- Look, do you see the camera? - Camera. - Camera. Camera. - [Chris] How would you
describe your daughter? - I would describe her
as loving and sweet, and she loves people,
and joyful, and funny. - [Chris] How would you
describe what Batten disease is doing to your daughter? - I would say that it's slowly taking her away. She can't walk anymore, and
she can't talk as much anymore. And she used to have this energy about her that she would just dance and sing around the house all the time. She would do a beautiful rendition of "Let It Go" (chuckles) from "Frozen", and she loved dancing to
Taylor Swift "Shake it Off", and "Uptown Funk" by Bruno Mars. She used to know some of those words, and would dance all
around the house to them. And so there are parts of
her that are missing now, and but we just look
for the parts of Ellery that are still there. - Strawberry Shortcake. Say cake. - Cake. - Yeah. (Ellery vocalizes) Her hair? What color is her hair? (Ellery vocalizes) What color is her hair? Is it pink? Pink? (Ellery vocalizes) Strawberry. (Ellery vocalizes) Yeah, on her shirt. Yeah. (Ellery vocalizes) Yeah, there's a strawberry up there, too. - She has so much in her mind that she can't get out with words. And before she, well, before
the onset of her disease, she knew so much that I would've said that she
was almost kindergarten ready. You know, she knew her letters, she knew number recognition. She would count to 20
with varying accuracy. She would say, "One,
two, three, four, five, six, seven, eight, nine, 10, 11, slelve." And then she would say,
"Aughteen, aughteen, aughteen, aughteen, 20!" (chuckles) There are times when she tries
to communicate something, and can't get her point across, and will find a different way to tell me. And whether it's by pointing
or showing me something, she'll get her point across
even if she can't say it. - [Chris] Ellery, it's really nice to be
here with you today. - Yeah. Can you say, "Hi?" (Ellery vocalizes) What would you like? - Milk. - Milk. Milk. (chuckles) When she was first diagnosed, I thought things like, "I identify so much
with being Ellery's mom. Who am I if I'm not Ellery's mom anymore?" And then it occurred to me that I will always be Ellery's mom with her here or without her here, I am always Ellery's mom, and that will always be
a part of my identity that I am most proud of. - [Chris] This is a
difficult question to ask, but I want people to know the
reality of Batten disease. Is it okay if I ask question
about life expectancy? - Yes, so typically life expectancy is about five to 10 years from diagnosis. So we are now about two
years from diagnosis, almost. She used to be able to say, "I love you", and things like that, but now we say it to her and we know that she knows what it means. - [Chris] How old was she
when the symptoms onset? - She was four years old.
It was March of 2020. - [Chris] Has Ellery
expressed frustration to you over this regression? - Oh, yeah. There were times when
she would try to walk and she would get frustrated because she couldn't walk right anymore, and she would get frustrated
that she kept falling. And there are many times when
she tries to say something, and she can't and she gets frustrated, or she tries to say something and we can't understand her,
and she gets frustrated. - [Chris] What was it like
when Ellery started to regress? (Ellery vocalizes) - So when Ellery started to regress, she would be walking, and then she would fall
and her legs would shake. And so I took her to the doctor, and they said that she was
doing it for attention, and that they would give us
a script for physical therapy if we wanted that for clumsiness. And I said, "Yes" because I
will always take more services than less services for my child. And so we went to the physical therapist who was able to elicit the shaking just by playing with Ellery's limbs who said she needed to see
a neurologist right away. And so I asked the pediatrician for a physical therapy script, or for a neurology script, excuse me. (Ellery whimpers)
It's okay. And they gave us the neurology consult, or the neurology prescription, and we went to the neurologist. (Ellery whimpering) And... It's okay. - [Chris] Do you think she would like if we put the TV show on and muted it? - Yes. (laughs) - [Chris] Yeah, let's do that. - Okay.
