It is the mission of The Olive Branch Fund: A Thisbe and Noah Scott Legacy to promote research, awareness, and support for families of all pediatric motor neuron diseases, including Brown-Vialetto-Van Laere.
"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."
Laurian and John Scott created The Olive Branch Fund: A Thisbe and Noah Scott Legacy in the spring of 2008, in order to raise awareness and research dollars for pediatric motor neuron diseases.
Since 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 have been 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 is the genesis of The Olive Branch Fund: A Thisbe and Noah Scott Legacy, which continues to raise money to fund research and awareness for pediatric motor neuron diseases. Their hope is that these efforts will ultimately result in the eradication of all motor neuron diseases 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 is Brown-Vialetto-Van Laere (BVVL)?
How common are MNDs?
Is there any treatment?
How can you help?
How can I contact The Olive Branch Fund?
What are motor neuron diseases (MNDs)?
Motor neuron diseases (MNDs) are a group of progressive neurological disorders. 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 is Brown-Vialetto-Van Laere?
The medical term is progressive bulbar palsy (PBP), which is not a specific disease but more like a constellation of symptoms. These include cranial nerve palsies which cause the loss of swallowing, breathing, speaking, communicating, seeing, laughing, smiling, and eating. When this syndrome also involves hearing loss, it has been named Brown-Vialetto-Van Laere (BVVL). If the other palsies manifest without hearing loss, the syndrome is referred to as Fazio-Londe.
This syndrome falls into a category of diseases known as pediatric motor neuron diseases (MND). Simply stated, motor neurons are tiny nerve cells that send signals to muscles to contract or move. When the motor nerve cells die, they stop sending signals to the muscles, and the muscles atrophy. While different motor neuron diseases can target other muscles, PBP and BVVL specifically target motor neurons within the cranial nerves and the muscles that are responsible for the basic functions of life.
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 MND, the obvious conclusion is that carriers for MNDs at large are even greater.
Is there any treatment?
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.
How can you help?
We need to get to a place where everyone knows the name: "motor neuron disease." Just as there are many types of cancers, there are many types of motor neuron diseases, and though each cancer may have a different origin, they all have a common "front line" of treatment by way of chemotherapy and radiation. Similarly, although each MND may have a different genetic or environmental predisposition, there is likely a common thread of treatment for all of them.
Researchers need funding to identify the genetic and environmental factors responsible for MNDs. If we can solve the mystery of how and why motor neurons die in these diseases, 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 Olive Branch Fund through tax deductible donations as well as by lending a hand to A Mighty Voice: Fundraiser for Children’s Motor Neuron Diseases.
How can I contact The Olive Branch Fund?
You may contact us via email at info@theolivebranchfund.org or info@amightyvoice.org.
