All right, guys, so we're gonna get started. Thanks, Mike. My name is Zach Bauhardt. I'm a physiatrist in the Boston area. This is a presentation that we're giving today on Baclofen pump troubleshooting 101, and I guess 102 and 103 and so forth. I am really delighted and honored to have with me two really tremendous leaders in our small but mighty field, Dr. Kimberly Heckert. And you can see her credentials right there and also Dr. Michael Salino. I've known both of them for several years now, and they are really tremendous in what they do. So it's really wonderful for having them with us. So thank you to both of you. So first, I'm just gonna give my own personal disclosures. I am on the Speakers Bureau for AbbVie, also the advisory board, and I've done some speaking also for Medtronic and Pyramidal Critical Care, which makes Gaplofen. You can see Dr. Heckert's right there, and she will give hers when she starts giving her talk, and Dr. Salino will discuss his as well. So first, I'm just gonna go over the goals and objectives, and then Dr. Heckert is gonna give her lecture just to kind of align with the goals and objectives. And then Dr. Michael Salino has some really tremendous images that he's gonna share with us as well to kind of bring it on home. So first, we're going to discuss how to approach a patient with a baclofen pump experiencing a loss of efficacy in the outpatient setting. And you know, this stuff isn't overly complex, but you have to know how to do it. And Dr. Heckert was just saying that this is really one of the big reasons why a lot of people don't go into the field of intrathecal baclofen pump management, because there's kind of confusion about how to really approach these patients and how to approach these systems and these situations. And we tend to think it's a lot more difficult than it is, or sometimes we don't really pay baclofen withdrawal quite the respect that it's due. So having Dr. Heckert and Dr. Salino here kind of going over this will really be very helpful and hopefully help raise the bar for all of us who are attending today and are really interested in this patient population that by the way, is really undertreated, really does need our help, and physiatrists are better equipped than really any field out there to really take care of this patient population. So it's something that our field can really help with dramatically. And I also want to thank AAPNR for sponsoring this and for putting together. The second part of our talk, the goals and objectives are to understand the approaches to pump malfunction in the ED and really the importance of developing an institutional protocol. And that's more what I'm going to talk about. Dr. Heckert is going to be talking about the first goal and objective. And then the third one is going to be Dr. Michael Salino discussing diagnostic imaging techniques. And there's several to assess for baclofen pump catheter malfunction. And I know personally that he has a lot of really, really cool slides that you're going to enjoy looking at. So I'm going to close mine out for now and I'll let Dr. Heckert take over. So I'm going to stop sharing. Okay, great. Can everyone see my slides now? Yes. All right, let me put us into presentation mode. All righty. So thank you so much for the warm introduction. Thank you all for being here. I'm Kimberly Heckert, and I am the director of the Spasticity Management Fellowship at Jefferson, which is in Philadelphia. And we are beginning our, we're in our sixth year, we're soon to take our seventh fellow, and it's been a wonderful ride. So I'm going to go ahead and turn it over to Dr. Heckert to provide some of the slides. Here are my disclosures, as were mentioned before, I've been a speaker and a consultant and been on advisory boards for some of the toxin companies. And also while not directly benefiting me, my fellowship program has received support from the pharma companies that are listed here. Okay. So the piece of this presentation that I'm going to cover is loss of efficacy that we would manage in an outpatient setting. I'll discuss what a presentation of this may look like and potential etiologies, beginnings of the workup and some mitigation strategies that you might use along the way while you're trying to figure it out. And then also, if there's time, I'm going to present a case that will highlight this type of workup and management. And I'm going to set a little timer to make sure I don't go over. All right, as we know, intrathecal baclofen can be a very powerful tool for relief of problematic spasticity. And the great thing is that patients can enjoy the spasticity relief without the side effects of oral baclofen. Now, what dose will give a patient great control and relief? Well, that may vary based on a number of considerations, such as the severity of the spasticity, the body areas and total body areas involved and the disease etiology. Now, friends of mine published a study back in 2015 and showing the total daily dose of baclofen and how there were some trends that they observed based on etiology where patients who had an underlying diagnosis of brain injury or CP or SCI tended to need higher total daily doses than patients with MS or stroke who tended to be a little bit more sensitive. And I would say that while there can be some outliers, I have found this to be similar to my practice as well. But once we do find that sweet spot with a total daily dose, for the most part, we can expect that that is going to be relatively stable. Now, some exceptions will occur. These would primarily be in the cases of progressive disease. So something like MS, where we might expect the patient to worsen, their spasticity could also worsen. Growth of a patient, if a pump is placed in childhood and the patient grows and their muscles grow, we might anticipate their dose could increase over time or development of a painful condition. So we know a lot of these diseases can cause problems such as heterotopic ossification, or our patients could get regular run-of-the-mill arthritis or they might develop wounds. Any of these things can increase spasticity and potentially affect the dose that will keep them stable. But for those who have enjoyed good spasticity relief with ITB, a loss of efficacy can be very concerning and it can be complexing to the physician as well as the patient. So what might we consider if we encounter a loss of efficacy? Perhaps there is a failure somewhere in the pump catheter system. That could be a problem with the pump itself, the motor or other hardware, which is fairly rare. More commonly, it could present as a problem with the catheter, a leak, a disruption, a kink, or a problem that results as a programming or drug formulation error. As mentioned, it could also be the disease state worsens or somehow there's a noxious stimulus. So upon presentation, you might be lucky and this patient could be your patient that you've been following for a long time. But it might be like me and you might have patients present to you for this problem from other physiatrists. That can be one of the reasons I'm sometimes consulted. So perhaps the patient is presenting with spasticity that is not as well controlled as they had previously been. So in this case, you're going to have to take a very careful history to understand the onset, the duration, and the severity of the loss of efficacy. So if you don't know that patient well, you might have to ask, when was the last time that your spasticity was well controlled? And then you will need to ask quickly if there are any symptoms of withdrawal. And these could be mild symptoms of withdrawal. They could be intermittent. You want to make a determination about whether or not they are progressive. And what you are doing in this line of questioning is really making an assessment about whether this workup should