May 22nd, 2014- The MRI

'Don't forget to set the alarm for 3 AM.'

Ally's MRI was scheduled for 7:30 AM the next morning.  Since she had to be sedated, she was not allowed to eat for 4 hours prior (and no formula 6 hours prior, so I had to make sure it was not a fortified bottle).  We wanted to make this as painless as possible, so instead of waiting on her we figured it would be easier just to get up at 3 and wake her to eat.  She's never really had a 'schedule' of napping and feedings since leaving the NICU so we didn't want to leave it up to her!

Russell got up with her and fed her, and she did great with it.  Ally had to be at the hospital an hour before she was scheduled, so she didn't have to go too long after that to go without eating.  I needed to be at school for testing, so Russell was the one who took her for the MRI.  I kept my phone with me all morning and he would text me updates.  

She was a bit fussy as they were leaving for the hospital (getting hungry again!) but otherwise not too bad.  They got there and got her checked in, then went up to the imaging floor.  After a brief wait, they took her back.  Russell was not allowed to go back with her.  He said he could see her through some windows before they sedated her- the technicians were all cooing over her!

He said it took about 30 minutes, and then he was with her when she was done.  He said as she came out of sedation she would get fussy and mad and could hardly wait to eat!  She did so great!  He texted updates to me most of the morning until she was out, ending with this picture, captioned "Hi Mommy!"  

My coworkers thought I was crazy for being at work, but it's a crazy time of year at school and besides, what good was I going to be in the waiting room versus being at work?  I was, however, a bit emotional that morning and went straight to the hospital after work.

We had found out about 2 days before that because of her age and prematurity she would actually have to stay overnight in the PICU due to the sedation.  They told us it was a common procedure amongst anaesthesiologists and was just a precaution.  We were bummed about that but knew they were just looking out for her best interests.

Russell stayed with her most of the day.  His Mom came and sat with her at one point when he had to go take care of a couple of things.  When I got there it was just Russell and Ally in the room.  She looked none the worse for wear, and her nurse just fawned over her.  It was my first experience with the PICU so it was a learning time for me, taking everything in and reading everything on the walls around the ward.

I stayed with her by myself while Russell went with the family to dinner.  Then I took the boys home for bed.  Russell called me not long after that from the hospital, saying that neurosurgery was planning to come by either that night or tomorrow before discharge to speak with us about the results.  I told him to please call me so I could be on speaker with them if I wasn't there when they came and he said he would.

The next day I got the boys to school/daycare and got myself to school.  I kept my phone with me all day but Russell never called.  I texted him at lunch and he said he was still at the hospital!  No one had come by yet and they weren't going to discharge Ally until we had spoken with them.  He was a bit frustrated because since he had stayed the night at the hospital he wanted to come home to shower and change clothes!

By the time the school day was over he was still there.  I went to pick up Luke from school since Russell was otherwise engaged.  In the process, my phone rang.  I stood in the hallway and spoke with our neurosurgeon.  He had been in surgery most of the day which was why he was delayed.  What he told me I didn't want to hear, but I'd been expecting.

Ally was officially diagnosed with hydrocephalus.  A shunt would be necessary.  The pediatrician working the PICU had told Russell he was pretty sure she had hydrocephalus just from feeling her head- he could tell the skull plates were a little bit farther apart than they should be, so we weren't surprised.  While it wasn't urgent (there had been talk of doing the surgery over the weekend!) it needed to be scheduled.  I spoke with him about the option of waiting (the next week was my last week of work, and the following week we were spending most of it at my parents' home almost 3 hours away, to visit with them and my grandmother who was up visiting from Florida, so I kind of wanted to wait until afterward if it was safe) because I didn't want her to suffer or have brain damage due to waiting too long, and he said he thought that would be fine.  If we notice any changes that indicate the fluid build up has become more severe (vomiting, lethargy, loss of appetite, bulging soft spot, unconsolable fussiness/crying) then we may need to take action a bit sooner, but he thought we would be fine to wait until the 2nd week in June.  I asked him about other types of surgery for hydrocephalus, and he said he thought a shunt would really be best for her.  I also asked about specialized shunts (programmable or magnetic) and he said traditional would be all she would need.

So there we have it.  On June 11th at 8:15 AM our girl has the first of many surgeries through her lifetime.  I read a statistic somewhere that of all of the 'implants' that can be done surgically (stints, pacemakers, etc.) shunts have the highest failure rate of all of them.  Many people with shunts have dozens of surgeries in their lifetime.  Our prayer now is that not only will this first surgery go well and she recover quickly, but that she will be protected from infections, failures, and blockages that will necessitate surgery more frequently.  Thanks for your prayers!  More exciting updates soon! 

