Top Ten

Here is our Top Ten list of what I was told at Ayden Jane's diagnosis when she was 8 months old. These things were discussed as near absolutes for her future. I looked at my sweet, silent infant in her car seat and listened to her Doctor paint a bleak picture of what her future would become. A second doctor later advised me to accept she was disabled and stop trying to fix her. That diet nor supplements nor.... would change things. The first doc was kind, just uninformed about improvements that were possible but I can gladly say that he has followed Ayden Jane and has changed his PWS tune. The second doctor ... well, the last I saw him he threw her chart across the room and stormed out. He did not like that I questioned his authority. Sort of wish he was still there so I could let him meet Ayden Jane now.

As a side note, I have to add, we found doctor 3 at age two and she was very different. She straight up told me to remember most of Ayden Jane's genetics were intact and she was much more Gulley than PWS. She had no idea how true that was!!! And of course, there is Dr. Miller who shows us Ayden Jane is more than PWS with the way she talks to, plays with, laughs with and cares for Ayden Jane. Each of her patients is an individual first with their own unique make up and needs who happen to also have additional needs due to PWS. 

1 - Behavioral Problems/Autistic Tendencies
Doctor - High risk for Autism and would at minimum have autistic like tendencies. Behavioral problems would occur because she would not be able to control her emotions and have sensory processing problems. These would likely make it impossible for her to be taught in regular education classes or participate with typical kids in activities. 

Ayden Jane/current- Ayden Jane is in regular classes at school, is on regular rec soccer team, rec swim team, goes to summer camp, church groups... and very well behaved. We do see some of the "autistic like tendencies" here and there. She is very literal and not great at reading social cues. She does need things repeated and notices things others do not. She does get over emotional at times but because she feels deeply. She cares to the 10th degree. She is the best cheerleader, encourager, defender... anyone could want because once you are on her "team" she will have your back forever. 

2 - Appetite
Doctor - PWS will cause her to have hyperphagia. This means her brain will tell her she is starving constantly and she will do anything she can to get food. You will need to lock your cabinets and refrigerator and never, ever leave her unsupervised. She will steal food from garbage, stores, friends and neighbors. She will not be able to help it. In addition, she will only require 60 percent of the calories that other kids her age will need so you will need to feed her much less. The only dietary recommendations - cut her calories.

Ayden Jane/current - We chose a diet that was low in carbohydrates, moderate protein and high in good fats. This was against doctors recommendation at that time. She thrived. Once under the care of doctors that had a clue, diet was supported and tweaked for her individual needs. She currently is NOT HUNGRY. She leaves food on her plate when she is FULL. She understands why she eats the way she does and monitors herself. She is not bothered in the least by siblings or friends feasting on carbs she can not eat and is likely to tell them they would feel a lot better if they did not eat them either. I can leave her to make her own breakfast and go for my run. She is still learning, but honestly has better skills in the kitchen and makes better food choices than most any 11 year old kid. 

3 - Lack of Energy, Balance, Coordination ...
Doctor - PWS will cause her to have poor balance and coordination. Her motor milestones will be delayed. She will not be able to learn to ride a bike or play sports. She will have low muscle tone and a lack of energy and will not be very active. She will likely be overweight or obese which will make all of these things even harder.

Ayden Jane/current - Ayden Jane reached her motor milestones at the late end of typical development. She learned to ride a bike while in Kindergarten and loves to ride all sorts of scooters, power wing, whatever. The faster and crazier the better. She has even learned to surf! Does she have low tone? Yes. Does she let it stop her? No. She climbs, bounces, rides, swings, swims, plays ... all with her entire being and loves every minute of it. I don't even pretend to watch at times because she also falls, crashes, and lives on the edge. The effects of PWS on her activities make doing all these things a bit harder and I do not anticipate her winning the MVP trophy, but she is the true meaning of sport and love of the game. 

4 - Sleep Disturbances
Doctor - PWS will likely cause her to have sleep apnea and wake early. She may also wake and look for food during the night so locks are important.

Ayden Jane/current - Ayden Jane does not have sleep apnea. We have not done anything to make that the case, she has simply not been given that bonus feature. She did begin to wake very early and often the months to year prior to pitolisant. Woke repeatedly starting around 4-4:30 AM. The rule was she had to stay in bed until 6:00. She also was unable to stay awake past 6:30 without a significant after school nap. Interestingly, the doctor did not mention daytime sleepiness. He spoke of a lack of energy ... which maybe a different way of looking at what I see as daytime sleepiness. We addressed the daytime sleepiness with Pitolisant which is for narcolepsy and aims to regulate sleep wake cycle. It was the correct choice for Ayden Jane and she now sleeps well at night, going to bed about 9 and sleeping through until 7. With the quality night time sleep she goes hard all day everyday and can think more clearly and learn much better. 

5 - Cognitive Delay
Doctor - PWS causes cognitive deficiencies. The average IQ is 65 so you can expect a her to be below average and require specialized instruction in school with an emphasis on life skills. 

Ayden Jane/current - First of all, that is just plain old news. Average IQ is now closer 95 if given gh early in life. (typically in the first year). Ayden Jane has an IQ in the average range and scores academically in the 70th-80th percentile on standardized tests of typical kids. So, in a room of 10 typical kids her age, Ayden Jane ranks in the top 1/3. 

6- Learning Problems/Disabilities
Doctor - Even if your child does manage to have an IQ close to the average range she will likely have learning disabilities which will further require special instruction in school and make it difficult for her to learn.

