Community, Neighborhood, Family

This past week we had a wonderful time with some of our "PWS family." It is frustrating that the reason I know so many people now all over the US and even in other countries is because of the common bond of having children with Prader-Willi Sydrome. However, the bond is sort of undeniable.

Imagine the chat's you have with other parents about everything from recipes to bedtimes to milestone to potty training. Now, imagine that you have no one in your community who's child operates like yours. Of course you still like to chat and you strive to include these conversations to keep up your needed connection to friends, but the conversations really don't apply. (and often make your heart hurt as you realize how far you have yet to go)

You have questions about PT, OT, ST and want to discuss AFO's vs SMO's. You wonder how to make decisions about things as basic as what and how much to feed your sweet child.

Add to that being told by the 'experts' in the beginning as your child was still just a sweet, quiet, sleepy infant that they would never be able to hang with typical kids and would struggle with a giant list of hard challenges. Then finding hope in new doctors and a group of friends who are fighting along with you to change that prediction. We cheer for each others children and we hurt with the families who's kids are struggling despite all they are doing. There is still much work to be done.

So our families - ones affected by PWS - become a tight knit community, just like a neighborhood of old and many like family. Well part of our neighborhood came to visit from Indiana. It was perfectly comfortable and wonderful to have them stay a couple days with us. How do we know them? Well, I met up with them once on our way to Chicago last summer so Ayden Jane could play at a playground and T and I could chat for an hour or so. Oh, and we met up at the conference last summer. Yep, of course the next step was to have the entire family stay a few days in our home. It sounds crazy!

We had a wonderful time. We went to the SC aquarium and the beach and the kids played like they had been cousins or something their whole lives. I think it was nice for them to to have friends around who live the same way around food. Who find almond milk and keto candies and coconut bars and nuts for snacks perfectly normal. Friends who are not phased by nightly shots or daily supplements. Other kids who automatically say "fix your legs" when little ones sit in W sit, who have the learned ability to safely play with siblings who's tone is low and body awareness sometimes non existent. No explanations necessary.

So nice for the parents to be able to just talk openly about our every day stuff. Share recipes and therapy ideas and milestones and challenges on common ground. Wonderful to be able to talk about research and medical stuff like it is typical dinner conversation.

It is certainly a unique neighborhood, but I'd say this is the best part of having a child with PWS. Gaining a whole new neighborhood that is filled with wonderful people you would have never met any other way; getting close enough to many of these PWS neighbors to truly feel like family.

Time to Breathe

When Ayden Jane was little, I mean just diagnosed through preschool years, I felt like the term special needs described her at times. I could also interchange delayed, or struggles with sensory processing, or speech apraxic, or .... all sorts of other terms I only ever learned after having her. I felt like she was hanging on to 'typical' in some ways but in a lot of ways was still stretching to get there. It felt like one bump in the road would put us forever unable to claw our way back into the 'typical' ranks.

Honestly, it was a really stressful place to live. You know, not really special needs so she didn't fit in that camp, but not quite typical either. It was exhausting and a time where siblings and wonderful family friends filled the gaps of having a typical crew of friends to run with. Add to that the medical complications of Prader-Willi Syndrome and the fact that we were on a limb with the way we were feeding her. Living outside the traditional medical approach to treating PWS. Sometimes I wonder how we managed that stress!

I also recognize that a lot of awesome people we have met because of PWS are in these stages now. Bless you. I hope that Ayden Jane and the other older kids like her offer you hope. I know it is hard to trust the hope of the 'new generation' of PWS because it is, well, new. I recognize that there are so many examples of PWS gone bad.... That it is a risk to believe your child's life will be the exception to the old literature. It is a risk that places your heart exposed to dashed dreams.

I have been told this past year on more than one occasion that although Ayden Jane is doing great now, it's only a matter of time. These things have come from well meaning parents and 'experts' who truly believe that all the challenges of PWS are unavoidable. I have had older parents say that as she goes up in years in school she will no longer be able to handle being in the typical classroom. I have been told that the gap between Ayden Jane and the other kids socially will become too wide. And, of course, I have been told that she will become constantly hungry and not be able to manage it on her on.

