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California Opens First Group Home for Adults with PKU-Aug 2004

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California’s first group home devoted exclusively to treating adults with Phenylketonuria (PKU) was opened in April in Burbank. the new facility offers residents a special diet that has helped reduce their assaultive behaviors and the need for psychotropic medications.

PKU is an inherited metabolic disease that, when left untreated, can cause mental retardation and other neurological disorders. While newborns since 1966 have been screened for PKU, there are a number of older adults with PKU-triggered mental retardation who have fallen through the cracks of appropriate treatment. In California, there are about 170 adults with untreated PKU living in developmental centers, group homes, or living with their families.

The Koch Vagthol’s Metabolic Residential Home, owned and directed by Ann Seisa, receives support through state funding and can accommodate up to five residents. The home is specifically intended for Californians with PKU who are transitioning out of state developmental centers, and is named after Dr. Richard Koch, an internationally recognized authority on PKU, who serves as the home’s medical advisor.

Background: Seisa became interested in treating adults with PKU about eight years ago when she brought in 49-year-old Katie Stepanek with untreated PKU into another group home that she operated. (Seisa owns Vagthol’s Residential Care, Inc. in Los Angeles) Stepaneck had lived in a state developmental center for 30 years, was on several psychotropic medications, and was very combative. Seisa asked Dr. Koch, an expert on PKU at Children’s Hospital Los Angeles, for help. He prescribed a strict diet that depended on a commercially made product along with vegetables and fruit. After awhile, “things really calmed down,” Dr. Koch said. “She really improved”-so much so, they were able to cut her medications down to one. Stepanek is enjoying a much more normal life now and is alert and cooperative, caregivers report.

“This was inspirational and led to the movement we are engaged in now,” Robert Erio, Regional Options Unit at the Frank D. Lanterman Regional Center (FDLRC) in Los Angeles. (FDLRC is one of 21 regional centers that provide developmental disability services in California). Encouraged by the success with Stepanek, the FDLRC approached Ann Seisa three years ago about the possibility of creating a special home to serve former developmental center residents diagnosed with PKU who were returning to the community. “FDLRC had identified several individuals that, in our opinion, could be better served with the combination of dietary treatment and a focused, customized environment with well-trained staff,” Erio said. “Residents can remain in the home for their lifetime, if they so choose.”

Funding: The California State Department of Developmental Services’ Community Placement Plan provided start-up funds to establish the Koch, Vagthols Metabolic Residential Home, and the FDLRC provides ongoing support from its purchase of services budget. “It is estimated that the average resident of a state developmental center may cost anywhere from $170,000-$200,000 [per year] depending on a variety of factors,” Erio explained. “The rate for this particular customized community home–plus day programming, tranportation, etc., — may be closer to $120,000-$132,000 per year. Still a ‘win-win’ if you can demonstrate improved quality of life.”

The California PKU Late Treatment Project: Following the decision to establish a specialized community treatment environment for individuals with untreated PKU, the next step was to form a partnership between the California Department of Developmental Services (DDS), FDLRC, and Children’s Hospital Los Angeles, “where Dr. Koch and Kathryn Moseley [a metabolic dietician] do their good work,” Erio said. In 2002 the partnership created the California PKU Late Treatment Project to study the efficacy of the special diet, and facilitate the transition of other adults with PKU out of state developmental centers into group homes.

DDS approved $120,000 for FDLRC to fund the project for one year, and the FDLRC distributed those funds to Children’s Hospital to carry out the project. Recently, DDS approved $90,000 to continue the project through July 2005.

“We are hoping that this project will serve as a model for other states in improving the quality of life for [adults with PKU’,” said Kathryn Moseley, who serves as the project’s principal investigator. “It also saves money.”

Center Activities In February, the Center co-convened a training seminar with Agnews Develipmental Center in San Jose for medical doctors, nurses, dieticians, and social workers. The Center is being encouraged by DDS to conduct similar seminars in northern California.

In early July, Moseley and Dr. Koch sent letters to each of the regional centers in California to introduce the project, explain PKU, and invite them to set up a meeting.

Moseley also has developed a new protocol for treating PKU in developmental centers. The commercial products essential to the diet don’t taste very good and require a specialized kitchen to prepare them, so it has been difficult for a development center or other large institutions to implement the dietary program. Conflicts over a mix of diets among clients in one setting is another problem–giving a whole new meaning to “food fights”. That’s why a group home devoted exclusively to the PKU treatment is so effective, Moseley said. Nevertheless, her new protocol–that includes an oral agent and a less restrictive diet–addresses various stumbling blocks so adults with untreated PKU in institutions can improve while they wait to be re-integrated into community.

Moseley discovered the general extent of untreated PKU nationwide when she prepared a conference presentation in 2001. She sent surveys to 149 metabolic clinics throughout the United States to see if they were caring for adults with PKU. She received 66 responses and found that 17 clinics serve adults with PKU with the special diet. A total of 249 individuals started the diet, and 165 have stayed on the diet. Moseley plans to post survey results on the Center’s new website at http://www.pkulatctx.com.

Now that she has received state Institutional Review Board approval to conduct research on human subjects, Moseley can proceed with plans to study the effects of the diet on adults with PKU in California. She looks forward to publishing the results because it is critical to document evidence that the diet works in order to justify the treatment regiments to the state legislature.

FMI: Visit the website for the California PKU Late Treatment Project at http://www.pkulatetx.com. To learn more about PKU, go to http://www.lanterman.org/news/healthviews/whatispku.asp.