It is the mission of the Thisbe and Noah Scott Foundation to promote research, awareness, and support for families of pediatric neuromuscular diseases in general and motor neuron diseases like Brown-Vialetto-Van Laere in specific.
"There are more than 25,000 Americans with spinal muscular atrophy (SMA), which is the most common pediatric MND."
"The world of pediatric motor neuron diseases [is] a world where not one single disease has a viable treatment."
The Olive Branch Fund: A Thisbe and Noah Scott Legacy has become the Thisbe and Noah Scott Foundation.
Same founders. Same purpose. Different name.
Laurian and John Scott created The Olive Branch Fund: A Thisbe and Noah Scott Legacy (now the Thisbe and Noah Scott Foundation) in the spring of 2008, in order to raise awareness and research dollars for pediatric motor neuron diseases.
With their daughter's "diagnosis" in the spring of 2006 with a pediatric motor neuron disease (MND), specifically a rare type called Brown- Vialetto-Van Laere (BVVL), the Scotts began searching the world for doctors and researchers who may know something about the etiology and treatment of BVVL. Finally, in February 2008, five months after their son was also diagnosed with BVVL, they located two physicians in the United Kingdom who had been looking for the BVVL gene. With that knowledge, the Scotts partnered with the Genetic Alliance in the U.S. in order to aid the researchers in the UK with their search - most importantly, to hasten their progress.
Though the discovery of a BVVL gene seemed to be on the horizon, and physicians from the Child Neurology Foundation and the Columbia Motor Neuron Center were eager to work with the Scott family, not enough could be done in time to save their children, Thisbe and Noah.
Such was the genesis of The Olive Branch Fund: A Thisbe and Noah Scott Legacy, which sought to raise money and finance research for children’s motor neuron diseases as a fund through a community foundation in Nashville, Tennessee. Then, in the fall of 2011, the Scotts made the decision to come out from under the umbrella of a community foundation and instead to stand on its own, transitioning The Olive Branch Fund: A Thisbe and Noah Scott Legacy into the Thisbe and Noah Scott Foundation, as a nationally-recognized public 501(c)(3) charity. It would mean even more work for the Scotts and their volunteers, but it would remove the limitations associated with a community foundation while increasing their ability to reach farther in scope for future fundraising goals, as well as broadening their mission to include not just motor neuron diseases but all children’s neuromuscular diseases. Their hope is that these efforts will ultimately result in the eradication of all motor neuron diseases and its broader neuromuscular counterparts, so that no family will ever have to suffer as their family has and still does every single day.
FREQUENTLY ASKED QUESTIONS:
What are motor neuron diseases (MNDs)?
What are neuromuscular diseases (NMDs)?
How common are MNDs?
How common are neuromuscular diseases?
Is there any treatment for MNDs?
Is there any treatment for neuromuscular diseases?
How can I help?
How can I contact the Thisbe and Noah Scott Foundation?
What are motor neuron diseases (MNDs)?
Motor neuron diseases (MNDs) are a type of neuromuscular disease that is progressive in nature. They destroy the cells (motor neurons) that control essential muscle activity including speaking, walking, breathing, and swallowing. MNDs may be inherited or acquired, and occur in all age groups. In adults, symptoms often appear after age 40. In children, particularly in inherited forms of the disease, symptoms can be present at birth, appear before the child learns to walk, or appear in the late teenage years. The causes for these MNDs are not well understood.
More common and well-known MNDs include ALS/Lou Gehrig's disease, progressive bulbar palsy (PBP), primary lateral sclerosis (PLS), progressive muscular atrophy (PMA), and spinal muscular atrophy (SMA); however, there are a host of rare versions of motor neuron diseases, like Brown-Vialetto-Van Laere (BVVL). These affect both children and adults, who suffer through these diseases with normal cognition or thinking, as they become trapped in a body that is wasting away.
What are neuromuscular diseases (NMDs)?
