We’re back! Yohan yelled, sliding the door to the garage open. I noticed he was walking funny, not CMT funny, but like he had a stiff leg, a heavy foot, an injured limb? My perplexed look begged the question… What now?
He brushed off my concern, acting if I were being overly paranoid and concerned (like usual). “We had a great ride and of course, on the last mile, I was tired and I took a spill. I just sprained my ankle… and…well my elbow is banged up. Oh…. and sharp stick pierced the palm of my hand when I hit the ground, but overall, I’m fine. It’ll be better in a couple of days, he said, limping down the hallway.”
After a hot shower and short rest, I took a look at the damage. “My ankle is hardly swollen,” he insisted. I pointed out that the last time I had looked at his ankle, I could see a prominent ankle bone. Now it looked puffy and bloated, as if a small jelly fish had snuck in there and took up residence. So, the crutches came out of the closet, along with the sickening memories of past orthopedic surgeries, months and months of plaster casts, pain, boots, stinky feet, scars, blood and sores.
Our bald kitties ran across the hardwood floor to greet him, but one look at the clanking crutch made them hit the brakes and off they went sliding uncontrollably, face planting into the wall. Thank God the cats make us laugh!
Recognizing the all too familiar clunk, thud, clunk, thud of Yohan’s footsteps as he made his way across the room, I too wanted to hit my head against the wall, cursing CMT to eternity and back.
Yohan’s had so many trips, falls, ankle sprains and surgeries, he knew the drill. No, not RICE. We changed that acronym to RICED. Rest. Ice. Compression. Elevation. Drugs…can’t forget the Tylenol, Aleve, etc.
There wasn’t a lot of pain, but by Monday, Yohan intuitively knew he’d better get it checked out by his doctor. This was the foot on which he has had 2 reconstructive surgeries, with mediocre results.
When the films were placed on the viewing pane, there were a lot of aahs and oohs. His x-rays lit up the room like lights on Christmas tree. The techs were amazed at the hardware holding his foot together. But who was the jokester who took a pen and drew a fine line across the outer leg bone?
Diagnosis? A hairline fracture of the fibula requiring 2 months in a walking boot, 24/7, except to bathe. Ugh. Well, it could have been worse, but it still sucks.
Home we went, trying to wrap our heads around the news. The first order of business was to purchase an even-up foot riser to avoid throwing his opposing hip out of joint. He learned pretty quickly that the even-up foot risers are treacherous as they get caught on everything…..even air. The utmost caution is warranted. And if you don’t catch the edge of the foot riser on something, the cats will make sure you fall…..hard.
Walking on 2 CMT feet is tough. Put one of those in a boot and now balance on one very high-arched foot, whose toes curl in and up, and sprinkle in some ankle supination (outward turning ankle). Not for the faint of heart!
Next, he looked for additional boot liners. Seriously, they send you home with 1 boot with liner. The “hand wash only” liner smells like death after the foot is enclosed within its sweaty fabric for only 24 hours. Yohan ordered a second boot (the lux version of the same brand) so he could wash the liner every other day. Luckily, when a piece of the plastic from the first boot started digging into his heel, he had a cushy alternative.
The pain from the fracture was bearable, but he started to get a painful pressure sore on the bottom of his foot which thankfully abated when slipping his orthotic into the boot. The first several nights sleeping with the boot were tough, but gradually his body got used to the inconvenience.
This morning, he woke up with a smile!! He was celebrating his 2 weeks down, six weeks to go in a boot! He’s a glass half full kind of guy.
Taking a step back, here are my reflections:
Yohan’s new electric mountain bike offered a rainbow of opportunity, where, for the first time in quite a long while, Yohan could just be one with his friends, his dad, and his people. He did some pretty amazing rides, going places and seeing things not seen before. And these days are not gone, just suspended for a short period of time. Not the end of the world, but a bummer all the same.
I guess we all have ups and downs. Personally, I prefer the ups, but don’t we all? I believe the ups build experience and self-esteem and the downs build strength of character. And then there are all the in-betweens. Every emotional state –happiness, sadness, anxiety, fear, anger – is transient, so accepting the peaks and valleys with calm and acceptance makes each situation a little easier to handle.
Life’s highs and lows are an inevitable part of being alive, so I try to enjoy the highs, learn from the lows and experience everything else in between with an open heart. I know….easier said than done.
Yohan will get through this small setback. It will become a faint but unforgettable memory that will be part of his ongoing arsenal when dealing with upcoming challenges, setbacks and successes. He’ll get back on that bike…of this I’m sure. Why? Yohan is no stranger to adversity.
He’s not giving up or giving in. He’s found an activity he thoroughly enjoys, an activity which is exhilarating and fun, creating long-lasting memories of freedom and adventure. Also, he’s determined to cycle in the Cycle 4 CMT event with his Uncle Chris, his dad and maybe even me! The Cycle 4 CMT (in person or virtual) is so much more than a cycling event……It’s a celebration of strength, resilience and community.
Join us in the spirit of uniting with like-minded people, to fund research to put a stop to CMT. There is no cure for CMT… yet. But there will be because we, our CMT warriors, friends, family, loved ones, are going to make it happen. Grateful, so grateful for our community. Xo
The CMTA hosted a very informative talk on Zoom in September, 2020 to the CMT community featuring well-known orthopedic surgeon, Dr. Glenn Pfeffer. I’ve transcribed this talk for you! Enjoy!
Elizabeth Ouellette: Welcome Dr. Glenn Pfeffer. Dr. Pfeffer is the Director of the Foot and Ankle Surgical Program at Cedars-Sinai in Los Angeles.
I can’t tell you how much I appreciate and admire Dr. Pfeffer. He spends all his time with people with CMT. He wants to better their lives. He wants to see people walk. He has devoted so much time and energy to the CMT community. I am just so honored to have him here as a doctor, my son’s surgeon, as a friend and as a colleague. So welcome, Dr. Pfeffer, and thank you for coming on.
Dr. Pfeffer: Well, thanks for having me, and I’m surprised so many people came on a Saturday!
Elizabeth: First, I’d like to get to know Dr. Pfeffer a little bit more. And when I was doing some research on him, I saw that he did magic. I’m like, what surgeon does magic?
Dr. Pfeffer: Actually, magic is important to me. I did this through high school, college and medical school. I actually performed in nightclubs. I spent the summer in Nantucket at the Rose and Crown as their magician. And whatever it is that attracted me and still attracts me to magic, is the exact same feeling I get two weeks after surgery when a CMT patient is sitting in the office. Your foot’s going to be a little bloody. You’re going to have some sutures to take out, and we open up the cast, and you’ll see some pictures of this, and I hold their foot and I say, “Take a look here, your foot.” And it’s a new foot. It’s a foot they haven’t seen, perhaps ever, and the look on their face is identical to the look on people’s faces when you do a magic trick for them.
So whatever that is that attracts me is why I continue with magic. Magic is very simple you know. You can do things like this where you can take something and have it disappear. You can make it a little more complicated, which I do in the office for kids. You just take the same little piece of foam or whatever, and you just put it into this hand and you can just show people that it’s empty. So that’s sleight of hand, which of course is great for the magician. I still do this at orthopedic parties. But not to belabor it, if I were going to do this I wanted this to have the same absolutely startling response that people have with CMT when they see their new foot.
Elizabeth: Your father was a surgeon?
Dr. Pfeffer: He was a general surgeon.
Elizabeth: Oh and so is that what inspired you to be a surgeon?
Dr. Pfeffer: Yes, absolutely – I don’t think I’d ever have even gone into medicine if not from my dad’s influence. You know like all of us, I’m a mix of my dad’s DNA and my mom’s and they were very different people. But dad really inspired me with his surgery, and he was hard-working and I think it was my destiny. I probably have very little free choice in life. He didn’t make me but in terms of who I was, it was sort of my destiny, like the Jedi.
Elizabeth:You horseback ride or you did in the past. You scuba dive, you dance, you create bonsai, and I read somewhere you made a correlation between bonsai trees and surgery. Could you tell us a little bit more about that?
