Julie Hoover-Fong of the
Greenberg Center for Skeletal Dysplasias
and the Institute of Genetic Medicine
on the challenge of improving health care
for Little People:
How did you end up at the Greenberg Center?
HOOVER-FONG: I didn’t ever really plan on being a skeletal dysplasia specialist, but I did know since the sixth grade that I wanted to be a doctor. I was a geeky kid who liked science and had a sister who was very ill, so I knew something about children’s hospitals and was intrigued by medical treatment in pediatrics.
I’ve always gravitated toward patients who had unique problems: people who needed help from multiple kinds of services. That’s why I went into genetics. During my fellowship here, when I was also involved in finishing my Ph.D. in clinical investigation, I had the opportunity to attend a national Little People of America event. It was a real immersion experience with a great patient population. A couple years later, this position opened for clinical director.
What motivates you to come to work?
HOOVER-FONG: The realization that there’s so much we still don’t know about skeletal dysplasias, and so much more we can do to improve health care for this community—much of which is very basic.
Basic in what way?
HOOVER-FONG: For instance, we’re coordinating a multicenter study on blood pressure. When I became clinical director of the Greenberg Center, there was anecdotal evidence that short-stature persons had a greater incidence of heart problems than the general population, and people were clamoring for us to look into this. The way to start, of course, was by assessing blood pressure, height and weight. But there weren’t even blood pressure cuffs that properly fit short-stature people, so many simply didn’t check their pressures because the readings wouldn’t be accurate anyway. Just a couple of years ago, a cuff was designed specifically for adults with disproportionately short upper arms, so we designed a study and we’ve been collecting data ever since. It seems that little people do have a higher prevalence of hypertension than average-stature persons, maybe up to twice the prevalence.
It’s surprising that such a fundamental tool was lacking until recently. What other challenges do you face when providing health care for this population?
HOOVER-FONG: When I started directing the clinic, families would come to clinic for basic visits and ask if their children were fat, thin or appropriate? I couldn’t tell them, because we didn’t have any weight curves: You talk about a basic need! Every child who goes to a pediatrician has his or her height, weight and head circumference plotted on a growth curve to assess whether or not it’s appropriate or not. For achondroplasia—the most common form of skeletal dysplasia—curves were developed in 1980s for head circumference. Height curves were also available. But weight was not. So we built a collaboration with Dr. Charles Scott at AI duPont, and Dr. Kerry Schulze and Dr. John McGready at the Bloomberg School of Public Health and developed a new weight curve for achondroplasia that we published in 2007 in the American Journal of Medical Genetics. Also lacking were body-mass-index curves, which all children now are plotted on because of the increasing risk of childhood obesity. We published new BMI curves for achondroplasia in the American Journal of Clinical Nutrition, August 2008. And we’re hoping to continue doing this for other common skeletal dysplasias.