Ayden Jane/current - Yep. She has a couple learning challenges. Namely, she processes information a bit slow. This means there are times she misses instructions or information. She does not miss the instructions because she was not listening, but because the words were coming faster than she can process. There is a difference! Additionally, she takes longer to memorize facts and works slowly. Like a tortoise some days. So what to do? We choose to provide accommodations in the regular classroom. She has friends who repeat instructions sometimes. We love a checklist. She works harder to memorize and is provided with extra time to complete assignments. She loves to learn. She is smart and proud of her grades and the effort she pours into them. She will gladly put in the extra effort and amazingly never complains. 

7 - Temperature Intolerance
Doctor - Honestly, he did not mention this one on diagnosis day. I have heard about it since and typically kids with PWS do not maintain body temperature well. They can get too cold or overheat and become lethargic.

Ayden Jane/current - Ayden Jane used to NEVER wear a coat and claimed to be warm even when blue. She would also overheat super easily. Pitolisant has improved this one a fair amount, although I wouldn't give her the med just for this. I consider it a positive side effect. Ayden Jane still has issues with this but it seems to be isolated to indoors when buildings are over heated and "stuffy". I don't claim to understand it... just saying what it's like. This was oddly one of our biggest challenges when the weather started to turn cold. Her body just gets all sorts of confused when it's cold outside but overheated inside. 

8 - Skin Picking
Doctor - Listed among the difficulties of managing PWS is skin picking. People with PWS will pick at their skin until they bleed and beyond. These sores will commonly become infected and it can be frustrating problem. There is not a lot you can do but cover the sores and try to distract them.

Ayden Jane/current - Ayden Jane does not skin pick. Never has thankfully. Living here where mosquitoes could be the state bird I was concerned each bite would turn into a rough road. It is just not the case for AJ. I am sure it is a frustrating problem but I have heard some great results from the use of PharmaNAC. (we love the stuff and use it for other reasons)

9 - Aggressive Behaviors
Doctor - People with PWS often develop aggressive behaviors as they age. You may need to look into group homes for the safety of you daughter and your family. 

Ayden Jane/current - Ayden Jane does have big emotions and this can include anger. She can be crazy excited, heart-broken sad, incredibly joy-filled, fighting mad ... None of her emotions or responses are odd other than they are magnified over what you would expect in some situations and less than you would expect in others. So yes, the intensity can be a bit off, but parenting, patience, consistency, discipline, and high expectations are all necessary. Add in the strong-willed nature, at least in Ayden Jane's case, and the need to start young teaching them they are able to stay in control and skills to manage big emotions is important. All kids get big eventually and PWS kids are no exception. If you want them to stay in control and not become physically aggressive when they are big, the time to teach them is when they are little.  In the end, I love the big emotion. It's a way she is a bit different, but the joy and excitement this kid can get over something tiny is BIG. 

10 - Scoliosis/Orthopedic Issues
Doctor - scoliosis is common

For Ayden Jane a 24 degree curve seemed to pop out of no where when she was approaching 5 years old. We were given a few months to see what we could do with PT etc and the appointment was made to consider serial casting. Lots of PT and swimming and hippo-therapy and running and.... her curve gradually reduced until it was just minimally monitored.

Fast forward to this March. Poof. Curve jumped up again and we started night bracing and we are ramping up the running etc. It is extremely uncommon for a curve to be reduced at this age and the goal is simply to keep it from increasing and avoid surgery. Of course, Ayden Jane says don't worry she will have it back under 20 by the time we head back for our next check in September. That's how she rolls!!

Absolutely not an exhaustive list. There are other things to watch for like diminished pain, stabismus, speech issues/delays, fine motor problems.... I could keep going but sheesh! In the end, kids with PWS are kids. They are a member of a family. They have awesome personalities and strengths and weaknesses that have NOTHING to do with PWS. As parents, we value those that look at our kids and love them for the amazing little ones they are. The teachers, the therapists, the doctors, the friends that see our kids as quirky, cool, little kids and love them unconditionally. 

Top Ten Part 2- Food, Hunger and Satiety

My sweet, beautiful little baby sat quietly in her car seat as the doctor described the HUNGER that would surely come with Prader-Willi Syndrome. His advice was to restrict her calories and lock up the food in our house. Not to allow others to eat around her and be prepared for her to think she was starving but not allow her additional food. Yeah.... that sounded horrific to me as well.

The combination of hunger and slow metabolism was described as the main characteristic of PWS. Where are we now? Ayden Jane not only accepts that she has to eat differently, she can make the choices on her own. She can order her own meals at restaurants and fix her own meals at home as needed. There are no locks on our fridge or pantry and Ayden Jane is left unsupervised regularly. These are things that were unheard of by her original doctor.

The most amazing piece with Ayden Jane, however, is that she can 'read' her hunger and full signals. I do not think they are they same as a typical person, nor are the quite as reliable, but she has learned to use them to help guide her. She will stop eating and tell us she is full. This I NEVER get tired of. We can trust her when she says she is hungry, or still hungry and requests a bit more at meal time. It is not frequent and she always talks through it. Mom, I'm still a little hungry. Should I wait a few minutes and then decide if I need more food? Or, I am still hungry, would more vegetables be the best choice? I think I had plenty of meat....

Most kids with PWS will also talk, think, dream about food. I mean, hard to focus on school or anything else food on the brain. Also, they worry about food. What food will be there, what will I be able to have, how much will I be allowed.... Since Ayden Jane is not constantly hungry she does not have food always on her brain.

