Surgery date

Today was a big day!

Meghan had OT and PT this morning.  This was the first time Meghan had therapy since finding out about surgery.  I talked to them about Meghan possibly needing SMO's.  They suggested that we wait until she has her surgery and then request a consult with a PT at Children's while she's recovering.  They said a PT at Childrens will do a consult and determine if she needs to be fitted in SMO's or not.  She might not need SMO's in hopes that the surgery will improve her low muscle tone.  If she would need SMO's, they said we don't want her to be fitted in or around New Ulm as they don't deal with fitting kids for braces often and might not get the best fit for her.  The PT staff at Children's deal with it on a daily basis and will get her the best fit if she needs it.  So, we will wait until January for this.  They also brought Meghan special utensils to use to learn to start feeding herself since her range of motion in her wrists is very limited.

We also got confirmation on Meghan's surgery date, which will be January 2.  We will go to the Cities January 1 to do lab work.  We will stay in the cities over night.  We have to check in at the hospital at 6am and surgery will start at 8am.

We also ordered awareness silicone bracelets for her 2 conditions.  We are excited to get them and spread awareness!  I will post pictures of the bracelets when I get them in the mail, but that will probably be a couple weeks yet.

I found a place in Mankato that does screen printing and I got the artwork back today!  He originally told me it wouldn't be until next week.  He's really on top of things and is honored to do the project for us.  Once we finalize the artwork, we'll be able to take orders for tshirts and sweatshirts!  Things are starting to move along!

Meghan has been taking more steps lately!!  She went a few weeks without doing much walking, but has been walking more the last couple of days. 

It was a busy, but very successful day :)

Attempt to explain new diagnoses....

Since Chiari is a rare disorder and not very well heard of, many people have asked what Chiari (kee-ar-ee) Malformation and Syrinx is, which is what Meghan has recently been diagnosed with.  I will do my best to try to explain these two conditions.

There are 3 different types of Chiari Malformation.  Type 1 is the most common, and is what Meghan has.  Type 2 is more severe and is accompanied by Spina Bifida.  This type can not be operated on.  Type 3 is a very serious and very rare type and also can not be operated on.  Luckily, Meghan's type can be operated on and the hope is that her symptoms will go away after surgery.

Chiari malformation is a brain abnormality that causes the cerebellum, the part of the brain that controls coordination and muscle movement, to protrude into the space normally occupied by the spinal cord. Some children are born with the condition, and others develop it as they grow (Meghan's neurosurgeon believes Meghan was born with Chiari, along with Hydrocephalus).  Chiari malformation sometimes happens because the space at the back of the skull, where the cerebellum sits above the spine, is too small or is unusually shaped. These "cramped quarters" squeeze the cerebellum and even part of the brain stem, which controls the nerves in the face and neck, down through the foramen magnum (a funnel-like hole below the skull through which only the spinal cord usually passes).  The pressure caused by Chiari malformation on the cerebellum, brain stem, and spinal cord can keep them from working correctly. It can also block the movement of cerebral spinal fluid (CSF), the liquid that surrounds the brain and spinal cord and protects them from injury. When CSF doesn't flow properly, it can build up in the brain and spinal cord and block communication of the brain's messages to the body (In Meghan's case, she has total blockage of CSF flow.  This alone warrants surgery).   If a Chiari malformation is severe enough to block the flow of CSF or put significant pressure on the brain or spinal cord, a child's nervous system can be affected. Symptoms can include dizziness and problems with balance and coordination. This means a child might fall down a lot, walk unusually, have trouble grasping items, or have poor hand-eye coordination.
Other symptoms can include:

• neck or chest pain
• headaches that are brought on by coughing, sneezing, or laughing
• difficulty swallowing, which may cause gagging, choking, or vomiting
• difficulty speaking
• rapid eye movements or vision problems like light sensitivity or blurred vision
• hearing problems like a tinnitus (ear ringing) or hearing loss
• weakness, numbness, tingling, or other abnormal feelings in the arms and legs
• insomnia
• depression

Signs of Chiari malformation in babies include:

• irritability when being fed
• excessive drooling
• weak cry
• trouble gaining weight
• arm weakness
• developmental delays

Children with Chiari malformations might have other conditions that are associated with the abnormality, such as:

• Hydrocephalus: An excessive buildup of CSF in the brain that causes an enlarged skull and, if left untreated, can lead to brain damage, a loss in mental and physical abilities, and even death.  Meghan was diagnosed with this in April 2012 and had surgery to place a shunt in her head.
• Syringomyelia: A disorder in which a cavity called a syrinx develops in the spinal cord. This cavity usually fills with fluid and stretches the spinal cord, which can cause permanent nerve damage and paralysis.  Meghan also has this on her lower spinal cord.  The hope is that after her decompression surgery, that her syrinx will diminish.  If it does not, she will need another surgery a few years down the road to remove the syrinx.
• Scoliosis: A condition that causes a curvature of the spine. Scoliosis can be a complication of syringomyelia, which causes an imbalance in the strength of the muscles that hold the spine straight and upright.  Meghan does not have this........thank goodness!

