Wow!  It has been quite a while since we posted an update.  I really fell behind, but will update as much as I can.  Sorry for the long post!

In June, we flew to Maryland to spend some time with Jenn, Meghan's IR4 runner, and her family.  We stayed with Jenn's in-laws, who graciously opened their home to our family.  We spent our first full day in Maryland running the Maryland Special Olympics Torch Run, which was such an honor to be a part of and was incredibly moving. There was a program after the run where the Special Olympians were honored and they even involved Meghan!  Jenn lined all of this up to have Meghan included in the run and we will forever remember that day!  They even talked about how Jenn and Meghan were matched through I Run 4.  The remainder of our time in MD was spent by eating some (a lot!) of crab, had snowballs, saw some of the beautiful Maryland scenery and celebrated a birthday party for Meghan on the last day with Jenn's extended family.  It was great to get away and spend some more time with the Saulsbury's as they are such a blessing to our family!

Meghan had a routine abdominal ultra sound in July to check on her dilated kidneys.  Once again, there was more fluid and a new finding of urine blockage, so that puts her Hydronephrosis at Grade 2.  She will have another ultra sound this Friday.  If more dilation is seen, we will need to consult her Urologist, which we were discharged from a year ago.  Her AFP level remains normal, so that's great!  AFP is a tumor marker blood test.

Meghan started her second year of preschool in September.  This year she is in an inclusive class which consists of 10 typical kids and 6 special needs kids in the same class.  The staff consists of 2 teachers (1 of the teachers focuses on special needs kids) and 2 paras.  Meghan has a more one on one Para with her this year due to her size and being in a larger, faster paced class.  Even though shes little, she doesn't let that stop her and she makes her way through the crowd and let's her presence be known! So proud of her!  She is doing well in school.  We had conferences a month ago and got a great report!  She knows all of her shapes, colors, and numbers 1-10 and she can count to 18, so that's wonderful!  A few areas that are tougher for her are the alphabet, writing, using writing utensils and maneuvering a scissor.  She only recognizes 6 letters and is unable to write any letters or numbers.  But, while these are proving to be more difficult for her, we know that it will all come to her in Meghans time.  She is doing well in speech and is being pushed very hard!  Speech continues to be an area she has to work extra hard at.  We have discovered that her right side of her tongue is thicker then the left side and she's rolling her right side up when she talks.  This will just take time for her to figure out how to get words out by talking this way.  There is nothing we can do to correct it, as it is how she is genetically made.  We are all working with her on speaking slower.  When she talks, she doesn't necessarily talk fast, but in order for her to get her words out in an understandable way, she must talk slow.  Otherwise, her words all run together and gets hard to understand; especially if she's telling a story.  OT/PT is pushing her hard as well.  She uses a treadmill at school so she can work on making larger strides and walking further distances without tiring.  She is up to walking for 4 minutes on the treadmill.  Balancing on one leg, hopping/jumping, throwing are a couple things we are working on.  She also uses a small trampoline at school to help her learn to repeatedly jump.  We will hold her back another year to start Kindergarten, so she will do preschool one more year after this school year.

We saw the Endocrinologist a couple months ago. Meghan gained 9 ounces (if I remember right) from the previous appointment. She decided to give Meghan until January and will decide at that time if we will continue growth hormone shots, or take her off of the shots for a year and then retry. She had blood work done at this appointment, which showed low IGF levels, so the GH dosage was increased again. We discussed Meghan's stomach issues and agreed that the best plan for Meghan is to have strict set meal times, portion sizes and keep food out of sight. This is NOT to be mean to Meghan at all, but rather keep her mind focused off of food when it isn't time to eat. While we understand that people want to "help her out" by giving her food/treats, this is opposite of what should be done. It takes a lot of patience and effort on us as her parents to work with Meghan on when it's time to eat and when it's not time to eat and to keep her mind focused off of food when it isn't a meal time. For Meghan, it's best to keep food out of her sight...simply because if it's out of sight, it's out of mind. This is hard for us as parents to do, as parents should never have to hide food from a child, but with her obsession and inability to stop herself from eating, this is what we need to do. Our ultimate goal is to help Meghan by teaching her about how our stomach feels when we eat too much. The biggest misconception is that she is little because she is under fed. Reality is, she is little because her body produces very little growth hormone, and is medically considered growth hormone deficient. We give her shots to give her body the correct amount to growth hormone it needs to help her and her organs grow. We monitor her food intake so she doesn't over eat; as she literally does not know when to stop herself from eating (her inability to feel full is most likely linked to her undiagnosed genetic condition). Over eating can cause your stomach to burst, which can be deadly. Meghans stomach is super small (roughly the size of an 18 month old) so it doesn't take much. Trust me, we have seen her over-distended belly when she over eats and it isn't pretty and it sure isn't comfortable at all for her. All we can do is to keep working with her on this!

She recently had her yearly MRI.  She did great!  The older she gets, the harder these appointments are.  She knows whats going on.  She hates being poked and prodded.  But, she tolerates it all very well.  She LOVES helping the nurses and doctors out while they are doing her vitals.  And I love that they all let her help!  She just might be a nurse or doctor some day!  There isn't much change from last year's MRI.  The size of her ventricles are where they should be and her shunt is working well!  Her shunt drains CSF from her ventricles about every 4-5 seconds.  Her Chiari is stable at this point.  The space posteriorly to her brain stem is becoming crowded.  CSF flow is adequate for now, but could pose to be an issue down the road if the crowding continues.  We will continue to watch that.  One thing we have noticed in the last couple months is the chocking/gagging is back when Meghan eats or drinks.  This went away after the decompression surgery, but has recently returned.  For now, we will be keeping an eye on it and if it becomes worse, we will be talking to her Neuro.  The cysts on her spine have not increased in size which is good news considering last year there was mention of placing a shunt in her spine to attempt to drain the cysts.  Since the cysts didn't increase in size, this is not needed at this point which is great news!!!  She is being closely monitored for scoliosis.  We have known for a couple of years that the liklihood of Meghan developing scoliosis at some point is high, but for now she is in the clear.  The odds are stacked against her due to her leg length difference, the cysts on her spine and the fact that her hips sit uneven.  All of this puts a lot of pressure on her spine.  We love, love, love, Meghan's Neuro!  He listens to us and asks for our input.  Not every doctor/specialist does that, so when you have one that does, you appreciate it.  He is looking into a genetic condition called Macrocephaly Capillary Malformation, M-CM for short.  He hasn't had any patients with this condition, so he wants to do his research and talk to Meghan's Geneticist about it.  MCM is a genetic condition which includes every single condition that Meghan has.  Every. Single. One.  If this is the genetic condition that Meghan has, it gives us our answer.  It doesn't change anything for her.  This is a sporadic mutation that happens during development in the womb.  This condition can not be passed down, or inherited.  It is not a progressive, nor is it a regressive condition (meaning those with MCM do not worsen through time and they do not lose skills they have already acquired), so that is good news.  People with MCM have Macrocephaly and can also have Hydrocephalus, Chiari and some can develop Syringomyelia.  Meghan has all of those.  Other conditions that those with MCM have are: fused toes, hypotonia, hemihypertrophy, limb length difference, seizures, some have growth issues and food obsession.  The only thing that Meghan doesn't have are vascular anomalies.  But, her mysterious pustular rash could fall into that category.  I feel like this is our answer and I pray that this is the answer that we've been searching for.  We will not give up, as Meghan deserves our best fight for her.  We are proud and honored to do that for her.

Overall, Meghan is doing excellent and is having fun doing 4 year old things.  She keeps us in line and she thinks she's a Princess.  She loves to sing and dance.  She still loves Dora and Mickey Mouse.  She's a little sweetheart who steals the hearts of everyone she meets! 

 

Meghan had a couple appointments yesterday.  First we met with her Geneticist.  Meghan's regular Geneticist moved out of state, so this was the first time we met with her new Geneticist.  We were pretty impressed with him and I loved that he got to her level and joked with her throughout the appointment.  It made her feel at ease.  To sum up the appointment, he is putting Meghan in the category of having an undiagnosed genetic condition.  He feels very strongly that she has a genetic condition that can tie most, or all, of her conditions together.  However, it could take years to find our answer.  She's had some genetic testing done already, which has come back with no answers so far.  But, there are other tests we can do to see if we can find our answer.  She's been tested already for Beckwith Wiedeman syndrome, which came back 80% negative.  He said there are a handful of people whose test comes back as 80% negative, but end up having BWS.  So, our first step is to completely rule out BWS, which will be done with a blood test once insurance approves the testing.  If that comes back okay, our next step is to have a fibroblast cell test done, which will be done using the cells from the skin biopsy she had done in November.  The lab that did the test on her skin biopsy froze some cells, and if we end up needing to do the fibroblast, the lab will grow new cells from the biopsy and the test will be done on the new cells.  If, after we don't have answers from that, we will have genome sequencing done.  There are 22,000 genes in our bodies and this test looks at every single gene.  However, this test is extremely expensive, so it could require the Geneticist to write a letter on behalf of Meghan as to why the test is needed, which he is on board to do.  If all of this comes back without answers, we will re-submit all of the bloodwork/cells in 5-10 years.  Science is always evolving and even if we don't get answers in the next year or two, 10 years down the road there could be a test available which could give us our answer.  He also feels that Meghan's lack of growth is probably due to her undiagnosed genetic condition, especially since growth hormone shots aren't helping her grow adequately.

