Hereditary angioedema (HAE): Rare and hard to treat

Annual drug treatment costs for HAE can range up to $1 million or more

July 31, 2018

What was the study about?

HAE is a rare and potentially life-threatening genetic condition. It occurs in about 1 in 50,000 people. Treatment costs range from thousands of dollars a year to more than a million per year. Available HAE therapy includes preventive (prophylactic) and on-demand medication.

Guidelines recommend anyone with HAE have on-demand medication available for two attacks and always carry on-demand medications.1 Not every HAE patient will need or use preventive treatments.

A new HAE medication in 2017 and other HAE treatments in the pipeline generated new interest. Plan sponsors want to understand more about members with HAE, their drug utilization and spend.

What did we learn?

  • The average 12-month total cost of care was $409,925; HAE drug cost totaled $395,507 (97 percent) of that. All other medical and pharmacy costs equaled $14,418 (3 percent).
  • Cinryze® and Firazyr® were the top two drugs in HAE drug spend, together holding 87 percent of total HAE specialty drug spend.
  • 37 percent of the members in the group were new starts to drug therapy.
    There were 10 members whose total cost of care was more than $1 million.


For the study, Prime looked at pharmacy and medical claims data of 15 million commercially insured members from July 1, 2015 to June 30, 2016. 226 members had at least one claim for an HAE specialty drug in the first half of 2016.

About half, 111 (49 percent) members met continuous enrollment criteria. The average age was 43 years and the group was 75 percent female. The study analyzed and compared their use of five HAE drugs, medical and pharmacy claims, emergency room visits, inpatient hospital admissions and total cost of care.


HAE drugs are processed on both the medical and pharmacy benefit; only with integrated medical and pharmacy claims data can you understand patterns of use.

Among the 111 members with HAE that met the study criteria, there were 10 high-cost outlier members. Their costs were more than $1 million/year each. This small group accounted for 30 percent of the $43.9 million overall HAE drug spend in Prime’s commercial book of business.

If you remove those members whose costs were more than $1 million a year from the list of 111, the average total cost of care for HAE becomes $302,496.

Of the members whose costs were more than $1 million a year, none of them were new starts.

What does this mean for you?

Members with HAE vary widely in how they use the drugs that manage their condition. Drug costs drive 97 percent of HAE treatment expenses. There is no opportunity for medical cost offset; any savings must come from appropriate use of HAE drugs.

The preventive drug Haegarda® (C1 esterase inhibitor) was approved in June 2017. More drugs for HAE are in the pipeline. Plan sponsors will need to reevaluate their utilization management and medical drug programs.

Prime recommends diligent pharmacist case management following a patient’s first use of HAE drugs. This can help ensure appropriate use and realize drug cost savings, whether the drug is billed under the pharmacy or the medical benefit.

Prime uses integrated medical and pharmacy claims data to inform its clinical programs. Real-world data provides insights to improve health outcomes and help control costs.

Drug names are the property of their respective owners.


1. Preventive and Followup care for Hereditary Angioedema Attacks. Medically reviewed by Graham Rogers, MD on May 17, 2016. Written by Sarah Keller. © 2018 HealthLine Media. accessed at

Hereditary Angioedema: A Comprehensive Integrated Medical and Pharmacy Claims Analysis of Utilization and Costs Among 15 Million Commercially Insured Members (Spring 2018)

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