PWS Awareness Day 19: Abe's Perspective

What is it like to have Oscar as a brother? I should know. I’m Abe, Oscar’s older brother (Read about me on the right side of this page).

Oscar is much more “normal” than you might think. He has relatively strong social skills, and he is articulate and coordinated enough that on first glance, he could easily be just another teenager out with his family. I think that’s how most people see him—people who don’t know him anyway.

To close family friends he is much more—a funny, talkative, brother of Ruby or me, or son of our parents, who is a huge A’s fan and loves animals. Oh and he has Prader-Willi syndrome too. But that doesn’t quite tell the whole story.     

To me Oscar is a funny, hardworking, earnest, and stubborn brother.  Our relationship is similar to that of other siblings who aren’t disabled (I think). We talk (and argue) about baseball; we plot against our parents, our sister; we play board games together. Doing these things with Oscar can be very rewarding. Yet in these situations he requires some extra scaffolding from me, as my mother would say.

In order to keep him in a good mood, I have to be careful that I don’t question his opinion, start winning by too much, or set him off in any other way.  I try to walk the line of stretching him as far as he can go, or close to snapping, because I think it helps him grow as a person. Later in life, I want him to be able to win arguments, to be gracious in defeat, to do things that other people can do with less difficulty than he can.  

I set my hopes high, but his goals are even higher. I want to give him a chance to reach them.

That’s my mindset on a good day.

There are also days when I don’t have such an optimistic view. Days when Oscar is anxious, upset, worried; when he throws tantrums over minor details. Days when I can’t make him laugh. These are the days in which I wish he didn’t have a disability, I question why it had to be me, I wonder about life with a “normal” brother.

We always hope there will be fewer of those days in the future.

PWS Awareness Day 18: Why is Oscar so thin?

People are often surprised when they see a picture of Oscar or meet him for the first time.  He's very thin, not what one expects when they hear he has Prader-Willi syndrome and insatiable appetite. In part, he comes by it naturally -- Paul is very tall and thin, and family genetics are a big factor.  We also have food security in place to ensure that is healthy and safe, and getting the right amount of calories.  But there's more to it than that.

Many years ago, at a National PWS conference we heard that the ideal weight for a person with PWS is a quartile below their height percentile.  So, if Oscar's height is in the 50th percentile, his weight should be at about the 25th percentile. 

My understanding is that the main reason for this rests in how PWS affects body composition.  We've not done the fancy tests, but Oscar has less lean muscle mass and relatively more fat mass than a typical person - and therefore his healthy weight is actually less than that of a typical person of the same height who has more muscle.  If he were to gain excessive weight, my impression is that he would gain fat mass at a more rapid pace than lean muscle mass which would put extra strain on his organs.  He'd also have extra weight to support but not the increased muscle strength to carry it like typical people do when they gain weight.

Growth hormone therapy helps increase lean muscle mass and reduce fat mass (along with many other positive effects). Oscar has been getting nightly growth hormone shots since he was four months old, and there's no doubt he has benefited greatly.  My guess would be that his lean muscle mass is still below average though.  And, with less muscle, he looks thinner than expected for his height.  I notice this especially in his arms but also his legs.  

We work closely with Oscar's primary care physician and his endocrinologist to make sure that he is growing and gaining weight at the right pace.  And sometimes we need to make adjustments. Just last fall we all agreed that we needed to up his calories to ensure he had enough fuel for puberty.  (In case you are wondering, we made these decisions with Oscar out of the room, since discussing increasing calories in front of him would have created major anxiety!)

My two thin (and good-looking) guys. 

PWS Awareness Day 17: Thoughts on a Meltdown

I called home from Lit Camp today, hoping to get to talk to Abe, Ruby and Oscar.  I hadn't heard their voices in a couple of days.  Abe told me a little about coxing the quad and the four in the final race of the season.  (He hasn't been able to row since he broke his wrist three weeks ago but he still found a way to be in a boat.)  Ruby told me about the sleepover at her friend's house. She slept there last Friday too, and she giggled when I asked if she'd be made a plan for next Friday as well.  But Oscar couldn't come to the phone.  He was in his room, screaming, Paul said, frustrated that his favorite baseball video game wasn't working out the way he wanted it to. 

