Homeschooling for children with special needs is suitable in many scenarios.
When special schools are insufficient, costly or too far, home education is ideal.
Talk with children about homeschooling to help them understand.
Consult doctors, counselors, your family before you begin.
Contact parents that already have their children homeschooled to get more info.
Homeschooling allows to focus on the strengths & weaknesses of the child.
Children with special needs feel more comfortable at home & learn better.
Parents should prepare for the educational needs of the children.
If done correctly, homeschooling helps in effective growth and development of children with special needs.
For more infomation >> Is homeschooling a good option for children with special needs? Here is some information! - Duration: 1:09.-------------------------------------------
HERE'S HOW TO KNOW IF YOU NEED MORE PROTEIN - Duration: 3:54.
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Amir Obè - Dont Belong Here - Duration: 3:58.
[Verse 1] You just feelin' sorry for yourself
All that drinkin' bad for your health You been smokin' way too much
You been on the road too much You don't ever tell me how you feel
You don't ever wanna keep it real Lately, you been lookin' stressed out
You should prolly go and get out, ay You lost a lot of friends on the way
Heard them say that you been actin' fake Heard them say that you been fallin' off
When the fuck you gonna drop a song?
You should prolly go and get some help right now
Thinkin' you should do it for yourself right now
Need to start actin' like you care right now I'ma get the keys and take you there right
now
[Chorus] I don't belong here (I don't belong here)
I don't belong here (I don't belong here) I don't belong here, hey (I don't belong here)
(Bad, bad, bad) I don't belong here (I don't belong here)
I don't belong here (I don't belong here) I don't belong here, hey (I don't belong here)
Bad, bad, bad [Interlude]
I don't belong here I don't belong here
I don't belong here
[Verse 2] You don't ever look them in the eye
Always giving up before you try Saw you on stage looking shaky
Prolly why you fucking up lately (look at you, look at you)
Now you lookin' restless (look at you, look at you)
You gon' learn your lesson Why you stress you exes?
You know you ain't helpless I'm glad that you felt this
Quit looking for the exit Look at you
Disgusted, you've been disappointed ever since you dropped that one shit
All them fuckin sad songs, you ain't drop shit
You've been on that drunk shit (you, you, you, you, you)
That's enough, that's enough, that's enough
That's enough, tough love gon' hurt when you out of love
When you out of love
[Chorus] I don't belong here (I don't belong here)
I don't belong here (I don't belong here) I don't belong here, hey (I don't belong here)
(Bad, bad, bad) I don't belong here (I don't belong here)
I don't belong here (I don't belong here) I don't belong here, hey (I don't belong here)
Bad, bad, bad [Interlude]
I don't belong here I don't belong here
I don't belong here
-------------------------------------------
Incredibles 2 are Here | Disney Pixar | Elastigirl Dash Violet Frozone Mr Incredible Jack Jack - Duration: 30:05.
We are introducing the ever so funky family of super heroes, the Incredibles.
And they are back with a new movie Incredibles 2 and we are bringing them to the channel.
Of course, like all Disney Pixar animations, it took on a charming twist to what could
be a typical super hero genre.
Unlike other super hero stories, it's funny and family oriented.
So let's open them up.
First up is Mr. Incredible and Jack Jack.
His human name is Bob Parr and he is born with super powers.
In the Incredibles universe, these super humans are known as the Supers.
Mr Incredible is the most powerful superhero in his universe.
Is he a super dad?
Dun dun dun.
That is the biggest question in Incredibles 2.
Can he handle a teenage girl going through confusion emotions?
How about a hyperactive energizer teenage boy?
Is he ready to handle a Super baby with 17 super powers?
Oh did we mention, these kids all have super powers too?
It turns out he is not really ready to be a dad.
But who is?
He also wants to be a good husband for his wife too.
Can he do it?
I think what is interesting about the Incredibles is that the entire family is super heroes.
Everyone from a baby all the way up to the parents.
Not only do they run as a family, but the parents encourage their children to become
superheroes too.
Mr Incredible seems normal.
You would never even guess he is a superhero just by talking to him.
Something about him just seems like a next door neighbour.
He seems so likeable.
He seems normal as normal comes.
But he is anything but.
He is the strongest of the strongest.
He is born with super powers and he knows how to use it.
He is a strategist and a good fighter.
You mess with him then he comes back with his entire family.
Yep, that's Mr. Incredible.
The box advertises that these dolls are fully poseable.
He has seven points of articulation.
Mr Incredible comes with a very shiny red suit and it is sewn very well.
It definitely adds quality to the doll.
But here is the problem.
Mr. Incredible is a very big guy and has a disproportionately exaggerated body shape.
While his knees are intentionally kept such as it can't bend, perhaps so he can stand
on his own.
But rest of the joints are having problems moving too.
Mainly because his suit is too tight and also because of his weird body shape.
The arms really have no place to go.
I think it's good that the animators tried different body proportions rather than keeping
it same as other typical super heroes.
It comes with a mask and it took a few minutes to figure out how I'm supposed to put it on.
The mask just kind of sticks to the eyes.
It's not secured or anything, but the material kind of has a slight suction power and it
kind of stays put.
That's kind of cool.
Now let's go find some profiles for Mr. Incredible and Jack Jack.
Mr. Incredible
His human name is Bob Parr.
He lives in Metroville.
His super powers include superhuman strength, invulnerability and superhuman senses.
Superhuman Strength
According to the Pixar's Wiki, Mr. Incredible can lift in excess of 66 tons.
But he can lift a locomotive which weighs 153 tons, so he can lift some really heavy
things.
With his incredible strength, he can jump very high and run very fast.
Invulnerability
He can withstand a lot of physical trauma including multi-story falls, direct impact
of a train and even 100,000 volts of electricity.
Enhanced Senses
He can feel dangers before anyone notices.
His Abilities
He is a skilled strategist, skilled swimmer and an expert hand to hand combatant.
Weakness
He doesn't have any.
Cool.
Jack Jack
His human name is John Jackson Parr.
He lives in Metroville obviously with his family.
His super powers are...he has too many.
According to the wiki, he has 17 known superpowers.
Or at least that's how many his dad thinks he has.
So if he can keep all of his super powers into the adulthood, he is going to be the
most powerful Super ever.
He can shapeshift into a monster, teleport, and vibrate at a molecular level.
He can pass through force fields or solid objects.
He can crawn walls and he can chew through almost anything.
He can move objects with his mind and levitate.
He can shoot green lasers from his eyes and he can generate electricity too.
He can duplicate himself and change his size.
And he is pretty strong.
That's kind of scary if you think about it.
I hope he doesn't turn into a supervillain later in his life or that's going to be really
scary.
Next up is Elastigirl.
A mother of three kids.
A superhero in the day.
A supermom in the evening.
But definitely not someone anyone should mess around with.
Her civilian identity is Helen Parr.
What is interesting about Elastigirl is that she is not just Mrs Incredible and a mom,
but she has an identity of her own, Elastigirl, an identity she had before she got married,
which she kept even after.
I heard from someone that once you get married and have kids, you lose your identity as yourself
and people start to recognize you as somebody's mom.
And to certain extent, it happens to dads too.
That you are no longer called by your original name, but somebody's mom and somebody's dad.
It begs the question, who are you as a person after you have kids?
She is an interesting character, because what does it mean for a woman to be independent
and strong but is also a wife and a mom in our modern times.
Where do you cross a line between don't mess with me or else and being caring and nurturing
for your family.
A woman's role in our society has always been set within very narrow boundaries of what
is considered good and acceptable and usually that meant taking care of domestic needs.
Any behaviors outside of those boundaries are not only scrutinized by the other gender,
but also by other woman as well.
Mom as a superhero is pushing so many boundaries that it not only brings fresh perspectives
to the super hero genre, but also what is considered acceptable in our times for somebody
to be a woman.
The doll comes with 13 points of articulation.
She is wearing a beautiful red shining suit and she also comes with a removable mask.
Much like Mr. Incredible's mask, this one just kind of sticks and stays on top of her
eyelashes.
As I bring this character to my channel, I have to ask myself what do I do with this
character?
Is she going to be the main character?
Or is Violet going to be the main character?
But also some characters are more liked by girls than others.
And I discovered, that's a fine line to walk too.
Let's be honest.
Girls hate being boring.
Evil is good.
Trouble is better.
Chaos is to be desired.
But love conquers all.
Happy ending is better than sad ending but hardship is required.
Being mean is fun, but so is being innocent.
So then is Elastigirl kind of outside of what my audience can relate to?
That's obviously a question I need to figure out.
Elastigirl
Her name is Helen Parr.
Her superpowers are mostly like her name says is full body stretching.
She can stretch up to 30 meters, but with her full body, she could stretch up to 300
feet or 90 meters if she wants to.