I can't say how Ayden Jane has reached this point but I can say she has eaten low carb, moderate protein and high fat since the start. We have also talked about food and how her body uses food extensively. She understands the science behind how she eats and how PWS effects her nutritional needs. I am also very aware that different kids with PWS just vary in this symptom like all the others. 

PWS Awareness Month Top Ten - Part 1

May is Prader-Willi Awareness month. I struggle a lot with this. On one hand, PWS is a crazy exhausting, syndrome to manage which seems more like wrestling a constant moving target with no way to know what "joy" is coming our way next; and on the other hand, Ayden Jane is pulling of quirky typical kid to most people.  I get frustrated at the chronic nature of the genetic syndrome and wish Ayden Jane could spend her days with her biggest worry being if the waves are good.

When Ayden Jane was diagnosed 10 years ago we were given the sage advice of the day. Otherwise known as the list of all the things Ayden Jane would never be able to do and the ways she would struggle/suffer.

I think this year for PWS Awareness Month I will make a sort of top 10 list of things that were predicted and nearly guaranteed and where we are with them now.

Number 1 - Scoliosis

I don't say number one as in this is the number one challenge, I am just putting it out there first because the wonderful horse barn that has loved on Ayden Jane since she was an 18 pound 18 month old riding a sweet little horse all the way until now!

Scoliosis is very common in PWS. Absolutely not a guarantee, but definitely something that has to be monitored and addressed as needed. For Ayden Jane a 24 degree curve seemed to pop out of no where when she was approaching 5 years old. We were given a few months to see what we could do with PT etc and the appointment was made to consider serial casting. Lots of PT and swimming and hippo-therapy and running and.... her curve gradually reduced until it was just minimally monitored.

Fast forward to this March. Poof. Curve jumped up again and we started night bracing and we are ramping up the running etc. It is extremely uncommon for a curve to be reduced at this age and the goal is simply to keep it from increasing and avoid surgery. Of course, Ayden Jane says don't worry she will have it back under 20 by the time we head back for our next check in September. That's how she rolls!!

Scoliosis

A few days ago we headed to Shriner's for a back check. We have been doing this for years and up until this trip Ayden Jane has mostly received good news. Her streak of strong and straight came to an end.

She did her thing with standing in the cool x-ray machine they have. It scans from the top down and the image comes up on the screen where I can see it. My heart dropped. What was once a slight curve was now a very obvious curve. I did my best not to panic Ayden Jane, saying it looked bigger but we will see what the Dr. says.

Doc came in and as I looked at the x-ray and heard the numbers. In the past 4 months her curve has gone from 11 to 26 degrees. The doctor continued to outline options and future. Night bracing. Daytime bracing. Surgery... I was trying to focus. All the while Ayden Jane was whispering in my ear. We will work hard. We can fix it. We did it before.... I put my hand on her leg and quietly told her we need to listen to the doctor right now. "Okay mom, but have FAITH. We will have everyone pray and Jesus will tell us what to do. He always does. He is faithful to take care of us. I'll just surprise the doctors again."

Now I'm not going to pretend that as she was picking out patterns and being measured for the brace that the reality of what's coming didn't bring forth a few tears. It was a roller coaster of a couple days, wavering from very worried she will be able to sleep in the brace to determined to straighten her spine so fast she won't even need it.

In the end, she is ready to fight. New running shoes were purchased and day one of running already accomplished. Contact was made to the amazing Mrs. Jen, her PT of old that helped her conquer a 23 degree curve at age 4/5. Lots of people praying that we will know just what to do and that Ayden Jane will have the strength and determination to do it. We return to pick up the night brace in 4 weeks.

In the end this song is what she is running to and the life she lives.

Confidence

Schedules and Flexibility

Ayden Jane is having a fantastic year. I may say that at the beginning of every post because I will never get tired of typing that! I got such a kick out of her the other night as processed through a change coming up.

Her schedule so far this year was to have exploratory from 8-9 and then settle in to LA until recess time, somewhere around 11:30, give or take a few minutes depending on the day. So it is along academic block, but Ayden Jane has done fine with that. Recess and lunch puts them back into the classroom and hard at work from 12:30 - 2:30 for dismissal.

The change is simply to flip math and LA so that she will have math in the morning and LA after lunch. (and yes, social studies and science are in there as well, I'm just not exactly sure when) When Ayden Jane heard of the change her initial reaction was to get a bit upset. "But I'm doing so good! Why do they have to change things?" Ha, valid.

In the end, she asked me to be sure to include some math foods at breakfast (no clue what a math food is) and keep in mind she has LA after lunch so pack accordingly.  Sure kid. I'm on it. Hopefully she will have a great day Monday and will be 100 percent on board by days end. 

Growing Up

Yep, I'm back. The full time work is done for now and I am hoping to get a bit of writing back on track.

Ayden Jane is really growing up. I'm not talking about how tall she is getting, even though that is happening too. I'm talking about her conversation. About dealing with social things at school. About caring for others. Okay, parts of her are growing up. There is plenty of her that still loves princesses, and fairies, and playing with toys, and imagination. I am thrilled those parts are content to stay young a little longer.

Growing up is challenging for any kid. Most kids have fears that other kids will laugh at them. Most kids worry that they will get picked on. Some kids worry about tests and grades. A few kids glide through pretty much unscathed but I think if we are honest, that is very few.

Now, how about heading into the wild world of growing up when you are a bit, "quirky, different, unique, weird, interesting, complicated, ...." just pick a word. These are the words that make fascinating and successful adults, but targets in school.

