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The Direct Medical Costs of ₁-Antitrypsin Deficiency^*

C. Daniel Mullins, PhD; Xingyue Huang, MS; Sanjay Merchant, MS, MBA; James K. Stoller, MD, FCCP and for the Alpha One Foundation Research Network Registry Investigators

^* From the University of Maryland School of Pharmacy (Dr. Mullins, Mr. Huang, and Mr. Merchant), Baltimore, MD; and Department of Pulmonary and Critical Care Medicine (Dr. Stoller), The Cleveland Clinic Foundation, Cleveland, OH. A complete list of investigators is presented in the ^Appendix.

Correspondence to: C. Daniel Mullins, PhD, University of Maryland School of Pharmacy, 6th Floor, 100 N. Greene St, Baltimore, MD 21201; e-mail: dmullins{at}rx.umaryland.edu

	Abstract

TOP Abstract Introduction Materials and Methods Results Discussion Appendix 1 References

 
Background: For individuals with emphysema because of severe ₁-antitrypsin deficiency, specific therapy called IV augmentation therapy has been available since 1989. Such therapy consists of IV infusion of pooled human plasma ₁-antiprotease.

Methods: To assess the direct medical costs of having ₁-antitrypsin deficiency, the current study surveyed members of the Alpha One Foundation Registry for Individuals With ₁-Antitrypsin Deficiency regarding their annual expenditures for treatment of this disease. Data regarding demographic features, ₁-antitrypsin status, and health-resource utilization were collected from a self-administered questionnaire. Respondents were asked to provide total health-care expenditures, but costs by specific items of care (eg, drugs, physician visits, etc) were not available.

Results: Mean annual cost estimates were higher for PI*ZZ-phenotype individuals ($30,948, n = 292) than for non-PI*ZZ–phenotype individuals ($20,673, n = 53; p = 0.049). Among PI*ZZ-phenotype individuals, self-reported costs of health-care services were further analyzed for those 288 individuals whose ₁-antiprotease use status was reported. For the 185 current ₁-antiprotease users, the mean annual cost was $40,123 (median, $36,000).

Conclusions: Annual health-care expenditures by individuals with ₁-antitrypsin deficiency are very high, whether or not they are currently receiving augmentation therapy. Augmentation therapy adds substantial costs, especially for heavier individuals who are receiving weekly infusions.

Key Words: ₁-antitrypsin deficiency • cost • registry

	Introduction

TOP Abstract Introduction Materials and Methods Results Discussion Appendix 1 References

 
₁ -Antitrypsin deficiency is an autosomal codominant condition that predisposes to early-onset emphysema, chronic liver disease (ie, hepatitis, cirrhosis, hepatoma), panniculitis, and vasculitis.^{1 2 3} Although population-based studies show a frequency of approximately 1 in 3,500 live births in the United States and predict a prevalence of 100,000 severely deficient Americans,^{4 5 6 7 8} < 10% of affected individuals have been detected to date.⁴ Putative reasons for this underdetection include underrecognition of ₁-antitrypsin deficiency by health-care providers⁹ and freedom from clinical consequences despite severe deficiency in some individuals.

For individuals with emphysema because of severe ₁-antitrypsin deficiency, specific therapy called IV augmentation therapy has been available since 1989. Such therapy consists of IV infusion of pooled human plasma ₁-antiprotease. To date, only a single preparation of pooled human plasma ₁-antiprotease has received US Food and Drug Administration approval (Prolastin; Bayer; West Haven, CT), although other preparations as well as other treatment strategies are currently under study. Limitations of the current treatment include the substantial expense of the drug and the associated infusions, as well as current inability to produce enough pooled human ₁-antiprotease to treat all appropriate candidates.

