BDSRA - Dallas » About Batten Disease

2010 Hope For Batten Cure

Admin — Mon, 01 Mar 2010 12:00:19 +0000

2010 Hope for Batten Cure
5K Run / 2 Mile Walk
1K Fun Run

In Honor of McKenna and Eric Lowden

Saturday, May 15th, 2010
Bachman Lake
3500 West NW Hwy.
Dallas, Texas 75220

SPONSORED BY
BATTEN DISEASE SUPPORT AND RESEARCH ASSOCIATION (BDSRA)

Message from Scott Lowden

Admin — Mon, 01 Mar 2010 12:00:14 +0000

Batten Disease
Batten Disease is a relatively rare genetic neurodegenerative disease that tends to strike boys and girls of Northern European ancestry. It is always fatal. Juvenile Batten shows few, if any, symptoms in the first five years; so the child begins life normally. Visual impairment, an early manifestation, begins at age six or seven. A trip to the eye doctor, not uncommon for children that age, often initiates the process that will result in an unexpected and truly horrifying diagnosis.
Clinically, juvenile batten disease is characterized by the mutation of a gene called CLN3. The mutation reduces or prevents the production of enzymes that are necessary for normal neurotransmission. In real life, the defect results in a progressive chemical chain reaction that produces blindness, epileptic seizures and the loss of cognitive and motor skills. These symptoms occur episodically at first, then with increasing frequency. Eventually the child will lose the ability to walk, to communicate and, finally, to carry on any life functions. The process can take 20 years.
Our Story
When Chris and Danielle brought their six year old son Eric to the eye doctor in 2003, they could never have expected the nightmare that would follow. Several diagnostic failures eventually led to a test and confirmation in September, 2004, that Eric had Batten Disease. There was no known incident in Chris’s or Danielle’s family histories that would forewarn them that they each carried a rare recessive gene. And in Nature’s lottery, even though they carried the gene, Eric still had only a one-in-four chance of inheriting the disease.
Eric’s sister McKenna was born before Eric’s diagnosis. Despite the three-to-one odds in her favor, she too was diagnosed with Batten in October 2007.
Eric and McKenna are our grandchildren. Chris is our son. Both Chris and Danielle are college graduates. Chris has a PhD in medicinal chemistry and works for a pharmaceutical research company. They have two younger daughters, Sophie (4) and Tori (8 mos.) who do not have Batten Disease. They will provide the best care possible for their children, but their family can never be normal.
Hope for Batten Cure
Because it is rare in America, Batten Disease does not attract large government grants or charitable funding. Our focus is on a cure, even if it may come too late for our own family.
Nancy is active in the running community and for the third year has organized a 5K Run, 2 Mile Walk and 1K Fun Run scheduled for May 15, 2010. The race will be held at Bachman Lake, 3500 West NW Hwy., Dallas, 75220. All net proceeds of the event will be contributed to BDSRA and designated for research.
If you do not attend the event but wish to contribute, you may write a check to BDRSA and send it to Nancy Lowden, 12116 Cross Creek Dr., Dallas, TX 75243. BDRSA is a non-profit organization qualified under 501 (c) (3) of the Tax Code. It supports over 1,200 families worldwide who have children with Batten Disease.
Don’t hesitate to call Nancy at 972-407-9705 with any questions. Many thanks for your consideration.
Scott Lowden

A Personal Note To You…

Admin — Mon, 01 Mar 2010 12:00:08 +0000

A personal note to you…

First, I thank you for the opportunity to educate you about Batten Disease–a very rare inherited, degenerative, neurological disease which affects persons of any age, but primarily infants, toddlers and school age children. It begins unexpectedly and leads to a progressive loss of brain function eventually leading to an early death. At this time, there is no treatment or cure available and it is always fatal.

In the United States, one child is diagnosed with the disease every week! Over time, affected children suffer mental impairment, worsening seizures, and progressive loss of sight and motor skills. Eventually, children with Batten Disease become blind, bedridden, and unable to communicate. Presently, there is no treatment for Batten Disease and the disease is always fatal. Because of its rarity, Batten Disease does not attract large government grants or charitable funding. For more information go to the BDSRA website: www.bdsra.org

Our only grandson, Eric (14) was diagnosed with juvenile Batten Disease in the fall of 2004. In the fall of 2007 our granddaughter, McKenna (8), was also diagnosed with juvenile Batten Disease. Eric is now almost completely blind, but plays baseball and hockey on special leagues, and enjoys swimming and building leggos. McKenna also participates in the special hockey league. She enjoys watching “Underdog” cartoons and playing Barbie dolls with her sister, Sophie (4). Both Eric and McKenna are entertained by Zorro (the family dog) and Kamarhi (the family cat). The newest addition to the family is Tori (8 mos.) Sophie and Tori have been genetically tested and they do not have Batten Disease. Let us hope their parents, Chris and Danielle, will be able to handle, mentally and physically, the many difficult challenges they will face in the coming years.

