Wesley's MPS 'WarriorHeart' Support
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Wesley's Warrior Heart: Help Keep It Safe & At Rest!
At four years old, our sweet Wesley has been diagnosed with Hunter syndrome or MPS 2.
Hunter Syndrome/MPS Type 2 is a very rare inherited disease (2000 cases worldwide) in which long chains of sugar molecules (MucoPolySaccharides) build up in the body's cells due to a missing or deficient enzyme called Iduronate 2-sulfatase. It's so rare, it is considered an 'orphan disease' as it doesn't draw much support from medical researchers, doctors & pharmaceutical companies.
MPS2 affects the ENTIRE body, causing progressive damage to the heart, and other organs, bones, joints, the respiratory system, central nervous system and brain development. Hearing loss is common.
There is no cure at this time, but there is a treatment to help give a better quality of life. An Enzyme Replacement Therapy (ERT) drug called 'Elaprase' (manufactured in England) is infused weekly, through a surgically implanted port into the chest. Once a week for life...though the damage cannot be reversed, it can help slow down the rate Wesley's body collects these molecules that are not being 'recycled' correctly, in his body. Elaprase does NOT cross the blood/brain barrier so that is another issue altogether. Clinical trials are underway for this type of treatment.
Currently, Wesley is undergoing many 'baseline' appointments with the cardiologist, ENT (EarNoseThroat), Eye Dr., etc., to determine his present organ function levels so that changes or decline in the future, especially in his heart & respiratory functions, can be monitored.
In June, Wesley will begin making the weekly 4 hour drive to Children's Hospital, sit (or hopefully sleep for some of it!) for 5 hours during the infusion, then travel 4 hours home. For the first few months he will have to be monitored for allergic reactions to the Elaprase and then, we hope, a local treament facility (oncology clinics can best administer this type of ongoing treatment) will be willing and be approved by the pharmaceutical company. Our hope is that Wesley will be able to receive treatments a little closer to home.
Due to the develpmental delays (that are now known to be part of the MPS2 diagnosis), Wesley will ALSO continue in Physical, Speech and Occupational therapies twice a week. After two ear tube surgeries, he's now been fitted for hearing aids (they should arrive in another month) to help with the hearing loss that began before the age of two. Physical therapy is helping keep his joints/body more flexible. This is an important year in Wesley's life as he needs to learn as much as he can & stay as strong as he can based on the prognosis timeline, with the life expectancy for Hunter boys being about a decade.
We are so thankful for the gift of God that Wesley is and for the many precious blessings he has brought to us & the lives of others. He is brave, loving and courageous and loves the outdoors, tractors, trains & his new baby sister.
He expresses compassion and mercy for others in ways you wouldn't think a boy with a terminal illness could even comprehend:
In this video, Wesley is comforting a sad friend on a Sunday afternoon at church:
We are also deeply grateful that these treatments & therapies are currently covered by insurance. But there is work time lost for appointments, therapies, fuel cost for travel, car maintenance & repair, etc. that is a huge strain on our family's limited budget.
Currently, we are looking ahead for creative ways we can give Wesley the best life possible as well as to raise awareness about MPS.
One way, will be to stay overnight near our Children's Hosptial so those 13 hour days for treatment & travel can be broken up during the beginning months they monitor for allergic reaction to the Elaprase. Ten weeks, ten nights.
Secondly, Wesley's safety during these precious years we have him is of great concern. Currently our home in the country has a great yard, but NO FENCE (and runs directly along a well-traveled county road).
Due to Wesley's condition & especially the hearing loss, he has difficulty processing and understanding these important boundaries (another factor of the MPS) and tends to explore & wander beyond the safety of the property, so he is often restricted from enjoying his time outside. The most immediate need will be to get the Deaf Child signage ordered & posted ($180.00 for 4 signs & 2 posts: back to back signage in two areas).
The fence estimate is in, we'll utilize family, friends & volunteer professionals for the equipment necessary and the installation, but the fence materials for the size of our yard (& to match the neighbors shared fence) were much more costly than we'd anticipated ($1300).
Wesley has the Heart of a Warrior:
Brave & Courageous, yet so Loving & Compassionate!
Please help Wesley safely get the daily exercise & sunshine he so desperately needs in his own protected, fenced yard, just right for him.
And, as you are lead, please help Wesley & our family endure those long treatment days in the beginning with a night of rest.
No amount is too small. Every little bit counts & is greatly appreciated.
Thank you so much!
Check out our blog: Wesley's Warriors
Read more about Hunter syndrome!
