In patients with sickle cell concurrent pulmonary issues such as asthma, allergies, sleep apnea and acute chest syndrome are common. Alvin Singh, MD, is helping save these sickle cell patients an extra trip to the hospital through the multidisciplinary sickle cell pulmonary clinic. Dr. Singh ensures that patients are well oxygenated, ventilated and that pulmonary issues don’t affect their sickle cell disease.
Listen in as Alvin Singh, MD explains that although acute chest syndrome is the second most common complication of sickle cell disease, it can be difficult to determine the source of the problem, especially in young patients with a predilection for asthma.
Multidisciplinary Sickle Cell Pulmonology Clinic
Featured Speaker:
Learn more about Alvin Singh, MD
Alvin Singh, MD
Alvin Singh, MD, is an Assistant Professor of Pediatrics at the University of Missouri-Kansas City School of Medicine. He received his degree from St. George’s University School of Medicine and completed his residency in pediatrics at the University of Nebraska/Creighton University. He is fellowship trained in Pediatric Pulmonology at the Children’s Hospital of Pittsburgh.Learn more about Alvin Singh, MD
Transcription:
Multidisciplinary Sickle Cell Pulmonology Clinic
Dr. Michael Smith (Host): Our topic today is a Multidisciplinary Sickle Cell Pulmonary Clinic. My guest is Dr. Alvin Singh. He’s an Assistant Professor of Pediatrics at the University of Kansas City School of Medicine. Dr. Singh, welcome to the show.
Dr. Alvin Singh (Guest): Thank you. I appreciate it.
Dr. Smith: Let’s first just talk about Sickle Cell for a moment. We know that there’s a lot of comorbidities with the disease. Tell us a little bit about Sickle Cell, the comorbidities, and why it’s necessary to have such a multidisciplinary clinic like you do.
Dr. Singh: Of course, well, as we all know, Sickle Cell disease is a hemoglobinopathy that changes the red blood cells, which are essential for hemoglobin and oxygenation. When these cells change, they can affect various parts of the body – bone marrow, for instance, which leads to pain crises in various areas of the body – but will concentrate in the chest where it can affect the pulmonary vasculature, cause possible spasms and symptoms that involve chest pain, shortness of breath, hypoxemia – which usually is diagnosed as acute chest syndrome.
I think the biggest problem with acute chest syndrome is – is it inflammation? If it’s inflammation, is it inflammation that is typical of asthma because these patients have the same symptoms, right? They cough, they wheeze, they have hypoxemia, shortness of breath, and chest pain, and some may or may not respond to inhaled bronchodilators. In a lot of these young, Sickle Cell patients, the real key is teasing out whether or not these patients with recurrent hospitalizations for, quote, acute chest – is it due to the acute chest? Is it due to asthma? Is it due to a combination of both? Is helping one helping the other? I think that’s the biggest question that hasn’t been looked at very rigorously, and that’s where we’ve started to help and see and manage these patients.
Dr. Smith: What is your opinion, then, Dr. Singh – how many patients are coming in being diagnosed with acute chest syndrome and it really is just asthma? Do you think it’s a significant number?
Dr. Singh: I think, from my limited experience, I would probably say maybe half. A lot of it is teasing out a history of asthma. Is there a strong family history – a maternal and paternal history of asthma? Is there a significant response and improvement with inhaled bronchodilators or systemic steroids? Do PFTs or pulmonary function testing show bronchodilator reversibility that’s typical in asthmatic patients? Do these patients have a lot of eczema, or allergic rhinitis, or atopy that can make asthma worse?
Half of my clinic visit for initial evaluation is just really teasing that out and looking at the charts to see how they responded to medication when they were admitted for acute chest syndrome. Do they have wheezing on exam? Do they have significant pulmonary function findings that would make me say, “Okay, why don’t we trial a daily inhaled corticosteroid and see how you do?” I can, with confidence say that my Sickle Cell colleagues have noted a decrease in hospitalizations for acute chest when I treat them aggressively for asthma. Now, am I treating asthma? Am I treating the acute chest? Who knows?
Dr. Smith: That’s interesting, right? You mentioned the pulmonary function test. How do you determine the source of the problem if the patient is just too young to undergo the PFT? What do you do in those cases?