Over the years Ayden Jane has had her share of being made fun of. If that makes you gasp, breathe and think about it. SOME kids are just not nice. Some kids do not understand that all people are valuable. Some kids do not like others who are not the same as they are. She's had a bit of a resurgence of being on the receiving end of unkind words. We had a long chat about the stuff that is said and goes on in school. In the end though, here is what she had to say about it all:

1 - don't worry Mom. I know what he/they say is not true. God made me awesome.

2 - he/they are just mean. I think maybe they had a rough life but I'm not letting them take that out on me any more.

3 - if he/they were ever really, really, mean, C would punch them in the face. (I did NOT encourage this one. don't worry)

So there ya go. Confident, compassionate, protected. I'll take it. I mean, to hear negative things about yourself from your peers at age 11 and truly know and believe that those things are not true? To not lash out but to have at least some compassion for them?

Working on knowing when to ignore and when to speak out. - things like don't touch my desk or stay away from me. Although not nice, those you can just ignore. Calling you names, using bad language, threatening you, telling others to say bad things about you. - speak out.

Basically, Ayden Jane is a great kid. She is smart and perceptive and just when you think she is clueless, you find out she wiser than you could imagine. Some day she will be able to express all the cool stuff that rolls around in there. In the mean time, I enjoy the glimpses. 

It's Been a While

I am surprised how long it's been since I have posted. Well, sort of. In November I went back to work for a few months. Then December just full on flew out of control!

So to recap highlights, Ayden Jane managed straight A's for the first time. She was so proud of her all A's. She worked hard for it and totally earned it! As a reminder, she is in all regular mainstream classes and did not receive one bit of special education support the first quarter (more on that later)

Thanksgiving came and we had a nice family meal with all the girls. Ayden Jane does really well at these sorts of times and honestly, I just didn't worry too much about portions being a bit bigger. She was excited to bring over some keto candies that we made as a special desert and she was happy.

On to Decemeber. It was so very, very, very, beyond busy. With the rain and flooding of the fall, most activities were pushed into December and all the typical December excitement and Christmas things were mushed together with them. It was overwhelming for me and Ayden Jane loves to do EVERYTHING so we just over did it. On top of that we passed a nasty, long lasting cold around much of the family. So, much of December is a blur.

I think we FINALLY are putting together the last pieces of handling illness in Ayden Jane. She caught the nasty, snotty, cruddy, endless cold. She handled it pretty well the first few days and then, she didn't. It was like she hit a wall and in a matter of hours she went from okay to looking like a truck ran over her. Her skin color was bluish on her arms and the whitest white on her legs. She felt cold to the touch. Her energy disappeared and a headache came on strong. At that point, we went to the doc and he gave us a script for hydocortisone at Dr. Miller's recommendation. It was definitely the way to go! She turned around pretty fast and was back to fighting the cold well. After another week of fighting the cold we hit up the antibiotics since Ayden Jane has such a horrid ability to fight infection. In the end, Ayden Jane managed to kick the cold with no major complications. Woohoo.

It did, however trigger the strangest tics. She often fidgets her fingers quickly and sometimes makes a few odd facial movements when eating, but nothing life altering. Kayla nicknamed it chipmunking and with a reminder she stops Then this crazy uncontrollable ticiing came on. She could not stop it even when we pointed it out. It became nearly constant. After some thought, consulting smart folks, lots of reading, I decided to try treating it sort of like PANDAS even though I believe it was triggered by a virus rather than infection. Non of this is actually a PWS thing, it's a sucky immune system thing. Anyway, treatment is basically naproxen for inflammation and PharmaNAC because there is some evidence that it increases the effect of the naproxen. (and it can't hurt)

Sure enough,, after one dose of both Ayden Jane was more calm/movements more quiet. She told me after about 30 minutes when I went to check on her that she thought it was really helping because she could make it stop when she noticed she was doing it. That was yesterday and this morning it was still a bit better. I dosed her again and I really think it's working. So hoping she will be back to herself in time for school in 2 days.

She's one tough kid. One thing after another....

Carnitine

So before we ever went to see Dr. Miller I started to notice Ayden Jane was losing weight and looking scrawny across her shoulders. In the past this was a sign her gh was low. She was also starting to get tired earlier in the evening.

I talked through it with Ayden Jane, trying to figure out what she was feeling and she honestly amazed me. We talked about things that could be causing her reduced energy feeling. She described it as a physical tired, not a brain tired. She felt that it was not Pitolisant related because it felt different. She was excited to get lab work done to see if maybe it was gh. I was a little concerned about how labs would go in terms of gh because she was a little sick the week before and that always messes up the gh level. Sure enough, her IGF- 1 was a fair amount higher than it was 6 months ago and raising gh was off the table even though her dose has not been increased in a year and a half.

So now what? After a chat with Dr. Miller she told me I had room to play with Ayden Jane's carnitine supplements so play I did. I made some pretty risky moves and we all paid for it. Oops. Here's the scoop. Ayden Jane requires significant amounts of carnitine and always has. Over years of experience I have learned that Carnitine Fumarate provides physical energy and a dash of Acetyl carnitine seems to really improve brain energy and speed and over all 'enhance' the carnitine usage. I stopped the ACL with each Pitolisant dose increase and then slid it back in, with the exception of this last increase. ACL is apparently a natural remedy for narcolepsy so it all makes some scientific sense. (sorry, I am being tough to follow)

I decided to add back in both CF and ACL  The result was Ayden Jane's little brain was in over drive. Her poor teacher had her hands full with Ayden Jane partnered with a boy she was sweet and kind to the week before and had ZERO patience with the early part of this week. It was not pretty and none of us liked Ayden Jane on this dose. ha. I decided to ditch the ACL for now and keep the added CF and see how this rolled. It was so much better. Sweet, focused, happy Ayden Jane came back to us.

