Tuesday, June 19, 2012

Sarah is scheduled to get a helmet on Thursday.  After a few weeks of complicated logistics, we were very excited to finally get that date nailed down.  The sooner it is done, the more good it can do.

Last week some of the hardware from Sarah's most recent surgery had come loose.  We saw a few little pointy bits poking out from her head.  So, we called her plastic surgeon and her neurosurgeon.  

On Friday she saw her plastic surgeon.  From his perspective, she is good to go.  

The neurosurgeon was concerned about pressure.  The neurosurgeon has always been concerned about pressure.  Between her larger than average brain and extra fluid, there has always been a question of how much pressure there is, and how much is too much.  He ordered regular CT scans to monitor changes, but especially pressure.  Today we heard what we have been hoping for months not to hear: Sarah needs a shunt.  

It is not the end of the world.  It is just another thing with another set of complications and issues.  

A shunt is a tube that drains excess fluid.  It drains out of her head and into somewhere else in the body.  The internal design protects Sarah from infection.  It is relatively simple.  There are complications. They fail pretty often.  I forget the exact numbers, but something like a quarter of them fail in the first year, and a quarter of the remaining fail in the second year.  A new set of complications and issues.  

This also means that the helmet is off the table, at least for now.  I do not know whether we will revisit the idea later.  The older she gets, the less good a helmet can do.  I am very disappointed.  Obviously, protecting her brain trumps other concerns, even when the other concerns are too many surgeries.  It is hard to argue.

I was at a family party over the weekend and granddad looked at Sarah and asked if her forehead was tighter.  I did not even hesitate before saying no.  As it turns out, he was right.  Her head circumference has grown between three and four centimeters in the past three weeks.  That is pretty dramatic.  Not dramatic enough for someone who looks at her every day, apparently.

The screws could also be a clue that there is too much pressure.  It is possible that they were displaced by the internal pressure.  One of the plates is pushing out as well.   (Doctor said he had never seen that before.  Do you want to talk about things a Momma can tire of hearing?)

The CT showed less room and thinner bone.  Both red flags.

He said that a few months ago her had two or three reasons to get a shunt, and seven or eight reasons not to,  and the opposite is true now.

Instead of a helmet, this Thursday Sarah will get a shunt.

For months and months, Josh and I have been afraid to schedule anything in the future more distant than a week.  The summer looked wide open though.  Sarah was not supposed to have another surgery until the fall.  So, we went ahead and scheduled a trip.  It had been confirmed about forty-eight hours before we got the news that Sarah needs a shunt and she needs it now.  We are optimistically refusing to cancel, even though it was supposed to start this Saturday.

Things like this are discouraging.  We were discouraged.

Last night we lay in bed chatting with little Sarah.  Her huge eyes looking brightly up at us.  She was not tired, though it was very, very late.  She is a pretty quiet baby and she was quietly listening to us worry and try to sort out practical details.  (Where do we take Lily?  Should Josh take off work?  What about the vacation days?  Who should stay with Sarah in the hospital?)  Sarah listened.  Then she spoke.

"Da da."

We cheered and smiled and laughed and wondered if it was a fluke baby babble.  Sarah put her hand on her forehead and signed, as she spoke again.

"Hi Da da."

She closed her mouth, and looked at me.  "Mom" did not come out, but she formed her mouth correctly and tried, as she tapped her hand on her chin to sign Mom.  Then she looked at the empty doorway and tapped the tip of her tongue at the front of the roof of her mouth twice.  Lily.  She was looking for Lily.

Amazing little wonder.  To chase away any remaining doubt, when she saw Dad this morning, she looked right at him and asked, "Da da?"

We still have some practical considerations to work out, but we are far to delighted to be discouraged.  Alleluia!  God is good.

Friday, June 8, 2012

What is going on in the wonderful world of Sarah?

Sarah's surgery in early May went well, and has mostly healed.  She had follow-up visits with both surgeons involved in the surgery. 

