Tuesday, July 7, 2009

More from the Hawn Family




SMA Treatment Acceleration Act of 2009

I have become aware of a bill currently in Congress to help find a cure for SMA and fund research. This link will take you to the full text of the bill http://www.opencongress.org/bill/111-s1158/text . I have created a petition as a link on our blog and am URGING you to PLEASE sign this petition and pass our link on to everyone you know to sign also. When we reach our goal of 100,000 signatures, I will send the petition to Congress so they can see how important this bill is to so many people.

When Ryan was first diagnosed with SMA, I thought it was a rare disease that didn't effect many children. Since, I have discovered hundreds of families, devastated by the disease. I have been brought to tears more times than I can count by the stories of others. Please, help us fight for these families and children. Help find a cure for SMA.

Jennifer Hawn

http://www.thehawnfamilyweekly.blogspot.com





SMA


SMA is a muscular disease passed on genetically to children by their parents. You can not "catch" SMA by being around someone who has it. It is a "Recessive" genetic disease, meaning that BOTH parents must carry a copy of the recessive SMA gene. There is only a 25% chance each pregnancy of the child having SMA and a 75% chance each pregnancy that the child will be healthy. One out of 40 people is a carrier of this recessive gene. The brain is not affected, and they have been tested to have at least average to above average intelligence. Please do not make the mistake of treating them as mentally impaired!! Their bodies may not be perfect, but their minds are, so be sure to treat them that way! SMA affects a child's muscular development, and the severity depends on what 'type' of SMA the child has. There are four "Types" of SMA, Type 1,2,3 & 4. The earlier the symptoms are noticed, the more severe the type of SMA. Type 1 is the most severe, affecting children while still in the womb or shortly after birth. Type 4 is the least severe, affecting adults.

Type 1
children are diagnosed usually before 6 months of age, more often before 3 months of age. Symptoms may even start in the womb. Many mothers later recall the baby not moving as much the last month or so of pregnancy. They are not able to hold up their heads, roll over, crawl, sit up without support, or walk. All of their muscles are extremely weak, with the weakest muscles being the legs, upper arms, and neck. Their chest may appear concave, or very skinny at the top, with a big belly. Bell-shaped. SMA affects all muscle systems as well including sucking, swallowing, digesting food, and excretion. Constipation is a common problem as is being able to control excessive drooling (secretions), and getting proper nutrition and calories for proper weight gain. A common cold can easily turn into pneumonia which is what usually takes the lives of these children, along with "respiratory failure" or when they no longer have the lung or chest muscles to be able to breathe on their own. Two major decisions must be made with Type I children...whether or not to insert a feeding tube to prevent pneumonia and prevent starvation when they have lost their ability to suck or swallow; and whether or not to put them on a ventilator or other breathing machine when they experience respiratory failure. Current statistics show that the average lifespan of a child with SMA Type I, not put on permanent ventilation or "life support", is only 8 months of age, with 80% dying by the age of one, and the majority of the rest dying by age 2. HOWEVER, these statistics are not a hard and fast rule. Each child is affected so differently by SMA that they do not all follow the same path or progression. Also, as more is learned about SMA, the lifespan of a Type 1 child can be lengthened depending on the severity of the symptoms for each particular child. Last but not least, the line between each Type of SMA is not clearly defined, and it is common for a child to exhibit patterns of two types, thus confusing the issue of "life expectancy" for that child.

Type I children most often have very little leg movement, very little upper arm movement. Many times their hands remain fisted and their hands/wrists are turned the "wrong" way. The physical characteristics that often "gives them away" to having SMA is a bell shaped body, legs that stay in the "frog" position, moving the arms from the elbows down only, and the head tilted to the side because of lack of neck muscles. They often have bright, expressive faces and eyes.

Type 2
children are diagnosed before 2 years of age, usually more like 15 months. These children are usually able to be in a sitting position without support, but often can not get there by themselves. They can sometimes crawl with bracing and therapy, and on occasion may stand with braces. Feeding and swallowing problems are not common in Type 2 children, though they are still possible. They will usually never walk. The lifespan of a Type 2 child varies so widely, there isn't one! They could pass away at an early age or they could live well into adulthood. As with all forms of SMA, weakness increases over time.

