Pre-Op Tour *Highly Recommended

The short run to the surgery date is here.  Two weeks to go and it seems the to do list continues to grow.  G is handling it well.  There have been a few bouts of high anxiety, and then she remembers why we are choosing to do the procedure.  Honestly, her biggest fear is the IV.  The initial IV will be placed after she goes to sleep, but she is still a bit anxious about having the IV tube in her vein.  I guess I would be the same way at that age as well. 

It has been a week since our pre-op tour at Cincinnati Children’s Hospital. If your hospital offers this type of class/tour, I would highly recommend it.  We drove 3 hours just for the tour, and it was well worth it to put G at ease, as well as DH and me. 

Currently, at Cincinnati Children, they offer this tour on specific weeknights and Saturday mornings.  We scheduled ours on a weeknight since it is a 3 hour drive. We would much rather drive home a bit late, then get up super early on a Saturday.  We scheduled our day so we would arrive a few hours early and catch a short day at the Zoo.  The hospital provides free and discounted tickets to some area attractions, so we picked up a couple for the zoo.  It is located blocks away from the hospital, so it was an easy commute to and from the campus.  Both kiddos enjoyed themselves and it was a good distraction of the surgery stress. 

We arrived at the hospital campus around 6pm and were greeted by the Child Life Specialist who would be taking us on the tour. These folks are wonderful!  Their only job is to help the kids feel at home at the hospital.  They will bring crafts, movies, games etc to the room, or escort the kids to the playroom if they are able. 

Our first tour stop was the “Getting Ready Room” where she will change into her gown, get vitals, and meet with the anesthesiologist.  From there, we were taken to the Induction room.  This is where G will be given “sleepy air” to go to sleep, and then they will insert the IV after she has drifted off.  This is where you can tell it’s a kid’s place...  The “sleepy air” is flavored.  G was able to smell the scents offered and think about which one she wants.  Her choice?  A combo of two… Cherry Bubblegum.  I wish I had been given that choice the last time I had surgery!

Attached to the Induction room by a sliding door, is the operating room.  G was awe struck at the number of lights and machines there were in the room.  It didn’t scare her, but rather made her feel good that she will know what to expect.

The recovery area is next.  They have it decorated in a Sea Shore theme.  We picked out a few focal points for her to remember, so when she wakes up, she will know where she is.  Coming out of anesthesia can be a little scary, so I am curious if this will work. The bright yellow sun found in each room and the Fish curtains were where we called her attention to.   

Then it was up a few floors to the ICU.  G will spend 1-2 days there directly after surgery to ensure everything stabilizes.  We were able to meet a few of the nurses that will be around that day.  We confirmed there is a limited number of visiting hours in the ICU, but not for parents.  Parents are encouraged to stay all day and night.  (I was going to stay even if they had advised otherwise).  Cincinnati Children’s has a nice ICU parent’s lounge with a mini kitchen and lockers. They provide free laundry facilities and a very limited number of sleep rooms.  Within the ICU room itself, there is a pull out, single chair/bed. I have to be honest, it does not look comfortable at all, but we will see.

The Neuro/Trauma unit is the floor where decompressed kiddos end up.  This floor has a smaller nurse to patient ratio given some of the complications these kids can have.  It was a busy floor with only a handful of rooms open. Again, there is a single chair/bed in the room for mom and dad.  Unfortunately we were told one pull out chair per room is the maximum.  Since neither of us want to leave her side, I’m not sure how to make this work, but I’m sure we will figure it out. 

The very last stop was the play room.  It’s down a few floors from the Neuro unit, but not far.  It is stocked with movies, paints, puzzles, crafts and lots of toys. Plus there is always a Child Life Specialist in the room to help with the activities.

The entire tour took about 90 minutes.  G had tons of questions, so ours may have been a tad longer than normal.

To-Do LIst

There are so many things to do to prepare G, DH and myself for decompression. Given the prolonged recovery, I want to get her in to the dentist, eye doc, and all the other normal follow up visits she needs for the fall. 

The rest of the time will be spent in pre-op bloodwork, pre-op exams, one more MRI, Pre-Op Surgical Class/Tour, FMLA Red Tape, Packing, Getting the house in order for our return, making Ronald McDonald House/Hotel reservations. Goodness Gracious, I'm sure there is a ton of other things that I'm not listing!