At four years old, our sweet Wesley has been diagnosed with Hunter syndrome or MPS 2.
Hunter Syndrome/MPS Type 2 is a very rare inherited disease (2000 cases worldwide) in which long chains of sugar molecules (MucoPolySaccharides) build up in the body's cells due to a missing or deficient enzyme called Iduronate 2-sulfatase. It's so rare, it is considered an 'orphan disease' as it doesn't draw much support from medical researchers, doctors & pharmaceutical companies.
MPS2 affects the ENTIRE body, causing progressive damage to the heart, and other organs, bones, joints, the respiratory system, central nervous system and brain development. Hearing loss is common.
There is no cure at this time, but there is a treatment to help give a better quality of life. An Enzyme Replacement Therapy (ERT) drug called 'Elaprase' (manufactured in England) is infused weekly, through a surgically implanted port into the chest. Once a week for life...though the damage cannot be reversed, it can help slow down the rate Wesley's body collects these molecules that are not being 'recycled' correctly, in his body. Elaprase does NOT cross the blood/brain barrier so that is another issue altogether. Clinical trials are underway for this type of treatment.
Currently, Wesley is undergoing many 'baseline' appointments with the cardiologist, ENT (EarNoseThroat), Eye Dr., etc., to determine his present organ function levels so that changes or decline in the future, especially in his heart & respiratory functions, can be monitored.
In June, Wesley will begin making the weekly 4 hour drive to Children's Hospital, sit (or hopefully sleep for some of it!) for 5 hours during the infusion, then travel 4 hours home. For the first few months he will have to be monitored for allergic reactions to the Elaprase and then, we hope, a local treament facility (oncology clinics can best administer this type of ongoing treatment) will be willing and be approved by the pharmaceutical company. Our hope is that Wesley will be able to receive treatments a little closer to home.
Due to the develpmental delays (that are now known to be part of the MPS2 diagnosis), Wesley will ALSO continue in Physical, Speech and Occupational therapies twice a week. After two ear tube surgeries, he's now been fitted for hearing aids (they should arrive in another month) to help with the hearing loss that began before the age of two. Physical therapy is helping keep his joints/body more flexible. This is an important year in Wesley's life as he needs to learn as much as he can & stay as strong as he can based on the prognosis timeline, with the life expectancy for Hunter boys being about a decade.
We are so thankful for the gift of God that Wesley is and for the many precious blessings he has brought to us & the lives of others. He is brave, loving and courageous and loves the outdoors, tractors, trains & his new baby sister.
He expresses compassion and mercy for others in ways you wouldn't think a boy with a terminal illness could even comprehend:
In this video, Wesley is comforting a sad friend on a Sunday afternoon at church:
We are also deeply grateful that these treatments & therapies are currently covered by insurance. But there is work time lost for appointments, therapies, fuel cost for travel, car maintenance & repair, etc. that is a huge strain on our family's limited budget.
Currently, we are looking ahead for creative ways we can give Wesley the best life possible as well as to raise awareness about MPS.
One way, will be to stay overnight near our Children's Hosptial so those 13 hour days for treatment & travel can be broken up during the beginning months they monitor for allergic reaction to the Elaprase. Ten weeks, ten nights.
Secondly, Wesley's safety during these precious years we have him is of great concern. Currently our home in the country has a great yard, but NO FENCE (and runs directly along a well-traveled county road).
Due to Wesley's condition & especially the hearing loss, he has difficulty processing and understanding these important boundaries (another factor of the MPS) and tends to explore & wander beyond the safety of the property, so he is often restricted from enjoying his time outside. The most immediate need will be to get the Deaf Child signage ordered & posted ($180.00 for 4 signs & 2 posts: back to back signage in two areas).
The fence estimate is in, we'll utilize family, friends & volunteer professionals for the equipment necessary and the installation, but the fence materials for the size of our yard (& to match the neighbors shared fence) were much more costly than we'd anticipated ($1300).
Wesley has the Heart of a Warrior:
Brave & Courageous, yet so Loving & Compassionate!
Please help Wesley safely get the daily exercise & sunshine he so desperately needs in his own protected, fenced yard, just right for him.
And, as you are lead, please help Wesley & our family endure those long treatment days in the beginning with a night of rest.
No amount is too small. Every little bit counts & is greatly appreciated.
Thank you so much!
Check out our blog: Wesley's Warriors
Read more about Hunter syndrome!
Give $100 to help get this fundraiser to its goal
Organizer
Rena Leffert
Organizer
Huntsville, AR