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Melanie Cole (Host): Acute flaccid myelitis, or AFM, is a rare but serious condition. There's been a sudden rise in cases of this frightening polio-like condition that can leave children paralyzed or with weakened limbs. My guest today is Dr. Sue Mukherjee. She's the Medical Director of Spinal Cord Injury and Rehabilitation at Shriners Hospitals for Children - Chicago. Dr. Mukherjee, first let's start with what is it? What is AFM - acute flaccid myelitis?

Dr. Sue Mukherjee, MD, FRCPC (Guest): AFM is a condition that results in inflammation of the gray matter of the spinal cord in a specific area. Typically it results in weakness of a part of the area in a part of the body. It can result in weakness in the neck and upper trunk and shoulders, or it might affect the lower part of the body. In some cases, it can cause weakness in the body from the neck down.

Host: Do we know what's causing this outbreak?

Dr. Mukherjee: There are a number of viruses that are implicated in the condition of AFM. So initially in 2014 when the CDC first started tracking cases of this, it was described as monoplegia, which means weakness of one limb, and it was occurring after a virus called enterovirus D68. It now seems to be caused by additional viruses including enterovirus 71 as well as a coxsackievirus as well seem to be associated with it. So the children will have typically a viral- what we call a prodrome, which means a viral illness in the days to weeks before the muscle weakness sets in.

Host: Why are only children affected?

Dr. Mukherjee: We don't know. About 90% of cases that are affected seem to be in children, and we don't know why specifically children are more at risk.

Host: Dr. Mukherjee, are there any confirmed cases in Chicago?

Dr. Mukherjee: The cases that we've seen have been a diagnosis AFM from a clinical standpoint, but my understanding is the viruses have not been isolated in those cases, so they can't be confirmed cases. Oftentimes the virus has passed through the body already, so we can't link it directly, but it is becoming a more recognizable clinical picture of sudden onset of weakness following a viral illness.

Host: So, you pretty much started with the symptoms there. So, a sudden onset of a virus. There's a lot of viruses, and parents worry every time their children get a virus. Are there any specific symptoms of the virus that we would notice?

Dr. Mukherjee: Not necessarily. It seems to be a pretty typical enterovirus type symptom, and enteroviruses and rhinoviruses are common in the fall, which is when AFM seems to hit a peak, and the symptoms tend to be upper respiratory symptoms. So runny nose, sore throat, the usual typical cold symptoms, sometimes with some GI symptoms as well, meaning gastrointestinal symptoms with vomiting and/or diarrhea. But those symptoms typically have passed.

In some of the cases that we've seen, interestingly some of the children have had ongoing illness or a lot of difficult recovering, having had bacterial infections after the viral infection had passed, and some of them had received antibiotics. I don't know if that's related or not, but it's an interesting fact that we've been following.

Host: What are some of the complications or long-term effects of AFM? And how do you diagnose it?

Dr. Mukherjee: Well, one of the goals of treatment is to recognize the symptoms pretty quickly, and while the inflammation is active and the antibodies are affecting the nerve tissue, the goal is to try to stop that process quickly. We don't know exactly what the most effective treatments are, but in general treatments that work against antibodies attacking nerve tissue include things like plasmapheresis, intravenous immunoglobulin, and steroid treatment. So they're all being used with effectiveness, and if we can halt the process of the antibodies affecting the nerve tissue, we can hopefully limit the damage, and then try to get recovery in those nerves once the swelling settles down, and the nerves can have a chance to start functioning again.

So the goal is to get these children to rehabilitation fairly quickly once the initial nerve-related treatment has been completed and the workup has been completed, and then to work on gradually getting them to use those muscles as they can in novel ways and in novel environments to help their recovery.

Host: Tell us a little bit about your intensive rehab for these children and the services that you have for acute flaccid myelitis.

Dr. Mukherjee: Yeah, our interdisciplinary rehab program includes physical therapy, occupational therapy, and speech therapy, as well as child life and recreation therapy. So the combination of different modalities and different ways of working on things including trunk strengthening and arm and leg strengthening as well as speech and swallowing issues work together to try to promote and maximize muscle recovery and stimulating the nerves to recover.

We also can use some medications, especially in cases where there is pain, which we've also seen with kids with AFM, and they do benefit from medications that can help decrease nerve pain as well. Whether those influence the ultimate recovery is a little unclear, but we do think it's helpful in general to facilitate their recovery.

Host: I understand you have a good story for us. Tell us about Emily's story.

Dr. Mukherjee: Yeah, Emily is a sweet young girl who sustained weakness. She initially presented with neck pain and subsequent weakness in the arms and in her neck and swallowing ability where she was having difficulty holding her head up, difficulty walking, and she was affected both in the neck and shoulder muscles as well as in the leg muscles. So she could not sit up, she could not walk, she couldn't move her legs very much.

After her initial treatment by neurology, she started showing recovery and the family was seeking rehabilitation care. We discussed it with them, and they decided to come to Shriners once we described our program. Emily and her mom came and stayed with us for several weeks, and she started to show fairly quickly recovery in her legs, which was very positive. She was having some difficulty with neck and shoulder pain, which we were able to treat, and that helped to facilitate her progress with the other therapies.

She ultimately went home to local therapists and was able to work on walking, running, flossing, which was one of her favorite dance moves. She could do that pretty impressively, better than I can, and was able to return to a lot of her prior activities. The most difficult thing for her was activating her shoulder muscles and her deltoid muscles initially, and before she left here, she was starting to show some really nice movements in those areas as well.

Host: Dr. Mukherjee, as we wrap up here in summary, tell the listeners what you would like them to know about AFM, as parents worry when their children get viruses, and you've cleared up everything so nice for us. Please let them know what the take-home message about viruses in general and AFM is.

Dr. Mukherjee: In general, I think we have to be cognizant of the fact that this is still a very rare condition. Although it is alarming in the sense that it does cause significant neurologic issues, it is still very rare. So only one or two in a million children will be affected. The peak times seem to be between August and October, and of course the main ways of preventing are the usual ways of preventing virus transmission, which is avoiding contact with people who are ill, keeping children home when they are acutely ill, and making sure children know to wash their hands and that their families also follow those precautions; the usual viral precautions and washing their hands and avoiding touching their face.

If a child shows signs of weakness after a viral illness, it is imperative to seek out neurologic care quickly as well as following the initial acute treatment to seek out rehabilitation care quickly. An inpatient setting is sometimes the most appropriate so that the children can have a chance to rest in between therapies as well as progress in multiple therapy modes.

Host: Thank you so much, Dr. Mukherjee, for coming on and explaining for parents really what this is. We hear about it in the media, and it can be worrisome, but you've cleared it up so nicely for us and told us about your multidisciplinary team that can really help children with AFM. Thank you again. This is Pediatric Specialty Care Spotlight with Shriners Hospitals for Children - Chicago. For more information, please visit www.ShrinersChicago.org. That's www.ShrinersChicago.org. This is Melanie Cole, thanks so much for tuning in.