Because ₁-antitrypsin–deficient individuals may experience several chronic debilitating illnesses and may receive life-long, costly therapy, the clinical and economic burden of illness can be high. In a modeled cost-benefit analysis of IV augmentation therapy, Hay and Robin¹⁰ estimated that at 70% efficacy, the cost per life-year saved with augmentation therapy was $28,000 to $72,000 (1990 US dollars), and that at 30% efficacy, the cost ranged from $50,000 to $128,000 per life-year saved based on an assumed yearly treatment cost of $30,000. A more recent study by Alkins and O’Malley¹¹ showed a lower incremental cost-effectiveness ratio of $13,971 for severe ₁-antitrypsin–deficient individuals. The estimate by Alkins and O’Malley¹¹ is more current, uses a discount rate of 7%, and focuses on survival data from the National Institutes of Health Registry. Although estimates by both Hay and Robin¹⁰ and Alkins and O’Malley¹¹ are useful in estimating the cost-effectiveness relationship of augmentation therapy, they may fall short in estimating the economic burden of ₁-antitrypsin deficiency because they are based on models, they fail to consider patient-reported information, and they do not capture information regarding the full spectrum of associated conditions related to ₁-antitrypsin deficiency.

To assess the direct medical costs of having ₁-antitrypsin deficiency, the current study surveyed members of the Alpha One Foundation Registry for Individuals with ₁-Antitrypsin Deficiency to assess the annual expenditures by affected individuals for treating this disease.

	Materials and Methods

TOP Abstract Introduction Materials and Methods Results Discussion Appendix 1 References

 
The cost of treating ₁-antitrypsin deficiency includes the cost of treating attributable illnesses and the cost of augmentation therapy. The cost of ₁-antiprotease treatment includes the cost of the drug and the cost of the infusion. Furthermore, because the dose of ₁-antiprotease is weight based, treatment costs were calculated for three patient types: females (50 kg), average-weight males (75 kg), and heavier males (100 kg). Drug cost and administration estimates were based on 1999 Medicare reimbursement rates for the relevant current procedural terminology (CPT) codes and estimated costs for Prolastin, assuming a standard reimbursement rate of 95% of the average wholesale price. The CPT code used for these calculations was 90780, representing the first hour of ₁-antiprotease infusion (listed at $36.73 for 1999). Calculations for the monthly cost of infusion were based on the same CPT code (90780) for the first hour of infusion, and also code 90781, which represents each additional hour of infusion therapy (listed at $18.48 in 1999).

Table 1 presents the estimated reimbursement amount for ₁-antiprotease treatment per infusion with the annual costs based on whether infusion was administered weekly (at a dose of 60 mg/kg > 1 h) or monthly (at a dose of 250 mg/kg > 4 h). Reliable estimates were unavailable for several other costs, including the costs for physician outpatient visits and for emergency department visits, the cost of skilled nursing facilities, and the cost of other complications associated with the disease. In the absence of reliable estimates, the current analysis did not consider these other costs and so likely underestimates the true costs of health care associated with having ₁-antitrypsin deficiency.

Data Collection
Data regarding demographic features, ₁-antitrypsin status, and health-services utilization were collected from a questionnaire distributed to all ₁-antitrypsin–deficient registrants in the Alpha One Foundation Registry. At the time of this study, the Registry consisted of 688 individuals who agreed to complete and submit the questionnaire. These 688 respondents represent a small but incalculable percentage of the total number of questionnaire recipients, as the precise number of questionnaires distributed (approximately 12,000) was not tracked and some individuals may have received more than one form.

The research registry questionnaire (which is available from the Alpha One Foundation, Miami, FL) was designed to query utilization of health-care resources over a 12-month period. Individual respondents completed the survey at various times during the interval from 1997 to 1999 and were asked to provide estimates for the prior 12 months. Estimates included all medical visits (scheduled and unscheduled), medications (whether prescribed or over-the-counter), and all other expenditures for other health-care services (eg, emergency department visits, etc) in the aggregate. Cost estimates for individual types of utilization (eg, office visits, hospitalization) were not reported. All costs were expressed in US dollars for the year in which the survey was completed.

	Results

TOP Abstract Introduction Materials and Methods Results Discussion Appendix 1 References

 
Between 1997 and 1999, questionnaires were completed by 688 participants. As shown in Table 2 , the mean age of participants was 49.7 years, the mean age at diagnosis was 41.2 years, and 52.3% of the participants were male. Of the 80.0% (n = 548) who provided responses regarding race, 96.2% were white. Of the 81.7% (n = 562) who reported a specific phenotype, 84.5% self-reported being PI*ZZ phenotype. As demonstrated in Table 2 , the current study cohort generally resembles the largest available cohort (n = 1,129) reported from the National Heart, Lung, and Blood Institute (NHLBI) Registry of Individuals with Severe Deficiency of ₁-antitrypsin.¹² Self-reported data on physician visits over the previous 12 months were available from 629 individuals (91.4% of respondents). Only 17 individuals reported no doctor visits, while almost one half of patients (n = 308) reported between one visit and five visits. Approximately one fourth of patients (n = 174) reported 6 to 10 visits, while approximately 10% reported 11 to 15 visits (n = 68) or > 15 visits (n = 62). Patients also provided information on the use of home oxygen. About one third of patients (n = 235) reported using home oxygen in the previous 12 months.