be undertaken on a more urgent basis versus whether you can continue this workup in the office. So have this patient's dose been kind of slowly titrated for a long time? That might give you a different understanding than per se if it were sudden. Could a noxious stimulus be causing a worsening of spasticity? We would want to ask about constipation, bladder problems, infections, wounds, arthritis, or joint problems. Or was it more sudden? Did it correspond with a recent refill? Could it be attributed to a concentration or programming error? Was there a history of trauma that might lead us to think about a catheter disruption or kink? And I'll mention also to ask, when was the last pump or catheter replacement? I have seen a couple of cases in recent years where a problem occurs after a replacement in the area of the pump catheter connection, where that first connection is around what I call the nozzle and the catheter tubing. Sometimes shear can occur there or a disruption could occur there. So it might be important to ask if the change corresponded with a pump replacement or catheter revision. You will want to do a good physical exam as your next step. So anyone who presents to my clinic with a concern for loss of efficacy is going to get totally naked. And my fellows can attest to this. They're going to be transferred out of the wheelchair or onto the table. And we are looking at the whole body to do a skin assessment, to assess their tone and to interrogate the pump. So what are we looking for when we interrogate the pump? A number of things. But you're going to be hearing from Mike Salino soon. And he is a person who taught me, I would say almost everything that I know about pumps. And one thing I'm very grateful for is very early on, he taught me to check for logs every single time of a pump refill. So most of the time we're looking for motor stalls in logs or some sort of critical error. And typically I would have thought about that with an MRI. Magnetism is one of the main things that can cause a motor stall. But interestingly, more recently, even years after he taught me this, we are seeing that some of the newer Apple devices can also cause motor stalls. And this has been reported in a couple of states. So things like the newest model of iPhone or iPad Pros and MacBooks can stall a pump if they're placed over the pump. So this would be another reason why it's a very good idea to look for motor stalls and check your logs each and every time you interrogate a pump. That is an extra step in the interrogation of the pump, but you should do it. And then again, we're looking at the records to look at the dose over time. Okay, some steps in initial management you might want to think about. Consider checking the actual reservoir volume and comparing it to the expected reservoir volume, the same way that you would in a pump refill. Or better yet, if you think there's a problem with the concentration, just take it out and refill it with the medicine that it should be, just perform a refill. The other thing you can do is go to the closet and grab one of those catheter access port kits and aspirate from the catheter access port. A reminder here, there's a one-way valve so that when you do aspirate from the catheter access port, you are aspirating contents of the catheter and not the reservoir. And if you take enough of that fluid, you'll get CSF. So if you don't get fluid at that point, really you're going to gear your next assessment more toward a nuclear scintigraphy study, which Dr. Salino will show you a little bit about that in a few minutes. And that is because you really cannot put dye in a catheter at that point. If you do get fluid, if the fluid is positive, you can send that aspirate for beta-2-transferrin PCR. And beta-2-transferrin is a protein that is found almost exclusively in the CSF and would indicate that your aspirate does contain CSF. Afterward, if the fluid is positive, you would program a priming bolus. And then another consideration you might do at that first visit is just program a bolus with the pump. You would want to consider the timing of that appointment and the total dose so that your patient who maybe drove in to see you that day is going to get home safely. You want to make sure that there's enough time that you can monitor the patient and see their response and that you're not giving a dose that is going to be too much for them. Another initial management step could be changing the concentration of the baclofen that's in the pump. So if you did not change the overall dose at all, but you simply made the concentration more dilute, you have made a higher flow. You have caused the pump to distribute the medication at a higher flow rate. The other thing that you can do is change your settings to add bolus doses. So if the patient is on simple continuous, you can switch them to flex mode, or if they're already on flex mode, you can go ahead and increase those bolus doses. So there again, the shorter the duration of the bolus, the higher the flow. So these are some steps that can be taken to change the flow in the system. And why are you doing that? Well, again, if there's a leak or a partial obstruction, a higher flow may get a response where a lower flow does not. At this point, you can also consider some additional imaging. X-rays are very low-hanging fruit. You can get X-rays of the abdomen and the spine. Sometimes that will give you the answer. Kinking or a fracture or a disruption might be observed just on plain X-rays. Now the older catheters were a little more radiopaque, a little easier to see on X-rays. The newer ones, not as much, but you can sometimes still appreciate them on X-rays. So worth doing, because again, it's low-hanging fruit. A CT myelogram can be done where you're placing dye into the catheter, but again, only if you were able to aspirate the catheter contents. The reason that I say this is that if you tried to force dye through the catheter access port and did not first aspirate the contents of the catheter, you risk giving that patient a very large bolus. You can calculate exactly what that would be based on the length and the volume of the catheter. And the concentration of the drug. And that may not be an insignificant bolus dose. It could even potentially be life-threatening. So you really don't want to do that. As I mentioned, you could do nuclear scintigraphy. In this study, instead of placing dye in the catheter, you are placing tracer in the central reservoir and allowing the pump mechanism itself to continue. And then you're going to observe that tracer for 48 and 72 hours and follow where it goes. And then finally, sometimes even the imaging studies don't give us the answers and programming boluses don't give us the answers. So something that you could consider is another bolus via lumbar puncture, similar to what would have been done at the time of a pump trial. So you can compare a bolus delivered by lumbar puncture with a bolus delivered by the pump. And if there's a significant benefit from the LP versus the pump, that gives you a very strong indication that there's a problem in the pump catheter system. So again, friends of mine published this great study. It's actually a series of best practices for intrathecal baclofen troubleshooting. And this is published in Neuromodulation. And this is a diagram that is a bit of a review of some of the considerations that I just mentioned. So if you are interested in this work, this is going to be one of the series of articles that will be very, very useful for you. I won't say whether or not I have it on my phone for quick reference. Okay, so I'm seeing that we have 13 minutes here down. Dr. Bohart, should I continue with this case? Absolutely. Yeah, please do. Okay, this is a true story, a true Hollywood story of a real patient referred to me. He was a 