Riley, at 1 month on Steroids

It's been 4 weeks since our vet visit where we started Riley on Prednisone (5 mg, every 12 hours).  He started eating within 36 hours of the medication and, basically, hasn't stopped since.  His appetite is voracious.  He pees a lot, but he's adjusted to this very well and, such a good boy that he is, there have been no accidents in the house!

Also, we started him on antibiotic therapy for Helicobacter, just in case that has been playing a role.  Dr. Mlekoday wanted him to start when he was more than 3 weeks into the steroids and tolerating them well, so we started them this week, and he's tolerating those well, too.

Anyway, today's visit was with Dr. Chad West at Animal Medical Center.  He is Riley's new Neurologist.  Such a rock star, that guy!  Amazing mind and a kind personality.  Riley really likes him, and so do we.  This is the gist of things, and I'm quoting from his follow up email/consultation note to Riley's family vet (Dr. Mlekoday):

"Riley looks great and has been doing well at home. He seems to be handling the steroids fairly well; although, he has developed muscle wasting in his abdomen and along his spine. 

We discussed the typically recommended steroid tapering course and decided to initiate the taper at this time. I discussed that the taper will be gradual and unless there is recurrence, will follow this course:
Prednisone 2.5 mg po bid X 4 weeks,
Then 2.5 mg po Q24 X 4 weeks, 
Then 2.5 mg Q48 for 4 weeks. Then discontinue. 

I recommended blood work be checked in 6 weeks to include cbc/chem panel/cpli, +/- gi panel. This should serve as a baseline to see how Riley handles the continued taper."

So, as you can see, we're going to be doing steroids through the end of January.  Not bad, so long as Riley stays hungry and asymptomatic. 

I asked Dr. West his frank opinion about all of the pancreatic markers, the concerns about Riley's diet, the possiblity of this being chronic pancreatitis vs. helicobacter, etc... and he said that he really thought those were all incidental to the central issue, being the immune-mediated inflammation in his brain.  He he said that the cpli and gi panel are really sensitive tests and they often pick up on minute changes that, while clinically in the "abnormal" range, really tend to be in the "it's fine, leave it alone" range for that individual.  His hope is that when we repeat bloodwork in 6 weeks, either things will be different and back to normal or else we'll come to realize (if there is little change from the other numbers) that this is just Riley's "normal".  He said that, while it's possible that as we start to taper, Riley will start to lose his appetite again, it's incredibly unlikely and he'd be totally surprised if that happened.

In any case, today was a great day.  And as I type this, Riley is ravenously and enthusiastically chomping down on his plant-based dinner downstairs.  It feels like all is well in the world, again.  

Here's hoping...

What We Know Now....

It's been an intense past 24 hours and I'm overtired. I've also got a full schedule of patients to see, myself, today. I'm trying to bang this out in my one break in my schedule today because, by the time I'm done and back home tonight there's no way I will be able to write coherently. 

I also want to say, from the get-go, Michael and I both appreciate the barrage of calls, text-messages, emails, Facebook-comments, etc... and we're sorry that we just don't have it in us right now to respond to everyone individually. Please, please, please; understand it's not anything personal. There's also the fact that repeating the same information over and over from person to person makes it feel incredibly awful for us. That's one of the reasons we have this blog. We can put the info here and it can save us the upset of discussing and re-discussing the latest on Riley. Which brings me to that: Riley had a bunch of tests yesterday and we have a lot of information. 

 This is the order in which the information came to us: 

RILEY'S EGD (Esophagogastroduodenoscopy): For the most part, it was pretty normal. Abnormal findings were some grass in his belly at the pylorus (opening to the duodenum (small intestine) from the stomach. Also, there was a single piece of paper towel sitting in the pylorus and across it into the duodenum. It was adherent to the lumen-- as though it was pasted onto the lining of the "tube" of his small intestine. It wasn't causing a blockage-- clearly, food has been passing through just fine. But this was an odd finding.  We're not sure how he got the paper towel, though we know he was eating grass.  Lots of dogs eat grass when their tummy hurts.  All of this said:  The lining of his esophagus, the sphincters on the stomach, the lining of the stomach and the duodenum were all very normal.  No masses.  No ulcers.  They still took some biopsies just to look at and make sure they are as histologically normal as they appear.  

