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.

Tuesday, February 23, 2010

Contact v2.0

Sometimes you need to abandon email, mobile devices, etc... And just PICK UP THE PHONE!

I came home from the gym at 6:30 am and there was still no email response from Dr Rusbridge so I called over to her office in London. Her assistant was very sweet and helpful. Apparently, she has been emailing me but the messages never made it to my inbox(or spam box, which I've been checking nearly hourly.) SO GLAD I CALLED!!! Now, with the proper address and other info, I am on my way to the bank to get an international bank cheque and then we shall overnight all of Riley's stuff. Hopefully, in a few days, we might have her expert recommendation.

Of course, all of this has me thinking...

I think I've been hoping that she will make a clear-cut, "Yes you must" or "No, you mustn't" recommendation. On some level, this would alleviate some of the responsibility that comes with having to make this kind of decision for a loved one. Of course, there is the possibility that she could come back with an equivocal recommendation and that would leave us right back where we are now- totally torn. I suppose, on some basic level, I need to come to terms with the reality that Michael and I still ultimately must make this decision and be able to live with it, either way. However I may wish to emotionally abdicate responsibility and let someone else step in and take control (and "be on the hook" for the consequences) it's just now hitting me that even the choice of following Dr. Rusbridge's ultimate recommendation is still a choice that I/ we would be making. In the end, I want to do what's best for Riley. Nearly equally important, however, is that we make a choice that we both can live with and that will not haunt us for years to come, regardless of the outcome.

Monday, February 22, 2010

A Difficult Weekend

So we're still waiting to hear back from Dr. Rusbridge. It's been 5 days since our one and only email contact with her and she has yet to respond to my last email asking for information as to where I should be sending this material. I can only surmise that she's traveling, away from the office, or that she has Tuesday-Thursday office hours (which seems likely, based from some stuff on her website.) Sadly, this means that we're still waiting to find out where to send all of the materials, consultation reports, MRI films, etc... so she can do the consultation.

Meanwhile, after speaking with our family vet, we decided to start Riley on 5 mg of Omeperazole, daily. This is one of the first line medications used/recommended in the medical management of Syringomyelia, as it has a side-effect of lowering the production of Cerebral Spinal Fluid (CSF). This, in turn, could potentially lower the pressure in the syrinx at the base of the brain/spinal cord and alleviate his symptoms.

Sadly, this doesn't seem to be working. If anything, Riley has become more sensitive, his "episodes" are more frequent, and he has some hind leg lameness. He's not jumping up or down as much as he used to, and he's taking stairs (both up and down) very cautiously and that's both in and out of the house. Clearly Riley's not feeling great. Yesterday afternoon, he was lying on my lap. I was petting him gently, stroking his hair and as I often do, I told him "I love you, little man." He looked up to give me a kiss (which is what he usually does when I tell him this) and as he crooked his neck upwards he let out a frightening yelp and then he froze, locked in place, guarding against this horrible pain that suddenly gripped him. He seemed unsure as to whether he should lay down, sit up, or what to do... so he just froze in place. It was heartbreaking.

I'm starting to become very conscious of time, ticking away. I'm getting really concerned that we might be unnecessarily delaying a surgery that needs to happen. Still, I'm even more concerned going into this and making the decision without Dr. Rusbridge's input. After all of my research over the weekend there's one thing that's very clear: The research is conflicting. There are no large sample populations, no large prospective studies and it really seems to be a case-by-case situation with respect to doing surgery or not. For that, I want the world's premiere expert on Syringomyelia to have some input into this decision. So let's all hope we hear from her by this time tomorrow.

Thursday, February 18, 2010

We Have Contact!!!

After emailing Dr. Clare Rusbridge (the leading expert on SM in the CKCS) I heard back from her (via email) this morning. I am gathering all of the documentation, consultations, reports and MRI images and getting them international-express mailed out to her this afternoon.

