Eric Weber, LCPO, FAAOP, National Orthotics Specialist with the Hanger Clinic talks about how ankle, foot orthoses can help patients with CMT (Charcot-Marie-Tooth disease).
What exactly are AFOs and how do they help CMT patients?
Eric Weber: AFO is an acronym. Within the orthotic and prosthetic field, we tend to use acronyms for anatomy. So AFO is ankle, foot orthoses, specifically designed to assist or prohibit specific movements from the middle of the calf all the way through the foot and specifically with CMT, it can do a couple of things. Most folks are trying to prevent tripping, pain or prevent a deformity. It is goal and task oriented and tailored to the patient. Everybody is different, so it is an awfully long answer to why somebody with CMT might use an AFO.
Could you talk a little bit about the science and how it has helped your patient Julie?
Eric Weber: Specifically, the AFO is trying to prevent a weak foot from dragging on the ground or tripping. Further, we assist with standing balance and prevention of falls while standing. Also, it assists in correcting certain alignments and preventing maligned joints from forming. The material science is what has evolved over the years. The field has moved from leather and metal – think of Franklin Roosevelt and his long leg braces that he would wear. We can now use thermal plastics that are formed around the custom model and in the last decade we have come to utilize custom composite materials. These composite materials consist of carbon fibers, Kevlar, polyethylene, and plastics that are all interwoven in a way that we can specifically define the motions that we want to assist or inhibit. In Julie’s case, she utilizes two different types. One that she uses as something that is premade and sized. We then customized it in the laboratory. But it was made specifically to prevent her foot from dropping when she would swing her leg. She really wanted more time at work without having to focus on her walking or standing ability. Then when she realized she was able to stretch out her day a little longer without pain or fear of falling, she wanted to be more active. That is when she said the premade devices were not enough to help her balance when she wanted to go, let us say, for a hike in the mountains. So, we utilized a custom fabricated design, where we were able to redirect the forces that are acting upon her when she is walking and redirect them so that it keeps her balanced and in less pain. It can keep her moving through the day longer and with less energy.
When we talked to Julie, she was telling us she was against them in the very beginning. So, when you came with this customized version for her, how did you convince her to give it a try?
Eric Weber: Recognizing that CMT often does not manifest physical symptoms until the first, second, or third decade of life and the symptoms come on slowly. For a long time, Julie captured what was going wrong with things that she would buy at the drugstore. For example, I feel like my ankle is weak, so I might try an ankle wrap. I feel like I have some pain in my feet, so I will try a pair of squishy insoles in my shoes. That usually lasts for some period but what is remarkable to me is when an individual finally accepts or realizes that they might need some assistance beyond their own capability. So, it is really heartening when I meet somebody like Julie for the first time. I feel I am in a privileged position because here is somebody who has lived with or battled emerging symptoms for a long time. So, when the day comes where they finally say I just cannot trip anymore or I just need to stand at work a little bit longer, that is where I can guide and help. Part of our vision and values, at the Hanger Clinic is to empower the patient. We also give them tools and help them reach their goals. So, it is an important process. So, it is not a conversation centered around convincing somebody. I feel like I am there to listen and find out what are you lacking in your life, what you functionally need, what your goals are. Then we can tailor the treatment to that specifically. So, it is not a conversation centered around convincing the patient. There may be some functional biomechanical aspects that we are hoping to employ. We attempt to give tools to patients to reach and empower them to reach their goals, and we are ascertaining their specific goals. The goal might be to stand a little bit longer at work, to trip a bit less when they walk along the ground, or to increase walking on uneven ground. So, our treatment programs are tailored to help reach the potential they have and specifically in the CMT community that is a very progressive and ongoing target that we are constantly chasing. For me, it is a matter of providing something as little as possible for as long as possible. So, the minimum amount that the patient needs to fulfill their own goals in their life right now, recognizing that will probably change because their needs might be greater later. So, you want to want to provide the tools, knowing that they are putting in all the work. It is our honor to guide them through that process.
So right now, Julie has the braces. How long will she have these for?
Eric Weber: Every CMT case is completely unique. But there are opportunities to predict. Beginning in the late 80s, there was a lot of work surrounding the muscle weakness patterns, the predictable sequence and the way that CMT affects the body. In general, the longer nerves are affected first, so we know the arms and legs have the longest nerves affecting those muscles. We also know that if we have a chance, we can prevent some deformities that might be a large challenge later in life like where Julie is right now. Specifically, in Julie’s case, we have worked hard to capture her foot in a corrected position, both at the back and in front with things that are inside the shoe and then when her needs grew to needing a bit more assistance to prevent her foot from dragging as she walked. Then we moved on and crossed the ankle a bit. Most recently, she has expressed that she needs to work on walking on uneven ground and trails in the mountains. That is when we moved into these custom composite designs for her. I imagine as we continue, we can see that some of the muscles that are weakened might or might not be able to overpower the muscles that are pulling in the other direction. So, what we do is adapt and move forward with our designs and there is no real predictor of how quickly that might happen. It is vitally important for us to maintain a good relationship with our patients and as we walk through it know this might be a lifetime of utilizing our services. So, we see them quite consistently. Julie and I specifically are in communication via email. She might say I am feeling this, I am worried about falling, or can we modify the device to help me in this part of my life. So, it really is a lifelong friendship and integration into developing further treatment plans.