40-year-old man with spastic paraplegia from a remote gunshot wound, referred to me by a good friend and colleague for loss of efficacy and worsening spasticity. I would say he had mild withdrawal symptoms. He had essentially just severe spasms. He did not have itching and he did not have irritability aside from, you know, being irritated at how bad his spasms were. And this was occurring in a setting of previously having enjoyed very, very good control with intrathecal baclofen. So the onset was six months prior, just after he was hospitalized for an episode of sepsis. And while he was hospitalized, he previously did clean intermittent cathing, but they had to catheterize him with a Foley, and that was traumatic, and that necessitated a temporary placement of a suprapubic tube. So fast forward, he gets out of the hospital, has eventually the suprapubic tube is removed, the urethra heals, and yet his spasticity is still worse than baseline. And so they begin escalating his doses and these are not helpful. So after a period of time, he is referred to me. At the time of referral to me, his dose was 300 micrograms per day, simple continuous 2000 mics per mil concentration. And on exam, I find him to be a motor complete paraplegic man with severe spasms that extend both of his knees and plantar flex his ankles with transfers. So he tries to transfer to the table and his legs go out even moving like supine to sit on the exam table, he goes into these marked spasms. And he requires now a transfer board, which he previously did not use. We did a full skin survey, we did not find skin breakdown, but we found an area of hypersensitivity around his scar from his suprapubic tube where every time we would sort of touch that area, it would also elicit these spasms. So I began to be a bit suspicious about that. Upon interrogation, there were no motor stalls. Upon meeting him that first visit, I did perform a catheter access port aspirate. The fluid was very easily aspirated, and it was positive for beta 2 transferrin. I went on to order x-rays of his abdomen and pelvis and I scheduled him for a CT myelogram and I ordered a urinalysis. The x-rays showed that he had developed some heterotopic ossification at both hips. There were no obvious catheter disruptions, we could see his catheter. And then I did the CT myelogram, it was normal, his UA was also normal. But an interesting finding was that both times I accessed the catheter access port, both in the office and then again in radiology for his CT myelogram, both of those aspirates resulted in a temporary improvement of his spasticity that lasted about six to eight hours associated with the priming bolus. Additional interventions, I took out all the 2,000 mics per mil, I compared the volumes, actual unexpected, there was no problem there. I refilled him with a 1,000 again to improve low. And then I put him on flex dosing. I gave him a 25 microgram bolus in the office, it provided temporary relief, so then I scheduled them in flex dosing. And then I began to increase his boluses up to 75 micrograms three times a day. And each time I went up, he would get a little bit of temporary relief, but then it just faded over time, then even my temporary improvements were not helping him. So okay, additionally, I went up to flex dosing, I had brought him all the way up to 600 micrograms a day on flex dosing, and nothing was really working. So ultimately, I did give him a bolus via lumbar puncture. And interestingly, he had a much better response with a lumbar puncture bolus compared to any bolus that I was giving him through the pump, and those those doses were higher. So then I made a referral to neurosurgery and began weaning him. And his pump was five years old at the time. So he went with a full pump replacement and catheter revision. The findings operatively were the catheter at the site of the pump connection was embedded in scar tissue. So there was a partial obstruction. And after his surgery, he required only 225 micrograms a day, simple continuous. To this day, he's now stable on this dose for more than 18 months. So my take home points from this case were that he had a normal catheter access port aspiration, and he had a normal CT myelogram, those do not always rule out a problem with the catheter, there can be a more subtle problem. And the other point here is that, you know, if I could say, you could argue, maybe he just had HO, and that's why he needed a higher dose, or there was this area of sensitivity around the suprapubic tube. But even with those two conditions, his dose should have stabilized, and it never stabilized, even if higher, it was always coming back to no control in a man who was previously very, very well controlled. I even sent him for PT, they did some desensitization around the scar, nothing really gave him good relief. So, you know, all these temporary relief, all these temporary measures did not help him and ultimately, the loss of efficacy progressed. So that's why I became convinced that even in the presence of these normal findings, there had to be a problem and the lumbar puncture bolus confirmed that for me and for the patient. So those are the take home points. Loss of efficacy deserves investigation, please do it, don't just keep escalating the dose, and a systematic approach can help you identify the problem. I'm going to save questions for now, we'll do those at the end, or you can feel free to email me and I'm going to turn it back over to Dr. Bohart. All right. Thank you, Dr. Hecker. That was fantastic. I really like it. I'm going to be a little bit of an emcee here. I like it how your studies were basically normal, yet obviously the situation was abnormal. And, I think one of the big lessons to really derive from this is that we have to listen to our patients. And if they're, if despite what we do, if we don't come up with an answer, we still have to explore. Yeah. And not just keep turning up and up and up, because I'm sure all of us who have been treating patients like this for a while, you sometimes get referred a patient who's at a really mega high dose of the back up and pump. And I think that basically what was done is that it just kept getting turned up and up and up without the proper investigation having been made. So that's excellent. Thank you. Let me share my screen. There we are. Okay. Okay, so Dr. Heckert already really went over a lot of what I like to talk about as well when I give these presentations, but I also want to talk about a few additional things, situations that might present in the ED, and I know that there's probably some residents here and some attendings who've been doing this for a while, probably maybe some med students who know, so I'm just going to really start at the more basic level and just kind of try to, you know, cover more basic stuff as well, but I just do want to emphasize that baclofen withdrawal is a very dangerous situation, really poses severe risks, it can be life threatening, I liken baclofen withdrawal, you know, to basically when I was an intern in Cambridge and And, you know, I had all these rule out MI cases and probably 90, 95% of them turned out not to be MIs, but we admitted all these patients just to make sure it wasn't MI because MI can be so catastrophic. And same thing with backlog withdrawal. You know, if you're in doubt, admit it, just admit to either your service or neurology or neurosurgery or medicine or however it works within your institution, just admit them and observe them if need be. Because the worst thing that we can do is give them oral baclofen and just send them home, you know, and then say, call us on Monday, because at that point, it really may escalate into really life-threatening backbone withdrawal. And that's obviously a situation that we want to avoid. Just to review the basics, the mild symptoms, increased