Talk from Quinn's service in Wenatchee

Quinn Sunsanee Hardy OCT 2013

Lexie We had the honor and privilege to be Quinn’s parents. We’ve decided to speak together. We worked together as Quinns parents so it makes sense to speak together.

Barlow Quinn Sunsanee Hardy was born November 28 2012.  She weighed 6 lbs. 2 ozs.  We struggled to find the perfect name for her – it wasn’t until she was almost here that we settled on Quinn.  We wanted to honor her Thai heritage, and chose Sunsanee for her middle name – which translates to “One who is praiseworthy”.   As she grew we developed many pet names for her – Quinny, Squish, Tuk-Tuk, and our favorite, Quinsanee.  Her defiant spirit shone through early in life when Barlow tried to dress her in the hospital, and she grabbed hold of her clothes and refused to let go.  She was small but full of vim and vigor.  As a newborn she was alert and sweet , but also sometimes demanding.

Lexie At around 2 months, Quinn began to cry continuously and inconsolably, sometimes lasting 18 hours a day.  No one could find anything physically wrong with her, and it was believed she had colic and would improve over time.  Just before she turned 4 months old, she lost the ability to breastfeed or suck from a bottle.  It got to the point that she was being fed by dripping milk into her mouth using a syringe.  She had stopped growing at an expected rate, and her head circumference was abnormally small.  She was admitted to Seattle Children’s Hospital, where she received a feeding tube that went in her nose down to her stomach.  An MRI showed that the white-matter in her brain was abnormal, and the Drs. Began running tests to try and determine the cause of her leukodystrophy. 

Barlow At around 6 months of age, Quinn began having seizures.  They escalated to the point where she was having a seizure every 30 minutes. Another admission to SCH, and a prescription later, the seizures abated.  After months of tests coming back negative (some of them for truly horrible diseases), we finally received a diagnosis of Aicardi-Goutierres Syndrome.  Not much is known about AGS because it is an extremely rare genetic disease – there are fewer than 200 known cases worldwide.  No one could tell us what to expect, and there is no cure.

Lexie What is known about AGS is that it is a genetic defect that affects many parts of the body. The brain is the most severely affected. Most Children stop developing new skills and also have some developmental regression. It affects the ability to move, to see and to eat.

Barlow Because of Quinn’s condition, we met several great people we would not have had the opportunity to meet otherwise.  Our eyes were opened to the many families around us who have children with all sorts of debilitating illnesses.  We’ve learned not to compare our children or each other, but to recognize the love between parents and children.  We’ve gained strength from each other, cried together, and supported each other.  We also were able to meet some wonderful Drs.  We were also able to meet some wonderful Drs, nurses, and therapists. 

Lexie Along with the ability to eat, Quinn also lost most of her vision as well as the skills she had learned and developed since birth.  We worked with the Drs. Here in Wenatchee and in Seattle to make Quinn comfortable and to help her develop the best she could. We had weekly physical therapy with her occupational therapist Heidi and we worked with her daily at home. We would do stretching, work on rolling from side to side, We worked on holding her head up and developing trunk strength, and of course we had the dreaded tummy time. She learned to shake a rattle, grasp a ball, and roll to her side.  She learned to eat baby food (though she didn’t always enjoy the flavor, as far as we could tell).  And while these are normal skills for most babies, they were huge accomplishments for Quinn. 

Barlow We have been greatly blessed by the people at church and friends in the community as we worked to raise Quinn.  From meals to watching McKay (our poor neglected dog) when we had to make emergency runs to Seattle, Mayra and Mac became very good friends.  We are grateful for Marin and Sarah as they came over hold and snuggle Quinn so we could get a wink of sleep or do a much needed load of laundry or dishes.

Lexie Shortly before her birth, Barlow’s parents moved to the Wenatchee Valley to be near their soon-to-arrive granddaughter, away from the waters of the Pacific which they love.  They watched Quinn when Lexie returned to work.  Grandma Toland also came to watch Quinn for the summer.  Her grandparents doted on her, giving her lots of snuggles and kisses. They also helped us with the through love of making her do her physical therapy and helped with feeding her.

Barlow We had visits from family from Utah and Wyoming and everyone who met her thought she was sweet and lovely. It has always been so puzzling as to how a baby so cute could have so many challenges.

Lexie In the last month Quinn had been doing very well. She’d been making progress in her therapy. She’d begun to transfer a ball from hand to hand. Her neck strength was improving. She was doing better with her tummy time. She had not had a seizure for over 4 weeks.