I also noted with the increased carntine that she is not hungry. As in, leaving food on the plate and saying she isn't hungry and doesn't want any more. Time will show if she increases the muscle mass back in and if I see the return of her physical endurance at swim team. Hoping for the best!

And, because I always mess way too much, I will likely try a small amount of ACL eventually. 

I Don't Know When It Changed

This past weekend Ayden Jane and I had the pleasure of heading to Indianapolis for a One Small Step walk hosted by some of our favorite people. Hurricane Florence was blowing into town at a snails pace. Well, honestly, I think a sloth would have beaten the storm in a head to head race. I felt we were safe to stay through the storm but as the approach dragged on and the days of rain and possible power outage lie ahead the big kids voted us off the island. They were unconcerned about the impending hurricane but they had no desire to be locked inside for days on end with our own little tornado of energy.

So Ayden Jane and I made a last minute decision to pack up and head out. Ayden Jane is an easy travel companion and was even willing to read aloud to me to help pass the time.  It was like my own little audio book.

We stayed with wonderful friends who open their home (and giant basement) to us any time we are in town or in need of a stop. Ayden Jane had a wonderful time with their sweet kids. She was thrilled for the attention of H, a teenager who has not lost her ability to be playful. She enjoyed hours of fun playing with B, a sweet, energetic boy a few years younger. And, of course, loved seeing E, a younger version of Ayden Jane.

The biggest purpose of the trip was the walk on Saturday. There were several families with children who have PWS, some I already knew, some I had not. After the day and the conversations and the talk of independent futures "one day" .... I got to thinking.

I don't know when it changed, but my mindset has changed from:

IF Ayden Jane will graduate from HS with a diploma to WHEN she will.
IF Ayden Jane will go to college to WHERE she will go to college
IF Ayden Jane will be able to have a job she loves to WHAT job she will choose.
IF Ayden Jane will live independently to what do I need to teach her to make all of these things come true.

This entire post likely sounds crazy to 99 percent of people. If you have a child with PWS then you likely can't imagine them having such a typical life, learning to be fully independent and manage job, food, money, medical things .... If you don't have a child with PWS, and you know Ayden Jane, then you probably think I'm crazy to have once worried so much about her future.

Somewhere along the way, my thinking just changed. We were told a lot of things when she was diagnosed and one of those was she would never live independently. That she would need to live with us or in a group home. It weighed heavy on us but now, I don't really worry about the future or what it will look like for AJ. I mean, we think about what options will be good for her. We know that she may take the round about route and need extra instruction or help from time to time, but I have no doubt she will find her way. 

Intro to Grains

Since Ayden Jane was 8 months old we have been "grain free". Basically, grains in the form of infant cereals proved to cause improper body composition, in ability to grow, and mute her personality so we threw them out. There were a few select exceptions like occasional quinoa and flax meal.

It was likely a drastic response as the parent but they felt like desperate times (and they kinda were). I needed to make a big change and, although it may sound like it was a hard thing to suddenly remove all crackers,, bread, cereal, pasta along with the obvious cake and cookies, it was a simple straight forward rule. It forced me to get creative and come up with other things to feed a nearing one year old. The improvements were undeniable and I never wanted to go back.

I know it likely sounds strange but even the thought of Ayden Jane eating grains panicked me a little. I looked at food so differently and even went grain free myself for a time.

Now, Ayden Jane is nearing eleven - years that is. After discussions with Dr. Miller and the need for slow burning complex carbs to be available for growth rather than relying so heavily on protein she wants me to try introducing a very small amount of complex carbs into Ayden Jane's diet on a regular basis.

Ayden Jane has occasionally had beans like in chili, but certainly not regularly. Oats are a choice Dr. Miller would like us to try since being a girl with PWS by UPD, cholesterol levels run a bit high. She told me this back in January but I knew I needed to get Ayden Jane to a "better place" before messing with something new. Quite honestly, after she was doing so much better and I was home for the summer and could really watch, I forgot completely about it.

So.... This past Tuesday I worked up the courage to give her something we swore off 10 years ago. I gave her the full fat plain greek yogurt with frozen blueberries she loves AND added 1 Tbs raw steel cut oats and 1 Tbs healthy granola (oats, nuts and seeds). I gave it to her after school and she crashed early that night - 7:00. Then woke up with a belly ache around 3:15 and was up for hours.

She finally fell back asleep about 5:30 and I did not wake her for school. She did wake on her own at 7:15 though and was chipper and ready for the day. It was almost crazy! She went all day long with no problem and had a great day.

So.... where do I go from here? Experiment will continue on the weekend and I will give them to her at breakfast. (that's the goal anyway since I need to find something other than eggs for breakfast). We will see how it goes.

It is crazy how sensitive she can be to tiny changes in diet, supplements or medication. I am so very thankful, however, that I am home and able to work through the nuances that are Ayden Jane.

Grade 5 off to a Great Start

We are 7 days into the new school year. Ayden Jane is confident and happy and learning. Whew!!!

K-3 were such amazing years. There was struggle for Ayden Jane and much to overcome but she was supported in all the right ways and each year brought many moments of joy and much for her to be proud of.