Unfortunately, there is no cure for Chiari malformation. Most children who don't have symptoms won't require treatment.  When symptoms do occur, and are serious enough to warrant surgery, your neurosurgeon will decide if surgery is necessary.  If surgery is necessary, it is called decompression surgery.  The surgery will help to relieve pressure on the brain and spinal cord, restore the flow of CSF, and prevent additional damage. This surgery usually takes several hours and involves removing part of the skull and a portion of the spine's top vertebra.  Surgery isn't a cure but it can help to greatly improve the problems caused by a Chiari malformation. It does have drawbacks, though. Surgery can be painful and can put a child at risk for infection and other complications.  Many people believe once the decompression surgery is done, that they are cured.  That is not the case.  The hope is that the decompression surgery will improve Chiari symptoms.  However, it is a possibility of a person that has been decompressed to have the Chiari come back.  This is common in kids whose brains are still growing, or if a person is involved in a whip-lash  type accident.  The whip lash motion can trigger the Chiari and a person can experience the Chiari symtpoms again.  30-40% of people who have been decompressed report being symptom free after surgery.  Another 30-40% report their symptoms improved slightly and another 10-20% report no relief of symptoms after surgery.  We ofcourse are hoping for a completely successful surgery for Meghan and that NO other surgeries will be needed for her down the road for Chiari, or her syrinx.

MRI results

Well, Meghan's MRI was just two days ago.  It was a rough day.  She had been fighting a cold a few days before her MRI so we were concerned she wouldn't be able to have her MRI.  A day before her MRI, her cold disappeared, so we were happy with that.  Of course, as Meghan's luck would have it, we were 15 miles away from Childrens for her MRI and she vomited.  I thought foresure she would not be able to have her MRI.  But, luckily, she didnt have a fever and I told the nurse and ICU doctor that Meghan vomits when she gets over heated and I felt that is what happened this morning in the vehicle.  She got cleared for her MRI. The MRI took a little less than 1.5 hours.  She had images taken of her brain checking the Chiari Malformation, the spine checking the syrinx, and what's called a CINE (sin-e) MRI, which checks the flow of cerebral spinal fluid along the spinal cord up to the brain.  Fluid flows up the spinal cord, over the brain and back down the spinal cord with each heartbeat.  They are checking for any blockage of CSF fluid flow.  Meghan took a little longer to wake up from the sedative this time, but she did great!  Once she was awake for a while, we headed over to the Neurosurgeon - Dr. Petronio.  He is wonderful!  He asked us a bunch of questions, asked about her symptoms, asked about her development since the shunt was placed, and did a neuro evaluation of her.  Then he went over the MRI images.  He told us she definitely has Chiari Malformation.  Her brain herniation out of the skull pertrudes by 9 mm.  Doesn't sound like much, but it is a decent size herniation.  He then talked about the syrinx on her lower spine.  It was the same size from 3 months ago.  He then talked about the results from the CINE MRI.  She has complete blockage of CSF fluid.  Given her symptoms and that she has complete CSF blockage and a syrinx, it was determined that she needs to have decompression surgery within 6 months.  This is an invassive surgery.  The surgery will take 2-3 hours.  Meghan will be in PICU the first night, then will be in a regular room for another 4-5 nights.  The FULL recovery time at home is up to18 months, but she should be somewhat back to herself 3 weeks post op.  Anytime you are dealing with opening the brain, you need to allow your brain plenty of time to heal.  The advantage we have is her age, and kids her age usually bounce back faster than an adult.  We are working on scheduling a date, but right now, it is looking like the first or second week in January.  We hope for a successful surgery and that no further decompression surgeries will be necessary.  During her neuro exam, he mentioned that Meghan has Hypotonia in her ankles.  He defined them as being floppy, very low muscle tone.  She also has flat feet.  He talked to us about Meghan possibly needing braces.  He said that as she gets older, she will eventually walk on the inside of her feet if not corrected.  I will be talking to her PT and OT next Wednesday about this and to see if they think we need to take her to an orthopedist.

2 more weeks

EEK!  In just two more weeks, we will be on our way to Children's for Meghan's MRI.  I try very hard not to think about it, and to keep busy.  But, it's always in the back of my mind.  The waiting game is getting hard on Jeff as well.  We just have so many unanswered questions that we know will be answered soon.  I just keep telling my self that I believe in miracles, and I believe God will bless us with a miracle at her next MRI, and everything will be just fine.