We need your help. Please consider participating in my third annual Hope for Batten Cure 5K Run, 2 Mile Walk, 1K Fun on Saturday, May 15, 2010 at Bachman Lake in Dallas. If you cannot attend the event but wish to contribute, you may write a check to BDRSA and send it to Nancy Lowden, 12116 Cross Creek Dr., Dallas, TX 75243. All monies raised will be designated for research into finding a cure/treatment for this horrible disease. BDRSA is a non-profit organization qualified under 501 (c) (3) of the Tax Code.

It is my hope and prayer that you will participate or donate to my annual fundraising event.

Yours truly,

Nancy and Scott Lowden
Eric and McKenna’s Grandparents

SPONSORED BY
BATTEN DISEASE SUPPORT AND RESEARCH ASSOCIATION (BDSRA)

2009 Race Photos

Admin — Fri, 15 May 2009 07:38:54 +0000

2009 Race - Group Photo of Team Competion Runners

Grandad, Nana, Eric and Chris Lowden

2009 Race - Lady Winners from Race Nation Team Competition

2008 BDSRA Run Photos

Admin — Mon, 16 Mar 2009 02:13:00 +0000

Saturday May 10th, 2008

Eric Lowden (12) with his Grandad, Scott Lowden finishing the 1K Fun Run.

Eric, Scott and Nancy Lowden after the 1K Fun Run.

Chris Phelan interviewing Eric.

SPONSORED BY
BATTEN DISEASE SUPPORT AND RESEARCH ASSOCIATION (BDSRA)

2009 Run For Hope Sponsors

Admin — Sat, 14 Mar 2009 17:49:56 +0000

THANK YOU to our 2009 Sponsors

Silver Sponsorship ($1,200)

Scott and Carol Lowden

SAP (matching program)

Bronze Sponsorship ($500)

Stevens Entertainment, Inc.

Ron and Kathy Saunders

A-1 Servomotor Repair

Contact: Nancy Lowden at 972-497-9705 or at nslowden@swbell.net to learn more about sponsorship opportunities for this event.

With Your Help, There Is Hope!

Kari Elisabeth Anderson

Admin — Sat, 14 Mar 2009 17:33:39 +0000

2009 Hope for Batten Cure
5K Run / 2 Mile Walk
1K Fun Run

In Memory of Kari Elisabeth Anderson
May 25, 1988 – August 6, 2008

Saturday May 9th, 2009
Bachman Lake
3500 West NW Hwy.
Dallas, Texas 75220
(west of Lemmon Avenue)

SPONSORED BY
BATTEN DISEASE SUPPORT AND RESEARCH ASSOCIATION (BDSRA)

About Batten Disease

Admin — Sun, 23 Mar 2008 06:46:25 +0000

First described in 1826 – more than 170 years ago – Batten Disease (Neuronal Ceroid Lipofuscinoses), thought to be one of the most common neurodegenerative diseases, remains an unsolved mystery today…a puzzling disease that assures its victims of only one consistent manifestation…early death.

An inherited, degenerative neurological disease, – Batten may affect persons of any age, but primarily affects infants, toddlers and school age children, beginning unexpectedly and leading to a progressive loss of brain function that later destroys bodily functions, eventually leaving the victim totally helpless.

Whether in the case of infantile (Santavnori), late infantile (Jansky, Bielschowsky), juvenile (Batten, Spielmeyer, Sjogren), or adult type (Kuf, Parry), the early symptoms of Batten Disease are confusing ones. It strikes without warning, affecting vision and causing seizures or convulsions.

Possibly most frustrating of all is the fact that Batten Disease is rarely diagnosed immediately, often being mistaken for epilepsy or mental retardation, even schizophrenia. And once diagnosed, there is no satisfactory treatment and no cure. The clinical course of the disease includes a marked decline in cognitive function; personality and behavior changes; loss of communication and motor skills; poor circulation; decrease in muscle mass; hyperventilation; hallucinations, and, finally, deterioration to a vegetative state that ends in death.