Then we saw the Endocrinologist.  She agrees with the Geneticist that her lack of growth is most likely due to her unknown condition.  We just don't know how to treat her growth because we don't know what the cause is from.  She would've expected to see much more growth by now.  3 months ago Meghan was at -3.79 on the growth chart.  At yesterday's appointment, she was -4.07% behind.  She increased the GH dosage and we are going to give it another 4 months.  If in another 4 months, there still isn't adqueate growth or catch up growth, we will discontinue GH shots.  We can always try again at a later date.  We all agreed that if it isn't working, there is no need to continue giving her the shots.  She did enroll Meghan in a study that will watch her growth and will follow her on what works and what doesn't work on her.  We discussed a problem that we are running into with Meghan lately.  We have been trying the "eat what you want" approach with her.  However, this has not been a good thing at all.  Meg will over eat and will be up all night long with bad stomach pains/nausea.  Meghan just doesn't have the ability to stop eating when she is full, and over eating could cause her stomach to burst open, which would be extremely devastating.  So, we are to limit her portions so she doesn't over eat, as well as have strict set meal times, which we already have.  We are learning as we go with her food.

She was due for blood draws yesterday, which resulted in 3 failed attempts.  The nurse tried taking blood from each arm and was unsuccessful in getting any blood at all.  Her veins kept rolling.  So, we need to let her arms rest for a few days and try again to get the blood.  We need to check her AFP tumor marker.

Meghan is almost finished with her first year of preschool!  I think she will really miss going to school and it's been great to see her develop great social skills!

Three years ago yesterday, Meghan's life was literally saved when she had her brain MRI.  Finally, after almost 10 months of searching for answers, we got answers!!!  While the news was a huge relief and the biggest scare of my life at the same time, I knew she would now be okay.  I am so so so proud of Meghan's accomplishments and her will to fight each and every day.  Three years ago today, her shunt was placed and our baby got a second chance at life.  It is amazing how much happier she became after her shunt was placed.  We are very blessed!!

Holding Meghan in pre op room. 

After surgery

This is the picture that I think of when I think of Meghan's first smile.  This is the first picture of her smiling that I don't see pain in her eyes.  Thank you God for leading us to the right doctors!!



Happy Spring!  Now that winter is officially behind us, we look forward to spending a lot of time outside!

Meghan started participating in a program called Special Olympics Young Athletes.  The program meets for 1 hour every Monday night for 7 weeks.  There are 6 or 7 other kids in the program - some she knows from school.  Since she is unable to participate in contact sports, this is a wonderful program for Meghan to be involved in.  Last Monday was the first get together and Meghan had a blast!  She is excited to go back next week!

Jeff and I met with Meghan's school a couple days ago for an IEP meeting.  The meeting went great and everyone adores Meghan (who doesn't?!?!)  Gross motor and speech continue to be her biggest struggles, but she continues to progress at her own speed and there has been absolutely no regression in any area!  Cognitively, she is doing super!  She is able to count to 6 consistently and recognizes numbers 1-9.  She knows the major colors and shapes and can label them.  She knows her first and last name.  The alphabet is a little tougher for her.  She recognizes the letter M and is trying really hard to write that letter.  This school year, Meghan was in the Special Ed classroom which consisted of 6 special needs kids in the class.  For next year, we all agreed that it will be best to place Meghan in the preschool class that has 10 typical kids and up to 8 special needs kids in the same class.  She is ready for this transition cognitively.  There will be a few modifications needed for Meghan and due to Meghans size, she will have a more one-on-one para with her at school, mainly for her safety.  It is a faster paced class, so that concerns me a bit, but she will be allowed extra time to do her work. 