Dr. Pfeffer: I’ve always been interested in bonsai which are, you know, plants; they’re trees, and you keep them miniature by trimming their leaves, by trimming their roots, and you keep them in small pots. Everyone knows what they are, and what you do to shape them is you wire the branches and you hold them down so they look like a tree. And I realized only a year ago that what I’m doing with bonsai is identical to what I’m doing with CMT feet. I was sitting there in surgery and we were wiring a foot down and putting screw into it, and somebody who knew about bonsai said, “You know, Glenn, that’s exactly what you’re doing with bonsai.” And it was startling to me. You would think it would be obvious but it wasn’t.
As you may know I’ve got my own foot problem.
Elizabeth: I was just about to ask you; when you told me you had foot issues, I’m like, “He gets it.He knows what it is like to have a foot problem, and I think that’s a bonus for your patients.”
Dr. Pfeffer: Well I was just going to say, though, that’s how I got into horseback riding. Because I’m athletic by nature, but I couldn’t run. You know, I didn’t know what I had. It’s very poignant for me, the CMT world, because patient after patient, everyone who’s listening knows this, everyone, unless you had a mother or father who had CMT, you grew up not quite knowing what was wrong with you, right? And I didn’t either. I didn’t know I had a problem with my foot for 40 years. It sounds dumb, right? But if someone’s out there with CMT and no one told them they had CMT for 40 years, and they just thought they walked funny and they couldn’t keep up, and they were a little unbalanced, nobody would know. So I took up horseback riding because I could do what I wanted to do. I could fly through the air and jump, and I’m sure all the people listening have modified their lives in way so that they can function with their CMT.
Elizabeth:I think you can really relate to people with CMT and understand foot issues and the inability to do certain things.
Dr. Pfeffer: Well there’s no question, you know I don’t like to talk about it too much because you don’t want to get a little corny on a Zoom chat with all these people, but yes, I 100 percent relate to what people are going through. I’m not in a wheelchair. I don’t have problems breathing. My hands are strong. But for the isolation that somebody feels, the difference that somebody feels growing up with CMT, that is exactly what I felt for sure, and I don’t know that you can teach somebody that. I’m not sure you can really learn it, but it’s just in my soul. I’m not as bad off as most of the people with CMT at all but that’s why I get it, you know, that’s why I think it’s probably why I was attracted to all this.
Elizabeth: So why don’t we start your presentation, I think you are a fascinating person, and I know you’re an expert surgeon and the best of the best. And you’re also a great presenter, so I’m sure people want to hear what you do every single day.
Dr. Pfeffer: I want to just show you my world of CMT, and I have a certain kind of person that comes to me, right? Somebody who was unfortunately paralyzed in a wheelchair would not be getting to my office, so I do understand that I’m seeing a segment of the CMT population. But this is my world and what I go through every single day. At this point I’m confident that we at Cedars are operating on more CMT patients than anywhere else in the United States and we have a plethora of experience with it.
We’re lucky enough to have a large CMT program at Cedars with some of the most famous CMT neurologists on the face of the planet, such as Rich Lewis and Bob Baloh, and with amazing geneticists. If you have an issue, you can come to see the program. You’ll see a lot of people. Instagram is as alien to me as, you know, speaking Russian or French and yet it’s been a tremendous success for me and the people who follow it. I mean, my gosh, I didn’t even understand that at one point I had two people following it. Now we have close to 1400 people across the world, and I communicate with them all. It’s sad when someone from Ethiopia says “How can I come and have surgery?”, and of course the chance of them having surgery is almost zero for financial and travel reasons.
I encourage you to follow me on Instagram: #CharcotMarieToothSurgery
The foot’s complicated, right? It’s got a lot of muscles in it. There are 20 muscles in the foot, more than there is in the entire leg.
The tibialis anterior muscle is the strongest dorsiflexor (muscle lifting foot up toward the shin) and helps to lift the foot from the ground. The Tibialis Anterior Muscle also facilitates flexion of the foot upwards and extension of the toes. The Tibialis Anterior Muscle originates from the outer surface of the tibia and inserts into the first metatarsal bone in the foot which is located behind the big toe.
Above are the toe extensors. These are what lift up your toes, and interestingly with CMT, if the tibialis anterior that lifts your ankle gets weak, these toe extensors will start working harder, which is why so many of you will have a toe deformity. As the Tibialis Anterior muscle gradually weakens and the foot drops down, a contracture of the Achilles will occur because the tendon is no longer being stretched out during gait. The worse the contracture, the harder it is for the weakened Tibialis Anterior to lift (dorsiflex) the ankle.
Above is an image of the Peroneus Brevis, one of the key muscles that weakens in CMT. Why it happens exactly is still unknown, but this muscle, when it weakens, destabilizes the ankle and the foot starts to turn in, because this muscle is weak. And the other reason the foot starts to turn in so commonly is because this muscle in the right foot, looking from behind, stays strong.
So one weak muscle, and one strong muscle causes the foot to start to deform.
So let’s talk about that what causes the CMT deformity. I’m talking about this common cavovarus (very high-arch) foot. This is what happens every millisecond in our body. Muscles are pulling back and forth but keeping us balanced, right? With CMT, because of the paralysis that’s uneven, involving some muscles but no other muscles, they become weak. There is CMT. Some muscles are powerfully strong, and others are weak and that causes a deformity. It’s called a cavovarus, and you can really see it on this right foot.
If you took your hand, put it in your pocket and left it there for a year. Not only will you not be able to move it, you’ll probably never be able to open it up again because all of the soft tissue contracts. And that’s what you don’t want to have happen. If there’s one message I can give you, don’t let that happen.
The minority of patients I think benefit from surgery with CMT: Most patients do not need surgery.
The person below is wearing ground reaction force braces. This type of brace, made from carbon fiber or plastic actually bends and stores energy.
So when you have no function in the leg and the Achilles tendon isn’t working, these braces are just terrific. This person could walk a hundred miles if they had to because their foot’s flat on the ground and they’re doing great. Now, here are all the types of braces there are. Some are off the shelf. Some are custom made. Which is better than the other? I don’t know.
I absolutely think chocolate ice cream is the best. Does anyone disagree? Some people like strawberry or maybe vanilla, and the problem with the braces is you can’t try them all on unless you go to a spectacular brace shop, which I’m lucky enough to work with at our Center of Excellence. Would anybody say that a size six dress of a certain brand is perfect for you? Of course not. You’d want to at least get it in the mail, try it on and send it back. That’s what you have to be able to do with braces. Unfortunately, we can’t try all these braces and some of them cost thousands of dollars, so try to go to a brace shop that has a wide selection to let you try some of them off the shelf.
Now that is not the right brace for a for this crooked foot. Some of you have it. I see this situation every day of the week. Would anyone put a foot like this into a brace? It’s like putting a square peg into a round hole. This is from Friday.
I was a little delayed today getting my talk all set because this gal just came in. She lives on a ranch. That’s the shape of her foot. That’s the shape of her brace. Shame on everyone taking care of her. Shame on her brace maker. She has pain walking on the side of her foot in a brace that looks like it’s something to a caged up an animal. That foot should never be allowed to walk the face of the Earth. That can be made flat, and even if this woman can’t get out of her brace, she can get into a brace with her foot balanced and her body weight plumb lined with no pressure walking on the side of the foot.
I can’t see you all, but how many of you have had or have a callus on the side of your foot? Right. That’s what happens. So these people should all have surgery, that’s how I feel. Now some people don’t want to wear a brace.
This is Katie’s Story
“Hi, my name is Katie. I live in Florida and I have CMT. Katie didn’t want to wear a brace. It was in sixth grade when I first started getting made fun of for the way that I walked and noticed that running and keeping up with my friends was becoming more difficult for me, and my parents started taking me to some doctors to try to find out what I could do to help me with my CMT. With each doctor that I met with, I felt like they didn’t understand my specific case of CMT. And they gave me some options, like braces and orthotics, and some of them would help me temporarily but nothing ever really helped me. So when I was in high school, I started falling almost regularly, and I missed out on my homecoming and my prom and just gave up on trying to find shoes that fit and started to become really discouraged.”
So Katie was a young woman, and she didn’t want to wear braces. She could have actually been in a brace. So she came from Florida. She said, “I don’t want to wear braces the rest of my life.” So I said okay and she had some muscles that were working. We operated on one foot, and she was incredibly brave and so we operated on the other. Katie could hardly walk without a brace without holding onto a wall.
Many of you know that kind of person. So the CMT type foot deformity is a tricky surgery. That’s the problem, right? People have had a lot of issues with it. There’s so many components to CMT surgery. The surgeries will take at least three to four and a half hours. There’s no way to get through it quickly. Basically, the failure of CMT surgery is when we don’t do enough.
So this is what we put on the operative board at Cedars-Sinai. We put all the procedures someone’s going to have. “Hi, we’re going to fuse a part of your toe joint,” you’d say to a patient, “Okay, let’s schedule you for surgery. Hi, we’re going to do a tendon transfer on you. Okay let’s schedule you for surgery.” But the tendon transfers are some of the most complicated and extensive surgeries that there is in all of orthopedics except for some spine surgery or hip surgeries with dislocations and acetabular malformations.
Now how do we know what to do? Well, the problem is there is no good consensus on what to do. At least there hasn’t been, but we’ve done a lot of studies on this at Cedars and much thanks to the CMTA for their help.
This was a study we did. It was published in 2018. It was sponsored by the Charcot- Marie-Tooth Association (CMTA) and this was really incredible for us because we won a prize for this operation telling us how to correct the heel varus. And we won a prize for this as one of three research studies of the year most likely to change orthopedics. I just happened to have it here.
We took a print of one of my patient’s foot – Sarah. And we printed out 18 “Sarah’s”, 18 of these and then we studied them with different operations in the lab, but very exactly, and we showed which was actually the best operations for correcting heel deformities in CMT patients with her type of problem. Since then we’ve done other research more on heal osteotomies. We’ve looked at extensor transfers The most difficult, competitive organization in the world and the most academic is the orthopedic research society. And Max, who’s going to be joining us, he’s the second author, won a prize here just this past spring for a young investigator’s prize. I mean this is like winning a Nobel Prize in orthopedics and I was actually stunned by it. But part of the reason is because the whole area of CMT surgery is so poorly investigated, it’s not that hard to do some landmark work if you do the research.
Now there’s a big hole on how you should do CMT surgery and how it ends up in people and how do people do it. We’re just starting now. Some of you I’ve operated on. I always say I’m a pretty accessible guy and I don’t hear about too many people doing poorly. I know the ones I’ve had to re-operate on. I just spoke to a woman this morning where a young girl in New York is not having the motor strength that she needs. So I think I have a sense of it, but we’re going to study it and hopefully publish that by the end of the year.
So this was a remarkable thing – years in the planning. There were seven past presidents of the American Orthopedic Foot National Society and some of the most famous foot and ankle surgeons on the face of the planet, and with the sponsor of the CMTA sponsorship and Elizabeth’s fire in her belly. we brought these people together. And it almost killed me, literally, but we finally published a paper on our results, which just came out, and this is a consensus. We didn’t get everyone to agree, but I think just to sum it up here, this is accessible. You can get it through the CMTA and other places and if you’re going to have surgery locally in your area if you can’t come to Los Angeles for some reason, give this to your surgeon and say, listen I’m sure you know all about this but would you mind taking a look at this paper. And if they haven’t seen it they’ll be grateful to have it. https://www.cmtausa.org/news/breakthrough-guide-to-orthopedic-surgery-for-cmt/
The goal of surgery is to give you a flat foot, and to balance your foot. My goal is to keep you out of a brace if I can, and I usually can if I’m willing to operate on you. It doesn’t always work that way, and if we can’t get you out of a brace, at least we’ll get you into a smaller brace. Much better to wear just a small piece of plastic than some of these bigger, bulky braces. So what do we do? We transfer tendons, which is moving muscle. We move strong muscles that are deforming the foot to weaker muscles which are letting the foot become deformed. We cut through the bone. Here you can see a bone. This is a right heel we’re looking at.
We take a wedge out of the bone. We twist the heel around. Here you can see it. So this is the heel from behind on the right side. You can see what we do. We take a wedge out and then we just simply shift the heel.
Okay, now who can you trust to do surgery? I hear there’s a lot of talk about me in chat rooms and stuff. I said, is there anything I ask my patients they don’t like. One person said, well you kept them waiting a lot, and another person apparently said, well, I just didn’t like him, but I guess he’s a good surgeon. But look out there. I don’t hear anybody saying about me, anyway, oh he sold me a bill of goods. He said I’d be good but I’m not. I just do not hear it. And if you do, tell Elizabeth and she’ll tell me because I want to hear about the failures I’ve had. I know the failures. I know why they’ve occurred but most of them have just been in the hands of God. Find someone who does at least one CMT surgery a month.
That’s a fair number of CMT surgeries to do. Most very experienced, busy surgeons will be doing three CMT surgeries, maybe two a year. But if you can find people around the country. that’s a good thing.
When should you do your surgery? Do it as soon as you know that you or your child can’t live with their foot the rest of your life. The sooner the better. Dr. K, my partner, because I don’t operate on people really under the age of nine, he just operated on a four-year-old yesterday from Utah, both feet. All these people are the perfect age for me to be doing surgery. You know, 10, 11, 15, 16, but you can do it anytime. It just gets harder because things get stiffer the older you are. So every day, because of Instagram, I chat with people. Some people are from Eastern Europe. Some people are from United States, and I have met most of them. I guarantee you I will make you better. I don’t know how much better. I’m not 100% sure I’ll keep you out of a brace, but I tell people you’re about a C-. I’ll at least get him to a B +. All right. So all I need is one strong muscle.
All I need is that muscle to move. That’s it. I just need any muscle. Is there risk? Of course.
The biggest reason surgery fails are that not enough was done. To do 18 surgeries at one time is a lot. T
Sarah was one of the most amazing. You may have seen her. She was 16. She couldn’t walk. We operated on one foot. All she said she wanted to do was walk down the high school aisle without having to hold on to her father. I called her years later. I said, “How are you doing? What’s going on with you?” And she said, Dr. Pfeffer, you don’t understand.” She said, “I just walked 10 kilometers around London with my boyfriend in cute shoes.” So that’s a magic trick all right. This gal, she said, “I don’t want to wear braces. I’ve had surgery. I’m going to be the first woman President of the United States. I’m going off to college.”
There she is. I mean, I can’t do that. Could she? She texted me a while ago. She goes, “After five or six hours of walking around campus, I need a little co-op brace. You know, ones that go into your laces because I get tired at the end of the day and sometimes need a little bit of help.” That’s better than I ever thought.
Q & A
I’m wondering how you deal with toes. Yeah it’s a great question. Very succinctly, toes are among the most difficult, actually, of surgery to do and the the longer people wait, the worse off they are. I can still fix the rest of the foot, but the toes become more and more problematic and there’s no easy answer for that. Sometimes as simple as just transferring the tendons. Sometimes we just cut the flexor tendons but I don’t like to do that in someone who has a motor disease. And what I’ve been doing lately with severe problems is we actually have been fusing these joints. It’s okay because the joints are useless. It’s not doing anything for anyone except getting in the way. So we fuse the joints and leave the tendons alone and and we can have some beautiful results with that. The problem is it’s a lot of surgery. That, in and of itself, that operation could take an hour, hour and a half. To add that onto a four hour operation … someone like the boy I’m operating on on Monday who has all that may have to come back, he may for the toe operation.