RILEY'S MRI:  Riley had a second MRI of his brain and cervical spinal cord.  As you recall, his first one was back in February 2010.  There is a LOT going on so, item by item:

* There is Otitis in his Right Ear; inflammation of the inner ear. (in the above picture, the arrow is on that area.) Unlike in humans, the shape of the ear canal and the underlying anatomy of the ear make it very difficult to assess the presence of deep, inner-ear inflammation or infection in dogs on routine physical exam.  

* There is a region of lower-grade inflammation in the medulla adjacent to the region with Otitis.  (in the picture above, this is the grey-ish area, to the right of the cursor.  Note:  the opposite side of the brain, which should be a mirror image, is not grey.)

* The large Syrinx remains.  When compared to the size/length/thickness on the previous films, there is no significant change, but it's still huge. (In the above picture, it's the long, white horizontal "cord" running from the base of his brain stem on the left into his neck.  The tiny black line at the bottom of it is his actual spinal cord.  The cord is compressed by the large, white Syinx.)  As Riley's Neurologist said, "It's probably the biggest syrinx that I've ever personally imaged on a patient."  Not a distinction one wants to have.  

* There is increased fluid in the fourth ventricle as compared to his prior films.  This, in turn, is putting pressure against the Cerebellum, and squeezing it in the skull.

Okay-- in  plain English?  The last two findings basically show a relatively stable case of Caudal occipital malformation syndrome (COMS)/Syringomyelia in a Cavalier King Charles Spaniel.  

The prior two findings, however, point towards the events of this past summer, when we thought he might have a deep penetrating inner ear infection.  The  inner ear "lights up" on the MRI on the right whereas it does NOT on the left.  The fact that the adjacent area in the brain (the Medulla) is lighting up, too, would hint at a possible local-spread or Meningitis.  Based on this, they wanted to do a Spinal Tap.  We said yes...

RILEY'S SPINAL TAP:  So ,technically speaking, they didn't do a spinal tap.  They did a Cisternal Puncture, which is a procedure where they pass a hollow needle through the posterior atlanto-occipital membrane into the cerebellomedullary cistern.  So they shaved the back of his head and "tapped" the cerebral spinal fluid from the area up high:

The rationale was that if there were anything infectious, our best chance of catching it would be right up in that local area, not all the way down in his lumbar spine.

They didn't expect this to be an infection- so this was mostly done to rule out that as a possibility.  

The CSF Analysis (results) came back overnight and are as follows:

These findings confirm that there is no infection. However, this also confirms that there is inflammatory disease in the presence of syringomyelia.   

The Neurologist was clear that he sees this very often in Cavalier King Charles Spaniels with a history of early-onset syringomyelia.  They can develop these autoimmune inflammatory processed in and around the structures of the syrinx.  Though there is very little by way of hard data in large sample prospective studies on the matter, he assured me that he is dealing with this all of the time and that steroids are usually the way to go.

They gave Riley a bolus injection of Solumedrol at a high dose (240 mg) and we are starting him up on 5 mg of Prednisone every 12 hours.  We will do this for the next 2-3 weeks before starting a very, very, very slow taper over a 2-3 months period.  The hope is that this will attack the inflammation and his neurologic reasons for inappetance will resolve.  

One potentially complicating factor in all of this is that steroids, on their own, increase thirst and hunger.  So it might be a bit difficult to figure out exactly why Riley is eating, if he does start eating again.  However, this should be readily apparent if he looses his appetite again when we start the taper.

Last night, when we came home from our 12 hours away from the house, Riley was fit to be tied.  He wanted nothing to do with any of us, much less his food.  He ate nothing.  He drank nothing.  He passed out and we were fine with that, because we know what he had been through.

I believe the steroids have kicked in because he woke me up crying at 3 AM to go outside and it was insane how much he urinated!  ....that's steroids for ya!  Still, he didn't want to eat his breakfast so we made the "paste" and "force fed" him again.  I am wondering if  his persistent inappetance this morning is in some way related to his being scoped/biopsied yesterday? Riley's Neurologist, in an an email  just a few minutes ago, said, "I cannot comment on scoping and its effects on anorexia, but I wouldn't be too discouraged yet... [the results]  do confirm inflammatory disease even in the face of SM, so I am very optimistic that the steroids will improve things."

Stay tuned...

