Josh Needs Our Help and Prayer

Meet Joshua Paul Spencer, three year old son of Leann and Paul Spencer of Longview, Texas. Isn't he a precious little boy.

  We have a story to tell you about little Joshua. About this time last year he began to have some difficulties with his breathing and he began to have seizures. Leann and Paul began a long series of trips to doctors from Houston to Dallas and experiencing the tedious and stressful agony of test after test, and wait after wait for results until finally a short 5 or 6 weeks ago he was diagnosed.

Joshua has Infantile Neuronal Ceroid Lipofuscinosis. In layman's terms it is called Batten Disease. It is a very rare disease, so rare it affects only about 3 in 100,000 and it is always fatal. This disease is a degenerative neurological disease that is inherited and usually affects young children, however has affected older children and even adults before the age of 40. It was first described in 1826 by a British pediatrician. The name Neuronal Ceroid Lipofuscinosis is a group of diseases and Batten Disease is the general name given for the group. What little Joshua has is the most common of the diseases and is also one of the diseases found in a group known as Lysosomal Storage Disorders.

Batten Disease is rarely diagnosed immediately and is often mistaken for Epilepsy, Mental Retardation, Retinitis Pigmentosa, and even Schizophrenia in adults. The onset of this disease is characterized by beginning vision loss, seizures, clumsiness, personality and behavior changes and causes continuing physical and mental deterioration until death.

It is an recessive inherited disease meaning both parents must carry the same gene. A child must inherit a copy of the bad gene from both parents in order to be affected. This explanation surely helps us understand why it is so rare a disease.

At this time there is no cure for this disease and the prognosis for Joshua is 6 to 12 years. Little Joshua has passed through the onset of the seizures and has already come to the stage that he has lost his ability to walk. The next stage is blindness, then retardation, then death. Joshua¹s Grandparents, Mary and Ray Bucknum of Lott, Texas tell us they are believing the Lord for a complete healing for Joshua and is asking you to join with the family and their church family in agreement in prayer for his complete healing.

Recently, the Make A Wish Foundation granted Josh's wish, and took him to Florida. Everyone involved believes that as a result of their prayers and faith, and God's faithfulness to answer prayer, Josh was able to walk during that trip, and enjoyed it immensely.

As we lift Joshua and his family up in prayer and believe for his healing there is a second request for prayer on another issue, and that is the cure for this terrible disease.

Rare diseases generally do not get the attention or the funding needed for research for many reasons and this one is not on anyone's priority list. However, there is hope and we are told the cure is just dollars away.

An international non-profit organization that has been created to provide information, support, and research called Batten Disease Support and Research Association or BDSRA is working very hard to eventually eliminate this child killer and have set up offices in the US, Canada, New Zealand and Australia. They provide services to the families to help them with information, mentoring, medical specialist referrals, sibling support, networking, participation in research, genetic testing, counseling and bereavement outreach. They work with medical professionals to obtain and disburse pertinent information to the public.

Mary and Ray Bucknum, owners of Maray's BBQ in Cameron, Texas are hosting a silent auction fund raiser to help promote the cure for this disease.

It will be held at Maray's BBQ on HWY 77, just across from KMIL Radio on December 13th from 11 am to 3 pm. FREE BBQ PROVIDED.

Any and all items that you can find to donate to be sold at auction will be greatly appreciated and all items will be accepted. Please contact Mary or Ray at 254-584-7051 or 254-697-8822 for information to donate your items to this very worthy cause.

Donations of funds will also be accepted to help the family with the costs involved with caring for little Joshua. You may mail your donations to:
Texas Bank & Trust
P.O. Box 3188, Longview, TX 75606
to the Leann Spencer Hope for Joshua Fund.
To speak with Leann or Paul Spencer you may call them with your support in Longview at 903-668-2732.

To learn more about the disease and see Joshua's web site please visit www.bdsra.org. Here you may see him and other children afflicted with this disease.

And remember, The greatest gift we can give is prayer and we quote here again "the effectual fervent prayer of a righteous man, availeth much"James 5:16.

See you at Maray's on the 13th.