One of the most difficult stages of the disease occurs when the child loses his/her speech. When that’s gone, it becomes a guessing game as to the child’s needs, pain, etc. This is followed by the loss of the abilities to eat and drink. Children will soon be fed through a tube inserted through the abdominal wall.

The disease causes death of brain cells. Death for the child happens when the brain can no longer sustain life.

Batten Disease was first identified 180 years ago. However, it has only been in the past ten years that progress has been made. Today there is a treatment trial underway, and we are hoping for two more trials to start next year.

BDSRA
166 Humphries Dr.
Reynoldsburg, OH 43068
Phone: 1-800-448-4570
Web site: www.bdsra.org

“A Light in a World of Darkness”

I wish to thank Joni Metcalf, President of the Minnesota Chapter of BDSRAfor her invaluable assistance.

We also thank Carol Lowden for designing the picture used on this website and Hope Lowden for coloring the picture.

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 (5) and Tori (1) 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 14, 2011. 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

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 (15) was diagnosed with juvenile Batten Disease in the fall of 2004. In the fall of 2007 our granddaughter, McKenna (9), 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 (5). Both Eric and McKenna are entertained by Zorro (the family dog) and Kamarhi (the family cat). The newest addition to the family is Tori (1).  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)

If you’re considering changing energy providers here’s a way you can help BDSRA and the environment.

Just click on links below, be sure to enter the sales code: BDSRA

Sign-up page:

https://www.whitefence.com/greenmountain/?mrc=GME&ReferenceCode=COFDAL&SalesCode=BDSRA

About Green Mountain Energy:

http://www.greenmountainenergy.com/texas/home.shtml?ReferenceCode=COFDAL&SalesCode=BDSRA

In Honor of McKenna and Eric Lowden

Vase and Flowers by Sophie Lowden.

Handprints added by Kathy Holman Norman.

Donovan Lusk

Saturday, May 12th, 2012
Reverchon Park
3505 Maple Avenue

Dallas, Texas 75219

SPONSORED BY
BATTEN DISEASE SUPPORT AND RESEARCH ASSOCIATION (BDSRA)

5K Run: Pre-registration: $25.00 ($30.00 on Race Day)

2 Mile Walk: Pre-registration: $20.00 ($25.00 on Race Day)

1K Fun Run: $10.00

Online Registraton

(Note: the link below will take you to the Get-Me-Registered website.)

https://secure.getmeregistered.com/HopeForBattenCure

Registration and Packet Pickup Sites

Wednesday & Thursday, May 9 & 10, 10:00 a.m. to 7:00 p.m. No Friday packet pickup.

Run-On

5400 E. Mockingbird Lane, #114

Dallas, Texas 75206

Google Map: Here

Register by Mail

You can register by mail, using this: Registration Form

Mail the completed form, and payment (before May 5, 2012) to:

Nancy Lowden,

2116 Cross Creek Drive,

Dallas, Tx 75243-4602

The 5K Race will start in the park and then connect to the Katy Trail and go north to the turn-around near Knox/Henderson and return to the finish line in the park.

For more information on this event, contact:

Nancy Lowden at 972-497-9705 or at nslowden@swbell.net

With Your Help, There Is Hope!

Race Day Registration – 8:00 a.m.
5 K Run – 9:00 a.m.
2 Mile Walk – 9:05 a.m.
1K Fun Run – 10:00 a.m.

1^st Overall Male and Female
1^st Overall Masters, Male and Female
1^st, 2^nd, and 3^rd in age Category
Customized trophies by www.khsculpture.com

Special Thanks to:

Braumiller Schulz & Co. LLP

for printing of race flyers

RunOn

for their assistance with race planning and

Registration and Packet Pickup

Bill Borowski of Baylor Sports Care

for providing two EMT’s

David Hartwig of DH Sports

for donating his race management services

Chris Stratton for directing the Team Competion

which raised $1,150 for batten disease research

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.