Misunderstood to Managed: Treating Patients with Sickle Cell Disease and Trait

But no, you don't have a lot of dentists that know anything about sickle cell disease. So for about three or four years, that's what we did. We lectured at the different dental societies to try to educate these dentists on the clinical manifestations of sickle cell disease in their dental patients. Welcome to the Phil Klein Dental Podcast. In today's episode, we're addressing an often overlooked topic in dentistry, managing dental patients with sickle cell disease and sickle cell trait. These patients face unique challenges, and unfortunately, many are turned away from dental practices simply because their condition isn't well understood. Our guest today is Linda Chandler. She's a registered dental hygienist who has spent more than 45 years working closely with sickle cell patients and educating general practitioners about the disease. She'll help us understand how to identify these patients, recognize the key radiographic findings that may point to sickle cell disease, and discuss the clinical risks that must be considered during treatment. We'll also explore why it's so important for dentists to distinguish between transient sickle cell pain and true odontogenic pain, so unnecessary extractions can be avoided. and patients can receive the compassionate, appropriate care they deserve without having to travel miles just to find a willing provider. So our guest today, as I mentioned, is Linda Chandler. She is a graduate of the University of Texas School of Dental Hygiene, past president of the Southeast National Dental Hygiene Association. She's written several articles on sickle cell disease and various health-related subjects, and voted Top Hygienist of the Year in 2012. Before we bring in our guest, I do want to say that if you're enjoying these episodes and want to support the show, please follow us on Apple Podcasts or Spotify. You'll be the first to know about our new releases, and our entire production team will really appreciate it. Linda, it's a pleasure to have you on the show. Thanks, Phil. It's a pleasure to be here, and thank you so much for having me. Yeah, we're very happy to have you, Linda, on the show, and I do want to recommend to our audience that you gave an excellent webinar. in August of 2025, Smiles in Sickle Cell Care, Preventive Dental Hygiene Strategies for Enhanced Health. To find that, just go to vivalearning.com, type in Chandler, C-H-A-N-D-L-E-R. And I think there's a lot of information that all of us as practitioners can benefit from. Before we hand it over to you with some questions, Linda. I do want to give the audience a little bit of a background on sickle cell disease. There's 120,000 Americans that have it. And if you, to use the general dental utilization numbers, let's say 50 to 70%, you might expect on the order of 50,000 to 70,000 dental patients that are seeing dentists with sickle cell disease nationwide. And I think it's important for our clinicians out there to know about what this means as far as dental treatment. So the disease itself involves abnormal hemoglobin and that's known as hemoglobin S. It has a lower functional capacity and it causes multiple systemic complications along with dental complications. The actual hemoglobin shape is crescent or sickle-shaped and it actually will become stiff. And that could cause blockages in the blood vessels. Now, specifically as an endodontist, I can tell you, I have seen patients that come in with severe pain, pulpitis, and then I take an x-ray and it looks perfectly normal. And what's actually happening is, in this kind of situation, is that in the dental pulp, there's a vaso-occlusive crisis going on, which is basically an obstruction of the microcirculation of the pulp. And that will produce, in many cases, a very severe symptomatic pulpitis. So keep that in mind, because by no means should you perform endodontic treatment or extract the tooth. We do not want to go in that direction when a patient like this comes in under this condition. So it's something that's very important that we know could occur in these patients, along with other things like transient numbness of the chin and lip. That's also due to vascular occlusion. around the mandibular and mental nerves, and we'll talk about that. And then, of course, there's the hypoplasia of the enamel, which could cause, obviously, progressive karyogenic activity, and we need to know about that as well. So turning it over to you, Linda, with your experience, and you've been teaching and writing about sickle cell disease and sickle cell trait for a long time, what are the challenges general dentists and hygienists face when treating these patients? Well, the challenge that we have is that we have a large number of people. We have about a 32-year span of people who actually do not know the sickle cell status, whether they have the disease or the trait. And unfortunately, most health history questionnaires don't ask this. So you have a patient in the chair, and if you have not asked that patient specifically if they have sickle cell disease or the sickle cell trait, they're not going