Barlow A few days ago Quinn had begun to have a hard time keeping her food down.  We all thought she had a stomach flu. None of us, not her doctors, or her family suspected hear heart was failing. She passed away on OCT 25^th at 12:37

Lexie We’ve told you a little about Quinn’s life, but now we’d like to tell you about Quinn herself. Quinn loved to be touched and held. She was instantly comforted by being snuggled by her loved ones. She loved to be sung to and to hear stories. When we were riding some place in the car she would cry in her car seat not understanding why she wasn’t been held, we could sing to her and she would calm down. She had eclectic taste in music she enjoyed everything from Metallica to the Beatles.

Barlow :During her therapy she would cry and protest when it got difficult or she had to work hard. Much like we all do when we have to exercise. She would often close her eyes as though she were asleep to avoid continuing. It’s almost like she was pretending to be asleep so we would stop therapy.  She had difficulty with hand control, but she would somehow  manage to reach up and grab my beard.

Lexie: Barlow likes to tease. I was grateful when we got Mckay and he had someone else to tickle and tease. And Both Mckay and I were grateful when Quinn came along and Barlow’s attention went to tickling and playing with Quinn.

Barlow: I would rub her face and her head and she would make an annoyed expression. It was like she was saying “ Ahw… dad!”  At first she didn’t really appreciate it, but she seemed to grow to like it. It was a special bond we developed. If anyone else were to try such a thing she’d cry and protest, but she tolerated as she seemed to know it was my way of saying I love you.

Testimonies

Barlow

Lexie

thoughts...

I don't really want to forget... just hurt less

I'm not sure if this true but I like to believe it is.

I know this for sure.

We will see Quinn again.

Occupational Therapy

I started this post before the recent tragic events... but I thought I'd post it anyway. It's not all the way complete, but I just don't have the heart to work  on it more right now. 


We've been really slacking in updated the blog. Once you get behind it's hard to get motivated to start again.

A big part of managing Quinn's condition is helping her develop her motor and oral skills. She lost a lot of skills she had early on due to the damage to her brain by the disease.

We have occupational therapy each week with a fabulous therapist named Heidi. She comes in to our home through the "Birth to Three program" or early intervention program (ESIT).


We do daily stretching with Quinn. With every diaper change we try to do hip stretches. For a long time her hips were very tight. At first she couldn't extend her hips enough to bring the back of her legs down to the ground.  It's been improving little by little... and now she can lie almost flat.


Quinn has a strong grip, but struggles to get objects into her hands. We work on passing objects back and forth, reaching and picking up items.

This is Quinn's favorite toy. The ball has a few little beads in compartments that rattle. She will actually track the ball with her eyes. This is is something new she's not been able to do in the past.

This is a sensory drum. Quinn can tap on the drum and feel the beads roll around inside. It helps with visual, audio and tactile development.

We work with Quinn to interact with things in her environment.

" I need a break!"

working in developing head strength

Interview with an AGS patient

AGS Conference Washington DC - Jonah Interviewed by Adeline Vanderver, MD

https://stream.manchester.ac.uk/Play.aspx?VideoId=12566

little by little

Quinn has been making very small improvements. She is just starting to be able to shake a rattle. For a normal baby this is normal but for Quinn this has been a huge step.

Our Little Zebra

 

OUR LITTLE ZEBRA

In medicine there is a saying " When you hear hoof beats think horses not zebras." This is to explain that common things happen often and rare things are not as likely - it's more likely you have a cold than cancer. We see a lot more horses than zebras here in the states.

We have been going to several different specialists. Quinn has been poked, x-rayed and studied.

Our little Quinn is a zebra. She is rare and special.

 

It has taken a long time to find the cause of her symptoms, but wee recently got a diagnosis a few weeks ago. She has a condition called Aicardi-Goutieres Syndrome.  The prevolence is reported to be <1/million. This genetic disease is caused by mutations. Quinn has 2 different mutations in the TREX1 gene. She got one mutation from each of us.

This condition is characterized by microcephaly (slow growing head), feeding difficulties, seizures, and jitteriness. Quinn's has all of these symptoms. Her prognosis is unknown - 25% don't make it to one year of age, but we are pretty sure she'll be here long after she's a year old. There are a few children who grow up to be 30 years old. There are many possibilities. We need to work really hard to help Quinn to develop and grow the best we can. She has a lot of hard work ahead of her, but she's a tough kid and has already done a lot better than many children with this condition.

We think she'll be writing her own story.

More info:

Aicardi-Goutieres.com

The International Aicardi-Goutières Syndrome Association (IAGSA)

Nuclease Immune Mediated Brain & Lupus-like conditions

Diner at the "Crabpot" after our last appointment at Seattle Children's Hospital.