Grade 4 was rough. I honestly did not write much because I did not have a lot that was good to say. Something in the combination of personalities at school, the transition to a new school, me going back to work and who knows what else made for a train wreck of a year. It effected Ayden Jane deeply to the point that her anxiety was through the roof, she was acting like a cornered animal behaviorally, panicky at every minor thing, having night mares, developed significant tics, and even her labs were getting screwy. We pulled her and placed her on medical homebound to get everything to settle.

Needless to say, this year HAD to start well. After the last month being home last year, a summer of pouring positive into her and rebuilding confidence and reteaching behavior, it HAD to start well.

So happy to say it has. Honestly, I don't know what exactly her academics have been these first couple weeks but I do know she is happy. She is confident. She is excited to go to school each day. She is communicating beautifully with her teacher and at home. She talks of friends and things she is doing with a smile and no worry. She can problem solve and not just melt down.

As far as energy goes, the first couple days were exhausting as expected. So much to process, so many new faces, new routines, new books. I imagine old fears were lurking. It zapped most of her energy the first 3 days but this second week she has done fantastic. When I pick her up she is silly and chatty and NOT tired. She's able to just be a typical kid. Can't ask for more than that.

Summer

I know it is cliche to say that summer is going by/went so fast! That's the truth though. I'm not saying there weren't days I thought might not ever end, but overall I am feeling the "I can't believe it is August!!" 

Summer brings reduced stress. Opportunity for camps and fun. Lots of swimming and boogie boarding and a bit of surfing. Lack of schedules. This summer, a return to horse back riding and learning to ride on her own.  Lots of laughter. Ayden Jane is refreshed and ready to tackle grade 5. 

On the medical side, labs are good. Eyes were checked and we don't return for a while. Scoliosis check will be sometime in the fall. Next trip to UFL for endocrinology the end of October. Pitolisant still waiting on the eap to help with the financial piece but importation still open until that happens.

We have managed to do a bit of reading over the summer but little to no math. I expect this year to definitely be a challenge for Ayden Jane. She learned things last year, but I just had the feeling they didn't quite sink in like they needed to and she will have to fill in a little bit shaky foundation as well as build on that foundation this year. 

One thing we typically do during the summer was skipped. No running. It has been a very hot and humid summer. Then came lots of camps. Then the past few weeks rain and storms.... I am still hopeful we will get back on the running trail. Mostly because it is so good for her and she is super motivated to be in great shape when we see Dr. Miller in October. It's going to be a challenge but I will do my best to get creative and find the proper motivation for her. Truth is, she is a decent runner. Somewhere along the way though, she has decided she's not able to do it. I miss that confidence she had before the struggle of last year and the illness of last winter. 

Her health has rebounded. Still hoping for the confidence to make a full recovery. Of course, she is at an age where that can be hard! 

I Know What I Can Eat

Posts have slowed... obviously. It's partially because we are busy. Partially because life is sort of on cruise control. Mostly, however, it's because Ayden Jane is older. The things I would write about seem more personal somehow. I want the blog to reflect life with Prader Willi Sydrome for Ayden Jane. At the same time, she is just a kid who has struggles like any other kid and who wants their struggles broadcast.

That said, I haven't abandoned trying to write her story. I am just working on how to do so respectfully.

The struggle she had today is clearly a PWS struggle. Well, I guess it is really a 'medical diet' struggle. Ayden Jane works hard to understand her diet. She is learning well and proud of herself. Today she was at a camp and the director was concerned about giving Ayden Jane whipped cream on her blueberries. Ayden Jane knows she can have whipped cream but the director texted me anyway. (which I think was totally the correct choice. Better safe than sorry) Ayden Jane, however, is very bothered when situations like this come up and she knows, that she knows, she can have something and people doubt her. It may be more that she thinks they don't trust she's smart enough? Maybe that they think she is not smart? Maybe she partly worry's they think she is lying?  Whatever the reason, it REALLY bothers her. She held it in until she got home and then I got an earful of how mad it makes her that they don't believe her. That she knows what she can have and that she isn't going to eat anything she can't have because it would just make her feel awful and she wouldn't have any fun....

Having Prader Willi Syndrome means having a lot of rules about food. It means having to think with your head and not trust you body. It means having a great deal of self-discipline. At age 10 Ayden Jane is determined to use her head, follow the rules, and have more self discipline than most adults can muster. Great job AJ.

Houston

Last weekend Ayden Jane and I traveled to Houston to meet up with a few of the families that are also taking Pitolisant. Well, the point to the weekend was to meet with Drs. at Texas Children's Hospital to discuss pediatric narcolepsy and Pitolisant and PWS and better characterizing sleep in PWS.

Ayden Jane and I ended up flying in too late to actually meet with any of the doctors as the agenda switched a bit after our tickets were purchased but Ayden Jane had a wonderful time hanging out with her friends.

It was interesting to see how relaxing it was for her to be around kids who operate in her realm, so to speak. They have similar food rules, the have a little slower processing so Ayden Jane is never left behind. They all accept each other for exactly who they are. They totally get it that each one is being the best person they can be and that sometimes it's just hard.

I had a great time watching the kids run around, play hide-n-seek and just enjoy being together. No pressure. Ayden Jane was firing well for most of the weekend. She did crash a bit while we were at the airport. She really wanted to hang out with J for a little while before our plane took off but AJ was really just not much fun. After we said goodbye I finally got smart and found her some caffeine and poof! She was right back on track and just in the nick of time! We got on the plane and then it was delayed... yep, we had to sit and wait a while and if she hadn't rebounded so well it was going to be a long, long wait. She did beautifully. Pulled out a sticker book and dressed the movie stars in outfits and was GREAT.