I had foot surgery, now my knee and hips are not aligned with my feet and I am having gait problems as well as some knee and hip issues. I’m wondering if that is because she waited too long and the CMT foot made her walk differently or do you do gait analysis beforehand? Have you heard of these problems? Someone has foot surgery. They get deconditioned and the hip muscles which are vulnerable to begin with, and the knee muscles get weaker, right? So they just get deconditioned. I know of one person in my career from Santa Barbara, and she had foot surgery. She was, you know, recovering and then she had that problem. You know, and all of a sudden her hips started to have a problem. It’s really really rare if the foot’s have been bad … feet have been balanced properly. My first thought is that the surgery didn’t work on the feet and that they’re imbalanced. The key is to remain conditioned, even if confined to a bed. Get some five pound weights on your ankle and lift up the leg.
Do you ever do both feet at the same time?
Never. You could do double surgery, like in a four-year-old like yesterday where Dr. K did that. Where you can just carry them around easily, but no, an adult is going to be completely impaired because they can’t put any weight on their foot for six weeks. Yeah. They would have to be tiny. Carry them around to the toilet. Carry them to the chair. What weight would that be? I don’t even know. You know, 50 pounds if dad’s strong.
Is there a particular age you recommend surgery?
There’s not a specific age, but how do you know to bring your child to trust you with his feet or her feet? When do you do it? Well, it’s absolutely the most important question anybody can ask, and I don’t have an answer. To operate on the crooked foot at any age, operate certainly on the young adolescent at 12 or 13, as soon as you know that person’s not going to live with that foot ideally for the rest of their life. If you look at your child, your friend, and you say I don’t want them to have that foot the rest of their life, that’s the time to have surgery.
Since CMT is a progressive disease do you do surgery and then 20 years later you have to do it again because the foot deforms or what is your experience with that?
There’s no literature on that whatsoever. The study that we started in 2017 will be coming to fruition soon. I said I told you, anyone I’ve operated out there please answer us when we write to you, and we’ll follow these patients along forever, and the only way anyone will have an answer for that is in 2037 when those patients are still around. Most … many of them … many of them were young and we’re going to find out how they’re doing but I can tell you this. If anyone’s considering surgery and they’re not doing well, don’t delay because someone says, tells you, you’re just going to be paralyzed in 10 years anyway. That’s not my experience. I’ve been operating for 30 years in California and no one has ever come back to me and said the operation hasn’t worked because I’ve gotten weaker, ever. And I’m around, you know, so I’ve never had that. So I don’t think these things progress. and I personally think that when you do this on a young person … this is really critical … what do you think happens? And we’re doing a study on this.
Is it true you should not operate on children until their bones are finished developing? Is that sort of an old school philosophy?
No, it’s an old school philosophy. Just throw it out the window.
How can you find a foot and ankle surgeon, one who really knows what they’re doing on the CMT foot,
I want people to come here if they can. I’m amazed but I admire that COVID is not stopping anyone. Tomorrow there’s a girl from Texas and she’s driven up. There’s a boy next week from New York, and he’s flying in, you know. So it doesn’t seem to stop anybody much for the CMT and they think it’s a good time, right, because schools are virtual. So all of a sudden someone’s saying, well, this is not worth it to be freshman in college for seventy thousand dollars a year. I’m gonna get my feet taken care of. People come, I want them to stay. A lot of surgeons wouldn’t want that, but I want the person to stay here for two weeks.
Now it’s a big city and a lot of people have relatives and I would say what I tell everyone. You can get a hotel out of town for $59 somewhere a week and you can get a hotel in town for $5,900 a night, and I’ve had both types of patients. Normally the former than the latter but you stay and then the sutures will come out in two weeks and then you go home. Insurance will … we’ve never been denied insurance because most … if you’re in North Dakota … in your town the insurance knows that there’s nobody who’s going to want to do this, and if they do want to do it, the patient will say doctor how many have you done? Have uoi done hundreds of patients, the way they have at Cedars? It’s too difficult a surgery to take on you know so the the surgeons don’t want it. Even the HMOs will allow people that … one of those people I showed you is from an HMO, which is very restrictive healthcare, right, in Hawaii but the HMO doctor doesn’t want to do it. Medicaid and the medical patients aren’t allowed to come but almost all insurances will allow this.
I’m an employee. I don’t get a penny from doing the surgery, just not a penny. I’m an employee of Cedars and Cedars is part of almost all plans. And the last thing I would just say is if somebody doesn’t allow you to come at first, they will because all you have to do is say this is where I want to go. Are you willing to take the responsibility, doctor, insurance plan, but I’m not going to do well. And the answer for that 99% of the time would be, why don’t you go to Los Angeles? Now there are certainly people who do what I do in the United States, but they’re large areas where they’re not. Okay, that’s a really helpful answer. Great to know. I just said the cash price is just insurmountable. I just … it’s tens and tens of thousands of dollars. So you really need to to go through insurance and get that.
So in that regard we’ve never had anyone turned down. Sal Rosette, my surgery scheduler … I was working late night in the office, he was leaving late and I asked Sal about this. And he said to me is exactly what he said. I don’t know if it’s true or not. He said, Doc, he goes, we have no one ever turned down by insurance for you. Each doctor has something called an NPI number that designates them. He said he’s at the insurance company I’m talking to them on the phone and they say well what’s his NPI number and it’s CMT and the insurance just immediately says, oh yeah, you can go there. I don’t know if … I don’t know if he’s just reading into that or not. I said, so they’re tracking us with CMT and my doctor number? He goes. ” they must.” Blue Cross, he said … I don’t know if it’s true … but according to him he said Blue Cross knows about you and CMT. Anyway that’s a long answer. That’s okay, very helpful. Back to the age a little bit, and I know you spoke to the crooked foot being, you know, of surgical possibility at any age.
What are your thoughts on ankle fusion?
There was a consortium in Europe which you know about. Dr. Shy was there and others looking at this, the surgical issue, and we met and I was amazed that one of the surgeons there does a lot of CMT surgery said we do fusions in everyone. We do tendon transfers and fusions.
So I was born with a fusion You can do okay with the fusion of the joints that are usually involved with CMT, but you’re not perfect. And I had … if my foot moved perfectly, I would not have dislocated my ankle when I fell in the pool recently. You don’t want to do a fusion in a young person. If you have to, it’s not the end of the world. Let me tell you, if anyone’s telling you who has CMT that you need an ankle a fusion, you need another opinion from out of town.
But never fuse an ankle in a CMT patient as a general rule. Why? Because you’ll do much better with those braces. When you fuse the ankle, you take all the spring out of the ankle and you negate the ability of using those wonderful braces, those ground reaction force braces that are made now, and those braces are anything from over the counter or basically online to ten twelve thousand dollars a pair, so there’s a lot of options. But you have options unless you get your ankle fused.
Who should do my surgery?
There are orthopedic foot and ankle specialists. That’s who you want to see. An orthopedic MD foot and ankle specialist … M-D, someone who went to medical school. You know, that’s the first thing. Not a podiatrist. If there’s a podiatrist out there who’s done 100 CMT surgeries, then fine, I have no problem with that. You know, podiatrists are not medical doctors. There’s some natural competition between orthopedic surgeons and podiatrists.
I’ve operated on always over 40 000 people with CMT. Wow. In this one you need somebody with gray hair. I am so much better at this than I was 15 years ago. Go see somebody. Contact me if you want. Set up a telemedicine visit, and I’ll tell you whether I agree with your opinion or not.
And I know everybody, and I’ll secretly tell you if I think it’s a good person to go with.
The Consensus Paper – Take that paper, put it in your pocket bring it to your surgeon … learn it. CMT patients are pretty smart people. And learn what’s in that paper. Ask a few questions and see what the answers are. What we wrote in that consensus patient paper will change and it’s not the final answer, but it’s a very good start to knowing who the right surgeon is for you, right?
Elizabeth: I can’t thank you for being so passionate about our cause, and you’re very approachable, and even in the world of orthopedic surgeons. And it’s you guys are very very busy, and very task oriented, but you care. You have heart. You have soul. You follow up with your patients. I mean, the quality of care that you give is amazing. So not only are you a very competent surgeon, but also you’re somebody that’s approachable and you can talk to and you’re interesting. And so A+. Thank you so much. Thank you so much for this wonderful presentation and caring about our community.
