Transcription:
Pediatric Lupus

Melanie Cole (Host): There's no handbook for your child's health, but we do have a podcast featuring world-class clinical and research physicians covering everything from your child's allergies to zinc Levels. Welcome to Kids Health Cast by Weill Cornell Medicine. I'm Melanie Cole and joining me today is Dr. Virginia Pascual. She is a Gale and Ira Drukier Director of the Gale and Ira Drukier Institute for Children's Health at Weill Cornell Medicine and Ronay Menschel Professor of Pediatrics at Weill Cornell Medical College. She's here with us to talk about lupus in children. Dr. Pascual, it's a pleasure to have you join us today. People hear about lupus and they don't typically think of children. Can you help us to understand a little bit about this and how it occurs in children, the different types? At what age? Give us a little brief overview of pediatric lupus.

Virginia Pascual, M.D. (Guest): Yes. So I agree with you that people normally think that lupus is a disease of young females and that children might be spared from it. But this is not the case. Unfortunately, up to 20% of patients with lupus started their disease before the age of 16. So that means if there are about 250,000 people living with lupus in the US, up to 50,000 are children.

So it is not a very common disease, but definitely can affect children.

Host: Thank you for clearing that up. How do patients present? Because as I understand it, Dr. Pascual, this is a difficult diagnosis to make in children. Do they go to a dermatologist? Do parents tend to think it's eczema and so they don't pay too much attention to it. Can you tell us a little bit about how it presents, the symptoms and what would parents notice that would send them to their doctor?

Dr. Pascual: Yes, that is a very good question. And I can tell you that what you are asking me, is absolutely one of the major challenges that we have in the field of lupus. Lupus is a great simulator. That means it can give rise to symptoms that may involve basically every organ in the body. I like to say, when I try to explain what lupus is, it's a disease that can affect anywhere from the hair follicle to the deep of the toe. That gives you an idea. When there is such tremendous heterogeneity in the clinical presentation, it's obviously going to be difficult to make the diagnosis. So patients with lupus can start by presenting rashes in the face or in sun exposed areas in general.

And these might be the reason why parents take the children to the doctor. Or the presentation can be very silent and mainly for example, affect the kidney again, in a very silent manner so that when the patient and the parents start to notice that there is something wrong, the kidney might already be involved and there might be inflammation that needs to be treated pretty soon. So, it is hard for me to tell you what are the most, or what are common manifestations of lupus that may take parents to think about the disease, because it is even difficult for doctors, for pediatricians to recognize the disease in many cases.

Host: So once the disease is recognized, what kind of physician is working with children? Are these pediatricians with a specialty in this? Is it pediatric rheumatologists? Speak a little bit about who then the parents would take their children to, and then speak a little bit about how kids typically respond. What kinds of treatments and things are available?

Dr. Pascual: Yes. So obviously the first physician who is going to encounter a child with lupus is a pediatrician and again, it requires skills to recognize that this could be lupus. But there are some blood tests that help in this case. From the pediatrician, as soon as the pediatrician realizes that the disease could be lupus, then the patient would be referred to a pediatric rheumatologist. Pediatric rheumatologists, like myself are pediatricians who have done extra training in rheumatic diseases. And obviously one of the diseases we handle or we treat the most in our field is lupus. So, typically when we encounter a patient with lupus, the first thing that needs to happen is a very thorough examination of every organ system in the body.

Because as I already said, lupus in children, as in adults, can affect every organ from the brain to the skin, to the kidneys, to even the blood vessels. So, after the diagnosis is made, treatment has to, or should start as soon as possible. Because if lupus is not treated, it can progress from one organ to many organs in the body.

How do we treat children with lupus? Well, we treat them exactly in the same way adult patients are treated. The medications that we use to control lupus in general, have a purpose, which is to suppress the immune system because the main problem that patients with lupus, children with lupus have is that their immune system is overreacting, is reacting to the child's tissues like it's reacting to foreign invaders, like it's reacting to viruses, to bacteria, to microbes in general. So in patients with lupus, the microbes are not there. What is there, is an immune system that is targeting the tissues of the patients. So we need to suppress that immune system and the way we do it is using drugs like steroids, which are very broad immunosuppressants. That is the first line of therapy when we encounter a child with lupus. Steroids are effective at controlling the symptoms of the disease. But as many of you know, carry many side effects. So because of this, we do not want to use steroids for a very long time or at very high doses.

So what we do is add very quickly additional immunosuppressive drugs that are going to help us try to get the rid of the steroids as soon as we can. In the field of lupus unfortunately is not like in asthma, for example. An asthma attack is treated with steroids for maybe a few days, few weeks. And that's it. When a patient, when a child comes with lupus and we start the steroids, those steroids are going to be used for a much longer time. And again, in addition to the steroids, we would use other immunosuppressive drugs that are different kinds of immunosuppressants. For the most part, they work and we are able to control the flares of the disease, but, because the disease goes with flares, remissions, and again flares again, we need to treat these children for a very long time.

Host: Can you tell us a little bit about lifestyle, behaviors, things that parents can help their children with at home to make some changes that can help ease some of the burden of what you've been discussing here today. And even being on steroids, for example, comes with its own complications as you say. What can parents do to help this be a little bit less of a on their child?