- [Chris] Yeah. - All right. Zach, where's the remote? It's okay. (Ellery whimpers) - [Zach] What do you want
me to put on? Mickey? - Yeah. Do you want Mickey? (Ellery vocalizes) - House. - House. Mickey house. Okay. - [Zach] Yes. "Mickey Mouse Clubhouse". - House. - House. Okay.
- House. - [Chris] Has she always loved Mickey? - Yes. She goes through phases, but Mickey's been for a while. - Mickey. - Mickey. - House. - House. - Mickey Clubhouse. - House. - House. - [Chris] Do you love Mickey Clubhouse? - Mickey. - Mickey - House. - House. Do you like Mickey? Yes or no? Hey, do you like Mickey? Yes or no? - Yes. - Yes. - Donald. - [Mom] Donald. - Donald. - [Chris] Quack quack. - Quack. - Quack. Donald Duck. (Ellery vocalizes) Yes. - What are you smiling at? What are you smiling at? - [Chris] Something funny
happening on the show? - Mickey. - Mickey. - House. - House, yeah. Mickey house. - A house? - Yeah. - House.
- Mm-hmm. - Mickey. - Mickey. - House. - House. - [Chris] Do you two
watch it together a lot? - All the time. You know, Ellery has always been a child who likes to be in my lap all the time. From the time that she was born until now, she's always wanted to be, you know, if there's times during the day when she will get down and
go play and things like that. But really most of the time
she wants to be in my lap. And you know, I've always loved having her in my lap, but especially now given
what we've been told, I'll hold her all she wants. In the beginning of her diagnosis, I pre-grieved all the time and just looked at what
the future was gonna hold, and what my life was going
to look without her one day. - [Chris] How did you learn
to live in the moment again? - Therapy. (laughs) Medication. She does love her Mickey. Donald Duck is her favorite. Right? Donald Duck. Duck. - Yeah. Donald Duck is her favorite, and she loves the way he talks. And my dad will can talk like Donald Duck, so we'll call him and he'll
talk like Donald Duck to her. And she always smiles and laughs. - [Chris] Do you love
Donald Duck? Yes or no? - Do you like Donald Duck? Yes or no? (Ellery quietly vocalizing) Now she's making quack, quack noises. (Ellery vocalizes) Is that how you make a duck noise? - [Chris] Quack, quack. (Mother gasps) Quack, quack. Quack, quack, quack, quack, quack. (Mother chuckles) - Quack, quack, quack. - [Chris] Hey. - Look at our friend, Chris. - [Chris] Can I sit with you? - Yes - Yes. - I know you love Donald. (Ellery quacks) So I wanted to do... Nice Donald impression. Quack, quack, quack. - Look. Look at Chris. - Quack, quack, quack,
quack, quack, quack, quack. - Quack, quack, quack. - That's a great Donald impression. - Quack, quack, quack. - [Chris] Do you like seeing
her do her impressions? - Oh, yeah. I think it's so cute. I love it when she tries
to oink like Peppa, she goes, "Ka, ka, ka."
