Elementary School Spreads SMA Awareness Across the Country and Around the World

The SMA community is constantly trying to spread awareness for a disease that most have never heard of.  SMA is relatively unknown despite being #1 genetic killer of children under two.  We are always looking for unique and creative ways to help get people informed.  When Mrs. Sheldon, the librarian at Rolling Ridge Elementary School (school Jadon attends) came to us with her Flat Stanley idea, we were overwhelmed with her creativity and enthusiasm as well as the potential “reach” of this project.

We feel Mrs. Sheldon explains it best in this short video:

All 450 students at Rolling Ridge Elementary have read a funny book titled, Flat Stanley, written by Jeff Brown.  The book is about a young boy named Stanley Lambchop.  He is accidentally squished “as flat as a pancake” when a bulletin board falls on him.  Stanley is fine, except for the fact he is very flat.  Stanley discovers some advantages to being flat.  He can slide under doors, go into sidewalk grates, and even fold himself up small enough to fit into an envelope.  When Stanley wanted to visit friends in California, his parents folded him up and placed him in the mail.

All the students made their own Flat Stanley and they have been sent near and far.  Accompanying each Flat Stanley is an SMA warrior or angel.  Over 100 SMA kiddos are represented!

The project is simple, when someone receives a packet we simply ask that they take their Flat Stanley and SMA Warrior/Angel on some adventures. Take them to fun, exciting places, hang out with you at the movies or just have them at Christmas dinner but make sure to take pictures and send the pictures back so we can see where they have been.  We hope to have pictures from all over the world.  Pictures can be emailed to:  flatsrr@olatheschools.org

Here is another blog telling what we want you to know about SMA: SMA Awareness – What We Want You to Know

Many Flat Stanleys and SMA Warriors/Angels have already been out doing some pretty awesome things.  Check out these adventures!

 

SMA Awareness – What We Want You to Know

 

We are often asked what we want people to be aware of in regards to SMA.  This blog could be 20 times longer but it encompasses the basics we would like everyone to know.  It’s not that a person needs to be an expert but if  the need arises having read this information may make a difference.

First – We want people to know the disease exists.  SMA = Spinal Muscular Atrophy.

– Most people have never heard of Spinal Muscular Atrophy, including medical professionals (We certainly had not).  We want everyone to have heard of the disease.  When I say “Spinal Muscular Atrophy” I want people to say “I’ve heard of that” rather than “Gesundheit” because they think I just sneezed.

To get people’s attention we must tell them how severe the disease is
– #1 Genetic killer of kids under 2
– 90% of the kids diagnosed with SMA Type 1 don’t live to see their second birthday

Second – Symptoms and Diagnosis

There are 4 main types of SMA with Type 1 being the most severe.  As a general rule the type is dictated by age of onset and furthest milestone reached. The other 3 types would have similar symptoms but less severe.

These are symptoms for a type1 (Usually diagnosed between 0-6 mnths of age):
– Muscle weakness, limpness in the arms and legs. “floppy baby”
– Most type 1s will never sit on their own and won’t be able to hold their head up
– A weak cry, cough, and swallow
– Their belly will be very active during breathing
– The chest will be narrow and the belly rounded giving the torso a pear shape
– Most will develop a frog-leg position when lying flat on their back
– The tongue often twitches

These are several symptoms but the child may have all or only a few of these. 

Third – You can be tested to see if you are a carrier of the disease. 

-1 in 40 people carry the gene that causes SMA.
– If you are considering having children it is advisable to be tested.  Knowledge is power.
– If anyone in your family is a carrier of SMA (even second or third cousins) your chances of being a carrier increase.  If you have a first cousin that is a carrier, your odds increase to 1 in 8. (I am a carrier so I hope my cousins are paying attention.)

Two carriers having a child present a 25% chance of the child being affected by SMA.

Fourth – HOPE ENDURES

-Newly diagnosed families NEED to know that there are options of care that can extend the child’s life.  Despite the staggering statistics there are care options that can (not always) extend the life of even the most severe cases.  This care relies on the help from many machines and a specialized diet.  These options may not be right for every family but every family needs to know they exist.  Doctors definitely need to know these options exist and offer these options to newly diagnosed families, not just tell them there is nothing that can be done; just take your child home and love them.

Fifth – There is a lot of very promising research.