An issue that has come up within the last couple of months is sound sensitivity.  Meghan has always been sensitive to louder noises, but it has gotten noticeably worse recently.  It seems to be the higher pitched sounds that will bring her to instant tears, as well as sounds she has heard all her life that never bothered her before, but they do now.  Other than having her avoid hearing sounds that are sensitive to her, there isn't anything we can really do.  I do not think it is shunt related at this point.

This is still in the works, but when we will be in Maryland visiting Meghan's runner and her family, the Special Olympics torch run will be going through their city.  Meghan's runner has been in contact with an organizer for this event, and it's a big possibility that Meghan, her runner and her family, as well as Jeff, Luke and myself, will all be able to participate in the torch run!!  We are super excited about the possibility of this becoming a reality and are truly, truly blessed to have some pretty amazing supporters in our life! 

Growth hormone shots are going well for the most part.  There are still some days where she cries, but overall, it's going well!  Next doctor appointments will be in May and then I believe we will have the summer off from appointments...YAY!!!

Happy 2 year Zipperversary to my little Chiari warrior!!!!  It's hard to believe it was 2 years ago today that Meghan had her second brain surgery.  It seems like it was just yesterday.  I remember the day so clearly.  So many emotions and these anniversary dates always bring back all the emotions from surgery day.  I'm so proud of Meghan for the fight she fights.  She may be little, but she sure is fierce and she won't let anything stop her, or slow her down!  She doesn't have school on Wednesdays, so she is home with me today, which is what I need today.  We are getting lots of snuggling time in today!!

In PICU

2 weeks post op

Meghan's scar today!  Barely even noticeable, except a bald spot where hair still hasn't grown in around the scar.

This is one of my favorite pictures.  Meghan was in PICU and all she wanted was the purple Chiari Warriors bracelet on her.  She also wanted her puppy (who was able to be in the surgery room with her and got it's own wristband) next to her. 





 

 

New shirts for the Hydrocephalus walk/raising awareness are now available for ordering!  All shirt designs are the same but there are 3 different order forms - 1 for tshirts, 1 for non hooded sweatshirts and 1 for hooded sweatshirts.   I will close the orders on February 22 and will place the order that day.  If you need help ordering, let me know and I would be happy to help!  Please note that the prices listed on each order form are estimated prices based on a minimum quantity.  Prices could change depending on how many shirts are ordered.  If there are more shirts than what the minimum is, the shirt prices will go down.  $2 from each shirt sale will go towards the Team Mighty Meghan walk team donation to the Hydrocephalus Association.  The money donated to the Hydrocephalus Association will be used to fund research to find a CURE!!  If you know anyone else that is interested in ordering, pass this info on to them.  You can copy and paste the order form link by clicking on the blue 'share' down arrow towards the top of the order forms, then copy and paste the link.  As of right now, youth sizes are not available in either of the sweatshirt styles. However, if I have enough interest in youth sweatshirts, I can create an order, so be sure to let me know if you are interested in youth size sweatshirts.  Thank you for your support!

T-shirt order form:
Price based on minimum quantity of 25 orders
Estimated price $15.73 + $2.00 donation to TMM walk team = $17.73/shirt
**Youth sizes available**
https://www.customink.com/g/snd0-0016-2enz

Hooded sweatshirt order form:
Price based on minimum quantity of 10 orders
*Price is based on 10 orders, but need minimum of 6 orders to order this shirt*
Estimated price $40.19 + $2.00 donation to TMM walk team = $42.19/shirt
**Youth sizes are not available**
https://www.customink.com/g/snd0-0016-2euf

Non-hooded sweatshirt order form:
Price based on minimum quantity of 10 orders
*Price is based on 10 orders, but need minimum of 6 orders to order this shirt*
Estimated price $35.18 + $2.00 donation to TMM walk team = $37.18/shirt
**Youth sizes are not available**
https://www.customink.com/g/snd0-0016-2etp

I am a very happy Mommy!  Meghan had her first growth check up this week with her Endocrinologist since she started growth hormone therapy.  When she started GH 2.5 months ago, she weighed 20.8 pounds.  Today, she weighed 22 pounds.  To put this into perspective, Meghan has been at 20 pounds since her last surgery in February 2013!  In 2.5 months, she gained 1.5 pounds!!!  She also gained 1 centimeter in height!  I didn't think we were ever going to get her past the 20 pound mark.  The doctor would like to see a little more growth next time we see her in 4 months.  According to the growth charts, she is still -3.79% below the 0 percentile for her age.  The only concern from the appointment is that Meghan has been complaining for a few months about leg and knee pain.  She complains about pain in her right leg, which is her larger leg.  She is going to check with Meghan's Orthopedic doctor to see if she wants to see Meghan sooner than October, which is our next scheduled Ortho appointment.