to list it, and you won't know. But as a clinician, when you go in, like I showed on the podcast, when you look at the x-rays, we can actually look at the trabecular pattern of those x-rays and kind of give us a clue right off the bat. Also, another thing with you being an adonis, Phil, another thing that we see in our x-rays, especially the periapicals, we'll see where the pulp is dying or the pulp is calcified. And that's one of the another signal that we have when we're looking at it. So a lot of times our diagnosis comes from just looking at the x-rays and also a few other of the characteristic signs that gives it away. like one of the things that we can also tell once we look at the x-rays and we see that trabecular pattern we can literally look at the chest because a lot of them because they're suffering for oxygen they have the barrel chest They have the dry lips. They have the dry gum tissue because they're trying to breathe through their mouth. They're trying to get as much oxygen as they can. So these are some other things that tells us this. And then also, if I've had a couple of patients that came in that did not know they had sickle cell disease or the sickle cell trait, and they've already had to have knee replacements or hip replacements, these are some of the other common. things that go along with sickle cell disease, having to have a kidney transplant, having their spleens removed, their gallbladders. You have all kinds of medical problems that if you don't have it on the health history questionnaire with the associated medical problems, that's usually a signal for Dr. Ramirez and ourselves that, hey, this patient may have sickle cell disease at a sickle cell trach. So there's a lot of things. And when we're out there lecturing, we let those dentists know that if you don't have that on the questionnaire and you have somebody in your chair that's had to have a kidney transplant or is going up for a hip replacement, the first thing we ask is, have you been tested to see if you have sickle cell disease or the sickle cell trait? And now a lot of the MDs are doing that when they come up against people of African descent, especially here in the United States of America. they're starting to ask more questions. And it's also prevalent in the Hispanic population, is it not? Oh, definitely, yes. We have a large Hispanic population. In fact, a lot of the sickle cell patients come to the United States from the Hispanic countries. They come to the United States because we have... We have just better medicine. We have better facilities to help treat the sickle cell. Yeah. Now, what about the color of the mucosa, the gingiva and the buccal mucosa? Isn't it paler than what it would be typically? Yes. And it has like a whitish color to it and it's dry. And, you know, generally we can look at that and tell. And mainly that's because... of some of the medications that they take. The medications cause a lot of problems. And just like with the, most of them are on hydroxyurea. A lot of them was on the Oxbrida, but they pulled that off the market. But they have some fascinating drugs that are being tested right now that's going to be able to help them. But even with those drugs, we have to work carefully with their hematologists. You know, you just can't go in there and start doing a bunch of stuff. We have to work with the hematologist. Because a lot of them are on a daily regimen of antibiotics, and we're getting ready to introduce a lot of antibiotics into their system. So sometimes we need to put them on a stronger antibiotic. And with that, we have to work hand in hand with the hematologist. So in your experience, Linda, at the Powder Smile, you're a practice in Houston. What is your biggest challenge when you first see the sickle cell disease patient? Well, the problem that we have here at the Potter Smile is that most of the time, by the time they get to us, a lot of damage is already done. Most of the patients that are full-blown sickle cell disease, or SS, like we like to call it, or SC, these are the ones that has the most problems, that they have the most vaso-occlusions, or we call them sickle cell crisis. They're on Medicaid until they're 18, unless they have a private insurance. Fortunately, we have quite a few of our sickle cell patients with private insurances, but if they haven't been under care of a dentist from, say, age three or four, because what we find in a lot of these patients, especially when they're children, they will have enamel hyperplasia where the teeth don't actually develop. We also find that a lot of our patients with... ss disease or sickle cell disease they will be missing teeth they will be genetically missing teeth just like my granddaughter has the trait and she's genetically missing her second bicuspids and also we'll find in the gum tissue it'll be very very fibrotic so a lot of times when the teeth are trying to break through they don't break through the gingival tissue in the normal eruption pattern because as you know as we learned in dental school that the permanent tooth is right under the deciduous tooth and it dissolves the deciduous tooth and it comes