-There is so much scientifically known about SMA that we truly believe there will be a cure!!  Many pre-clinical research is currently active, five of which are now in clinical trials.
-Gene Therapy is the most promising research right now and it entered human clinical trial earlier this year (2014).  This is very, very, exciting!!!!

–Isis just announced that it will begin a pivotal Phase 3 study evaluating ISIS-SMNRx.

Sixth – Newborn Screening

– All newborns are tested for several diseases but SMA is not one of them.  It should be included and there are many people diligently working to make this possible.  It would be beneficial to know an SMA diagnosis as early as possible.  Once a cure is discovered this becomes even more critical.

This is the very basics of SMA Awareness.

Check out our Facebook page the entire month of August as we provide more insights into the SMA world and please, if you have questions, don’t hesitate to ask: www.facebook.com/JadonsHopeFoundation

Jadon’s Hope Foundation Partners with Jacey’s Journey to Fund Angel Arms

The gift of mobility is often lost on many.  Truth be told, prior to Jadon’s diagnosis it was something we too took for granted.  Our eyes have since been opened.  With a disease like SMA, watching your child be robbed of this gift is heart-wrenching.  Often times we find ourselves yearning and longing for movement.  Any movement.  No matter how minimal or slight.

We would like to introduce you to Melissa and Allan Kloiber, Mom and Dad to adorable Jacey and founders of Jacey’s Journey.  Like so many SMA parents, they wanted Jacey to experience what had been lost.  They wanted to see her thrive and be able to reach for the world around her like so many kiddos her age.  And so began their quest to make it happen and the invention of the Angel Arm.

 

 

 

 

 

 

 

 

Gravity is the worst enemy to a child with extremely low muscle tone.  By reducing gravity, movement can often occur when it would not have otherwise.  The counterweight of the Angel Arm takes the pull of gravity off the child’s limb enabling them to move.  The device is developed to be used in any direction, on any limb.  While we could go into the technical aspects of the device, seeing is truly believing.

 

 

Simply put, the Angel Arm helps SMA Type 1 kiddos move.

 

Grace Grutter, mom to sweet Nella, summed it up beautifully when describing Nella’s initial use of the Angel Arm:

“I was in tears yesterday…With the help of Nella’s new angel arm, I was able to see her reach for a toy for the very first time in her life! It was such an amazing moment that I will forever be grateful for. Nella was so determined to grab it, and was SO proud of herself. She was smiling SO big! What a huge blessing to be able to have this moment.”

We have been inspired by the determination the Kloibers have demonstrated in developing this device and building them for other SMA families.  Melissa and Allan only charge for materials and shipping, not for the extensive time spent lovingly building or packaging the Angel Arm.  Despite only charging for materials, the device may be too expensive for some families.  Because of this, they have set up a wish list for families asking for funding of the arm.

 

Jadon’s Hope Foundation is proud to announce we have issued our first grant to help fund the Angel Arm wish list.  JHF wants to see every child who wants an Angel Arm to have an Angel Arm.  We are committed and prepared to do more if the need arises.    We believe so strongly in what the Kloibers have created that our hope is to do all we can to help get kids moving.

As SMA families it is important that we all continue to support each other through this life storm.  Necessity is the mother of invention and where there is a need SMA families find a way.  If you would like to directly support the efforts of funding Angel Arms you can do so at the following link.  Every dollar donated through this link will go directly to funding Angel Arms for families.

 

 

If you are an SMA Family wanting to learn more about the Angel Arm, please click on the following link.

 

 If you would like to apply for financial assistance for an Angel Arm please use this link.

 

 

 

SMA Awareness- The Ella Hunt Story

Despite the fact that SMA is the #1 genetic killer of kids under two years old most people have never heard of it.  Even doctors in major hospitals are extremely uneducated about the disease.  This is a story that exemplifies why SMA awareness needs to be spread. Ella Hunt came into the world on August 2^nd 2011 and was welcomed by her parents Erica and Dan as well as her two very active brothers, Noah and Carter.  When she was just 4 months old she presented as “significantly delayed” and the tests began.  The Hunts were given their beautiful daughters devastating diagnosis of SMA Type1, the most severe, on December 20^, 2011. Erica writing about that day:

“Picture this moment, I (mom) sit in a dark hospital room at 4 am waiting for the clock to tick as this is the day we are running tests to determine what is wrong with our precious baby girl. I had heard the letters SMA mentioned by both the Developmental Pediatrician and the Neurologist. I had also been warned NOT to google anything by the doc’s. By 4 am I couldn’t stand it. I googled it and there it was……

SMA, a fatal illness with life expectancy of less than two years. A disease that limits kiddos from having (any) muscle movement, a swallow or respiratory function. I was heartbroken and with momma’s intuition I knew this was it.

