Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Disease)

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Shirley
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Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Disease)

Post by Shirley »

Hello,

We're new here. We expected our first visit would be for our senior dog Lady not for our 5 year old Dalmatian.

We were recently told that Domino probably has Batten's Disease. Neuronal Ceroid Lipofuscinosis. It is an inherited neurodegenerative lysosomal storage disease. There is no cure. I have not been able to find anyone else who has a dog with this disease or knows of someone who has. If anyone does I would like to talk to them about how they are taking care of their pet. Any special diets, therapy, etc. Domino is an important part of our family and we would like to keep him with us for as long as possible.

We are trying to figure out ways to help him with his low vision, balance, and turning front paws under and other problems as the disease progresses. Any suggestions would be helpful.

Also has anyone been to a Veterinary Nutritionist?

Thank you,
Shirley
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critters
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Re: Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Dise

Post by critters »

I have to say that Batten Disease/Batten's Disease was a new one on me, although I was reading about another lysosomal storage disease somewhere recently (no idea where).

I guess you know this is a human problem, too, and perhaps you could find something of interest and/or help on the human sites? There are TONS of them, although there doesn't seem to be agreement as to Batten v. Batten's. Googling <"Batten's Disease",dog> showed some treatments, including bone marrow transplants.

Dog-specific articles include http://www.vetpathology.org/cgi/content/full/37/5/488
http://omia.angis.org.au/retrieve.shtml?pid=2677
http://www.dogstuff.info/cl_in_border_collies.html

and 10 more pages of stuff.

You'll probably need wheelchair info; Karen Andy's mom can help with vision issues. Anything else you can think of?

I'd suggest a quad cart (4-wheeler) because of the likelihood your baby will eventually need all-over support. Do you need manufacturer or homemade info?

Are you ready for expressing and/or diapering info? This can help A LOT with skin care and general cleanliness.

While I don't know that anybody here specifically has a critter with Batten's, MANY people have experience with the issues you will face--paralysis, incontinence, etc. All you have to do is ask.



http://www.vetpathology.org/cgi/content/full/37/5/488
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GabrielDeafBlindPupFamily
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Re: Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Dise

Post by GabrielDeafBlindPupFamily »

Blindness, yes. I am so very sorry for this diagnosis. It's a new one on me, but I've been very lucky.
I have 2 blind pups. 1: baby gate stairs. 2: scent areas ie: water dish, door, steps to the yard. 3: doggles outside. 4. bell yourself and other critters (sorry critters) so puppers knows where you all are.
My blindpup excelled immediately and can do anything. Here's a wikipedia article on blindness. Understand that as soon as pup gets "used" to the blindness issue, that you will hardly know there is a blind issue. My deafblindpup is even more amazing, as he has no audio to guide him. But you could swear he can see.

I know this should be posted under blindness, but it'll be easier for you to see if I post here :)

CHAPTER ONE
If your pup is born blind, this is an advantage in many regards, as they don't develop quite the startle and fear response as a formerly sighted dog. A blind dog in the wild is a dead dog, and this is the first thing you must understand. The second thing is that a blind dog can do anything a sighted dog can do, except catch a frisbee on the fly.

If you find yourself with a blindpup, first understand that your view of the world is going to change. Your blindpup will teach you things you never thought possible. A born blind pup learns as she begins to crawl around (all puppies crawling around are blind at birth and for 3 weeks or so thereafter) and she compensates for bumping into things by developing her other senses. Your heart will break as she prances with a toy and BAM into the couch, the wall, the door, you. She will shake it off and continue her prancing, remembering that there is a couch, wall, door, mom at a particular place. She is developing her map. The map is the thing that enables these pups to exceed and excel. I am not sure how it works, but I am stunned by the accuracy. My blindpup has mapped two houses, two yards, and racing around willy-nilly chasing the cat (yes, she chases the cat at 100 mph) and never ever loses her map. She knows exactly where she is at every moment, despite running around furniture, rooms, moving objects. The map. My deafblindpup has it and when he gets fearful or very excited, he loses his map. You can visually tell when this happens. He doesn't lose it often and when he does, I touch him softly and he relaxes and gets his bearings.

With any blind dog, the first thing you do is get on your hands and knees, crawl around with pup (this is extremely fun for them) and look for pokies and owies. Inside and out. You want to babygate all stairways, some folks pad hard corners and surfaces (I have never needed to, one good thump and that thing is mapped) and some folks get doggles to protect eyes (in my house, I would be the one wearing them, as my pups are airborne. Also they can get them off in a skinny minute. You can also use a surgical e-collar to work as a "bumper". There is a product called the AngelVest, which I recommend, but I have never had to use one). A lot of folks use scents to mark different areas, again, my pups don't need it. Bell train your pup. Put her on short lead, sit on floor and teach her the come command as any sighted dog, only use a bell. Then when she hears the bell, she knows where you are. My pup would get "lost" in a room by herself and cry her eyes out. The bell became indespensible then. She could track the bell more easily than my voice. It's also helpful in the park when she's on long lead, to get her to come to you when she found a stinky in the grass, to keep her from doing what dogs will do.