tone, itchiness without a rash is really one of the kind of hallmark signs. People just feel a lot of itchiness, irritability, mood changes, and then severe backbone withdrawal can result in really severe rebound spasticity and hypertonicity, coma, seizure, altered mental status, hallucinations, hypertension, tachycardia, hyperthermia, and rhabdomyolysis. In my experience, mild backbone withdrawal can become severe really quite quickly. This isn't like a matter of, you know, days or weeks. I mean, it can really happen very quickly. So please do take this seriously if you do wind up treating this patient population that's already fragile to begin with. A very important point that I've kind of discovered in my career is that it's really important to have a protocol in place to treat the patient experiencing withdrawal, you know, and we all work in different situations. Some of us are exclusively private, some of us are exclusively academic, some of us are in group practices. There's no one protocol that's right for every situation, but really develop one that's right for you. And in my own personal practice, I am a combination of private practice, but I'm also a Tufts Medical Center in Boston where I teach the residents. And I'm also in a physician-owned private practice called University Orthopedics, which is the orthopedic department for Brown University. And in Rhode Island. And so I've kind of had to kind of crazy glue a protocol for these patients who are all throughout Massachusetts and Rhode Island and beyond. And really what I do in the situation is I say, you know, if you have to go somewhere, go to the ED at Tufts. And why is that? Because we have the physiatry and neurosurgery and neurology residents who are able to take care of these patients. And I always have my PMR residents go to the ED to basically greet these patients, even if I can't be there if I'm still more than else. And that's a protocol that really works for me. And I've written a protocol and I've shared it with my residents and I train them on it. And I will share this with you later. It's also very important to educate the ED staff. You know, we don't necessarily want our patients going to an ED where they don't know how to manage a back of a pump. So it really is best to have them go to your ED where there's a team that can take care and has experience in treating this patient population. Definitely consult PMR or neurology or neurosurgery. And, you know, this is a great way to have residents involved. You know, all of our residents, or a lot of them are really now going into more outpatient procedural fields, you know, sports, spine, and they're all great fields. But, you know, this is also a great outpatient procedural field as well. And we're going to make a tremendous difference in these patients' lives. And, you know, leaning on your residents to treat these patients, if there's a question of back of a draw in the ED is, you know, a great way to really empower them to, you know, in a hands-on manner, get involved with taking care of these patients. And in return, you obviously, you know, I look upon this as a team, as a posse in a way. So, you know, they'll see the patients in the ED, and in return, I train them throughout the three years that they're with me. So it really is a wonderful relationship. And definitely admit to the ICU if you suspect acute withdrawal. And I just want to emphasize that the treatment for acute back of withdrawal is not, if there's one take-home point from all of this, is do not just give them oral baclofen and send them home. I really have to admit them for IB benzos, and I'll show a nice list of medications that the head of our neuro ICU at Tufts shared with me. As Dr. Eckert says, always first rule out other causes of spasticity exacerbation first. And again, it's important to educate the ED staff about this. I've had a patient with an interesting diagnosis, neuromyelitis optica, and she was on a pretty, kind of like Dr. Eckert's patient, she was on a pretty stable dose of the baclofen pump. A pretty low dose was probably 50, 75 micrograms a day. It was a pretty low dose, if I remember correctly. And all of a sudden, her spasticity just skyrocketed. And I modified her score of threes and fours. I could not bend her knees at all. She had a horrible abductor tone. It really happened very quickly. And we did a huge workup on her. Everything was negative. Obviously, you know, UA was negative. CT dye study was negative. I did a nuclear medicine study on her as well. I had side access for desperation. We basically did everything on her and it was all negative. And then it turned out to be COVID, you know, and who knew, I mean, COVID just really just made her spasticity skyrocket. And then once the COVID dissipated, then her spasticity kind of returned back to baseline. But you never really know really what it can be because really any infection or any noxious stimulus can exacerbate spasticity. So it really is best to discuss with your residents who are on call, to discuss with your other attendings who may not be, who are also on call, who may not be as comfortable managing this or also with the ED staff to really rule out any other type of noxious stimulus. Because it can be anything. It can be a skin breakdown, ingrown hair. You know, obviously a lot of our patients have spinal cord injuries, so they are insensate below a certain level. So who really knows what's going on there? So it really can be anything. The most common cause is probably UTI. I would say in my experience that UTI can really exacerbate spasticity. But again, oral baclofen is very unlikely to be significantly helpful in cases of baclofen withdrawal from a mouse-functioning baclofen pump. And I've seen this from experience. I've seen, you know, patients go to other EDs in the New England area where they're not really accustomed to dealing with this. And they're given oral baclofen and, you know, it doesn't really work. And the patient still goes through withdrawal. And why is that? And the big reason why is oral baclofen really has a slow onset of action. And most importantly, it does not reach the high level of concentration in the intrathecal space in the CSF that the baclofen pump does. And that's really why the baclofen pump is so effective at what it does because it really just, you know, floods the GABA-B receptors in the intrathecal space, whereas oral baclofen doesn't do that. So, you know, do I put my, if I suspect someone's about to go through withdrawal or maybe in the early stages of withdrawal, do I still put them on oral baclofen? Yes, but that is by far, that is not the only thing that we do. We really do, again, administer IV benzos and titrate for efficacy. You know, normally when we start oral baclofen in an outpatient setting, we usually start at a low dose and we kind of go up pretty slowly. I don't do that. My own take is that if I am gonna put somebody on oral baclofen, in addition to IV benzos, I'll give them a pretty high dose of oral baclofen because I'm not really worried about fatigue at that point. I'm really more worried about withdrawal. That's really the 800-pound bill in the room. And really the goal of treatment is to have baclofen reintroduced ASAP to the intrathecal space. So as Dr. Heckert was saying, even a lumbar puncture, you know, can be helpful, but that's obviously not gonna last too long. If a catheter is patent, sorry, if a catheter is patent, sorry, let me do this. I can't see. Sorry, I'm just trying to get this out of the way. There we are. If a catheter is patent, you can consider refilling the pump reservoir and administering a bolus. But just know that if a patient is coming in with withdrawal symptoms, and if the pump is simply empty, and if you refill and administer a bolus, it may not be immediately effective if the catheter and the pump