Welcome to Holland

When early intervention first came to see Quinn they left this great essay about having a child with a disability. It has really given us strength and helped us to keep things in perspective.

 

WELCOME TO HOLLAND

by

Emily Perl Kingsley.

c1987 by Emily Perl Kingsley. All rights reserved

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this......

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.
After months of eager anticipation, the day finally arrives. You pack your bags and off you go. Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland."

source

"Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must stay.
The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.

So you must go out and buy new guide books. And you must learn a whole new language. And you will meet a whole new group of people you would never have met.

It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.

The night watch by Rembrant

But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."


And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.

But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

Shaking things up

Our sleeping beauty shortly after discharge from the hospital.

It's been a while since we updated... sorry about that. It's easy to get in the day-to-day grind and let things slip.

We are still doing tube feedings ever 3 hours and are getting used to taking turns being up at night. It's almost like she's a newborn all over again.
Quinn is gaining weight like crazy! She's up to 13 lbs 4 oz. - that's about a 2 lb gain over 2 months. She's got little fat rolls on her arms and legs and her chubby cheeks are precious.

We're sure every parent thinks their baby is the cutest... but Quinn really is the cutest.

Our chubby cheeked chipmunk in her car seat, last week. 

Grandma T was here for a month and watched Quinn while we were at work and now Grandma H is watching her during the day. We are so grateful for these brave ladies who have really stepped up and helped us out. Heavenly Father sent us to such wonderful families and we are so blessed.

Quinn has always had a little tremor since birth, but it's been chalked up to a startle reflex. In the last week that's changed. She has had events where her arms extend and sometimes shake a little. This has progressed to arms and legs being extended and her head being tossed back. It's a very strange sight and has gotten a worse. It's been happening when she is in the swing, car seat or the bath, but is now at rest as well. We sent a video recording of it to our Dr. who sent it to her neurologist in Seattle.
These events are suspicious for what they call infantile spasms or west syndrome. We are going out to Seattle this week for an EEG and a consult to determine if these are infantile spasms. Hopefully we can determine if this is a serious problem. We are really looking to make her more comfortable, and these spasms are stressing both Quinn and us out. Lets all hope we can find a way to lessen their effect on her.

We have the best friends, family, ward and co-workers. Thank you all. We've had great meals, lots of kind emails and phone calls, and so much support.

 Thank you to both our work places who have been so understanding of us having to rearrange our work schedules and sometimes take time off at a moments notice.

What we know so far…

Everyone wants to know what exactly going on with Quinn. We haven’t meant to keep anyone in the dark.  The truth is we don’t know, her doctors don’t know.

Quinn has not been meeting her milestones. She was not getting the nutrition she needed despite trying to feed her around the clock.

 She was admitted so she could be fed and assessed. The NG tube is helping her get the nutrition she needs. She is already up to 12 lbs and was at 10 lbs when she was admitted over 2 weeks ago. The tube looks scary but has been a blessing. She still tries to nurse and she takes milk through the binkie-trainer, but this is more for recreation rather than nutrition. We need to keep doing these things so she does not develop an oral aversion. 

In the hospital an MRI was done and it showed that Quinn’s brain does not appear like a normal 4-month-olds brain. There was a lack of development in some areas and calcium deposits noted as well. It is not clear if these findings show that Quinn’s brain has not developed right or if it is degenerating or both.  In either case her development is somewhere around 2-3 months and seems to have slowed.

Quinn waking up after being sedated for the MRI

 Her gastrointestinal tract has also been affected. She has not been able to digest food normally and shows signs of malabsorption.  She has had continued diarrhea off and on and we have been struggling in vain to find a magic cream to cure her diaper rash. We had no idea we were fighting a losing battle because her stool is so acidic. There was a time when it looked like her bottom was burned. We have since put her on a mixture of a formula called Alimentum (It’s spendy) and breast milk. Her rash and stool is improving a lot and as a result Quinn seems a lot happier.

We are not sure if Quinn’s agitation and crying is caused by her discomfort due to her abdominal pain from the gas and stool from the malabsorption or from her neurological condition. In either case we have been working on both problems by the formula change and a medication to help with agitation.  We still have several hours of crying a day but it is dramatically decreased. She seems much more comfortable now. 

There have been several theories examined. The calcium deposits were concerning for a disease that Lexie may have picked up while she was expecting. These diseases include things like Chicken pox and CMV. She was also worked up for Toxoplasmosis which is why pregnant women shouldn’t be around litter boxes.  To our relief these tests have been negative.

Other tests were done to examine other diseases that may have caused her neurological symptoms and some truly horrific diseases have been excluded. 