Pre-op bloodwork is complete.  I couldn't sleep the night before because G freaks out with her flu shot.  This would be a disaster, I just knew it.  Once we get to our local pediatric floor they give me the wonderful news that they have some numbing cream they can use at the draw site.  It worked wonderfully.  G was still a nervous wreck, but the nurses were great and were very patient with her.  I think playing one of her favorite movies, Frozen, in the background helped tremendously too. 

Now, I plan to dive into the FMLA paperwork and process. Wish me luck. -SCM

More symptoms- Decision Time

We took a 2 week vacation to New York and then to Indianapolis the end of May/early June.  During this time G was out of school for the summer. Her headaches got quite a bit worse in both frequency and severity.  We have begun speaking in numbers.  G has a good understanding of the 0-10 pain scale and can tell me at the end of the day how many headaches and the pain scale numbers.  A typical school day would be "three fours".  Or, 3 headaches with a severity of 4 on the pain scale. Since school has been out and more activity has ensued, it has increased to seven-ten headaches a day with a severity of four-ten on the pain scale.  It has been an obvious difference that has begun affecting daily life.

She has now begun complaining of constant tingling in both arms with no relief and a few instances of losing balance/loss of control of her eye muscles. Given these new symptoms, we made an appointment with our Cincinnati doc in mid-June.  We decided then to pursue decompression surgery.  He placed us on a wait list, as we would love to get in well before school, so G can begin school on the first day.  We know she will be returning part time, but no one wants to miss the first day of school, right?  Especially social butterflies such as G.

We received a call recently advising of an opening on July 23rd.  And now, the clock and calendar pages begin flying by. Lots of stuff to do and very very little time. 

FEES Swallow Study- Epic Fail

One thing that was mentioned during our long appointment in Cincinnati was the fact that swallowing difficulties and oral defensiveness is common in Chiari kiddos. G has had both since she was an infant.  She was on baby food consistency until she was almost 18 months old due to gagging, choking and vomiting.  Even today, at 7, she knows what she can eat and what will make her gag and choke. This prompted yet another referral for a FEES test.  It is Fiber Optic scope that is inserted through the nose and records the vocal cords during swallowing.  This allows a good view of how this part of the anatomy is working, and whether there is concern if a need for intubation arises.  I.e., If surgery is needed, the anesthesiologist would know about anatomy anomalies.  

This test takes a ton of personnel coordination. An ENT doc performs the test, while a radiologist and radiology tech runs the computer.  Then there is a team of nurses who helps during the test.  So, it took weeks to get it coordinated.  We were in the office by the end of May to have the procedure. We brought a variety of foods we knew G has problems with.  G's anxiety was under control (somewhat) and she was ready to get it over with.  The ENT doc said that the scope would be in less than 1 minute if all goes well.

That was good news, but that's where the good news stopped. The scope began to be inserted, and the software locked up.  Thus, preventing the doc to know how far she had inserted the scope.  This caused some pain and major anxiety for G... and for mom and dad.  They could not get the software to work, and thus resorted to a "Mirror Test".  This test is not recorded but is a visual observance of the vocal cords using a bright light and a mirror by the ENT doc.

All turned out ok, and no anatomy anomalies were found.  However, she was diagnosed with moderate to severe gastric reflux during this exam and thus is being now followed by an ENT and a Gastro specialist.

Seriously, can we see any more physicians in less than 6 months?  Her Medical Care Team is getting pretty extensive. 

-SCM

Chicago Geneticist Referral

So many referrals, so little time. The geneticist located in Chicago was on a 6 month wait list.  However, our Neurosurgeon was adamant on G being seen a lot sooner.  Our Pediatrician and the Neurosurgeon both called to plead our case and they finally placed us on an expedited wait list.  We were able to be seen the first week of May, which was so much sooner than we expected.  Our appointment lasted close to 3 hours.  He does a full exam, then incorporates an hour of physical therapy evaluation and then comes back in to discuss his findings.  Again, it was a wonderful experience in his office.  They are all a bit old school, with paper charts and such, but he definitely knows his stuff. 

We were able to leave with a therapy plan to help build up muscle tone before surgery incase we decide to go that route.

Referral #3-Cincinnati

The whirlwind continued in March as we were able to get in to see the neurosurgeon in Cincinnati only a few weeks after our Chicago trip. After a trip to University of Chicago, the commute to Cincinnati Children's seemed like a walk in the park!  Parking was only steps away from the check in desk and they validate parking!  None of this truly mattered to us since we only care about the care G will receive, but was a nice change from Chicago.