are empty. Why is that? Because depending on the dose that the patient's getting, could take a long time for the baclofen that you've now injected into the baclofen pump to actually reach the intrathecal space. So don't think that you can just refill the pump and send them home, because that could really lead to a withdrawal-type syndrome down the road. If the catheter, if you're positive that the catheter is empty, but you have to be positive about this, you can consider a priming bolus, which would basically fill the catheter up, up until the catheter tip. If you're not sure about the catheter, you can always perform, as Dr. Heckert said, a side-axis port and expiration, which empties the catheter, and then follow up with a priming bolus to fill the catheter. Sure, a lumbar puncture with injection of baclofen can be helpful. If they're in a community hospital and there's no interrogator present, and they're at a hospital where there's no team that can manage baclofen pump, but this is really more of a temporary effect. And some neurosurgeons or anesthesia can also put in a lumbar drain as well, for similar reasons. This is what the head of our neuro ICU shared with us. I'm frequently asked, what are some of the initial doses of medications for acute baclofen withdrawal? And if you look right here, lorazepam has a suggested starting dose. But the point is, with all of this, is you really wanna titrate to comfort. For those of you who aren't too experienced with this, this is almost, baclofen withdrawal is almost like the CWAS scale. You really wanna just keep giving them the medication until their vitals and their spasticity have kind of normalized. And if anybody wants this, please feel free to email me and I can send this to you. I just wanna share with you quickly before Dr. Salino goes over his images. This is the protocol for my residents. And this is what I want them to be comfortable doing by the time they're done with their PGY-4 year. It's meant to, this document is really meant to support the PMR resident on call, when called from ED and in cases of questionable baclofen pump malfunction. If it's within 30 days of implementation, then definitely get neurosurgery involved. So first, this is just our, again, this is our ED protocol. I notify the PMR attending on call, evaluate the patient in the ED, check for vitals, look for other causes of spasticity, which I've already gone over. Ask the patient if he or she has heard the pump beating, because you have a single tone or two tone, single tone is a non-critical alarm. Two tone, non-critical meaning that it may not be empty, but it's getting close to empty. So there may be less than two CCs of baclofen left over versus a two tone alarm, which is really more of a critical alarm, which means that the pump is really just not functioning, period, or it's truly empty. But again, assess for other causes of increased spasticity. Interrogate the baclofen pump and yes, definitely look at logs and alarms, which is on the tablet in the upper right-hand corner, you have to click logs and alarms. And then, because that'll tell you, sometimes the pump is turning on and off for one of many reasons. Sometimes the battery dies early. I've even seen that. And I have seen a case with an iPhone shutting off a pump. So you really never really quite know what it is, but also make sure that the refill date and the ERI, which is the Elective Replacement Indicator, have not passed, because then you may have an answer right there and check for motor stalls. And now this is what I want our residents to be comfortable doing as well. Use sterile technique and take one of those baclofen refill kits and aspirate the contents of the baclofen pump and compare to the volume that is calculated to be in the pump. Because when we interrogate the pump, and this is for those of you who aren't really too experienced with this, that tells us what should be happening. It doesn't tell us what actually is happening. The only way to really know if there's baclofen in the pump is to put it in the pump and aspirate. And then you can re-inject it back if it's close to full, if need be, but use one of those blue filters. Then if all of those above steps are normal, you can also order imaging, as Dr. Heckert said. And then with approval of the attending, consider programming a bolus, either 50 to 75 micrograms, sometimes 100 micrograms, administered over several minutes. And then the next steps to be performed, with me present or another attending present, would be more of a side access for aspiration, which Dr. Heckert was discussing, which really checks for the patency of the catheter, or a nuclear dye study or a CT dye study as well, which are really meant to be more, if a patient admitted or is an outpatient, the patient is stable. This is really a protocol that I want our residents to be able to really run the show with when the patients are in the ED. So I'm gonna end mine right now, and I'm gonna let Dr. Salino take over. Gotcha. Very nicely done, Dr. Heckert and Dr. Bohart. Actually, Zach, I wanna ask you a question that was recently put to me, just to get your opinion, and Kim, you could chime in also. Do you have, I'm with you, I tend to go with the IV benzodiazepines, if I truly believe I'm in withdrawal. The question that was put to me, do I have a favorite? Do I have one preferred over another? And my answer to that question is, is whatever IV benzo, that particular unit is most comfortable with, is what I choose. I mean, if they use propofol routinely, that's fine. If they're more comfortable with Ativan or Midazolam, whatever that ICU does for IV benzos, I don't wanna reinvent the wheel with it, but I'd be interested to hear your opinion. I'll get started and share my screen while we're chatting. I would say if the patient's already on a benzodiazepine at home, and you have a proven record that that works well for them, that might be a reason to consider using one particular one over another. And there's also the duration of effect, right? So, I mean, Klonopin is obviously longer acting, if I remember correctly, than Ativan. And again, this is not really my wheelhouse. I'm fortunately outpatient, and I very happily allow our inpatient neurointensivists to deal with this. But the duration of effect would be important as well. Gotcha. All right, let me go ahead and share screen. Can you guys see that okay? Yep, I see it. Looks great. Gotcha. So, an absolute pleasure to share the virtual podium with friends and colleague, Dr. Bohart and Dr. Heckert. I have learned a ton from both of them through the years. Wholeheartedly agree with Dr. Heckert's thoughts about doing a thorough investigation into potential noxious stimuli. It always hits home with me on a particular patient that I recall that I was about to do a CT myelogram on, and this is the first time I had seen this individual in a gown, and I was just about to access the catheter access port when there was this big necrotic area on the individual's thigh. And I said to the patient, what was that? And they said, oh yeah, I forgot to tell you, I spilled some coffee a couple of weeks ago on my pant leg and didn't know it, and actually had a pretty significant burn. So, I guess when I said to the patient, are there any problems with your skin? And they said, no, that didn't count. So, totally wanna go down that road of chasing down all the noxious stimuli possibilities. My task for the next couple of minutes is to talk about the imaging of intrathecal delivery system as it relates to troubleshooting. Despite all of our good efforts, many, if not most of our troubleshooting does come down to imaging studies. There are relatively few physiologic things we can do. One thing that Dr. Heckert mentioned was actually repeating a lumbar puncture to see if that results in return of effect. There