Syringomyelia in Dogs: The Dark Cloud Of Syringomyelia⁠—Fight For Ella

Syringomyelia is one of mankind’s “great gifts” to dogs–Cavalier King Charles Spaniels to be specific, although it can affect other breeds as well.

Thank you, Annie Mac, for sharing Ella’s story.

Syringomyelia in Dogs: The Dark Cloud Of Syringomyelia⁠—Fight For Ella

As we selectively breed for our own specific purposes, it is our dogs that end up on the short end of the stick. I believe that we should, for once,  put our interests aside, and start doing what is right for our best friends. Let’s breed responsibly with the welfare of our dogs in mind!

In the meantime, it is very important to make yourself familiar with any hereditary diseases that might affect the breed of your choice.

It is estimated that over 50% of Cavalier King Charles Spaniels are affected by syringomyelia!

What Is Syringomyelia?

Syringomyelia (SM) is a disease that is just as nasty as it is trying to pronounce it. However, phonics aside, if you are considering getting a Cavalier King Charles Spaniel, or already have one, I urge you to learn about this condition.

Technically speaking, syringomyelia is caused by a partial blockage of the flow of cerebrospinal fluid (CSF). As a result, pressure builds and the fluid is pushed into the spinal cord where it forms fluid pockets called “syrinxes”, damaging the spinal cord. Affected dogs can suffer significant pain, weakness, incoordination, and even paralysis.

Syringomyelia can occur as a complication of trauma, inflammation or a tumor. The most common cause in dogs, however, is a hereditary malformation.

In Cavalier King Charles Spaniels, syringomyelia is caused by a skull malformation that compresses and often herniates, the back part of the brain near where it attaches to the spinal cord. In other words, the skull is too small for the brain.

Symptoms of syringomyelia in dogs

Not all affected dogs will show symptoms, or their symptoms can be overlooked or misinterpreted. That’s why owner awareness is so important! It can save your dog’s life.

One of the typical symptoms is scratching the air near the neck, often only on one side of the body. That’s why syringomyelia is often referred to as the ‘neck scratcher’s disease. Some of the other symptoms can be

  • sensitivity around the head, neck or shoulders
  • restlessness
  • lethargy
  • reluctance to exercise
  • difficulty moving or incoordination
  • limb weakness
  • symptoms associated with pain

The only way to conclusively diagnose syringomyelia is with magnetic resonance imaging (MRI). Your vet will likely want to exclude other causes of scratching and discomfort first.

Ella’s story

When I received the call in January 2010 from Ella’s neurologist with her MRI results telling me she had a severe case of Syringomyelia with a large syrinx, my world stopped. I thought I did not hear it right. I was the one who read about Syringomyelia and knew all the symptoms and demanded she is seen by a neurologist. Ella’s first signs caught my attention probably when she was about 2 years old.

Ella’s initial symptoms

She was almost 4 when she was diagnosed. It was nothing major, it just seemed like she scratched more than usually.  I tend to notice everything about her. Her vet said that it was probably food allergies so we changed her food and I didn’t really think anything about it.

Then several months later she just looked funny walking up the stairs. No one else could see what I saw. I even took her to see her vet watch her go up the stairs.

It felt like taking their car to the shop and it works only when you are there. People thought I was crazy. She would put one paw in front of the other and she always seemed to walk on one side. She always seemed to scratch at her ears, which was diagnosed as an ear infection.  All of these things may sound like typical dog behavior and some of it could be but looking back on it, they were pieces of a puzzle.

Lethargy

It was probably in April 2009, I noticed the lack of energy. I would think she was just tired from daycare. I would laugh and say what is wrong with you? In April a Cavalier Meet-Up group began in Charlotte and I said I would write a newsletter and I included some health information.  It was there that I read about Syringomyelia.

My heart stopped. I immediately bought the book For the Love of Ollie and donated to SM research. I don’t know, maybe deep inside I knew that something was wrong with Ella.  All of my friends could not see it and the only way to really diagnose it was with an MRI which for me cost $1700.
The other things I noticed was the restless nights. She seemed to never get comfortable. Always making a bed or rubbing her face on the covers.  I heard about the “phantom scratching” and I didn’t really see that. I started to look to see if she was scratching on one side.  She was always scratching on one side.  I paid close attention to all the things she was doing.

Ella declines

It then went downhill real fast.  In about two weeks she was hiding under the bed, under the table, laying on the floor, shaking her head constantly, hardly able to walk up the stairs, dullness in the eyes. Again I went to my vet and he said not to jump to any conclusions it’s probably an ear infection and allergies and we still want to rule those out before I recommend her to go to a neurologist.

I knew she had Syringomyelia.  That weekend I took her to my cousin’s house and the thing Ella likes to do more than anything is run after the ball.  I threw the ball and she did not move. It made me cry and I called and demanded them to let me see a neurologist.  She went that following Monday.  I thought I caught it before anyone.  My friends did not even think she needed to see a neurologist so when I found out her condition was severe I was shocked.  I think it was because of the rate of her progression.