Less than 6 hours later, my worst fears were confirmed. Only it was worse news than we thought. Upon diagnosis, we were told we had weeks to months left with her. ‘Go home and hold her.

‘She is going to die in your arms or in her sleep’ the doctors told us.

I vowed then to never put her down until that time came. We cried out in agony realizing this gift of life would be taken home way too soon.The hospital sent us home (a 3 hour drive) and had us immediately placed on Hospice. All her physical therapy services were cancelled and little to our knowledge at the time, Ella was also discharged as a patient (not just discharged from the hospital).

THERE WAS NO HOPE.

We were encouraged to sign a DNR (Do Not Resuscitate) form which neither Dan nor I could get ourselves to do. Looking back, it was a whisper from God telling us there is more to this story and it is not over yet.”

Thankfully Erica and Dan heard that whisper as their prayer for more time was about to be answered.  A few states away a stranger, another SMA mom, was about to learn about Ella’s story.  Sarah, proud mother of two boys and a daughter Stella (SMA Type1), received a message from a friend in California about a blog she had come across.  Because Sarah actively spreads awareness her friend knew she would want to read about this little girl in Illinois.  A woman was making purses from Ella’s dresses and wrote a blog about it.  Sarah contacted the woman and asked if it would be ok for her to speak to Ella’s mother.

Sarah’s fateful phone call to the Hunts came one day before their scheduled meeting with the funeral home, which was the 8^th of January.  Ella’s celebration of life was scheduled for the 15^th of January.  They wanted family and friends to celebrate her life while she was still with them although they had accepted that the funeral would follow shortly after. Erica refers to this incredible SMA ambassador, Sara Turnbull, as their personal angel.  She introduced them to her daughter Stella. Her (then) 5 year old SMA Type1 daughter.  5 Years old? She explained that there are options in care that can give a family more time.  Time is what the Hunt family was after so they listened intently to the information the doctors had failed to mention.  Sarah described a life that would not be easy, a life with lots of machines, but a happy life.  A life with potentially years, instead of only months with their daughter.  While there are no guarantees with anything, they wanted to try.  Sarah suggested they contact Dr. Schroth (SMA specialist) in Madison WI.  Sweet Stella proved to the Hunt family…

THERE IS HOPE

Erica writes about the days after that phone call:

“We called Madison directly the next day. Instead of planning a funeral we began planning her life. Instead of planning on her dying, we began to plan on her living. We made the trip to Wisconsin two weeks later and left shocked. Dr Schroth did not give us choices like IF you choose this, it was more like ‘so, you are here, and if you want more time, this is what we need to do.’  We left armed with resources and most importantly hope and a vision for her life.

On the second day of this SMA awareness month (August) Ella turned 2 years old.  A huge milestone for all SMA families.  Not only is she surviving but she is striving.  She has some speech, a huge smile, and an active lifestyle with lots and lots of “Dancing in the Rain”. So why do we need to spread SMA awareness?

Because families wanting more time should know ALL of the options.

Look at this smile.

CLICK HERE FOR VIDEO

SMA Awareness – Care Decisions (Type1)

Picture courtesy of SMA Awareness Ribbon

An SMA Type 1 diagnosis is devastating to say the least.  The doctor comes in and tells the parents their beautiful baby, the light of their life, the life they had so much hope for is going to be cut short.  It is hard enough to learn there is something, anything wrong with their precious angel but to find out something like an SMA prognosis is simply earth shattering.

THEN…These devastated parents are asked to make decisions no parent should ever have to make.  They are asked how much care they want to give their child.   They may even be told that there is no hope.  They may not be given any options at all.  They may be told to go home and simply enjoy the little time they have.  Others may be told the only way they will leave the hospital is to have a tracheotomy and not even be offered other options.

That is why awareness so very important.  

There are basically 3 options of care for an SMA Type 1 child:

*Palliative Care – Also called comfort care.  The child is kept comfortable with the use of oxygen and pain relieving medications until the time they receive their wings.

