Chandler Crews was born with the rare, genetic condition achondroplasia, the most common form of dwarfism. In 2010, she decided to undergo a series of limb lengthening surgeries, which over a four-year period increased her height to 4’ 11” from 3’ 10.” We spoke to Crews, founder and president of the nonprofit patient organization The Chandler Project, about her experiences living with the condition, her decision to undergo the surgery, and how she views new therapies working their way through clinical development that seek to promote growth in people with achondroplasia.

 

Editor’s note: This transcript has been edited for clarity and readability.

Daniel Levine: Chandler, thanks for joining us. We’re going to talk about achondroplasia, what it’s like to live with the condition, and the changing treatment landscape. Let’s start with achondroplasia itself. For listeners not familiar with the condition. What is it?

Chandler Crews: Achondroplasia is the most common form of dwarfism. It basically makes the arms and legs grow shorter and disproportionate to your torso. Everyone with achondroplasia has an average size torso, but the bones for our arms and legs grow at a slower rate and shorter. Then by the time they’re done growing, it’s kind of like the gene thinks they’re fully developed when technically they aren’t, since the bones didn’t fully grow. It’s more than just the short arms and legs. The gene also affects other things within the body as a whole. For example, people with achondroplasia have a very narrow foramen magnum. We also have a depressed nasal bridge–our nasal bridge has a deep slope. We also have spinal stenosis, which means there’s not a lot of breathing room really for our spinal cord within our spinal column. So, it’s more than just having short arms and legs. Usually someone with achondroplasia when describing it says, my arms and legs are shorter, and that’s the most physical attribute, but it does affect the body as a whole.

Daniel Levine: I think a lot of people do think of this being a condition where, okay, so you’re short, how serious are some of the health consequences you talked about?

Chandler Crews: They really can be life threatening. When you have a baby born with achondroplasia or you have a kid with achondroplasia, early on in their life, one of the things you really have to focus on is their foramen magnum and everything involving their breathing, because they do have sleep apnea and can stop breathing. Anytime you hold a baby, you have to support a baby’s neck, that is just what you’ve got to do, but especially for babies with achondroplasia. We also have hypotonia, which is low muscle tone, so we are delayed when developing core muscle strength. They don’t hold their head up as soon as the average baby. Their milestones are delayed. Personally, I know families who lost their babies with achondroplasia because they stopped breathing, they just had trouble breathing and they couldn’t breathe on their own. Another issue or complication that people with achondroplasia are aware of is that they have a 50 percent chance of passing on the gene to their children. When two people with achondroplasia decide to start a family, they know that they each have a 50% chance of passing on the gene. The hope is that if they have a baby with achondroplasia, the baby only gets the achondroplasia gene from one parent and not from both. Getting the achondroplasia gene from both parents makes that baby double dominant. When that happens, they just don’t make it home from the hospital. It’s heartbreaking and that is something that you have to think about when starting a family– do we want to just adopt or do we ‘risk it.’ It really is more than just being short like a lot of people think. You see it in the media with all of these shows and they’ll say, “we’re just like everybody else, we just do things differently or we’re just shorter.” That’s honestly a pet peeve of mine because it’s really not true. There are a lot of other concerns that come with having achondroplasia and other forms of dwarfism as well. You see all these shows about these different families or individuals living with achondroplasia or dwarfism, and the synopsis of all these series is: we’re just smaller and do things differently. They never really dive deep into the actual genetic side of things. I think that’s really important and needs to be discussed more.

Daniel Levine: Well, you’re talking about some of the hidden aspects of condition. From the flip side, the manifestation of the disease in its most obvious way is very visible. Are there psychological consequences of having the condition?

Chandler Crews: It does not affect the psychological sides of things.

Daniel Levine: I mean more in the sense of being in a world where people can obviously see there’s a condition. What’s the emotional life of having the condition like?

Chandler Crews: Growing up, I was the only person with achondroplasia in my family and we just treated it like a medical diagnosis that I dealt with. It was easy for me in grade school to explain it to my friends or anyone as, ‘I’m short because I have achondroplasia,’ given I didn’t know anything about the gene when I was eight years old. Then as I grew up and started to understand it more, I would be able to say, ‘it’s more than just being short, I’ve also got to deal with X, Y, and Z.’ Over the years, especially this past decade, it’s really become important for me to advocate and to spread awareness about how it’s more than just the bones and it’s more than just being short, ironically, because I actually went through orthopedic surgeries to lengthen my arms and legs. I find it funny how it’s so important for me today to advocate more for the genetic sides of things because of what I’ve done orthopedically. A lot of people only see the orthopedic sides of achondroplasia, and don’t really see the genetic side of it. Having gone through that, I realize how it really is such an issue that people aren’t aware of how severe the gene is and how it really does impact you for your whole life. So, it’s really important to me to spread awareness, that it’s more than just being short and it’s more than just what’s in the bones.