Oscar had big hopes for this game -- he wanted to be able to create his own team, just like the current Oakland A's. He wanted to make trades and play against other teams.  He wanted to pretend he was A's general manager Billy Beane and play out a virtual (and very realistic) season.  But the game isn't an exact replica of the MLB.  And it's complicated, too complicated for Oscar to play by himself.

"It's just too hard for him," Paul lamented, "I need to figure it out myself so I can help him."

"I probably should have researched it more before buying," I said.

Paul hung up quickly so he could go be with Oscar, who was still yelling.  I couldn't hear him through the phone but I could imagine him kneeling on the floor, his upper body splayed over the bed.  I could him imagine him crying "It's not fair! It's not fair!" over and over.

But Paul wasn't anxious or worried about Oscar being so upset.  Neither was I.  This isn't the the first time he has melted down over this particular game.  He'd recover of course.  But it did make me reflect on how Paul and I both recognize that it is up to us to control the environment for Oscar.  We don't fault Oscar for this tantrum, I realized. We fault ourselves for not providing him the support he needs.  It's back to that thin environmental buffer -he just doesn't have the resources to manage this kind of disappointment, this kind of challenge.

Here's our big A's fan...at the ballpark in April.  Smiling.

PWS Awareness Day 16: Social Skills

Last weekend, after Abe's crew practice and Ruby's soccer game, we packed the whole family (visiting grandparents included!) into the car and drove an hour north to Sonoma Valley to Oscar's friend Eli’s birthday party.  Oscar had been talking about the party – a baseball game and swimming – for weeks.  He couldn’t miss it.

Oscar and Eli had bonded at school over their sports fanaticism.  Oscar, an Oakland A’s and Warriors fan, had finally found someone else who devoured the sports section every morning, someone else willing to talk trades and records and stats for hours.  Oscar was even able to accept that Eli is a Giants’ fan, something that might have been a deal-breaker a few years ago. (“Yeah, but he likes the A’s too, Mom,” Oscar would add.)

We pulled up the gravel driveway and spotted the “baseball field" off to the left.  Oscar leaped (yes, Oscar leaped) from the car and ran across the grass.  "Eli! I'm here! I made it, Eli!!"  The big game was already in progress, but I could hear several enthusiastic calls of "Hi Oscar!!"  There may have been some high fives.   

Abe and Ruby joined in and next thing I knew Paul was pitching and Grandpa was taking pictures. It didn't matter how many pitches someone needed - everyone got a hit.  Paul expertly bobbled a few catches, and then Oscar's friend Eli smashed one past the outfielders and several runs came in.  GrandMary and I sat with other parents on the deck stairs by the pool, chatting and looking out at the vineyards and surrounding hills. And I pretended that it was always this way.  That Oscar was always so engaged, so included, without any scaffolding or prodding.  That I didn’t need to be standing on the sidelines making sure he was following along and that no one was getting frustrated with him.

Oscar has made great strides socially this year, and has some genuine friendships centered mostly around a shared interest in sports and music.  I know why -- his teachers work on social skills every day, in the classroom, in social skills groups, and in one on one sessions. He, like most people with PWS, has needed to be taught every little aspect of interacting with others. The consistent school-wide push has been crucial.  And, importantly, Oscar’s peers at school, a school for kids with learning differences, are well-matched for him.  As the head of the school says, these peers “mirror and match” Oscar’s skill set. There is a nice balance of kids who are working on the same things that he is, and others whose strengths are his weaknesses.  The mix means that Oscar fits in, but also has models.

Years ago, when we attended our first Prader-Willi conferences, many of the speakers emphasized the importance of social skills.  Those conferences were packed too with strategies for navigating food and behavior, the benefits of growth hormone intervention, information on managing GI issues, plus tips for supporting gross and fine motor development.  I couldn’t absorb it all.  Raising a child with PWS is a never-ending triage situation.  There were years when social skills were the priority, and others when scoliosis or GI issues took the front seat.  I can’t look back and wish I’d pushed harder earlier.  But I am happy to see him so engaged now, so interested in connecting with friends, and able to carry on a conversation of more than two exchanges about something other than just his favorite topics. 

