6 months and Going Strong!

Well, our 6 month "zipperversary" has official come and gone.  I thought it was only appropriate to write an update since I totally spaced writing an update after G's follow up MRI in October.

Quick overview of G's 3 month follow up with MRI from October:

Scheduling this appointment was a bit of a hassle.  I was jockeyed between a few different people in the office and seemed a little disjointed.  I later found out they had just hired new office staff, and miscommunication may have caused the majority of the issues.  Finally, G was given an appointment and subsequent MRI almost 3 months to the day of surgery.  I was very disappointed that we were not going to be able to see Dr. Mangano, but his Physician Assistant was very very good.  She was able to pull up the before and after MRI scans and the difference was quite impressionable.  However, even after surgery, G still has a fairly severe herniation, but her CSF flow is better.  Remember, her herniation was 21mm upon diagnosis, so even with the best decompression results, it would only decrease it, not resolve it altogether. Her CSF flow is not perfect by any means, but much, much better.  There is less compression of the brain stem as well, which is very good news.

All in all, the procedure worked as it should have. It have the cerebellum enough time to heal, and everything seems to look as text book as it can.  We were very relieved to hear this news. 

Below, you can see the before and after MRI scans.  I have highlighted the area that has the most noticeable difference.  It is truly amazing how much of a difference that a few millimeters will make when it comes to brain and CSF compression!

So, how is she doing?  In one word... Great!  Her recovery has been text book, and honestly there are now days that I forget anything even happened.  And I believe she has these days too.  Her incision has healed wonderfully, and her hair is a good 2-3 inches long around the site.  I have to look for scar as it is almost completely obscured by the hair.

Symptom update: She has improved immensely in the last 3 months.  Numbness, tingling, and weakness have continued to be resolved, and have not reappeared.Her headaches gradually have improved, and are down to 1 every 1-2 weeks.  This is such an improvement I cannot begin to express how thrilled we are for this progress!  The main symptoms she continues to have is the ringing in her ears.  As I mentioned in previous posts, we did not expect this to resolve, but was hopeful after she woke up in ICU reporting there was no ringing.  She does mention that the ringing continues to change. Currently, it is louder, and a lower buzzing that changes frequency throughout the day. At times, the "booming" still occurs, but not as often.  She has lived with this type of sound all of her life, and does not seem to be bothered by it.  For this, I am grateful.

Dr. Mangano has left most of her restrictions up to us.  He heavily recommended no roller coasters, trampolines, or contact sports.  These can cause whiplash type injuries that may trigger additional symptoms.  I do have to share a quick story about this...

Well into G's first semester of school, all students who behaved in accordance of the school's behavior policy were rewarded with a surprise party one day after lunch. I had received the email alerting parents to this party, however they did not elaborate what would be included in this party.  As I dropped G off at school, I told her that she will have a surprise after lunch, and to have fun.  Unknown to me, a bouncy house had been rented for the party, and was the focus of the surprise. G, thinking that I knew about it, had fun bouncing around.  After school, she said she couldn't believe that I was OK with her participating in the surprise bouncy house.  To say the least, I was shocked to hear of it.  I asked her how she felt afterward, and she mentioned that it did give her an 8 headache for about an hour afterward, but recovered with a dose of ibuprofen.  So, lesson learned- Even though your 7 year old knows what he or she is supposed to do, they will do it anyway, especially when they think they have your blessing. 

Let's talk restrictions for a moment.  I am very glad that G is rule oriented, and that she will, more than likely, obey all restrictions placed on her.  She understands the need to be restricted and the relapse of symptoms that could occur if she were not to follow them.  I cannot imagine the frustration and concern parents have when they have children who are too young to understand, or those who choose not to obey them.  We have chosen the non-helicopter parent mentality, but have fully educated G on the consequences of her future choices.  Restrictions we have placed on her follow Dr. Mangano's suggetions.  No roller coaster, or jerky rides, no rowdy or contact sports, no bouncy houses or trampolines.  Thankfully, she has taken a liking to Chess Club and Theater, so she has extracurricular activities without the risk of much head trauma. Our true test will come later this year when we head to an amusement park that will tempt her.  I am curious how she will handle this, but we intend to do many other activities and lower risk rides.