Yohan is now in Graduate school in Claremont, CA. He is WALKING on his own 2 feet! In fact, he’s walking A LOT!! Finding his way around the first day of classes was physically and mentally exhausting, and when he looked at his Fitbit at the end of the day, he had walked right around 5 miles – a record-breaking feat! And, he has much less pain, too, which was an unexpected benefit of the surgeries.
Thanks to Dr. Glenn Pfeffer’s relentless striving for perfection, he never, ever let us down. He remained by our side every “step” of the way. Thanks to Dr. Pfeffer, Yohan is walking better than ever before. We are forever indebted to him for his surgical skills, fabulous bedside manner, and the care and compassion he showed over the past 12 months. He’s an amazing doctor and a brilliant surgeon. We are forever indebted to him.
And special thanks to Robin Tobias, his longtime physical therapist who taught him to use crutches, gave him exercises to rehab his withered leg and tortured him for 2 months, twice a week, to get his legs strong and muscles stretched out. We love you!
Uncle Chris, who created and organized the Cycle 4 CMT events also played a special role in keeping Yohan upbeat and smiling during these past 12 months. Many others have been overwhelmingly supportive over the past year too and to everyone who supported Yohan through thick and thin, and to those who sponsored his most recent 1-mile walk for the Cycle (and Walk!) 4 CMT this year, he writes,
Today, I write this email from my studio apartment in Claremont, CA where I am in a graduate program, studying Psychology. I cannot tell you how grateful I am to be able to further my studies and move on with my life.
I am happy to report that I was able to walk a full mile during the Cycle (and Walk!) 4 CMT on August 27 (see the picture of my Fitbit below, if you want proof). Your continued support was instrumental in motivating me to work towards and eventually meet my goal. As you know, this year has been extremely difficult, but I have managed to push through, overcome the challenges and succeed! Your unwavering encouragement and friendship have truly helped me to keep my spirits up – the idea of giving up was never, ever an option. I am eternally grateful for your generous gifts and steadfast belief in my capacities. Thanks again!
Below are a few of the pictures, taken by my brother, George Ouellette of the Cycle 4 CMT event:
Tonight, the house is much quieter, a bit too quiet, in fact. Gilles and I feel Yohan’s absence acutely. The first couple of days did not feel comfortable. We both experienced melancholy, pride, anxiety, and relief – relief that it all worked out which means that Yohan was able to pursue his dreams. We’ve talked to him off and on, and he’s adapting to his new digs very well and continues to walk as much as he can. When I asked him why he doesn’t bike to school, he says, “I like walking. It’s good for me.” Who is this child? I wondered, too stunned to respond.
Why? Because 2 years ago, when Gilles and I encouraged him to remain active by biking for 10 minutes a day, he unraveled, “I do not like to exercise. I’ve never liked to exercise. Exercise does not fit into my life, now or anytime in the future. I have issues with fatigue, my feet hurt when I walk, and I have all I can do to get through every day, so please stop asking me to be active.”
Today, he sings a different tune. I mean, he’s no Arnold Schwartzenegger, but he wants to stay fit and ambulatory. When a basic function like weight-bearing is taken away, and you are lucky enough to get an improved version back (walking heel to toe with less pain), every step becomes a small miracle. Never will we take the small things in life for granted.
It’s a rigorous agenda; and certainly not for the faint of heart. Some end up here willingly, and others just happen to land here unknowingly, without a means to escape. Yohan, a victim of the latter scenario, is counting down the days of this grueling summer when he flies free into the real world.
“Yohan, ” I venture, saying his full first name for the effect, “Once that walking cast is off, boot camp starts here at home – immediately – and comes to a close with your departure at the end of August.” He furrowed his brows in annoyance and sounding more irritated than usual, he shot out, “OMG, what are you even talking about? You get weirder and weirder every day. Could you please leave now? I have to get ready to go.”
Well, that went well, I thought. Hmmmm…. Note to self – Plan B -must be more abrupt.
A couple of days later, the walking boot came off and I cautiously began to implement and move forward with my cunning plan. If you want a sense of what his days have been like the past 2 months, read further for the highlights, and a few lowlights.
Rise and Shine! It’s 11:00 am on a Monday morning, quite early for a gamer who plays World of Warcraft or League of Legends into the wee hours of the morning. I carefully crack open his bedroom door, wiggle my bugle into the narrow sliver of darkness and blast “Reveille” at a piercing tone, which only Yohan hears as most of the neighborhood moms and dads have already left for their 90-minute commute to work.
Click below to hear exactly what went on that morning:
“Time to GET UP! UP! UP! UP! Today is the first day of the rest of your life!” I yell with parental satisfaction.
I hear moaning and groaning and a sudden thump of some hard object against the door. Uh, oh….maybe that was a bit over the top. I think I’ll retreat and let him sleep a bit longer. If he actually falls back asleep, there is a small chance that he’ll think that entire episode was just a bad dream.
Well, so much for that technique. Maybe a more gradual approach?
Yes, soldiers. Boot camp HAS changed since you were kids. Deal with it. Firing off commands and expecting immediate action don’t work as well as it used to. Subtle manipulation really is the way to go.
Once Yohan started speaking to me again, we established a routine. Most days go something like this:
11:30 AM – Breakfast
11:45 AM – Ankle range-of-motion exercises
11:50 AM – Break
12:00 PM – 10-minute walk
12:15 PM – CMTA-related calls and work.
3:00 PM – Lunch
3:30 – Ankle Range-of-motion exercises and 10-minute walk
4:00 PM Free Time
2-3am – Bedtime
And at 11:00 AM the next day, we start all over again. Honestly, I’m not sure how he does it all with only9 hours of free time, but he manages to fit in all sorts of other activities too: allergy shots, physical therapy 2X/week, doctor appointments, play with the cat, bring out the trash, help around the house, etc…. It’s truely amazing.
Seriously though, he’s doing well. Very well. Although I may poke fun, I told him that his only real job this summer, besides his 2-3 hours/day working for the CMTA, was to take care of himself, get physically fit, and to strengthen his calf, leg, foot so that at the end of August, he can walk right out of this house and on to grad school in southern California. And that is exactly what he’s going to do.
Dr. Pfeffer checks in frequently, for which we are all very, very appreciative. We’ve sent him pictures, video clips of Yohan walking, more and more pictures. At first, I was concerned because his foot still looked overly pronated. We were told not to worry about it…..yet. Healing takes a while and all the muscles in his foot and leg need to strengthen and relearn their own particular jobs. And, he has been walking without blisters, pressure sores, ulcers, pain, which is the best news ever.
In all of this, Yohan stood unwaveringly positive. Just last week, he again taught me a very important lesson about expectations.
Me: Walk back and forth down the hall again. Geez, ya know, that foot still does not look straight to me.
Yohan: I’m walking pretty well.
Me: I know, but I wish the foot did not turn in like that.
Yohan: Listen, Elizabeth. I’m walking. I am not getting sores on the bottom of my foot. It’s not too painful. The pressure is evenly distributed across my entire sole. I’m getting orthotics next week. You just gotta’ stop worrying. It’s not helping me. It’s not helping you.
He was right. Absolutely, 100% right, and I told him so, “Thanks for the reality check, buddy.” I constantly need to remind and convince myself that good enough trumps perfection. Always striving for perfection, a concept that is not even humanly possible, is defeatist – it will only lead to continual disappointment and dissatisfaction. I don’t want to live like that. Why do I set myself for failure time and time again? Old habits are hard to break. As Yohan would say, “Your perfectionistic tendencies are a “you” problem. You should work on that…”
On second thought, kicking him out of the nest may not be such a bad idea.
Year after year, our sponsors generously support the Cycle 4 CMT with silent auction items as well as donations of money, time and service. A big, heartfelt thank you to all our generous sponsors, without whom the Cycle 4 CMT would not be possible. As a sponsor, you will be mentioned on our website, in our printed materials, and at the event. In short, you will get tons of exposure while supporting a worthy cause – CMT. It doesn’t get any better than that! Join our growing team of Cycle 4 CMT sponsors. Email Chris Ouellette: firstname.lastname@example.org
The 4th Annual Cycle 4 CMT is open for registration! The event is scheduled to take place at the Old Lantern Inn in Charlotte, VT on August 27, 2017.