straight in that deciduous tooth is actually a pathway for that permanent tooth so when you have a very fibrotic gingiva, that permanent tooth developing may come out on the buccal side or it may come out on the lingual side. So then you have a dentition of both permanent and deciduous teeth. So let me ask you this, Linda. For routine SRP, would you consider a patient with active sickle cell disease a high-risk patient? yeah full-blown sickle cell disease yes there's a high risk and the first thing is a lot of times they their clotting factor is so low we have to watch the clotting factor because most of them, when they come in, they have anywhere from six to eight millimeter pockets, a lot of tartar. They have a lot of decay. They have a lot of infection. So that's when we really have to work hand in hand with the hematologist because once we go in to do that SRP, we're introducing a lot of bacteria into the blood system. And then if they have other problems, like we had one patient that needed an SRP, but this patient was on dialysis. and her kidneys were blown. So we couldn't afford to go in and put a lot of additional things in there with that kidney problem without covering it with the hematologist because she was already challenged enough. And then we introduced a lot of microbes into their blood. strain so they may not be able to control that so we can create a whole different set of problems so we have to watch that do you put them on antibiotics when you do srp yes we do what do you put them on typically most of the time we put them on amoxicillin unless the doctor or the hematologist wants them on something stronger and how long is that regimen for Well, generally we'll have them to take the antibiotics a couple of days before they come in and then a couple of days after they come in because we definitely want them to be covered so that if anything comes up, they already have the antibiotics in the system to fight it. So you're obviously doing a lot of deep scaling and root planing. Do you find that these patients with sickle cell disease and sickle cell trait, are they more uncomfortable post-SRP than the typical patient? Is their sensitivity heightened? Well what we generally do is once we do that deep cleaning we have exposed a lot of the enamel, especially the cementum, and you have a lot of sensitivity. And unfortunately, you know, we have to really be careful because the enamel is soft, also the cementum is soft, and it will cause a lot of sensitivity. So what I generally do is after I've done that SRP, I put a desensitizer on there, and that's one of the products, as we were talking about earlier, I use is the Voco products, the varnish. It comes in several different delicious flavors, and most of those patients really like it. And I even will give them some to take home to put on some of the areas that they're really, really sensitive because you need that constant barrage of that varnish on there to kind of desensitize that tooth. And you're talking about proflorid varnish from Voco. Yes, and that really helps with the sensitivity. And I do that after the deep scale. I go and I dry it, I isolate it, and I go on and put the varnish on there. before the numbing even leaves them. And then we give them just regular pain medication for the pain. What about a sickle cell crisis? Has that ever happened in the dental office when you were treating a patient? Well, we've been very, very blessed. In fact, we've only talked with one patient that wasn't our patient here that had a crisis in the chair. We were supposed to get back with her to find out what actually happened with that crisis, but we did not get the opportunity to talk with her to find out what actually happened. But no, we've been very, very blessed. Most patients feel no when they're going to have a crisis. They know their bodies very, very well. What is that? Tell us what that is so we understand what a sickle cell crisis actually is. What's actually happening? Well, when you have a sickle cell crisis, it's according to where that crisis is hitting. If it's hitting in the chest, they'll have what's called acute chest syndrome, and they have excruciating pain in their chest. If it hit in their legs, they just double over in pain with pain in their legs. If it hits in their... up in their head or their brain, they have a stroke. If it hits the heart, it's a heart attack. If it hits the spleen, it just clogs everything up. Is there an age range where this happens or it could happen at any age? It can happen at any age and at any time. When these patients have a sickle cell crisis, they end up in the hospital for what, a couple of weeks, right? Isn't it like 10 to 12 days? Anywhere from 12 to 14 days is according to what has to be done. Like if they have a crisis in their kidneys, they're going to have to stabilize those kidneys. They usually have to go on dialysis. They have to go through a bunch of changes and have to go on a lot of antibiotics after that blood transfusions and the blood transfusion. They try to limit the amount of blood transfusions they give to sickle cell patients. But a blood transfusion is based on where