Now that I knew she had it, the hardest part for anyone that finds out their dog has Syringomyelia is deciding what treatment option to take.  I can not recommend which is the best option because it is different for each dog.  Her neurologist said he could not tell me she would be around in 3 months and I couldn’t live with that. Ella had a severe case and it was progressing fast. She was first put on medication but she had surgery a couple of weeks later.

The fight is not over

Sadly, her fight was not over as Ella’s symptoms seemed to be making a comeback. And there was a chance that she might need a second surgery. She does need a new MRI to properly assess the situation.

One of my cavalier books from barrons said: “if the eyes are the window to the soul, the cavalier king charles spaniel has the kindest soul in dog-dom. To peer into the eyes of a cavalier is to enjoy a vision of utter devotion and to fall completely under their spell.” Staring to ella’s eyes when she is in pain deeply hurts me. I wrote about Ella needing a 2nd MRI and that is because surgery may not have been successful. The reason I know this is because I see the pain I once did before her surgery. I read stories of people and Cavaliers with Chiari and Syringomyelia needing multiple surgeries and I knew this was a chance, but I had hoped it would not be Ella.

Ella used to cuddle with me on the couch but now she lays on the floor. She looks at me with those eyes of pain and I can hear faint sounds of pain. I miss here and have to call her to come out from her hiding. They sometimes do this because it can be painful to be touched. How much I miss those days of her laying with me. She has good days but recently her behavior has changed. No one can tell you how hard it is to see this.

A lot of dogs don’t even do surgery and manage fine on medications but some are not so lucky. I read articles about studies and they will say state dogs as cases and they will say was euthanized after 1 year or another case that has shown no new symptoms and improvement.

I know I will do anything for her but I am scared. Please pray for her. The surgery is scheduled for September 1st. I will have my paycheck then and will need to figure out next month how to manage. I am trying to come up with fundraiser events.

Cranial/cervical decompression

The most common surgery to relieve the effects of syringomyelia is cranial/cervical decompression (also described as foramen magnum or suboccipital decompression). The flow of the cerebrospinal fluid (CSF) is freed by removing both parts of the skull and part of the first vertebrae. That is the surgery Ella had done.

This surgery has a good success rate in reducing pain and neurological symptoms. However, 25% to 50% of cases can develop scar tissue that will obstruct the CSF flow once again and lead to relapse. Ella has been put on additional medication and considering the signs she is showing there is a good chance that she is one of the dogs who relapsed.

Ella’s update

There is no cure for Syringomyelia. Those words keep playing in my head. There is also no guarantee that Ella will not relapse after she has had an expensive surgery where they removed part of her skull and she spent months recovering from this procedure.  I knew that when I decided to take that chance.  It was the only chance I felt she had and if she was one of the ones that were not successful at least I did something.  I thought that would make me feel okay and I would worry about that later and figure something out then. I just prayed that she would be one of the lucky ones. Surgery gave her time. That was a given. It would at least relieve the pain but there is a possibility that when part of the skull is removed, scar tissue can form.  This will then cause relapse and dogs will require to have another operation.

Why has this been on my mind recently? There was a post on http://www.cavaliertalk.com with notes from Dr. Dewey who is a neurologist that pioneered foramen magnum decompression (FMD) surgery and spoke recently at the AVMA.  Someone took notes on what he said and his presentation was targeted to vets so most of it was not new information but reading again some of these statistics made me realize just how realistic relapse could be for Ella.  Dr. Dewey created a procedure using Titanium Mesh with the hope to reduce the rate of recurrence.  I knew about this procedure when deciding on Ella’s surgery but it was more expensive, more dangerous, and at the time. What would the long term benefit be?

I am constantly worrying about Ella getting worse.  Some days she is better than ever but then there are the bad days and I see the signs that she had before she had surgery.  I am reliving everything over again.  I know it has only been 6 months and we still have a long way to go.  Every head shake, every stumble up the stairs, every time she hides under the bed, every time she flinches when I try to touch her head my stomach drops. I have these statistics in my mind. She is just too young with too much to offer.  I know she has so many good days that to harp on the bad ones is not fair.

More medication

When I explained to her neurologist some of the things she had been doing, he seemed concerned and put her on some more medication. He mentioned doing another MRI and that was extremely scary for me. I know the more knowledge is better but if the MRI results are not good, then I am faced with the same thing as before. I worry but I have reason to worry because she has a 50% chance. One person told me to face the facts and that she is getting worse. To just adjust her medications and that is the answer. There are several articles you can read about statistics and dogs that are euthanized and success rates but it just makes me too sad. I am posting this to share my worries but I also know there are people that need hope. I also long for hope too and there is hope for many of them.  Ella is her own case. She is a special case and a special angel.

I have been avoiding this since Ella’s neurologist told me Ella would need a follow-up MRI in August.

The chances of a relapse are relatively high and even though I have seen the symptoms reappear, I have been scared. One reason is that I know I will not be able to do anything about it. If scar tissue has developed they recommend surgery to remove it. Surgery that I will not be able to afford.  Knowing that she is on medication and I still see some symptoms of pain and eventually she will get to a point that I don’t want to imagine and there is nothing I can do, will kill me. I will need all the support and prayers through the next few weeks.

