Living With Proteus Syndrome

May 30, 2009 7:58 AM

Just imagine if your legs continued growing long after the rest of your body stopped.

Born with a rare condition, Mandy Sellars' petite 84-pound frame is supported by abnormally large legs that weigh 210 pounds. Her doctors, who are still baffled by her disorder, have tentatively diagnosed her with a form of Proteus syndrome (made famous by The Elephant Man) for lack of a more definitive explanation.

posted by miss lynnster (47 comments total)

That poor woman.

I'm not sure what other kind of reaction there is to have to this, minus snark (or someone with more medical knowledge than I have).
posted by availablelight at 8:05 AM on May 30, 2009

Sometime in the last year I watched a whole documentary about this woman and other people with Proteus Syndrome. It seems to strike random parts of the body with no explanation. There were people with a single finger that was growing crazily. I always feel vaguely voyeuristic when watching these shows about strange diseases, but it's amazing how many things medical science has no explanation for.
posted by kimdog at 8:06 AM on May 30, 2009 [1 favorite]

Have a look at the comments section, if you ever need a reminder that mefites are generally decent people.
posted by kuujjuarapik at 8:07 AM on May 30, 2009 [7 favorites]

Tremendous timing, miss lynnster, after the content of Wurtzel post a few below. Glad I read it.

The writer says - with such good grace: If you're curious about something, rather than stare and laugh, come up and ask that person. I'm guessing the majority of people with a disability would rather people ask them what their condition is rather than stare at them.
posted by Jody Tresidder at 8:10 AM on May 30, 2009 [1 favorite]

"In terms of people coming in to help me, no. I'm too stubborn. I like to do everything myself, whether it's the cleaning or the washing or the ironing or something like that."

Good on her. She's not letting the condition own her. She's remaining in charge of her situation.
posted by netbros at 8:12 AM on May 30, 2009 [1 favorite]

I wonder if she's considered amputation of the limbs, since she's already wheelchair bound and driving with hand controls, and the condition continues to worsen over time.
posted by availablelight at 8:15 AM on May 30, 2009

I read about this lady earlier and I believe that's what she's going to have to do eventually.
posted by dunkadunc at 8:18 AM on May 30, 2009

I'm guessing the majority of people with a disability would rather people ask them what their condition is rather than stare at them.

Given the choice, yeah, but mostly I'd rather be left the fuck alone. That said, this woman is an entirely different league of having to deal with that, and I sure don't envy her.
posted by desjardins at 8:18 AM on May 30, 2009

She addresses the elective amputation question in the interview - as an inevitable possibility.
posted by Jody Tresidder at 8:22 AM on May 30, 2009

I wonder if she's considered amputation of the limbs, since she's already wheelchair bound and driving with hand controls, and the condition continues to worsen over time.

If you read the second page, she addresses that. She was talking to doctors about it but it was too dangerous. If her heart gets worse she's going to have to consider it.
posted by miss lynnster at 8:22 AM on May 30, 2009

My, god, the AOL comments. I'm going to Youtube to salve my soul after that.
posted by Devils Rancher at 8:27 AM on May 30, 2009 [3 favorites]

If you read the second page, she addresses that. She was talking to doctors about it but it was too dangerous. If her heart gets worse she's going to have to consider it.

[thanks...the paleolithic browser I'm using at work--don't ask--doesn't even show a page 2]
posted by availablelight at 8:37 AM on May 30, 2009

What? People check Metafilter from work?
I can't even imagine what it's like to have that luxury.
posted by dunkadunc at 8:41 AM on May 30, 2009

I don't know how to express the amount of sympathy I have for this women without it sounding like pity. I'm quite petite, as well, and I can recognize my gangly little stick arms in Ms. Sellars, and it has got to be hell just getting around. I can't even do a pull-up, with my normal legs. The idea of them weighing 200 pounds makes me think I'd just give up.

The fact she hasn't displays a strength much more important than any upper body strength.
posted by Juliet Banana at 8:42 AM on May 30, 2009 [1 favorite]

My legs are growing independent of my body [sorry]
posted by you at 9:05 AM on May 30, 2009

I was going to flag the AOL comments and move on, but it fires up an email app. I forgot we were dealing with AOL here.

They really shouldn't allow comments.
posted by cjorgensen at 9:05 AM on May 30, 2009

I couldn't finish it but I thought she was waaay cool. My heart goes out to her. She was so extremely matter-of-fact about it.

I always bear this sort of thing in mind and am grateful for my good health. Not a day goes by I don't think this - even when I'm sick (because I always assume that I'm going to get better, so far truthfully)...
posted by lupus_yonderboy at 9:58 AM on May 30, 2009

Now there's somebody to inspire you. And look at the pictures - she's beautiful!