Neuromuscular diseases comprise different categories of diseases that affect the nerve cells (neurons), which control the body's voluntary as well as involuntary muscles. When the neurons' ability to tell the muscles to "move" is affected, then movement is decreased either in part or full. These can occur at the muscles, at the nerve junction, on the myelin sheath (or nerve coating), or within the nerve cell itself. The latter falls into the MND realm.
Examples include groups like motor neuron diseases such as ALS, SMA and Brown-Vialetto-Van Laere syndrome. Examples also include groups like muscular dystrophys such as Duchenne’s, Myotonic and Becker’s. Still others include names like Guillan Barre, myasthenia gravis, and Charcot-Marie-Tooth.
How common are MNDs?
According to the ALS Association, there are currently more than 30,000 Americans affected with amyotrophic lateral sclerosis (ALS) today, most of them adults. Every year, 5,600 more are diagnosed with the disease. In the world of pediatrics, there are more than 25,000 Americans with spinal muscular atrophy (SMA), which is the most common pediatric MND; and although there are different types of SMA, together they make up the leading genetic cause of death of infants and toddlers (www.smafoundation.org).
When you simply look at the statistics of SMA alone, it is staggering. According to the SMA Foundation, approximately 1 in every 35-40 people carries a gene that causes SMA, which translates into approximately 7 million people. Thus, the child of 2 people with the same SMA gene has a 1 in 4 chance of developing the disease. In other words, you and your spouse could each be carriers, and yet you could have four healthy children, all of whom had a 25% chance of being affected by the disease. If each of your children is healthy, then they hit the 75% jackpot; if this is the case, you will never know that you carry this gene or that you have passed the gene on to your children - that is, unless a grandchild or great-grandchild develops the disease. That, in turn, is how approximately 1 in 6,000 infants are born annually worldwide with SMA. There are no compiled statistics on MNDs in general, but if SMA is only one form of MN
How common are neuromuscular diseases?
Neuromuscular diseases affect human beings at every stage of life, from infancy through old age, and they do not discriminate according to ethnicity or geography. They affect millions worldwide. The incidence in general has been noted to be about 1 in every 1,000 births.
Is there any treatment for MNDs?
There is no cure or standard treatment for MNDs. Most forms are fatal, and all forms are severely disabling due to paralysis of muscles.
Sadly, many Americans have never heard of these diseases. Because of the intense national focus and fundraising efforts for pediatric cancer research in the past 20 years, the survival rate for a large number of pediatric cancers has increased dramatically, from as little as 4% in 1962 to 94% today (www.stjude.org). With cystic fibrosis, where in 1955 most children didn't make it to elementary school, today the median survival age is 37 years (www.cff.org). The same can and must happen in the world of pediatric motor neuron diseases, a world where not one single disease has a viable treatment, even though we've known about them since the late 1800s.
Is there any treatment for neuromuscular diseases?
Many neuromuscular diseases are genetic. Most of them have no cure. At this point in time some have, at best, treatment or therapy to try to increase movement, improve symptoms and lengthen life expectancy.
How can I help?
We need to get to a place where everyone knows the names: "motor neuron disease" (MND) and “neuromuscular disease.” Many of these diseases are genetic, and most have no cure. First, researchers need funding to identify the genetic and environmental factors responsible for these diseases. Next, investigation into potential therapeutics begins. For instance, if we can solve the mystery of how and why motor neurons die in MNDs, we can better understand how to reverse this process; regenerate the neurons themselves; and even create new neurons from other types of cells to forge new pathways to the atrophied muscles. The possibilities for treatments and a cure are endless and very much within our grasp.
You can aid the mission of the Thisbe and Noah Scott Foundation through tax deductible donations as well as by lending a hand to A Mighty Voice: Fundraiser for Children’s Neuromuscular Diseases.
How can I contact the Thisbe and Noah Scott Foundation?
You may contact us via email at info@thisbeandnoah.org.