For someone with CMT, how would it impact their quality of life?
Eric Weber: It has been remarkable how so little is known by the time you eventually are diagnosed with CMT as it often takes years to come to a diagnosis. There is a lot of genetic testing during the time you are searching. So that is a journey in and of itself. So by the time somebody who has either accepted the fact that they might require a little assistance or they have recently felt their physical symptoms have worsened enough to have the conversation, it is really important to have a direct conversation with a test with your doctor and partner it with physical therapy, and as a team come up with what is the best approach to manage your treatment. So, developing that relationship is important much like anything else in health care these days. Make sure you are comfortable with your team, with the therapist and the orthotist testing your doctor, and manage your symptoms because often you are going to be the one in charge. Somebody might not realize that the best conservative treatments are to move to bracing before we try and attack them in a different way. There is research now and medicine surrounding CMT, but in the current environment there is not any great pharmaceutical option. The treatments tend to be in the therapeutic rehab and orthotic world. So, the ability to communicate needs and respond accordingly from both the patient and orthotist is key. There is not one solution that is matched to any one person. So, whether it is orthotic treatment and or if you take a cookie cutter approach to the disease, I do not think patients will fulfill their biggest potential. So, if I had a message for anybody who might be thinking they may need a brace I would say we need to find somebody who is willing to listen and really tailor your specific treatments because it is different for everybody.
Are there any questions they should ask their doctor or the physician?
Eric Weber: Tell your doctor, I am tripping more, my foot hurts a lot when I stand for more than 10 minutes, or my steps are noticeably short. Ask the doctor, are there options to assist me? The doctor may or may not be familiar with exactly what you are talking about. But almost everybody would at least have an idea of conservative treatment using braces. It is really our job and the relationship between the doctor and the orthotics team to come up with the orthotic treatment plan. So, all you really need to do is express the need and the goal. For example, is there something out there for me? I would not expect them to come up with a solution, that is my job, but the need needs to be expressed.
Anything I did not ask you that you feel people should know?
Eric Weber: CMT is an umbrella term, so it covers many different types of disease states, also known as peroneal neuropathy. It is commonly referred to as hereditary motor and sensory neuropathy. It is one of the most common genetically inherited neurologic diseases out there in one in twenty-five hundred people. So, it is more common than we think and to seek out specific testing is not really a patient’s job. But if you are noticing in your late teens, 20s, or 30s that your balance is off, you are tripping, or there is pain in your feet than you need to seek treatment. I ask the questions because there is such a high prevalence in our society. I think it is one of the more undertreated groups of people. So, there are support groups and centers of excellence scattered around the country, that are constantly working on emerging research and pharmaceuticals, so do not accept it. If you are utilizing a brace, do not accept that is your only option. If you are not getting the results that you feel you need to then ask more questions. If somebody you are working with is not able to listen and tailor to the orthotic treatment to your needs, ask more questions with more people. It has been surprising when I have met some of these groups, how little is known about what is out there. We are such a small world, such a small part of the health care system that it really needs to come down to that conversation. It is our job to compile the potential options for you. One last thing is that the devices that are premade often do not have enough custom composite, and functional ability to control. When you are standing, they may be light and keep your foot from dragging, but anything beyond tripping really needs to be explored in a more customed and tailored cathartic treatment.
So, your patient Julie is keeping active and will that be good for her?
Eric Weber: There is a sense in the CMT community that you get tossed around and I mean that in the best possible picture because you might not have a concrete diagnosis. Often questions or needs that are brought up in a doctor’s office or even with a physical therapist may not be this progressive, interactive genetic neuromuscular disorder and they say you should just do some exercises or grab a couple of ankle wraps at the local drugstore or my foot hurts so here is some Tylenol or other pain medications. I think in Julie’s case, it is a little frustrating because she was looking for someone to help enable her or give her the tools to solve the problems. She was not looking for anybody to tell her what to do but she wanted somebody to recognize and listen to what she was needing and come up with some solutions to get her to the next level. Really that needs to come from somebody integrated and knowledgeable about the processes of CMT and what the progressive nature of it is because otherwise you are left with devices or choices that may not suit her needs. I think Julie felt a little frustrated if she was presented option A and option B, then stop and decide what you want and move on. Recognizing the nature of the muscle loss sequence can really inform how you attack it and in the least complex way so that she has a simple solution to the next step that she wants to go to, and it leaves the door open for further management later.
Interview conducted by Ivanhoe Broadcast News in November 2020.
END OF INTERVIEW
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