Riley's Great Ordeal of 2012

It's been too long since my last blog entry and I have been remiss in following up- so I'm sorry. Perhaps it's been avoidance on my part? I guess I've been hoping that this was not something clinically significant enough to warrant a blog entry. At this point, it's beyond obvious that it is. Nearly 3 months ago, the week of July 4th, Riley started looking funny. By that, I mean that he didn't look like his normal self. He was mouth-breathing in a sort of a low-grade pant, and he was keeping his mouth ajar. All of the time. Whenever we would lean in for a kiss or try to touch or rub his face, he would push the right side if his face and his right ear into us. It was almost as if he wanted to scratch an itch or something. After a few days of this, we had enough (because he had enough) and we went to our vet, Dr. Mlekoday. She did some tests and, initially, found that he wasn't producing tears in his right eye and diagnosed him with keratoconjunctivitis sicca. We started him on lubricating eye drops and cyclosporine ointment. This reversed things pretty quickly and within a few days Riley was producing a normal amount of tears again. But his face rubbing, scratching and his mouth-breathing was still present, so we went back to Dr. Mlekoday and she did more blood tests. While almost entirely normal, one test came back positive titer for Rocky Mountain Spotted Fever. She immediately started him on two antibiotics: Baytril and Doxycycline. Riley was a trooper and he handled these meds very well without any apparent side effects. His mouth-breathing and the face-pushing stopped after just a few days. Dr. Mlekoday said that, just in case there were a deeper inner ear infection, we keep him on the Baytril for six weeks to be able to get a concentration that will penetrate to bone in the ear. In retrospect, I now realize that it was just days after stopping the antibiotics that Riley started to develop anorexia. Originally, this was a mild inappetance. We would feed him his usual dry kibble and he'd walk away from it. Michael would try and hand-feed him. Sometimes he'd take it, other times he wouldn't. Eventually, the times he wouldn't take the dry food became too frequent and so we tried giving him the same food but in their wet canned food formula. Initially, Riley loved this. However, it wasn't long before he started rejecting this, too. We tried doctoring up his food: We'd make combinations of dry and wet food, sometimes with some baby food (which he loves) and sometimes with homemade tomato sauce (also something he loves). In the end, he just stopped eating. I think we were in denial. Meal after meal, Riley would eat maybe a quarter of his food or none of his food at all. He would skip his breakfast and we would think, "That's okay, he'll eat at dinner." But then he'd only eat a teaspoon of food at dinner. This pattern went on for a few weeks while we were focused more in our other dog, Phoebe, who tore her ACL. Thats not an excuse: its an explanation. We were treating her non-surgically, and that involved 24/7 monitoring of her to keep her from walking and jumping. Though we were very successful with her and she has since recovered beautifully, it resulted in an unfortunate and unintentional ignoring of Riley's worsening anorexia. I don't know how I'll ever be able to forgive myself for that. Anyway, it was about three weeks ago when we realized that Riley hadn't had a full meal- breakfast or dinner- for weeks. His spine was suddenly palpable and visible through is thick coat. His ribs were more prominent. His harness was so loose I could fit four fingers through it. Now, Riley was a bit overweight and could have stood to loose a few pounds, but this was too much, too fast. In fact, at the next vet visit, it was clear that he went from 20 lbs to 18 lbs in just a few weeks. I know that sounds minuscule, but that's 10% of his body weight which is considered clinically significant. We went back to Dr. Mlekoday. This time, she did an abdominal ultrasound, an X-ray and every blood test under the sun. The x-ray showed him to have an abnormally small liver, though his blood work showed normal liver levels. On ultrasound, there were some "cysts" in his spleen, though these were not hyperechoic (i.e., they didn't light up) so they're probably old and reactive from some old infection or injury, a long while ago. There were no signs of any infection or disease. The only other "positive" findings were an abnormal cobalamin level and and increased PLI. Dr. Mlekoday felt that this was consistent with a malabsorption or malnutrition issue, which made sense since he wasn't eating. She wanted to repeat a bile acid challenge test, just to rule out any issue with his bile levels so we went back for that, and that was negative, too. But Riley wasn't eating. AT ALL. We started to get panicked. So we started grinding up his dry kibble into a fine powder in a spice grinder. Then we'd add water, turn it into a paste and force-feed him. The