Type 3
children are diagnosed between 18 months of age and early adolescence. In the beginning these children are able to stand and walk but usually have difficulty doing so. They typically have a normal lifespan; however, as with all forms of SMA, weakness gets progressively worse and they usually will be wheelchair bound.

Type 4 SMA is an adult SMA, with symptoms beginning around age 35. They also usually have a normal lifespan; though, as with all forms of SMA, weakness gets progressively worse.

SMARD: Info from JTSMA
Spinal Muscular Atrophy with Respiratory Distress Type 1 (SMARD1) is a neuromuscular condition causing weakness of the muscles. It is the second anterior horn cell disease in infants in which the genetic defect has been defined. SMARD1 is not linked to the SMN1 gene locus on chromosome 5q13 (classic SMA), but is caused by mutations in the IGHMBP2 gene on chromosome 11q13. Despite a substantial overlap in clinical features, the phenotypes of SMA versus SMARD1 can be distinguished. In SMARD1, the predominating symptom is a severe respiratory distress due to a paralysis of the diaphragm. Most patients present [show symptoms] at the age of 1 to 6 months with respiratory failure and progressive muscle weakness with predominantly distal lower limb muscle involvement. Sensory and autonomic nervous systems may also be involved. Early involvement of the diaphragm and predominance of distal muscle weakness clearly distinguishes SMARD1 from Type 1 SMA. Essentially, in SMA1, symptoms manifest in reverse order. Infants with SMA1 will become floppy due to weakness of the proximal limb muscles and assume a frog leg position before they suffer from respiratory failure. In contrast to SMARD1, SMA1 infants have intercostal recessions and develop inefficient respiration due to paralysis of intercostal muscles.

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About Our Family

My photo
Missouri, United States
Clinton and I live in Missouri. Clinton works for CED, Inc. It is an electrical distributing company. He works in Inside Sales right now and is training for Outside Sales. I am an Independent Marketing Consultant for Melaleuca. I work from home and I really love what I do. Cora is 3 years old and she is so excited about starting Preschool in the fall. She loves everything Princess and her favorite color is Pink! Miles is 2 and he loves CARS the movie and is really into Thomas the Train as well. He is a ball of Fire and keeps us on our toes! And in keeping with stereotypes, loves the color Blue. Well, Wyatt Evan Fisher is here now!! He arrived at 5:03pm on June 22, 2008!! We are so very blessed and so very excited! He's 7 pounds and 5 ounces, 20 inches long! Healthy and so very handsome! We are so blessed and so proud of our new baby boy! Precious blessing from God!

Fisher Farm Family Cookout!

Fisher Farm Family Cookout!
Cora was swinging with her cousins.

Fun Fall Pictures!

We had a blast taking the kids pictures! It was a gorgeous fall day and the kids were in great moods! We really loved Miles "cheese" face! We tried to get him to say something else so he'd actually have a smile instead of the "cheese" lips! :)

I have included some of Hannah, Major and Cannon. They are great friends of Cora, Miles and Wyatt. We have had a great time being neighbors and friends! We have been really enjoying doing things together.



Cheese Lips!

Again with the lips! :)

The Ulitmate Mud Puddle Adventure

Miles LOVES Puddles! We have had a lot of rain lately and so the dead end fills up quickly with the run off. This is such a great joy for Miles. He loves water and jumping into puddles and making quite a mess of things! Take in mind that it's October and quite chilly out~this means nothing to Miles~he feels no cold. Now, since daddy took the pictures, please excuse the fuzzy ones! :)

Painting the Pirate Ship!

Cora and Miles love painting! They had a great time painting and I had a wonderful time watching them, taking pictures, laughing, being amazed at how quickly they are growing up, in awe of their creativeness. I am so blessed to be their mom. What a precious gift God has given me. I am so thankful!

Puddin'

I decided that it would be fun to let the kids play with their snack as a sensory activity! So, we had chocolate pudding to "paint" and granola and dried fruit to "decorate the painting" and their bodies as the "canvas"! It was a hit!