May 17th, 2014- The Realization & The Run

I was getting excited for the coming weekend- to see my family again, and to do Carter's Run together.  Thursday night after the boys were in bed I was on Facebook, browsing my newsfeed.  I saw a post from someone in our Dandy Walker parents group (a closed group) where they had posted pictures of their child and how blessed they were by them.  (I believe it was a newborn, we did the same on the group page when Ally was born to introduce her to the 'Dandy-Walker world.') 

What struck me, however, was something that I've seen many MANY parents in this group post, but for some reason at that time it hit me like a ton of bricks: 'I'm so glad I didn't listen to the doctors when they recommended I terminate.'

I'd had enough.  It was the final straw.  I was so SICK of seeing parents post similar statements on Facebook, underneath pictures and videos of their smiling children.  Doctors are recommending termination of dandy walker pregnancies across the country, telling parents their children will be 'vegetables' or have 'no quality of life'.  Obviously, if a person was missing a part of their brain you would think that they would have some severe issues- myself, not being from a healthcare or medical background, might have thought before this that if part of your brain was missing you would not be able to even survive.  However, this could not be further from the truth- and these are DOCTORS telling this to parents.  So many Dandy-Walker kids defy their diagnosis daily and grow up to be well-functioning adults.  Of course, every case it is different, and some are more severe than others (especially if paired with a genetic abnormality, which Ally does not have), as we were told early on in Ally's diagnosis and have seen firsthand since- but 'no quality of life'?  What a lie!  It made me sad- these parents on Facebook had chosen life, but how many more had been terminated that could have had fairly normal (if not completely normal) lives?

I sent a message to Eric Cole, the founder of the Dandy-Walker Alliance, with my frustration and outrage.  He responded that this was exactly why the Alliance was formed, (I finally understood why the car magnet says "A life worth saving" on it) and would I happen to be attending Carter's Run since I was in the same state?  When I told him we would, he said to come to the Dandy-Walker Alliance tent to meet him and some of the Dandy-Walker kids. I was excited to realize that for the first time I was going to get to meet other kids living with Dandy-Walker and talk with their parents.  That night I also sent a message to Carter's Mom, Lisa, via the Carter's Run Facebook page to let her know we were going to be there and that we would like to meet them, but we knew they would probably be really busy during the event.

Russell has been gung-ho about wanting to raise money for the Alliance to use for its projects.  But that is the furthest thing from my mind now.  Not to sound completely pessimistic, but I don't believe we will ever find a 'cure' for Dandy-Walker.  I could be wrong, but if there ever is one I don't believe it will be found in my lifetime, or even Ally's.  How do you 'cure' a developing fetus from not having a fully developed brain?  How could a brain transplant be possible- or even a partial brain transplant (of the cerebellum)?  Maybe one day this will be possible, but for now I don't have a lot of enthusiasm about the possibility.

My mission is education- I am an educator by profession, after all.  People have to know what Dandy-Walker is, and that it is NOT a death sentence, or a 'no quality of life' sentence.  And while a lot of that can be done by parents of Dandy-Walker children (and Dandy-Walker adults) with the help of the Alliance, my belief is a lot of it must also come from within the medical community itself.

As mentioned in some of our earliest posts, our doctor at Maternal Fetal Medicine was excellent.  She did not sugarcoat anything, but she also didn't leave us without hope.  And she helped us systematically eliminate possibilities (the infection, genetic abnormalities) with testing (first blood tests, then the amniocentesis with SNP array, along with a fetal echocardiogram and fetal MRI) but always gave us all the options with full details about each.  Termination was only brought up twice- once with the initial consult with the genetic counselor where she gave that as an option and we said no way (and she never brought it up again) and then with our doctor when she told us that if any of our testing showed an issue with Ally that would cause her not to survive, (or survive for long, such as Trisomy 13 or 18) she would do everything to make sure I was fully protected during the birth process and termination may be an option with that.  It was NEVER pushed on us, and we were NEVER made to feel we were in a hopeless situation.  In fact, they directed us to the Alliance and to local support groups for special needs families as well.  They always made sure we were fully informed and were open to questions anytime.  EVERY PARENT needs to have the same care and concern we did- with ACCURATE information about their child's diagnosis and condition.

My hope is to get in touch with our doctor and ask her to help us figure out how to get the word out to the medical community about the truth of Dandy-Walker.  I'm really not sure what that looks like, but I'm hoping she can help out and give me some ideas.  The Alliance has put together a great brochure for genetic counselors and doctors to give to families when they first get the diagnosis that is full of good information and encouragement and is very easy to understand.  If we can get that out across the country to the people that need it- doctors and new DW families- that will go a long way in stopping unnecssary terminations of DW pregnancies.  Educating the medical community is my first and ultimate goal.