interesting thing was, he never fought us. He would lick his lips, as to indicatemthat he was hungry, but he wouldn't take the food from our hands, the bowl, the floor. The only way for him to eat was if we'd pry his mouth open, put a ball of the food on his tongue and then close his mouth. He'd swallow the food, but wouldn't really chew it. At this point, Dr. Mlekoday asked us to go back to Dr. Jason Berg who is the Neurologist who is an expert in Syringomyelia. Since it was 2 1/2 years since he last examined Riley, she wanted him to rule in or out the possibility that all of these symptoms could be neurological and come from his Syringomyelia. We went up immediately. Dr. Berg did a quick and minimal exam which we found to be somewhat upsetting, but he was adamant that this was not Riley's Syringomyelia getting worse. He said, "If this were my dog, I'd scope him. It seems like its G.I." He put Riley on Cerenia and said that if this didn't suppress nausea or induce eating in a few days, to get off the Cerenia and get him to a G.I. specialist. After 5 days, Riley was still not eating on his own and we were force feeding him. At Dr. Mlekoday's insistence, we stopped the Cerenia and made an appointment with Dr. Doug Palma, an Internal Medicine Specialist at The Animal Medical Center in Manhattan. She felt that a new set of eyes and ears on this case may bring about a different thought about what's going on. Last Thursday, we brought Riley to Dr. Palma. I really like Dr. Palma. He is a doctor's doctor. Very thorough. Very methodical. He spent over 30 minutes examining Riley and then he called us back to talk. He said that, on clinical exam, this is not a dog who is manifesting signs and symptoms of any obvious GI disorder. He said, "my clinical gut instincts are that this isn't G.I." I like a doctor who listens to his gut. That's a big part of my clinical judgement and decision making process as a doctor, myself. He continued to say that there were obvious and reproducible pain responses on physical exam which were suggestive of advancing Syringomyelia. He was clear to acknowledge that he had just seen a neurologist and didn't want to disagree with him, but his clinical index of suspicion was very high that this was his Syringomyelia. He called his colleague, Dr. McCue who is a Neurologist at The Animal Medical Center, to come in and do an impromptu examination on Riley. After that exam, he was very clear that we should treat this as Syringomyelia-pain related first. As he said, "We can always go back and scope him if we need to." So that's what we did. On their instructions, we brought Riley home and increased his 10 mg of Omeperazole dose from just once in the evening to every 12 hours (we doubled the dose. ) Again, as a reminder, Omeperazole is used in Syringomyelia in dogs to lower cerebrospinal fluid levels so that the brain swelling (that causes pain) is reduced. They also added Gabapentin (Neurontin) and wanted to see if this worked to resolve his pain and get him back to eating. The rationale behind this is that, since dogs who are in significant pain stop eating, if the Neurontin knocked out his pain, then he'd eat. After one day on the Neurontin, Riley was "stoned"-- his gait was ataxic, beheld his head lowly, and he was (generally) out of it. However, for the first time in over two and a half years, his "hot spots" were shut off! I could touch his chest, his right side if his neck, I could rub him there and he didn't scratch! It was shocking. Even more shocking is that, lat Friday night, we put his dry kibble in is bowl and he put his head in the bowl and ate his dinner! We were so excited that this nightmare might finally be ending, with an answer as to why he wasn't eating. That hope was short lived. Riley never ate again without being force fed. Dr. Palma was so generous with us, keeping in touch with us daily- sometimes many times a day- via email. Two days ago, we agreed that the Neurontin wasn't really "working" for Riley; It's just making him sedated but his scratching/Syringomyelia symptoms are back and he's still not eating. Clearly, this isn't Syringomyelia. At least, not entirely. We woke up early this morning and, with a fasting Riley, came into the Animal Medical Center. We met with Dr. Palma before saying goodbye to Riley. The plan is to start with the upper endoscopy to look in his mouth and throat, down his esophagus, into his stomach and small bowl to look for any potential issues, like ulcers, masses/tumors, irregularities, etc... Then, while still under anesthesia, they'll take him up to the radiology suite and do an MRI of his brain and spinal cord, to check on his syrinx. They'll also be looking at his ear and the whole right-side of his neck. If there is any reason to suspect meningitis (which is not uncommon in these cases) they'll then do a lumbar puncture and cis-puncture (spinal taps). It's been two and a half hours and he's still upstairs. We're waiting for an update and still holding good thoughts. More to come....