- George. - And George, I'm so sorry. I always forget it to say George. - [Chris] What sound do
Peppa and George make? - Ka, ka, ka. - Yep, that's it. - Peppa and George. - And George, Peppa and George. - [Chris] Oink, oink. (Zach snorts) If you want to make Ellery
smile, what's your go-to thing? - Hey, Ellery? Ellery? She really likes, it's not appropriate, but she likes toots. (mother blows raspberries) (Chris blows raspberries) (all laughing) (Ellery vocalizing) - Did he toot? (blows raspberry) Oh, are you giving kisses? Are you giving strawberry kisses? Ellery, that's so nice. (Ellery vocalizes) Yeah. Kiss. Oh, that's so nice. - Do you think she
really loves these shows and different toys that
she's talking about because it's something easy for her to bond with with people. She knows the words? - Yeah, definitely. It's something that she can
interact with, you know? Oh, and what the times
that she has the TV on, a hundred percent of that
time she's talking back at it, and singing to it and- - Anna. - Anna, I know. And so it's really something
that she can interact with. Whereas like other people, it's a little tough to
interact with other people because sometimes you get forgotten about if you're somebody who
doesn't talk very much. - Quack, quack, quack. - Good job. - [Chris] What is it like for you to see her play and make jokes like that? - It's really, you know, it's wonderful anytime I see Ellery do anything that seemed like Ellery before
the onset of her disease. So when she smiles, it makes me happy. When she laughs, it makes me happy. When she says a word that she
knew before the disease onset it makes me happy. Even when she crawls now, you know, she doesn't walk anymore, but when she crawls now it makes me happy because I know there where
this disease is headed, and you know it's wonderful
to see her doing things that are functional. (Ellery whimpers) Even when she eats my
mouth, I'm happy, you know? Milk, it's okay. So yeah, I love seeing her
do just about anything. - The nose. - The nose. That is a nose. (Ellery vocalizes) - That's a cool nose.
Strawberry Shortcake. I like that you're
showing me all this stuff. Does she like to show
people her interests? - Oh, yeah. Yeah. In fact, ever since she
was a little, little baby, if she liked you, she would hand you
something that was her toys. - Share. Share. - Oh, is that for Chris? - Oh, did you say share? - Share. - Share. - Does she say share? - Share. Yeah.
- Thank you for sharing. - Share. - Share, yeah. - I found Strawberry Shortcake's hair. - Yeah, hair. - I found her nose.
- Nose. - I can't find her eyes though. Where are her eyes? - Eyes. - Are her eyes down here?
- Eyes. - Where's her eyes?
- Wait, that's her shoe. That's not... That is her eye. - Good job. That's her. - That's her hair. Thank you for showing me her hair. - Hair. - Yeah, that's hair too. You said you went to therapy
after Ellery's diagnosis. Can I ask you a question about that? - Yeah. What are some coping
mechanisms you learned to deal with your family situation? - So, grounding techniques, right? Like, if my mind run runs away with a thought of like,
sometimes I would daydream, and be caught on the
idea of like her funeral or, you know things like
that, that were just, it would paralyze me. I am paralyzed, but I
meant like emotionally, and you know to get out
of that, I would say, "Oh, look Ellery is watching
Mickey Mouse Clubhouse, and she's happy, and she's giggling, and she's sitting on my lap, and I can feel the
weight of her on my lap. And I'm hugging her and she's hugging me. And you know, just sort
of like talk to myself about the things that
are actually happening, and not what's going to happen. - [Chris] Do you have
a diagnosis yourself? - I do. I have spina
bifida myelomengocele L4/5. And when I was born almost 41 years ago, my parents were told
that I would never walk, that I would never talk, that I would have
intellectual disabilities, that I would have trouble
separating from my parents, that I would have trouble making friends, and so forth and so on. Pretty much all of that was false. - [Chris] How would you
describe the physical impact spina bifida has on you? - So I like to say that
the way that I think of spina bifida is like it's
an inconvenience, right? So like, I can't carry a laundry basket. I have to use two crutches. So I, you know, the one thing
that I would've liked to do that I really couldn't do, was carry Ellery, my daughter. So I carry her in my arms,
I did carry her in my body. - Zach, how would you
describe your family? - I would describe us as fun, and loving, and a lot of work. (Zach and mom laugh) - [Chris] What is it like to be a mom with a physical disability and have a daughter with a
regressive terminal disability? - That part is the hardest part, right? So it means that a lot falls
on my husband, you know? He has to carry her, he has to push her. It means a lot of physical burden on him. And if I could take on anything for him, I absolutely would in a heartbeat. He's a wonderful man, and
he does so much around here and I try to pick up where,
you know, where I can, but it's just a lot of
physical strain on him, and that's what that means for him. So, yeah, that part is very hard because I wish that I
could help out and I can't. - Jen has shared what it was
like from her perspective with Ellery being diagnosed
with Batten disease. Would you mind sharing
it from your perspective? - Sure. I, so Jen and I are very different in how we dealt with the diagnosis. I mean, you know, obviously
it's like life shattering, but I am a more, when it comes to this, I'm a more hopeful person, and I live more in the now so I don't really think about the future. So, you know, I'm not like
depressed all the time about the disease because I'm thinking about, "Okay, what does Ellery need right now?" - Mickey.