Finally, the presence and number of associated conditions was classified according to participants’ self-reports. In the context of patients’ self-reported conditions (eg, emphysema, chronic bronchitis, asthma, chronic liver disease, etc), patients with self-reported chronic lung disease reported the highest annual health-care costs (mean, $39,470; median, $15,000; Table 5 ). The annual costs for patients with other associated conditions were as follows: chronic bronchitis (mean, $36,598; median, $10,000), emphysema (mean, $33,320; median, $11,051), asthma (mean, $31,376; median, $6,000), and chronic liver disease (mean, $8,442; median, $2,750). Individuals reporting four or more associated conditions incurred higher annual health-care costs (mean, $45,107; median, $30,000) than those with fewer (ie, one to three) such conditions (mean, $30,209; median, $6,000), or those with none (mean, $9,640; median, $700; p = 0.0007; Table 4 ). In order to compare self-reported cost estimates with projections based on Medicare reimbursement rates, the projected costs for ₁-antiprotease were calculated for the 292 PI*ZZ-phenotype individuals using the values presented in Table 1 and stratified by gender and frequency of augmentation therapy. Based on the reported distribution of weekly vs monthly ₁-antiprotease recipients and the gender distribution (assuming an equal number of 100-kg and 75-kg men), the mean annual cost of ₁-antiprotease therapy was > $50,000 in men and > $30,000 in women. Moreover, these estimates are based solely on the cost of infusion therapy and do not incorporate the costs of disease complications. Comparison of these projected ₁-antiprotease costs with health-care expenditures actually reported by current ₁-antiprotease recipients shows that subjects appear to be underreporting their total costs, either because they report only out-of-pocket expenses or because they are unaware of how expensive their treatments actually are.

	Discussion

TOP Abstract Introduction Materials and Methods Results Discussion Appendix 1 References

Because this is the first study, to our knowledge, to assess health-care costs in individuals with ₁-antitrypsin deficiency, comparisons with prior findings are not possible. On the other hand, it is noteworthy to compare the cost estimates in the current study with drug costs assumed in the models used by Hay and Robin¹⁰ and Alkins and O’Malley¹¹ in assessing the cost-effectiveness of augmentation therapy. Our estimate of patient annual health-care expenditures based on self-reported data for individuals receiving ₁-antiprotease is similar to assumed cost (Hay and Robin¹⁰ ) of $30,000 per patient per year in 1990 dollars (which is equivalent to $44,613 in 1998 dollars based on the consumer price index for medical care¹³ ). Our estimated Medicare reimbursement amount for augmentation therapy ($48,356 to $51,050 for a 75-kg person) is similar to the estimated cost (Alkins and O’Malley¹¹ ) of $52,000.

To put the cost of ₁-antitrypsin in perspective, it is important to compare the associated costs with those of related clinical conditions, eg, ₁-antitrypsin–replete COPD. In this regard, Strauss et al¹⁴ estimated the annual cost for patients with chronic obstructive lung disease in the late 1980s to be between $6,234 and $7,192 (equivalent to $12,371 to $14,272 in 1998 dollars), based on whether a patient visited a family practice physician or a pulmonary specialist. In another study, Bergner et al¹⁵ reported that the annual cost of care for patients with chronic obstructive lung disease was $7,647 in 1981–1982 dollars, which is equivalent to $20,014 in 1998 dollars.¹³