She could also jump 80 feet or 24 meters by stretching herself.
She is also invulerable.
It's kind of hard to get injured because she can stretch so well.
She is bulletproof and she is good with hand to hand combat.
She can shape shift by stretching her body into different forms.
She can also change her body size.
Her weaknesses are blunt force, elasticity and freezing tempratures.
If she gets hit with a blunt force, she gets dazed.
And sometimes she has problems with her body getting stretched at wrong times at wrong
places.
She loses her powers in extreme cold.
Next is Violet.
I couldn't get Violet from the same toy series.
This one is from a different toy company.
The one from Jakks Pacific is completely sold out.
I guess people really like Violet more than the other family members.
So instead I got this one.
This one talks.
It says she has 30 sayings.
So let's open her up.
I suppose a superhero, specifically a teenage girl superhero has been tried before.
Fantastic Four comes to mind.
There is also Starfire and Raven from Teen Titans Go.
But probably she is the only one who runs with her parents.
She is the oldest of the three kids and naturally she is kind of given more responsibilities
than other kids.
Even in the first action scene in Incredibles 2, she's told to babysit Jack Jack, which
she kind of does but not really.
She has to juggle school, boyfriend, two annoying younger brothers and just kind of annoyed
with everything in general.
But who wouldn't if somebody had her life?
She is a character full of irony.
I think she wants to be a superhero, but she seems to be fed up with it too.
She likes Tony, but she can't express her feelings.
She wants attention but her superpower happens to be invisibility and force field.
I think she is an embodiment of teenage girls.
She wants attention, but not wrong kind of attentions.
She wants to be good, but not become a pushover.
She wants to express herself, but not criticized for it.
But most of all, I think she's trying to figure out all of the confusing feelings.
The thing is it takes time to embrace your gifts and truly realize what it means to be
different.
But when you are a teenager, being gifted is same as being weird.
You are the outsider.
Fitting in can be difficult.
Probably because her peers have no idea how to handle the difference.
Your gifts might look like an abberence.
So you act like everyone else.
She has 10 points of articulation.
Her head is supposed to move, but not really because of her hair.
Her torso rotates at the waist, her upper thighs do not move.
Probably because of the speaker and chips inside of her.
So technically she can never sit.
She comes with a shield and a doll stand.
So what is interesting is Violet is clearly a force to be reckoned with, but she doesn't
brag about her powers.
And people just don't notice her all that much, which adds to her insecurity.
I think she is an interesting character to delve into.
The doll operates with three button cell batteries.
On the back, there is an IR button, which interacts with other Incredibles in the same
line.
So if Violet says something, other Incredibles will respond back.
But we are not going to try that.
We are going to try the button in the front.
So let's hear what Violet has to say.
Bet you are wondering what it's like to create a force fields.
Well, I gotta admit, it's pretty cool.
How am I?
I'm super duper.
So how are things?
Seen any good movies lately?
That did not sound good.
I better put up a force field just in case.
Don't worry.
I got you covered.
Now that Jack Jack has unpredictable powers, I think my babysitting fees should go way
up.
So um this is fun.
What should we do now?
Dad.
You better not be in my room.
Come here quick.
I'll protect us with a force field.
Wow.
This might be the strongest force field I've ever made.
Hello.
Mom?
Dad?
Where is everybody?
Sometimes my family fights crime together.
I think it's kind of cool.
Don't tell mom and dad I said this.
But I think it's great the way they always put our family first.
Sounds like bad guy to me.
What do you think?
Up for some hero work.
Hi.
My name is violet.
It's nice to meet you.
Oh, hey, so you are a Super.
Me too.
Uh oh, I see smoke and fire.
Here.
This will keep us safe.
I'll hold up the bad guys.
You go get help.
Don't worry.
Nothing's getting through this force field.
Fighting crime with you wasn't too terrible.
We should hang out more often.
There are a few more sayings on the back in IR mode.
It has a few that we already heard from the front.
Look at you.
Aren't you adorable?
You don't know me.
Let's change that.
I'm Violet.
It's nice to meet you.
What do you say?
Up for some hero work?
I'm a super remember?
Check this out!
Sounds like a bad guy to me.
Come here quick.
I'll protect us with a force field.
I've got you covered.
Don't worry, nothing's getting through this force field.
Have you seen Jack Jack?
Now that Jack Jack unpredictable powers, I think my babysitting fees should go way up.
Wait.
You were supposed to watch Jack Jack.
Look at you.
Aren't you adorable?
Now let's go look up her profile.
Her name is Violet Parr.
She seems to be the only one in her family to use her first name as her superhero name.
She is 14 years old and she is shy, awkward and easily annoyed.
But she's also brave, reserved, sympathetic and strong.
Her super powers include invisibility and force field generation.
She can also levitate by creating a force field bubble.
Her invisibility power seems to only work on herself and only extends to her superhero
suit.
Her force field is nearly indestructible.
It can deflect bullets and in Incredibles 2, Violet has learned to use the force field
for offensive attacks too.
She is smart and adaptive.
She is also very athletic.
She is weak against blunt force.
If enough heavy force is applied to her force field then Violet can be knocked over.
Both of her super powers drain a lot her energy.
She can't turn her clothes or any other objects around her invisible.
Unless she is wearing her supersuit, her invisibility is kind of well...pointless.
Extremely corrosive substance can also penetrate her force field.
Next is Dash and second Jack Jack.
But mostly we are going to talk about Dash.
Dash is reckless and competitive.
He believes he should use his super powers to his advantage.
But when he's not allowed to show off his super powers, he goes a little rebellious.
He gets into trouble with her older sister Violet all the time.
I think to certain extent, Dash is an embodiment of a teenage boy as well.
He knows he is special.
He knows he can do incredible things, or at least he feels like he can.
He is bursting with so much energy that he can barely contain it himself.
But everyone else around him is telling him to stop and go slow.
While his messages are lost in chaos usually, he does seem to have a point most of the time.
He is good-hearted and playful.
But his impulsiveness gets interpreted in wrong ways.
Although he competes and gets into trouble with Violet, he cares for his siblings.
He tries to take care of his brother, Jack Jack.
During missions, he shows a lot of focus and cooperation.
He does have brilliant his moments although it gets lost in actions.
The doll comes with five points of articulation with a red plastic suit.
His name is Dashiell Robert Parr.
He is 10 years old.
His superpowers include superhuman speed, superhuman agility and reflexes.
He is also good with a combat using his super speed.
His speed is 190miles per hour or 305 kilometers per hour.
But maybe he's faster, because he can run on water or walls or ceilings.
He can react at a superhuman speed and improvises during his mission at a super human speed
too.
We don't know of his weakness yet.
Also Math doesn't seem like his favorite subject in school.
Now, let's talk about Edna.
She is a fashion designer.
Not just any fashion designer.
She designs for superheroes.
She takes a lot of pride in her work and she wants to be known as the best designer for
superheroes.
She is like Q from James Bond.
The Butler from Batman.
The brainiac who supports the superheroes and makes everything possible so superheroes
can be superheroes.
She hates capes and she hates supermodels.
She hates rival designers and well...hmmmm...I guess she has strong opinions about things.
She apparently has a brother who is a doctor and he also works exclusively with superheroes.
I guess they really understand the needs of the Supers.
When she designs the suit for superheroes, it's not just the looks, but it must be functional.
It seems like a lot of science and technology goes into making it.
When she was making the superhero suit for Jack Jack, she seems to be using a lot of
technology to either counteract or enhance the existing superpowers.
So while she is a fashion designer by title, she is also a scientist and an engineer.
Her dress is quilted and it feels really nice.
Somebody went through a lot of effort to sew her clothes.
Like a truly famous designer, the doll comes with a very nice outfit too.
The doll comes with 5 points of articulation and she comes with a pair of glasses that
makes her eyes look super big.
The eye glasses can come off, but it's kind of stapled in, so I'll just keep it there.
Her full name is Edna Mode.
She is half Japanese and half German.
She often calls herself "E", probably a reference to Q or M from James Bond.
She has a one brother.
Doc Sunbright.
She lives in Metroville.
We also have Frozone, the monster Jack Jack or
the Firey Jack Jack and the infamous supervillain none other than the Raccoon.
Oh who could forget the Raccoon.
But first, let's open up Frozone.
He is the best friend of Mr. Incredible and his super power is freezing everything.
He uses humidity and he turns water molecules in air and freezes them.
He moves around on top of his ice constructions like he's surfing.
He is always cool and laid back.
I know he is a side character and I know male dolls are generally made with less regard.
I guess the target audience for these dolls, or rather action figures, if they are made
for boys, is probably a much younger population where extra joints will just lead to more
breakage, but still...this is a little disappointing.