I wish I could post a photo of Oscar with one of this friends, but I'm hesitant to without permission. So, instead, here is Oscar with his favorite furry friend -- his cousin A's dog Penny. (Penny, you're famous!  And thanks to Grandpa for this and probably every other photo I've posted.)

PWS Awareness Day 15: Pushing Through

Yesterday morning, a friend texted me a hilarious exchange that she and her husband had over pre-dawn coffee about my blog posts.  Not about the posts themselves, but the fact that I am managing to write them every day.  The exchange was so funny that I started cackling. When I tried to read it aloud to Paul, tears streamed down my face and I couldn't finish.

I really needed that laugh.  And I really needed the acknowledgment that I am posting every day.  The truth is, many nights I am so exhausted that I almost give up.  I do try to start early but I often have trouble settling on a topic.  I start two or three posts and abandon them or save them for later.   It's hard.

Two nights ago I spent an hour looking through pictures thinking I’d take a break from writing and just post pictures for a few days, but then returned to the post I’d started earlier about “the plan”.  As midnight approached and I still wasn’t done, I really wanted to give up.  Just like I wanted to give up three nights earlier when I was writing about the pervasiveness of food in our culture.  But then, and this sounds sappy, I realized I couldn’t.  I had to keep writing.  I had to post something.  Oscar lives with PWS every single day.  He doesn’t have the option to give up, to step away from the challenge.  So this is one small way I can honor him. 

That said, I am away at Lit Camp, a writer's conference in Calistoga, through Sunday where I'm meeting other writers as well as agents, editors, and publishers.  I'll be getting feedback on the first pages of my memoir about learning to cope with Oscar's disability and then helping him to do the same as his understanding of PWS grows.  It's exciting, and I'm nervous.  And the internet is spotty.  But, somehow I'm still going write these blog posts!

Here's Oscar, on his third attempt to climb to the top of the wall. He made it!

PWS Awareness Day 14: Enough is Never Enough

Some describe people with PWS as being always hungry.  But Oscar and many others are actually better described as never full.  It's an interesting and important distinction, and not one I can claim to have made.  I heard it first from those two PWS experts Drs. Gourash and Forster who also developed the concept of food security after working with many patients with PWS at the inpatient crisis center at the Children's Institute in Pittsburgh. 

As I've mentioned, Oscar will almost always finish every last bit of his meal.  Yesterday the grapefruit quarters I'd given him for snack were completely stripped of all the pink flesh, and most of the white pith.  He would eat the gelatinous strip of fat on the steak if Paul or I didn't cut it off.  He will drink the rest of the soy sauce after the sushi is gone.  But we don't talk about "full" or "hungry" at all, and I never ask if he's either.  The question is irrelevant because the answer won't change anything.  He's done eating when the food is gone.  If Oscar says he's hungry I remind him when the next meal is.  If I were to instead, even one time, give him a little bite of something, it would undermine his food security and create anxiety.  He would know, or have the impression, that he could acquire more food, food outside "the plan" at any time.  He would never let go of the possibility that I might cave again because I did that one time. He's never forgotten a "one time" breach.  The one time I let him get out of bed before official wake up time. The one time he had screen time before he was done with his homework. These breaches often end in meltdowns when denied the next time.

The reason that the distinction between always hungry and never full is important is that telling others that Oscar is always hungry doesn't elicit the desired response.  If Oscar is always hungry, well-meaning people would be tempted to placate Oscar by giving him just a little bit more.  Instead, if we say that he is never full, and that enough is never enough, no one is tempted to give Oscar more food, since more food won't solve the problem.  No matter how much you give him he could always eat more. So simple..but I would have never thought of it myself.  Thank you again, Linda Gourash and Jan Forster.