Our staff experience at Cincinnati was similar to Chicago.  Everyone was so very helpful and kind.  They truly understood we were deer in headlights and needed a ton of direction.  I have found that even though I have 10 years of healthcare background, most of my clinical knowledge goes down the drain when it comes to the care of my own child.  Both facilities were great about recognizing this and talking to us at a level that both DH and I can understand.

I was so very impressed as soon as I walked into the waiting area.  I saw that they had a full bulletin board with a ton of information on Chiari Malformation.  I knew we were in a good spot.  We arrived about 25 minutes early, and were called back within 5 minutes of registering. The lack of wait was amazing to me.  I figured it was a fluke, but when we returned in June, it was the same wait time.  They have something figured out!

During our appointment, we met with both the physician and his Nurse Practitioner.  Both were extremely well versed in Chiari.  Being the type of person I am, I had a book full of questions. Some of the questions were used to see HOW they were answered, not WHAT information they were answered with. The impression I got from this office, is that they are conservative, and do not operate unless symptoms prompt them to do so.  Their surgical incision approach is somewhat more conservative as well. This particular doc makes a smaller dura incision, but in a 'Y' formation.  This allows for more expansion, with less cutting.

We met for approximately an hour with both the MD and NP in the room.  G's exam didn't seem to change much since the Chicago appointment which was good.  A long discussion revolving around the connective tissue disorder occurred.  This doc's take was that there was some precautions he would take if there was a chance G had it.  He requested that we see a geneticist in Chicago who specializes in the connective tissue disorder before deciding whether to perform surgery.

We left with a great feeling about this office.  IF we decide to do decompression surgery, Cincinnati is the front runner as of late March. 

Neuro Referral #1 and #2

As with most parents of newly diagnosed Chiarians, we jumped into the interwebs to find out all we can on the subject.  Some was informative, most was terrifying, very little was comforting. This is when we found the websites Conquer Chiari, Ben's Friends, Chiari Support and ASAP. (See links to the left)  There are so many helpful folks out there to help you along the way.  It is especially nice when you meet someone in your neck of the woods and can help identify the physicians and facilities where they have had good experiences.

Our first neurosurgeon was a blind referral from our pediatrician office. The physician was a very nice guy, who treated G very well.  He recommended surgery within 2 months given the severity of the herniation.  His main concern was further injury if G was to have a whiplash accident.  However, he did not meet our criteria.  The office only performs a dozen or so Chiari Decompression surgeries per year.  This was not enough for me to feel comfortable, so we decided to move on to some recommended providers from the moms I met on the Chiari websites.

Our pediatrician was very supportive and referred to us to the neurosurgeons we requested.  We decided to pursue referrals to two of the top Chiari surgeons in the Midwest.  One was located in Chicago and the other in Cincinnati.  Both were hard to get into, and the Cincinnati doc required a spinal MRI before our consult.  So, we decided to get the additional MRI before seeing each doc so they both had the same imaging to review.  The spine MRI showed no syrinx, and we were relieved. 

It was early March before we could get into the doc in Chicago.  Let me tell you about University of Chicago...It. Is. Huge.  Parking is valet only due to the pure number of patients being seen at once.  Getting there is quite a chore, and then navigating the buildings are a bit tough.  Once we made it to the neurosurgery department, the staff was AMAZING.  Front office, clinical staff and the physician were all great.  What really struck me about the physician, was the fact he only spoke to us once in the first 10 minutes.  He walked in, looked at DH and me and said "Neither of you look 7 years old".  Then proceeded to talk to us very indirectly through G.  He asked all of his questions directly to G and only glanced at us to ensure we agreed with her answer. 

Then, he came and sat down next to us to discuss his thoughts.  His suggestion was to adapt a few items at her school desk to see if it improved her symptoms, then return in June for a follow up.  He was not keen on surgery since she was not exhibiting one of his 3 prerequisite symptoms.  (Sleep Apnea, drop attacks, synrix present) We were so very relieved to hear this since we had been told G would need surgery. We still had some concern given the severity of her herniation, and decided to get one more opinion.

An important side note, is that we have a strong family history of a connective tissue disorder that is sometimes correlated with Chiari.  When I specifically asked about this correlation, and how it pertains to how surgery is handled, I was met with a nonchalant answer.  He had no concern that this disorder ran in the family, and he advised that there would be no difference in surgery or outcome even if G has the condition.  This struck me as wrong and raised the "mom's inner voice" flag.  We left feeling good since we had decided no surgery at this time, yet conflicted to due the known severity of her condition and the possible underlying connective tissue disorder. 

On to the next provider we go...