Cycle 4 CMT
This year, come out to cycle one of four beautiful cycling routes that travel through the scenic Vermont towns of Shelburne, Charlotte, and Hinesburg. All routes take cyclists over a covered bridge offering beautiful views of the Adirondack Mountains while experiencing the rolling hills and farm country of Charlotte VT.
Register to attend the fun after-party and enjoy great food, drink, live music, friends, and family. And if for some reason you are not able to come to the event, sponsor a rider or make a donation to the event.
Over the past 3 years, the Cycle 4 CMT has raised an amazing $425,000 for CMT research that will stop the progression of CMT. It means more than you can imagine to Yohan and to our family, who will fight to the end to find a treatment for this insidious disease. Please join us again this year, on August 29, for a fun and memorable event!
A Message from Yohan
Thanks for reading my story!
Did you know that June 20th marks one year to the day of my first surgery? This means that I’ve been stuck for just about a year, at home, with the parental units. I have got to get out of here. HELP!
Nevertheless, I am feeling hopeful. On May 27, I took my first steps on the surgically repaired foot and it wasn’t as bad as I had feared. Truthfully, I was really nervous about walking. Would this surgically repaired foot full of pins and screws be able to hold my weight? Would I feel the screw in my heel or my big toe? Will the surgery be successful?
So far, so good! I’m still wearing the walking boot 24/7. I can’t put full weight on it yet, and in all honesty, I’d rather go slowly and ease into walking. I also started physical therapy last week so I have to do daily exercises to get the muscles and tendons in my foot and leg working and stretched out. The worst part is the desensitization exercises where I gently rub the skin on my foot and leg with a soft bristled brush to get my nerves to calm down. They overreact to the tiniest of stimuli; simple touch feels like pain and tickling at the same time. It’s very unpleasant.
My soft protection boot comes off this week and I finally get to put my foot in a shoe. Wish me luck. I feel pretty anxious.
Thank you for all the support, thoughts and prayers. You’ve buoyed me up when my spirits were down and now, I will not let me or you down. And don’t forget to support the Cycle 4 CMT event. You are my lifeline and never will I forget your efforts on my behalf. Here is the link to join our team: cycle4cmt.com
The recovery experience, so far, has been nothing, and I mean nothing like the first surgery in June of 2016, which is the best news ever. And this is how I’m feeling about that:
We took preemptive measures: The anesthesiologist beefed up the nerve block so it would last longer. And we started his pain medication well before the block wore off. No ER. No crying out in pain. No hiccups.
Well, that’s not altogether true. He had a bad case of the literal hiccups-the involuntary spasms of the diaphragm kind, the day after surgery. Not once, not twice, but 17 times. 17 bouts of annoying hiccups!
After the 5th attack, we Google searched solutions. He tried almost all of them – holding his breath until he passed out, breathing into a paper bag, freezing a metal spoon and sticking it on his temple (don’t try this at home…you’ll just end up with a headache and freezer burn), gargling with salt water, etc, etc, etc. He was about to call his doctor when he came up with one last remedy – swallowing a tablespoon of honey. It worked, people! Relief. Hiccups can be caused by acid reflux and the honey soothed and coated his esophagus. Score!
2 weeks post-surgery, the stitches needed to be taken out. Last time, every angry suture came out screaming, zapping Yohan with electric shocks. It was extremely unpleasant and almost intolerable. This time ‘round, Dr. Pfeffer told him to take extra pain meds before the dreaded plucking of 75 pieces of rough and tough nylon delicately holding his skin together AND, he suggested numbing spray. Yohan relaxed. When strings were no longer attached, Yohan admitted that the process went, “1000 times better.” The suture removers at Pfeffer’s office were all like, “Hey, why don’t we use this stuff more often?” And “I hope he lets us use this spray on every patient….it works wonders!” I’m not even going to comment on those last 2 sentences because all I feel like saying is, “Duhhhhhhhhhhhhhhhhhhhhh.”
The first week is always the hardest…on me. Zoned out on powerful medication that eradicates short term memory, communication with my son got somewhat frustrating.
Every “conversation” went sort of like this:
Me: “Want something to eat?”
Me: “To eat.” I repeat, forcing my first four fingers together pointing to my open mouth.
As I stare at him, waiting for him to answer me, his eyelids get heavy, his head drops forward and off he nods. Sigh.
Me: “Yo” I nudge, gently touching his shoulder.
Yohan: Quite startled, as if I had screamed in his ear and shook him hard by the shoulders, he shouts wildly, “HUH? WHAT?” with terror in his eyes.
“Where did I go wrong?” I wonder.
It’s right about now that I felt like jumping in the car and going shopping. Shopping always seems to lift my spirits, for at least 10 minutes, until I look at the receipt and realize just how much I did not need whatever is in the bag.
When my husband Gilles gets sick, he acts like a wounded cat. He isolates. We make scarce, get out of his face and leave him alone. When I feel ill, I crave attention. When I was young and growing up in Vermont, I used to try to catch a cold by opening my bedroom window wide, letting snowflakes land in my hair and frigid wind blow into my airways. It never worked, not even once. I loved school but I wanted my parents to stay home and take care of my every need and desire. I’d say that Yohan got my genes in that department.
I literally sat next to Yohan for the first week, supporting him through the boredom, the pain, the medication regime. I even tried providing humor (apparently he does not think I’m very funny).
For example, I changed his pee bottle up. This time, instead of an “Honest Pee” container, I offered a Chia Seed container, which we conveniently renamed the “Pia” (Pee-yah) seed bottle.
I bought tons of his favorite fruit: Sumo mandarins, the gigantic, juicy mandarins available for a limited time only in February and March. He asked for one, peeled and segmented, so I took one of our teensy tiny mandarins from a young tree in our yard, and gave him a bowl with the smallest mandarin pieces I’d ever laid eyes upon. Yohan looked at the bowl in his lap, blinked once, twice and then just stared at me, completely confused. “HAHAHAHA, Just joking! ” I cackled. “Elizabeth, don’t do that to me! I’m already having a hard enough time focusing, he complained. And then he mumbled something to the effect of, “ Please Universe, send someone to my rescue.”
Someone in the infinite cosmos was listening. That same day, the phone rang. My sister Kathy from Vermont asked if I could use a hand.” Can you find a flight that comes in tonight?” I inquired sleepily, and only half-jokingly. She called me back later that day. I got my ticket!! I’ll be staying a month (a month? I hope we’re still friends after a month), and I arrive in 48 hours. “Hang on sissy,” she said encouragingly. “I’ll be there before you can say Pee- ya seeds”!
Can you say life saver? That’s exactly how I feel about Kathy’s visit. She cooks, cleans, gardens, laughs and takes great care of Yohan. I’ve been able to catch up on my sleep, for which friends, family, husband, and colleagues are immensely grateful.
To everyone who has sent us messages, offered words of encouragement or just simply checked in to see how things were going, I can’t tell you how much we all appreciate it. Thank you for your kindness. How grateful we are for each and every one of you.
Yohan now is sporting a boring white cast (that will soon change) and a full beard which he is not shaving off until he can walk again (OMG). He’s doing quite well. I have a good feeling about this last surgery. We’ll get this kid up and walking yet. He’s a survivor!
PS: Maybe the beard is not so bad. I think it is growing on me.
Yohan had his second 7-hour surgery ( a redo of his left foot) on Wednesday, March 29 at Cedars-Sinai, in LA with Dr. Glenn Pfeffer. As you may have guessed, his first surgery was not successful. We will probably never really know why, but apparently, some of Yohan’s muscles in his lower leg were unusually strong, which made for a very successful tendon transfer (a strong tendon replaces the weak tendon to correct foot drop.)