Edna who is equal in terms of being a side character got a wonderful outfit, but Frozone,
his elbows are kind of stuck in that position.
I don't know if I should be disappointed for Dash too, because Dash was also given only
five points of articulation.
Next,we have the monster Jack Jack and the Raccoon.
I supposed this isn't really the monster Jack Jack, but close enough.
Jack Jack can shapeshift and make himself look like anyone.
Him choosing to be a monster is I guess by choice, because he could've turned into anything
really.
Frozone and the Firey Jack Jack both have five points of articulation.
The raccoon on the other hand has six points articulation, because his tails move.
Kind of cool.
The raccoon is Jack Jack's first namesis.
Hey, who says being a supervillain is easy, especially when your opponent is Jack Jack.
In Raccoon's defense, he didn't start the fight.
Jack Jack did.
Is he going to let a little baby tell him what to do.
No way.
Raccoons have egos too.
Jack Jack won that round.
One to zero.
But life must go on.
As the raccoon nearly escaped from that faithful encounter, I bet the Raccoon shrieked and
vowed to return with vengeance.
His advantage?
We know nothing about him and surprise is on his side.
Frozone's civilian name is Lucius Best.
He is 40 years old and he is happily married to Honey, who only appears in voice.
He lives in Metroville.
I suppose almost everyone lives in Metroville except for villains or a few other minor characters.
His super powers include cryokinesis and super strength.
He can make ice from his fingertips.
He can quickly move by creating ice chutes which he creates by blasting ice right in
front of him.
He is not as strong as Mr. Incredible, but he has shown several instances where he seems
to have superhuman strength.
He wears special goggles and his boots can turn into skis or skates.
His real name is unknown.
His species procyon lotor.
They are known to walk the nights.
They scavenge garbage cans.
They are clever and mischievous.
His superpower unknown.
His weakness also unknown.
But clearly if he survived a fight against Jack Jack, he must have super powers, right?
His intentions unknown.
His likely time and locations for the next attack, unknown, but he will probably appear
near Jack Jack's residence near the garbage cans again.
And surely Jack Jack will be waiting for him.
Can he win against Jack Jack?
He will need to make very detailed plans.
He must observe and train.
He escaped from Jack Jack once and he can do it again.
After all, the night belongs to him.
When I was making videos with Super Hero Girls, some of you left comments that Super Hero
Girls are not Disney.
While I mostly use Disney dolls, I never restricted myself to just Disney dolls.
But this would be a good mix.
We are adding the Disney's super heroes too.
The Incredibles.
So here they are.
The Incredibles.
-------------------------------------------
Here & Now Wednesday August 29 2018 - Duration: 1:03:54.
For more infomation >> Here & Now Wednesday August 29 2018 - Duration: 1:03:54. -------------------------------------------
Here's How To Get VERIFIED On Instagram - Duration: 3:16.
For years the mystery behind the magical blue checkmark was the holy grail for instagram
users, but now, instagram will allow just about anyone to request a verification badge.
Welcome back to IO I'm charlotte dobre.
Over the last few years, I must have googled 'how to verify my instagram' like at least
10 times.
Theres something about that pretty blue checkmark that says, hey mom, I made it.
But the way to get verified was pretty much unknown.
No one really knew how to get verified, you definitely couldn't apply or email instagram
about it.
And if you did, they would probably laugh at your request and ignore the email.
Apparently there was also a black market where people charge thousands of dollars to hook
you up with verification, but more likely they would probably just take your money and
disappear.
What was clear though, in order to be verified, you had to be famous enough or instafamous
enough that the people at instagram had to know about you in order for them to verify
your account.
Or for there to be enough fake accounts claiming to be you.
Well friends, that is no longer the case.
Instagram has a brand new feature, where any user can request to be verified.
And here's how to do it.
First, go to your profile.
Then you gotta get to settings by clicking on those 3 little bars at the top right hand
corner.
At the bottom of that page, theres a settings option.
Click it.
Once in the options page, scroll down to request verification.
The next page is a form that says . A verified badge is a check that appears next to an instagram
account's name to indicate that the account is the authentic presence of a notable public
figure, celebrity, global brand or entity it represents.
Fill in your username, your full name, and then attach a picture of a government issued
piece of ID.
Like a drivers license, passport or national identification card.
Either that, or if you're a business, attach your utility bill, article of incorporation
or tax filing.
The piece of id has to include your birth date.
Then, you wait.
If your account gets verified, you'll get a notification, and then that beautiful new
checkmark will appear next to your name.
But then theres the catch.
Submitting a request for verification does not guarantee that your account will be verified.
And instagram will be focusing on accounts with a large number of followers.
But having a lot of followers doesn't mean that someone with a lesser amount wont get
verified.
I have a friend with 4 thousand followers and his account is verified.
I have other friends with over 100k who aren't verified.
So kids, go forth and get that pretty blue checkmark.
May the gram be with you.
Bomb – am I the only one who has a massive crush on charlotte.
Ohhh you guys know how to make a gal feel special.
Rodrigo Estrada – You should make bloopers a once every 2 to 4 weeks thing.
I actually don't mess up that often but I guess now I have a motivation to mess up?
We are thinking of making bloopers a monthly thing though so stay tuned for the next one.
Discord Night – Charlotte the scandal hunter.
I wonder if I can put scandal hunter on my resume.
Mercy – IO is coming for keemstars job.
Yep.
Hear that keemstar, I'm coming for YOU! he probably wont see this, he only watches
videos with his name or drama alert in the title.
I know this because he retweeted a recent video we did on him.
The video is over!
If you wanna keep watching IO, theres a playlist over here for you with a bunch of videos just
like this one, and obviously if you enjoyed this video, smash the like, subscribe and
notification bell, and I will see you in a future IO video.
-------------------------------------------
We Could Live Here! One of Our Favorite Cities | Milwaukee Travel Guide - Duration: 10:03.
have you ever visited a place and thought I think I could live here? well
that's how Kait and I felt about Milwaukee Wisconsin after our first
visit. the city left such a positive impression on us that we put it on our
list of favorite cities in the u.s. did I mention there's great beer and good
food and the city gets extra bonus points for being dog friendly Leo is
with us on our first trip to Milwaukee and needless to say the locals love them
you excited for bread?
I say let's go sit on the patio it looks delicious
we've got cranberry walnut bread and sourdough one of my favorite things to
do is to find a good local bakery always exciting to see what kind of baked goods
they come oh man knows food
the crust is perfect of course there's more to eat than artisan bread in
Milwaukee during our first visit and sampled beer
cheese sausage even a root beer float if Pizza is your thing check out
transfer pizzeria this pharmacy turned to pizzeria dishes out some of the best
pizza in the city we love their garlic sauce pizza topped with sausage and
pepperoni it's fantastic pepperoni is thick cut and spicy
transfer pizza good job for brunch any day of the week head over to Blues egg
and snag a seat at the bar Kate's favorite is their house-made English
muffin served with whipped butter and jam although our diets have changed
since our first visit to Milwaukee we got to revisit some of our favorites
including the Wisconsin cheese Mart and despisers
okay gotta feed the meter how much time should I put in dollar fifty an hour
let's do this
I love the smell of this place smells amazing stocked up on all of my favorite
spices now I can officially begin testing and working on the cookbook love
the spice how
really fun to be walking through this area again because we were here on a
food tour the first time we were in Milwaukee it's fun to pop into the
cheese shop it just wasn't really good it's really
good to cheese travel as well but I love this cheese sign we'll have to take a
picture in front of it
ooh smoked Wisconsin cajun Gouda
I don't think I've ever had this much cheese and we ate at mater's on the food
tour that was a lot of fun sauerbraten cabbage
before we move on let's talk about the Friday fish run live music and a great
beer to go with your fish head over to lakefront brewery while you're here you
may as well go on their brewery tour complete with samples and a good laugh
we had a great tour with Kyle I was introduced to a new beer the Arturo and
it is absolutely fantastic I think we're gonna go back inside have a couple more
enjoy this wonderful day in Milwaukee by the river and see what else this leads
us to you know there's no shortage of festivals in Milwaukee but there are
several to choose from one day we stumbled upon the Brady Street festival
in full swing with live music on multiple stages
a festival in Wisconsin wouldn't be complete without a cheese curd eating
contest although I couldn't convince Kate to sign up we did stick around to
watch the contestants chow down on their to pound a pile of cheese curds then we
proceeded to sample every cheese under the tent including the ghost pepper
cheese
after watching all those people trying to eat two pounds of cheese curds I
decided I had to try some squeaky Milwaukee cheese curds good
I can't imagine eating two pounds though no I'm glad I didn't sign up for that
contest it's a good thing Milwaukee is a very walkable city because a leisurely
stroll was an order after all the cheese Kate and I went for a walk along the
Milwaukee River where we hung out with the fond and hopped on a relaxing cruise
around the city
if you're looking for more adventure kayaking is a great way to experience
the city and its architecture Kate and I went on a four hour kayaking trip the
first time we visited Milwaukee and it's still one of our favorite things to do
the challenge with a great city is there's way too much to do and not
enough time if you're a motorcycle enthusiast like me a trip to the
harley-davidson museum is important if you're interested in history a tour of
the Cape's mansion is a must the guided tour reveals some fascinating history
about the taps family along with a detailed tour of their 20,000 square
foot mansion time to grab a cold one over at best place and learn more about
how Pabst Blue Ribbon came to be what's the word of the day
Gambrinus it means to be content with a stomach full of beer all right which way
ice-cold beer this way gift shop this way this way
ice-cold PBR pregnancy so far this has to be one of the best brewery tours
because we're not learning about how the beers made we're learning about the
company itself this little nugget if you don't know what that is look it up find
out what the B stands for very interesting story let's go while we're
on the subject of brewery tours we had a meet up at MKE Brewing Company with our
WT our camp head brewer Kirk gave us a private tour with several refills along
the way including a sample of OG a beer beloved by the locals and one of Kurt's
creations Kate and I have been on quite a few brewery tours and they're all a
bit different MKE brewery it's a glass and ham tour where you can sample as
many beers and as much beer as you can handle that's a nice thing about having
home with us we can walk back to the van and take a nap when we're done but
there's no time for rest and relaxation German Fest is in full swing
curtain family offered to drive us to the festival where we devoured not one
but two giant pretzels I've loved bugs ever since I was a
little kid but I particularly liked the Baha bugs and we actually thought about
towing one behind the van and it's still something I want to get to this day so
that is one cool vehicle behind me and if there aren't already enough reasons
to go to Milwaukee here's another one to add to the list a live glockenspiel
performance at German fest
granted we've never experienced the winters in Wisconsin but we enjoy
Milwaukee so much we just might plan a winter road trip to get the full
experience
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RHONY: Is Andy Cohen Scared Of Bethenny Frankel? (Season 10, Episode 21) | Bravo - Duration: 2:53.