My second goal is to partner with the Alliance to create even more resources for families, especially newly diagnosed families.  I am hoping to find a few 'point families' in every state (with the help of the Facebook group) that the Alliance and doctors can list and provide for new families so they have someone close by to get in touch with and talk with when necessary and would be familiar with doctors and services in their state.  Another Mom on the Dandy-Walker parents group was asking for the links to all the blogs like mine to link to hers- I'm hoping we can actually ask all families with blogs to send the links to the Alliance so they can have a whole page with them for families to have a reference for life with Dandy-Walker and read messages of hope and encouragement (hopefully, also listed by state). 

I was greatly encouraged Saturday at Carter's Run.  My family had a great time at the event.  Everyone except Dad, Luke, and my youngest nephew walked the 5K (well, Ally and Ben were pushed) and my brother even ran part of it.  Afterward Luke did the 1K fun run with me (whining almost the whole way, but he did it).  When all the racing was over, the boys went to watch the magician at the fun fest and I went with Ally to the Alliance tent to meet Eric Cole, his wife Andrea, and their son Ryan, age 9, who wears glasses and a huge smile and uses the aid of a service dog.  We also met Noah, age 2, who has a shunt but otherwise has developed normally and on schedule; Shawn, age 6, who walks with the aid of braces and who thought Ally was SO cute :-), and Carissa, age 24, who was shunted at 10 (and has never had a revision!) and lives under her mother's care.  I really enjoyed hearing from the other families about their children and the journey they have been on, and being able to get advice from them as Ally has barely started her own journey!  I unfortunately did not get a lot of pictures while at the tent, but here are some pictures from our day.  (Sorry, some of them are sideways and I can't get them to turn!)

 Mom, Luke, and my nephew warming up.

 Ben is ready to race!

 My brother and sister-in-law warming up

Waiting to start!

 The Great Zucchini!

 The 1K Fun Run...at least he looks happy at the finish!

Can I tell you the best part of the run?  I was pushing Ally the last leg of the course.  There were 2 women walking a few paces in front of me.  The course wound through a neighborhood and came out onto a street with some shops.  A man was going into a nearby building and I guess saw all the people walking and the course being protected by local police and asked the 2 women what cause were we walking for?  They replied, "For Dandy-Walker syndrome."  He asked what that was, and they couldn't really give him an answer.  I slowed down and said I could tell him about it.  I explained in about 20 seconds the best I could- that Dandy-Walker is a brain malformation where part of the brain is missing in the back, the cerebellum.  Often because of this gap it results in hydrocephalus.  Children with Dandy-Walker may show no signs of having it, or may have developmental delays or issues with motor skills and mental or learning issues, among other things; it is a large spectrum of possibilities.  He said, "Thanks, I'll have to look it up at home."  I told him to go to www.dandy-walker.org, and continued down the course.

And that, my friends, was well worth 3.1 miles.  

Ally and I finished together a few minutes later, running the last bit, in about 45 minutes.  The culmination of the beginning of a journey for me, as Ally continues hers.

May 12th, 2014- The Freak-Out

The very night that I posted the last update, I had a very scary mom-moment.  Ally had just finished her bath and she was laying on her towel on the floor.  I was drying her off, and her head was turned to the side, looking toward the wall.  And I saw it.

The hydrocephalus.

I could see the enlargement of her head.  I could see it going up and out the back.  It was nothing drastic, but it was like my eyes were opened and I could see the issue.

Maybe.  Was I just being dramatic?  Of the two of us, Russell is the paranoid one when it comes to our kids getting hurt (physically or otherwise), not me.  Maybe I just thought I saw something.  The only thing I did know was that I didn't know, but it made me feel that we were definitely on the road to a shunt as the resident neurosurgeon had suggested at our last visit, which made me sad for the journey ahead of Ally.  While hydrocephalus can be easily controlled, it can also be very complicated.  Shunts can get blocked or infected, and after awhile in children they are outgrown.  It meant a number of surgeries throughout her life.

I just wept.  With all of the great news we'd gotten on Ally over the short month and a half of her life, I'd become hopeful maybe she wouldn't need the shunt.  Now I didn't see any hope of that.

I was also scared.  The neurosurgeon had put in an order for an MRI, but we had heard nothing about when that would be scheduled.  If I really DID see head expansion, I wanted her to be seen as soon as possible.  I did not think she was in any kind of immediate danger, (no soft spot bulging or anything) but if I could see a difference then it was probably significant.