Touch training is simple combined with basic obedience training, and you will charm judges at all silly pet trick contests. For GabrielDeafBlindPup a touch on the nose is sit, a pat on chest is down, a touch on head is up. AllicksBlindPup sits on touch on nose, down with touch on foreleg.

Housebreaking is the same as a sighted dog. Get them out first and last thing of the day, same place each time, and after every single meal. A hearing blindpup will be ecstatic over the sound of your voice, so praise in a high, sing-song voice, using their name over and over. Sound is so important, so keep it soothing, upbeat and fun.

Retrieved from "http://www.handicappedpets.com/mediawiki/index.php?title=Blind_dogs"
Karen, Andy's ^i^ mom
Lethal White Aussies Rule!
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Shirley
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Re: Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Dise

Post by Shirley »

Thank you so much for the information. You brought up things we hadn't considered yet.

Yes we did know that it was a children's disease. That was the first thing the neurologist told us. I had not seen the links you provided. Thank you so much.

The website www.bdsra.org. offers booklets on the disease. I'm going to call and see if I can get copies. I'm also writing the veterinarian who is doing current research on Tibetian Terriers to see if he can offer any information.

Nutritionally there are some things that I have read that help slow the disease in children. Vitamin C & E, and carnitine are supposed to be helpful. The neurologist said Q10 may help but did not want us to give it yet. I also read that Vitamin A was bad. Domino has been on Hill's Prescription U/D since he had surgery for bladder stones in April. That has A added and carnitine. I don't know if they cancel each other out. For children it states that physical and occupational therapy may help. A dog article stated to restrict activity. I've also read that this is a cholesterol degradation/transport disease. I'm wondering if cholesterol lowering foods would help.

I emailed Hills and they didn't have the information and said my vet should call their Veterinary Consultation Service.

We want to see a Veterinary nutritionist and talk to our vet about the bone marrow transplant. We also need to find out about vaccinations, heartworm and flea control. I know dogs with cancer and some with immune problems are not supposed to be vaccinated.

Regarding quad cart (4-wheeler) I think we'd need information on both homemade and manufactured. My husband's pretty handy but he'd have to see design to see if he could do it.

We've also been talking about pet ramps for vehicles. Domino has problems jumping up. But with his balance problems I'm afraid he'll fall over the edge.

We're not ready for expressing and/or diapering info yet. Domino has still been able to go out. Dave has had some experience with expressing the bladder with a cat we had. Diapers we'll need some pointers on. Domino absolutely hates having his backend touched.

We're never sure how Domino will wake up. Some days he's almost normal. Later he looks like he's had to much to drink. He gets confused at times. Aggression is something else we're going to have to deal with. I also read that he can go deaf. The list of symptoms keeps growing. I guess it depends on what part of the brain is affected.

Something I am worried about is eating. When its meal time he crouches down to scoop food from the bottom instead of eating from the top. We've tried raising his dish. The way he eats it just goes all over the place. We sit there putting it back in the bowl for him. He's hungry all the time and just gulps it down.

Thank you for all your help.
Shirley, Domino and the gang
Shirley
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Re: Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Dise

Post by Shirley »

Thank you so much you've been very helpful.

We'll start with the new training and see how it works. I'm also checking out the book "Living with Blind Dogs" from my library. Eventually Domino may go deaf from this disease too.

Yesterday morning I woke up to find Domino had a cut under his eye. Have no idea what he ran into. Because this disease has affected his balance we never know if he's running into things because he can't see or stumbles.

We have a total of four dogs and five cats. Two are seniors- 1 dog (Lady) and 1 cat (Andy). Andy still acts young. Hasn't had any problems. Lady has arthritis, spondylosis, low thyroid and kidney insufficientcy. But she has become our energizer bunny. She has problems with her vision too.

This is probably a question I should post to everyone. What do you do with the humans involved. My husband doesn't seem to have alot of patience when it comes to Domino. Which really surprises me. He was great with our dog Ernie Bear who had terminal cancer and with Lady.

