SMA Blog Party! What is SMA?

What is SMA?

SMA stands for Spinal Muscular Atrophy. A disease my husband and I had never heard of until our daughter was diagnosed more than three years ago. Spinal Muscular Atrophy Type 1 (SMA1) is a genetic disorder that occurs in 1 in 4 children born to parents who both carry a recessive gene for it. (1 person in 40 carries such a gene.) There are five main types of SMA, type 1 being the most severe and the most common. SMA affects one in every 6,000 live births. This disease is degenerative and affects the motor nerves, resulting in muscle wasting and weakness. Children born with the disorder gradually lose strength in their nervous systems and become paralysed. The paralysis includes the chest and therefore Type 1 children die from repiratory related illness, usually by the age of two. There is no known cure or long-term treatment.

After learning Kaitlyn’s diagnosis we went home and researched anything we could find on the internet about SMA. The information was overwhelming for us and there were no options given to us from our local hospital about approaches we could take in caring for our daughter. There are really three options when caring for a type 1 child:
-Let nature take it’s course
-Using NIV approach (the method we chose for our daughter)
-Tracheotomy

Kaitlyn has been through a great deal in her short life. She has become dependant on machines to help her breathe, to keep her lungs cleaned out, to feed her, to monitor her 24/7, and to help her move. She has had four surgeries and was intubated for each. One intubation was for major bowel surgery to repair a torn and perforated bowel from a misplaced J-tube. Kaitlyn has never been intubated for an illness (a bit of a rarity for Type 1 children). She has a port-a-cath, an internal catheter, placed for venous access. She has a blood disorder that causes her blood vessels to form clots. She has two holes in her heart (also a bit rare, so we are told).

And in light of all this.....she laughs. Kaitlyn laughs because she is happy, because she is smart enough to know she has a reason....a right to be here! She is precocious, she is determined and she is strong. We adore how strong she is and how she never gives up (AKA stubborn). She is a joy and we are enjoying each and every moment with her!

But what would Kaitlyn wish for....what would she most want, if she could tell us? I think she would ask us for a cure. A cure for this horrible disease! She would ask each of us to do what we can to spread the word about SMA, raise awareness, and help fund a cure for SMA. Well guess what folks...Here's an easy way to help Kaitlyn in our quest for a cure!! Go to: http://www.voteforsma.com/ and vote for the Gwendolyn Strong Foundation in the Jimmie Johnson Helmet of Hope Contest as they are trying to win $20,000 towards a CURE for SMA. Please vote every day until the 29th of September. Ask your friends to join us in voting and feel free to join our blog party and tell your readers about SMA. You can talk about Kaitlyn :)

Thanks for your time, and happy voting :)

Kaitlyn Goes to Pre-school

Kaitlyn had her first day of pre-school today :) The entire day was packed with excitement! We started Kaitlyn's day early to finish her respiratory care before we had to leave the house. Tim did Kaitlyn's care while I got both boys ready for school and caught the live bird flying around in our living room. .....Wait a minute...did I say a live bird??? YES!!! I walked into the living room to see what had the cats all in a fit! Who was staring me back from the back of my couch but a real live bird!!! The poor thing was crashing into our big picture window in our living room and freaking out as our two cats were staring it down. The poor thing MUST have made it's way down our chimney! I have no idea HOW, but no doors had been open before I discovered him. Anyway, I got a towel and trapped him in the window. Then boys both wanted to see him up close and then I took him outside and he promptly flew away. He paused for a breather in a tree not too far from our deck, but was otherwise unharmed :) It's a miracle the poor thing didn't get hurt!

We made it to pre-school on time and Kaitlyn enjoyed most of her day. She liked the free play time first thing in the morning. I layed her on the floor with a pile of toys and a few of the kids came right over and started playing with her. A couple of girls in particular played for extended periods of time :) Kaitlyn can already say one of their names and was asking for her tonight after supper. She kept saying the little girl's name and then saying please!! Then she called for the little girl.....Oh Iya! Oh Iya! We played with playdough, built block towers, played with dinosaurs (STOMP, STOMP, STOMP...good times:), sat in circle time, painted a picture during group time, and "ate" a snack! Kaitlyn got to "eat" cucumber slices with the rest of the kids :) Then the worst part of the day was watching the rest of the kids line up single file to go out through a door in the back of the classroom that leads to their inaccessable playground :( We got to go outside and around the building to watch the kids play, but could not participate. I think we will have to use this time for something more constructive for Kaitlyn, as it was quite sad.

