A day in the life..

People who have visited our home have told us that they did not have an understanding of William's daily medical treatments until they saw it first hand.  We are posting these videos to give a portrayal of Williams's daily care.  DISCLAIMER:  These are not medical videos or "how to" videos.

To start our morning, William gets a nebulizer treatment of multiple medications including albuterol and pulmocort.  These medications are administered through the ventilation tubing (circuit).  This is done every morning and evening.  In times of sickness the medications may be administered every four hours.  The purpose is to loosen secretions so they can be suctioned out with a suction catheter.

The next videos show the shake vest.  It is also called a smart vest or percussion vest.  This vest uses a vacuum to inflate and deflate a wrap that goes around William's chest.  The purpose of the vest is to shake out any mucus that might be stuck in the airways.  William has to endure this for 20 minutes in the morning and 20 minutes at night.  We might do this more often if William has a respiratory infection.  This is one of the machines that we believe had kept William out of the hospital.  Notice the suctioning in the first video.  Suctioning is done throughout the course of treatments to remove the secretions.  A suction catheter is attached to a suction machine.  The catheter is inserted directly into the trach.

After the breathing treatment and the shake vest we move to the cough assist machine.  This machine blows air into the lungs with force and then sucks it out with force, mimicking a cough.  The machine mimics five coughs and then the suction machine is used to pull out the loosened mucus. The process helps prevents mucus plugs.  This machine is used immediately after the nebulizer treatments and shake vest.  In tandem, these treatments remove the mucus that cause breathing difficulties.  Again this is done twice a day with additional treatments as needed.  The entire  process takes about 45 minutes.  So we spend at least 1.5 hours per day giving these treatments.

Food.  William cannot swallow without aspirating.  His food is pumped directly into his stomach though a G-Tube.  As you can see the food placed into a bag.  A pump moves the food through the G-Tube into his stomach thereby avoiding the airway.

Summer Kick Off

One of my goals for the summer is to take the kids places. To see and do fun things!

William will be 2 years old next month and in those two years we have had long hospital stays, countless doctors appointments and almost daily therapy sessions (physical, occupational, developmental & speech). I have decided that it is time we get out and explore! So far we have checked out two different parks in our subdivision. I posted a video of William driving his power chair in a previous post. We attended Mason's birthday party and this week we went to the Cosley Zoo in Wheaton and the Pride of the Fox Riverfest in St. Charles!

Cosley Zoo: Wednesday, June 5 2013

Cosley Zoo is a small local zoo that is part of the Wheaton Park District. It is less than an hour from our home so we decided to check it out! We picked up Juliet from her last day of Pre-school and went to the zoo.  Juliet loved it. Her favorite animal was the peacock. We circled back several times to see if the peacock was showing their feathers and finally we saw peacock's beautiful feathers. William is a cautious boy and had to process the day. He defiantly was unsure of the situation, but I saw him checking things out. Later that evening I caught him playing peekaboo barn app on his ipad.  It is a simple app that has animals hiding behind a barn and when you tap on the barn it says the animal name and has the animal sounds.  I have not seen him play with this app in a really long time. I would have to think it is not a coincidence that he came home from the zoo that has lots of barnyard animals and then selected this barnyard app on his ipad.

William and Juliet see a sheep

William checks out the horses

Juliet poses by the birds

William looks at the birds

Pride of the Fox: Friday, June 7, 2013

Second adventure of the week, Pride of the Fox in St. Charles! They had lots of children's activities. First we started with the bounce houses.  Juliet jumped and William took in the sites.  Next we headed over to the face painting tent.  Juliet decided that she wanted her face painted like a princess and we picked out a dog temporary tattoo for William's arm.  William was funny.  He sat patiently like he always does watching all the kids.  I do not think he realized that he was in line too.  As soon as he got up to the front and saw that he was next he started shaking his head no. We convinced him it would be ok. We then visited the animals at the petting zoo and Juliet went for a pony ride.  William watched all the animals and had lots of smiles! He even smiled for the picture in the below photo!!