Dr. Singh: Right, it’s really the same kind of history and physical more than anything. A few patients have chronic lung disease of prematurity, so that sets them up to having asthma-like symptoms on top of their Sickle Cell disease. Sometimes half of it is just trying some medication that you treat for asthma and seeing what the response is. Are they coming to the hospital more often? When they get a cold, are they coughing and wheezing and ending up being placed on a course of oral steroids? Do they actually respond to inhaled medication to the point to where they have a cold and can be managed at home with no issues? A lot of it is trial and error, a good history, a good physical exam, reviewing the chart to see if there are any patterns consistent with bronchodilator reversibility or treatments with asthma and whether or not they responded to that treatment.
Dr. Smith: Let’s talk about sleep apnea in the context of Sickle Cell. How often do you see it, and what kind of role do you think it plays in Sickle Cell disease? What are the challenges in treating sleep apnea in young patients?
Dr. Singh: I think that’s a great question. I tend to – the more I’ve been seeing these patients -- I would say about maybe half – it’s pretty frequent in terms of the occurrence of either sleep apnea symptoms, frequent snoring, constant nasal congestion or mouth breathing, enlarged tonsils on exam. Usually, I will either screen for a sleep study or start them on some medication such as a nasal steroid to help shrink those turbinates and possibly shrink those tonsils and help – and just ask the parents, “Is the snoring getting better?”
If it’s not, then I obtain the sleep study to see how bad the obstruction is. Half the time some of these kids will be hypoxemic at night and just need some Oxygen, and they do fine – because of their Sickle Cell disease. I think screening with a good sleep questionnaire during the initial evaluation, trialing medications, getting a sleep study or polysomnogram, can help tease these patients out. The best-case scenario is they need a little bit of Oxygen. More significant cases would involve an ENT evaluation for an adenotonsillectomy, which we’ve done on a few patients.
Dr. Smith: You have a really interesting clinic here. It’s known as a multidisciplinary pulmonary clinic – walk us through what might be a typical patient visit to your clinic – exactly who is involved, what healthcare practitioners are going to see the patient at this visit, and just walk us through that.
Dr. Singh: Yeah, absolutely. The patients will get checked in. I usually come to the hematology clinic and just set up shop there and usually, one of our respiratory technicians, to perform pulmonary function testing, will also come along with one of their portable PFTs and set up shop as well. Usually, they’ll get checked in. A nurse will see them first, do intake, do medication reconciliation, and after that, usually, our respiratory technician will grab them if they’re old enough and do pulmonary function testing.
Depending – we usually see probably seven or eight patients in a half day – it’s one-half day a month. It’s usually the fourth Tuesday. While that’s going on, if there are any other patients that come in at the same time, we just talk amongst each other with myself, the hematologist, the nurse practitioners, and decide who gets to see who and when. We usually do a preclinic huddle to decide who needs to see pulmonary more so than hematology and we just go. I would probably say it’s a nice controlled chaos situation usually involving myself, hematology -- and then when we’re all done seeing the patient, we’ll constantly communicate each other what documents they need. I will usually prescribe the respiratory medication and hematology will prescribe the rest.
I review PFTs with them, and we go over an asthma action plan or a pulmonary action plan. If they’re on inhaler medications, we review the techniques for inhaler medications with the respiratory technicians, and usually, the patients go on their way after. And then after clinic, we have a post-clinic huddle just to talk about what we were thinking, when we need to follow up, what tests I need to get or what hematology needs to get in order to coordinate labs or other studies that might be needed.
Dr. Smith: Okay, and this kind of approach, how do the parents feel about it? Is there a good feeling about the care of the patient and they like this kind of approach where they can come in, and everything can get done at once? This must go over really well with everyone?
Dr. Singh: Right, exactly. Follow-up is difficult, and one of the main reasons is transportation and coast of transportation. A lot of families are very thankful that it’s almost like a one-stop-shop. You can get your asthma or airway inflammation addressed as well as your Sickle Cell disease and anything else. If you need to see nutrition, social work, anybody else that can be seen in the multidisciplinary clinic is there. You can get labs drawn at the same time. They are really appreciative. I don’t think we’ve got any negative feedback. I think it’s all been very positive.
Dr. Smith: Yeah, it makes sense. When you hear about these types of clinics that are able to do everything in one day – cut down on the patient visits, it makes things easier. Of course, when you hear about it, it makes sense, and it’s like why haven’t we been doing this all along, right?