We are so excited that she will be reviewing this case, and we're really grateful for her generosity with her time, expertise and we're hopeful that she can see through this, understand our concerns, and help us to make a better decision for Riley.

More to follow...

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.

Wednesday, February 10, 2010

Riley has Syringomyelia

It was those three simple words that instantaneously crushed our hopes of enjoying a normal future with our 17 month old puppy, Riley. All of the hopes, plans and dreams of our beautiful puppy growing up into a healthy, active dog who lives well into his teens seem less sure with the uttering of these three words: Riley has Syringomyelia. We found out yesterday, at 3:05 pm, after his MRI was done and the radiologist was kind enough to take us aside and slowly go through his films.

A bit of history on Riley: We have had concerns about Riley since he was a puppy. Specifically, we've been worried that he may have Syringomyelia (SM) which epidemic with the Cavalier King Charles Spaniel (CKCS)breed. Our initial concerns were, admittedly, based on some very broad symptoms that are not uniquely specific to SM. We brought this up with our vet in NJ when he was a puppy and he poo-pooed it. When we switched Riley over to our old vet back in New York City last fall, we mentioned it to Dr. Mlekoday and she said (correctly) that the signs and symptoms of his "air scratching" and his extreme tenderness (that causes yelping) when touched under his arms and around his neck should just be presumed to be a tick unless (at some point later on) it is suddenly accompanied by pain. Well, it never has been until now. Of late, his scratching can go on for quite prolonged periods of time. It's totally maniacal behavior. Compulsive. Uncontrollable. But he's only 17 months old, and lots of behavioral quirks can go on until after he's two. So, really, there has constantly been this underlying belief of, "It's nothing and he'll grow out of it."

The tipping point was this past weekend. Ethan was sitting quietly on the couch, Riley was fast asleep next to him. All of a sudden, for no reason, he leaped up out of his sleep and started shrieking and yelping in pain. We couldn't calm him. He did it a second time later that night when sleeping next to Michael and then he started to favor his back left leg. All of this together triggered a gut instinct in Ethan and it made us very suspicious. (Ethan's side note: Call it clinical intuition, but my practice of podiatric medicine and surgery is -like most good clinicians- based on my gut feelings in many ways. My gut told me this was not good.)

We decided that, even though an MRI is phenomenally expensive, the cost of not knowing and not treating this early enough could be greater. So we called yesterday and scheduled him for the MRI today. We just came back from his MRI and he's resting comfortably at home. Sadly, my instincts were correct; Riley has Syringomyelia (SM). A pretty significant case of it, too. The lesion (syrinx) goes from the brain-stem/Cerebellum as far down as the MRI imaged (past C-6). More than likely, if we scanned him all the way down to the tip of his tail, there'd be more lesions, too. That's what the veterinary radiologist said.

We spoke with our family Vet, Dr. Jen Mlekoday, this morning and she felt that we should head over to see Dr. Jason Berg up in Yonkers, NY. She said that he was involved in some of the earlier studies and research in the United States on the phenomenon (and subsequent epidemic) of SM in CKCS. So we have an appointment with him for Monday morning and will likely have more information then. We have been looking, though, at surgical specialists in SM in The CKCS around the country and there are quite a few, so we'll need to do our research before committing to anything one way or the other. We have started Riley on Rimadyl 25 mg daily to help decrease inflammation around the inflammed structures. This is step one and we'll see if this helps at all.


Below on the left is a "Normal" MRI scan of the Brain and Spinal Cord of a Cavalier King Charles Spaniel. It is labeled and for anatomic structures:

Below on the right is not Riley but a labeled scan of another Cavalier King Charles Spaniel with Syringomyelia. Note the differences from the above (normal) scan:

And here, below, is Riley's MRI from yesterday. Note the severe swelling of CSF about the spinal cord, the extrusion of the medulla through the foramen magnum, the hydrocephalus and edema in the ventricular system. The syrinx, though not visible in its entirety on this view, extends the full length of his C-spine: