Intro

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, April 6, 2010

5 Weeks and Counting....

It's been 5 weeks since raising Riley's dosage of Omeperazole from 5 to 10 mg. He's still yelping-free. He's running, jumping, playing, teasing his sister something awful... and, basically, doing all of the things we would expect a 19 month old Cavalier King Charles Puppy to be doing. The "air guitar" scratching persists, but as we've been told, that will always be present. In truth, it doesn't bother him at all and it's more of an unconscious "tick" than an annoying habit or repetitive behavior.

We are so lucky. He is so fortunate. We feel tremendous gratitude for this pain-free time he is having. We still take each day, one day at a time, grateful for this amazing "recovery".

Here's a quick/short iPhone video of him running around in the yard this past weekend:

video

Saturday, March 13, 2010

Decision

It's been a while and I apologize for not getting back here in the past two weeks. Between getting Dr. Rusbridge's consultation, discussing it amongst ourselves, coming to a decision and then reviewing it with our family vet, Dr. Mlekoday, we found ourselves on our way out of town for 5 days and it's been nuts since we came home. Let's catch up.

We decided that we are going to hold off on surgery. We realize that this is a risk, but so is the surgery. The bottom line is that, with all that we know now in March 2010, there still isn't a lot of data from prospective, large-sample studies about longer-term outcomes in Syringomyelia in the CKCS. While Riley's condition is likely to deteriorate over the course of his life there is no such guarantee. Ultimately, surgery to decompress is still evolving and new additions and techniques continue to emerge. One of our hopes is that in forestalling surgical intervention we might buy Riley time until better surgical outcomes are established with newer procedures and techniques.

Another concern is that we have to live with the outcome of the decisions that we make. I know that might sound strange to say but having been through life-challenging illnesses with our other companion dogs (Blackie and Chandler) we know intimately the process that goes on in your mind after the fact. Even when you do everything "right" you still second guess yourself wondering if you "Could have done more?" or "Should I have done less?" Etc... One thing we knew for sure is that if Riley went immediately for surgery and had an undesirable outcome, we would forever be chastising ourselves for not having attempted less invasive/medical treatments, first. So with that information, we decided as a family to follow Dr. Rusbridge's protocol for treatment of SM with medication.

The first thing we noticed was that Riley was on a very low dose for Omeprazole. At that point, he had been on it for just over a week and he was still in discomfort and having yelping "episodes". We decided to take him to the higher-range of the dose for Omeperazole and doubled his 5 mg evening (once a day) dose to 10 mg. Within 24 hrs, his yelping calmed down and he began to start running around the house and acting more spry and puppy-like. It was almost too good to be true.

We left for Las Vegas for Michael's birthday weekend just 2 days into the new dose. Our dog-sitter was well briefed on what to do and what to expect. We were actually looking forward to being away so that we could see if there was a notable difference 5 days later when we came home. And what a difference there was! Our dog-sitter reported just one middle-of-the-night yelping "episode" which was quick but with no other issues and no apparent distress. We came home 5 days ago and it's been 5 yelp-free days. As we expect, he continues to scratch and do the "air guitar" though it is markedly decreased. Riley is chewing his toys, running around the yard (and our living room) chasing Phoebe, kissing, snuggling and being very delicious and puppy-like, again. He's in no apparent distress and he's just the happy ol' Riley that we know and love.

We know that there is a very good chance that this might not last. It might not last for long, even. But he's doing well now. He's had a few good days. Today was a great day. And, if at some point we have a run of bad days, then we'll follow the protocol and switch him to Cimetidine, and if that's not working, Neurontin, or Lyrica, etc... and if the day comes that we can't manage him with medication, then we'll find out what the best information on surgery is.

In the end, we're all learning about this as we go along and just doing the best we can. One major "life lesson" we learned last year when we suddenly found ourselves facing a diagnosis of severe and aggressive malignant melanoma in our dog Chandler, was that you take every day, one day at a time. You know that awful dreaded day will come at some point. But, as cliche as it sounds, none of us know how long we have here. None of us know what's in store for us tomorrow or the day after. All we can do is give gratitude that today, the Omeprazole seems to be working and Riley isn't in pain.

Today is a good day.

Monday, March 1, 2010

Dr. Rusbridge has Spoken

Below is the Report from Dr. Rusbridge. She makes very specific recommendations with regard to medical management as well as surgical management, though she stops short of making a recommendation/ endorsement of one route over the other.

Below her report followed by our response to her, followed by her reply back to us. Clearly we have a lot to think over.

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28th February 2010

Dear Dr Ciment

Re: Riley, a 18mth male Cavalier King Charles Spaniel

Thank you for sending me Riley’s medical notes and MRI scan. I was sorry to hear of the difficulties you
have been experiencing with him.