For more infomation >> RHONY: Is Andy Cohen Scared Of Bethenny Frankel? (Season 10, Episode 21) | Bravo - Duration: 2:53. -------------------------------------------
Bow to Blood and Torn are here! | PS VR - Duration: 0:31.
[SLOW MUSIC PLAYING]
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Deputies pulled over a car and found a baby not breathing. Here's how they saved him. - Duration: 3:57.
By Lindsey Bever August 29 at 1:13 PM A squad car came to a hard stop outside a hospital in Southern California, and a sheriff's deputy hopped out carrying an infant
Officials with the Los Angeles County Sheriff's Department said the 9-month-old boy had been discovered unresponsive Monday during a traffic stop — prompting a team effort to save him
Authorities said that one deputy began driving the child to the hospital while the other deputy worked quickly to resuscitate him, performing CPR inside the patrol car until the child started to breathe
Video obtained by ABC affiliate KABC shows one of the deputies running into an emergency room with the child in his arms
"What a heroic and lifesaving action," Capt. Richard Harpham of the Los Angeles County Sheriff's Department said of their efforts during a news conference this week, according to KABC
[Watch two police officers save a choking baby in a busy intersection] Harpham said Deputy Tyler Milton saw a vehicle been driven "erratically" about 10 p
m. Monday in Lakewood, outside Los Angeles, and pulled over the driver. When he stopped the car, a parent jumped out and alerted Milton that the child was not breathing, Harpham said
It's not clear why the child, who has not been publicly identified by police, was not breathing and unresponsive
"Deputy Milton sprung into action, grabbed the 9-month-old, summoned help from other deputy sheriffs," the captain said
#LASD Lakewood Sheriff Station Deputies Save Baby's LifeOne Deputy began administering CPR as a second deputy drove to LB Memorial Hospital
Our heroes were able to resuscitate the infant prior to arriving at the hospital
The infant is listed in stable condition. pic.twitter.com/Ub8jjblynJ— LA County Sheriff's (@LASDHQ) August 28, 2018 KABC reported that another deputy, Alissa Farrington, answered the call and transported Milton and the child to nearby Long Beach Memorial Medical Center — in what authorities called a "coordinated effort
" The captain said that as they were rushing to the emergency room, they radioed other deputies as well as Long Beach police officers, who blocked intersections for them so they could get through
Meanwhile, Milton was in the back seat, performing CPR on the child. Officials with the Los Angeles County Sheriff's Department said in a statement that Milton was able to revive the child and get him to the hospital
The child was listed in stable condition, authorities said. The hospital did not immediately provide a status update Wednesday on the child's condition
The deputies were not available for an interview Wednesday with The Washington Post
Read more: How an Idaho couple survived 30 cold and wet hours trapped in an ice cave She sent her husband a 'nagging' text about the car seat — and it may have saved their son's life
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Experts shut down Trump's conspiracy theory about Google -- here's where he really gets shredded - Duration: 2:03.
Donald Trump opened up a war with Google on Tuesday, starting his day with a rage tweet about his search results, apparently inspired by YouTube stars Diamond and Silk
Google's algorithm is a closely guarded secret and evolves constantly. No one gets the exact same results, as the search engine also looks at user's past behavior and location
However, as the Washington Post points out, experts have shown that conservatives and liberals see no significant differences in results
But location is a major factor in results. The Post ran searches for Trump from Europe and found that his results were even more brutal without having Fox News and Breitbart to buttress them
In Italy, one of the top results was headlined: "Donald Trump says that Google is rigged but maybe he does not know how it works
" Then a story about Aerosmith banning Trump from using their songs and Harry Potter author J
K. Rowling trashing the American president. German media trashed Trump from both the left and right
German Conservatives dislike Trump's style and the way he's made it impossible for the opposition to work with him
Poland's version of Newsweek has been railing on Trump, and last week's cover compared Trump to the Polish Prime Minister, saying both are "liars" and "the exact opposite of Senator McCain
" Read the Post story here.
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Comfy 3: Hoodie: Here is Why the Best is Yet to Come - Duration: 11:37.
Comfy Clothes Chic.
You can have both style and comfort, learn how to wear and style the most comfortable
pieces in your wardrobe.
Let's chillax and give thanks to our extra comfy clothes that are like Chicken Soup for
the Fashionista Soul.
Toss aside your high heels, throw out your spandex, and relax into these stylish ensembles.
Look at all the comfy clothes we are going to focus on in this style series.
We're going to look at fleece, hoodies, thermals, muumuus, even sweatpants, and of course, leggings.
So in the first two videos of this series, we've actually already covered two of the
topics.
So we've already looked at the two style ingredients of fleece and thermal, so if you're interested
in learning how to create chic outfit from those comfort pieces, just go back and watching
those videos.
In this particular video, in this episode, we are going to focus on the hoodie.
Now, let's get the rundown on the basic stats of a hoodie.
So it's super warm, like a lot of the items featured in this style series, it's perfect
on a bad hair day because you can just put up the hood and run around and do what you
have to do, and really, when you have the hood on from your hoodie, it's better than
a keep away sign.
People just leave you alone.
They think you're a badass, so for all those reasons, I love hoodies.
This would be the point, if this was a traditional-style series, where I show you super cute outfits
featuring very cute pieces that you probably don't have in your wardrobe.
So I'll show you a sweatshirt like this with gold lettering.
I'll show you a hoodie like this with the side zip or diagonal zip, and even another
hoodie in a very pretty sea green color, and then you would go, "Those are beautiful, Aisha,
but that doesn't look like anything I have in my wardrobe.
My sweatshirt is left over from high school PE, or my sweatshirt, I got for free for a
5k that I signed up or but never completed because I faked an ankle injury," so if you're
one of those people that you have hoodies that you have just acquired by various circumstances
but not have locally gone out and bought hoodies, and that is many of us for the items that
make up the comfort pieces in our wardrobe, I have outfits for you as well.
So even if you don't have the pretty ones shown here on the screen, we can make do with
what you most likely have in your closet.
This is the standard grey hoodie.
I think Rocky Balboa wore this in the first Rocky movie.
So this is the most basic, standard hoodie you could have, and most of us have a variation
of this in our wardrobe, so I'm going to give you a way to where you can look comfortable
and be stylish.
So the key to this is that you're going to wear a super smoking-hot leather jacket over
it.
So you're going to wear the leather jacket over it and then you're going to pull out
the hoodie over the collar of the leather jacket so it hangs out back, and you can see,
to make this stylish, black jeans, black moto boots, you can see there's not really a heel
on them, super comfortable to walk around in all day, and then just very simple earrings.
