No squeamish pictures, just squeamish talk

If you're one of those people who can't hear about medical procedures without getting sick, you may want to stop right here...because Emma's got 5 medical issues to address.

Let me start from the beginning.

Issue #1: As some of you loyal followers know, on March 16, 2010, Emma was born with her spinal cord exposed (medical term: myelomeningocele), the worst and most common of the 3 types of spina bifida.  The day she was born, her neurosurgeon, Dr. Troup, closed up her spinal cord, tucked it away, and closed her skin.  His plan for her (as with all his SB kids) was to lie on her stomach and let gravity do its work, pulling her spinal cord away from all the other "stuff" around it so that scar tissue wouldn't adhere the spinal cord to anything.  You see, your and my spinal cords just float around our bodies freely at the bottom, and that was the ultimate goal for Emma.  However, her incision opened and was infected for a few really gross weeks.  Any of you nurses who would be interested in seeing what this was like, message me - I have day-to-day pictures to measure progress.  Anyway, this likely led to spinal cord tethering, which is quite common in kids with spina bifida.  The spinal cord isn't floating around - it's stuck to all the other "stuff" by scar tissue.  This problem manifests itself in various symptoms, none of which we have really seen in Emma.  However, we have known it was there and knew that she would eventually have to have a detethering surgery to fix it - in other words, scrape scar tissue away from her spinal cord at the original site of her surgery.  And then lie on her stomach for 5 days, intubated and sedated - because how else do you get a 3-year-old to lie still for 5 days?

Issue #2: After Emma had been with us for about 8 weeks, we learned that she was having side effects most likely from a Chiari II malformation (also present in varying extremes in kids with spina bifida).  An MRI has shown us on multiple occasions that Emma's brain stem doesn't stop at the base of her skull - it keeps going down into her neck and is compressed all the way down to her third or fourth vertebra.  Common sense will tell you it's not good to have your brain squashed.  However, Dr. Troup was only about 50/50 on whether decompressing her brain stem would actually relieve the side effects.  Emma's MRI looks awful, but the appearance of it doesn't really jive with her present symptoms, lack of some symptoms, and severity of symptoms.  But we knew at some point down the road, she would probably need to be decompressed.  This is a process of either shaving off bone from her skull to allow her brain stem more room or a combination of that bone shaving PLUS cutting through the outer membrane of her brain.  Of all the surgeries that kids with spina bifida have to endure, this is one of the most difficult to recover from, especially when you cut through the membrane.

Issue #3: Along with Emma's awful-looking Chiari malformation, she also has an enormous cyst in her spinal cord called a syrinx.  It's dreadfully large, and Dr. Troup has always been amazed at how asymptomatic she has been.  But again, this is something that we have always known would have to be taken care of at some point.

The picture below illustrates a Chiari Malformation at the top - see how the brain is pulled down into the neck?  And the black mass at the bottom in the middle of the spinal cord is a syrinx.  That's where Emma's syrinx starts, but it goes ALL THE WAY DOWN HER BACK.

Issue #4: In the most recent past, Emma was diagnosed with a severe case of scoliosis.  This is mainly an orthopedic issue, but with Emma, nothing happens in isolation.  I posted recently that Dr. Troup feels that the spinal cyst could be contributing to the scoliosis.  There's also a chance that the tethered spine, could be contributing.  But there's also a chance that neither the tethered spine nor the cyst have anything to do with the scoliosis.  A lot of kids with spina bifida have scoliosis just because.  Well, not exactly "just because," but it's too much to get into right now.  Since the spinal cord tether and the cyst have to be dealt with sometime, Dr. Troup wants to do the surgeries now, while Emma is initially being treated with a back brace for the scoliosis.  The treatment of the scoliosis with a back brace and these neurosurgeries is in no way an immediate fix, and there is no instant gratification.  However, Troup's thought process is to go ahead and treat these problems that could be leading to scoliosis since they have to be addressed at some point anyway.  PLUS, who knows - maybe the draining of the syrinx and the detethering could actually improve her mobility - think about it: anything affecting your spinal cord affects your mobility.  Here's a picture of what scoliosis looks like and how to measure it in degrees (Emma's is 55 degrees, which is pretty severe, and I would estimate that this measurement is only about 35 to 40 degrees):

So what about the Chiari Malformation?  Troup would never say that it has nothing to do with the cyst, the tethering, or the scoliosis, but for the most part, Emma's compressed brain stem really has nothing to do with the other problems.  As compressed as her brain stem is, her spinal fluid flows through and around her brain stem like nothing's wrong, so the spinal fluid isn't pooling the cyst...which is usually what happens.  The cyst is literally just standing on its own, unrelated to anything else.  The beauty of Emma being on her stomach for 5 days, sedated and recovering from a detethering and cyst draining, is that she could also be spending that time recovering from the most difficult surgery - the decompression.  So we'll just move forward with that one, too.  Some potential benefits (potential - not certain at all), are less choking on thin liquids (this has been happening since birth) and the reduction in severity of central sleep apnea (her brain doesn't tell her to breathe properly when she sleeps).

And let's introduce another issue: #5.  This isn't really a medical problem, but one that really bothers Troup on all his spina bifida kids.  He knows that they go through enough in life, and it's salt in an open wound (no pun intended) that they have these ugly surgical scars.  When Emma's original incision opened up and got infected, it healed in a pretty nasty way.  The scar is awful, and her skin just looks terrible around it.  So Troup feels bad for her, and he's going to do a little plastic surgery to fix it up.  Kind of a bonus surgery.

So did you keep up?  Surgeries to correct the compressed brain stem, drain the spinal cord syrinx, detether the spinal cord, and clean up her scar, in the hopes that 2 of the 4 procedures will lessen the severity of her scoliosis.  There will be three incisions: at Emma's neck, where Troup will scrape away skull bone; in the middle of her back, where he will drill through a vertebrae, insert a catheter into her spinal cord to drain fluid, and close it back up; and on her lower back, at the site of her original incision, where Troup will scrape away scar tissue from her spinal cord, close up the incision again, and tidy up her skin.

It all happens on Friday, January 3.  Emma will be in pediatric ICU for at least 5 days.  And no offense to anyone - we welcome visitors to see us, but not to see Emma.  Three incisions, three access points to her spinal cord/brain, in the middle of cold and flu season.  We will unofficially quarantine her.  Being sedated for 5 days makes her more susceptible to pneumonia, and if she has to be woken up to fight the pneumonia, she will be sore, in pain, and cranky.  I think you see where I'm going with this.  We've been told that if everything goes as planned, she should be back to herself two weeks after the surgeries.

Now breathe.  That's all we've been doing here lately.  Just breathing sighs of relief that we know when these impending surgeries will actually be happening.  And breathing sighs of resolve to get through this current moment, task, hour, or day...and just move on to the next.  Not looking too far ahead, but also preparing for what lies ahead.

Words we have and continue to live by: Matthew 6:26-27, 34:  Look at the birds of the air; they do not sow or reap or store away in barns, and yet your heavenly Father feeds them. Are you not much more valuable than they?  Can any one of you by worrying add a single hour to your life?...Therefore do not worry about tomorrow, for tomorrow will worry about itself. Each day has enough trouble of its own.