We returned late Sunday night and I was tired as well but we both got up Monday morning and went about our days.

I need to spend the summer getting her lots of opportunity to hang out with kids. Being a youngest by so many years and having such awesome big siblings, she spends a lot of time with "adults". We pulled her from school and she has been spending even less time with kids her age not to mention that she started acting a bit crazy from all the stress before we pulled her.

I'm out in a couple weeks. Noah comes home. Summer camps and beach/pool days begin. Hoping it is a wonderful summer! 

Overdue Update

I can't believe it's been a month! Okay, I can believe it because time has been flying. When I left off we were considering bumping Pitolisant. I chatted with Dr. Miller and for a few reasons we opted to wait. Mostly, I was grasping at straws to figure some things out that honestly, had nothing to do with Pitolisant.

Ayden Jane did not have a banner year in school. Not sure exactly where it went off the rails but it became more and more obvious that there was no way we were going to get things back on track. It was beginning to really hurt Ayden Jane's health, well being and ability to learn. By this point, Ayden Jane was not sleeping well, waking with night mares, snapping defensively at most comments, her posture was tense and hunched over, her voice unsure and the tics... oh how the tics were developing. She was becoming anxious about food and her weight was rising. Basically, PWS was creeping in as the anxiety escalated. I spoke with Dr. Miller about all of it and she said to pull her from school. She would fill out whatever form I needed but that Ayden Jane needed to be removed from that setting.

I was honestly unsure how it was all going to work but I could not handle watching the tics that shook her body take over. After about 3 days the anxiety seemed to wane. A week in there was so much more laughter. So much better sleep. No more night mares and the tics were down to mild movements. The food anxiety disappeared and eventually the defensive snapping at us relaxed. I was getting my sweet, confident, funny Ayden Jane back. I began to realized just how bad things had been as I saw the relief.

So how has the schooling piece gone? Great. Ayden Jane works so very hard and Gary is home with her. Their routine is to go to dunkin' donuts and get a coffee and work there for a couple hours. (Ironic, I know) Ayden Jane is focused and gets a lot of work done. Her love of learning has returned and she is like a sponge. The other great thing is that she is truly excited to go back to school next year. She is relieved to finish the year this way, but does miss seeing other kids and the activity of school.

Last week she went to her school to take state standardized testing and did great. No problems, no anxiety ... almost gleeful to go. Here's to 4 more weeks of medical home bound, and then SUMMER.

Camp Out and Spring Break

The week before spring break, Ayden Jane's school did a 4th grade camp out. The kids spent arrived at the state park early on Wednesday, spent the night and stayed Thursday until time for school to end. They had lots of activities and learning and fun. Ayden Jane really enjoyed Wednesday - I was not there for most of it - and we slept in a tent Wednesday night. That was hard on her. She did not sleep much and Thursday we ended up going home a bit early because she was DONE. Just tired and not having fun so no reason to push through. 

I received good reports on how she did through the day on Wednesday and enjoyed chatting with a teacher that helped with her class. I was told that Ayden Jane was well behaved and made great connections. The teacher even said she was smart and she liked how Ayden Jane's brain works. So fun when there are people that "get" her and appreciate how she thinks.

The camp out rolled us pretty much right into a road trip for spring break. It was a whirl wind trip with stops in Asheville for hiking and visiting water falls, Chicago to see many friends and to visit with Noah. On the return trip we swung by Indianapolis for a very quick visit with more friends who are part of our PWS family. 

Overall, Ayden Jane did well. The hiking was tough, but she did enjoy most of it. Spending the night in multiple homes, a hotel, lots of car travel. Not typically a great plan for kids with PWS but she rolled with it pretty well. She did get tired which causes over emotional and snacky. That's never fun. 

On this side of the trip, we are settling back into a routine. She is definitely 'mushy' and her core need lots of work from the winter and travel. Her weight is not good. Her sleep is still not back to where it should be. So, what do I do? Make a PLAN.

First up, we are going back to horses. It's just so great for her and we have had a long break. Next, the pool and ocean are beginning to warm up so there will be lots of swimming and boogie boarding in our near future. I am working on a running plan with motivators along the way. And finally, I am going to bump up her Pitolisant on Friday. It typically comes with a bit of weight gain, but at this point we may as well just add that to what we are already working on. Hopefully, the bump with help her get her sleep schedule back on track and maybe give her a bit more daytime stamina. 

We shall see!

Good Computer, Bad Wifi

At times, I need to sum up Ayden Jane's brain for people. The other day this analogy came to me. It is as if Ayden Jane's brain is an excellent computer. She is smart and can connect things well. She loves to learn and has some great skills. She is capable of learning everything she needs to learn to do very well in school.

So why does she have days/times where it seems she is very smart and days/times when she seems to struggle academically or to even put together a sentence?

I think the best description is that she has a good computer (brain), but the wifi is not always great.

-Sometimes it is all in there but waiting for her to respond, or "load the page", is like watching that little circle on your computer spin as say loading.

-Sometimes that loading sign means she has taken in more information than she can process and she needs time for all of it to file in the right slots.

-Sometimes that loading sign means she wants to concentrate on the words you are saying but her brain can't turn off the uncomfortable feeling of her shoes, or how hot she feels, or the background noise you don't even notice, or the new decorations on the wall, or.... you get the picture.