- Mickey. (Ellery vocalizes) They should do a crossover.
Mickey and Strawberry. - [Chris] Those are two favorite things. - Yeah. At the moment. She also likes Peppa, right? - And George.
- And George. Yes. We can't forget about George. I always have hope that
there will be a cure, but assuming that Ellery's
trajectory looks like how the trajectory for
the disease is right now, then I always tell her we're gonna have the rest
of our lives to grieve. So let's not grieve now 'cause
it's not doing us any service and it's not doing Ellery
any service, you know? We need to be here for Ellery. So yeah, I mean, I'm sure, I don't know if it's totally healthy that I don't think about
everything, but I don't. - If somebody does wanna bond with her, make her feel included, what
do you recommend they do? - Like, sometimes the kids at school will go up to her and give
her a toy or give her a book, or the girls will come up to her and sing Twinkle Twinkle Little Star. And she loves that. - Do you like Twinkle Twinkle? - Oh, Twinkle Twinkle Little star. - Star.
- Star. ♪ Twinkle, twinkle, little star ♪ ♪ How I wonder where you,
I'm just making it up, are ♪ ♪ Up above the sky so high ♪
(Ellery vocalizes) ♪ Like a diamond in the sky ♪ ♪ Twinkle, twinkle little star ♪ ♪ How I wonder what you are ♪
(Ellery vocalizes) - Yay!
- Yay! (Ellery shrieks) - Oh, you like that one. - Thank you for singing with me. (Ellery vocalizes) - She really liked that one. - You're a wonderful singer. - Good job.
- Twinkle. - What?
- Again? - Twinkle. Yeah. - Twinkle. ♪ Twinkle twinkle little star ♪ ♪ How I wonder what you are ♪ ♪ Up above the sky so high ♪ ♪ Like a diamond in the sky ♪ ♪ Twinkle Twinkle little star. ♪ ♪ How I wonder what you are ♪ I liked that you copied my pointing. - Yeah. Good job. - [Chris] How would you
describe Batten disease, and what it does to somebody
who's never heard of it? - Batten disease is a disease that slowly takes away your function. So for Ellery, first it
took away her walking, her potty training, then
most of her talking, her ability to eat solid food, so now she eats is only a soft food diet. It will eventually, it
eventually causes blindness, and she will eventually be G-tube reliant, and then the child dies. - I have something really
important to tell you. (Ellery vocalizes) - Look at Chris. - Are you ready? I'm gonna tell you
something really important. Quack, quack, quack, quack,
quack, quack, quack. (chuckles) - (chuckles) Oh, now you're doing it. - Quack, quack. - (singing) Do, do, do. Do, do, do. - (singing) Do, do, do. Do, do, do. - Is that a song from it? - (singing) Do, do, do, do, do. - That's actually a common
noise that she makes. - (singing) Do, do,
do, do, do, do, do, do. - Very nice singing. (Ellery vocalizing) - Thank you for singing me a song. (Ellery vocalizing) - Oh yeah? - [Chris] How is Batten disease causing that result in the body? - So Batten disease causes
the cells in your body to collect trash. And instead of like, our bodies naturally wash
the trash off of our cells, and we're fine, but
hers collects the trash, and doesn't get rid of it. So it kills the cells, and
then kills the function. - Ponies. Ponies. - Yeah, ponies.
