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.

Incision Photos

As promised, here are the incision photos in order, with a short description of each one.  I have added a few lines of spacing to allow anyone who does not want to see these photos, to exit. :)

Food for Thought: We were instructed to wash it everyday until suture removal and were told at our NS follow up that they can always tell the patients who have followed these instructions. Suture removals are much easier and less painful if instructions are followed. It seemed to work for us.


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Post Op Day 2. Bandage removed approximately 44 hours after surgery.

 

 

 

 

 

Post op Day 5

 

 

 

 

 

 

Post Op day 6. We began to see some redness and slight puffiness appear at the very bottom of the incision.

 

 

 

 

 

 

Post Op day 8. Redness is increasing, but only around the sutures.  Watching it closely, but no drainage or excessive tenderness.  G is beginning to complain about itchiness.  She likes to have the water run over in the bath to help with the itchiness.

 

 

 

 

Post Op Day 10. Redness is very pronounced now, but still only around sutures and no drainage.

 

 

 

Post Op day 12. Redness is somewhat subsiding.

 

 

 

Post Op day 14.  Day of Suture Removal.  Confirmed with office that the redness was a local reaction that will improve now that sutures are out.

 

 

>photo<

 

 

Post Op Day 22. At about a week after suture removal, the puffiness and most of the redness is gone.  Incision line is still tender, but able to use a very soft brush with shampoo during the bath to remove the remaining scabs.

School Nurse Instructions

I want to provide the outline of our If/Then statements that I sent in to our school nurse.  I tried to be as detailed as possible when writing these, and included symptoms that G has never even had, just in case. This has been copied and pasted from a Word document, so I apologize if the formatting does not work with your interface. 

August 7, 2014

School X Nurse Office

Re: G DOB: xx/xx/xxxx
 

Upon returning to school, G may have residual symptoms given her recent Posterior Fossa Decompression procedure on July23rd due to her diagnosis of Chiari Type 1 Malformation. Below are a list of symptoms that she may have, and what I request happen if these complaints occur during the school year.

 

Headache with pain scale of 1-4	·         Lie down for 10 minutes to help alleviate symptoms. ·          If no relief, give 320mg Tylenol. ·         Call Parents if headache lasts longer than 30 minutes.
Headache with pain scale of 5-10	·         Give 320mg Tylenol ·         Advise to lie down ·         Call parents
Pain or Tinging in Extremities	·         Lie down for 10 minutes to help alleviate symptoms. ·         If no relief, call Parents
Dizziness	·         Lie down for 10 minutes to help alleviate symptoms. ·          If no relief, call Parents
Neck pain or stiffness	·         Give 320mg Tylenol ·         Advise to lie down ·         Call parents
Any swelling, pain or discharge at incision site	·         Call parents
Confusion, light headedness,	·         Call parents
Seizure, Dyspnea	·         Call 911 ·         Call parents
If she presents to the nurse for any reason	·         If call is not warranted, then please send a note home with details of visit.

 

Please use the following numbers, in this order to contact parents:

·          555-555-5555

 

If you do not reach us at the above numbers, please use numbers below:

·          555-555-5555

 

Post Op school tips and tricks

As the first week of school comes to an end, we have had a steep learning curve. I want to offer the tips we learned and to help you know what to think about before you send your kiddo back to school post-op. 

First, think about school bus riding.  This was a restriction I placed on G right away.  I dropped off and picked up each day this week to eliminate any extra bouncing and roughness.  I was also concerned with the elongated day if she were to ride the bus.

Secondly, be aware of their noise sensitivity.  We had thought about it in terms of home life, but didn't dawn on me to plan for the noisiness of school.  Fire and tornado drills were discussed at my school meeting prior to school.  But, HELLO, what about the lunch room, you know, the nosiest place in school, and possibly the planet?  I had to come visit the school after G's lunch on the first day because the elevated level of noise in the lunch room caused a level 6 headache. The headache resolved with rest and she was able to return to class.  Our long term solution is still being worked out. I bought a custom ear plug kit from Amazon (link here), and the mold itself worked well, but I don't think I have the skill to successfully produce usable ear plugs.  Once at school, she could not insert them into her ear, and neither could the nurse.  I have enough to try one more set, but if those do not work, we may need to go the professional route.