The plan is to attend pre-school two days a week for now and see how it goes. It is tiring and risky being exposed to so many germs, but to see our girl's face when she got to do what the rest of the kids were doing....priceless :) I am not sure if we will attend for the entire winter or not. I guess we will have to see how the winter unfolds.

We are Driving!

Kaitlyn's wheelchair has finally arrived! We have a custom pink bubble-gum paint job...custom seating...and very sensitive fibre-optic drive controls :) This has been and is going to be a learning process for all involved. Kaitlyn has been such a trooper through all of this!

Kaitlyn had her first try with the fibre-optic drive controls last week! Like a dummy, I forgot my camera :P I took some photos with my phone camera, but alas I have Vista and Vista does not like my phone :( LOL We had training session #2 today. We are having some minor issues trying to find exactly the right place to put the fibre-optic drive controls so that Kaitlyn can access the lights easily! She has so little movement left...even in her little fingers. With the fibre-optic lights all Kaitlyn has to do is interrupt the light beam with her finger and the chair will move until she moves her finger away from the light. She is doing well recognising that when she interrupts the light, the chair moves :) A little too well...LOL! Her OT was trying to get her to stop on command, and she would ask Kaitlyn to stop. Kaitlyn of course is looking all around and ignores the OT. The OT then corrects Kaitlyn by lifting her finger off of the drive control and Kaitlyn proceeds to holler at her :) As soon as we let her finger go you have to jump out of the way :) Move it or loose it!!!

MDA Walk 2010

Yesterday was our Walk for the Muscular Dystrophy Association here in Halifax. It was a beautiful sunny day. It was rather hot though :( Kaitlyn gets a little fussy in the heat. She made it through the day though and seemed to enjoy the Walk for the most part :)

There were a lot of people at the walk this year. Most individuals/teams either met or exceeded their goals for fundraising. We are thrilled to be able to give back. MDA has been very supportive to Kaitlyn. They have provided many peices of medical equipment over the past three years. Thanks MDA Canada!!

Here are a few photos of our Walk. Sparky the firedog was at our event :) Sparky paid Kaitlyn a special visit after the walk! She was thrilled...she adores Sparky!!

EHS & Sparky the Fire Dog

We are in the process of updating Kaitlyn's Special Patient Card with Emergency Health Services. A special patient card is a protocol we set in place so that Ambulance drivers know what to do in the event that we should have an emergency with Kaitlyn and need their services. In setting up this new protocol we have had the pleasure of meeting an EHS supervisor who has taken an interest in Kaitlyn. He has requested that our hospital set up an information day where they can learn more about Kaitlyn's condition :) They have also asked the firefighters at our local fire department to attend. The firefighters are our first responders :)

As a result of our meeting with the EHS supervisor, our local fire chief payed Kaitlyn a visit a couple of weeks ago. He brought us a reflective house number; much more visible signage for EHS and anyone else needing to find our house! They even took the time to find us a sign post, pounded it into the ground and hung the sign! The firefighters also arranged to have our road sign moved. The sign was originally placed on a telephone pole about 15-20 feet from the main road. Thank you very much to our local Fire department!

When the fire chief visited he said that he would send Sparky the Fire Dog over the next weekend to see Kaitlyn :) So today Sparky the Fire Dog walked into a very happy little girl's room!!! She was beaming from ear to ear :) She absolutely LOVES dogs and to see this great big "dog" in her bedroom tickled her :) Sparky gave Kaitlyn a stuffed dalmation dog and a firehat :) She LOVES both! Kaitlyn took the dalmation dog to bed with her :)

The Fire Chief also brought several firefighters and a firetruck over. They opened up all the compartments on the firetruck and let Kaitlyn, Alexander and Owen check out anything they wanted :) This was so nice of them!! Our children don't get to go out as much as we may like so they were thrilled to have a visit from a firetruck and Sparky. They will talk about this for days now :) Thank you again to our local Fire Department! You guys are great!

This is how Sparky arrived for his visit :)

Kaitlyn and Sparky :)

Kaitlyn and her loot :)

She likes big trucks after all...LOL

Sparky was so nice to Kaitlyn

She even tried to wave to him :)

Alex loved the firetruck too!

Firefighter Alex...hehehe

Kaitlyn took "Sparky" to bed :) (She calls her dalmation Sparky)

Ready...Set...Drive :)

Sorry I haven't written in a while. I wanted to update everyone on Kaitlyn's fundraiser for her power chair!! The fundraiser was a success! We were able to reach our goal. Kaitlyn WILL have her wheels! Our next fun....driving :) Look out brothers, walls, kittens....here she comes! We are thrilled for her to say the least :)

The chair will take a couple of months to build! We are having them build specialized vent trays to carry all of Kaitlyn's machines. She is getting an Invacare base. The vent trays are being built from scratch. Her seating is also being custom built :) It will fit her like a glove. We will of course have to replace the seating more often this way, but it is much more comfortable and supportive for Kaitlyn. She will use fibre optics for drive controls. This will take Kaitlyn longer to figure out than a joystick, but ANY mobility is better than no mobility!!! She will eventually figure it out and drive like a pro!!

I cannot wait to take pictures and video for ALL who have supported us! Speaking of which I want to thank everyone for your heartfelt support! People are extremely generous when it comes to the needs of a child! We had people attend our fundraiser that won the fifty/fifty draw and donate their winnings back to our cause.....people that could have used the money themselves!!! This overwhelming support for our daughter is NOT unnoticed!! We are just so grateful to our entire community for once again pulling together and reaffirming once again why we are so proud to be part of such a small town :) We may have a city address but our hearts are rural rooted!!!

We have also been so proud of our SMA community!! You guys are above and beyond! People facing the exact same struggles as us and they scrape together a donation for our cause!!!! This kind of act is unheard of in other "communities". We are so very greatful for your support. Our daughter would NOT be alive today if it were not for the SMA families and what they have already shared with us! Then this.....it's just so overwhelming for us! We don't know where to begin to say THANK YOU! The thanks will be in our daughter's face when she figures out the freedom the power chair has to offer!!

On another note, we have been battling a string of nasty lung infections! Bacterial pneumonias, back to back, and NOT fun!!! Kaitlyn colonizes pseudomonas (two strains...oral antibiotic resistant!), Stapholococcus Aureus, and Stenotrophomonas Maltophilia! The infections almost always hit in the same way! She starts with increased secretions, developing to a level that she becomes bi-pap dependant to control them. She almost always has fever (high 38's to 42)! Secretions are always at some point green or greenish! Then her lungs get "junky" (wet)! This can sometimes develop over a couple of days or sometimes happens as fast as 12-18 hours!!

She was sick just two weeks ago! This past illness hit her fast! She woke up a Monday morning with slight increase in secretions. She had a fever (39.4) by late morning. By afternoon she was bi-pap dependant! By evening we could finally hear the junk rattling around in her chest...up to that point she had been clear! We decided to keep her home and call the pediatrician in the morning. I had planned to get up with her through the night! When Kaitlyn gets junk in her lungs she needs chest physiotherapy (chest clapping/CPT) and cough-assist every two to three hours around the clock!! After her 2:00am treatment she was maintaining sats of 82-84 on 6 litres of O2 through her bi-pap (ventilator), with increased settings!! We decided to wake the boys and take her in to the ER!

As always she grew the same things in her cultures. She had white cell counts of 27.9 (normal 6.0-15.0)!!! Her platelet count was 107 (normal 150-400)!!! Crazy! This typically indicates severe infection! There has never been any indication of bladder or blood infection. Sometimes her ear (right-the one she lays on) looks infected.

Our question to the experts is can this happen with aspiration (that fast)?? Or does it sound more like bacterial infection in the lungs?? We are wondering if there is anything we can do to prevent these pneumonias!! It is so hard on our girl being sick!! She has been on antibiotics since last September...almost constantly!! Our longest stretch is 20 days!!

We hope everyone else has had a healthier winter so far than us! We are very impatiently awaiting spring and warmer weather!!