This is a so-easy outfit that most of us have black jeans, most of us have a pair of boots,
and most of us especially if you've been following me for a while, you should at least have a
black leather jacket.
You just put that all together and you're ready to just run around on the weekends and
you'll look stylish and put together, and really, you're wearing the most comfortable
pieces possible.
So there's outfit number one.
So again, I'm not going to give you an outfit with a super fancy colorful print sweatshirt.
I used the most basic line most of us have in our wardrobe.
Again, here is a gray hoodie, right?
How can you make this stylish?
And again, I'm going to turn to inspiration from workout wear.
So especially a lot of us go and do our workouts on the weekends anyways and a lot of times
you have to run errands or you have to go meet somebody afterwards.
You may not have time to change or you don't want to run into Mr. Right and you're looking
like Mrs. Wrong, right?
So you want to still look put together, so you have the grey sweatshirt, and the pieces
that we're going to add to it, we're using pops of different neon color in this one to
really make this outfit.
As I said in other videos, a lot of times when you are wearing these comfort pieces
and then you want to make them stylish, you're going to have to maybe up your accessories
game, so you see here, we have the neon yellow purse again.
There's pops of neons, so there's that and you see the neon pink shoes that are there.
They are just sneakers.
You have on a pair of workout pants, they're the capri style one.
I have a fun watch that I put with it, a striped watch, super fun.
So you have these just pops of personality and color with this outfit but you look at
it, the essentials of this outfit is a grey sweatshirt and black workout pants, okay?
Then, I have just simple stud diamond earrings that you would put in your ear.
But again, it's stylish all when it's put together because it is little bit thought
out, that you're like, "Okay, I'm wearing basic black and grey.
I want to do pops of color and then do something super fun like, "Oh, how can I have pops of
neon color?"
So again, you're wearing a sweatshirt but you're going to up your accessory game, you're
going to up your handbag game, and for this, I love it because it's a big purse, you're
running around in the week and doing errands, going all over yonder as they say to do a
whole bunch of righty things and you need that space, so that handbag, so why not get
really a stylish one to help dress up the rest of the outfit?
It's full of personality, super stylish, you will feel bad at all running into Mr. Right
wearing this outfit.
And at the beginning of this series, I said, this was all about giving thanks for all of
the comfort we've received from the clothes in our closet but I also wanted to share a
story of when someone provided me with comfort.
I'll tell you in a little bit why I've been Incorporated this into the series but later
on, I'll offer you to an opportunity to say thank you to people that have provided you
comfort over the years.
So for this episode, here is the story of comfort.
So we all have those very early relationships, whether yours was at the end of high school
or the beginning of college.
I remember it's like my first real boyfriend.
We had got cell phones so that we can talk to each other and not run up the phone bills
too much, and it was one of those where you have some friends and family plans and you
can put different people on it so it wasn't too much to call them, and then they had a
cell phone plan where if you guys were on it together then it's free for anybody that's
on the plan for you guys to call each other.
So I think it was one of the summer breaks between college and me and that boyfriend,
I was like, "Hey, why don't we do that?
Why don't we get a plan together?"
We've been together all ready for I would say one or two years, and so it was like,
it just makes sense to do, right?
We've been together for a long time and you're young, and you just think you can be together
forever, and so I thought I'd ask him, "Why don't we do that," and he was very hesitant
and didn't want to do a cell phone plan with me, and I remember, I was so hurt.
Like I said, I was home for spring break and my younger brother knew that I was upset and
what was bothering me that my boyfriend at the time didn't, to me, feel like he didn't
think enough of our relationship for us to get a cell phone plan together so that we
could talk to each other whenever we want, and my younger brother came in the room, I
remember, he came in and he was like, "Oh, don't be upset about that.
He's just not ready for that.
It has nothing to do with you.
You shouldn't feel bad about it," and I'm like, how did my younger brother get so wise?
I was probably crying or on the verge of tears but like I said, my younger brother came up
and gave me that advice like, "It has nothing to do with you.
He's just not ready for that yet.
You shouldn't feel bad about yourself for thinking it has anything to do with you."
So that was very cold but he took time out to say that to me.
It was interesting to see your younger brother full of wisdom and what he said did actually
make it feel a lot better and it wasn't a big deal to me anymore.
So for that, I'm thankful for that moment of comfort and many others that my brother
has provided over the years, very appreciative of that, and so in hearing some of these stories
in this series, it is making you think of these moments when people have comforted you.
I will offer you the opportunity to send them a thank you.
You can send them a greeting card slideshow to tell them exactly how you feel about them
and what you appreciate they have done for you, and by doing that, you will have the
opportunity to win a scarf.
So in this series, like I said, you're wearing the comfortable clothes.
a great way to up your style game doing that is wearing accessories, and what is cozier
or more warm than a scarf?
So we're giving away scarves in this series and I will show you exactly how you can enter
to win a scarf and how you can send that greeting card slideshow to somebody that's very special
to you and that has comforted you before.
Just let them know exactly how you feel and to say Thank You.
So this is very excited to do that in this series.
Use the link below the video to access the slideshow.
Scroll down, fill out the form with your email address.
Scroll down, let us know who you sent the greeting card to.
Check the box that says, "I'm not a robot," and then hit submit, and you'll be entered
to win a comfy scarf.
To view this slideshow, it plays on its own but you can also use the arrows to forward
through the different slides.
You'll be able to open it in full screen to read all the text
or just look at it in the regular slideshow format.
You can share the slideshow on Facebook, just click the Facebook button.
It'll pop up in an open window and click post to Facebook.
You can also tweet this out, just click on the Twitter button at the end of the slideshow
that.
It'll open up in a new tab and you hit the Tweet button and you can share it with your
followers.
You can also highlight the link at the bottom of the slideshow and just paste it into any
email or into any social media platform to share.
Remember to fill out the form and forward the slideshow to someone that you want to
say thank you to.
Fill out the form, hot submit, and you'll be entered to win a scarf for this Comfy Clothes
Chic series.
Don't forget to do it, and that'll be it for this episode of Comfy Clothes Chic.
Sad to say, this is the end of this episode but I will see you on the next one.
Thank you for watching.
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Dragon Con is more about the costumes: Here's how they also give back - Duration: 0:58.
For more infomation >> Dragon Con is more about the costumes: Here's how they also give back - Duration: 0:58. -------------------------------------------
Neighbor's dog keeps barking? Here's how to solve it - Duration: 1:17.
For more infomation >> Neighbor's dog keeps barking? Here's how to solve it - Duration: 1:17. -------------------------------------------
Flu Season Almost Here - Duration: 1:26.
For more infomation >> Flu Season Almost Here - Duration: 1:26. -------------------------------------------
If Trump slaps auto tariffs on Canada, here's what you'll pay - Duration: 3:04.
For more infomation >> If Trump slaps auto tariffs on Canada, here's what you'll pay - Duration: 3:04. -------------------------------------------
Here's why the ethical priorities of the Catholic Church are so badly warped - Duration: 9:16.