Thirdly, the bright sun during recess caused a headache for G during her first day.  This had not been a problem before school, but I have noticed that she has been closing her eyes on her way home due to the bright light.  I am not sure what to make of this.  Our resolution was to leave a pair of sunglasses at school for times she is outside. She is currently enjoying quiet recess inside with a buddy, so she is not routinely outdoors during the school day.

Fourth, The collared shirt, oh the collared shirt.  I am still running for mother of the year since I placed my newly decompressed 7yo in a nice collared shirt for her second day of school.  It didn't even cross my mind that the collar would rub the bottom portion of the incision.  So, back to school I went with a backup shirt.  As a side note, I obviously do not learn quickly, because the shirt I laid out for the last day school... You guessed it, another collared shirt.  I do wonder about my sanity sometimes.  Luckily G caught it. :)

Fifth, Be open minded about their desk.  G's school supplied a type of tilted area on her desk to elevate her work so she would not need to look down and cause a kink in her neck. After her first day, it was obvious that it was not elevated enough.  I sent in the laptop desk from Amazon (link here) that was working well at home. This allows it to be positioned at, pretty much, any angle and height required.  We are going to try this for about 5 school days, and if it continues to work as well as it does, then we will buy a second one for home. 

All in all, the first week has gone well.  I am super glad that it was a shortened week!  G was picked up early one day due to a level 6 headache with dizziness that was not resolving. But the other times I have been called to the school, we determined that she could return to class. The school nurse has been wonderful about contacting me each time G visits the nurse's office.  She is averaging 4 trips to the office and 1 dose of Tylenol per day.  Most headaches are relieved by resting and an occasional dose of Tylenol.

Questions from a 7 Year Old

During the last couple of days, G has had an uptick in anxiety.  She can see the calendar flying by like I can.  So, I had her recount every fear she has and we talked about each one. All were very normal fears for a 7 year old. 

· What if a mistake is made during surgery?

· What if I start throwing up?

· What if I wake up during surgery?

· What if they make you and daddy leave me? 

We discussed the likelihood of each one and what will be done to prevent them.  She knows she has a wonderful surgeon and the hospital is one of the top in the nation. Discussing her fears and the positives  made her feel much more at ease.  I'm sure we will have this same conversation many times over the next few days to help drive home the "chin up" mentality.

All we can do now is finish up a few projects, and concentrate on having some fun. Her current project is to decide which of her stuffed animals get to go.  She only wants to bring the ones that can be easily washed. You know, in case she throws up on them.  This made me smile, as this concern had not even hit my radar. 

Germaphobia

One thing I had not expect, was level of germaphobia that would consume me it gets closer to surgery.  I fear that G will get sick just before or after surgery and either cause a delay or complications post-op. As much as I do not want the 23rd to get here, I do want to get it over with.  If a delay became necessary, my level of insanity would become public knowledge. We are limiting her trips to places that are known to have the most germs. Further trips to the store and public restrooms will have to wait a month or so!

Earlier in the week, I put some of my germaphobia away and took her school shopping.  I am usually the parent who dreads the school supply list because it means the kiddos are going back to school.  Honestly, I think I am in the minority of parents...I want to hang out with the kids later in the evenings and not worry about alarm clocks. I know, I know, give me a few years and I will mark time until school starts. But this year, I was so glad that the stores had their school supplies out early.  If we had waited until after we returned, I don’t think G would have felt up for picking out her new backpack.  So, we decided to do it in advance of the surgery date.  Just one more thing marked off the to-do list!

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. 

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...