Meghan also had an abdominal ultra sound.  She did super!  She even helped the ultra sound tech while she was having her scan.  She has a little more fluid in her right kidney than previous scans, and Urology was consulted, but it's not concerning enough for them to see Meghan!

I have spent the last few weeks talking to Meghan's teacher, OT and neuro about Meghan's last MRI in November.  When I got the MRI report in December, one of the findings listed on the report is that Meghan has a slipped disk in the C2/C3 area.  She also has early disk degeneration disease in that same area.  After reading the report, I talked to Meghan's OT about the findings (she is super knowledgeable and has many times helped me understand things more than doctors).  Her concern was if there should be any activity limitations and if Meghan needed some kind of stabilization and advised me to talk to her neuro.  I talked to Meg's neuro who has decided to limit some of her activity.  He doesn't want her doing any tumbling of any kind, no summer saults, jumping on big trampolines or bouncy houses, or doing any activity that would put pressure on her neck area.  No stabilization is needed at this point, but it is something that will be monitored over time.  Luckily, Meghan is very mellow and not much of a risk-taker, so we don't have too much limiting to do.  The neuro is also recommending that we have a neurophsych assessment done on Meghan.  Jeff and I have known about this for a couple months and we have decided that at this time, we do not feel this is something we need to put Meghan through.  I think a year down the road when Kindergarten gets closer, we may decide to have this done, but not right now.  Meghan's teacher and OT agree with us that it will be beneficial as Kindergarten approaches, but no need to do the assessment right now.  A neurophsych assessment is broken up into three 2 hour testing sessions.  An assessment includes tests of the child’s intelligence, academic skills, attention and concentration, learning and memory, processing speed, visual spatial perception, language skills, visual motor and fine motor skills, sensory perception, executive functioning (such as planning, organization, initiating and inhibiting behaviors) and emotional functioning.  A pediatric neuropsychologist interprets the pattern of results in the context of the child’s developmental stage, their current setting and the child’s medical history.  An intervention plan is developed to support the development of skills and/or how to use the child’s strengths.  Why would an assessment be beneficial for Meghan?  It would give us a description of her strengths and weaknesses, give suggestions on what we can do to help her, give us recommendations for educational programming, and help us know what is fair to expect from Meghan at this point in time and suggest further therapy if needed.

 

Some good news is that the biopsy from November is normal!  We will meet with the Geneticist in 4 months.

We will have a team IEP meeting end of Feb/early March to discuss schooling next year.  I am hoping we can transition Meghan into the preschool class that has typical kids and special needs kids combined so we can ready her more for Kindergarten.


Meghan helping the tech while having an ultra sound

FINALLY!!  This sweetie has officially gotten past 20 pounds!!!

 

Happy New Year!  2014 was a great year for our Mighty Meghan!  It was the first year since she was born that didn't consist of a hospital stay!  It was also the healthiest year she has had since birth!  Woohoo!!!  We hope to have another great year in 2015!

Meghan is getting much better with taking her growth hormone shots.  She doesn't cry as much when she is given her shot!  We will have a check up with her Endocrinologist in a couple weeks to see how she is doing and to see if she has grown at all since starting growth hormone.  We have already been told not to expect to see much growth at the first check up, but we will see! 

We are very excited for this summer! On Christmas Day, we surprised Meghan's runner and told her that we booked tickets to fly to Maryland to visit them in early June!  We are excited to have this opportunity to visit Jenn and Ryan again and to meet their 2 adorable little boys!  While we are in Maryland, we are going to try to meet up with a family who has a little girl with Hydrocephalus.  The Mom of the little girl and I have been friends since Meghan was diagnosed. 

Otherwise, there isn't a whole lot going on with her right now, which we are very thankful for.  We - like everyone else - are doing our best to dodge the influenza bug that is going around right now.  It seems to be everywhere.

The annual Hydrocephalus Association - Minnesota walk will be held on Sunday, September 20th, so mark your calendars if you would like to join our team and walk for a cure!  I will post more information as soon as it becomes available!