Daniel Levine: I want to get into some of that. In terms of daily living, what is it like to live with a condition in a world that is built for people of different proportions and sizes? What’s it like to go to the grocery store or use a public restroom?

Chandler Crews: When I was 16, I was fully grown, and my full adult height was 3’ 10” tall. People always ask me, what was that like? How was that? And honestly, it was just my normal. I had nothing to compare it to. I could see how my friends did things, how my siblings did things, and I saw it was easier for them to do different daily tasks than it was for me. At the same time, I had been doing it my whole life and that’s all I knew. So, when people would ask me, how do you do things? I’m like, this is how I do it. Yet, I decided to go through these procedures and I knew the benefits that I would get from that. But to answer your question, it was a challenge and I know it is a challenge for everyone with achondroplasia. It is unfortunate but true that there is a functioning height in society, that anyone who is from five to six feet tall doesn’t really struggle with everyday things, but if you’re under five feet or if you’re over six feet, you do struggle. Just doing everyday tasks, I remember there would be times where, my mom would pick me up from school one day and she’d be like, I need you to run into the store for whatever while I’m in the car. There would be times where I couldn’t, because I was too short. Sometimes the automatic sensors wouldn’t sense me, and the doors wouldn’t open unless I was walking next to somebody the door could sense. Also, the doors in public restrooms are so heavy or the handles are so high. When you’re 3’ 10’’, you can’t reach up to pull the handle to open or close the door. So, we always had to think about that when we would go out. I remember in my high school there was this one bathroom in our upstairs hallway where you could get in and out from both ends of the bathroom, but at one end you would push the door to get in and on the other end you would have to pull the door to get out. When I would go to that bathroom, I would go in on the end where I could push the door open and then leave on the opposite end to push the door to get out. I never went in and out of the same door because I couldn’t reach the handle to pull it open. But again, that was just my normal and that’s what I knew. I think that is what it’s like for anyone with dwarfism, it’s just their everyday norm. But when I was 16, I decided to go through these procedures and I went through two leg lengthening procedures. So today, I am 4’ 11’, just shy of five feet now. I never realized how much my height did affect everyday things because I was just so used to it. Once that changed, I started to do things that I never even realized I couldn’t do before, and it really was eye-opening.

Daniel Levine: What was the biggest change? What was the most eye-opening experience?

Chandler Crews: For me, it wasn’t until after my second leg lengthening that I realized, and it’s weird how I didn’t realize it until after the fact, where I was able to have face-to-face conversations with my peers. I always knew I wasn’t having face-to-face conversations with them, but since I was so used to it, I never realized the actual impact that it had. That did change for me. Before having my procedures, I knew I couldn’t drive a car without assisted equipment but now I can drive a car without needing any assistance, and other everyday things. Really having that face to face communication was the biggest impact for me. Looking back, I remember having uncomfortable interactions where I could tell somebody was like, okay, do I kneel when I talk to her, do I bend over, do we need to both sit down at a table? I never really had to think about that, but the average-height peer I was talking to would always have to think about that. So, for that not to be an issue for me anymore was eye-opening. I never realized just how important and how impactful it is to be able to just go up to anybody on the street and have a face-to-face conversation with someone. That was the most impactful thing that had never occurred to me, for whatever reason, that I would be able to do that after going through my procedures. I also lengthened my arms and that itself was also a big impact because I am able to deal with hygiene and drive a car and sit a safe distance away from the steering wheel. I knew those things were a benefit going into it, but then actually doing it myself, I realized how much people take for granted being able to do those everyday tasks.

Daniel Levine: Before you underwent limb lengthening procedures. There was another moment that you had this kind of self-awareness, you were actually watching a video of yourself performing in a dance performance. What was your reaction to that video and how did it change your perception of yourself?