The bone that they had to work with was "paper thin."  That made the reconstruction work difficult.  It is thin because it the bone has been pressed from the inside.  I discussed it a bit when she had her first surgery in September.  Normally the plates in your skull are free floating at first.  The brain grows, the plates come apart, the bone grows and around again until you are done growing.  If the plates fuse to early, the brain still grows, and it grows where it can.  The growth can cause dangerous pressure concerns.  The pressure pushes the bone which sort of molds around the brain.  The bones can be thinner than normal and not as smooth. 

So, that is what the surgeons are up against.  They have to make sure that Sarah's brain is out of danger.  They want to minimize surgeries.  They want growth to be as normal as possible, both cosmetically and developmentally. 

This surgery was a big one.  And it seems to have gone very well.  About a week after the surgery we noticed something pointy pressing from underneath the skin on the top of her head.  We had already scheduled our follow up visits, and we were glad.  We showed both surgeons and neither seemed particularly worried that one (or a few) of the screws would have come loose. 

Side note: Before I was a mom I was a preschool teacher.  Once, a four year old boy climbed into my lap with a tool box and a look of consternation.  He picked through his tools very carefully, and chose a bright orange screwdriver. 
"What's wrong?"
"You have a few loose screws," and he set to work fixing them, just above my temple.  Maybe loose screws are genetic. 

In any event, we were told to be sure to call if the screws actually poked through the skin, in which case it would be a simple matter to remove them.  Otherwise, we were not to worry. 

In the follow-up visits, we also discussed the next step for Sarah.  Both surgeons have worked on many children with Sarah's syndrome, and other craniofacial issues as well.  Sarah's case is, apparently, unique and difficult.  One suspects that all cases are unique and difficult. 

The next step for Sarah is not typical for children with Apert syndrome.  The next step is a helmet. 

The helmet is custom made.  You have seen the kind of helmet she will get.  When children sleep only on one side and they develop flat spots, this is the helmet that is used to help correct the problem.  The helmet does not push and shove the head into shape, as say braces on teeth work.  Instead, it is molded very precisely to the shape of the head.  It is form fitting, without pressure.  Then they carve out the interior of the helmet where they want the bones to grow.  The head grows into the helmet.  Then they carve out more.  The idea is to train the growth of the bone, not to remold what has already grown.  So it only works when the kids are babies, because that is when the most significant growth happens. 

For Sarah, there is hope that this helmet will help to reduce the necessity of future surgeries.  Also, if it works well, it will help her head to grow in a more normal, round shape.  So, win-win.  Cosmetically useful, medically a good choice. 

There is risk though.  And I think this is why it is not typical for kids with Apert syndrome.  These kids have larger than average brains and typically more fluid in the brain.  Pressure is already a concern.  Add a helmet, and you can add pressure.  That is obviously not insignificant.  Pressure can damage her brain, as it did before she was born. 

Furthermore, the actual molding is more art than science.  This might be true for most of Sarah's interventions. 

For the next few months, after she gets her helmet, we will follow-up once a month with the orthopedist who is making and reshaping the helmet.  We will follow-up once a month with the plastic surgeon, who wants to keep a very close eye on the work, because it is atypical, and typical is already not simple.  We will follow up once a month, with a CT and a visit with he neurosurgeon to make sure we are monitoring intracranial (in the head) pressure very closely.  We will watch closely at home for any neurological symptoms.   It is a little intimidating, the number of doctor visits that will be required over the next few months. 

We are excited, and confident that it is the right thing for Sarah. 

Thursday, June 7, 2012

Why has it been so long since I posted?  Well, there are excuses.  Sarah got sick, then the surgery then home then sick again.  Three hospitalizations.  The Lily got sick, awfully sick, twice.  The second time she was sick with both a nasty virus and Lyme's disease.  Then Sarah got sick.  It has been a rough couple of months. 

But the truth is, there are wonderful excuses too.  We have been out sightseeing and picnicing.  We have been playing and cooking and gardening.  I should learn to add pictures, because there are some wonderful pictures. 

