  Both were encouraging.  I got to hold my baby.  I held her in my lap for hours.  She calmed down and slept peacefully in my arms.  It was wonderful.  Amazing.  Peaceful.  

As you can imagine, we had a lot of visitors in the next few hours.  Her nurse came in often to check everything and make sure Sarah was getting better not worse.  The doctors came in, one after another, to check on her.  She looked and sounded better every minute.  She was working hard that first hour, but her work was successful, and slowly, slowly, she was able to calm down.  By the time we would have been waking up on a normal morning at the hospital (5:30 or 6) her breathing looked normal.  It was supported, of course, by high flow oxygen through a cannula, but she was not exhausting herself with labored breathing.  She was just breathing.  

We cannot predict the actions of God or our children.  In retrospect, it is much better that she did not have to go across the hospital to the OR to be extubated by strangers with Mom no where in sight.  In retrospect, it was wonderful to skip the hours of suspense and skip right to the breathing.  The best laid plans are sometimes best ignored.  

That said, Sarah has been given strict instructions never to do that again.  

I am not feeling very insightful.  I am trying to work through my nerves.

Tomorrow we hope and expect to extubate.  Breathe, baby!

I am more nervous than I would like to admit.  I am drinking my tea and trying to stay calm.  The doctors will round soon, and I will hear their plan to extubate.  I know that they are stopping her food at 6 a.m., which means that they are still planning to extubate, but not in the morning.

"Peace I leave with you; my peace I give to you. Not as the world gives do I give it to you. Do not let your hearts be troubled or afraid."  John 14:27

I pray for that peace as I try to go to sleep tonight.  Peace that I cannot understand and the world cannot give.

I am praying for healing, for my sweet Sarah.  I know God will heal her.

I am praying for the doctors who are responsible for figuring out a good plan to get her breathing on her own.

The word trache is bumping around again.

A little about what has happened.

When Sarah was extubated about a week ago, she did not do very well.  They tube was out for over an hour as we hoped she would improve.  She didn't, and they re-intubated.  That is always a risk, and the doctors minimize the risk in a number of ways.  They make sure that the vent is on a low setting.  They make sure that you are "breathing over" the vent- you are taking more breaths than the vent is giving.  They make sure that there is an "air leak," which means that some air is getting around the tube.  The air leak is important because it shows them that your throat is not swollen and that they can remove the tube without damaging the tissue or causing irritation that will swell and require re-intubation.  They look for minimal secretions.  If there is fluid in the lungs or so much fluid in the mouth that they worry about fluid in the lungs than they have to weigh that as a factor.  Will it cause a plug so the lungs cannot work?  Will it be too much for you to manage without help?

Of these, all signs looked great the first time with Sarah.  Except that she had (always has) more secretions than they would like.  The ERT (extubation readiness test) was excellent.  So they were surprised when she did not do well, but they are always prepared for that.  The re-intubation procedure went smoothly.

What happened?  We discussed it at length.  She was crying the whole time the tube was out.  They gave her some morphine for pain- it was only a week after surgery!  But morphine does not really work well for her, so it could have been that she was in pain and just too agitated.  It could have been secretions.  She could still have been feeling pneumonia too acutely.  Her lungs simply were not strong enough yet and she was not ready.

So we did a few things over the next few days to prepare her for a second attempt.  We got a better pain plan in place.  We gave her more time to recover from the pneumonia.  They put her on a medication to reduce secretions.  (Robinul, for the curious.)  After a few days, she had fewer secretions, she was back to low vent settings and she needed less pain/sedation medication.  She seemed ready.

She did her ERT.  It went very, very well.  There was a hiccup that morning.  The nurses went to suction her tube.  (As I explain to Lily, when you cough, you can get stuff out.  When she coughs, it gets in her tube and she needs someone to suction it out.  Ick.)  They had a little trouble- it was thick and it got stuck.  It was not an emergency- they were able to deal with it pretty quickly, but they did reconsider extubating that day.  A mucus plug can cause a lot of trouble.  They decided she was ready.

It is always a balance of risks.  When someone is intubated there are a million things that are more difficult.  You have to keep kids sedated, so they don't pull the tubes out before they are ready.  And sedation comes with its own risks, some of which increase the longer you are on them.  The tube itself is high maintenance.  Sarah is a wiggler with a lot of oral secretions (drool- she is teething!) so they have to get three people in the room about once a day to re-tape her tube.  They have to suction every time she coughs, and she coughs pretty often between the pneumonia (better now!) and just having a tube in her throat to stimulate.  Your body does not recognize the tube as helpful, so it fights.  Getting extubated in a timely manner matters, and not just because you want to get out of the ICU.

The second extubation was not as smooth as the first, as I explained in my other post.  It feels odd to describe a failed extubation as having gone smoothly, but the first had.  When they took the tube out the second time, everything happened quickly.  There were no minutes of waiting, watching and hoping.  She needed help in a hurry.

So what happened this time?  Again, there were a lot of theories.  Maybe the mucus was too thick, and there  was a plug?  Maybe she was too sedated to work?  One thing was clear upon re-intubation.  There was an upper-airway obstruction.  They had to use a smaller tube to re-intubate.  Swelling?

So... tomorrow.  We are addressing everything.  We are walking a fine line with her secretions.  The Robinul thickened everything up too much, that was clear even if it was not the cause.  But she has too many secretions to ignore.  So, we cut the dose in half and are monitoring very closely.  We pulled back further on sedation, but again we are walking a fine line.  If she goes into withdrawal, that will not be helpful.  But she has to want to work to breathe.  Swelling is a major concern, so she started a steroid today.

We also want ENT to weigh in, and to be present for the extubation.  I missed them today (I was at MRI) so I will not get the full report on their recommendations until tomorrow morning.

The plan, as it stands now, is to extubate tomorrow morning, but this time with ENT present and in the OR.  A more controlled setting.  Ideally, they will just observe.  We are hoping for a crowd of bored bystanders.

We are not going to go into the various ifs.  Tomorrow Sarah will breathe.

Singing:
Spirit, come, transform us. Come be our breath, be our hope!

My tea is cold.  I am going to get another cup.

"For my thoughts are not your thoughts, nor are your ways my ways."

Almost two weeks ago, Miss Sarah had her surgeries.  They went well.  Her palate is repaired.  She has a thumb and a pinkie finger, currently under wraps.  There are tiny little tubes in her ears to help drain fluid.  We should be home.

"For my thoughts are not your thoughts,
nor are your ways my ways." Isaiah 55:8

*Sigh*

After the surgery, the anesthesia team was not comfortable removing her breathing tube.  At the time, I did not really understand why.  Sarah is a complicated kid.  It was a mouth surgery.  I figured they were just being cautious.  So we would spend a night in the Pediatric Intensive Care Unit.  We have lots of friends in the PICU.  An extra night in the hospital was not a big deal.  Surgery was on a Tuesday, we had planned on a three day hospital stay, but knowing her and knowing how cautious everyone is with her (justly so) when I was told three days, I heard "through the weekend."

Tuesday night: "Tomorrow we will think about extubating."  She was not ready Wednesday.
Wednesday night, "Tomorrow we will think about extubating."  She was not ready Thursday.
Thursday night, "Tomorrow we will think about extubating."  She was not ready Friday.
So it went until Monday.  Sarah had Pneumonia.

But, on Monday the 20th she was ready!  They did an extubation readiness test (ERT)  for a few hours.  Essentially, they turn off the vent, but leave the tube in so Sarah has to breathe but if she gets into any trouble they can fix it in a hurry.  She passed with flying colors.  She was fantastic!  They gave her a break for a few hours, so she would not be tired when they extubated.  Then they pulled the tube!

She cried and cried.  I held her and tried to calm her down.  She could not catch her breath.  She could not stop crying.  Was it pain leading to extreme agitation leading to inability to breathe effectively?  Were there too many secretions leading to inability to breathe effectively leading to extreme agitation?  What was the chicken and what was the egg?  In the end, it did not matter very much.  It became clear that she needed help.  She was re-intubated.

We were all pretty discouraged.  It does happen, but we were so optimistic!  Monday was the day I had expected to go home.  (Can't trust that day.)  This was a setback.

That evening, I chatted for awhile with a wonderful family I met in the PICU waiting room.  They are a wonderful, optimistic, faithful, strong family.  I have enjoyed getting to know them.  One of them was talking about how to love our babies in this setting.  We cannot pick them up when they are intubated.

"I just want to hold her and hug her."

I fell apart.  Contrary to what you might think, since I keep a very public blog, I am not prone to public expressions of emotion.  I just broke.  In a moment I was crying on the shoulder of a near stranger.  I wanted so badly to hold my sweet Sarah.

Josh posted scripture on Facebook that night.

"Amen, I say to you, whoever says to this mountain, ‘Be lifted up and thrown into the sea,’ and does not doubt in his heart but believes that what he says will happen, it shall be done for him." Mark 11:23

She will be well.  God is in control.  It has only been a week.  We finally admitted that it was not going to be an in and out hospital stay, and we checked into the Ronald McDonald house.