Miles wasn't sure at first~he usually revels in messes, but this time Mom was "letting" him and he just didn't know what to think about that! It didn't take him long to get into it though.

Cora had a great time and she dug in right away! When Dad came home from work, she invited him to join the fun as well! He took off his shirt and soon he was the canvas that the kids were painting! Cora wanted to make sure that his entire back was covered!

Wyatt remained an outsider to the event~which I am sure he appreciated! He really enjoys watching his brother and sister play. He smiled and laughed and cooed a lot! It was a really enjoyable time for us all! If you haven't had fun with pudding, I suggest you try!

Cora and her "Pudding Art Creation"

Cora and her "Pudding Art Creation"
We have chocolate pudding, granola, and dried fruit!

Puddin' face!

Puddin' face!

Miles was just getting started!

Miles was just getting started!

Now we are getting the hang of it!

Now we are getting the hang of it!

Cora loved the feel of the "squishy" pudding in her fingers!

Cora loved the feel of the "squishy" pudding in her fingers!

Daddy jumping in the fun!

Daddy jumping in the fun!

HMMMM......

HMMMM......

Almost got it all......

Almost got it all......

The Finished Paintings!

The Finished Paintings!

Now with silly faces!

Now with silly faces!

Cora's First Day of School!

Cora's First Day of School!

It was chilly morning.

At School

At School
In front on her school.

Her Cubby

Mrs. Creason~Cora's Teacher

Mrs. Creason~Cora's Teacher

Oh bath time!

Oh bath time!

May I Help You?

May I Help You?

Here's lookin' at you kid!

Here's lookin' at you kid!

Wondering if we could just go on our walk already!

Please???

Please???

Wyatt always falls asleep in his backpack on our walks!

Playing in the sprinkler!

Playing in the sprinkler!

Hannah, Grant, and Cora~Umbrellas to "save them" from getting wet....didn't work out so well!

Hannah, Grant, and Cora~Umbrellas to "save them" from getting wet....didn't work out so well!
Hannah and Grant are our neighbors. Hannah lives next door and Grant lives caddy corner across the street.

Hannah's brother Major.

Hannah's brother Major.
Major and Miles are good friends~they love to play together!

Fort Umbrella~all the kids are inside.

Fort Umbrella~all the kids are inside.

Miles coming to join the Umbrella Crew.

Miles coming to join the Umbrella Crew.

Cora Loves Playing With Finn!

Cora Loves Playing With Finn!

Clinton Jumping Over Cora!

Clinton Jumping Over Cora!

We take Jason's Dog Disel, for a walk everyday! Cora likes to hold the leash by herself.

We take Jason's Dog Disel, for a walk everyday! Cora likes to hold the leash by herself.

Cora LOVES to ride her bike! Everytime we are outside, she's on her bike!

Cora LOVES to ride her bike! Everytime we are outside, she's on her bike!

Miles Messes

Miles loves to make messes! This montage of cereal was waiting for me one morning and boy was I happy about it..... This isn't the first (or the last ~ I'm sure) time this has happened. If you don't get him food the moment you hear him wake up~BEWARE! Oh the joys of parenthood!

Miles Helping Himself to Breakfast.....

Miles Helping Himself to Breakfast.....

Cora and Aunty A making Cheese Cake!

Cora and Aunty A making Cheese Cake!

BIG SISTER

BIG SISTER

Cora is always asking to hold Wyatt

Cora is always asking to hold Wyatt

How Sweet

How Sweet

Into the face paint from halloween.....

Into the face paint from halloween.....

Miles thought Wyatt needed some too!

Miles thought Wyatt needed some too!

Bath Time

Bath Time

4th of July

4th of July

Four Wheelin' Anyone?

Miles loves Uncle Paul's four wheeler! He just loves to run to the shed, get the helmet on (which used to terrify him) and climb on! If he can talk daddy into it (which isn't a hard thing), he gets to ride all over the farm!

Clinton Painting Cora's Nails

Clinton Painting Cora's Nails

Angela and Jeremy Reynolds

Angela and Jeremy Reynolds
This is my best friend and her husband.

Wyatt's first week!