The very next morning as soon as I got to school I called neurosurgery and told them my concerns.  I asked if she could be seen by the neurosurgeon.  After calling back after talking with the doctor, she said that they really needed the MRI imaging to tell anything, and put in an urgent request to get it scheduled.  (When I told her I wasn't really sure about whether her head was actually bigger and that I might be seeing things, she said, "No, we know you always listen to the Mama.  They have instincts about these things."  Right on sister!)  She asked if I'd measured her head and I said no (not that I would have anything to compare it to, I hadn't written down her last measurement or had access to it).  They also asked if she was having other symptoms (vomiting, lethargy, loss of appetite, change in diapers, etc.) and I said no.  The hospital called later to say they'd scheduled her MRI for the 22nd.  I took down all the information, but in my mind I was thinking "That's almost a month away!"  She had her 2 month check-up on the 6th, so I decided to keep an eye on her and see what our pediatrician said.

I did manage to dial back the paranoia; usually I freak out and then I'm ok (or vice versa) when it comes to things like this where I get really scared or stressed.  Since that day, we've had some more updates that have calmed me more.

On May 1st Ally had her first Early Intervention appointment with the physical therapist and an educator from Easter Seals.  Since it was scheduled in the middle of the day I was unable to make it, so Russell took her alone.  He said she did really great!  Being a preemie she has a chronological age of 2 months, and an adjusted age of almost 3 weeks.  Her physical development is on track for a 2 month old!  So depending on which age you are looking at (chronological or adjusted) she is either on schedule or ahead of schedule for physical development milestones!  While this is great news, delays can start anytime so we will continue to keep a close eye on her and continue with Early Intervention in case she does have a need for therapy in the future.  One of her best milestones is her smiling at us and her brothers.  She usually does it really fast and we haven't gotten a picture yet, so we will be working on that for future posts :-)

May 6th was her 2 month check up.  I was only able to catch the tail end of the appointment, but I was glad I got to speak with our pediatrician.  She is now 8 lbs, 10 oz, so she has officially doubled in size since her birth!  (I forgot to mention in the last post that she was officially out of preemie clothes and in newborn/0-3 month clothes.  I LOVE all the girlie clothes and dresses and headbands we have for her!)  This puts her barely on the charts- first percentile for weight, second percentile for height.  Her head, of course, with the hydrocephalus, is at the 39th percentile.  I wasn't there for the actual measurement, but our pediatrician said that while her head has grown (which, of course, is to be expected) it is still in the same percentile as last month, so the growth is not exponential.  She made me feel a lot better because if she had been concerned I was going to ask her to 'stretch some muscle' and move up the date of her MRI, but she seemed unconcerned about the later scheduling.  One thing that was frustrating was I had requested that neurosurgery send her imaging to our pediatrician, and that had not been done.  Guess I will need to be reminding them of that next time!

May is one of my all-time favorite months of the year.  School is winding down, weather is awesome, we celebrate both Luke and Ben's birthdays (Birthday party extravaganza weekend!  They are now officially 5 and 2 years old!), and now it is Dandy-Walker and Hydrocephalus awareness month.  This must be 'proclaimed' in each state by the governor.  Virginia has had it in the past, but apparently it is a yearly request, which I did not know.  I put in Virginia's request April 26th, but haven't heard anything yet (not surprisingly, since it was so late).  Though it is mid-May, it could still happen!  I saw South Carolina, New Jersey, and Nevada just got theirs recently.  However, it is still unofficially Dandy-Walker Awareness month, so please spread awareness in your school, work, or church by going to this website and printing the poster and displaying it.  http://www.amywaggs.com/CartersRun/wp-content/uploads/2013/10/Dandy-Walker-Flyer-20141.pdf
You could also go to the Dandy-Walker Alliance website to learn a bit more: www.dandy-walker.org and request a wristband or car magnet from this website: http://www.dandy-walker.org/magnet-request/
You could also join us for Carter's Run this weekend www.cartersrun.org or the Dandy-Walker Alliance Day at the Washington Nationals in June.

 The picture above is a common slogan from the Dandy-Walker Alliance.  What isn't shown is the 2nd part of the tag line, which says,

"That's a lot when your child is the 1."

Never has that been more true than now.

Please continue to pray for decisions for next school year for our family.  We have had some questions answered, but still have a lot to figure out.  And of course pray for Ally, especially on the 22nd for her MRI (she has to be sedated for it :-( ).  I will have a few more updates before the end of the month, especially after this weekend at Carter's Run and her MRI next week.