School has been going very  well.  Everyone at the school has continued to be very supportive. Up to very recently, G has been using the custom ear plugs during lunch. Now, noise is much less of a concern for her.  We continue to drop her off and pick her up from school, and will more than likely continue to do so through this year.  We will begin the bus routine at the start of next year.  She is eager to get back on the bus, so I will probably relent in the fall. Her school nurse visits have pretty much diminished with an average of 2 or so visits per month.  These visits are typically due to a headache being brought on by an external source, such as bumping heads with someone, or looking down for extended periods.  She began PE in November, and she seems to be doing well.  The PE instructor does a wonderful job observing the students and pulling her in a direction with less risk.  I have no worries about this at all.

We return to Cincinnati  in July for her 1 year appointment.  I was clear that I wanted to see Dr. Mangano during this follow up, and they were happy to oblige. We can only hope and stay positive that the MRI continues to show improvement or at least static information.  With her being so young, and still has a ton of growing to do, we do not know how that will affect her Chiari. Only time will tell.

I have to give another shout to all of the support our family has had in the past year.  What a crazy, whirlwind year, and it was bareable due to the wonderful support from everyone.  People continue to text, email and stop by and ask about G.  And I am always thrilled to tell them how well she is doing.

For all the parents out there reading these posts, know that you are constantly on our family's mind.  We were there, with the same deer vs headlight eyes and I only wish I had a blog similar to this to read.  I wanted to hear the good, positive stories, and to follow along with a family who has gone through the journey that we were only starting.  Lord only knows, you hear the scary, unfortunate stories everywhere.  Do not get me wrong, they are important to tell and to hear as well.  But along with everything else in the world, moderation is key.  If you hang heavy on one side, it could set you up for failure no matter the outcome. Arm yourself with knowledge of the good stories as well as the bad, and you can't go wrong.

Anesthesiology Consult

Party update:  All went went fabulously. G was so very surprised and had a great time hanging out with her friends.  It also gave DH and myself some social time that was very good therapy.  We have now filled one of our tubs with donations to take to the Ronald McDonald House! 

Tomorrow marks the last pre-op related appointment.  Our anesthesia consult is set for tomorrow.  We opted for a phone consult since it is a 3 hour drive and G does not need to be present.  What is an anesthesia consult?  I am not 100% clear on this, so being the newly manifested OCD mom that I am, I call on a good anesthesiologist friend of mine.  We have known each other all of our lives, and I trust her more than most in the healthcare world.  We spent about 40 minutes on the phone discussing G’s current state and what the consult will probably consist of.  She gave me a line of questions that I had not considered.  A few examples are: If G will be put to sleep away from us, can they use an oral sedative, to help her with anxiety? Who will be the anesthesiologist?  Will there be any Residents/Fellows/CRNA, etc in the room?  If yes, what are their experience levels? Do Neuro cases use specific anesthesiologists, or is there a general pool of anesthesiologists? Do they give pain meds prophylactically, before she wakes up?  Or do they wait until she complains of pain? Is it typical to use a pain pump for IV meds in the days after surgery?

Many anesthesiology questions were answered during the pre-op tour, so that was helpful as well.  However, it was mentioned that the Neuro cases are treated a bit differently, so I am curious how anesthesiology fits into this difference.  I suppose this will be answered tomorrow.

Pre Op Party and RMH Wish List Fullfillment Drive

We are marking the “One Week to Go” date with a surprise party for G.  We have invited all of her friends to the local mini golf course for pizza and fun.  It’s an evening dedicated to getting her mind off of surgery and being able to run around with friends before surgery.  She has no idea this is being planned and she will be super excited when she sees 15 of her closest friends!  Most of whom she hasn’t seen since school was out in May.

G is such a giving person.  She asked me last weekend if we could take some donated goods to the Ronald McDonald House before her surgery date.  So, we have begun a “Wish List” fulfillment drive at my work and among our family. I also included donation information and the official wish list info on the invitation for the surprise party.  I hope to be able to drop a couple tubs worth of needed items on our way down, the day before surgery next week.  We have very generous friends and co-workers, so I don’t have much doubt we will meet this goal.

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. 

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.