they've had the occlusion at. If they've had. The occlusion in the brain, if they're having a mild stroke, they have to get oxygenated blood there immediately. If it's the kidneys, they give them a blood transfusion because they have to get oxygenated blood there. Wherever, if it's a vital organ, they're going to get a blood transfusion because the key here is getting oxygenated blood so that tissue does not die. And to once again reiterate for our listeners what a crisis is, essentially the sickled cell. adhere to the vascular endothelium and to each other and they create micro vascular blockages and as a result of that normal blood flow is impeded of course that leads to reduced oxygen to the tissues and then you have This whole vaso-occlusive activity triggers a cascade of events, inflammatory events, and this causes tissue injury, severe pain. All of this is characteristic of a sickle cell crisis. And once that crisis happens, these patients, as you mentioned, Linda, go in the hospital for 12 to 14 days, I think you said, and their dental hygiene during that time is very little or none at all. So when you see them after that, they must be a mess regarding their oral hygiene. Well, generally, now they, when they're in so much pain, brushing their teeth is the last thing on their mind. And then if their caregivers aren't brushing their teeth, by the time, in about 14 days, under all the medication, not brushing your teeth, when we see them, we are almost right back to an SRP again. You know what I mean? Because the teeth. And then you have a lot of necrotic and granulation tissue. You have a lot of sloughing of dead tissue. And their mouth is dry. And a lot of times, if they have to be incubated, they have tubes. And it can really be bad. So when you're examining the patient, whether you're doing an SRP or just doing a hygiene exam, and you notice a tooth that is hopeless and it has to be extracted, you know the patient has sickle cell disease or sickle cell trait, what is your risk management protocol for that patient as far as getting that tooth extracted? Well, first of all, we have to clear it with the hematologist to make sure that their blood is within normal clotting range. And usually it has to be anywhere between an eight and a 12. As far as the hematologist, they'll give us numbers and they can tell us if we can go on and remove the tooth. A lot of times we have to wait. Sometimes we have to do what's called a prophylactic blood transfusion, where they're giving them a blood transfusion so they'll have enough oxygenated blood so that we can actually extract the tooth or do anything that's going to cause a lot of hemorrhaging. So that is certainly protocol, Linda. that should never be deviated from, which is contacting the hematologist to get the green light on an extraction. Because if you don't, and the patient has active sickle cell disease, which means low oxygenation of the area and subpar clotting factors, you're looking at, as you mentioned, hemorrhage, which could be very serious, and infection of the surrounding tissue. Osteonecrosis, where the bone actually dies. In fact, when... Here at the Potter Smile, when Dr. Marinci removes the tooth, he does not apply pressure to the bone because I've actually seen him try to remove the tooth and take half the jaw because the bone is so weak. So he meticulously dissects the tooth so that it's not a lot of trauma placed on the socket of the tooth. Yeah, atraumatic extractions. That's exactly how he does it. And it takes a little bit longer to do it that way. And then he makes sure that... We suture it, you know, make sure that, you know, you want to hold all that blood in there that you can so it can clot. And then, of course, we cover them with antibiotics. And Dr. Mercy always checks the extraction site two to three days later. Right. And you're calling the patient and you're monitoring the patient very closely. You mentioned earlier, Linda, that many of the patients... don't put it in their medical history that they have sickle cell disease. And sometimes they don't even know they have it. But we are able to detect certain things on radiographs, commonly seen on a pan actually, related to the trabeculation of the bone and other visual telltales that give us the idea that, okay, this patient is likely to have sickle cell disease. Tell us about that. Well, when you're looking at a radiograph, if you see a lot of radiolucencies especially close to the apices of the tooth and we have seen some as i showed in my presentation and we could show you some more that's even worse than that the radiolucencies or you just don't see any bone at all in those areas and then a lot of times that right along the ramus is very very thin And the trabecular pattern is gone. And generally, if you look at the distal of the first molar, there's always a six-millimeter pocket. And that pocket can come and go. But that's usually a tattletale sign. And in most of our sickle cell patients, that first molar, that six-year molar, is the one that they lose actually seeing pockets in a bifurcation. And then what we've noticed, we have a pedodontist that we work