- Ponies. - Oh, you like My Little Ponies? - Ponies. - Ponies - Ponies. Yeah, ponies. - Ponies.
- Yeah. (Ellery vocalizing) - [Mom] Storm King. - Is Storm King over there? - [Chris] What is Storm King? - Storm King is the villain
in the "My Little Pony" movie. She loves villains. She also loves- - Storm King. - Yeah. You also love Hans in "Frozen". - Hans. - Hans, he's yucky. - Hans. - Hans falls in the... - Hans. - Hans falls in the... - Hans. - Hans. He falls in the water. Yeah, water. - Water. - Yeah, water. - And you said she likes villains. - Oh, yeah. - Why do you think she likes villains? - For some reason she
thinks they're really funny. - Hans. I've never seen
Frozen. Hans is the villain? - Oh, yeah. But she likes the part
where Anna punches him, and falls in the water. - Hey look, I'm Hans. - Look. - I'm Hans. Ow! (all laugh) I'm Hans. - And Anna. - And Anna, yeah. - Wait, who punched Hans? - Anna. - Hey look, I'm Hans. I'm Hans. - Look, look, look. - And here's Anna. Ow. Anna got Hans. (chuckles) - Anna. - Anna. - Anna and Hans. - Hans. - Anna got Hans? (Ellery vocalizes) - In the water. - She still has all the interests. - Yes. - Of any kid. - Oh, yeah. - Yeah. (Ellery vocalizes) - Yeah, do you want me
to get the Storm King? - Storm King.
- Look. - [Chris] Oh, he looks
scary. Ah! He's scary. - Storm King. - Yes. And you'll notice
that Storm King and Mickey sound the same the way
she says she says, "Ocky". Also Forky from "Toy
Story 4" is also "Ocky". Yeah. So sometimes we often
have to try to figure out contextual clues as to what
she's trying to ask for. - Ocky. - Yes. So if she says Ocky, we'll say, "Do you want Mickey Mouse Clubhouse, or do you want Storm King Ponies?" And then she'll either
say house or ponies. And that's how we know
what she actually wants. - Yes. - What do you want others to
know about Batten disease? - I want everyone to know
that Batten disease is so, Batten disease is not as
well known as it should be. And it is not as
researched as it should be. And we need to work to
find treatments and cures because no child should suffer the way that my child has suffered, and the way that my family has suffered. Because although we look for the joy, and we always look for the happiness. and we always look for the giggles, there will always be a part of our family that is destroyed because
of Batten disease. - And I was told that
they were researching it, but there wasn't enough money 'cause not enough people
are diagnosed with it. So they just stopped the research. - Right. Yeah. They
stopped the clinical trial that did the genetic testing 'cause there weren't enough,
there wasn't enough profit. And that makes me sick. (chuckles) And they said, you know, while it only gave the kids
who had it a couple of months, well, I'll take a couple of
months, I'll take a month. - What's it like to know they look at your daughter's
life in terms of profit? - Yeah, that makes me sick. It's really sickening to know that they can know that
children are dying, children are losing their function slowly. And it's not that they're not aware of it. Ellery knows that she
used to be able to walk, Ellery knows that she used
to be able to talk easier. You know, it's not like they're carefree, and losing these functions,
you know? It's hard. And they just give up the research. I think that when you become a parent, you have these expectations of what parenthood is going to be like. And so you think, you assume, you know, they're gonna grow up and go to college, and get married and have children, and you're gonna experience
all these things together. And so when something like
this happens in your family, you have to readjust what
parenthood looks like, and what to expect from parenthood. And so I've readjusted what
a good day looks like, right? You now, before a good day looked like, "Oh Ellery learned her letters today, and we got to sing our ABCs
together, and she knew them." And now a good day is she crawled, and she was happy, and she
smiled, and she laughed. And you know, that's what
makes a good day now. (Ellery whimpers) It's okay. It's okay.