Yohan is now able to lower (plantarflex) and raise his foot (dorsiflex) his foot (that’s the good news). The bad news is that something went awry. The end result was an extremely pronated and flat foot, a misaligned heel and a big toe that was completely crooked.
Bottom line – he was not able to walk on that foot. He’s been using a knee scooter and crutches for the past 10 months. HE’S BEEN COMPLETELY OFF HIS LEFT FOOT SINCE THE 3rd Annual CYCLE 4 CMT IN AUGUST OF 2016!!
Dr. Pfeffer looked far and wide for answers, and more importantly, a solution. Several ideas were floated around and the option we like the least was to fuse the subtalar joint or the joint directly below the ankle (due to the possibility of it being hypermobile). He wasn’t thrilled about this option as you lose lateral movement and fusion is usually last resort procedure.
After much discussion, this is what ended up happening:
Pfeffer straightened Yohan’s big twisted toe with a fusion. He no longer can bend his big toe, but he has no toe movement anyway, and it was just getting in the way. Now it will stay put. He took his big toe tendon, which he no longer needs, and put it on the inside of the foot, creating a normal arch.
He also unscrewed the screws from the first heel osteotomy, filling the holes with cadaver bone, realigned the heel and reinserted the screws to hold the new position. The three other toes were lengthened by cutting the tendons and shaving off toe bone to straighten them.
He also lengthened the Achilles tendon, which has always been very tight (this is why he walked on his toes when he was little). With such a contracted, stiff foot, surgery is always that much harder Also, he had an overabundance of scar tissue, which very difficult to work with.
I keep obsessing over the cadaver bone part. Like, whose bone did he receive anyway and what was that person’s story? Was her name Eloise or Gertrude? Was his name Emilio or Tom? Was the person young or old? Where did the bone come from a hip, arm, knee or foot? Did that person have osteoporosis or bones of steel? I guess we’ll never know the answers to the questions which haunt me. I just want to thank that person, whoever you are for your bone donation. Your selflessness may help my son walk once more.
Dr. Pfeffer believes that this second surgery has a 95% success rate, but really, we will not know for months, when walking and rehab begins.
We are all so glad it is over.
After surgery, Gilles and Yo drove back from LA the same night and did not arrive until 4am. Since he has such problems with pain the last go round, we decided to medicate him well before the nerve block wore off. That seemed to have worked, as he has not complained of much pain to speak of. A Relief.
Yohan is pretty out of it at times, but we are already starting to bring down his level of pain medication. Gilles and I are taking turns medicating him every three hours, especially at night. Yo returns to Cedars in 2 weeks to get the soft cast off and the fiberglass cast on.
We are very hopeful that once the foot heals, he will be able to walk and get around.
Yohan has been accepted to 2 graduate schools in the fall, both in LA, so he is excited about the possibilities that await him in the future. As much as he loves us, and we love him, regaining an autonomous life is the ultimate goal.
Please keep him in your thoughts and prayers. And, thank you for your love, support, and kindness. It means the world to us.
I started writing this post last night, as a way of getting my feelings out and on “paper.” But, the more I wrote, the worse I felt. I shut down my computer and used my “call a friend” coupon. When feeling blah, I usually hide in a shell, stick my head in the sand, curl up in a dark corner. This time, I chose to change things up a bit. “Hey Bethany, Got a few minutes?” After ranting on and on for a while, I was able to balance out my thoughts and get neutral; I may have even sensed a twinge of positiveness, a crack of light in a darkened room.
Here is what I wrote before speaking with Bethany:
In June of 2016, Yohan had reconstructive surgery on his left foot. From the get go, he had more than his share of issues.
The nerve block wore off quicker than expected, and his pain spiraled out of control.
The day after surgery, he waited for hours and hours, in unbearable pain, at the local ER. After a 6 hour wait, he was called back, examined and admitted. Joint, bone and nerve pain continued to be prevalent at least 6 – 8 weeks after the surgery.
Once he was put into a walking boot, pressure sores developed on the bottom of the foot.
We made numerous trips to San Francisco (45-minute drive….. one way) to have orthotics made, adjusted and readjusted, and readjusted again to take the weight off the ball of the foot where the sores thrived. No go. They hung around, uninvited, like ants at a picnic, and threatened to multiply should he decide to put any pressure at all on that foot.
Every time the sore healed, it reopened when he tried to walk. He’s been off that foot, on crutches/ knee scooter, for9 months (That’s enough time to get pregnant and produce a little person).
For reasons unknown, the first surgery failed (except for the tendon transfer, which worked amazingly well).
After several trips back and forth to Los Angeles, where Dr. Pfeffer practices, it became obvious that a second surgery on the same foot was a necessity. There will be more bone cuts, tendons transferred and lengthened, heel realignment, toe fusion, and straightening.
Surgery is tomorrow, Wednesday, March 29. We are all anxious, want it to be over, but especially successful. He wants to simply walk, one step in front of the other. Is it too much to ask?
After my conversation with Bethany, where I released the pent-up negativity, I was able to open myself up to more positive energy and look on the bright side. During the past 9 months:
Yohan got to play A LOT of computer games.
He received tons of gift baskets (and money) from his grandmother.
He had the time and focus to apply and be accepted to graduate school in the fall.
He gained work experience as a CMTA intern.
Gilles and I were able to do a few short trips because he “volunteered” to take care of our high maintenance cat, Tortellini.
He rediscovered a childhood friend, and their friendship has taken hold and blossomed. (No Eva, he does not have CMT).
Vincent and Yohan
He did not have to make his own meals or do his own laundry for the past 10 months.
We played games as a family and enjoyed each other’s company.
Yohan now embodies the word – patience.
We had time to devise schemes and play practical jokes on Gilles ( for example, we put no tear toilet paper in his bathroom-hahahahaha)
Dr. Pfeffer has spent the last several months investigating what went wrong. He solicited second, third and fourth opinions from the world’s best orthopedic surgeons. He’s admitted that things can go wrong the first time around, “…but there is no margin for error the second try”. He’s done his homework, knows what he’s going to do and is proceeding with confidence. We are in good hands. We just have to remember to remain patient, determined and positive. And when you can’t stand it anymore, pick up the phone and call a true and trusted friend. The results can be mood-altering!
Elizabeth, Bethany and Jeana
Tomorrow, Dr. Pfeffer will perform an operation to straighten Yohan’s foot. Here are a couple of very cool images:
I hear voices. Eavesdropping, as if I were an innocent bystander, I witness the back and forth volley of words, arguments, and reasoning. Often, I put a harsh stop to the banter, for fear of losing too many precious minutes to internal disputes and emotionally draining debates. Trying to reach my neutral, rational space often feels impossible, like I’m swimming against a strong current intent on overwhelming my frantic efforts. Yet, somehow, someway, I usually manage to quiet the noise, at least temporarily, and transition into a state of calm deliberation, frantic activity or self-imposed numbness.
Yohan had his first ever foot reconstruction surgery in June and 8 months later, he’s still not walking without using crutches or a knee scooter (see previous blog posts for the whole story). As soon as he starts to put full pressure on his foot, sores develop.
When that happens, staying off the foot until it heals is the remedy. To offset the pressure, he had his shoes modified, new orthotics made, which were adjusted again and again and again, only to have the sore reappear when weight-bearing. I know that all surgeries do not go as planned. You probably also know that surgeries are risky, in many, many ways. But I thought the surgeries that went awry happened to other people. But this time, Yohan is that other person.
The holidays came and went, and in January we found ourselves at a standstill, not knowing what direction to turn. The last pictures I sent to our surgeon showed how Yohan’s foot had healed, and it did not look right. For some reason, his heel looked misaligned, causing excessive pronation and weight distribution imbalance. Our surgeon, Dr. Pfeffer, was perplexed, but to his credit, he’s promised to make it right. Putting ego aside, Dr. Pfeffer is determined to make the next surgery the “last surgery” by asking for second and third opinions from well-respected colleagues. He wants to make 100% sure that no rock is left unturned before going back in to correct the lopsided foot and straighten Yohan’s toes. His humility, commitment, and compassion command our genuine respect.
Left Foot After Surgery
Nevertheless, I dread this second surgery. I just want Yohan to be able to walk with ease, even if it is only short distances. And in all honesty, I may have felt, if only for a microsecond, discouraged, angry and saddened by all the challenges Yohan’s already faced throughout his 23 years on this planet, due to the effects of CMT. He complains rarely and manages his day-to-day with laughter, humor, and hope. Yet it is difficult to witness his debilitating fatigue, chronic pain and now, successive surgeries. I wish it weren’t so, but it is so and that’s what is true.
In general, I tend to live in my head, not my heart. Why open up to intense emotion, when the risk is getting sucked up into a vacuum of never-ending despair and misery? When my thoughts become dark, the voices in my head try to cheer me up, scold me for being negative and/or neutralize the negative with positive thoughts. This process has become so automatic that I often no longer know what I feel inside. Many believe that raw emotions, in all their complexity, are an undeniable part of the human experience, serving as a profound source of inner guidance and direction.
Yeah, well my inner feeling mentor acts more like a sneaky stalker than a trusted ally, ready to pounce when I am least protected. I’d rather stay in the neutral zone and avoid the war-torn areas of my life. Yet I often wonder where those intense feelings go. Do they evaporate? Remain in the body unexpressed and ignored until the pressure builds so, they just explode? Do feelings have feelings? So many questions, so few answers.
The second surgery does not yet have a date, but it will be soon, in the near future. We’re all doing our best and trying to live in the moment. We’ll get through. Thanks for listening. And if you have a moment, let me know how you cope through difficult times. You never know, your advice and feedback may just be of help to others someone else.
Good Friends are Hard to Find: CMT feet, Cheetah legs & Time travel
It was in the fall of 9th grade, and Yohan had just twisted his ankle, yet again. It was a bad sprain, nothing a couple weeks of icing and crutches wouldn’t cure. But, a bum ankle was just the tip of the iceberg. Chronic sprains, neuropathic pain, footwear woes were more the norm as his CMT progressed. His good friend Will, trying to make Yohan feel better, innocently came up with a solution to stop the madness. “Yohan, why don’t you just get below-the-knee amputations? I bet they could give you an awesome, high-tech pair of Cheetah Legs, and then you could run, play sports, hike….you could do it all!, ” he said as his thoughts drifted to Yohan’s first gold medal sprinting win at the Paralympics.
As crazy as this idea of artificial limbs sounded at the time, it didn’t seem so far-fetched today.
Here are the facts:
-Yohan had reconstructive foot surgery in June.
-As soon as he was given the go ahead to walk, he developed a pressure sore on the ball of his foot.
-Pressure sores are persistent and in his case, a sign that his foot mechanics are off.
-Orthotic modifications have not been helpful.
-One surgeon suggested surgically lifting the big toe bone, and straightening all the toes, while a second surgeon had a completely different perspective. They do agree on one detail: Both think another surgery is imperative to get him back on his feet. We were hesitant to get a second opinion- it often confuses the picture even more, and then the patient is left to figure out the “right” solution.
Lately, I find myself saying the “F” word ….a lot.
Why? I am angry, frustrated, scared, disappointed, and did I mention, scared? It feels as if we are rolling the dice or playing Russian roulette: One wrong move and BANG!! You are no longer walking. Maybe a classic case of negative thinking, but that’s the analogy which came to me, so I used it.
Today, I’ve had a bad case of the “shouldawouldacouldas,” otherwise known as regret or backward thinking. If only if we could just go back to May, to the joy and happiness of Yohan’s graduation day, with the knowledge we have today. Maybe the surgery would have gone differently. Maybe we would not even have had the surgery. Maybe he did not even need surgery. High? No, I am not high….a bit delusional perhaps, but not high.
Balderdash!! STOP. REWIND. The only way forward is to look ahead, not back.
If I invest in anything, it will be in Yohan’s future and the future of so many with CMT. At least in this realm, the CMTA is making tangible and reality- based progress. If you do not know about the STAR or Strategy to Accelerate Research initiative, click here: http://www.cmtausa.org/research/our-star-strategy/
We are not really ready for the cheetah leg prosthetics. Having chronic pain myself, I too fantasize about getting cool-looking prosthetic limbs. It’s tempting. I mean, who wouldn’t want to look and get around like actor, activist and athlete Aimee Mullins?:
But, both Yohan and I are kind of attached (literally and physically) to our own feet and calves, And then, of course, there is that problem of nerve pain, phantom limb pain, emotional turmoil, financial considerations, etc…..it’s a big decision, one we are seriously not considering. But Will was right, they sure are impressive. Love you Will!
6 months later, he is still crutching around the house. Every time Yohan attempts to walk on the surgically repaired foot, he develops open sores and blisters on the ball of his foot. We were hopeful that the custom-made orthotic would shift his weight to a better, more functional position. It does, but his fragile skin just plain cracks under pressure.
Infection is also an on-going concern. With little sensation on the bottom of his feet, the seepage from the sore was the only indication something was amiss. Now, to avert danger, he inspects his soles day and night with a telescoping mirror.
“You need another surgery on that same foot?” I repeated incredulously as if I had not heard him right the first time. Sounding deflated, he explained, “Yeah… Dr. Pfeffer wants to take the pressure off the ball my foot, by surgically lifting the bone. He also wants to straighten my toes, and I’m not too sure about that. I’m going to think about it. Anyway, all in all, it’s a 6-8 week recovery period.”
Remaining calm and collected on the outside, I steadily asked a few more questions, showed my support and told Yohan we would do whatever it takes to get him walking again. But inside, I’m all like, WTF? Another surgery? You gotta’ be kidding me. My friend, Bethany had her feet surgically repaired and now she is walking all over the place. In fact, she can walk further and longer than most people I know. And Jeana had surgeries…ONCE on each foot, and now her feet look great!She has funky pinky toes, but hey, she can dance, walk, and exercise.
As I thought about this new piece of information, I suddenly remembered all the community members on our Facebook group who had mentioned having multiple surgeries – 10, 15, 20 operations over the years. I had put these people in the category of anomalies. They must have had really bad surgeons or maybe the procedures were done a long time ago when surgeons did not know CMT and really hadn’t a clue as to what they were doing. Today, surely, with advanced technology and techniques, a second surgery on the same foot within a 6-month time frame is probably unheard of.
Many DO have multiple surgeries to correct CMT feet. It may not be news to you, but it just hit me that I must be completely delusional.
As I count, I realize that it is more common to require several procedures on each foot, and then some over the years, than to have one surgery and be done with it. In fact, just last week I ran into a woman at the local fruit stand who commented on the Shark-O-Marie-Tooth bumper sticker on my car. “Oh, I see you have that too,” she muttered as she whisked by me. “What do I have?” I asked a bit confused. While feeling the ripeness of a cantaloupe, she offered, “ That thing, you know- Charcot-Marie-Tooth. My husband has that too.” She moved on to the kiwi, popping bite-sized samples into her mouth. “He’s in his 70’s now and he’s had over 23 surgeries on his feet over the years.”
I almost choked on the chunk of apple that lodged itself in my throat. Funny how reality changes once you open yourself up to the world. Haven’t you ever heard a new word, idea or process, and then you hear about it again and again, wherever you look. This phenomenon actually has a name: The Baader-Meinhof Phenomenon. Never heard of it? You will. Now that you are in the know, your unconscious will be scanning the environment looking for this word and you will be surprised at how often it will crop up from this day forth. This phenomenon even has its own Facebook page: https://www.facebook.com/TheBaaderMeinhofPhenomenon
Where was I? Ah, yes…back to Yohan.
The second surgery on the same foot is not scheduled yet, but come January 3rd, a call to Dr. Pfeffer’s surgery nurse will be made. Welcome 2017…….it can only get better, right?
PS: It’s not all doom and gloom. Yohan is finally using the knee scooter we’ve had since day 1. He wheels himself around the house with ease, running into people, objects, walls. He’s become quite adept at steering and getting around. Wheeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeeee!