Amy Moore, MD, a Nerve Surgeon at Washington University St. Louis talks about AFM, how the nerve surgery is executed and how this disease effects families.
Interview conducted by Ivanhoe Broadcast News in March 2019.
What is AFM?
MOORE: Acute flaccid myelitis is a disease process that affects the spinal cord. And predominantly it is affecting children who are under the age of 8, but we’ve seen adults as well. And it’s rare. Only about 500 cases have been diagnosed since 2014. But unfortunately we’re seeing an uptick in incidents. And I just want people to be aware that there are options for these children.
It doesn’t present all the same with all these kids? Some are worse than others, is that right?
MOORE: Correct. What we do know is that the children have some type of respiratory or G.I. illness early on followed by paralysis, whether it’s a single limb or multiple limbs. Some children, it affects their ability to breathe and others even to hold their neck up straight.
Who is at risk for this?
MOORE: Unfortunately we have not been able to pick out unifying features for this diagnosis. But usually it’s healthy children who are coming down with acute flaccid myelitis.
We don’t know what causes it?
MOORE: We don’t. We think it’s associated with a viral illness. Enterovirus D68, A21, coxsackie virus – these are your common colds. But unfortunately the families will get sick and only one child will come down with the paralysis. So we’re not really sure.
Was the first case seen in 2014? This has not been identified before?
MOORE: It’s the first time it was recognized as a disease entity in 2014. I don’t think we know if it had been just something misdiagnosed previously.
I see. But still very rare. Obviously the common cold and these viruses are not very rare. So millions of people are going to get these and have no issues.
MOORE: Correct. The importance is understanding that if your child is not acting normal and is not able to move and their limbs are feeling weak, that’s the common story I’m getting from my patients is that they have a respiratory infection, and the inability to move their arm or they are tripping because their leg’s not working. They need to head to the emergency room.
You said it’s an infection of the spinal cord?
MOORE: It’s a virus that attacks the anterior horn cells of the spinal cord, which then leads to the motor dysfunction. So where it hits along the spinal cord is variable. But unfortunately it results in lack of motion of either an extremity, a muscle group, even disability of the diaphragm to work.
Do most of the kids have some degree of mobility issues with this?
MOORE: Yes. I’m not on the front line unfortunately. But of the patients I’ve evaluated, all of them had have at least one extremity involved.
Before we get into nerve transfer, what has been going on with these kids? Has there been any treatment to help the mobility or does it sometimes recover on its own?
MOORE: Fortunately, many of the children are recovering some part of their function that they’ve lost. What I’ve been able to predict is that the extremity that’s affected first is the last to come back and is the most severely affected. They’re still recovering at six and eight months. And so we’re holding off on interventions right now.
It’s not the earlier the better. You’re waiting to see how much they can get on their own.
MOORE: Correct. So before with nerve injuries, if they have no function, we want to be intervening early. Because regeneration happens at a millimeter a day, an inch a month, a foot and a half a year. So if the injury is at the spinal cord, to get to the muscles of the arm before the muscles no longer receive the input, we want to be there early. The difference with AFM is that we don’t know the extent of the injury. And these children are going to recover. But because we haven’t been looking at these children, we don’t know expectations of when they will recover. Our goal and part of the task force through the CDC is to figure out when, as surgeons, should we be operating. And those discussions are ongoing.
That’s still not determined. There is no cut and dry – this is when we should intervene. This is a learning process for you too as well as the patients and the parent.
MOORE: Absolutely. There’s so much to learn and to understand with this disease process. And from a surgical intervention, we’ve been successful seeing outcomes. But we got to those children from the 2016 epidemic over a year out. And so this gives us an opportunity for all of these children. And as soon as they plateau, we intervene. And hopefully that’s going to give us better outcomes.
When you say nerve transfer, what are we talking about?
MOORE: A nerve transfer is taking a nerve that’s powering a muscle – and there may be more than one muscle that does the same movement, such as two muscles that bend the elbow – multiple nerves – to a muscle that wiggle the toes. And we can take one of those nerves and transfer it to the muscle that’s not working because of the injury at the spinal cord. And we cut it and remove it and I suture it under a microscope. And at a millimeter a day, inch a month, foot and a half a year, we get muscle function.
Wow. So these are extra nerves. You’re not harming another part of the body or a less important nerve?
MOORE: It’s less important.
MOORE: If our goal is to get back use of your upper extremity, we want the elbow to bend so you can feed yourself, then the shoulder, then your hand motion. In your legs, we want to stabilize the hips, we want to be able to use the quads, which are the muscles that extend the knee and bend the knee. And so that will help us move to walk. And so our focus has to be, I don’t need your toes to wiggle, I want you to be able to stand. And so we just use a hierarchy. And most of the time, these children don’t even know that I’ve taken the extra nerve.
Are there different places that you’re taking them from?
MOORE: The reality of the surgery is depending on what muscle I want to reinnervate. Meaning what muscle do I want to bring the electrical activity to allow it to move. And the good news is all the nerves come from the spinal cord and they all run together. Depending on where the nerve to the muscle is, that’s where I can tap into the live wire and make the transfers. I don’t have to be at the toes stealing the toe nerve, I have to just be where the live wire is active and I can transfer it at that level.
And transferring it is just exactly what you said – cut it, move it, suture it.
MOORE: Exactly. Right.
Wow. How come people don’t do this for other people with paralysis?
MOORE: Yes. We actually do.
MOORE: Nerve transfers were designed and first really came into use in 1998 for injuries to the plexus, that means the brachial plexus, which is nerves that allow you to move your arm. So trauma was our original use of these nerve transfers. And all we’ve been doing for AFM is applying these principles. Some of my partners have used it for patients with spinal cord injury. The idea is these nerves to the muscle, if we can get there in enough time, they will work.
I see. How many surgeons are doing nerve transfer for this condition?
MOORE: I think there’s probably many that I don’t know of. But 15 of us are on this surgical workgroup through the CDC. I’m sure there’s many more who see these patients. But there’s a focus of us who are really becoming centers.
A lot of patients around the country are getting referred to your 15, right?
MOORE: Absolutely. Yes.
How many kids have you treated here?
MOORE: I’m up to 13 surgeries, 12 from the 2016 outbreak. And I have almost 10 surgeries scheduled over the next two months. It’s just starting with these children. But even a patient last week that I was going to operate on was showing recovery. So we pushed it off because I really want him to plateau. So I know that I’m intervening at the right time and doing the right thing.
What are the ages of the kids you treated?
MOORE: The youngest I treated was 23 months, the oldest was 14.
Wow.
MOORE: But most under the age of 7. They mostly are sitting in that 2 to 6-year-old range.
How long is this kind of surgery?
MOORE: The surgery itself, the operation, takes all day. It’s not that it is tremendously long in the sense of hard to do, it’s just getting the right exposure, working under a microscope – very detailed work – and then time for me to think. What do I have? What’s working, what’s not? So the intraoperative analysis just takes time. I tell the parents it’s a long day, but the kids do great. They do great.
And what have you seen so far in these kids? The kids you treated in 2016, tell me about the results you’ve seen.
MOORE: With nerve injuries, the recovery is long. And we’re just now being able to see the results. And what are those results? Three of the children who came to me in wheelchairs are actually able to ambulate with assist device. I’m not making them normal. But I’m waking up muscles to make them stronger so they can stand and so that they can walk and or just even move to transfer themselves. I’ve seen children who are able to bend their elbows from those that I did from the upper extremity. But honestly, we are still waiting to see the results from those first surgeries.
Some of these children are walking with a device, an assistant?
MOORE: Assist device. Such as a walker or a cane or with braces. Anything is better than just being able to sit in that chair. And we love this quote from Sterling Benelle, “those who have nothing, a little’s a lot.” And that’s what we’re seeing in these kids. You just give them a little and they can do so much. It’s incredible.
It’s been three years, roughly, since you’ve treated a lot of the kids from 2016. Do they continue to get better every time you see them? Or do you think after a year or so, we’re not going to see any more improvement?
MOORE: No. For nerve injuries and nerve recovery, we give up to four years to say this is as good as you’re going to get. The surgery is mostly done about a year to 18 months ago because we got them so late. Although the diagnosis was in 2016, I wasn’t operating until 2017. So we’re still in that window. I do think the regeneration with AFM kids is a little slower. I would have expected for them being so young to see these instant results, that may be because of the virus or the entity of where the virus is attacking. But the good news is we’re seeing results. Until we get more time, will we know more.
When you say the virus, do they still have it? Is it like a cold? It comes and goes and then it’s gone. Or whatever attacked their spinal cord is always going to be there?
MOORE: That’s a great question. I don’t think we know. I think it’s that the body fights the virus and then allows it to get better. But the damage is already done. These children who have been diagnosed late I don’t think have active virus and it doesn’t mean that they don’t have the paralysis. So I would say that the virus has been shed, but unfortunately, the damage is done.
The virus that causes this is clearly contagious. You can spread that to a sibling. You can spread that to a friend at school. But AFM is not contagious, right?
MOORE: Correct.
MOORE: We don’t understand why, take for example, a family of five. They all have their runny nose, the cough, but only one child becomes paralyzed. We have not seen multiple children of a family be infected with the paralysis component. So there has to be some type of immunological response that that child is susceptible to. And that is where the research has to happen. We need big brains thinking about it. And raising more awareness that this is a diagnosis we need to know more about.
Is there anything else that’s helping these besides nerve transfer that’s helping these kids ambulate again? Or is this their only option?
MOORE: Well, their early treatment is using immuno modulators such as IVIG, plasmapheresis and steroids. And we hope that that is improving their outcomes. But we don’t know. There’s not a shot or an immunization that you can get to prevent this or to cure it.
For the kids who have it and they are paralyzed, they’ve lost mobility, this right now is the best option for them? And for a lot of kids it will work to some degree, but does it not work in some kids?
MOORE: We don’t know. There’s not enough children to figure out who is this going to help and who will it not help. The idea is that the virus attacks at the spinal cord level. But we don’t know. It may be along the entire nerve and maybe at the muscle as well. Our goal from the surgeon workgroup from the CDC is to take samples of the nerves of the muscle and study it. And until we can and understand more, will I be able to say, well, I can predict that this child will recover if I do a nerve transfer, this one may not. There has to be a genetic component. I just don’t think we understand enough yet.
This might not sound like much, just to bend an arm again, but for a child, that means everything. What are you hearing from the kids? And what are you hearing from parents?
MOORE: I still cry with all of the families when I see some motion after a nerve transfer or the child being able to walk down the hallway towards me. Brandon’s (ph) a great example. Every time I see him, I’m just grateful to be part of it. He could go out and play with his friends and not be worried about where his chair can go. It’s really hard to put to words, that’s why I stumble because I just feel very grateful. Grateful to be part of it.
Wow. And for the parents, what do they say to you?
MOORE: You know, if I reflect on the parents that I operated on, they were themselves happy that we took the leap together. Because there was a big unknown if it would help or not. And we knew we were doing something. And I think that felt good. My practice is talking to them before they even come to St Louis and really agreeing that their child may be a candidate. But then this is a leap of faith and it may not help. But are we OK to undergo the risks of anesthesia. I think everybody’s been really happy and excited. I would say that’s the biggest response is hope. It’s going to help them.
Awesome. You’re a mom?
MOORE: I am.
You seem very passionate about this. You’ve got this very maternal instinct in seeing these kids who are the ages of your kids.
MOORE: That’s right. I think as a mother of three, it is hard to separate yourself from these families. Although many of the stories have similarities, every time I hear the parents give their experience, it wrenches at the heart. And I’m like I’m going to help your kid. And that’s what comes out. And you meet the children. And they are just fighting. And one child can’t breathe, can’t hold their neck up. But they’re trying to participate in my exam. And I’m just hopeful that I can help them in some little way.
What’s the next step?
MOORE: I think there are many next steps. We need to categorize which children we’re operating on and what are their outcomes? And follow them over time. We need to take the nerve samples, little pits of nerve, and study it under the microscope to figure out are there cells that are different? Is there something we can intervene early? Can we create a vaccine that fixes it? We also need to come up with more creative ways to potentially help more muscle groups. Because right now we’re limited by what’s working. But if we could save the muscle to give it more time that we can provide more function to these children, that’s the goal.
Of the few articles I’ve read, almost everyone calls it a polio-like illness. That’s what reporters have grabbed onto, polio-like illness. That scares people. Especially scares grandparents because they lived through that. Right. Is this similar? And could this really could get out of control. This really could be a major epidemic. Or are you seeing this level off?
MOORE: Well, if we look back to 2014, we see that there’s upticks every other year of diagnoses. And that’s been pretty stable. What’s different now is we’re more aware. I think where we were missing diagnoses in the past or calling it something else, like a transverse myelitis, now AFM is more at people’s forefront. I think we’re going to see higher numbers. But not necessarily that there’s more kids being affected, it’s just they’re being recognized. And I don’t know that we’re in this epidemic. But we’re only working on about four years of data. So I think there’s just so much more to know.
Is there a theory why it’s every other year?
MOORE: No. We don’t understand. Or at least I don’t understand. And I haven’t heard even speculation why. But it’s very interesting.
I did I read this, where there’s also a season for it?
MOORE: Yes.
Is it the fall?
MOORE: All of these children from 2018 are in September. Maybe early September or late September. I have one child from October, 1 child from November. But the most are all within a week in September. All over the country, week in September. Crazy.
Wow.
MOORE: Crazy. How? Why? Where? What? I mean, it is every question. And we have to just chip away. And there’s good, great, really smart people working on it. And that’s great. And there’s the support that, let’s talk about it. Because for that one child it matters.
The parents of AFM are unique to any patient population that I’ve treated. They have a Facebook group. And they talk. And they understand. So my patients who are coming, the families have already, started naming other kids and this is what they had and their questions. But the support is something just so notable that I’ve been really impressed with. These parents help each other and that’s what it’s all about.
They need to know they’re not alone.
MOORE: And to have options and to support each other all across the country and have new friends going through the same but sort of different experiences, I think, is just incredible.
Most of the children come from all over the country. And I haven’t had the chance to see them back. They go to their local physicians who sent them to me. And I get videos. Or I see the Facebook page.
But they do come back for follow up visits with you?
MOORE: They do. Most do. Some of them are following up at closer centers. So they came for their surgery, but because of the financial means, it’s just difficult to come back and see me. And so thankfully we also have a network of doctors who are willing to step up to the plate.
END OF INTERVIEW
This information is intended for additional research purposes only. It is not to be used as a prescription or advice from Ivanhoe Broadcast News, Inc. or any medical professional interviewed. Ivanhoe Broadcast News, Inc. assumes no responsibility for the depth or accuracy of physician statements. Procedures or medicines apply to different people and medical factors; always consult your physician on medical matters.
If you would like more information, please contact:
Judy Martin, Director, Media Relations, Washington University St Louis
314-286-0105
Sign up for a free weekly e-mail on Medical Breakthroughs called First to Know by clicking here