As Pennsylvania investigators worked to confirm up to 1000 cases of sexual abusecommitted by Catholic priests, a panel of Catholic ethicist-theologians appointed by the bishops was also hard at work
Like the Pennsylvania team, the panel serving the bishops sought to ensure that Church-affiliated institutions weren't ignoring sexual evils
Good on them! you might think. They're finally taking responsibility for the mess created by their obsession with priestly abstinence
You'd be wrong. Bad, Bad Birth Control The goal of the panel wasn't to investigate, punish, heal or prevent child sex abuse
It was to ensure that Catholic-controlled healthcare systems don't look the other way while doctors and other care providers offer contraception, vasectomies, tubal ligations, or abortions (or sexual transition care or death with dignity)
The panel concluded that the bishops must prevent these evils in any institution where they have a say, including secular hospitals that have been acquired by or affiliated with Catholic healthcare corporations
In the past, mergers between Catholic-owned and secular hospitals have sometimes carved out separate legal entities to allow continued provision of reproductive and end-of-life services that are prohibited by the religious directivesgoverning Catholic healthcare "ministries
" (As an aside, it should be noted that Catholic charities and hospitals mostly serve non-Catholic people
Further, they obtain less than five percent of their revenues from Catholic offering plates, the bulk coming from taxes and private insurance
It should also be noted that they provide less free care for poor people on average (2
8 percent in 2011) than do public hospitals (5.6 percent). Church control is largely an artifact of history and accumulated wealth
) At this point in history, the irony of the bishops posturing as ethical authorities in matters of sexuality is lost on no-one but the bishops themselves
A New York Times article on care refusals in Catholic hospitals provoked over 400 comments expressing outrage from Catholics and non-Catholics alike
Have they no shame? More Members, More Guilt, More Abuse The Church has long sought to control sexuality and procreation, of course
Competitive breeding, sanctified by theologies that glorify childbearing, has been part of the Church's expansion strategy since the origins of Christianity
"Women will be saved through childbearing," promised Christianity's most noted early misogynist, the Apostle Paul
By the Middle Ages, the Virgin, Madonna, Whore trichotomy—a woman must be one of the three—was well ensconced, shored up by stories of virgin martyrs and saints
As for sexuality itself, what better marketing strategy for an institution selling absolution of guilt than to persuade people that most sexual desires and pleasures are sinful? The Church's antagonism to contraception (it allows sex for pleasure) and abortion (it enables women to refuse childbearing) and trans care (it garbles the carefully-delineated theology of gender binary) all can be seen as extensions of two age-old institutional objectives: more members and more guilt
Problem is, the second of those—the guilt—worked a bit too well, so well that it backfired, creating a culture of secretive, abusive sexuality
Why Such Warped Priorities? You might think that excising this festering socially-transmitted infection, making amends to victims, and transitioning to a married priesthood that recognizes the range of healthy human sexualities might be a full-time focus for the Catholic hierarchy right now
So, why have the bishops decided that now is the time to instead double down on judging such contrived sexual sins as using or inserting an IUD, or terminating an ill-conceived pregnancy, or getting the snip, or removing an unwanted uterus? Sigmund Freud would have had an answer for that
He might have called it reaction formation. Freud would have said that the Catholic hierarchy's shame and guilt and outright horror at their inability to suppress their own sexuality has lead them to try even harder at controlling the sexual and reproductive lives of others
Most people are familiar with Freud's concept of defense mechanisms, including, for example, denial (I do not have a problem), repression (I can't remember), and projection (the problem isn't me, it's you)
Reaction formation is part of the same list, but often less understood. Here are some excerpts from the Wikipedia definition: The concept of reaction formation offers one hypothesis about why the Catholic hierarchy is willing to go to such extremes to suppress and control the sexuality of people who aren't part of that hierarchy, whether through refusing medical services or through propagating harmful myths and stereotypes about sex that affect many of us
Freud had a lot of ideas about child development and the human subconscious that have turned out to be wrong
(Even a science as squishy as psychology is self-correcting over the course of a hundred years
) But what's interesting about this particular situation – the problem of the bishops and their sexual obsessions—is that Freud developed his hypotheses about human nature in precisely the kind of situation that Catholic dogmas have created: a culture that is highly repressive of sexuality, one that layers on rituals and rules in an intricate system of proprieties aimed at distancing us from our animal nature
In Freud's case this system was the European culture of the Victorian era. But the parallels make his analysis particularly relevant when it comes to making sense of the inside-out, upside-down "ethical" priorities of the Catholic bishops
Just Say No Part of being psychologically healthy is learning to recognize when other people are putting their stuff on you, and learning to say no–kindly when possible, but firmly
The Catholic bishops need to be told that their sexual hang-ups are not ours and that they cannot force their harmful theologies on the rest of us, especially not on the public dime, and especially not in healthcare systems that disguise their religious affiliations and where we may be sent unknowingly
When we are sick or injured and seeking care, we are vulnerable, especially at the beginnings and endings of life
We must be free to make important and even sacred decisions according to the dictates of our own conscience with support from people we know and trust
It's time to ask the bishops to leave our bedrooms and hospital rooms. Valerie Tarico is a psychologist and writer in Seattle, Washington
She is the author of Trusting Doubt: A Former Evangelical Looks at Old Beliefs in a New Light and Deas and Other Imaginings, and the founder of www
WisdomCommons.org. Her articles about religion, reproductive health, and the role of women in society have been featured at sites including The Huffington Post, Salon, The Independent, Free Inquiry, The Humanist, AlterNet, Raw Story, Grist, Jezebel, and the Institute for Ethics and Emerging Technologies
Subscribe at ValerieTarico.com.
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National Slinky Day is here and it's awesome - Duration: 2:17.
For more infomation >> National Slinky Day is here and it's awesome - Duration: 2:17. -------------------------------------------
Prion Disease/CJD (Creutzfeld-Jakob Disease) Basics - Brian Appleby - Duration: 33:43.
so I'm just going to go over a little bit of just basically what prion disease
is what a prion is. So a prion stands for a protein that's infectious most
infectious organisms have DNA or RNA-- what we call nucleic acids-- and prions
are unusual in that they are transmissible but they don't have any
DNA and RNA so they're kind of a group all into themselves and they're not
degradable by typical sterilization so in order to sterilize you have to use
specific procedures and the way prion disease work is that we all have normal
prion proteins that's the yellow PrPc that we have in our body most of it's in
our brain some of it's in our stomach we don't know exactly what it does but it
probably has some housekeeping function in the brain and we all have that and
then for whatever reason we come across and infectious prpsc which stands for
scrapie protein that's the prion protein that causes the disease comes in contact
with the normal prion protein and then it converts the normal prion protein
into itself in kind of this auto catalytic cycle so in order to have
prion disease you need to have PRPC or normal prion
proteins--and this is important specifically for Dr Collinge's talk
tomorrow, when he talks about his treatment study and the importance of
blocking PRPC. so just keep that in mind once you have the abnormal prion
proteins they tend to aggregate and form seeds and then they cause things like
oligomers and fibrils and you get this kind of exponential curve that you see
where you get rapid spreading so that's what happens at the molecular level. if
you look at the brain tissue you can look at it in a variety of ways one of
the ways it's called H&E staining where the brain develops these holes or
vacuoles which gave it its original terminology of a spongiform
encephalopathy because the brain resembles spongy tissue then you can
also do immunohistochemistry and take an antibody
that binds to the abnormal prion protein and stain for that and that's what
those brown deposits are in the right hand slide is the abnormal prion protein
itself so when someone goes for autopsy at the surveillance Center both of these
are done and that's how we screen for prion disease and brains so animals also
have prion diseases and sheep and goat they have a prion disease called scrapie
which came about because one of the symptoms that sheep and goat get is they
get this itching and it causes them to scratch up against surfaces and then
cows get something called bovine spongiform encephalopathy what is
sometimes referred to as mad cow disease and then in the u.s. we have something
called chronic wasting disease which is a very unusual prion disease that affect
deer elk moose and caribou and at the end of the talk I'm going to talk
specifically about CWD and why we care about it specifically as family members
and also scientifically especially in the US. so if you look at the
epidemiology or the occurrence of CJD in the american population actually
across the world the one statistic that's usually thrown out is the one per
million statistic and this is something called an incidence and what it means is
that there's one new case per million individuals per year across the entire
population but as we'll learn later not everyone gets CJD in fact it's usually a
mid to late life illness and some people live longer than a year so that
statistic whereas it's important for public health because that's how you
look at incidences of the disease psychologically it makes it sound like
it's much rarer than perhaps what it is. so perhaps a better way of framing how
common the disease is is to look at how many deaths per year occur in the u.s.
due to prion disease or CJD and if you look at that statistics it's about one
in every six the one in every 10,000 US us deaths are due to prion disease so
that's not a common disease but it certainly is maybe not as rare
that one-in-a-million number may initially make you think so when you go
to your legislators on Monday I would advocate that you use the one in 10,000
deaths per year of CJD as opposed to the incidence members of one in a million
because most people are going to realize what incidence is so to take an example
of Ohio we have about ten and a half million people so if you look at the
incidence we would expect about ten and a half new cases per year as we'll talk
about later about 25 percent of cases lived longer than a year so you would
have about two and a half cases carried over from the year before. so if you look
at kind of a low estimate it wouldn't be unusual and actually be expected to have
13 active cases in Ohio alone at any given time. so again this is not a common
disease but I think it's a much more common than people might think again
from that one-in-a-million number. if you looked at the causes of prion disease,
most people especially in the medical community and in the media know it to be
an acquired illness mostly from the BSE epidemic or mad cow epidemic but in fact
the vast majority of human prion diseases are due to sporadic illness and
what we mean by sporadic illnesses we think what happens is the normal prion
protein sometimes undergoes of spontaneous Misfolding on its own and
converts them to the abnormal prion protein and starts that Auto catalytic cycle
that we talked about in the first couple of slides but it's not acquired from an
infection that we know if it's not genetic it seems to happen on its own
accord so that's about 85% of cases. about 10 to 15% are due to what we call
genetic etiologies and that could be genetic CJD, fatal familial insomnia or
GSS and I'll talk specifically about what those different genetic prion
diseases are later and then by far the least common cause of prion disease are
the acquired forms and that's well under 1% and that includes things like kuru
iatrogenic CJD and variant CJD which we'll talk about
later so if you look at the age of onset of the disease it tends to be a disease
of middle to late life but if you break it down by etiology you do see certain
peaks and that is variant CJD in general tends to occur and much younger people
so people in their teens 20s and 30s genetic CJD tends to occur more in
midlife like 50s and 60s and then sporadic CJD tends to be more of a later
life illness with a mean age of onset of about 62 but it's important to remember
that there's a lot of variation in between so for example we've seen
sporadic CJD and people as young as 13 or 14 and we've seen variant CJD and
people as old as 50 so you know this is just a generalization there's a lot of
variability within it the oldest in the US was 98 sporadic yeah so if you look
at how long people survive with prion disease as you well unfortunately all
know it's a very quick illness. the vast majority of people pass within one year
but there are some cases that live longer than a year about a quarter some
of them can live to three years but again that's unusual
the vast majority will pass away within four to six months of the symptoms
starting and many of you are well aware of the symptoms of CJD
what you may not be well aware of is that CJD can present in a variety of
different ways so in fact people very rarely present with what we would call
the classic CJD presentation which would be cognitive
problems or dementia problems walking and steadiness and the myoclonus or the
jerking movements that's only going to be a presentation in a very small subset
of people a lot of people will just present with cognitive problems so they
may be misdiagnosed as having Alzheimer's disease or another form of
dementia some may affect with very sudden visual changes and may be
misdiagnosed as having stroke some may just present with what we call
ataxia or an unsteady gait and be misdiagnosed as something else so in
general the rule that I have is there's no rule in how a CJD can present we
certainly seen a variety of different presentations but at least as time goes
on they tend to look very similar but of course you don't have that luxury in the
beginning of the illness. so diagnosing CJD it's a clinical diagnosis in life
the only way to definitively diagnose it is by looking at the brain tissue
preferably at autopsy and we do this by looking at the clinical symptoms so
most patients will have dementia many will have myoclonus or that's the
twitches that we talked about They'll have cerebellar or visual symptoms so
cerebellar symptoms are unsteady gait almost like a drunken walking or they
may have incoordination and visual symptoms can be a variety of things like
visual hallucinations or it could be a depth misperception they can have
weakness and tremors and sometimes even have parkinson's-like symptoms of kind
of a shuffling gait and then at the very end of the disease people reach a state
of what's called akinetic mutism which is a lack of voluntary speech and
movement unfortunately rather not specific as you see this in most end
stages of dementia but you typically do see this in most
cases of CJD towards the end and then we use a variety of different diagnostic
tests but we'll go about in detail after this one of them is called the
electroencephalogram or EEG which looks at brain waves and there's a finding
called periodic sharp wave complexes that can be suggestive of CJD and I'll
show you an example of that and then there's a variety of different spinal
fluid tests that can be done the who classification World Health Organization
classification only includes one of them currently called 1433 but a variety of
other different spinal fluid tests are also now used and
we'll talk about them and then the brain MRI also can be suggestive of CJD
by having different abnormalities in different areas of the brain on a very
specific sequence called diffusion-weighted imaging and I'll show
you an example of that as well so this is an example of periodic sharp wave
complexes on the EEG. EEGs --I agree with Richard we were talking about this at
the last session-- become kind of unhelpful in the diagnosis of CJD
currently but it is important to rule out other things like seizures so we
still use that. this is an example of a brain MRI of a patient with sporadic CJD
the specific sequences that we like to look at is called diffusion weighted
imaging and that's done on most MRIs nowadays and we look at two specific
areas and CJD one is the basal ganglia or the middle part of the brain and that
tends to show brightness or it could be the outside part of the brain called the
cortex in kind of this cortical ribboning pattern and this can be quite
suggestive of CJD in the appropriate clinical context meaning that the
patient looks like they have symptoms of CJD
and other things have been ruled out I think in some cases the brain MRI is
sometimes then the first initial suggestion of CJD so one case that we
had many years ago was a case from DC that presented to us four suspected CJD
and I examined him and he was pretty normal he had a normal memory testing
his exam was normal and then we looked at his MRI and this is actually his MRI
his only complaint was he used to do yoga and he could only do head stands
for two minutes every morning whereas before he could do it
five minutes and the only reason he had gotten an MRI was because he had been in
a bike race and he fell off and hit his head and the neurologists ordered a
brain scan and this is what happened so of course this is a case of someone who
had an MRI that looked like prion disease and a patient who did not look
like to add prion disease and this was how the suspicions arose
and then we followed the patient over time and they they did develop prion
disease and past two months later so in certain circumstances I think this has
led to an earlier diagnosis in some cases when clinicians may not have
suspected the disease so in that regard I think it's been helpful and then there
are a variety of different spinal fluid test there's two spinal fluid test that
we use that are kind of just markers of brain cell damage and that's 1433 which
at the surveillance Center is generally reported as positive negative or
ambiguous and then we have the total tau protein which is reported as a number so
you could have a tau protein level of one or it could be in the tens of
thousands and in general the higher the number sometimes the more suggestive of
CJD it can be in the right patient but these are only markers of brain cell
damage so they can be elevated in other conditions so for example people that
have seizures at mass brain tumors head injury they can all have elevated
markers of brain cell damage so it's not very specific for prion disease itself
and then we recently started using a very disease specific test called
real-time quaking induced conversion otherwise known as RT- QuIC which
actually detects the abnormal prion protein itself so it's kind of the first
disease specific marker that we have and you're going to hear a lot about that in
the conference this weekend and people doing this test on a variety of
different tissues so doctor Zanusso will talk about using it on nasal epithelium
Dr. Zhu will talk about using it on skin so it will pop up a lot and it's
very specific for the disease itself because it looks at the abnormal protein
the way it works is they take a plate with normal prion proteins in it and
then they take a sample that presumably has abnormal prion protein in it and
then they label it with what's called Thioflavin T and put it for lack of
a better term a shake and bake oven --sorry dr. Rhodes that I just described
as that -- but and what will happen is it kind of
exploits that prion paradigm where it converts normal prion proteins into
abnormal prion proteins and you start getting aggregation of these proteins
that the star fluorescent binds to and then you can see that and that's a
positive test that lets you know that there's presence of abnormal prion
proteins in there. now, the reason why it's rather specific is we would not
expect to see any abnormal prion proteins and any other condition so
that's its power and then when we talk about different diagnostic tests we
often talk about things like sensitivity and specificity. Sensitivity is how
good a test is at detecting a specific disease and generally and that's not a
specific problem in our field because we have a lot of tests that have relatively
high sensitivity. it's the specificity that until fairly recently has been a
problem and specificity is how sure you are that the disease you're trying to
detect is the actual disease that you're trying to detect. so in RT-QuIC the
sensitivity is high at 95% and relatively almost 100% specific for the
disease because we wouldn't expect it in anything else I'm going to talk a
little bit about the difference between the surveillance Center and the Mayo
Clinic because I know some families have had specimens sent to both and the
different results so the tests are done differently from a lab standpoint and
also when you send the specimen to the Mayo Clinic the physician has to
actually order specific tests they have to know to order 14-3-3 and if they want
tau they have to know to order that a lot of clinicians may not know that tau
could be helpful in their diagnosis so it's not ordered RT quic is also only
available at the prion surveillance center so if that went to Mayo you're
not going to get the RT quic. one of the benefits of receiving spinal fluid at
the surveillance center is in essence patients or clinicians don't really need to
know what they're ordering they just need to know that there's a suspicion of
prion disease and then we automatically do all three tests so
that can be helpful for the clinicians the other helpful thing is that if we
have a positive test then we have people that will contact the clinician saying
that we have this autopsy program that I believe you're going to hear about in
detail on Sunday that's funded through the CDC. I just wanted to end a little
bit on sporadic CJD talking about sporadic CJD molecular subtypes because
you'll hear about this and it's very confusing. so sporadic CJD has different
molecular subtypes that behave in different manners and the reason why we
care about that is because it looks differently on the brain tissue
it may look differently clinically and also the diagnostic tests may be