Chandler Crews: Every time I talk about this, it’s funny. I grew up in dance. I was dancing from the time I was two or three and dance was my life. My mom was a dancer. So, of course my sister and I got into dance. Dance became my thing. My sister got out of it pretty quickly. It was my life. I went every week and then as I got older, I started going twice a week. Every year you work up to the big summer recital. That wasn’t even the first time I ever saw myself dancing. My mom would get the tape or DVD every year of the dance recitals and we would watch them. As I got older, dance became more and more important to me and I took it more seriously. Dance is one of those things where when you’re looking at a performance and you’re looking at a group of people doing a dance performance, every person needs to be uniform, they need to be the same, and everyone needs to be on beat. If there’s one person that makes a mistake or stands out, you notice it. Again, it wasn’t the first time I ever saw myself dancing. I remember, my mom was playing the video for that year and we didn’t even get a few seconds into it before I told her to stop playing it. She was like, what? And I asked her, ‘Is that how I look when I dance?’ I could tell she did not know how to answer the question and didn’t understand what I was asking. I told her, when I’m on stage and dancing with my classmates, I feel like I look like them and that’s how I feel on the inside. When I’m out on that stage, I feel like no one can notice me and I feel like nobody recognizes me. Looking back at all of these moments in dance, people I didn’t know would always come up to me after the dance and say, ‘I loved watching you dance, and you were so great.’ I’d be like, I don’t know who that person was, but thank you. At the time, I was always like, they must’ve just thought I was a really great dancer. Looking back, they never said it to any of the other girls in my dance class. People would always gravitate towards me, people I didn’t know. I never realized that until after I had that moment where I asked my mom if that’s how I look when I dance. After that, I really thought about whether people that would come up to me were being honest or were they just doing that ‘inspirational porn’ where they see the girl that stands out because she has dwarfism, and they want to make sure they pat her on the back and tell her that she did a good job. Honestly, after seeing that one video, that’s kind of how I saw it. It didn’t do anything to my self-esteem, but I have a really hard time accepting compliments because I think back to dance. That really did impact me negatively. I think a lot of people with any sort of disability understand because ‘inspirational porn’ is a thing. For me, it goes back to watching that dance video. Anytime I would be on stage, I would think to myself, I just look like all of the other girls in my dance class and for whatever reason, it just never hit me until that one video. It was just one of those things where I finally thought to myself, that’s not going to change unless I do something about it myself. It was one of those moments where I was just like, I really am truly different from everyone else.

Daniel Levine: You made reference a couple of times to the fact that you have undergone limb lengthening. I don’t think most people are familiar with this. What is it, what does it entail, how many surgeries did you have to undergo to do this, and what’s the post-surgical period like?

Chandler Crews: So, limb lengthening is exactly how it sounds, you lengthen your arms and legs. It’s actually a fairly common procedure, not really for people with dwarfism, but for other conditions. Basically what they do, I’ll just kind of speak about my leg lengthening specifically, is they make an osteotomy in the both femur bones and then your tibias and fibulas in your lower leg. Now they actually can do it internally if there’s no deformity correction needed, which is great. To speak about my second leg lengthening experience, I needed correction done on my tibias. So, what they do for that is they cut the bone and then they’ll place screws in different areas on the tibias and then those screws stick out of your leg. They stay assembled together through these metal frames, which are called fixators. Those metal frames are held together by six different struts, it’s basically a screw, and you twist it almost every day. You get a schedule to turn the struts every day and it pulls the bone apart from where the bone was cut about a millimeter a day. Same with the femurs, but with the femurs they can put a telescoping rod right into the femur and within that rod there’s a little motor that is activated by an external remote control. The remote has a magnet on it, it sounds made up, but it’s true, and you put this magnet right where they cut the bone and the little motor is inside the rod and it activates it to pull the bone apart. Every day you’re lengthening your femurs and your tibias, and for achondroplasia you grow about a millimeter a day. If my calculations are correct, that’s about growing an inch in height every two weeks. Every two weeks I was about an inch taller, and with each of my leg lengthening procedures, I lengthened a total of six inches. That’s lengthening three inches in the femurs and three inches in the tibias for one surgery. Overall, it takes about three months to do the whole lengthening, but for every day that you are lengthening, they say you have a day of consolidation. You do three months of lengthening and then you have to do three months of consolidation. If you do have the external fixator devices on, you have to have them on during the consolidation phase, just to keep the bone in place and stable. So, you’re in these frames for about six months and then they come off and you’re six inches taller than when you went into surgery the first time. There is a lot of physical therapy and patients have to know that going in. It’s pretty extreme but it’s doable. It looks and sounds a lot more excruciating than it is. It is a lot of hard work and it is something you need to be committed to. You’ve got to go through this physical therapy. At the end of it, it’s very rewarding. I remember, after my first lengthening, being at this new height was a surreal experience. You never think you can actually make yourself grow taller. It’s exciting and it’s kind of weird at the same time, but overall very rewarding. I do also want to clarify that limb lengthening for dwarfism does benefit you medically. Before my procedures, my legs were severely bowed and I knew I was going to need some straightening done sooner rather than later at 16. I knew limb lengthening was an option and I knew I was going to get my legs straightened at some point. I just decided to do both. Also, limb lengthening the first time, and I’m not an orthopedist, they correct the pelvic tilt. People with achondroplasia, our pelvis is also affected. This helps alleviate pressure on the lower spine, which is very important. There are a lot of medical benefits that come with limb lengthening. It’s going to help you orthopedically and overall, that helps your overall well-being and your overall health, but it doesn’t change anything genetically and it doesn’t change any potential risks that I can have from the gene later on in life. It doesn’t change me passing on the gene to my children, should I choose to have any. It does help me live a better life for myself and a healthier life, but it doesn’t do anything to the genetic side of things.

Daniel Levine: A few years ago, you founded the Chandler Project, what is the Chandler Project and what does it do?

Chandler Crews: The Chandler project provides those affected with achondroplasia with the latest research and information in the different pharmaceutical and surgical developments that are happening and becoming available for patients. Within the past 10 years, there has been scientific discovery and pharmaceutical developments for potential therapeutic options for patients with achondroplasia. The Chandler Project helps to provide patients and families with access to that information. Unfortunately, there’s a large presence within the achondroplasia community that doesn’t support having therapeutic options, but there is also a community that does want those options. I saw that there was a void and decided to start my own non-profit and provide patients and their families with this information and help them be able to have access to these potential new treatment options, because they really weren’t able to get the information anywhere else.

Daniel Levine: It’s an interesting point you just mentioned. There are sensitivities within the achondroplasia community. There are people who feel there’s nothing wrong with them that needs to be fixed. How does this complicate the development of therapies and the treatment landscape?

Chandler Crews: It really does complicate the developments because there is such a large number of people who think they don’t need to be changed. The different therapies that are being developed aren’t to change anyone with achondroplasia but to help prevent and alleviate a lot of the medical risks and complications that can occur with having achondroplasia. Babies or people with achondroplasia can have sleep apnea, or they can have trouble breathing due to the narrow passageways, and with how the FGFR3 gene, which is the gene that makes up achondroplasia, impacts all of the areas in our body. These therapeutic options that are being developed are to help prevent those complications from occurring. When you have a child with achondroplasia, you’re not going to know what health complications they’re going to have. I was very lucky growing up. I went through with the routine MRIs that you have to go through and the different follow-ups, but I was really lucky that the only surgeries I ever had growing up, prior to my elective limb lengthening, were ear tubes. Unfortunately, I had a lot of friends that had to have spinal fusions, they had to have decompression, they had to have shunts, they had to have trachs, or they had to have their tonsils and adenoids removed. All these different things that you don’t know when you have a child born with achondroplasia. You’re not given this handbook of what complications they’re going to have. So, these therapeutic options, that will hopefully becoming available soon, are to help prevent the health risks and complications that can occur with having achondroplasia. I think it’s important that people know that it’s an option and that all of these therapies are to help prevent these complications from happening.

Daniel Levine: There are multiple therapies in development to treat achondroplasia. How do you view these and what expectations do you have for them?

Chandler Crews:

I view them all as different, but they all serve the same purpose. I think it’s very exciting. They are also different as far as how they’re administered, some can be injected once or twice a week, but overall, all of them serve the same purpose. I think it’s great that there’s going to be more than just one option for families, because the difference in how they are administered might be better for one achondroplasia patient versus another achondroplasia patient. To know the different options available is very important for families and patients. Everything is all still in development, but it’s exciting to have more than one option, especially for new parents who have a child with achondroplasia. It’s exciting for me, and I’m not a parent and not going to be one anytime soon but thinking about my future and knowing the risks of having a child with achondroplasia. They’re going to have more treatment options than I ever did. To have these different therapies available is very exciting and I know a lot of parents that are very excited.

Daniel Levine: Chandler Crews, founder of the Chandler Project. Chandler, thanks as always.

Chandler Crews: Thank you so much.

 

 

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