Thankfully, I have a small group of parents who believe as I do - that no one really knows what this new generation of kids is capable of. That their limits are not yet set.

And of course, Dr. Miller is the leader of the band when it comes to high expectations for our kids. She has already informed me she expects to be invited to Ayden Jane's college graduation and wedding.

Wow, that was not really the original intent of this post! Funny how writing sometimes takes turns of it's own.

Mostly, I just wanted to say that currently, at age 7 and in first grade, Ayden Jane is the most 'typical' she has ever been. Academics are on track. Her processing speed is finally seamless. Her energy is great. Her blood sugars are stable and the need for constant food monitoring gone. She has and is making friends with ease. Her behavior is, well, at least typical if not better than kids her age. She does not struggle with anxiety. As for 'pws hunger?" I have heard her say she is full more in the past few weeks than ever before. She is way more concerned with playing and being a kid than wasting time being preoccupied with food. She is advocating for herself when she is at friends homes and making great food choices.

I am finally - 7 years down this path - not living every moment in panic that today is the day it all falls apart. My heart does not cringe when Ayden Jane talks about what college she wants to attend. I don't worry about her when she leaves the house without me. I no longer live with the stress that I am racing against the developmental clock and need to do more with her. More PT, more OT, more exercise, more ST, learn more about food, study more and find supplements, understand more.

It's finally time to breathe. And to all the nay sayers... I know your intentions are good, but Ayden Jane and I choose to believe that although pws will always be a part of her life, it will not slow her down.

Local Endo

We see Dr. Miller once a year or year and a half but Ayden Jane is also followed by our local endocrinologist, Dr. Clarke.  She is awesome and the two of them are a phenomenal team.

Last Wednesday we had our 6 month check with Dr. Clarke. Ayden Jane's weight is higher than we'd like but Dr. Clarke thinks she looks great and is not at all concerned.  (I agree in my head, but my heart will always worry a little)  Ayden Jane grew a full inch since the last we saw her. We talked through Ayden Jane's recent illness and agreed to extend the augmentin and she also felt like the dose Ayden Jane was on was likely a bit low for her considering her history. Wow what a difference a few extra days of the higher dose has made. (more on that in the next post)

She took an x-ray of Ayden Jane's hand to check on her bone age. Basically, gh can advance bone age if the dose is too high or probably for other reasons... The end result of that is the growth plates will close earlier than they otherwise would and in the end height shortened. Height is not the main reason we use gh but it is nice to know that if the gh is administered correctly her end height should be about what it would be if she had not had PWS. The results were good news.

After the 'winter of infection' Ayden Jane's bone age was advanced by 11 months. Not good. She went from spot on to 11 months advanced in a bit more than a year... No reason to all out panic, but a trend that needed to be stopped or reversed.  Last year her bone age was 8 months advanced. That was pretty good news - we were going in the right direction. This year her bone age was just shy of 6 months advanced. Yay! Still going in the right direction. I like this trend!

A quick visual scoliosis check looked great. Lots of interesting conversation which Ayden Jane, of course, wanted to take over. Lot's of smiles and head shaking by Dr. Clarke over how awesome Ayden Jane is doing. Shared the pictures of handwriting changes with MCT....

Medical Appointments - Eyes

I have been wondering about Ayden Jane's eyes for quite some time and decided it was time to dig a little deeper into how they are doing. We have been seeing a local opthomologist since Ayden Jane was 3 and she has had glasses since just before she turned 5. For the most part she wore the glasses during school and I did not have much luck getting them on her at other times but she seemed to be doing great.

Recently I had noticed her sort of preferring her left eye. She was moving papers or turning her head to use the left eye. Sometimes turning books a bit counter clockwise to read them. She also will stand about an inch from the TV to watch and still has trouble putting letters or numbers right where she wants them when she writes.

I made an appointment at Storm Eye Institute in Charleston and off we went. I know that it is a world class place and now I just wonder why I never made the trip before...

In the end, her prescription is good but I understand a whole lot better what we are trying to actually do with Ayden Jane's eyes and am confident that there are not hidden issues lurking. Basically, she is farsighted and the right eye cross is corrected in her current glasses so that's the script she needs. This can cause her distance vision to be a little blurry, thus her reports of being able to see better without them. Her vision is actually a plus 3 but her glasses a plus 2. The doctor said with lots of compliance and wearing of her glasses he may try reducing the prescription in 6 months. The goal is to have the lowest prescription which will keep her eyes aligned.

As you can imagine that has put Ayden Jane on a mission. She heard that if she wears them enough that by the time she is done growing she may be able to go without glasses. It's a long shot but she is now determined to make it happen. She is wearing her glasses soooooo much more.

As far as eye health, her eyes are great. No stigmatism or any of those sorts of things either.

Definitely Sick

Well, not much time went by from my last post before I took Ayden Jane to be checked for infection.  Yep, ear infection and sinus infection and "slight wheeze."

I knew pretty much as soon as I saw her after school on Friday. It was late Friday afternoon and I knew there was no chance we were getting into the doctors office and I didn't want to wait until the next morning so we headed to the minute clinic at CVS. As I talked to the doctor (or nurse practitioner?) I told her Ayden Jane has PWS. Fully expecting either the blank stare of the panic look of - I remember hearing those words in med school. Instead, she said, "I have a nephew (or something like that, I went brain dead in shock) with PWS. How can I help you."

After checking Ayden Jane out and seeing a definitely infected ear, evidence of a sinus infection and even a low grade fever we talked antibiotics. I was so nice to just be able to say experience tells us amoxicillin does not really work and neither does zithromax. Augmentin just seems to be the one to work when she gets this stuff. Also, she typically needs 10-14 days rather than the typical 7-10. No problem, a 10 day script in hand and out the door we went.

Now as for lessons learned? Ayden Jane's immune system actually works to an extent now. That is great news but also made me miss the fact that she was sick. I know that sounds weird but I got good at reading her old signs. It seems that I need to trust some more subtle signs. I would not say her behavior became awful as it has in the past when sick, but she was definitely more easily upset and argumentative. Also, she was less hungry than typical - that's a strange experience for a parent of a kid with PWS and previously hunger took over when she was sick. Balancing her blood sugars has proven difficult, but things are smoothing out now. She has definitely put on some weight through the month but not as much as I am sure she would have without the lactoferrin improved immune system.

All in all, she has fared well through a winter with lots of people very sick all around her. I am counting it as a win and hopefully she will be back to full strength soon.

Sick ... or Not

I am really stumped by Ayden Jane right now. I keep wondering if she is sick. I mean the sort of sick that requires antibiotics. She had a stomach virus which was followed by her developing very stuffy sinuses. After a couple of days it all started to break up and come out so I was hopeful that she was going to beat it.

Now she is either just taking her time getting rid of the last of it or it is starting to turn funky on her. The big difference is I have no idea how to read her anymore. Her immune system is so much better than it was which is awesome, but I don't know just how to read it anymore. It used to be there were certain signs that told me bacterial infection was taking over. Now those signs are gone... No 'hunger' when she gets sick. No soaking through the bed at night. No horrid behavior. She can fight so much longer ... but how much longer? It's just so hard to know.

I really want to make it through the winter with no antibiotics but I also don't want to wait too long that we have a major infection to defeat.... ugh.  Just wish the stakes were not so high.

I can hear her squeaky little snore from the bedroom. Is that a sign? She has been tired in the afternoon this week after school. Is that a sign? or is it just that she has stayed up late every night this week. She has not finished her lunch any day this week... does she now get less hungry instead of more hungry when sick? So confusing...

I gave her some claritin this morning since allergy season seems to be upon us and I figured it couldn't hurt. I guess the next few days will either clear this up once and for all or send us packing to the doctor...

She has still managed to get her homework done, attend swim practice, soccer practice and Mckenna's soccer game this week. She has had something every night! She loves to be busy but it is hard when she is a bit under the weather and when she does not get enough sleep.