-Sometimes that loading sign means she is running low on food for her brain.

-Sometimes that loading sign means she is just plain too tired to summon the extra effort it takes to process the information you are sending her way.

I have to admit, I love the days were information process quickly and she can learn complicated things with ease. Oh how I love it when anything comes with ease for her.

I need to remind myself sometimes, when I am frustrated with that spinning circle, that it has to be even more frustrating for her. 

Pitolisant

I haven't said much about Pitolisant in particular lately. Mostly because the other things that have been going on sort of overshadowed it.

Here is where we are with it. Ayden Jane started a bit over a year ago. We went up fairly quickly, okay very quickly. Approximately every two weeks brought about a dose increase. The increments were in 4.5 milligram amounts until we got to 18. By that time we were close to the end of the school year and decided to let things settle in over the summer. Summer's are easy on pretty much all fronts. Of course, other than the keeping her busy front. She wants non-stop action in the summer. (for details on how all that went you can look back to Feb/March of 2017)

After summer I decided to let Ayden Jane settle into grade 4 on the same dose. Not thinking it was all she needed, but wanting her teacher to get to know her as she was on 18 and help me be able to note improvements if I increased the dose. Well, it started off okay, and I increased and then this year sort of crashed and burned on a number of levels. I have not been able to get good info from school. She got sick in December. 

I saw Dr. Miller at the end of January and she was still not well. Dr. Miller gave me the go ahead to bump her dose again. This relieved the need to cut her pills into fourths and allows me to give her a pill and a half, equaling 27 mg. My frustration is that I feel like her brain is better. I think she is processing well and can learn more easily. I believe she is communicating better in general and know she has been way more fun to live with. It's the feel, think and believe stuff. I really want an objective opinion that does not spend as much time with her to be able to help put some parameters on the gains she has made.

Enter her amazing teacher from last year. The one who walked us through those challenging first months and pushed Ayden Jane to new heights last year. Even more importantly, the teacher that Ayden Jane connected with, is able to communicate well with, trusts and loves. The wonderful Mrs. M has agreed to spend a bit of time with Ayden Jane with a two fold purpose. 

1) Help Ayden Jane improve/regain her confidence in her academic abilities.

2) Get a feel for how Ayden Jane is processing, comprehending and learning. 

I can hardly wait!!

 

Calm

As the days go by, we are enjoying a pretty calm time. Ayden Jane is getting up and out the door on time for school most days. That was not the case much of this year. I am not sure what the change is... In the past she was simply not stressed about whether or not she arrived on time. Nothing we said or did seemed to motivate her. We all know that no steps in the morning routine could be skipped. And, of course, push too hard and you just create meltdown central... Some days she would nearly make it out the door and then decide she would not make it through the day if she did not shower! Seems as though the past few weeks have been smooth sailing though!

Ayden Jane also seems to have a better grip on school in general as well. She comes home and knows exactly what she wants to work on. She does not get a sort of standard homework. She brings home anything not done or asks for me to go over with her anything she did not understand. I have no idea how she is doing from the schools perspective, but she seems much calmer and less overwhelmed by it all.

We have been working on a lot of Khan Academy in the evenings. I am still a bit unclear on whether these assignments are completely required, or if they are supplemental. I'm guessing both to an extent. Ayden Jane really likes when we do these together because she does not understand the video. The idea behind Khan Academy is that there are video instructions for each skill so you can watch the instruction, It sounds strange, but she is not good at obtaining information from video. Even as a small child, she did not watch TV or video. I remember being thrilled at about age 4 that she would watch an entire episode of Curious George and give me a break!

I have enjoyed the Khan Academy with her though. She truly does not want to get answers right just to get answers correct. She gets so excited about understanding what she is doing. I could give her correct answers and she would not enter them unless I showed her how. I hope she retains that love of learning and wanting to understand. If we can keep Khan over summer I will be happy to Khan and read the summer away and be on top of things for grade 5.

We need a win next year. This one has been rough.

Settling In

Ayden Jane is doing great. It took so long to get her back on track, and I'm sure there will still be some bumps, but hopefully we have turned the corner toward summer and consistent improvement.

After school she is soaking up any learning I can pour into her. Last night it was fractions. Equivalent fraction, ordering fractions, comparing fractions.... As she grasped one concept and was doing the practice she would say, "when I am done with this, there is another part I have a question about." I was tired and finally told her no more. I had to make her go play.

She also had a great practice at swim team. Her stroke looked really smooth and coordinated. It is so strange how you can even see in her movements when things are on or off track with her. There is a balance that, when maintained, allows her to think and move well. It seems to be immune related as it always falls apart when she is sick. Beyond that, maybe, inflammation? Gut? Nutrition? So many things to keep in check and no real way to know which one/ones are struggling when she struggles.

It is so frustrating to watch her and know that "something" s off. Know that she is getting stuck when she shouldn't be. Know that she is hearing but not comprehending. Know that she has things to say she can't express. Know that she is struggling to relate to her friends when she shouldn't be. Know that she wants to get out there and be active and do... but her body is just too tired. We are coming out of two plus months of this and finally seeing the balance return.

Soon the ocean and the pool will begin to warm up. The sun will shine and the days will be warm. Lots of surfing and boogie boarding and playing and getting pounded by waves. Lots of sunshine and fresh air and time to play with friends. Afternoons at the pool playing sharks and minnows or categories or who knows what else with other kids she meets there.

Oh summer. WE MISS YOU.