And, because pictures are fun (and remind me that I am writing about, and for, Oscar) here is one of the two of us along the California coast one beautiful Sunday last April.

PWS Awareness Day 13: Allergies, Anxiety and "The Plan"

Oscar is suffering from allergies this year.  He sneezed and sniffed and wiped his eyes all weekend.  I'd never given him medication before -- I am just not sure how he will react.  Can he take an adult dose? Will it make him drowsy?  Or spacy?  I just kept hoping the sneezing would stop.

It didn't.  He was still sniffling and stuffy after school today, so I asked one of the staff, a homeopath, at our local pharmacy what he recommended.  He pointed me toward a homeopathic chewable for kids and proceeded to describe a dosage plan that included Oscar chewing tablets at intervals throughout the day.  Oscar was with me, and must have interrupted five times, getting more and more agitated. 

"I can take it at school by myself, at lunch and snack. I can do that!" he jumped in.

Most of the medicines for kids have some sort of sugar. They taste good, I realized, as his voice rose and his eyes flipped between me and the homeopath.  I needed to adjust his expectations.

"Kids can't give themselves medicine at school. That's the rule," I said and turned my attention back to the homeopath.

"So when am I going to take it then?" Oscar interrupted again.  "Will Dad know I need it at breakfast? How many should I take a day?"

The uncertainty was triggering his anxiety.  He fired these questions off at me before I could start to answer. 

[So here's where I'm tempted to make up what happened next. A little creative license. I can do that, right? Yeah, well, then you all would get some picture of me that's not very accurate.  So here's what really happened:]

"Oscar, STOP.  I am in charge of 'the plan'.  I will GIVE IT TO YOU when it's time." I raised my voice and over-enunciated, catching the attention of other shoppers.

He didn't stop.

"But how will I know?" he asked again, pleading almost.

I puffed up my cheeks and let the air out slowly.  Then I tried again, a little more calmly. 

"Ok, here's 'the plan'," I said, knowing this was what he needed. "I am going to give you one today, and then I have to see how your body reacts.  I need to see if your symptoms get better.  But I will give it to you when you need it, at the right time.  That's 'the plan'."

He finally relented.

For many years now we've been using the "the plan" as a way to create a structure and eliminate uncertainty in the environment.   We use "the plan" to convey a schedule on a busy day, when there is a change in routine, anytime there is anxiety, to deal with crisis moments.  Teachers use the "the plan" as a way to end arguing and gain compliance on non-preferred activities.  "The plan is we are going to run a 1/2 mile and then play at the park."  I use the "the plan" to get the laundry folded and the exercise in before screen time on the weekend.  We invoke 'the plan' multiple times a day -- it's not personal, it's not a punishment or a reward.  It creates a structure and definitely helps allay anxiety about what is happening next, as well as manage spiraling expectations, like at the pharmacy. 

I don't have a picture of Oscar red-eyed and sneezing, so here instead is one of him with Paul and A's pitcher Dan Straily at Fan Fest. Highly-anticipated days like these require creating "the plan" well in advance.

PWS Awareness Day 12: Behavior (A Start)

Most parents of kids with Prader-Willi syndrome will tell you that the most challenging aspect of the disorder is the behavior, not the food. I would agree. We parents eventually get used to managing the food.  We start to eat more healthily ourselves.  We put in place a food security plan and stick to it.  We lock when the time comes.  We cry about that, and then we get over it.  We educate our schools, friends and families. We are clear and brave about asking others for what our kid requires to function.  And we avoid the situations that are unsafe or cause stress because of food uncertainty or access.  It sounds hard, and it definitely is.  But it's not the hardest part for many families.

People with PWS struggle with a wide assortment of behavior issues -- tenuous emotional control, rigid thinking, skin picking, perseveration, impulse control, and OCD-like behaviors to start.  Many behavior problems can be traced to or are exacerbated by stress....including a breach in food security, unexpected changes in the schedule or the plan, uncertainty or inconsistency of any kind.  My favorite graphic from two of my favorite PWS experts (Dr. Linda Gourash and Dr. Janice Forster) is one that shows a smiley face representing a typical person.  Lighting bolts of stress are striking a nice wide "environmental buffer" surrounding the smiley face.  A typical person has lots of inherent coping mechanisms to manage the stressors of every day life.  A person with PWS does not.  The graphic for a person with PWS is a sad face -- the lighting bolts are easily permeating their thin environmental buffer causing stress and leading to a whole array of behavioral issues. 