Thank you again.
Shirley
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critters
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Re: Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Dise

Post by critters »

Shirley wrote: For children it states that physical and occupational therapy may help. A dog article stated to restrict activity. I've also read that this is a cholesterol degradation/transport disease. I'm wondering if cholesterol lowering foods would help.
I don't know why activity would be unduly restricted when the disease process will continue regardless; somewhere in an article I saw reference to cholesterol, but I don't remember any details.
Shirley wrote: Regarding quad cart (4-wheeler) I think we'd need information on both homemade and manufactured. My husband's pretty handy but he'd have to see design to see if he could do it.
homemade ideas: http://www.handicappedpets.com/HomemadeCarts.htm

Not everybody makes a quad cart, but here's the manufacturer list. http://www.handicappedpets.com/wheelorder1.htm
Shirley wrote:We've also been talking about pet ramps for vehicles. Domino has problems jumping up. But with his balance problems I'm afraid he'll fall over the edge.
http://www.handicappedpets.com/wramps/
Make or find one with a railing?
Shirley wrote:Dave has had some experience with expressing the bladder with a cat we had.
Is Dave your hubby? This should come in handy!!
Shirley wrote:I also read that he can go deaf. The list of symptoms keeps growing. I guess it depends on what part of the brain is affected.
The deaf dog expert is also the blind dog expert, so I'm sure she'll be back for another message. LOL. I'd bet you're right about the part of the brain being affected.
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critters
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PS ramps

Post by critters »

Given the nature of this, I think I'd use a wide-ish, low-slope ramp with siderails wherever possible. Commercial ramps seem so steep, for the most part. Your handy hubby might have LOTS of entertainment for awhile! :lol:
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GabrielDeafBlindPupFamily
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Re: Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Dise

Post by GabrielDeafBlindPupFamily »

Dear Domino, Pup, your ma is so worried about you.
Shirley, try a surgical e-collar to act as a bumper. I have them for my pups, but Allicks is AIRBORNE.
You can pick them up at Petsmart for a fraction of what you pay at the vet.
Karen, Andy's ^i^ mom
Lethal White Aussies Rule!
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http://www.s8.createphpbb.com/lethalwhiteauss/
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Tiffany
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Re: Help Needed: what to do with them humans.

Post by Tiffany »

Hello
Maybe its because with the terninally ill and elderly there is an end in sight, I'm not trying to be heartless but as the caretaker of a not even 60 year old stroke victim I can tell you its heartbreaking to think about how much longer are they going to have to live like that, AT FIRST. You could try to talk to your husband and once he learns that your baby can be happy just the way he is maybe he'll feel better about it. Again please don't take what I said as a negative but just a statement from someone who as much as I hate to admit it has felt like that before, not with an animal but from the way it sounds your pets are just as important as people. I could also be waaayyy off base here Its just a thought.
I hope this helps a little bit.
Eros & Tiffany
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Shirley
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Re: Help Needed: NEURONAL CEROID LIPOFUSCINOSIS (Batten Dise

Post by Shirley »

Yes, Dave is my hubby and he is really handy. Thank you for the links. It will keep him busy.

Regarding limiting activity. The article also mention not using stairs. Dave thinks that maybe excercising produces more toxins that accumalate in the brain. Or maybe he burns up muscle and not the fat.

Rails for the ramps is a great idea. I told Dave and he was trying to figure where we'd stow it in the car. We have a small station wagon. We have to figure out how to make it so it folds up.

Dave called bsdra for me yesterday to get booklets. They gave him the number of a doctor who's working with dogs. So we're waiting for a call. If he can't offer any suggestions then bsdra said to call them back and they'll see what they can do.

Everyone has been so helpful.

Shirley and Domino[/list]
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critters
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misc. eating, ramps, etc.

Post by critters »

Keep your eyes open as you wander stores; you might find something perfect for him to eat out of; I was thinking of something deeper with rounded walls to funnel the kibbles back down, something sort of fishbowl-y.

Maybe you could buy a commercial folding ramp and have Dave make some sort of (clamp on?) railing so you'd have both portability and safety.

Since Dave has experience with expressing, you can use that when the time comes that your baby is leaking, to help keep mess to a minimum. He may need to adapt his technique to a larger victim (LOL), but he should have a really good start. We can post expressing, poop on demand, and diaper info when you're ready.
Shirley
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Re: Help Needed: what to do with them humans.

Post by Shirley »

Eros & Tiffany wrote: Maybe its because with the terninally ill and elderly there is an end in sight.

I see what you're saying. I did ask him why he was harder on Domino. He hadn't realized it.

Because Domino looks okay you forget he has a problem. [/i]
Tiffany
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Re: Help Needed: what to do with them humans.

Post by Tiffany »

Hello
Well thats good that you brought it to his attention, and he is going to try to be better. I wish I had more insight to help you out, but your at the right place.
We wish you the best of luck!
Eros & Tiffany
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Shirley
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Re: misc. eating, ramps, etc.

Post by Shirley »

Re:Keep your eyes open as you wander stores; you might find something perfect for him to eat out of.

We will.


Re: Maybe you could buy a commercial folding ramp and have Dave make some sort of (clamp on?) railing so you'd have both portability and safety.

Great Idea! Thank you
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