This is a blog dedicated to our Cavalier King Charles Spaniel puppy, Riley. He was just 17 months old when he was diagnosed with Syringomyelia. We are DETERMINED to over come this, make his life as rich, full and amazing as it was going to be before we knew of this diagnosis. We hope that other doggie mommies and doggie daddies out there who also have a baby with SM will be able to use this as a source of information and inspiration in their own personal journey through and to the other side of having a family member with SM.

Monday, February 15, 2010

Riley's Consultation with the Neurologist

We took Riley up to se Dr. Jason Berg who is the Neurologist up in Yonkers, New York who our family veterinarian Dr. Jen Mlekoday recommended.

First, a word about Dr. Berg. He was kind, affable, friendly and great with Riley. He gave us almost a full hour consultation without feeling rushed and we left having all of our questions answered to our satisfaction and understanding. Dr. Berg reviewed Riley's clinical history and his MRI "films" (quotations because they're all digital nowadays- no more film involved.) Here are the bullet points/ highlights of our discussion:

* Dr. Berg said that 3 out of 4 times when he gets a CKCS with SM, he recommends starting with medication therapy and holding off on surgery. This however is NOT his recommendation with Riley. Even though Riley has very mild clinical symptoms and his "episodes" of pain have been very few and far between (and very short lived) Dr. Berg feels that, in conjunction with the severe findings on his MRI, and his very young age, surgery is the better route. In his words, "On a scale of 1 to 10 where 10 is the most severe, Riley's clinical signs and symptoms are about a 2- maybe a 2 1/2- out of 10, but his MRI findings are an 8 or 9 out of 10."

* The most common procedure employed in surgical management of this condition is called FMD- Foramen Magnum decompression. The principle of this surgery is to restore normal Cerebrospinal Fluid (CSF) flow from the brain into the spinal cord and back by removing the bone at the back of the skull (supraoccipital bone) and part of top of the first vertebrae. This may be combined with a durotomy (cutting the meninges) with or without patching with a suitable graft material. Of late, in much of the veterinary literature, there has been a trend towards applying a Titanium mesh over the surgical area. However, this has been associated with scar tissue formation. In many cases, this compresses the spinal cord as much (if not worse) than the original malformation/ condition. It is for this reason, that Dr. Berg does NOT recommend this procedure. There is also a variation on this procedure where a "shunt" is put in- like a tube- looking to restore connection so that the CSF can travel through this area of narrowing at the base of the skull. He also does NOT recommend that procedure. He said that dogs like the CKCS are very spunky and active and an insecure shunt sitting at the base of the brain could jiggle around later on after the surgery causing irritation, compression or worse to the adjacent spinal cord. Dr. Berg said his surgical recommendation would be to do the FMD with interposition of the temporal musculature and fascia. While this procdure can be technically difficult, if not impossible, in smaller breed dogs, Dr. Berg said that this is very simple to accomplish in the CKCS as the breed has abundant musculature at the back of the head about the temporalis muscle and it's underlying fascia. He uses this muscle and fascia and sutures it into the space where the bone was removed, holding the contents in place. There is no chance for "rejection" as no foreign material is being implanted, and the scarring potential is lower.

* As far as starting with Medications, there is a treatment algorithm on how to approach medication vs. surgical treatment in the CKCS. Step 1 is to administer medications (like Furosemide or Cimetidine) which lower CSF production. Depending on responsiveness to this treatment, there are other steps such as NSAIDS, Steroids, Gabapentin, and the list goes on. Dr. Berg said that he has not seem much clinical improvement in pain levels using medications like the first-level CSF reducers in dogs as young as Riley with a large a Syrinx. Dr. Berg was very clear that, in his experience, both medication and surgery do very little to alleviate the "air guitar" or "Phantom scratching" that CKCS with SM do. Even if we opted for surgery, Riley would likely continue to do the "air guitar" and "Phantom Scratching" afterwards, but the treatment goal is to alleviate the pain associated with the activity, not the act itself.

So, our decision is boiling down to this paradox: Right now, Riley is mostly asymptomatic. If his current level of irritation/pain were to never change, then surgery would NOT even be on the table right now. However, as SM is a neurological condition that is progressive, the longer we wait until he has more constant and significant pain, the more potential damage is being done to his spinal cord and nervous system. So do we operate now on a young and healthier dog and put him through this significant surgery (even though he currently has less clear-cut signs and symptoms of pain) because we know that the severity of the disease in young (under 2 yrs old) CKCS with findings as severe as Riley's tend to suddenly progress really quickly and badly? Or do we chance it and wait and see if he will perhaps not progress and, hence, not need the surgery (though, knowing that if he did, he would be disadvantaged for having waited)?

Dr. Berg used the analogy of compressing a marshmallow with your finger. If you stop pressing it after just a short time, the marshmallow will puff back up. Maybe not 100%, but it will restore shape and volume to some degree. However, if you wait and hold your finger pressed into that marshmallow for much longer, it becomes less likely that any of that compressed space will decompress and restore back to it's original size/volume.

What he says makes sense. And I know that nobody wants to take their happy puppy and bring him into surgery one day and have them open up his skull, operate around his brain-stem and spinal cord, and then deal with the aftermath, the nursing him back to health, and the enormous cost associated with this surgery. Still, the cost of waiting or delaying treatment, could be greater in the longer term.

So, we have a LOT to think about. I have reached out to Dr. Clare Rusbridge, BVMS PhD DipECVN MRCVS RCVS. She is a Certified European Specialist in Veterinary Neurology who has written the definitive papers on treatment of SM in the CKCS, and she is arguably the world's leading Veterinary Neurologist on this topic. I'm hoping that if she has the time to take a look at Riley's case, perhaps she can help us negotiate some of these difficult decisions.

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