We have now moved on to genetic testing, and there will likely be tests done after that.

We may not know what is wrong with Quinn for a long time. No one can tell us how functional Quinn will be in the future. We don’t know if she will be a normal child, if she will be able to walk and talk and care for herself. We don’t know what her life expectancy is...

What we do know is... We LOVE Quinn, and we will love her no matter the outcome. We are going to do our best to make her comfortable and to help her thrive. Heavenly father sent her to use as a great blessing and he knew we would love her. We won’t disappoint him and with is help we can get through this. 

Thank you to everyone who has been reaching out to use and shown so much concern for our family. You have no idea how much it means to have support from so many wonderful people.

Coming Home

We have finally be discharged from the hospital. We had to do a lot training with Quinn at the hospital to be able to manage her feeding tube. We can now replace the tube if it comes out, hook up feeding, give medication and stop the feedings
We started Quinn on some medication to help with her agitation. she used to cry up to 18 hours a day. it is so much better now.
She is really starting to plump up and gain some weight.

We have been going to appointments getting Quinn settled and trying to make things work for her and us at home.

After Quinn's first walk in her new stroller before admission.

Today she went on a walk with her grandma Toland and our puppy McKay.

Quinn's walk after discharge with Grandma T. and Mckay,
 in her super fancy stroller bought by Grandma H.

Quinn's status

Yesterday marked being at the hospital for one week. The good news is that we are hoping to go home on Monday or maybe Tuesday. Quinn has been on tube feeds for nearly one week now. She has gained weight and she looks so much healthier. Her cheeks are filled out and her skin is pink.

She will be coming home with the NG tube. The tube goes from her nostril down her esophagus to her stomach so that milk can be pumped directly into her stomach. We are still feeding her orally as well. She takes a little less than 1/2 of her milk through her mouth either through breastfeeding or what is called a binki-trainer. A binki-trainer is a pacifier with a small tube hooked to a syringe that we can slowly push the milk through so it's like she's sucking it from a nipple. She has to work harder at the binki-trainer than she does a bottle. We don't want her to forget how to suck and use her mouth muscles.

We have been doing a lot of tests to figure out what is causing Quinn's symptoms. So far we have ruled out some truly difficult conditions, but we have a long road ahead of us. We will have to come out to Seattle from time to  time for follow up.

Quinn's Biography

Hi my name is Quinn,
When I was born, I was on the small side (6 lbs 2 oz).  I started having problems while my mom was in labor - I was feeling stressed out and my heart rate dropped several times so I was delivered via c-section. I had a bowel movement while I was in mommy so I came out green. I was also a little shaky but my blood tests were normal, so they just thought I was moving like that because I was startled.
I was so cute and everyone was crazy about me. I had my family wrapped around my finger. My mom and dad had so much fun getting to know me.

Shortly after birth with green hair.

My Aunt Haylee took lots of cute newborn pictures of me.

My parents love me so much.

 I did super well in the hospital. I had a hard time figuring out how to eat and I didn't gain as much weight in the 1st two weeks as I should have, but after a few weeks I figured it out and even though my parents were very tired I did fine until I was 6 weeks old

.

I started having a lot of gas and it hurt my belly after I ate. I had to poop all the time and I cried and cried; everyone thought I was colicky.We tried some medications (Ranitidine, Zantac) for reflux and it seemed to help a little bit. We tried lots of things - swings, gas medicine, lactase enzymes.  My mom tried eating different things and cutting things out of her diet to make me feel better.

 At my 2 month check I was still really small, but we thought I'd catch up. We thought I'd get better when I was 3 months old. 3 months came and went and I still cried a lot. I was only getting about 4-6 hours of sleep a day. Everyone thought I'd grow out of it eventually. I didn't feel like smiling much, and my parents worried about me a lot.

Coming home with Daddy

Princess Quinn

After I turned 3 months it started to get really hard for me to eat. It was hard for me to nurse or to drink a bottle. My mom had to drip breast milk into my mouth with a syringe. I got a new medicine for my tummy, and I was able to eat a little bit better for a week.

When I went into my well child check I was not growing like I was supposed to. My parents and doctor were concerned because I wasn't acting like a 4 month old. I have been getting behind and so I was sent to a hospital in Seattle so they could figure out how to help me.

Opening letter

Friends and Family,

Thank you for your prayers and concern - they mean a lot to us.  As there are so many of you that are concerned about our welfare, we are creating this blog in an effort to keep everyone equally informed and prevent people from 'slipping between the cracks'.  Following we'll be posting daily progress reports and pictures of Quin.

Thank you again for your prayers and love.
The Hardys