with, and what we've noticed is that a lot of times they think that they have juvenile periodontitis, and all it is is just a six to eight millimeter pocket on a patient with sickle cell disease or the sickle cell trait. And what do you do for that pocket as far as treatment? Well, generally what we'll do is we'll scale it out really good and flush it and put some arresting down there, you know, try to save it as much as we can. And then another thing we try to do is stabilize the bite because, you know, if they are not occluding right, it shakes that tooth loose. So there are a lot of things that you can do. But what we found, the best thing is just prevention. Now, you mentioned, Linda, some radiographic signs of sickle cell disease, and you emphasize the radiolucency that shows up. in the trabecular bone but there's also a stepladder configuration that's very indicative of sickle cell disease well it we they call it a stepladder we used to call it the h pattern 50 years ago i've been looking i've been following this for about 50 years 50 years ago they call it an h pattern but now it looks just like a stepladder And it's just the way the bone forms. It forms in layers instead of, you know, with a trabecular pattern, it's like all integrated. I say it looks like a condensed spider web. Right. Yes. And a sickle cell patient, it's like layers of bone and it looks just like a stepladder. Right. And that's a telltale sign right there radiographically. Yeah. When you see that, there's a high chance that that patient has sickle cell disease. In a given year, Linda, just curious, how many patients does your practice see that have either sickle cell disease or sickle cell trait? In a given year, we may see anywhere from 50 to 60. Do you think that that's something specific to your practice? Because that sounds like it's pretty high for a dental practice. Well, what we have is we have a nonprofit called The Potter Smile. And this is what we do. That's one of the things that we do. In fact, we're the only 501c3 in the United States of America that addresses the dental needs of those patients with sickle cell disease and the sickle cell trait. So we have a large clientele of sickle cell patients that comes in here. And they travel a long distance to go to you to get care? Travel from New York, Virginia, California. I had no idea these patients had to travel so far to get dental care. Your office is in Texas, right? Our practice is in Houston, Texas, in the medical center. And we are the dedicated dental office for sickle cell. The name of our practice is Fantastic Smiles of Houston. And it's Dr. John Marinci, and he is our potter. And Dr. John Marinci is a Harvard graduate, and he's one of the top 10 prosthetic dentists in the United States, which is the actually, we have to rebuild. mouths. If you saw the patient, I don't know if I used Walter, our poster person, Dr. Marinci took that patient, he only had six teeth, and we rebuilt the whole mouth. He couldn't have implants, no. But implants can be done under certain circumstances on patients with sickle cell disease. Obviously, you need the okay on the medical side and the bone needs to be up to par. Well, not only the medical side, but what Dr. Marinci does is he sends them and we do a bone density test to make sure that they have the density to do it. And then when he places the implant, it has to be done very delicately because you don't want to destroy a lot of tissue to cause osteonecrosis. Because we had one patient that came to us. And she had gone to another dentist, and they tried to do an implant twice, and the implant failed. It kept setting up an infection because the tissue was dying around it, and they would put on antibiotics and everything, and she just could not have an implant. So we ended up having to do a three-unit bridge on her. Yeah, it seems to me that the confidence level of the dentist is not high when it comes to sickle cell disease, and it's obviously linked to the lack of training that dentist has and understanding of the disease. And unfortunately, this results in patients being turned away, and then they have to travel long distances. like from New York to Houston to go to your practice. And really what would solve that problem would be a lot of these dentists getting additional training and developing a relationship with the medical community so they could manage the risks of these patients and get the general dentistry done. Yes, in the normal fashion. But the problem is most of the time when the dentists find out they have sickle cell disease, they don't want to touch it. And then also a lot of times they don't understand the disease. See, one of the things that we always talk about in our lectures, a sickle cell patient has a bad record of no-shows. But generally, if we have a patient that no-shows, we know that patient is in the hospital and has had a crisis because there's a high level of anxiety that goes along with coming to the dental office, and anxiety can actually throw you into a crisis. So generally, if they don't make it... I have a patient, I know she's, okay, she's in the hospital, she's had a crisis, you know