History

Riley was diagnosed with syringomyelia secondary to a Chiari-like malformation on 9th February 2010. You were prompted to obtain an MRI scan after a weekend where Riley had multiple episodes of postural pain. Riley is also having distressing episodes of pain which appear to wake him from sleep and is more tentative about going up and down stairs. Riley has displayed sensitivity to having his ears groomed on the right and also has the characteristic phantom scratching at the right side. However, despite having episodes of acute pain, Riley still has a good exercise tolerance and shortly after these episodes of vocalisation he is willing play with the other dog. The main questions are how much discomfort is he in and whether or not he should be medically or surgically managed.

MRI images

MRI scans of the brain and upper cervical spinal cord are available and they reveal a Chiari-like
malformation (occiptal bone hyperplasia/COMS) with secondary ventricular dilation and extensive
syringomyelia. The syringomyelia extends from C1 to the caudal extent of scanning (C6); it is likely to
extend further down the spinal cord. The maximum measured diameter was 7-8mms. There was
involvement of both spinal cord dorsal horns, but in keeping with the clinical signs, there is more damage on the right.


The phantom scratching behaviour is thought to be part of a neuropathic pain syndrome
relating to a disorder of processing through the spinal cord dorsal horn and generally
signs of scratching are seen with syrinxes which are greater than 5mms. There is
ventriculomegaly of all the ventricles and a small quadrigeminal cyst extending from the
4th ventricle. There is no middle ear disease.

Management

You are right in your assessment that the main treatment objective is pain relief. It is difficult to know what these dogs are feeling. Using my own pain score, I would give him a score of 3 where 0 is normal and 4 are dogs with compromised activity. I enclose details of this pain score (below). Regarding the decision of whether or not to have surgery, this is quite a difficult choice because surgical management at best improves the situation but does not cure it. In Chiari malformation and syringomyelia in humans a foramen magnum decompression will result in syrinx collapse and an improvement of clinical signs in
approximately 80% of cases. The improvement in clinical signs is mostly due to improved cerebrospinal
fluid (CSF) flow through the foramen magnum and patients may still have issues due to the syringomyelia.

In dogs, all of the long-term follow-up studies that I have seen, including my own, have also found an ~ 80% success rate in improvement of clinical signs. However, follow up MRI scans have shown that the syrinx has not collapsed. Also I have found that there is a high recurrence rate. I found that 45% of my cases still had a good quality of life two years post-operatively, however many of these dogs still had signs relating to the syringomyelia and ultimately many dogs will eventually deteriorate - this can be from as early as two months post-operatively. Therefore my general advice is that surgery is the best option if medical management is not sufficient in controlling the dog’s signs of pain or neurological deficits. However, surgery is unlikely to be a lasting solution and medical management may still be required.

However, in Riley’s case you also have to take into consideration his young age and quite severe disease. There is a high chance that his clincial signs will progress and there is an argument that surgery may slow that deterioration. If I was operating on Riley I would seriously consider doing an additional procedure (e.g. interventricular shunt) in conjunction with a foramen magnum decompression, just because I know from experience that a foramen magnum decompression is unlikely to give Riley a lasting quality of life. However I have no experience (yet) whether this will lower the failure rate.

If you decide to opt for medical management in the short or long term then I would advise a combination of a drug which would reduce CSF production in addition to a drug which relieves neuropathic pain and has a site of action at the dorsal horn. Examples of drugs which reduce CSF production are the antacids omeprazole and cimetidine and a diuretics such as furosemide. The drug I more commonly use, although there is no scientific rationale for it (yet), is cimetidine which is dosed at 5-6mg/kg three times daily. I tend to use this drug over omeprazole purely because it is licensed for dogs in the UK (although not for CSF reduction). The dose of omeprazole is 0.5-1.5mg/kg once daily. You don’t give Riley’s weight, but assuming he is approximately 7kg then a 5mg once daily dose of omeprazole seems an appropriate starting dose. If omeprazole has not altered Riley’s signs then you might want to try cimetidine as an alternative otherwise it would suggest either these drugs are ineffective or that a neuropathic analgesic should be given in addition.

The neuropathic analgesic I use most commonly is gabapentin dosed at 10-20mg/kg two to three times daily. I generally start at the lower dose and build up. Pregabalin (Lyrica) can be a better choice for some dogs as it can be a more effective pain relief for syringomyelia with less sedation; however, its considerable downside is that it is more expensive. The dose rate is 5mg/kg twice daily; both pregabalin and gabapentin are unlicensed for treating canine diseases, i.e. there are no published clinical trials although have been used for many years by many clinicians. Potentially they can cause drowsiness and are metabolised through the liver, so that I recommend that liver function is monitored on a six to twelve monthly basis. For some dogs I add a non-steroidal anti-inflammatory drug (NSAID) in addition to the other drugs. I do not have a particular preference at this time, although I am currently evaluating a long-acting drug called Trocoxil and I am quite happy with the early results. I am not sure if this drug is available in the USA. Otherwise any NSAID can be tried; the response to NSAIDs, as you know, can be quite individualistic. Some veterinary neurologists recommend corticosteroids for treating this disease; I tend to use them as the last option because of their long term side-effects, especially in younger dogs. They can be the most effective drug if the dog does have neurological deficits, for example weakness or ataxia (wobbliness).