We are all well now. 

I have decided that rather than blogging when I get around to it, I'll write every day.  Unscheduled things never happen when even the scheduled things are falling aside.  So, I am going to keep two blogs.

Wonderfully made

I will never forget when I first met my first daughter.  For months I had been losing sleep, changing my diet, scheduling my life around this little person.  For months, I had thought of myself as a parent, and I had enjoyed being Mom.  But there was something unique and beautiful in that first moment when I saw her.  I think it was the first time I had any real sense of the word 'awe.'  (Awe, like awesome, not Aw, like awww... how cute.  Well, maybe both.)

My world simultaneously grew and shrank.  Nothing seemed to matter outside of the little hospital room, and yet somehow that was bigger than anything I had ever known.  She was just beautiful.  Perfect. 

So this, I thought, is what it feels like to think that your child is incomparable.  I swore I was not going to be that parent.  You know, the one who cannot see any fault with their child.  But there she was, and she was perfect. 

And then an awful thought crept quietly into my head.  Would I love her less if she was not classically beautiful?  Would I love her less if she was not so perfect?  I did not want to dwell on the thoughts.  I did not allow the thought to formulate in words; it felt like treacherous anxiety.

In the years since then, I never thought about it again. 

These thoughts have been mulling in my mind for a few months.  Can I be honest?  Should I be honest?  How can I tell people this?  I have settled on Chronology for the 'how', and I am still not sure about the other two. 

There is a lot of contradiction in parenting (life?).  For instance, we often regret how fast time flies.  We are told we must hang on to every moment because the time will go by so quickly and we will miss it.  The truth is, things happen fast because so many things are happening.  If you ever had time to ponder the reality of the challenges and scope of parenting, would you do it?  Carpe diem!  Are you kidding me?  From day one challenges confront your sleep-deprived self.  How can one tiny person who sleeps all day require so much of me? 

Every step of the way the masses remind you to treasure this time because what is coming is harder.  This starts during pregnancy, and from what I can tell, it never ends. "Just wait until she is born, you'll never sleep again."  "Just wait until she speaks, you'll miss the days when she couldn't."  Just wait until she's walking, screaming, tantruming, borrowing your car and stealing your clothes.  Just wait.  Also, Carpe diem, or you'll regret it. 

Still, the set up is generally pretty good.  By the time I get around to wondering if I can handle it, it is generally over.  Because I am not living in every moment.  Some moments, I just survive.  And those quiet or sunshiny moments?  I seize those.  Forget the rest.  I don't want to exhaust myself seizing. 

I spent as much time as any other parent, I suppose, beating myself up because I did not want to cling to every minute of every precious day. There were enough wonderful days and pieces of days to fill hundreds of memory books, which I will never get around to filling because that would not be appropriate time seizing.  There are joys enough in the day; there is no need to invent them.  It took a second child for me to allow myself to moderate that common bit of wisdom guilt-free. 

When Sarah was born, I did not have time to think about much at the beginning.  There were so many things that needed urgent attention.  This surgery or that?  Surgery at all?  What about her heart, her lungs, her kidneys, her fingernails?  Can I hold her?

The first time I held Sarah, my heart filled in a very familiar way.  The world grew and shrank, inexplicably.  In utter amazement I saw my daughter.  "My God!" I thought, "Have you ever seen anything so beautiful?  So perfect?" 

Sarah, my tiny love, lifted my heart.  Her tiny hands would need surgery.  Her face showed tightness from pressure inside.  She was lovely.  Amazing.  Beautiful.  Perfect.  It is another moment I will never forget. 

"You formed my inmost being;
you knit me in my mother’s womb.
I praise you, because I am wonderfully made;
wonderful are your works!
My very self you know.
My bones are not hidden from you,
When I was being made in secret,
fashioned in the depths of the earth.
Your eyes saw me unformed;
in your book all are written down;
my days were shaped, before one came to be. 
How precious to me are your designs, O God;
how vast the sum of them!"  Psalm 139: 13-17