Dr. Singh: Right, exactly.
Dr. Smith: [LAUGHS] Dr. Singh, I want to thank you for coming on the show today.
Dr. Singh: Well, great, yeah, absolutely.
Dr. Smith: And thank you, so much, for the work that you’re doing at Children’s Mercy. You’re listening to Transformational Pediatrics with Children’s Mercy Kansas City. For more information, you can go to ChildrensMercy.org, that’s ChildrensMercy.org. I’m Dr. Mike Smith. Thanks for listening.
Multidisciplinary Sickle Cell Pulmonology Clinic
Dr. Michael Smith (Host): Our topic today is a Multidisciplinary Sickle Cell Pulmonary Clinic. My guest is Dr. Alvin Singh. He’s an Assistant Professor of Pediatrics at the University of Kansas City School of Medicine. Dr. Singh, welcome to the show.
Dr. Alvin Singh (Guest): Thank you. I appreciate it.
Dr. Smith: Let’s first just talk about Sickle Cell for a moment. We know that there’s a lot of comorbidities with the disease. Tell us a little bit about Sickle Cell, the comorbidities, and why it’s necessary to have such a multidisciplinary clinic like you do.
Dr. Singh: Of course, well, as we all know, Sickle Cell disease is a hemoglobinopathy that changes the red blood cells, which are essential for hemoglobin and oxygenation. When these cells change, they can affect various parts of the body – bone marrow, for instance, which leads to pain crises in various areas of the body – but will concentrate in the chest where it can affect the pulmonary vasculature, cause possible spasms and symptoms that involve chest pain, shortness of breath, hypoxemia – which usually is diagnosed as acute chest syndrome.
I think the biggest problem with acute chest syndrome is – is it inflammation? If it’s inflammation, is it inflammation that is typical of asthma because these patients have the same symptoms, right? They cough, they wheeze, they have hypoxemia, shortness of breath, and chest pain, and some may or may not respond to inhaled bronchodilators. In a lot of these young, Sickle Cell patients, the real key is teasing out whether or not these patients with recurrent hospitalizations for, quote, acute chest – is it due to the acute chest? Is it due to asthma? Is it due to a combination of both? Is helping one helping the other? I think that’s the biggest question that hasn’t been looked at very rigorously, and that’s where we’ve started to help and see and manage these patients.
Dr. Smith: What is your opinion, then, Dr. Singh – how many patients are coming in being diagnosed with acute chest syndrome and it really is just asthma? Do you think it’s a significant number?
Dr. Singh: I think, from my limited experience, I would probably say maybe half. A lot of it is teasing out a history of asthma. Is there a strong family history – a maternal and paternal history of asthma? Is there a significant response and improvement with inhaled bronchodilators or systemic steroids? Do PFTs or pulmonary function testing show bronchodilator reversibility that’s typical in asthmatic patients? Do these patients have a lot of eczema, or allergic rhinitis, or atopy that can make asthma worse?
Half of my clinic visit for initial evaluation is just really teasing that out and looking at the charts to see how they responded to medication when they were admitted for acute chest syndrome. Do they have wheezing on exam? Do they have significant pulmonary function findings that would make me say, “Okay, why don’t we trial a daily inhaled corticosteroid and see how you do?” I can, with confidence say that my Sickle Cell colleagues have noted a decrease in hospitalizations for acute chest when I treat them aggressively for asthma. Now, am I treating asthma? Am I treating the acute chest? Who knows?
Dr. Smith: That’s interesting, right? You mentioned the pulmonary function test. How do you determine the source of the problem if the patient is just too young to undergo the PFT? What do you do in those cases?
Dr. Singh: Right, it’s really the same kind of history and physical more than anything. A few patients have chronic lung disease of prematurity, so that sets them up to having asthma-like symptoms on top of their Sickle Cell disease. Sometimes half of it is just trying some medication that you treat for asthma and seeing what the response is. Are they coming to the hospital more often? When they get a cold, are they coughing and wheezing and ending up being placed on a course of oral steroids? Do they actually respond to inhaled medication to the point to where they have a cold and can be managed at home with no issues? A lot of it is trial and error, a good history, a good physical exam, reviewing the chart to see if there are any patterns consistent with bronchodilator reversibility or treatments with asthma and whether or not they responded to that treatment.