have been some investigational studies with electrodiagnostics and CSF pressure wave detection that have shown some promise, but have not really come into mainstream utilization. So, it is important to understand what the imaging looks like. The last thing I'll say before I dive in deeply to imaging is don't lose your head when you're dealing with an intrathecal baclofen patient who's not having optimal effect. The rest of medicine, their brains fall out. They absolutely do not know how to think through things. Let us show our colleagues that we can. Okay, with no further ado, let us move forward. These are my disclosures. I work with many of the drug and device companies involved in spasticity, pain, and neuromodulation. I will only use brand names when I felt it was appropriate for educational purposes. And nuclear medicine cisternography, which we'll talk about a little bit, is technically an off-label procedure, not that it has ever been observed to be harmful. It just does not undergo the full clinical testing in terms of workup. So, it is an investigational procedure. So, single objective for me is to describe imaging systems or imaging techniques that are utilized in the management of intrathecal delivery systems. The couple that I'm gonna talk about are plane radiography, ultrasound, a catheter dye study, CT myelogram, and nuclear medicine cisternogram. So, plane radiography, AP and lateral thoracic spine, as well as a flat plate over the pump site. The advantage of this is you could do it remotely, even send the patient an electronic prescription. Please, please, please always check your own films. I know I sound like your internal medicine attendings when you were an intern. Lots of radiology now gets done remotely. Sometimes it even goes to the other side of the planet. Someone gets an X-ray at midnight, it's a radiologist in Australia reading the films. Some of these folks have never seen a pump, and even though you specifically asked to check the continuity of the catheter, I have absolutely had films that were read out as normal, where there was a four inch separation from the ends of the catheter. Biggest disadvantage right now is that the newer Medtronic catheter is currently radio lucent. There is some investigation with an updated catheter that may give it some radio opacity, but that's not commercially available at present. And recognize that you're probably only gonna pick up super big things. Still is worthwhile, it's relatively inexpensive and can make the diagnosis for you, but in the more challenging cases, you're gonna go on to other imaging studies. You know, so this is kind of the classic picture of a pump catheter system. Pump in the right lower quadrant there, the catheter connected to, and then kind of working its way around the spine and then up the spinal canal. The challenges is when you either do a direct look at this, I mean, you could say, is that catheter appropriately housed on the hub of the nozzle, so to speak? Are any of those twists and turns kinks or breaks, or is it just a projectional issue? It can be kind of hard to sort out. So catheter fracture, pretty easy to see. You could see the catheter entering the canal, but then having sheared itself off, probably on the edge of a lamina and have a free floating piece of catheter that's actually all the way down lower in the fecal sac. This free piece of catheter does not need to be retrieved. It actually will cause more pathology with your patient to retrieve it than it will be to just replace the catheter and put a new one in. As Dr. Heckert mentioned, if you do see something like this, don't assume that patients are getting nothing. Wean them down and then start them up as a brand new pump. Here's another picture of a catheter fracture. Again, seeing the two ends demonstrated, pretty straightforward that this again is an individual who's had loss of effect. Where that catheter tip is might be in the epidural space. An epidural baclofen works, but it works somewhat inconsistently, certainly less consistently than intrathecal baclofen. Again, wean this person down and restart them. Catheter migration, this is an individual whose catheter completely migrated out of the spinal canal. You can see it kind of curled up in the paraspinal soft tissues and no catheter present in the canal. This actually happened twice to the same patient about four years apart. There was just something about this individual that made her prone to it. Looped catheter, here's an image where the catheter went in and instead of going cephalad towards the thoracic spine went caudal and is actually probably just delivering right to the sacral portion of the spinal canal and easy to understand why some of them might not be getting optimal effect. Radiolucent catheter, trust me that this is an individual who does have a intrathecal pump and you could see no catheter, but you see this straight line radio opaque structure. This happened at the witching hour of medicine, meaning it happened at four o'clock on a Friday afternoon and people were panicked. Was there a retained needle? They had made the patient NPO, they had called both general surgery and neurosurgery to take a look at the patient when in fact it was just a radio opaque connector piece. Sometimes depending on the patient's body habitus, a single catheter implant can't be done and there can be both a proximal and distal catheter segment and you connect those two with this connector that it is radio opaque. And here's sort of a troublesome plain film. Is this a projectional loop on the plain film or is it a kinked catheter? And that's why plain films are somewhat limited. Quick little thing about ultrasound. This is the ultrasound appearance or the sort of graphic appearance of a synchromed tube. You can visualize both the catheter access port as well as the reservoir access port. This is in distinction to the Prometra system, which has a different ultrasound profile. One thing I forgot to mention in my disclosures is the use of ultrasound with intrathecal delivery is also technically off label, not that we have any expectations that ultrasound will damage the system, but it just hasn't been formally studied. One thing that ultrasound is very good at is detecting the so-called flipped pump. There actually is a name for this, it's called Twiddler's syndrome, where people flip an implanted medical device first initially described with pacemakers. In this ultrasound image, you don't see either a reservoir port or a catheter access port because it's on the other side and your ultrasound doesn't see it. This actually happened to me two weeks ago where I had a suspicion of a flipped pump and confirmed it with ultrasound and that patient is going back for a revision to put things back. This is actually a pocket fill where an individual had the solution for their intrathecal delivery at least partially injected above the pump and not into the reservoir. In a baclofen patient, this will probably present as withdrawal because the volume inside the reservoir is less than what you programmed it will and eventually it would run out. If you're doing this on a pain pump, this is potentially fatal pretty quickly. And in that case, you wanna potentially try to aspirate out that hypoechoic fluid collection. Catheter dye study. This is where you inject dye through the catheter access port and view it in real-time fluoroscopy. If there's one little bit of teaching that I wanna absolutely, absolutely emphasize is that if you cannot aspirate the catheter contents, you do not inject dye. The reason for that, as Dr. Hackert mentioned, is you potentially bolus the entire contents of a catheter all at once. In a 2000 microgram per cc concentration, that could be a couple of hundred micrograms all at once and that could be enough to put the patient into overdose. There