The current estimates of expenditures by patients with ₁-antitrypsin deficiency have several potential shortcomings, some of which are because of the retrospective nature of this study. First, as a voluntary survey with an unknown response rate, the study is subject to reporting bias. Indeed, of the 688 eligible registrants, phenotype was reported by only 81.7% (n = 562). Under the most extreme assumption that all unreported phenotypes had the PI*ZZ phenotype, the 475 responding PI*ZZ-phenotype individuals would represent only 79% of the eligible pool of PI*ZZ-phenotype individuals. Thus, these results could be biased by failure to include responses by up to 21% of the eligible PI*ZZ-phenotype individuals. Furthermore, because the 688 respondents in this study represent a small (but unknown) fraction of all potential registrants, the generalizability of our findings to an unselected population of PI*ZZ-phenotype individuals is uncertain. However, in the absence of available data from a large population-based cohort of individuals with ₁-antitrypsin deficiency, we believe that the similarity of the cohort in the Alpha One Foundation Registry to that described in the NHLBI registry for individuals with severe deficiency of ₁-antitrypsin (Table 2) supports the generalizability of our findings. Another type of reporting bias relates to our having cost data on only a subset (60.6%) of the 688 registrants.

A further potential source of bias is that respondents may have either imprecisely recorded or inadequately recalled their health-care expenditures. Reasons for this may be recall bias, lack of knowledge of the total cost of care, or simply difficulty recalling or enumerating out-of-pocket costs. As evidence of this potential bias, patient self-reported costs were less than the projected annual Medicare expenditure for augmentation therapy alone. To the extent that respondents may have had incomplete knowledge of health-care expenses covered by their insurance or may have forgotten the expenses, we believe that the responses in this study may generally underestimate the true expenditures. Finally, another limitation of our analysis is our lack of data regarding the types of usual care received by survey respondents (eg, regularly used bronchodilators, vaccinations against influenza, and pneumococcus, etc).

Many unanswered questions remain regarding patient expenditures and the total costs of treatment for ₁-antitrypsin deficiency. For example, because the survey instrument used in this study did not carefully itemize expenditures by type (eg, pharmaceuticals other than ₁-antiprotease inhibitor, hospitalization-associated costs, etc), we cannot currently determine the major cost drivers of ₁-antitrypsin deficiency. To address this gap in current understanding, a second-generation questionnaire developed to permit a more detailed analysis by type of expenditure.

	Appendix 1

TOP Abstract Introduction Materials and Methods Results Discussion Appendix 1 References

 
Medical and Scientific Advisory Committee and Clinical Resource Center Membership in the Alpha One Foundation Research Network Registry
Medical and Scientific Advisory Committee Mark L. Brantly, MD, Co-Director

University of Florida College of Medicine

Gainesville, FL

James K. Stoller, MD, Co-Director

Cleveland Clinic Foundation

Cleveland, OH

Alan F. Barker, MD

Oregon Health Sciences University

Portland, OR

Frederick deSerres, PhD

National Institutes of Health, NIEHS

Research Triangle Park, NC

Robert J. Fallat, MD

California Pacific Medical Center

San Francisco, CA

Caroline Riely, MD

University of Tennessee, Memphis

Memphis, TN

Robert A. Sandhaus, MD, PhD

Director, Medical Affairs

Boulder, CO

Gordon L. Snider, MD

Boston, MA

Charlie Strange, MD

Medical University of South Carolina

Charleston, SC

Jeffrey Teckman, MD

Washington University School of Medicine

St. Louis, MO

Gerard M. Turino, MD

Columbia University College of Physicians & Surgeons

New York, NY

Cathy Valenti

Alpha One Foundation Board of Directors

Meridian, ID

Debbie Waldrop, RN, BSN

University of Texas Health Center, Tyler

Tyler, TX

John W. Walsh

Alpha One Foundation

Miami, FL

Bioethics Consultant Evan DeRenzo, PhD

Center for Ethics/MedStar Health

Rockville, MD

Administration Symma Finn, MA

Alpha One Foundation

Miami, FL

Clinical Resource Centers Richard Helmers, MD

Mayo Clinic

Scottsdale, AZ

Russell R. Dodge, MD

University of Arizona

Tucson, AZ

Robert J. Fallat, MD

California Pacific Medical Center

San Francisco, CA

Carroll E. Cross, MD

University of California at Davis

Sacramento, CA

Marvin Ament, MD

University of California, Los Angeles Center for Health Sci ences

Los Angeles, CA

Donald F. Tierney, MD

University of California, Los Angeles School of Medicine

Los Angeles, CA

Jack L. Clausen, MD

University of California, San Diego Medical Center

San Diego, CA

Emmet B. Keeffe, MD

Stanford University Medical Center

Palo Alto, CA

Robert A. Sandhaus, MD, PhD

National Jewish Medical & Research Center

Denver, CO

Ronald J. Sokol, MD

The Children’s Hospital

Denver, CO

Arthur Kotch, MD

Danbury Hospital

Danbury, CT

Stephen Grinton, MD

Mayo Clinic Jacksonville

Jacksonville, FL

Mark L. Brantly, MD

University of Florida College of Medicine

Gainesville, FL

Adam Wanner, MD

University of Miami School of Medicine

Miami, FL

Joseph P. McMahon, MD

Indiana University Medical Center

Indianapolis, IN

Jeff Wilson, MD

University of Iowa, College of Medicine

Iowa City, IA

Dermot N. Killian, MD

Mercy Hospital

Portland, ME

Steven Weinberger, MD

Beth Israel Hospital

Boston, MA

Sanjiv Chopra, MD

Beth Israel Hospital

Boston, MA

Frederick Gordon, MD

Lahey Clinic Medical Center

Burlington, MA

William C. Sheehan, MD

Pulmonary Care, P.C.

Fall River, MA

Michael S. Eichenhorn, MD

Henry Ford Hospital

Detroit, MI

William F. Bria, MD

University of Michigan Medical Center

Ann Arbor, MI

K.P. Ravikrishnan, MD

William Beaumont Hospital

Royal Oak, MI

Michael Krowka, MD

Mayo Clinic Rochester

Rochester, MN

Marshall Hertz, MD

University of Minnesota Hospital and Clinic

Minneapolis, MN

Jeffrey Teckman, MD

Washington University School of Medicine

St. Louis, MO

Stephen I. Rennard, MD

University of Nebraska Medical Center

Omaha, NE

Henry C. Bodenheimer, Jr., MD

Mount Sinai Medical Center

New York, NY

Frederick J. Suchy, MD

Mount Sinai School of Medicine

New York, NY

Edward Eden, MD

St. Lukes/Roosevelt Hospital Center

New York, NY

Richard W. Hyde, MD

University of Rochester Medical Center

Rochester, NY

James F. Donohue, MD

University of North Carolina at Chapel Hill

Chapel Hill, NC

William F. Balistreri, MD

Children’s Hospital Medical Center

Cincinnati, OH

James K. Stoller, MD, FCCP

Cleveland Clinic Foundation

Cleveland, OH

Zobair Younossi, MD

Cleveland Clinic Foundation

Cleveland, OH

Kevin D. Mullen, MD

MetroHealth Medical Center

Cleveland, OH

Mark D. Wewers, MD

Ohio State University

Columbus, OH

Alan F. Barker, MD

Oregon Health Sciences University

Portland, OR

Michael K. Porayko, MD

Jefferson Medical College

Philadelphia, PA

Charlie Strange, MD

Medical University of South Carolina

Charleston, SC

Marc Judson, MD

Medical University of South Carolina

Charleston, SC

Norman T. Soskel, MD

Memphis Tennessee Clinical Center

Memphis, TN

Caroline Riely, MD

University of Tennessee, Memphis

Memphis, TN

W. John Ryan, MD, FCCP

Dallas Pulmonary Associates

Dallas, TX

James M. Stocks, MD

University of Texas Health Center, Tyler

Tyler, TX

Edward J. Campbell, MD

University of Utah Health Sciences Center

Salt Lake City, UT

Richard E. Kanner, MD

University of Utah Health Sciences Center

Salt Lake City, UT

Robert E. Sandblom, MD

Eastside Specialty Center

Redmond, WA

Ian Waters, MD

Victoria General Hospital

Victoria, British Columbia

Affiliated Research Centers Christine Cannon, RN, PhD

University of Delaware

Newark, DE

William F. Brechue, PhD

Indiana University

Bloomington, IN

	Footnotes

 
Abbreviations: CPT = current procedural terminology; NHLBI = National Heart, Lung, and Blood Institute

Funded by the Alpha One Foundation.

Received for publication March 30, 2000. Accepted for publication October 19, 2000.

	References

TOP Abstract Introduction Materials and Methods Results Discussion Appendix 1 References

 

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