Kaitlyn being her cheeky self :)

My boys playing baby!! They pull their shirts down over their knees and waddle around the house =D

Kaitlyn sitting in her floor sitter talking to Owen!

Help Kaitlyn Get Her Wheels!

We have now officially been fighting for a mobilised wheel chair for our daughter for two whole years. When Kaitlyn was 15 months we started questioning about a power chair as we knew that it could sometimes take several months to get our own chair. We figured that by the time that Kaitlyn was 20-22 months (2 years old maximum) she would have her power chair and she would learn to be mobile. Children diagnosed with conditions that do not allow them to walk should be given some form of mobility around the same age that they would "normally" be starting to walk. It has been shown and printed in articles that children as young as 18-19 months are old enough to learn how to drive a power chair!

We put Kaitlyn in a power chair at around 18 months, and although the chair was a mismatched set of parts she totally GOT how to make that chair move! The parts were borrowed from a local wheelchair dealer and our local hospital. We never could get the drive controls right as we didn't have proper seating. I'll try to explain. Imagine that Kaitlyn has less tone than a newborn baby and is weaker too! She needs full body support from the top of her head to the tip of her toes in order to maximize all the strength she can muster!! Without full body support she cannot use her limited movement to operate drive controls on a power chair.

After several "trials" with mismatched parts it became more and more obvious that Kaitlyn needed a proper chair (seating, hand/arm positioning, drive controls, chair functions!!!). Something that could provide her with the support she needs and the features that would make learning to drive possible! As Kaitlyn's condition is degenerative she has lost a LOT of function in two short years :( This has made our task even more challenging! As a result, upping the price of a power chair.

Kaitlyn's new power chair will have power seating functions (tilt, recline) as she will need to adjust her own chair for comfort! She will drive her chair with fibre optic switches as we could not convince our OT that Kaitlyn would be able to drive with a mini proportional joystick. She needs to be able to move a body part in all four quadrants (left, right, forward, backward). We were unable to demonstrate this with the type of mini-joystick provided for two trials! The good thing is that Kaitlyn's chair will be able to support different types of drive controls, so her chair will adapt to her needs down the road :) This chair will also come equipped with vent trays so that we can lug cough-assist, vent, suction, feed pump and oximeter wherever we go!!

Wheel chairs can often be funded through various funding programs. However, since Kaitlyn's chair requires such an elaborate set-up and sensitive controls the price tag is much higher than most chairs. We have managed to raise the majority of the cost of the chair with the help of our local children's hospital. The major funding support comes from PC Children's Charity in the sum of $20,000!!! To all of you who have supported the Superstore when they have their yearly Charity drives (when they ask us at the checkout if we would like to donate 1-2 dollars to their Children's Charity) I thank you from the bottom of our hearts. Now you know where that money goes!!! Right back into the community :)

We were told in January that we need to raise the remaining funds needed for Kaitlyn's chair. The Children's Charity funds must be used before the end of six months from the time it's awarded. We got an extension, but that still only gives us until mid-March! As such, this fundraiser means a great deal to us as it is our last chance to get Kaitlyn her wheels!!

My mother and sister have been organising this fundraiser. They are working tirelessly to make sure that this event is a success! They have decided to have the event in Annapolia Royal at the local Firehall. The date is the 21 st of February, 2010 starting at 1:00pm. They have arranged for local musicians:

Fender Benders - Country/Rock

Janis Sheridan-vocals

Jacquelyn d'Eon - Fiddle

Matthew Stanton - Guitar/vocals

Cecile Martell - Guitar/vocals

And more...

We will also be auctioning off a few items, selling tickets on some items and a bake sale, among other surprises :)

Please come and join us for an afternoon of fun! Kaitlyn and I cannot attend the fundraiser in person, but we are hoping to be able to attend via Skype! We will be able to see everyone and everyone will see us through the web camera on my laptop :) If you cannot make the benefit and would like to make a donation you can mail it to "Kaitlyn Hatchard c/o Kathy Horton PO Box 316, Bridgetown NS, B0S1C0" Any check donations may be made payable to Tim or Kimberley Hatchard. "WE NOW HAVE A PAY PAL ACCOUNT!!" Just go to the link on the top right of this page :)