We've seen most PWS-related behaviors in Oscar: tantrums, skin-picking, anxiety, non-compliance, sneaking, hoarding, inflexibility, arguing.  Whenever behavior issues spike (as they have in the last couple of days) we consider any shifts in the environment.  Is Oscar getting enough sleep?  Is there a big event he's anticipating (an A's game, a grandparent visit, a trip to the zoo?)  Is he worried about something?  Is there uncertainty about the plan?

Oscar has demonstrated over and over that he needs a structured, predictable, consistent, low-stress, (and food-safe!) environment.  And so, the goal for him is not independence.  The goal is, in the words of Drs. Gourash and Forster, maximum function with support. 

He will, in short, never live on his own.   And that's due to both the food and the behavior.   But this does not mean that we have given up -- we work on that "maximum function" every day, helping Oscar develop coping mechanisms, helping him deal with uncertainty or unexpected changes.  Helping him navigate this complex and crazy world a little more comfortably.

Here he is, giving me a thumbs up after a five day camping trip in Yosemite with his school.  A trip that was successful in large part due to the safe environment and consistent approach his teachers were able to carry from school to mountains. 

 

PWS Awareness Day 11: Writing

Writing has always been Oscar's most challenging subject.  Many of his behavioral challenges at school could be traced to writing -- the physical act of putting pencil to paper to draw or write created so much anxiety that, in kindergarten, he would cry and hide under the table whenever the teacher brought out the markers.  It was so bad that I actually suggested that we pull him from art class. (Thankfully his teacher politely ignored me, and instead she and the art teacher worked to make that class one in which Oscar would participate and eventually thrive.)

A behavioral specialist helped us break writing and drawing tasks into smaller bits starting in kindergarten. Oscar was rewarded when he finished a step, such as writing his name on the line, with the goal of finishing three steps without refusal or a tantrum. By 3rd grade the tantrums over writing were less frequent but he still needed substantial scaffolding and he used a "break card" when he got overwhelmed.  His handwriting was still slow, and he struggled with the generation and organization of ideas, so for longer writing projects he would answer a series of questions created by me or his aide that would "magically" turn into a paragraph when the questions were removed. Or he would dictate entirely to his aide.  These pieces tended to be long and rambling but we celebrated Oscar getting words onto the page.  

In 4th and 5th grade Oscar started typing and using two programs -- Kidspiration and Co-Writer -- to improve speed and help with organization.  His teachers also introduced him to their own organizational tools.  My favorite method for research projects was the color-coded index cards. Each color was tied to a specific research question, and Oscar, with the help of his teacher and his aide, would take notes on these cards and then use them to form paragraphs.  The process was long and laborious, but he still managed to write decent research papers on Christopher Columbus and Green Day's Billy Joe Armstrong.   Even so, we were still working at the sentence level in 5th grade...getting him to write a sentence using the daily prompt was sometimes excruciating.  And he certainly was not able to independently write a five sentence paragraph on a topic of choice.

But this year, in 7th grade, we are seeing big progress in organization and independence. This year (with only one anxiety-based tantrum) he managed to write a 900 word novel with plot and characters through the NaNoWriMo project. This year he finally learned how to write a paragraph with an introduction, supporting sentences and a conclusion in a timely manner.  This year he came home from school excited to start his homework -- a five paragraph persuasive essay about why HE should be chosen as one of the five students to read an excerpt from his novel at a local bookstore.

"Mom, I have to get started on my essay right away," he said as he got off the school van.  "I really want to be chosen to read, so it has to be a great essay."  I raised my eyebrows and smiled.  Was this really the same kid?

