16-33 Segment 2: Little People and the Prospect of Cure

16-33A The Future of Little People

 

Synopsis: Many people with dwarfism also face skeletal abnormalities which can lead to disability. Experts, all little people themselves, discuss major causes of dwarfism, the hurdles they create, the struggle for respect, and the prospect of treatments that could one day make little people much more rare.

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Guests:

  • Dr. Jennifer Arnold, co-star, TLC’s The Little Couple and co-author, Think Big
  • Ericka Okenfuss, licensed genetic counselor, Kaiser Permanente, Sacramento, CA
  • Gary Arnold, President, Little People of America and Public Affairs Manager, Access Living, Chicago

Links for More Information:

16-33 Little People

Nancy Benson: Everyone faces challenges in life. But some are greater than others… and some we can’t even imagine facing ourselves. We have an innate interest in how people meet those challenges. It’s one reason reality TV shows are so popular. Among the many worlds we see on reality TV, a few have proven extremely popular—for example, the lives of little people, or dwarves, have been the subject of more than a half dozen shows. Dr. Jennifer Arnold is co-star of one of them, TLC’s The Little Couple, along with her husband, Bill Klein. They’re also co-authors of the new book, Think Big.

Jennifer Arnold-Klein: You know, I was a big baby, average sized baby and you know, the doctors knew something was wrong with me though because I had respiratory issues and I was a breech delivery. They knew something was wrong and that I had some abnormalities to my skeletal structure but they didn’t know what it was and I wasn’t diagnosed until I was about two when the doctors realized I wasn’t growing then at the same rate and accelerated height and weight that I should have. So they sent us up to Baltimore, Maryland to a genetics clinic where I was very quickly diagnosed.

Benson: Arnold suffers from a bone growth disorder called SED. As a neonatologist, she says she’s cared for many infants with similar disorders, which will result in dwarfism.

Arnold-Klein: I’ve had quite a few infants in my career that I cared for that had a skeletal dysplasia. Various types, probably most common I can tell you I’ve had children with achondroplasia which is the most common skeletal dysplasia today, which is similar to the type of skeletal dysplasia that our children have. And in addition to that though I’ve had some other types that are unfortunately typically lethal in the neonatal and there are certain severe types that children may or may not survive. And then there are others that are just much more orthopedically compromised where children have a lot more complications that can require lifelong therapy.

Benson: About five million people in the United States live with short stature. Officially, dwarfism is defined as being 4-feet-10-inches tall or less… but the average height of an adult with dwarfism is four feet. Achondroplasia accounts for about 75 percent of all cases.

Ericka Okenfuss: Achondroplasia is a skeletal dysplasia that primarily affects the growth of the long bones and it causes a disproportionate short stature, which means that the limbs are significantly short in proportion to the trunk.

Benson: That’s Ericka Okenfuss, a licensed genetic counselor for Kaiser Permanente in Sacramento, California. She says of more concern than stature, are the various medical problems that often come with achondroplasia. They include simple things such as ear infections, which are common because the head is shaped slightly differently, making ear tubes often a must. Hip joints are often problematic. The opening at the base of the skull may be too small or the bones of the spine may not grow correctly, putting pressure on the spinal cord. Gary Arnold is president of little people of America and public affairs manager for access living in Chicago.

Gary Arnold: So what you have as the person gets older is the vertebrae putting a lot of pressure on the spinal column. And then that can create issues with circulation and nerves in the arms and legs. And so what you see is a lot of people with dwarfism having spinal stenosis surgery where they kind of try to open up the vertebrae to some degree and also can result then in mobility of a person as they get older.

Benson: Officially, dwarfism is classified as a disability by the Americans with Disabilities Act. But Okenfuss says many in the little people community are reluctant to accept that label.

Okenfuss: I think a lot of people would tell you that in of itself is not a disability. But many, many skeletal dysplasia conditions or most of them come with other medical complications besides just being short. And often that’s where it kind of crosses over to disability. And you know, it all depends on how you view that word as well. I mean, most people with short stacks are differently abled, absolutely. They have to do things differently in order to exist in an average sized world. But does it end up being a terrible limitation on their quality of life? Not most of the time.

Benson: Still, Gary Arnold says the toughest day-to-day challenges for many little people come from simply living in a world designed for average sized people—reaching the pedals in a rental car, getting groceries off the top shelf at the store, or getting respect from people on the street.

Arnold: It’s just kind of natural to treat someone who’s shorter physically as a younger person. You find yourself being condescended or being treated like a child or being treated, you know, like you’re some kind of spectacle. Like, walking down the street and someone sees you and they treat you as if they’re seeing some sort of exotic animal at the zoo and you get pointed out to their friends or the worst is if a parent points you out to one of their children as if they’re at the zoo. I think that’s one of the most frustrating things cause that really shows that, like, you’re not seen as a person just like everybody else or if you are, you don’t want the same kind of respect that typical strangers will give to other strangers in terms of their private space.

Benson: One height of disrespect may be the use of what little people call “the m word.”

Arnold: The word midget is unacceptable and you know, if we were to ask just one thing, I think that would be the request of most little people is to stop using the m word. It evolved over the years into this derogatory term where really almost every time you see it used is used in an objectifying way or in a demeaning way.

Benson: Eighty percent of children with dwarfism are born to average sized people, usually as a result of a spontaneous mutation in a specific gene. Particularly in some other countries, social norms and lack of support make it difficult to raise a child with dwarfism. The little people of America run a strong adoption program for those kids… and a strong counseling program for new parents here.

Arnold: All of a sudden, you know, from out of the blue, might not know anything except for maybe they’ve seen a scene of “The Little Couple” or “Little People, Big World” on the reality TV then all of a sudden they find out that the son or daughter that they’re about to have is going to be a child with dwarfism. And we understand it’s not what you planned for and it could be a difficult thing to face but for people who do find themselves in that situation, I think the best thing to do is to just kind of step back and look at what’s out there and try to track down some people.

Benson: But will little people even exist in the future? Okenfuss says a drug is in clinical trials that could treat achondroplasia by interrupting the pathway of the genetic mutation… stopping it from telling bones not to grow. The main idea is to minimize complications such as an impaired spine… but youngsters treated with the drug would presumably also reach a more average height. And to some little people… that’s troubling.

Okenfuss: People in LPA are struggling with the idea that people like themselves may no longer exist in the future in a couple future generations. LPA has been all about accepting yourself for who you are and trying to make people feel like, even though they have dwarfism, they are fine just the way they are and they have a lot to contribute to this society just the way they are. So there’s a lot of pride even almost in being who we are and in spite of the difficulties, both medical and social that come with that, so we’ve never been out there looking for a treatment. A lot of people are really concerned that treatments like this not only will mean that there won’t be people like them in the future, but that the overall diversity of humans in general will be reduced.

Benson: You can find out more about little people of America at their website, lpaonline.org. You can find out more about all our guests on our website, radiohealthjournal.net. You’ll also find archives of our shows there, as well as on iTunes and Stitcher.

Our production director is Sean Waldron.

I’m Nancy Benson.

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