MOST HEALTH PLANS have disease management programs for diabetes, asthma and congestive heart failure down to a fine art. But what about less common and more expensive diseases, such as Parkinson's, Alzheimer's, hemophilia and multiple sclerosis?

Only a few disease management companies and plans specifically address these rare chronic conditions, probably because the populations are small and the ante is tremendous.

According to Drug Benefit Trends, 100 to 300 out of 100,000 people have Parkinson's, at an average annual cost per person of $2,161. About two million Americans have Alzheimer's who, the National Institute of Neurological Disorders and Stroke says, consume $2,500 per patient per year on medications alone. Accordant Health Services, based in Greensboro, N.C., is one of those companies that direct their disease management programs toward less prevalent chronic diseases, a roster of 15 conditions including Parkinson's, multiple sclerosis, cystic fibrosis, lupus, Gaucher disease and ALS (Lou Gehrig's disease). All of those diseases combined cost an average of more than $9,300 per person annually, representing more than $65 million in annual claims per one million plan members.

Accordant CEO/President Steven Schelhammer sees that as an advantage. "Because costs are high and prevalence low, we can more easily manage the population with a high-touch model and make a difference in savings," he says.

Accordant has been able to deliver more than $5.5 million in net savings to health plans for every one million lives and a realized net return on investment of at least 90%, the company says.

DIFFICULT TO MEASURE
"One difficulty, however, of low-prevalent conditions is that the smaller numbers make it difficult to measure outcomes in a statistically confident manner," says Dr. Warwick Charlton, senior vice president of product development and management for Accordant.

"Not only are these diseases less prevalent, but they are complex and progressive," Dr. Charlton says. "The conditions typically worsen each year even if they are treated. That separates them from the more common chronic diseases, such as diabetes and asthma."

He points out that there is also a lack of evidence behind managing rarer conditions, so it is necessary to make a comprehensive assessment of the conditions and development of the program to achieve a convincing strategy for management. "Our approach relies on establishing key targets for intervention based on literature, clinical studies and expert opinion. We have to analyze what we can really impact," Dr. Charlton says.

"These rare diseases may need as many as 10 interventions there are no silver bullets or readily available guidelines in the public domain," Schelhammer adds.

The complexity, severity and lower prevalence of these conditions create more demanding patients who struggle to get good care and make it a point to understand their disease. "This has led to a higher standard of program excellence required by the patient," Dr. Charlton says.

Accordant's approach is patient-centric, focusing on improving patients' understanding of the disease and teaching self-management skills and medication compliance. "We are trying to reduce the severity of events once they occur through case management, and prevent adverse events through education and proactive disease management," Dr. Charlton says.

The company combines telephonic disease assessments and 24/7 nurse support with information technology. Online features provide sponsored members with a health evaluation tool, linking them with relevant and personalized information on treatments, management of their condition and strategies for preventing complications a health journal for recording important information and tracking key health measurements a record of prescriptions and costs communication with a specially trained nurse opportunities to participate in forums and chat rooms and message bulletin boards.

Blue Cross and Blue Shield of Minnesota, headquartered in St. Paul, turned the management of 14 low-prevalence, high-cost disease states over to Accordant a year ago, pushing the total number of chronic conditions the plan manages to 33. "It is possible for a health plan to develop its own disease management programs for the more common diseases, but it is not cost-effective to do it alone for the low volume, expensive conditions," says Dr. Steve Eisenberg, medical director of the plan. That is why Blue Cross and Blue Shield of Minnesota relies on Accordant.

Dr. Eisenberg says that managing these less common conditions is not much different from programs that address diabetes or heart disease. However, the interventions differ, with many of them relying more heavily on pharmaceuticals.

The first six months of data since Blue Cross and Blue Shield of Minnesota partnered with Accordant has indicated fewer hospitalizations and emergency room visits, cost savings and more knowledgeable members. Although Dr. Eisenberg says that decreasing costs is one objective, he also anticipates increasing member and provider satisfaction and improving clinical status, quality of life and management of the conditions.