In summary, I do think from your description that Riley is in pain, however this pain appears to be short
lived and probably relating to impedance of CSF flow through the foramen magnum. Sensitivity to touch
and the scratching is most likely related to the syringomyelia and this sensitivity may become more apparent with time. In my opinion it is worth at least giving a trial of analgesics because it is difficult to know what these dogs are experiencing on a day to day basis and an improvement in demeanour, exercise ability, etc. would suggest that the dog was uncomfortable. If Riley is unchanged or is very sedated then there is an argument for not using these drugs.

With regard to surgery, there is no easy answer. It is likely that if surgery is performed now that the episodes of screaming, etc. will improve. The scratching will continue but may be improved. However, there is a chance that all of the signs will recur before Riley is a middle-aged dog. However, at that time he may be successfully managed medically. The converse argument is that if the signs could be successfully managed medically, is it better to hold off surgery until medical management is no longer successful. I am afraid I do not have that answer to this dilemma which is part of the reason why I work so hard trying to prevent this disease and develop a better surgery/medical management!

I hope that these notes are helpful. Please email me to clarify any further details. I am away from the 5th to the 13th of March.

Yours sincerely,

Clare Rusbridge BVMS PhD DipECVN MRCVS
RCVS and European Specialist in Veterinary Neurology

Stone Lion Veterinary Centre, 41 High Street, Wimbledon, SW19 SAU
Fax 020 8944 0871 (SLVC) 020 8786 0525 (private)
Email: neuro.vet@btinternet.com
http://www.veterinary-neurologist.co.uk/
for information on syringomyelia, FOPS, Lafora’s, epilepsy, spinal disease and more

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Dr. Rusbridge.

Thank you so much for your thoughtful and detailed report. We have have reviewed it twice and just two questions for you.

When we spoke with Dr. Berg, he made the analogy of Syringomyelia to someone who is squeezing a marshmallow. His point was that as long as the Chiari Malformation continues to exert pressure on the Cerebellum and the syrinxes and hydrocephalus persists, there is "pressure". He suggested that if a person were to pinch/squeeze a marshmallow for just a few minutes and then release the pressure, the marshmallow would revert to a more full state. Perhaps it would not revert fully to it's original state, but mostly. Compare that to someone who pinches a marshmallow for hours and hours on end. It is very unlikely that when they release the pressure that the marshmallow would puff up at all. His analogy suggests that the longer we allow the syrinxes to increase pressure in the CSF, the great a risk of permanent nerve damage that would be sustained even after surgery. He stated that he was advocating for surgical management because the sooner we did the FMD, the sooner the pressure would be off the nerves and the less likely that Riley would sustain longer-term nerve damage. Is there, then, an argument that to preserve the integrity of the nerve tissues, the sooner we decompress, the more tissue is salvaged? Clearly, Pregabalin and Gabapentin work to alleviate neurologic symptoms but do nothing to address the longer-term, mechanical injury to the nerves/spinal cord from longer-term compression. Would we be "losing time" and risking further (permanent) nerve damage by exploring medication options which may alleviate symptoms while not addressing the underlying pathology?

With regard to a surgical recommendation, you suggested placing an inter-ventricular shunt in addition to performing an FMD alone. When I asked about this procedure at our original consultation, Dr. Berg recommended against it. He said that he has seen longer-term issues with the shunt not being well tolerated. He said that he's noted it moving around/ slipping, irritating the dog in the longer term. His concern is that an active puppy who will be again running, jumping, etc... is not a great candidate for such a shunt placement. I wondered if you had different longer-term results with your I-V shunts than he? As a surgeon myself, I understand that we all have procedures we're better at than others. Perhaps an I-V Shunt is a procedure that Dr. Berg is not as comfortable with and therefore his outcomes are different? I don't know. Should we decide to follow your surgical recommendation, who would you recommend we see for this kind procedure (i.e., the FMD in combination with the I-V Shunt)? We are willing to travel quite a ways for this, if need be. Certainly within the United States (I'm assuming we couldn't get Riley to the UK for surgery with you due to quarantine laws and the amount of time he would need to be in recovery before taking a long flight home.)

Ethan Ciment
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Yes - there is an argument that the sooner the surgery is performed then the less chance of permanent damage. This makes sense as an argument - I am not sure that is being borne out in clinical practice but this "lack of being sure" is because of the absence of hard data not bad experience.

With regard to the other surgical options - yes shunts are prone to failure and complications. The other problem is that I cannot give you the direct benefit of experience because I have not done this combined procedure yet - which is why I said I would consider it. There are 2 cases similar to Riley that I have ongoing at the moment which I am also considering it for hence my choice of words in the report- the owners have yet to decide. There is a problem being the first and also doing something new when you have already got an established technique. The other option - and probably safer is to do the first surgery and if there is not a substantial improvement then add a shunt.

hope that helps

Dr Clare Rusbridge
http://www.veterinary-neurologist.co.uk/
for info on syringomyelia, FOPS, Lafora's
disease, epilepsy, spinal disease and more

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.

A PICTURE SAYS A THOUSAND WORDS:

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: