September 24, 2010

Can you hear me know??

Most of you know Tylor will lose his ability to walk, talk, eat, and breath on his own. So it is very important to get Tylor's hearing and vision checked on a yearly basis. Because of Ty's low communication ability he we wouldn't be able to tell if his hearing or vision was going bad. This past week it was time for his hearing test and he passed with flying colors. Jenny, who does the testint, is so good with him. I was a little worried half way through the test because he stopped telling her when he heard the beep so she had him repeat words back to her. She said that he could repeat her perfectly after hearing the word in a faint whisper, which means you have to have great hearing to hear that low. So we figured he was just getting bored with the first test and stopped responding. After the hearing test we went upstairs to see Dr. White. He checked his ears, nose and throat and was pleased with how everything looked. So unless something changes we will see him in a year.

Ty finishing his test

Could Cyclodextrin be one step closer?


By Amy Dockser Marcus

We’ve been following the efforts of one family, the Hempels, to draw attention to the potential of cyclodextrin, an experimental drug that may have potential against a fatal neurodegenerative disease called Niemann-Pick Type C.

Addison and Cassidy Hempel, twin six-year-old girls, have the disease, and their parents were part of a larger group that earlier this year successfully got orphan drug designation for the compound from the FDA, a key first step for drug development in rare diseases.

The FDA this week gave permission for cyclodextrin to be injected into the spines of the girls. They’ve been receiving the drug intravenously for over a year but new research indicated that it does not cross the blood-brain barrier.

Chris Hempel, the girls’ mother, tells the Health Blog that the family pursued the new approach for delivering the drug in the hopes it will get into the brain and stave off some of the neurological decline associated with NPC. She also hopes that the information they report to the FDA may help bring cyclodextrin one step closer to a trial.

A spokeswoman for the FDA says the agency can’t comment on drugs under agency review or on any individual’s private medical information.

It isn’t easy to get clinical trials going in children with brain disorders. The Children’s Neurobiological Solutions Foundation held a meeting last week with clinicians, researchers, advocates and others to discuss some of the barriers, including the potential risks to participants. (Those can leave drug companies and hospitals skittish.) The foundation is trying to create a national network of sites in the U.S. that will collaborate on setting up trials for pediatric brain disease treatments.

The NIH held a workshop on developing therapies for NPC disease earlier this year. Cyclodextrin played a prominent role there too. A working group of researchers and clinicians has been discussing what scientific questions need to get answered before a trial could be launched.

Hempel tells the HealthBlog that she hopes this latest FDA decision will help propel larger efforts to start a trial. “The biggest hurdle for a trial is showing something is safe,” she says. “If we show this works in the girls, we hope a broader trial can start for kids with NPC.”

Thank you to the Hempels for all their hard work and PERSEVERANCE!! We love you Addi and Cassi.

September 23, 2010

National Niemann Pick Online Auction

Main Article Image
With Niemann Pick Awareness Month right around the corner I wanted to let you all know that the National Niemann Pick Disease Foundation (NNPDF) is having an online auction to help raise money for research and to help families who have children with Niemann Pick Disease. The NNPDF has been a great support system for us. We have meet many great people through the foundation and it comforting to know you can talk to peolpe going through the same things your are.

Please take a moment to visit the online auction and please bid generously!! There is a Brett Favre signed Viking jersey donated from the Brett Favre 4ward Foundation in honor of Tylor. We wrote the 4ward Foundation after Ty met him asking if he would donate something to either the Make A Wish foundation or the NNPDF. About six months went by and we didn't hear anything and then the NNPDF calls me to ask about the Favre jersey they received. I was so touched that Brett did that he is truly a giving man. Thanks Brett!!

Help spread the word!! October is Niemann Pick Awareness Month

October is Niemann Pick Awareness month and we are asking for your help to spread the word. Recently Actelion Pharmaceuticals LTD interviewed two families that struggle with NPC everyday. Actelioin Pharmaceuticals LTD has been working with the National Niemann Pick Disease Foundation to get FDA approval for Migulstat (Zavesca), the experimental drug Tylor is taking to help slow the progression of NPC.
This video shows how differently NPC effects each child. But most have the same horrible beginning....the time it takes to figure out what is going on with your child. This is where you can help! Spreading the word will make people more aware of NPC which in turn with help in raising money for research. There are great doctors and scientists working to find a cure for NPC but this all costs a tremendous amount of money. The NPC families contribute what they can but we need your help to save our kids lives. Please help by passing this on to family and friends and ask them to do the same.