William smiles for a picture

Juliet rides a pony

Juliet pets a baby goat

Juliet's princess face painting

Birthday Party Fun

Jenn and birthday boy Mason

This past weekend we celebrated a special birthday with family! It was Mason's first birthday!! Mason is Dan's cousin Jenn's little boy. Mason is pictured below with his smash cake!

Mason and his smash cake!

Up to this point, we had never taken William to any family events. Our main concern has always been his health. We have tried to limit his exposure to illness especially during cold and flu season. When we got the invite in the mail for a party on June 1st, we wondered if maybe this time we could take William.

William with his talker

William has been great lately, so the day came and we decided to take him to Mason's birthday party! We loaded the family and all of William's medical equipment into the van and we were on our way. The trip to Jenn's house took around 2 hours, so the kids watched Elmo in Grouchland. (One of William's favorite movies.)

William and me

When we first got to the party William  needed to warm up to everyone and all of the excitement.  We gave him his iPad and talker. He took turns playing with each and would regularly glace around at the balloons, people and activities.  We found a comfortable place to sit down with him, where he could see the party and family could come and say hi.  I tried to be as comfortable as possible, but I too needed to warm up to the situation. (It is funny really to be nervous about a family birthday party considering that have taken him to appointments all over the Chicagoland region.)

William in his new hat!

William did great! By the end of the party, William was getting into the spirit of things and began acting silly. Dan's Aunt Noreen (Aunt Nonie) started asking him all sorts of questions. All of which William would shake his head no to with a grin. He was having fun! Thank you Aunt Nonie for getting William to act silly with you and all of these fun party pictures! William even got a new hat! Thanks Uncle Den for the cool new John Deere hat!

And most of all thank you Jenn for throwing such a wonderful party.  It was a perfect first family party for William! We look forward to many more Whiston parties!!

Juliet with her Nema and Papa
(Brian & Debbie Whiston)

William shows Aunt Katie his iPad

William's thinking pose

Amanda, Madi & Juliet

Juliet & PJ acting silly

Megan, Debbie & me

William Exploring his World

I am so excited to finally share a video and pictures of William in a Power Chair!! 

Back in April we took William to a power chair evaluation and I shared our experience in a blog post titled Power Chair. William was not excited about trying out a power chair at the appointment. However we asked that a demo or loaner chair be brought to our house for him to try.  We have now had the demo power chair for two weeks today. The video included I took yesterday at the park! 

William has shown consistent improvement with his chair.  At first he was very cautious and just tapped the joy stick. He then started with clockwise circles - pushing the joy stick inward towards himself. Now he is working on forward and backward.

He loves his power chair. He almost always shakes his head no when it is time to get out. It is so exciting to let him explore his world on his own for the first time. Yesterday we took him to the park! Of course he was very cautious about the new place at first, but then began driving around and had a great time. He shook his head no as we were leaving and after we loaded him into the van I asked him what he thought of the park. He smiled and gave me a high five.

William playing at the park. 

During our two weeks of power chair practice, we have spent a lot of time in our garage and/or in the drive way.  Juliet recently got a bicycle for her birthday, so we all head outside for driving/riding practice. I have determined with two young children that do not yet understand how to stop their moving vehicles, practice sessions require two adults.

William and Juliet with their new wheels.

Who is taller?

The other day I had the opportunity to get a picture of William and Juliet standing beside each other!

William's PT has been having him stand against our ottoman.  He wears his AFOs, knee immobilizers and his TLSO then she braces him from behind.

Juliet (4) and William (22 mo.)

William standing with the help of his PT

It is amazing to me how close they are in height. William has always been very tall for his age.  Typically in the 95th percentile or higher on the growth charts. And no, Dan and I are not tall people.  Typically MTM boys are tall. I first wrote about this back in October when we first got William in his stander. 

Kool-Aid Playdough

Today William's Developmental Therapist brought stuff to make kool-aid playdough!! Here is the recipe and some pictures. Enjoy!!

Kool-Aid Playdough

• 1-2 packets of Kool-Aid
• 1 cup flour
• 1/2 cup salt
• 2 tablespoons cream of tartar
• 1 tablespoon of vegetable oil
• 1 cup of boiling water

In a large bowl add all dry ingredients and mix well. Add the oil and boiling water to the mixture and mix until it becomes very firm. It should be cool enough to handle after a few minutes of stirring. (It will last a long time if refrigerated in a plastic bag.)

William takes a moment to think about this.

He decides he's in and helps stir!

Rollin Rollin Rollin

Playdough toys and a Cookie Monster Car

Mr. Potato Head gets a new head an a hair cut.

A Mr. Potato Head Masterpiece!

What are we making next?

Tasting the kool-aid playdough.

Thanks Brittney! The kids had a blast!!

Power Chair

Yesterday we took William for his first Power Chair Evaluation at Shriners Hospital for Children in Chicago, IL. All things considered, William did ok.

Our appointment time was at 10 am, so William's day started out earlier than normal so that we could complete his air way clearance routine (breathing treatment, 20 minutes of his smart vest & cough assist) and run a feeding. William is not a fan of getting up early and with a little over an hour of travel, by the time we got there William was ready for a short nap. We answered some questions about William's abilities and then moved him into a Power Chair.  He started crying immediately after we placed him in the chair.  Initially we made the mistake of taking William off the vent to try out the chair. Although respiratory wise he was fine, he was upset that we changed his environment so dramatically so we gave him the vent back. He calmed down, but was still not happy about being in a new chair, in a new environment with new people around. During the evaluation he showed interest in the control buttons and even pushed the joy stick a few times on his own. We did a short lap in the hall way of Shriners and then headed back to the room to discuss the next steps.  Once parked, William fell asleep in the Power Chair.

Next we are going to get a Power Chair brought to our house to borrow and use to practice with William. We are confident that William will be able to learn to use a Power Chair! Stay tuned for practice videos.

William learning to use a Power Chair

William in a using Power Chair for the first time

April 2013 Updates

I thought I would share some updates and pictures of what the Whiston Family has been up to lately.

William in his blue TLSO

Juliet at TLSO Appointment

Most recently, William just got has first TLSO (Thoracolumbosacral orthosis). It is a brace used to correct the curve the spine from scoliosis. In December William had an x-ray of both his spine and hips.  The x-rays have been seen by Williams MDA (Muscular Dystrophy Association) Orthopaedic Surgeon, his non-operative Othopaedic and Physiatriast doctors.  They are all in agreement that William's spine and hips look good! I had previously been told by them not to be surprised if the x-rays showed some curvature.  So I took this as great news!! I was told that there are no studies that have shown that the TLSO will prevent scoliosis. However his physiatriast and physical therapists recommended we get him a TLSO sooner rather that later.  I of course agree I would most defiantly rather be proactive rather than reactive whenever possible. We are going to trial it out and see how it goes.  I am curious to see if it will help him sit in his wheelchair for longer periods of time and tolerate feedings in his chair better. I am also curious to see how it will help with his independent sitting.   







Lately we have been reading books with the kids before bed. I usually sit with William, acting like his human bobby so that he can sit up and turn the pages with us. We take turns alternating between Juliet's and William's books.  William usually is described as a patient and easy going boy, however when it comes to his books this is not always true. When it is time for a Juliet book, he shakes his head no and tries to close the book.  

Debbie Whiston (Nema) reading with Juliet and William before bed

Earlier this week we went to the DuPage Easter Seals for a Assistive Technology Evaluation. 

William with his "talker"

About a month ago, William's speech therapist rented a Springboard Device for us to practice with using with William. I like to refer to it has his "talker". William of course being the techy that he is instantly loved it. First we worked with the book Brown Bear Brown Bear, but he soon learned that there is more to the devise than just reading Brown Bear.  He found the medical section and got everyone to laugh by saying "I need a band-aid" over and over. His day nurse tells me that often says "help" "stop" "working" when she is catching up on her charting.  (William of course does not need help.)