are reports of fatalities when someone tried to do this with a pain system. Advantage, definitely has better sensitivity compared to plain films. It does require a little bit of technical expertise and you're probably only gonna pick up big tears. You might still miss smaller tears and cracks. So here's an example of a catheter dye study. You inject dye and you could see that ballooning out right over the spinal canal, representing a crack in the catheter. And again, wean the patient down and then restart. Similar image here in the paraspinal soft tissues where you see dye ballooning out and nothing in the canal whatsoever. CT myelogram follows the same principle except instead of using fluoroscopy, you actually do a CT scan. I think we should begin to call this procedure the Turner procedure. It should be named after Dr. Mike Turner, a pediatric neurosurgeon in Indianapolis. Mike passed away a few years ago. I learned an awful lot from him. And he was the first one to describe the CT myelogram study. I would put forth that there are abnormalities that you could see on CT myelo that you cannot detect with any other mechanism. I'll show you that in just a little bit. You actually inject the dye right in the CT suite and then immediately go for scanning. And you scan all the way from two vertebral segments above the tip of the catheter down through the pump in the abdomen to look at all particular possibilities. Advantages of this study are that it has better sensitivity compared to both plain film and fluoro. And also you get a quote unquote free look at a lot of structures of the abdomen, the thorax, the lumbar and thoracic spine. I can remember one of the first CT myelograms I did, we actually picked up a pancreatic cancer as a cause of noxious stimuli that the patient didn't appreciate because they were insensate. Disadvantages, again, you can inject if you can aspirate, coordination with radiology, and again, a little bit of technical expertise both in performing the study as well as interpreting it. I would highly recommend if you're gonna do this that you discuss it with your neuroradiologist so that they understand what they're looking for. So this is a normal study looking at the axial images. You could see the dot there at about the seven, eight o'clock position that represents the catheter. And that crescent shaped bright white slice represents the dye. You could see that it is nice and even, it is symmetrical side to side. This represents a normal study. Here's an example of contrast that's loculated in the spinal fluid. You could see that it is asymmetric, not lying just on one side. The right-hand image actually shows some dye ventral to the cord. These are the patients that worry me the most. These folks, I think, have a sequestered pocket of baclofen hanging out in the fecal sac that is just waiting to burst. And if you've ever had a patient that sort of had an alternating overdose withdraw syndrome, I think that that's what this is. No one knows the natural history of catheter loculation or subdural encapsulation that I'll show you in just a moment. We know it typically does not happen originally at the time of implant, but somewhere a few years into the therapy, there was some dural reaction that results in these abnormalities. Here's an example of a subdural catheter. Again, instead of being on the dorsal aspect of the spinal canal, it's on the ventral aspect. And again, kind of sequestered. If you flip the patient, the dye will stay in the same position. It will not move. Here's an example of contrast extravasating into the paraspinal soft tissues and not seeing anything in the canal. Again, confirming a catheter problem. This is a relatively rare thing to see, but this is actually dye filling the epidural space. Nothing in the intrathecal space, but actually filling up the nerve roots and surrounding it. The expectation of this was that perhaps during the initial implant, when the surgeon went to implant, there was a little bit of slippage of the needle out. And instead of threading it in the subarachnoid space, it threaded in the epidural space. This is a CT Myla image demonstrating a intrathecal granuloma. Relatively unheard of in the intrathecal baclofen world. Rare, but not unheard of in the pain community. You can actually see the granuloma beginning to compress the spinal cord. And this has a whole different management scheme if you see that. Sometimes, let me see. This is actually a CT mylogram with 3D reconstruction, able to kind of spin the spine around, pull off all the paraspinal tissues, and actually find the twists and turns of the catheter. In this particular image, we were only able to do the CT recon on the posterior half of it. A similar video could be shot on the front. 3D reconstruction can be a little bit challenging to get approved by payers. For example, Medicare does not cover it typically, but it is a pretty effective study to look at. Whoops. Lastly, nuclear medicine cisternography. It's really the only imaging study that can be utilized if you cannot aspirate from the side port. This is an injection of 500 microcuries of Indium-111 DPTA. You then collect images at 24, 48, and 72 hours. Again, as noted, this is technically off-labeled because of the lack of compatibility studies. Two things not to do. Do not use the filter on the refill kit. The DPTA will actually stick to the filter. There are some older reports of using technetium-99. Technetium-99 has a very short half-life of about six hours, and you might miss some patients who have a late abnormality. This can be combined with CT imaging, as I'll show you in a moment. And rarely, both false positives and false negatives have been reported. Advantages, again, the primary advantage is the only imaging study you could do without aspirating from the side port. It's a challenge that it needs to be coordinated with nuclear medicine. It takes several days to get a result. And again, it doesn't show good anatomic localization, but it's really more of a functional study than an anatomic study. So here's some examples of normal studies depending on your nuclear medicine preference. Sometimes it will look bright on a black background, or white on a black background, or it could be black on a white background. Here you could see the big sunspot representing all the radioactivity housed in the pump, going into the catheter, filling up the canal, and actually going over the ventricles and the basal cisterns. You could actually see sort of a ventricular gram showing that dye. Here are a couple of images showing a catheter with a focal collection, or abnormal accumulation within the fecal sac that you don't see that nice, normal, even distribution. And again, it could demonstrate two images. Here's some examples of catheter uptake that is more prominent at the catheter tip, suggesting some degree of scarring or perhaps a subdural or catheter loculation. Don't see this too often. More likely, or the only time I've actually ever seen it when we did both ACT myelogram and nuclear medicines and sternogram on the same patient, and it wound up being a loculated bit of catheter. This is a catheter-to-pump occlusion, where literally you see nothing other than the sunspot of the radioactivity in the pump, and this person obviously needs a revision. Lastly, you can couple nuclear medicine cisternograms with SPECT scanning. SPECT scanning stands for single photon emission positive tomography. Where you need a CT scanner that is also a nuclear medicine scanner. The only person on the planet who I know who does this routinely is Elmer Delahas in the Netherlands at Rotterdam. These are a couple of images from his paper where you see the radioactivity superimposed on a CAT scan. You could see in the image D there, a kind of an accumulation of dye in the paraspinal soft tissue. In image