The Diagnosis

A popular question is "How was G diagnosed?"  Our story, like many others, wasn't cut and dry. Our journey began on the evening of Christmas Day, 2013.  G was sent to gather her coat before heading home, but didn't return for about 10 minutes. Upon returning, I asked her what took her so long and her answer made my heart sink. "Mom, my head started hurting so bad, I had to lay down on the floor until it quit."  During the 5 minute ride home, I must have set a record for the number of questions asked.  My neuro curriculum from nursing school kicked in and I tried to ask each question in non leading, kid friendly terms.  Each of her answers was a kick in the gut.  How could I have not known about the severe headaches, ear ringing, limb tingling/numbness and dizziness that has been going on for, who knows, how long.   

I came to the conclusion that she needed to be seen ASAP.  So, I watched my clock roll over at 8 am, the day after Christmas and called as soon as the office phones turned on.  G and our pediatrician have had a special bond since our emergency C-Section 7 years ago.  Actually, G has her wrapped around her finger. :)  We are blessed with one of the best pediatricians in our state.  She got us in the same day of calling.  After G's exam and discussing the symptoms that were just discovered, it was apparent that an MRI was needed.  Unknown to me at the time, our pediatrician had a neighbor who was diagnosed with Chiari at age 14.  She secretly suspected this possibility with G's symptoms, and thus pushed for an MRI.

When the MRI was ordered, she specifically asked the radiologist to address whether a Chiari Malformation was present.  She wanted a negative or positive narrative of this particular finding.  When she received the original report, there was no mention of Chiari.  Neither affirming or denying the presence.  This prompted her to send the imaging off to a neuro-radiologist at the closest children's hospital. The result was the finding of a 21mm herniation and positive for a Chiari Malformation. We are baffled to this day that such a severe herniation was left off of the original report by the original radiologist.  Professionally speaking, it is unacceptable. But, I digress. 

If we had not had our wonderful pediatrician, we may not have had the MRI so soon, and the Chiari may not have been caught due to the Radiologis'st flub.  I am so thankful for the circumstances to have brought us to a quick diagnosis.  We are much luckier than some, who suffer for years without a diagnosis. 

Our Intro

So, why am I picking now to begin our journey's blog?  I am hoping to help other families go through this wild and scary ride called Chiari decompression surgery.  My goal is to chronicle our ups and downs in the next few months. I'm sure there will be lots of both!  I have found a handful of blogs out there that have helped me know what to expect in the coming few months, and I figure the more out there, the better.  Each kiddo is different, and not one experience is like another, but I have found seeing some real life experiences out there is very helpful.

Let's start with who you are reading about.  My husband and I grew up and currently live in the Midwest.  We have 2 wonderful kiddos. W is our rambunctious, Star Wars loving, 4 year old son, and G is our good natured, rule oriented 7 year old "Chiarian" daughter. We are as normal of a family as they come. Two kids, two dogs, a house and a very supportive family. All of which we are so very thankful for.

G's surgery is scheduled for later this month, and it feels like the date on the calendar is flying at us at warp speed. So much to do and prepare for, so little time.

So, you have found this blog, and thank you for reading.  Given you are here, I assume you already know what Chiari Malformation is, but just in case you do not, below is a quick overview from the site ConquerChiari.org's Awareness Brochure" The full brochure can be found by clicking here:

"Chiari Malformations are a set of neurological conditions which afflict approximately 1 in 1,000 people (300,000 people in the US), causing debilitating headaches, neck pain, weakness and numbness in the limbs, balance problems, visual disturbances, and a host of other symptoms. Chiari Malformations are located at the base of the skull. Part of the brain, the cerebellum, descends out of the skull into the spinal area. This results in compression of parts of the brain and spinal cord, and disrupts the normal flow of cerebrospinal fluid (a clear fluid which bathes the brain and spinal cord). If left untreated, Chiari can lead to the development of a fluid-filled cyst in the spine (syringomyelia) and cause permanent nerve damage and paralysis. Chiari is a complex disease which is poorly understood, and being diagnosed is an overwhelming experience. Patients are thrown into a whirlwind of medical jargon, tests, and surgery, all the while not knowing how things will turn out in the end."

Thanks for stopping by, and happy reading.  Feel free to drop me a line or ask a question. 
-SuperChiariMom (SCM)