STFU Elizabeth Wurtzel.
posted by futureisunwritten at 10:06 AM on May 30, 2009 [2 favorites]

Since no one had made the direct link from here, there is her website, mandysellars.com.
posted by dances_with_sneetches at 10:13 AM on May 30, 2009

I really like how dismissive she is about it. Her attitude seems to be "Maybe I'm different, but it's normal to me, so I'm going to go and have fun."

I dig graceful acceptance of one's self. Way to be.
posted by Turkey Glue at 10:27 AM on May 30, 2009

Why is it called Proteus? The seal-herding son of Poseidon? Maybe the shape-shifting thing, but still. Is it just that all maladies have to sound like Bond movies?
posted by cmoj at 10:30 AM on May 30, 2009

Either Bond Villans, or hyphenated European nobles: Engleman-Camurdrie syndrome, Ellis-van Creveld syndrome, Foix-Chavany-Marie syndrome, etc.

I was surprised to read that she had liposuction, and that it made the problem worse. Proteus syndrome is uncontrolled growth of skin and bone, so I'm not sure how fat removal would really help, unless she's saying liposuction as a general phrase for having tissue removed. On the other hand, having many inches of bone, skin or muscle removed sounds pretty torturous too.

I wonder if she's volunteered to have her genes mapped.
posted by boo_radley at 11:00 AM on May 30, 2009

You forgot Münchausen syndrome. As in Baron von.
posted by miss lynnster at 11:14 AM on May 30, 2009

What about the possibility of amputating her legs? I'd rather live with amputated legs and have the possibility of walking again (with the aid of prosthetics) than not at all.
posted by Malice at 11:56 AM on May 30, 2009

When I first read about this women, I couldn't really see what the problem was. I think her legs were a bit smaller then, but she just looked "normal" whatever the hell that is, or "average" in size and stature, but whatever it was it's all how she carries herself, like she might have to point the legs out before people would notice on their own.

She's gorgeous and beautiful inside and out, and it shows.
posted by loquacious at 12:17 PM on May 30, 2009

Why is it called Proteus? The seal-herding son of Poseidon? Maybe the shape-shifting thing, but still. Is it just that all maladies have to sound like Bond movies?

I was surprised to read that she had liposuction, and that it made the problem worse.

The section of her website on Proteus Syndrome says that the condition is indeed named after Proteus because of his shape-shifting. It also states that lipomas are common in people with the syndrome, so I'm guessing that the liposuction was to remove them.
posted by Thoughtcrime at 12:38 PM on May 30, 2009

I watched the tv documentary on her last year. The problem with amputation is that they'd have to go so far up into the hip that it will be a dangerous operation - she still has some mobility too. I loved her attitude that it made her special and it was who she was, so she was OK with not amputating. Lovely person.
posted by jimmythefish at 1:20 PM on May 30, 2009

I always feel vaguely voyeuristic when watching these shows about strange diseases,

I know exactly what you mean. On one hand, the pictures of Ms. Sellars are really astounding, because I simply had no idea that something like this could happen. It's fascinating. But then I feel like a jerk-off for being so intriuged, because it's not some really kooky weather phenomenom, it's someone's life.

In any case, she seems to have adjusted to it much, much better to the condition than anyone had the right to expect, so good for her.
posted by Uppity Pigeon #2 at 1:33 PM on May 30, 2009

When I read about people like Ms. Sellars I feel ashamed of myself for thinking that I have problems. Shit, I don't know what I'd do if I had something like that to deal with. I can tell you this, though; smiling so brightly as she does on that website wouldn't be part of the equation.
posted by metagnathous at 1:42 PM on May 30, 2009 [2 favorites]

She's got legs... And she knows how to use them...

Hey, someone had to whip out the ZZ Top reference here!
posted by jamstigator at 2:37 PM on May 30, 2009 [1 favorite]

No, they didn't.
posted by applemeat at 2:39 PM on May 30, 2009 [20 favorites]

Hm. I hope she offers to have her genes mapped. This condition is pretty fucking rare, as in only 120-200 people are alive right now who have Proteus syndrome!

And if I were in her position, I'd probably have my legs amputated. (Fun story: I nearly HAD to have my leg amputated once because I had compartment syndrome, but thankfully I only needed a fasciectomy.)
posted by kldickson at 2:58 PM on May 30, 2009

jamstigator is going to hell for that one.
posted by dunkadunc at 3:01 PM on May 30, 2009

"this is americia folks wheres the sympothy at?"
posted by D.C. at 3:28 PM on May 30, 2009

(Restraining self from making a Chun Li joke.)
posted by JHarris at 5:49 PM on May 30, 2009

If you're curious about something, rather than stare and laugh, come up and ask that person. I'm guessing the majority of people with a disability would rather people ask them what their condition is rather than stare at them.

Children are great with this. My 5-year-old son asked a woman he didn't know why she only had one leg, and she told him. I think she was glad to tell him that it was just a part of her life, that she got very sick and had to have her leg removed, but she's OK now.
posted by Mister_A at 5:53 PM on May 30, 2009

See Fanny Mills who "wore size 30 shoes, each pair made from three goat skins, with pillowcases as socks." Apparently, her condition was called Milroy disease.

And what metagnathous said.
posted by madamjujujive at 7:26 PM on May 30, 2009

This lady has serious balls. Absolutely amazing.

I can understand, from a psychological point of view, a reluctance to go through an amputation until absolutely necessary. Holding her back or not, her legs are part of her body and thus part of her identity. She may have abnormally large legs, but that's normal to her. This is the condition that she's experienced for the entirety of her life. To have her legs removed might solve the problems of bodily stress, but it's going to be a huge psychological blow to have to shift to identify yourself as "person without legs."
posted by grapefruitmoon at 8:28 PM on May 30, 2009

AOL Health: During a documentary you did with TLC, you traveled to the United States to find out what could be done to reduce the size of your legs. Did anything ever come of that trip?

Sellars: No. I went to a surgeon to see what he thought about the possibility of amputation and also to see a prosthetic specialist about fitting me with prosthetics if I should decide to go through with the amputation. I haven't done anything about that because I've been told by quite a few doctors that the operation itself is quite a dangerous one to do. So my thought process is simply not to have the amputation until absolutely necessary, and so if my heart starts having problems due to the weight then amputation is something to be considered. At the moment my heart is fine, but in years to come that may change. It may not. I don't think anybody knows what's going to happen.
posted by TheOnlyCoolTim at 9:38 PM on May 30, 2009

I'm not seeing comments. Did AOL remove them, or am I bad at the Internet?
posted by Mikey-San at 12:24 AM on May 31, 2009

Commenting about comments. How lame. As if there aren't plenty of tasteless comments that appear here.. many times from my hand! Oh no, we're way above AOL's comments. Truly a strange disease. I'm kind of glad she doesn't care enough to amputate. The medical field would be interested to see how far this develops, and to have the opportunity to study said development over time. PLUS, I'm sure she'll donate her body to science.. When her skeleton is on display, it'll be awesome! And all you pro-amputee morons will realize what a bad idea it was.
posted by ChickenringNYC at 8:13 AM on May 31, 2009

PLUS, I'm sure she'll donate her body to science.

What? How can you be sure of the wishes of someone you've never met regarding their mortal remains? Are you trying to a make a case that you're sure of it because you think she has an *obligation* to donate her body to science?

Because she doesn't. Being an anomaly doesn't mean that she's obligated in any way shape or form to be treated now, or after she dies, any differently than anyone else. She's not a two-headed fetal pig. She's not obligated to be your "WICKED AWESOME!" skeleton. She's a person who has the right to make her own medical choices without having to consider what you, or anyone else, thinks is "awesome."
posted by grapefruitmoon at 8:35 AM on May 31, 2009 [1 favorite]

Of course she has that right. I just said I'm sure of it because she seems so down-to-earth and practical about things. If doctors discovered a cure and could guarantee her legs would normalize, she might take that chance. Plus, I don't know if you really got my subtle joke about how there have been plenty of tasteless comments on this site.. especially from myself, and then I immediately segue into making one. Lighten up. Another thing she probably does is joke about her ailment; laughter is the best medicine is it not?
posted by ChickenringNYC at 8:39 AM on May 31, 2009

Laughing with someone is fine with me. Joking about her ailment is cool. If you want to say that her legs should each have their own passport since they need an extra seat on the plane, I'm down.

Saying that it means she'll be a rad specimen is demeaning and puts her on par with conjoined twins in a jar. And there we part ways in terms of thinking that this is funny.
posted by grapefruitmoon at 8:45 AM on May 31, 2009

Thanks for the definition of humor. Thought that was a completely individual thing.. after all, Family Guy continues to be wildly popular, for some reason. I don't think it would be FUNNY for her to donate her body to science. Are you trying to tell me you WOULDN'T be interested in seeing her skeleton? I'm just a curious person! I don't think it's a tasteless thing to say at all. Big deal. I don't want her or anyone, except Susan Boyle, to die any sooner than they should.
posted by ChickenringNYC at 8:49 AM on May 31, 2009

Thanks for the definition of humor. Thought that was a completely individual thing.

You totally misread me. I said specifically "And there we part ways on thinking that this is funny." Meaning that you find it humorous, and I don't. Thus, it's a completely individual thing.
posted by grapefruitmoon at 8:53 AM on May 31, 2009

Yes, Family Guy is wildly popular. But not because it's smart. Other wildly popular things? STDs. Obesity. NASCAR. Divorce. Jeff Foxworthy. The Da Vinci Code. The Hills. Movies about talking chihuhuas. And people re-elected George Bush too. Point being: common taste is not necessarily good taste.
posted by miss lynnster at 7:13 AM on June 1, 2009