Dr. Pascual: Yes. Well, parents can obviously, as for every other aspect of childhood, they can do a lot. So, first I would say it's very important to have conversations with their children. It is a very scary situation all of the sudden to realize that your child has a chronic disease. It's a scary for the parents. It's very scary for the children. They don't understand that these kids are going to need to visit their pediatric rheumatologists quite often, that they are going to be treated with these broad immunosuppressants. Sometimes these drugs need to be given through the vein, IV and as you can imagine, this is a scary situation for every child and every parent.

So being very open about discussing first with the pediatric rheumatologists, then at home, discussing with the children, what is the nature of this disease. How to best cope with the challenges that are going to present in the following months and years, and also, to give a positive outlook, we do have today medications that clearly control the disease, and it's very rare to end up with very severe organ damage that is going to require, like in the past, for example, a kidney transplant. This is a situation that today, with the therapies we can for the most part, avoid. So it's very important.

To discuss this openly with the family members, and I would say that is the first step. The second step obviously is to help physically the child, to make sure that the child is going to continue being a child and he's going to be able to do what children want to do. So there are things obviously that need to be avoided. For example, we know that one of the triggers of lupus is exposure to sunlight. So wearing sunscreen, avoiding the hours of the day, especially in areas where there is a lot of sunshine, avoiding that exposure is very important. At the beginning, it's difficult for kids to understand that they need to care about being exposed to sun, but as soon as they understand they can cope with it and it's not such a big deal.

So those are very simple things, but very important things. And from there on again, I would say it depends on where lupus is affecting the child. Sometimes lupus affects their musculoskeletal system, it can cause arthritis. It can cause inflammation in the muscles that lead to weakness. So to learn how to cope with that, to recognize the symptoms and seek treatment as soon as possible. So that in the end, the child is not going to be physically limited or limited as little as possible by lupus. These are all very important components of how to handle lupus in the pediatric age.

Another very important aspect of the treatment and parental control of lupus is going to come with for example, diet. We were mentioning that steroids are the first line of therapy for this disease. Steroids bring many side effects, including retention of liquids or fluids in the body, high blood pressure. So, controlling the diet is going to be a very important factor. A low salt diet, exercise, and making sure that their blood pressure is going to be monitored at home. All these are simple measures that parents can do and are going to help very much in the long run.

Host: As we wrap up, this is such an educational podcast, Dr. Pascual. Thank you again. And can you please just speak about the importance of the medical home working with other professionals? You've mentioned a lot here today, exercise, diet, rheumatology. There's so many different providers involved. It's really a multidisciplinary approach, right from the get go. Can you please speak about the importance of all the providers working together? And if you have any information about alternative therapies. People are always looking for all these other kinds of things and diet and exercise could be considered two of them. But just wrap it up with your best advice about all of the providers to help a child that's growing with lupus.

Dr. Pascual: Yes, that is again, a very good question. So when you have a disease that can affect every organ in the body, you can imagine that a single specialist is never enough. So lupus many times affects the kidneys. Lupus can sometimes affect the brain. It can affect, as I was mentioning earlier the blood cells, the blood vessels, so a multi-disciplinary approach to a patient with lupus, in this case, to a child with lupus is fundamental.

So we pwdiatric rheumatologists work in very close collaboration with nephrologists, with neurologists, with dermatologists, with hematologists. I could go on and on. These are the essential components of the care of a child with lupus. And the good news is, we physicians know that lupus is a difficult disease to diagnose, it's a difficult disease to measure the disease activity, how sick or how much better a patient might be from clinic visit to clinic visit. So seeking the help of our colleagues who are specialists in each of these other domains that can be an affected by lupus is very, very important. So a multidisciplinary team approach to patients with lupus is absolutely fundamental.

Now regarding alternative therapies. I would say that we have mentioned what to me are the most important ones, diet, exercise, avoiding triggers of lupus. Those triggers can be anywhere from sunlight to sometimes infections. So we know for example, that bacterial infections can trigger lupus. So if a child with lupus develops any sign of infection it's very important to immediately seek medical attention. In addition to that, I do not think that any alternative approach, like, medicinal plants or any other approach in this other regard has proven to be efficacious in treating lupus.

I would say that even today in the year 2022, we have to rely on medications that are approved to treat lupus. The good news is there are many new things coming. There are many new forms of therapy, very sophisticated ones that are being tried in adults with lupus. And that as soon as they are approved, to treat adult lupus, would be tried in children with lupus.

And I can tell you that the landscape is phenomenal. There is a lot of research going on in the field of lupus, many new drugs that have shown already in small clinical trials that they can offer help to the patients. They still need to be tried in larger clinical trials, but overall, I am very optimistic that in the next 10 years, we are going to witness a number of drugs that will help us control the disease with hopefully much less side effects than some of the drugs we are using today.

Host: You've made some excellent points. So informative, Dr. Pascual. Thank you so very much for sharing your incredible expertise with us today. And Weill Cornell Medicine continues to see our patients in person, as well as through video visits. And you can be confident of the safety of your appointments at Weill Cornell Medicine.

That concludes today's episode of Kids Health Cast. We'd like to invite our audience to download subscribe, rate, and review Kids Health Cast on Apple podcast, Spotify and Google podcast. For more health tips, go to weillcornell.org and search podcasts. And don't forget to check out our Back to Health. I'm Melanie Cole.

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