what I mean? So they have a high no-show ratio. So between the no-show and the high risk and all the work that has to be done by communicating with the hematologist and everything else, the dentist feels it's better for everybody to refer that patient out to somebody else. Yes. And then a lot of times the patients would rather come to us and see a regular dentist because what they're looking for, sometimes they get here, Phil, and they're so relieved. They say, finally, finally, we have a dentist that understands what's going on in my body. Dr. Princey, he worked his way through dental school at a blood bank. So he has an impeccable knowledge of the blood diseases and sickle cell. How many dental practices are like you? in this country that specialize? We're the only one that we know of and we've researched it and we've had it researched. We're the only one. So what about the other 50 to 70,000 sickle cell patients that are floating around going to dentists? They're obviously getting treated somewhere. Well, now you do have that dental specialty. It's a dental specialty clinic that does treat them, but they're only in, I think they're, I don't quote me, I think they're in Richmond, but no, you don't have a lot of dentists that know anything about sickle cell disease. For about three or four years, that's what we did. We lectured at the different dental societies to try to educate these dentists on the clinical manifestations of sickle cell disease in the dental patient. How did you get so involved with it, where you're so engaged? Well, 50 years ago, I've been looking at sickle cell for 50 years, and one of the things that really got me engaged was my mother passed away at 72, and we didn't find out she had full-blown sickle cell disease to two days before she died. And see, my mother was born in 1912, and they didn't discover sickle cell disease in America until 1910. And then she was having a crisis after crisis. We did not know it. Her doctor didn't know it. She was going to a doctor. They knew she had severe anemia. But they did everything to treat her. But they didn't know she had sickle cell anemia. That's an amazing story. And the fact that you have this connection to sickle cell. just this passion to really understand the disease and what the patient is actually going through. It must be a tremendous feeling of relief for that patient to sit in your chair in your office to feel like these dentists actually understand what I'm going through. And they're very, very willing to help me in any way possible to improve my oral health in collaboration with my medical doctors. Yes. And we won't do anything until we get written. You know, not horrible. We had one hematologist called and gave us the A-OK to do some things. And we said, no, we need it written because if anything goes wrong, we have to cover ourselves. You know what I mean? So we have to have written verification that it's OK. And again, as I was telling you, sometimes they come in and they know they know how their body feels when they're getting ready to go to go into a crisis. So if they tell us that. What we do is we try to make them comfortable if we have to give them something for the pain and things like that. We collaborate with their hematologist, we get them stabilized, and then we bring them back. And then especially if they got to have a blood transfusion, we generally can do just about as much as we can do after 14 days after they have a blood transfusion because their hemoglobins are high and, you know, they had clotting factors high so we can get in there and do a lot more. Yeah. So in closing, Linda, and it's been very enlightening to hear your story. in this specific field of dentistry. What would you recommend to our listeners if they were interested in pursuing treating patients with sickle cell disease? Well, the main thing is that if they understand the disease and they understand the medication and they understand the precautions that they need, they shouldn't really have a problem. And then with most sickle cell patients, you should take them slow. Their appointments should be short. You don't want to give them a long, drawn-out appointment. And then you really have to be empathetic to what they're going to. And then a lot of times, if they come in with a toothache, for God's sake, don't pull the tooth just because it's a toothache, a lot of them are having a crisis around the tooth. And then once that crisis is over, that tooth is fine. But if you go pulling the tooth trying to stop the crisis, then they don't have teeth to eat with. horrible to think that that actually happens yeah that's why yeah it's our responsibility as dental professionals to stay educated there's so much continued education out there after dental school that we have to take advantage of that and this is just one example of that Linda thank you very much for your input it's been a very nice conversation God bless you for all the hard work you're doing over there and the practice that you work in that you're helping these patients in the way that you do. It's a great story. Thank you very much. Keep up the great work. Okay, thank you. Bye-bye.