New MRI results

We’ve been all waiting to find out what Ella’s new MRI results were.

Ella had her MRI last week and the results are not good. Ella’s syrinx is 50% larger now than it was at the time of her first MRI prior to the surgery. A lot of scar tissue developed at the surgical site.

Without a new surgery, Ella’s prognosis is not good.

Ella’s MRI confirmed what I feared and even though the results are bad I now have information that many people do not. Her symptoms were worsening and I needed to know what the situation really is. Ella’s results were not what I wanted to hear but I am glad because it might have caught the scar tissue before it attached.

Dog Conditions: Syringomyelia

The reason I decided to go through with the first surgery was that I felt her SM was progressing fast and her medications hadn’t been helping. Even with the surgery, which initially released the pressure and allowed for the CSF fluid to flow, and with several medications, Ella did not improve. In fact, she is worse now than she was before. Hadn’t she had the surgery, I doubt she’d be with me today.

Uncertain prognosis

Not all dogs with syringomyelia respond to the surgery the same way. While there was hope that surgery would reduce the syrinx from its original size, that was not the case with Ella. The neurologist explained that is has been determined that the main factor isn’t as much the size as it is the location of the syrinx. Ella’s is located in the worst place it could be.

I knew progression was bad, location is bad, medication is not helping, so I asked about alternative therapy. But the neurologist said that this is not a solution for Ella. I would like to point out that it is not because he does not believe in it—he actually gives Ella acupuncture—but it will not do in Ella’s situation.

Dealing with scar tissue

He talked about another surgery that would be done to remove the scar tissue that is blocking the CSF flow. He said the risk would be if the the scar tissue has attached which he will not know until surgery. If that is the case he would have to carefully scrape or remove each tissue—this could cause complications resulting in permanent neurological damage. It is not a given and I asked what the odds were and he said that there could be swelling, haemorrhaging, but he could say it was not as much as I feared.

He would put in a bone cement to cover the space that was removed to help prevent scar tissue to develop again.

We briefly discussed shunting which he said he would not consider.

If we decide to do the surgery, Ella’s neurologist is going to work with me and keep the cost as low as possible since he knows my financial situation.

This is what I found out today and I would tell anyone who is going to make such a serious decision to really think about it. I know I can not do anything and watch her get worse and suffer more pain. Putting her through the risk of surgery is scary and I have to figure out whether this is the best thing for her.

Surgery is inevitable

I was told that without the surgery Ella has months at best.

Someone in my family said that no one would blame me if I let her go considering how much it put me through and how hard it was. It was shocking to me because the only thing that concerned me was not what it would put me through, but what it would put Ella through.

Am I going to do this for her or for me?

If she was old and not have the life I see in her that would be one thing. It would be selfish to keep a dog barely alive just because I could not let her go.

But Ella has so much life left in her, and so much love to give. She saved my life when I needed saving and I am not going to give up. I am going to do everything i can (no matter if it is hard) for her. She is the most important thing and i care about her more than anything else.

I would like to thank all the great souls who helped so Ella could get her MRI and all those who remembered Ella in their thoughts and prayers. I couldn’t have made it without you!

Final notes

Ella eventually started feeling quite well. As fate would have it, Ella passes on since but to a completely unrelated issue. She succumbed to complications of a linear foreign body in her intestine. Her diagnosis came too late.

Related articles:
Syringomyelia Awareness: What is Chiari Malformation?
Syringomyelia Awareness: Teddy’s Story

Further reading:
Syringomyelia in Dogs

Categories: ConditionsReal-life StoriesSyringomyelia

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Jana Rade

I am a graphic designer, dog health advocate, writer, and author. Jasmine, the Rottweiler of my life, was the largest female from her litter. We thought we were getting a healthy dog. Getting a puppy from a backyard breeder was our first mistake. Countless veterinary visits without a diagnosis or useful treatment later, I realized that I had to take Jasmine's health care in my own hands. I learned the hard way that merely seeing a vet is not always enough. There is more to finding a good vet than finding the closest clinic down the street. And, sadly, there is more to advocating for your dog's health than visiting a veterinarian. It should be enough, but it often is not. With Jasmine, it took five years to get a diagnosis. Unfortunately, other problems had snowballed for that in the meantime. Jasmine's health challenges became a crash course in understanding dog health issues and how to go about getting a proper diagnosis and treatment. I had to learn, and I had to learn fast. Helping others through my challenges and experience has become my mission and Jasmine's legacy. I now try to help people how to recognize and understand signs of illness in their dogs, how to work with their veterinarian, and when to seek a second opinion. My goal is to save others the steep curve of having to learn things the hard way as I did. That is the mission behind my blog and behind my writing. That is why I wrote Symptoms to Watch for in Your Dog, which has turned out being an award-winning guide to dog owners. What I'm trying to share encompasses 20 years of experience.

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