So back the the evaluation, I was really worried that William would not interact with new people and just put his finger to his eye and assess them and the situation, but he did well.  He worked with them for a good hour showing them what he can do with the Springboard device we are renting and his iPad. They also trialed the NOVA Chat 7 device and the iPad mini with the TouchChat App. The hour seemed to be his limit. At about that mark, the therapist needed to adjust some settings and I made the mistake of letting him play with his iPad while he waited.  Well of course when it was time to work again and I took away the iPad, William got upset. He repeatedly pushed the stop sign saying "stop" and then when she hid that symbol he found the "I don't want to" button and navigated back and forth from the home key so he could say it over and over.  And when he still did not get his iPad back he shook his head back and forth to say no. At that moment I was so proud of him! He communicated three different ways what he wanted. Two of which were with a device that is brand new to him! Their assessment was that William will succeed and learn any device or app that we select for him and that his understanding and use of combined language and navigation is actually further than they would have expected for his age. Way to go William!!

William enjoying an Orange Sucker

While we are on the topic of speech, we have started to give William some suckers to work on some oral motor skills.  He loves them!! 
Some of you remember that William used to love his pacifier when he was younger. Unfortunately after his trach and vent, he gave it up.  I was quite sad when he refused to take it, I knew that it helped his oral motor strength and helped with swallowing. (I even tried to trick him once and give to him while he was asleep. He of course woke up, took it out of his mouth tossed it and stared me down.) It is funny really, most parents want their children to give up their pacifiers at about that age.


When his speech therapist first started to work with William after his trach and vent he would not even let her close to his mouth. He now lets her touch the inside of his mouth, he sticks out his tongue and he actually likes his electric tooth brush. This is wonderful progress! (I am wondering what he will do if I rub sucker on a pacifier.) 

Juliet and William

We have also been spending more time in our front room, looking out the window.  William likes to watch the neighborhood starting to come out this spring.  He especially thinks it is funny if I go outside and wave to him from outside.  I think I even got a wave out of him a couple of times!

Juliet and William

Cautious optimism: My visit to the University of Washington.

When Melanie and I decided that we would put our resources towards finding a treatment for MTM, we sought out the people that were working towards the shared goal.  Today I learned that the team of researchers are working tirelessly towards a goal that I once believed would never be accomplished. I am now excited at the possibility that one day we will beat this disease.

Where There's A Will There's A Cure was started to promote research.  Our family and friends dedicated themselves to raising money and awareness.  In under one year we were able to raise nearly $60,000.00 towards this goal. (If you want to meet some of the great people that have donated time to this cause go to the Will Power page at www.will-cure.org)  I had the opportunity to present $50,000.00 to Dr. Martin Childers in order to continue research of gene therapy.  In visiting the lab and meeting with the team, I came away with a wealth of new knowledge.  I thought I would share some of what I learned with you.

1.  The groundwork.
In listing to the oral history of MTM gene therapy, I was struck by the amount of time and effort that has gone into all of the work that has gotten us to where we are today.  The first lesson was that the Frase Family put in more WORK than most people understand.  When I use the word work, I am referring to time and effort.  I have watched Melanie work tirelessly to raise every cent that she possibly can, yet we have not had to find scientists and doctors to conduct the research. Prior to today I did not grasp the amount of effort to that came in the decade prior to William's birth.  The presentation made it clear that 10 years ago there was no framework in place.  I write this because I was truly shocked at the amount of time that has been dedicated to our common cause.

2.  UW Medicine.
The second thing I took away from this meeting was the dedication and excitement of everyone on the research team. In meeting the entire team, one could clearly see that each person is excited about the work and results. They believe they will be successful. They truly believe they are on the doorstep of a treatment.

3. The "wow"
Dr. Childers presented his colleagues with data from tests. As graphs and numbers appeared on the screen, two scientists gasped and said "wow". This reaction was the single biggest verification of Dr. Childers work. Clearly the early testing has gone well. While early results do not guarantee anything, I can say I left excited and optimistic, yet cautious.

The LONG Month of February

One of the things I find hardest to do is share our "real" day to day story. It is easy to post cute, happy pictures of William's progress, but I find it hard to write or illustrate the days that are hard and exhausting. Part of me thinks that it sounds like complaining and the other part of me is holding my breath and praying that things do not get worse. I find it hard to write about what seems to be a hard day or month when I know so many children like William that have been in and out of the hospital. We have been there and those days are so much worse. William has blessed our lives in so many ways and one of those is that we have been given a unique perceptive about life, love and family. I am so thankful for every second I am given with my son and my family.

With that being said, here is why I am ready for spring. The long month of February 2013. The month started out with us losing our Monday and Tuesday day nurse. She needed a different schedule and a case closer to her home to take care of her family. So a new nurse was orientated and set to start the first Monday of the month. Unfortunately, I had to send her home with in the first 2 hours. The entire house heard that she was unable to complete the least complicated test on the ventilator.  When the night nurse was trying to help instruct her she stated that she "hates these machines" and then blamed her test failures on the fact that she had stomach pains and diarrhea.  Our night nurse, who has been with us since we brought William home with his vent and is fabulous, reported this to me as it made her feel uneasy. So I then question the new nurse about how she is feeling explaining that it can be fatal for William to get sick and that if she is not feeling well it is better that she go home. She tells me that she is 99% sure she is not sick and attributes it all to the fact that she was up at 3:00 AM to shovel the snow (really?) and that the roads were really bad. She also said that she had not called in sick in 20 years (really?) and when I asked her if she got a flu shot she said that she once had a bad reaction and doesn't get them (really?). I asked her to go home and asked the nursing agency not to send her back. I will never know if she was really sick or just had a bad morning and was nervous, but my instinct told me that she was not the person I wanted to trust with the care of my son.  Coincidentally Friday evening William spiked a high fever and I called his doctor after hours to get a prescription for Tamiflu. Within 24 hrs his fever was gone and he was back to himself with just extra nasal secretions.

That Sunday Dan and Juliet went out to a spaghetti dinner fundraiser. Shortly after they left, I get a call from Dan. He tells me that he had fallen on ice and can not move his arm. He was sure something was broken. Of course it is a time of day we do not have nursing help so I am unable to leave the house.  Thankfully a very kind fellow Mason, Mark Jacobs, drives Juliet home.  Juliet being the strong, independent girl that she is gives this stranger, driving her mom's mini van, directions home and initiates a conversation about the Bears and Jay Cutler. Mark then goes to the ER and sits with Dan and brings him home when he is discharged. Thank you Mark!! Meanwhile I call Dan's parents to let them know what is going on and Debbie gets in the car and drives up to help! Thank you Debbie!!

And then it began, the sickness. Whatever illness we were all exposed to takes everyone out. (Except Juliet...she seems to have the strongest germ fighters.) Our week day night nurse called in sick the Sunday Dan was injured, our Wednesday thru Friday day nurse called in sick two of the three days and our weekend night nurse called in sick both days the following weekend. (Plus we still do not have anyone on Monday and Tuesday during the day.)

By the end of the week, William had gotten worse. His lungs were no longer clear. We aggressively get him a steroid prescription and give him albuterol breathing treatments, 20 minutes of his smart vest wrap and cough assist every 4 hours for basically the rest of the month until we could return to his normal airway clearance routine of just twice a day. (The smart vest wrap goes around his chest and is vibrated for 10 min. at one frequency and then another 10 min. at a higher frequency to loosen secretions. The cough assist helps produce or simulate a cough by forcing air in and out.) To all of our nurses credit, we were able to keep William from getting pneumonia and kept him out of the hospital. They took wonderful care of our son.  We are so appreciative and grateful.

We thought March would be return to normalcy.  A new nurse was scheduled to start on the first Monday in March.  Well, she did not last long.  On her first day on the job I helped show her all of the therapies and tricks to care for William. While I was upstairs switching the laundry, Juliet quietly went downstairs.  Unknown to me, Juliet starts quizzing the new nurse. According to the nurse, Juliet's interrogation went something like this:

Juliet:   Do you know how to use the feeding machine?
Nurse:  You put the formula in the bag and prime it through the machine.
Juliet:   Do you know how do you give him his breathing treatment?
Nurse:  The nebulizer hooks into the ventilator tube.
Juliet:   When do you give him his medicine?
Nurse:  Juliet, I know how to take care of William.
Juliet:   That is not the question I asked.

Needless to say, Dan was happy that his daughter was checking up on her little brother and was skilled on cross examining.

So, the nurse quit after her second day citing that she found Juliet's behavior offensive and that I hovered too much. I am not upset that she quit, we all knew that she was not going to be a good fit. It is just frustrating and exhausting to be short of help.

William getting his smart vest wrap treatment.