We had so much fun spending Wyatt's First Week of life with Nani and Pop Pop! We drove down right after we were released from the hospital and Wyatt proved to be a great little traveler! He never made a peep! Cora and Miles were great helpers and loved having their baby brother in between them. Every time we got out of the car, Cora would say,"Mom! Don't forget our baby!" It was really cute. It was a lot of fun for the cousins to get a chance to spend time together too. It was a great week!

Our First Family Pic!

Our First Family Pic!

Playing Time

Playing Time
In Nani and Pop Pop's basement playing!

Balloon Swords!

Balloon Swords!
Safe Play?

Ping Pong anyone?

Ping Pong anyone?
Miles couldn't wait for his turn to play ping pong

Nani and Cora

Nani and Cora
They had matching hats! It was so much fun!

Proud Grandparents!

Proud Grandparents!

All the Kids with Nani and Pop Pop!

All the Kids with Nani and Pop Pop!

Diaper Head!

Diaper Head!
He decided that it would be fun to put his swim diaper on his head! He made the return trip home a funny affair!

Cora wanted to do diaper head too!

Cora wanted to do diaper head too!

The Arrival of Wyatt!

We were so very excited for the arrival of Wyatt! He arrived two weeks early, much to everyone's surprise! On June 22, 2008, at 6:30am, I noticed that it was going to be a very special day! I woke Clinton up and we began the "run around". We had to get everything cleaned and packed and ready for the next week. We arrived at the hospital and everything took off from there! He arrived at 5:03pm and was 7 pounds 5 ounces , 20 inches long. Just perfect! He was our smallest baby, but the size of a regular full term baby! He just seemed so tiny to us! And to think that most people have babies his size! We couldn't be more thrilled with our new little man! He is so precious to us! He is very content and sleeps very well! You can't ask for more than that!

Baby Wyatt is coming!

Baby Wyatt is coming!
I wanted a picture before Wyatt made his grand entrance!

Our Precious Little Man!

Our Precious Little Man!
Just a few minutes after he was born!

Mom and Baby!

Mom and Baby!
I couldn't be more thrilled with him!

Dad and his Son

Dad and his Son
He is so proud of his boy!

Big Sis and Wyatt!

Big Sis and Wyatt!
She sang "Mary had a little lamb" to him! It was so cute!

Wyatt Evan Fisher!

Wyatt Evan Fisher!
Our Precious Baby Boy! Loves to look around!

Nani and Pop Pop!

Nani and Pop Pop!
Happy Family!

Pop Pop and Wyatt!

Pop Pop and Wyatt!
Pop Pop was able to cut the cord! How cool!

Wyatt and Nani!

Wyatt and Nani!
We loved having Nani there for the Birth!

Uncle Jason!

Uncle Jason!
It was great to have Uncle Jason come and visit!

Summer 2008!

We are having a lot of fun this summer right in our own back yard! We got the kids a little pool that they love and a swing that I (Amy) love so that we could enjoy being outside together as a family! God has blessed us with great children, great weather, and so much love for each other. I am always thankful for how well the kids get along. I am looking forward to seeing how Wyatt will fit with our little family.

Cora is growing up so fast and she is "teaching" Miles new things all the time! Miles grows so fast "learning" from his big sister and he loves to copy her. He follows her and repeats what she says and does what she does. It is really sweet to see them playing together and learning from each other.

Umbrella Time

Umbrella Time
Cora loves her little umbrella!

Fun in the Summer Sun!

Fun in the Summer Sun!
They love their little pool!

Pool Time!

Pool Time!
They love Splashing in the Pool!!

Eatting Pluots!

Eatting Pluots!
Snack time in the pool! Can't get any better than that!

We love eachother!

We love eachother!
They are best friends!

Cora and Miles Club House!

Cora and Miles Club House!
They love this little house!

Painting!

Painting!
The kids discovered that if you get chalk wet, it makes "paint"!

Coloring!

Coloring!
Cora loves to color.

My yellow picture!

My yellow picture!
Cora wanted to make sure that I took a picture of her with her picture!

My Favorite Cuties!

My Favorite Cuties!
They are such posers!

My Studly Man!

My Studly Man!
Miles is such a big boy!

Precious Princess!

Precious Princess!
Cora is such a Princess!