Dr. Smith: Let’s talk about sleep apnea in the context of Sickle Cell. How often do you see it, and what kind of role do you think it plays in Sickle Cell disease? What are the challenges in treating sleep apnea in young patients?
Dr. Singh: I think that’s a great question. I tend to – the more I’ve been seeing these patients -- I would say about maybe half – it’s pretty frequent in terms of the occurrence of either sleep apnea symptoms, frequent snoring, constant nasal congestion or mouth breathing, enlarged tonsils on exam. Usually, I will either screen for a sleep study or start them on some medication such as a nasal steroid to help shrink those turbinates and possibly shrink those tonsils and help – and just ask the parents, “Is the snoring getting better?”
If it’s not, then I obtain the sleep study to see how bad the obstruction is. Half the time some of these kids will be hypoxemic at night and just need some Oxygen, and they do fine – because of their Sickle Cell disease. I think screening with a good sleep questionnaire during the initial evaluation, trialing medications, getting a sleep study or polysomnogram, can help tease these patients out. The best-case scenario is they need a little bit of Oxygen. More significant cases would involve an ENT evaluation for an adenotonsillectomy, which we’ve done on a few patients.
Dr. Smith: You have a really interesting clinic here. It’s known as a multidisciplinary pulmonary clinic – walk us through what might be a typical patient visit to your clinic – exactly who is involved, what healthcare practitioners are going to see the patient at this visit, and just walk us through that.
Dr. Singh: Yeah, absolutely. The patients will get checked in. I usually come to the hematology clinic and just set up shop there and usually, one of our respiratory technicians, to perform pulmonary function testing, will also come along with one of their portable PFTs and set up shop as well. Usually, they’ll get checked in. A nurse will see them first, do intake, do medication reconciliation, and after that, usually, our respiratory technician will grab them if they’re old enough and do pulmonary function testing.
Depending – we usually see probably seven or eight patients in a half day – it’s one-half day a month. It’s usually the fourth Tuesday. While that’s going on, if there are any other patients that come in at the same time, we just talk amongst each other with myself, the hematologist, the nurse practitioners, and decide who gets to see who and when. We usually do a preclinic huddle to decide who needs to see pulmonary more so than hematology and we just go. I would probably say it’s a nice controlled chaos situation usually involving myself, hematology -- and then when we’re all done seeing the patient, we’ll constantly communicate each other what documents they need. I will usually prescribe the respiratory medication and hematology will prescribe the rest.
I review PFTs with them, and we go over an asthma action plan or a pulmonary action plan. If they’re on inhaler medications, we review the techniques for inhaler medications with the respiratory technicians, and usually, the patients go on their way after. And then after clinic, we have a post-clinic huddle just to talk about what we were thinking, when we need to follow up, what tests I need to get or what hematology needs to get in order to coordinate labs or other studies that might be needed.
Dr. Smith: Okay, and this kind of approach, how do the parents feel about it? Is there a good feeling about the care of the patient and they like this kind of approach where they can come in, and everything can get done at once? This must go over really well with everyone?
Dr. Singh: Right, exactly. Follow-up is difficult, and one of the main reasons is transportation and coast of transportation. A lot of families are very thankful that it’s almost like a one-stop-shop. You can get your asthma or airway inflammation addressed as well as your Sickle Cell disease and anything else. If you need to see nutrition, social work, anybody else that can be seen in the multidisciplinary clinic is there. You can get labs drawn at the same time. They are really appreciative. I don’t think we’ve got any negative feedback. I think it’s all been very positive.
Dr. Smith: Yeah, it makes sense. When you hear about these types of clinics that are able to do everything in one day – cut down on the patient visits, it makes things easier. Of course, when you hear about it, it makes sense, and it’s like why haven’t we been doing this all along, right?
Dr. Singh: Right, exactly.
Dr. Smith: [LAUGHS] Dr. Singh, I want to thank you for coming on the show today.
Dr. Singh: Well, great, yeah, absolutely.
Dr. Smith: And thank you, so much, for the work that you’re doing at Children’s Mercy. You’re listening to Transformational Pediatrics with Children’s Mercy Kansas City. For more information, you can go to ChildrensMercy.org, that’s ChildrensMercy.org. I’m Dr. Mike Smith. Thanks for listening.