different and generally you'll hear these terms turn around like mm 1 mm 2
VV 2 and what that refers to is those first two letters are what's called a
polymorphism at a specific part of the prion protein gene that's kind of like a
flavor that we all have so we all can have one of three flavors we could have
mm mV or VV and these sometimes dictate the course of the disease
and what they look like clinically and Neuropathologically so we take those two
letters and we also match it with the prion protein type that's usually
detected off a Western blot and that's where you get the number from so for
example a patient who has mm polymorphism at codon 129 and a prion
protein of type 1 would be mm 1 so just so you hear these you know alphabet soup
going around that's what that refers to
so we talked a little bit about genetic prion disease the way genetic prion
disease works is we all have a normal prion protein gene that makes normal
prion proteins but in some individuals there can be a mutation in that gene
that instead of allowing them to make normal prion protein it allows them to
make a mutated form of the prion protein that has a much
higher chance of converting to the abnormal prion protein and then causing genetic
prion disease so when people have genetic prion disease their blood
relatives have a 50/50 chance of also inheriting the mutation but the
likelihood of them developing the illness is something called penetrance
so not everyone who has a mutation will develop the disease there are some
people who will not and penetrance means that it's the likelihood that you'll
become ill with CJD if you have the mutation and this is from a very
important paper that Eric Minkel and a lot of other colleagues put together
looking at different penetrance rates in different mutations and what you can see
is at the top of the table those are mutations that have very low
penetrance so you know they have you know maybe a 1% 5% chance of developing
the disease if they had the mutation that's important to know if you're a
family member who's deciding or whether or not you want to be tested because you
want to know what your risk is as you would expect the right-hand column where
there's red bars are reflects the percentage of people that had a positive
family history and if you have a mutation that has low penetrance
logically you're not going to have a lot of other family members that have become
ill there are many mutations unfortunately in prion disease though
towards the bottom those like E200k or P102L where the penetrance is quite
high and generally is associated with aged nearing a hundred percent but it's
not the same penetrance level for every mutation so if you belong to a genetic
family it's very important to know what the mutation is because that's going to
reflect the likelihood of developing illness if you have it so you want to
make sure that you'll have that you have that information so I talked about three
different genetic prion diseases. genetic CJD is kind of easy because it's called
that because it resembles sporadic CJD the most common mutation of genetic CJD
is the e200K mutation and a lot of the clinical symptoms and
diagnostic tests that we use for sporadic CJD are also positive in
genetic CJD although not always there's another genetic prion disease called
fatal familial insomnia which is due to a very specific mutation as the name
denotes insomnia tends to be a prominent symptom but you can also have very
prominent neuropsychiatric symptoms of anxiety and hallucinations and patients
with FFI you do develop cognitive issues they usually very late in the illness so
this is a genetic prion disease that sometimes doesn't really resemble what
we classically think of as CJD similarly Gerstmann-Straussler- Sheinker
syndrome or GSS is due to several different mutations
it also looks very unlike most cases of sporadic CJD mainly because the illness
duration is much longer so most patients with GSS can live about five years
sometimes up to ten years and they typically either have a very early
cerebellar presentation meaning that they have incoordination or problems
walking or they may have an initial parkinson's-like presentation where they
have a shuffling slow gait and then they typically don't develop other symptoms
characteristic of prion disease until much later in the illness. so this is a
genetic prion disease that if you don't know that you have it in your family
often will go undiagnosed until someone has considered it a possibility in that
family and with FFI and GSS a lot of the diagnostic tests that we use like CSF
tests brain MRI they're often negative in these tests that also leads to
difficulty in diagnosing these conditions. Moving on to acquired prion
disease most people know the story of kuru it's happened in a tribe in Papua
New Guinea where they partook in cannibalism as part of their death
rituals basically probably what happened is one individual developed
CJD. the loved ones consumed brain tissue and then continued to pass from
generation to generation so once cannibalism was ruled out the incidence
of kuru drastically reduced and I don't think they have had any case of kuru in
some time now but kuru is important because it really is what kind of
suggested that prion disease may be transmissible and that led to the
discovery of something called iatrogenic CJD. So Iatrogenic means medically
induced so these are cases of CJD that are transmitted through very specific
medical procedures and these are things like corneal transplants or
neurosurgical instrumentation but most commonly due to material taken from
cadavers. for example human growth hormone used to be taken from cadavers
to treat patients with a short stature we don't do that anymore because we know
the risk of prion disease and fortunately we can also synthesize human
growth hormone in the lab so we don't have to do that anymore also . there's a
covering of the brain called the dura mater that used to be taken from
cadavers to cover holes and neuro surgeries and that also is no longer
done because we can create that synthetically but we learned a lot from
iatrogenic CJD in that sometimes the time from the exposure to the infection
to the time that patients became he'll can be quite extended sometimes up to
decades and just a general rule of thumb for acquired prion disease with one very
specific exception is that in order to transmit prion disease prion material
has to be taken essentially from the central nervous system and then it has
to be introduced in another patient central nervous system injected into the
body or and ingested in large quantities in order to transmit them the disease
the one exception for this is variant CJD because we do know that variant CJD
is a little different and that it resides outside of the CNS specifically
and things like the spleen tonsils and appendix
and can actually be transmitted through blood but at least epidemiologically
there's only evidence that variant CJD can be transmitted in that fashion
so variant CJD is CJD in humans that due to eating meat contaminated with bovine
spongiform encephalopathy or mad cow disease it tends to be a young disease
so onsets typically in 20s or the 30s and it has a little longer duration than
classical sporadic CJD often over a year and their initial clinical presentation
is a little different and that they tend to have more psychiatric symptoms and
sensory symptoms at the beginning of their presentation often times 14-3-3 and
EEGS are negative and they can have different brain MRI findings from what
we typically see in sporadic CJD something called the pulvinar sign or
hockey-stick sign that's somewhat specific for variant as opposed to
sporadic CJD and then at least only in variant CJD you can also do a tonsil
biopsy to detect the abnormal prion protein that causes variant CJD but this
is the only type of prion disease where you could do that because it's unique in
that mechanism that it resides outside the central nervous system so if you
look at the cases of variant CJD worldwide the mad cow epidemic happened
in the UK so the majority of cases a variant CJD have occurred in the UK and
then most others were in European countries we've had four cases in the US
but all of them were thought to be acquired overseas and I think probably
Ryan Maddox from the CDC will talk a little bit more in detail about that on
Sunday today we have a total of about 231 cases which is quite remarkable
because quite a number of people were exposed to meat contaminated with BSE
that have such a low numbers is fairly fortunate if you look at the death rates
of variant CJD in the UK over time the BSE epidemic happened in 1980s
then the peak death rate in the UK occurred about the year 2000 which leads
us to believe that the mean incubation period so time from exposure to BSE to
becoming ill is about 10 years and we've seen a very steady tail of cases after
that but clearly the diseases and the decline and I don't think there's anyone
currently alive with respect to variant CJD --am i correct?-- so that's good and
haven't been for about a year now right. there is another sticky point however
with variant CJD and that is although only 231 cases have occurred to date a
lot of people were exposed to BSE so the concern is are there asymptomatic
carriers of the infection who they themselves may or may not become ill but
also may or may not be transmissible to other people via things like blood
transfusion so the UK did a study that looked at many appendices --over 30,000-- to
look for the presence of the abnormal prion protein and what they found was
about 16 out of 32,000 cases were positive for variant CJD and that there
really wasn't any difference in the number of cases depending on when these
patients were born are you going to talk a little bit about that about what that
means no okay and that all codon 129 polymorphisms
were represented so this led to the estimation that about one in 2,000
citizens of UK are harboring asymptomatic infection of prion disease
so this is why when you get a give blood in this country they give you a laundry
list of questions some of which is have you resided in the UK from this time to
this time and that's because they're trying to assess the risk of your
exposure to BSE. so changing gears a little bit to chronic wasting disease
fortunately we really don't have BSE in this country but unfortunately we have a
lot of what's called wasting disease. this is the prion
disease and deer elk moose and caribou that is a little unusual and that it
tends to be fairly infectious amongst its own species so essentially the BSE
epidemic was man-made we were kind of perpetuating the infection because we
were refeeding neural tissue back to cow's and perpetuating the infection
kind of like kuru chronic wasting disease can be transmitted between its
own species through things like saliva urine and feces so much more easily
transmitted in the natural environment which means that it spreads a lot more
rapidly and this map is just the depiction of cases of chronic wasting
disease you can see it's usually out in the Colorado Wyoming area but it has
been spreading the Canada and also to some states in the East Coast and then
in the last year so we've also heard about different cases in Norway we don't
know exactly how it got to Norway although there are some theories one is
that it's a sporadic occurrence and that just other countries haven't caught it
yet the other possibility is that hunters use urine to attract other deer
and sometimes that urine can be imported into different countries so it's
possible that there's year and contaminated with CWD that was imported
into Norway and that these deer became ill that was chronic wasting disease is
obviously very important to our country because we want to avoid what happened
in the UK with BSE luckily there's been no evidence of transmission of CWD to
humans but as with the case with the iatrogenic CJD and with BSE we only
really know that if we continue surveillance so sometimes these
incubation periods can be a decade or more
so we may not know right away whether or not it's being transmitted to humans so
this is why the CDC work and the National prion disease pathology
surveillance centers work is so important because sometimes the only way
to detect abnormal or new prion diseases is by
looking at brain tissue so part of what the surveillance centre does is it looks
for atypical prion disease that may be suggested that chronic wasting diseases
being transmitted to humans but again today we don't have any evidence that
that could happen.
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