Summary edit of the Hollie and Annie films from sue lowe on Vimeo.

September 20, 2010

AFO casting

Last Friday Ty got casted for his AFOs. I wasn't for sure how it was all done so I told Ty if he was good and did what Pat told him to do we would be in and out of there. He was pretty tired because they had spirit day at school so he was outside most of the day! After the paperwork was done we headed into the casting room. 
Tylor waiting for Pat

Casting supplies
First he put a sock on Tylor that he marked where the brace need to start and where his ankle bone is and other bones in his foot. Then they use two packages of cast material, they dip it in water and start to wrap the leg from where the brace starts and work their way down. Under the casting they place a long tube to cut along after the cast is set. This way when they remove it they won't cut the patient. After they get all the casting on they rub over the outside and make sure the foot stays flat. It only takes about 3 minutes for the cast to form then it is time to cut it off. Tylor didn't mind the cutting of the cast but he hated when he cut the sock with a scissors. He kept saying ooch and wrinkling his face. I didn't hurt but I think the thought of a scissors cutting along your leg bothered him. 

Pat marking Ty's casting sock

Starting the first cast, see the long tube

First cast done, way to go Ty Bug

There it is...I told Pat we need to use these for Halloween!!

Working on the second cast

See what I am talking about, look at Ty's face!

September 15, 2010

Walk For Wishes

Most of you know that Tylor was granted a wish through Make A Wish in 2008. They flew us out to New York to meet Brett Favre. This trip meant the world to Tylor and he still talks about it, when he sees Brett on TV he yells out I met him. The memories made on that trip will forever be with us and we want to help our families make memories like ours.

Tylor with Brett at the Jet's practice

On October 3rd Make A Wish is holding a fundraiser called Walk For Wishes. The event will start at Murphy Park, registration time is 8 to 9:30 and the walk is from 9:30 to noon. After reading about the walk I thought what a great way to help raise money to grant more wishes. We have formed a team called Walkin' 4 Tylor and we would love for you to join us!! Please see the Make A Wish website to join our team or make a donation.

Click Here to Donate


Tylor two days old
I can't believe that Tylor is already 14!! Where has the time gone? Tylor has brought me so much enjoy. I was going through pictures and old video tapes the other night and we have made some great memories! On Saturday we made more great memories!! The Dragon Boat races were held on September 10th-12th at Camp 17. We have been going to the Dragon Boat races for the last 4 years to camp and celebrate Ty's birthday. Ty loves to see his friends and family so he was very happy to see how many of them showed up on Saturday. This year Ty's Dad and his family came too. It made his birthday that much more special to have both his families there.

Tylor got his first present on Friday night

After we ate lunch it was time to hang up the pinata! I knew there would be a lot of kids coming so I stuffed Scooby with over 200 pieces of candy!! Scooby only lasted for a few swings....the kids really hit hard. Sy and Ryan figured out a way to hang him back up so the rest of the kids could take a turn. After all the kids got a crack at it, Sy the big kid took one swing and rained candy.

While the kids finished picking up all the candy I got the cake ready. It was way to windy for the candles but that didn't stop us from singing Happy Birthday. Ty was slow at eating his cupcake so I thought I would help him get started and then oops I missed his mouth.....

Got Ya!!

Than of course it was time for Ty to open his gifts. It was close to his nap time and Ty was getting tired so he let the younger kids help open his gifts. He got a lot of homemade cards, movies, stress balls, and more. After all that excitement it was time to nap but guess what all the kids wanted to lay by him so he didn't sleep much.

When the sun started to set we started the fire and broke out the marshmallows, graham crackers, and chocolate.  Tylor loves S'mores and sitting by the fire but soon it was time for bed. He had such a long day but he did great.

Becky wouldn't let Ty go to bed