E, you could kind of see an uneven distribution of dye. And image H is actually a pretty interesting one. It's a collection of fluid actually behind the pump. If you read Dr. Delahas's paper, he does a very, very standardized protocol in which he dilutes the solution that he's studying to make sure that the flow rate reaches the cerebral cisterns by 24 hours so that the 48 and 72 hour images have enough radioactivity to detect some of these abnormalities. And that brings me to the end of my little piece of this. As mentioned, there's multiple imaging studies available for troubleshooting. As mentioned, sometimes you need to educate the radiology staff as to what you're doing. And even those of us who have seen and looked at a lot of these, sometimes we're still bollocks as to what we're seeing and have to go on to some other things. My email addresses are shown there. I'll be more than happy to answer any questions offline or if you want me to look at some images, I'd be more than happy to do that too. And I am gonna stop the share so we could have a little bit of a group discussion. I know we have a couple of minutes left. Can I ask you a question? Sure, go ahead. Good, excellent. So you're talking about with a nuclear medicine study to have a protocol of patients coming back at 24 hours, 48 hours and 72 hours afterwards. Correct. When a patient is on a lower dose, maybe receiving 100, 150 microns a day, it may take longer than that to go all the way through the catheter to the intrathecal space. So do you give a bolus to kind of speed it up? I mean, what do you do in that situation? So you can, or if they're on a super low dose, we might tell the patient to 24, 48, 72 and one week, bring them back for a fourth set of imaging. Okay. And I also perform, I like the nuclear medicine study, our neurosurgery department doesn't, they don't like it. But I like it because I just find it technically easier to perform than a CT dye study. Sometimes it's just simply hard to find the side access for, especially in patients with obesity. Do you agree with that? I tend to like the CT myelogram studies because I think it gives you really anatomic distinction that you're not going to see with the other studies, but I agree with you. It's technically easier for lots of people just to do the nuclear study. Thank you. I wonder also in the case that you mentioned, Dr. Bohart, if you could empty the reservoir and place dilute baclofen in the reservoir, say 250 micrograms per mil, so that the flow rate is a little higher and then maybe you wouldn't have to, you know, have them come in an additional time for the last scan. That's a good idea. Never thought of that. Right, and it's also a good maneuver, Kim, just as you pointed out in your presentation that maybe it's a volume dependent thing. We've seen folks who have gotten return of efficacy if presented with a higher volume. So there's multiple benefits with that. So I do see one question in the chat, and I'll repeat the question. We're seeing a slew of loss of efficacy in catheters over 15 years old without clear indication on workup that it's absolutely the catheter or where the catheter malfunctions after the pump replaced routinely, and routinely when they go back, they get a decent flow of CSF in the OR. So I think one thing that this points out is probably the bane of all our existences, and that's the infamous micro tears. There has been some description of catheters that have had hundreds, if not thousands of little micro punctures along the way. You know, think of a garden hose with lots of little holes in it that eventually the flow at the end of the hose is next to negligible. I think when you see that, the only way that you could do this reasonably is to repeat the trial with a traditional lumbar puncture, and if they get a positive effect with the LP, go ahead, wean them down, and start them up like a fresh catheter. The handful of times that I've done that, almost uniformly patients get a return of effect with actually at a much lower dose than what they are before or were before. Completely agree with that. I mean, if you've made a good faith effort to work it up and you just can't find it, I think that LP bolus can be very helpful, especially if you have to convince a neurosurgeon to do a catheter revision, which is not always easy, or if there's any part of your patient that isn't convinced. We have another question in the chat. In a relatively asymptomatic patient, is there a specific cutoff where you would pursue significant, greater than five cc's difference between predicted versus actual reservoir volume? I think maybe more importantly than the actual number of milliliters, which could be affected by when you tend to do the refill, is a trend. Is there a trend that this difference is increasing that is suspicious for a problem as opposed to someone that maybe always has a difference of four mls or something? Would you agree with that, doctors? Yeah. I try to, the first time I have a large discrepancy there, I just document it, and I just kind of keep an eye on it. Of course, as long as it's not empty or nothing to do with troubleshooting procedures, I document it, and then with the next refill, I see if it's the same, or maybe there was a partial pocket fill, for example, with the previous refill, or something happened, who knows? But yeah, if it continues to stay there and it gets worse and worse and worse, then sooner or later you want to work it out. So the challenge with that is not so much at any moment in time, but the biggest worry I have with the distinction between predicted and actual actually comes at the time of pump replacement. You know, if you're having a five cc volume discrepancy in a 20 cc pump, that's 25% of the volume, 12.5% in a 40 cc. You know, remember when we change the dose, what we're really doing is changing the flow rate. The dose is actually a calculated number, flow rate times concentration. So someone might be programmed to say receive 400, but they're actually only receiving either 25% more or 25% less of that when you go to do your pump replacement. If you program the same dosing, you risk both overdose and withdrawal. So my worry is that when you do that, be super, super cautious about the dosing at the time of pump replacement. Normally a pump replacement's a same day procedure. Maybe keep them overnight to make sure that there's no undiagnosed withdrawal or be prepared to raise or lower the dose accordingly. I haven't seen that a whole lot, but in the handful of times, it can be a little bit challenging. I will also add that the person who asked this question mentions the patient's asymptomatic. And I would really delve into that history. I was very surprised recently by someone I did a routine cap aspirate on prior to replacement of his pump for end of battery life. And he was dry and I never saw it coming. But when I really, really looked back, there were probably some subtle signs there that he wasn't getting the relief that he had previously, but I really had to look hard. So there might be even some very early symptoms before it really cues you that there is a loss of efficacy. So I would take a deeper dive. Any more questions, everybody? It is getting late for some of us. I hope you feel empowered a little bit by listening and I hope you choose to not be afraid to use this therapy. Thank you so much for your attention. Yeah, thank you, everybody. Thank you, Dr. Salino. Thank you, Dr. Hecker. It was really great to have both of you here. And yes, this will be recorded and we hope you found this useful. Thanks to you guys too. Thanks, bye everybody. Have a good evening.

Dr. Zach Bauhart

Dr. Kimberly Heckert

physiatrists

Baclofen pumps

loss of efficacy

protocol

ED staff

case study

spasticity exacerbation

Baclofen withdrawal

intrathecal Baclofen delivery systems