By Tuesday night, we were talking about extubation again.  Sarah had been started on a medicine to reduce secretions.  Her vent settings were low again.  They had a better plan in place for pain management.  Wednesday we would extubate.

Wednesday morning the ERT went beautifully.  She was amazing.  Lily and I were at the Ronald McDonald House, awaiting news that the extubation was successful.  Josh was with Sarah.  We had our PICU dream team in place.  This was going to happen.  I got a text message from Josh.

"Pray."  I did, with Lily.  Then a few minutes later, "Pray hard."  I did, silently.

After an eternity, Josh called.  Sarah had failed extubation.  Unlike the last time, this time in minutes.  This time she did not breathe at all.  This time the high flow O2 through a nasal canula did nothing for her.  They gave her a mask, but still no air was getting in.  Her heart rate plummeted.  They started CPR.  My amazing husband was in the room watching helplessly.  The sequence of events is a little jumbled in the retelling, but Sarah required twelve minutes of CPR.  They re-intubated as quickly as they could.

Small blessings: we had plans to visit with a dear friend.  She arrived just moments after I spoke with Josh.  So, I pulled myself together and got in her car and went to the hospital.  Josh did not have to leave.  I did not have to wait for mom.  Lily would have a ride home to the grandparents' house after a very brief visit.

By the time we arrived, Sarah was stabilized and sedated.  Lily would see her sister sleeping peacefully.

By the time we arrived, every specialist in the hospital had come through.  Josh tried to keep up with it all.  A social worker came in.  "How are you doing?  Can I get you anything?"  By the time we arrived, Josh and Sarah had been through Hell.

The biggest concern on the table was new.  Sarah coded.  This event had all kinds of frightening potential outcomes, but at the top of the list: brain damage.  She was hooked up to an EEG machine, to monitor for seizures.  Her eyes were pried open every hour to make her pupils dilated in response to light.

This was not a minor setback.  No food.  In fact, nothing in her belly.  We cannot know it is working and we cannot risk it not working.  Sedation is up.  Vent settings are up.  But most important, neurology.

For forty-eight hours, everything was going to monitored.  Even when everything looks good, the doctors are hesitant to say so because in the first forty-eight hours after an event like that, everything can change.

"I command you: be strong and steadfast! Do not fear nor be dismayed, for the Lord your God is with you wherever you go." Joshua 1:9

Are you kidding me, God?!  This is my baby!  I was looking for encouragement in scripture and I found that verse.  I was afraid.  Very afraid.

I had met a father at the Ronald McDonald house that morning.  He was really upset, and he just wanted to talk.  So I listened.  He was worried about his very ill daughter and struggling with his wife.  He was fighting insurance.  He was questioning.  He was trying to talk himself out of losing faith.  After listening for about half an hour I said all I had to offer.

"You just have to trust that God loves your little girl more than you do.  He is in control and He is holding her in His arms."

Now I had to own my words.  It is so easy to say things like that.  Our all-knowing, all-powerful Father is in control.  I'm not.  I never was, but neither had I ever felt quite so powerless.  God loves Sarah.  I will trust in His love.

Today the forty-eight hours are up.  She did not have any seizures.  She has had normal neurological behavior.  All the labs are positive.  She is back to her baseline.  They may still want an MRI of her brain to look for damage, but we are overwhelmingly happy with what we have seen.  It is within the bounds of what we understood was possible, but outside the bounds of what we had been allowed to expect.

We still have big hurdles.  Sarah is still intubated.  It is midnight, and I am up waiting for evening rounds.  I expect to hear a pretty coherent plan for extubation- possibly on Monday.

"The Lord’s acts of mercy are not exhausted, his compassion is not spent; They are renewed each morning-great is your faithfulness!  The Lord is my portion, I tell myself,  therefore I will hope in him."  Lamentations 3:22-24

Long days

On Tuesday this week Sarah had surgery.  This was the long awaited hand/palate/ear surgery!  We were very excited.  It took seven and a half hours for the three surgeons to do their thing- and there were no complications.

The ears needed a little help draining fluid, and so she has tubes in her ears.  This is a fifteen minute procedure done under sedation.  It is common, and usually outpatient.

Sarah had a cleft palate.  It was relatively uncomplicated.  This is never outpatient, because if there is swelling, as is common after surgery, it would be in the mouth and obviously that is dangerous.  Generally, an overnight stay in the hospital to watch is expected.

Sarah had the first of three syndactyly release surgeries.  That just means they are separating her fingers.  They released her thumb and pinkie.  This is not a simple surgery and the recovery stinks.  This is the one of Sarah's three which took the most time.  Our surgeon said that this is sometimes outpatient (!) but not with Sarah because she has so much else going on.  Even without the cleft palate surgery we would have expected to stay a couple nights in the hospital.

The surgeries went well.  The surgeons were pleased.  We were pleased.  Then the anesthesia team went to take out her breathing tube, and they couldn't.  Sarah was not breathing well enough on her own.  So, we went to the ICU, expecting to stay overnight and extubate in the morning.  She just needed a little time.  After all, a seven hour surgery is quite a stress on the whole body.

Four days later, we are still in the ICU.  Sarah is still intubated.  And now, Sarah has been diagnosed with pneumonia.  When kids (maybe adults, I have no idea) are intubated, they have to sedate them to keep them safe and comfortable.  It does not feel good to have a tube down your throat, but if she pulls at it, she will not breathe.  The problem is, that the medications that they give for sedation also depress respiratory drive- the drugs that make the tube safe and tolerable also make it harder to wean off support.

We have been around this block before with Sarah.

She has an amazing team of doctors and nurses here in the PICU.  Many of them remember her from previous stays.

The good news is that Sarah looks great!  Her pretty pink cheeks are back to normal size.  She wakes up and looks around.  Since she is intubated, she cannot talk, but she smiles around the tube.  And her eyes light up when she is happy.  She is alert and seemingly pretty happy.  Her heart rate has come down from crazy land into a normal range, so I think that her pain is under control.

The bad news, of course, is that she will be intubated until she shows us that she is ready to breathe on her own.  Status quo?  She is doing very little on her own.  She has pneumonia, which will slow things down and make her feel miserable.  She has a fever, which is manageable  but only with both Motrin and Tylenol on board.  She is having some trouble tolerating her food.  It is not out of the ordinary.  Again, those same meds that kill the pain also slow everything down, including digestion.  So, we are medicating side effects of medication.

But, it is working.  She is not in pain.  She is getting better.  She is making a little progress each day.  Every day since the surgery, it has been the optimistic hope of the medical team to extubate the following day.  Now I think they actually have a handle on her.  Her progression is slow, not normal.  If we push her, she gets worse not better.  Because of her history she needs more pain medication than one would expect for a child her size.  The X-rays look a little better every day.  She needs a little less breathing support every day.  She is not ready to pull back on pain medication.  These are the little lessons that are actually huge.

Hopefully she will be breathing on her own within just a few days and go home shortly thereafter.

On the third day of Christmas this year I got the best gift imaginable: Miss Sarah laughed.

Sarah is fifteen months old.  It is still fun to watch her learn.  Remember the first time your baby smiled, and everyone told you it was just gas because he was only three days old?  I have been looking at that smile for just over a year.  Nothing will convince me that our sweet little ones do not express involuntary joy in the form of a smile, even when they are just born.  But later, when it is inarguable.  When they laugh.  Their delight is so beautiful it is contagious and you laugh right along with them.

Yes, darling, you are right.  Peek-a-boo is the funniest thing in the world.  Daddy is gone and then he appears!  The world is full of magic.

Sarah laughed when her Daddy was playing peek-a-boo.  He hid his face in her belly and then popped up where she could see him.  Her face pulled into one of her rare smiles and a giggle popped out.  Was it a giggle?  Do it again.  It was a giggle!  And then a belly laugh!  Sarah was laughing!

Since then we have made two discoveries.  Mom is not as funny, even when she does the same thing.  Dad has other clever tricks which are all funny.

We are finally on a regular therapy schedule.  It took six months, but it is happening.  We are seeing progress.  Sarah is getting stronger.  Her upper back and neck muscles are not as strong as they should be.  She cannot lift her head.  Her head is bigger and the muscles are not as well developed.  Usually, babies start with tummy time in the early months.  They begin to lift and turn their heads and then they learn to push up on their hands.

Sarah spent those months in the hospital.  Then home from the hospital when I would put her on her tummy she would scream.  I could not tell if it was fear or pain.  I asked everyone.  Is she in pain?  There is no apparent anatomical reason for her to be in pain.  She probably just hates it because it is unfamiliar.  I kept asking.  What can I do?  All the usual advice for tummy time haters did not help.  She did not care if I was on my belly too and talking to her.  She did not calm down when I massaged her back and legs or sang to her.  Finally on one of our unscheduled hospital stays I met with a therapist who said that she might be in pain.  She worked with kids of all ages, and she said that though most kids with a Mickey button (that is the button in her belly where we attach Sarah's feeding tube) express mild or no discomfort.  Some find it intolerable.  She showed me a few tummy positions which got the pressure off her button, and, lo and behold, she was soothable.  She was not happy, mind you.  But she would settle down.  So at about a year Sarah began to develop the muscles which would need to be stronger than most kids need, but were in fact weaker.

We also got an adaptive chair for her from an equipment exchange.  She likes her chair, and I like seeing her sit up!  Again, the muscles that she needs are not as well developed as they should be, so she can only tolerate sitting up for about half an hour, but that is half an hour she did not have before!  And she is getting stronger.

We have big things planned in her future, both medically and developmentally.

Sarah will get her surgeries on January fourteenth.  Please pray she stay healthy for this important surgery!  I feel a little bad not taking Lily outside to enjoy the cold winter air, but it is so discouraging how many times Sarah has gotten sick.  I don't want to take her to the mall or for walks outside.  I mean, I do, but I won't.   I like the sensory stimulation of seeing other places than her house.  Riding in a stroller, looking at strange and interesting things, smelling new smells... It all seems good.  But at this point, it is a risk I will not take.  When Josh is not working, he takes Lily out and about.  Sarah stays in.  No play dates.  No mall trips.  No walks in the cold outside air.  I think the outside could do her some good, but I am too scared.  It is in the low thirties and she can wait two weeks.

This is the same surgery that has been scheduled before.  It is the first of three hand surgeries- this one will separate her thumb and pinky.  Her cleft palate will be repaired and she will get tubes in her ears.  I cannot express how excited we are for her for this surgery!

Our to-do list grew extensively over the holidays.

Her last serious illness was very discouraging.  We had to cancel the surgery, which was already delayed past the recommended timeline.  We had spent another few days in the hospital because she was not breathing well.  We were given a maintenance inhaler, which is great on the one hand because it should help her to keep minor colds from turning into major events.  It is great, objectively.  But it is one more thing to remember every day twice a day.  And we have to face the reality that this has not been a series of terrible illnesses; Sarah has something chronic going on.

Every time we go to the hospital in respiratory distress, they do the same thing.  Oxygen.  Albuterol.  IV in, then steroids and fluids through the IV.  No food until her breathing is under control.  Lung X-ray.  And, every time the X-ray has shown cloudiness in her right lower lung.  And, every time, this cloudiness is diagnosed as pneumonia and treated as pneumonia.  So, a course of antibiotics.  Once we went and she had not finished her last course yet, so they put her on a second course of different antibiotics.  This fall some doctor had the good sense to compare the X-rays.  They all look the same.  Whatever is going on, it is not changing.  She did not have a suggestion as to what that means, except that it is not pneumonia and whatever it is, it is chronic.  We have to get her to a pulmonologist.  

Also, her therapist is recommending all kinds of equipment to help her development.  Equipment is not simple.  You have to get measured and approved.  Then there is some question of who will pay for it.  Our insurance has not balked yet, but it has been strongly recommended to us that we get secondary coverage.  So, we are looking into Social Security and Medicaid.  We don't really know how these programs work.  Everyone we talk to refers us to someone else who does not seem to know either.  We have hit a few brick walls.  We have now been told three or four times that we absolutely will not qualify because we have insurance and a reasonable paycheck.  We have also been told that we absolutely will qualify because of Sarah's needs.  Status quo: we do not know what to apply for or where to apply.  Best advice so far: "Move back to Maryland.  It is very hard to get covered in Virginia."

The to do list:

• Surgery & pre-op consults, including a CT to get clearance from neurosurgery.
• Pulmonology
• Equiment: adaptive stroller, stander, batch chair and possibly adaptive car seat
• Secondary coverage for medical bills and equipment

That is what we get for taking a month off.  We decided after the surgery was cancelled that we were going to enjoy our holidays like normal people.  We were going to go visit family.  We were going to decorate the house.  We were going to shop.  We made a gingerbread house and cookies and fudge.  We made caramel popcorn. We stayed in and snuggled and watched movies.  Although we took phone calls and answered any questions asked, we took a break from what had begun to feel like a treadmill of medicine.  I feel a little guilty, but I also feel able.  Able matters.  Sometimes Sarah's medical needs are our whole life, and for a month we lived a normal life.  

And what detracts from the guilt?  Sarah laughed.  For the first time.  She laughed!  I know that we need to do all the things we need to do.  We will.  I know that part of the reason we were able to take a month off is that Sarah stayed well enough to avoid the ER for a month.  I know, I know, I know.  But I also know that laughter is more important than anything the doctors can do.

A joyful heart is the health of the body,
but a depressed spirit dries up the bones." Proverbs 17:22