In 1999, Philadelphia-based Keystone Mercy, the largest Medicaid managed health plan in Pennsylvania, initiated a disease management program for its 60 members with hemophilia, who only make a small dent in the plan's total population of 260,000. Prior to the program, the disease was costing the plan $10 million a year.

The program focuses on helping members better understand the disease, developing protocols to meet patient needs and obtaining the best unit cost for medications. To meet the second objective, Keystone contracts with specialty drug distributors to obtain discount prices.

Keystone coordinates efforts between members, their primary care physicians (PCPs) and community specialty treatment clinics to identify those with hemophilia, develop a care plan and educate families (most of Keystone's members with hemophilia are children), teachers and school nurses.

"We want to teach members early about the disease and what concessions they may have to make so they will be better prepared when they become adults," says Robin Washington Griffin, senior case manager at Keystone. Children are urged to wear emergency bracelets and, when engaging in sports, knee pads and helmets, as well as receive permission to participate.

Hematology specialists at affiliated treatment centers monitor lab values (amount of protein in the blood) to determine how much medication is needed, which is also based on weight and age.

Severe hemophiliacs receive prophylactic, intravenous medication two to three times a week, while others may only need to be transfused when they have an injury or a procedure like a tooth removal.

Home care providers teach patients and family members how to administer the drugs. Griffin receives monthly progress reports from the centers, which she shares with patients' PCPs, and makes herself readily available by phone. The program is patterned after one for sickle cell anemia started a year earlier.

Agreeing with Schelhammer, Griffin says it is worthwhile managing a disease like hemophilia because the small population lends itself to more control, and outcomes are more immediate than for other more common chronic diseases.

The patients' care is capitated and costs an average of $100,000 per patient per year for medications alone.

Keystone's two objectives have resulted in savings of $2 million in 2001, even though the population of hemophiliacs increased, with a 40% decrease in hospitalizations.

In addition, emergency room visits dropped from 80 per month in 2000 to 20 the following year.

INTEGRATING EFFORTS
AHF Inc. in Enfield, Conn., is a disease management company dedicated solely to hemophilia and related Needing disorders. It coordinates and integrates efforts among providers, insurers and patients, providing critical data to help identify hemophiliacs and treat them educating health plan case managers, patients and their families and serving as a specialized pharmacy offering the critical clotting factor used by patients, along with equipment and waste disposal.

With only about 17,000 hemophiliacs in the United States, many providers are not intimately familiar with the disease, making it incumbent upon AHF to educate case managers so they can become patient advocates. AHF teaches them a better understanding of patients' and their families' needs, appropriate amounts of medication and the necessity of gaining quick approvals for treatment.

David Roy, the company's national business manager, says that many patients are put on a prophylactic medication schedule, recommended by the National Hemophilia Foundation, to help eliminate emergency room visits and stave off severe bleeding if an accident or injury occurs. The cost of that regimen is $150,000 to $175,000 per year per patient.

GOING ONLINE
Protocol-Driven Healthcare (PDH) in Bernardsville, N.J., has extended its line of interactive Web sites catering to consumers with common chronic diseases, such a diabetes, asthma and congestive heart failure, to include rarer conditions like cystic fibrosis and Parkinson's.

Mary-Anne Becker, a nurse practitioner and director of program development, is quick to say that PDH does not diagnose or treat diseases, but does provide education and information, which are limited for these less common diseases.

"There are no set protocols or consensus reports for managing these diseases, so we provide a valuable and valid tool for patients to receive education and insight, gaining control over their chronic diseases and becoming active participants in disease management," she says.

"We do not make recommendations unless they come from a credible clinical source," Becker says. All of the company's 13 Web sites--MyParksinsons.com and Mycysticfibrosis.com, for example--function similarly. They encourage patients to fill out personalized daily journals tracking stiffness, medications in relationship to symptoms and quality of life (in the case of Parkinson's), and voluntarily share the information with their physicians.

Online graphs and charts provide personalized feedback to patients on their condition and progress, while online forums enable them to communicate with healthcare professionals and others with the same disease.

Finally, patients have access to a reference library with educational material, clinical research and peer-reviewed articles.

Even without a silver bullet for treating many of the less common chronic diseases, some health plans and disease management companies are making a dent by improving care and cutting costs.