*NIV (Non InVasive) Respiratory Care – A Bi-pap machine is used to assist the child in breathing.  Several other machines are also used in conjunction with NIV:  Cough Assist, pulse-ox, feeding pump among others.

*Tracheotomy – Instead of a Bi-pap mask, a ventilator helps the child breathe through a tube attached to a hole in their throat tube which provides a direct path to the airway.  Most of the same machines are used as in NIV.

There are NO guarantees with any of the care levels.  Each and every child is different.  Parents need to be told that there are options.  They need to be filled with hope, not fear.  The fear will be there, it is inevitable.  We have lived with that fear every single day for the last 4 years. But because of all those great moments in between, the giggles and the milestones, we think more of life than we do of death.

Judging a situation is always easier the further away you stand.  Some would say that a parent choosing to do nothing doesn’t love their child or a parent choosing a trach is holding on too long.  Neither could be further from the truth.  The devastation that SMA forces on a family is harsh, no matter the length of life and no matter the decisions that are made.

Each family must choose what is right for THEIR family and THEIR child.  There were so many times I wished someone would have told us what was right for us.  We simply prayed for those direction and looked into Jadon’s eyes for answers.  We firmly believe the path we have walked was intended for US and JADON.

We would never dream of judging anyone’s decision, we don’t have the right and we certainly don’t have the authority.  What we want is for every family to know ALL of the choices for their newly diagnosed child.  Some doctors are uneducated in the disease and give no options and no hope.  Others may even know the options but decide on their own what is “right” for the family.  In their mind there is no quality of life for a child who cannot move a muscle.  Even medical professionals don’t have that authority.

So this is why we spread awareness, because knowledge is power.  If a family has all the information and decides that palliative care is right for them, we want to stand by them and help them “Dance in the Rain” through their shorter storm.

There are too many stories of families who were never given the options but wish they had been.  Sometimes, just sometimes, because SMA awareness is being spread, a family simply looking for more time with their beautiful daughter will receive a call.  A call from a complete stranger, an SMA Mom, who is the answer to their prayers…be sure to check out our blog tomorrow (Saturday) for this exact story.

What we want you to know about SMA

A friend of mine recently said to me “I know you want to build awareness for SMA, but what is it that you want people to know?”  I thought this was a VERY good question with a not so short answer, but here it is:

First – We want people to know the disease exists.  SMA = Spinal Muscular Atrophy.

– Most people have never heard of Spinal Muscular Atrophy, including medical professionals (We certainly had not).  We want everyone to have heard of the disease.  When I say “Spinal Muscular Atrophy” I want people to say “I’ve heard of that” rather than “Gesundheit” because they think I just sneezed.

To get people’s attention we must tell them how severe the disease is
– #1 Genetic killer of kids under 2
– 90% of the kids diagnosed with SMA Type 1 don’t live to see their second birthday

Second – HOPE ENDURES –

-Newly diagnosed families NEED to know that there are options of care that can extend the child’s life.  Despite the staggering statistics there are care options that can (not always) extend the life of even the most severe cases.  This care relies on the help from many machines and a specialized diet.  These options may not be right for every family but every family needs to know they exist.  Doctors definitely need to know these options exist. (Be sure to check out our Facebook page this month to see all the amazing kids “Dancing in the Rain”.  There are so many beating the statistics. )

Third – You can be tested to see if you are a carrier of the disease.

-1 in 40 people carry the gene that causes SMA.
– If you are considering having children it is advisable to be tested.  Knowledge is power.
– If anyone in your family is a carrier of SMA (even second or third cousins) your chances of being a carrier increase.  If you have a first cousin that is a carrier, your odds increase to 1 in 8. (I am a carrier so I hope my cousins are paying attention.)

Fourth – There is a lot of very promising research.

-There is so much that is scientifically known about SMA that we truly believe there will be a cure!!
-Gene Therapy is the most promising research right now and should be in human clinical trial in the first half of 2013.  This is very, very, exciting!!!!

 Fifth – Newborn Screening

– Newborns are tested for several diseases but SMA is not one of them.  It should be included.  It would be beneficial to know an SMA diagnosis as early as possible.  Once a cure is discovered this becomes even more critical.

So there it is (the “short” version).  Now go shout it from the mountain tops!