Today, for Mother's Day, Oscar handed me a letter he'd written at school.  I know this letter probably started with a graphic organizer on paper or in Inspiration:  a main idea in a center circle with supporting ideas in adjacent circles.  I know he probably wrote a few rough drafts and passed it to a peer for edits. But I know he did it without a tantrum and I'm betting he came up with his ideas quickly and independently...because he wrote a similar note for his grandmother in ten minutes flat.

Like so many things Oscar has learned to do over the years -- walking, riding a bike, tying his shoes (never mind, we are still struggling with that one) -- it took many many many times the amount of instruction and effort of a typical kid but he was finally able to do it.

Here's that awesome letter, and a picture of us.  (He looks pretty proud, doesn't he?)

PWS Awareness Day 10: Counting Cookies*

Have you ever noticed how pervasive food is in our culture? I never really paid attention until Oscar was born with PWS and its signature insatiable appetite and caloric restrictions.  Now I see food everywhere. There are the obvious things -- every birthday, soccer game, or five-minute meeting requires food.  Over time we've encountered lollipops at the barber shop, tootsie rolls at the video store, mints at the dry cleaners.  Surprise Halloween treats at Ruby's preschool at the beginning of October.  Ice cream in Abe's 6th grade class for returning signed forms.  Random people on the street have offered my kids cookies.  And finally the more subtle but still distracting references -- the seemingly innocuous cake in the chapter book, candy canes in the preschool workbook, endless tv commercials advertising humongous messy hamburgers. 

When Oscar was in preschool I did my best to remove the food references from his environment. We tried not to sing "Who stole the cookies from the cookie jar", for example.  He perseverated over anything involving food, especially those toy kitchens with the plastic delicacies. Rubbery chocolate chip cookies, pink-iced cakes, miniature fries...all so alluring to a preschooler with PWS who had never eaten any of those items. He was fascinated and couldn't concentrate on anything else when food (pretend or real) was nearby.  Teachers quickly replaced those play kitchens with pretend veterinarian offices, rainforests, and post offices.

Now, at age 13, Oscar continues to be pretty focused on food.  And while he frequently demonstrates sneaky behavior (with food and other items) he's not routinely stealing. Not yet, I remind myself often.  Oscar's pre-occupation with food is not something we can expect to improve, and will almost definitely worsen.

We have an elaborate "food security" plan that we follow that helps alleviate anxiety and behavior issues that arise from uncertainty.  In short, Oscar gets only the food we give him, when we give it to him.  It's the No Hope-No Doubt plan.  No hope for more food. No doubt he will get what he needs. We follow a schedule of breakfast, snack, lunch, dinner so he is always assured of his next meal.  We have locks on the cabinets and eventually the fridge will be locked too.  We keep him out of the kitchen when we are preparing food. It helps that our kitchen is tiny and there is no room for food-gazers.

Several years ago, back in elementary school, one of Oscar's teacher asked me how we did it.  She knew we kept food out of the environment -- he'd been at the school for 3+ years at that point and they got it.  No extra food, not even for birthdays or holidays.  But that day she asked if we also screened every book he read.  He'd been reading one book at school that had a list of food in it, and every time she checked Oscar was reading that same page again, and again, and again. I was grateful she picked up on it, and even more grateful that she helped him smoothly transition to another book. Knowing how and when to interrupt his perseverative tendencies is a finely honed skill.

But, later that week, the math homework reminded me how impossible it is to police the food references. The food yes.  We absolutely police the food and make sure that Oscar is always in a food secure environment.  But those references are impossible.

Here are the math word problems (photocopied from a national math program):

1.  If you have 7 cookies and give 3 to your friend how many do you have left?
2.  If you have two cookies and your sister has 18, how many do you need to have the same number of cookies as your sister?

EIGHTEEN COOKIES? Who needs 18 cookies? What about buttons, or pennies?

(I just laughed. Sometimes that is all you can do.)


And, because pictures are fun and far more interesting than long blog posts about food security, here are some ridiculously cute photos of Oscar swimming with dolphins, taken around the time I originally wrote this post.  

*10/2009 post, somewhat edited and updated.