Issue 32 | July 2015

PROACTIVE TESTING FOR A HEALTHY FUTURE

ACCEPTING ALPHA-1 AND MOVING FORWARD

MEET CORAM'S ALPHA-1 ADVOCACY TEAM

A P U B L I C A T I O N O F :

TABLE OF CONTENTS

BETTY ROOT: ACCEPTING ALPHA-1 AND MOVING FORWARDReceiving the Alpha-1 diagnosis hasn’t kept Betty from living her life to the fullest, which includes daily walks through the park and visits from her eight grandchildren.

PROACTIVE TESTING FOR A HEALTHY FUTUREThanks to support from a special program, Dr. Benjamin Laracuente is able to increase his testing of Alpha-1. He now offers free screenings in Western Pennsylvania, an industrial area with a large population of at-risk workers and retirees.

MEET LORI WISECoram’s newest Patient Relations Specialist (PRS) has special insight into helping Alpha-1 patients — both she and her daughter have had experience with home infusion therapy.

GET TO KNOW CORAM’S ALPHA-1 ADVOCACY TEAMIt takes a special person with a unique background and skill set to be part of our team — read about each member’s path to arriving at Coram.

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JULY 2015 | ISSUE NO. 32

The Alpha-1 Advocate, brought to you by Coram CVS/specialty infusion services, is a publication that is provided free of charge to the Alpha-1 community. Opinions expressed by contributing authors and sources are not necessarily those of the publisher. Information contained in this magazine is for educational purposes only and is not intended as a substitute for medical or billing advice. Do not use this information to diagnose or treat a health problem or disease without consulting a qualified physician. Consult a physician or appropriate clinical professional before starting or modifying any course of supplementation or treatment. Never disregard medical advice or delay in seeking it because of something you have read in this magazine.

© 2015 Coram LLC. All rights reserved. No part of this publication may be distributed, reprinted or photocopied without prior written permission of copyright owner. All service marks, trademarks and trade names presented or referred to in this magazine are the property of their respective owners.

We welcome your comments, stories, and suggestions. All correspondence, changes of address, and subscription inquiries should be sent to:

Alpha-1 AdvocateCoram CVS/specialty infusion services555 17th Street, Suite 1500Denver, CO 80202

communications@coramhc.comalpha1source.com

Clinical Editor Lou Anne Epperson

Managing Editor Allison Pizzuti

Contributing Writer Valerie Hansen

Graphic DesignerCeleste Johnson

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© 2015 Coram LLC | COR01011-0715

Upcoming Events

Alpha-1 Foundation 24th Annual Education ConferenceJuly 24–26: Garden Grove, CA

The conference brings together Alphas from all over the country and experts in the field of Alpha-1. Informative sessions include content focused on genetics, public policy, pediatric liver disease, transplant and oxygen. There is also a breakfast sponsored by Baxter, a luncheon by Grifols, hospitality suites and a dinner with awards. The Alpha-1 Kids program will sponsor activities throughout the weekend for children and teens.

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For information, visit alpha1.org or call 877.228.7321.

Coram Consumer Connect Call SeriesAugust 27: Traveling with Alpha-1, 7:00 p.m. ET; 4:00 p.m. PTOctober 1: Everything to Know About Insurance, 7:00 p.m. ET; 4:00 p.m. PT

During these live conference calls, clinical and advocacy experts lead discussions on topics most important to Alphas. Feel free to listen, learn, share and ask questions about topics that concern you. _____

Call toll free: 800.242.8077, access code 7681241, or visit alpha1source.com/events.

Alpha-1 Foundation Education DaysSeptember 19: Cleveland, OHNovember 7: Orlando, FL

The Education Day Series provides people living with Alpha-1 and their caregivers the latest updates on topics such as Alpha-1 liver and lung disease, how to travel with oxygen, safe physical activities and the latest research programs. _____

For information, visit alpha1.org or call 877.228.7321.

Betty Root had been healthy and active her whole life. She worked in a school cafeteria for 15 years and then in retail for another 15. At the same time she was raising four daughters. “My husband was a truck driver and gone a week or two at a time,” Betty says, “so I was basically a single mom.”

In 2004, Betty started having breathing problems. Betty thought her smoking was the reason, and the doctor pretty much agreed. She was diagnosed with chronic obstructive pulmonary disease (COPD). His advice was to quit smoking.

“Then three years later, I was out doing yard work and just got to the point where I couldn’t breathe,” she says. The doctor sent her for chest x-rays, and showed two nodules (small tissue

masses) on her right lung. She went back the same day for a computerized axial tomography (CAT scan). They still didn’t know if she had lung cancer or pulmonary disease. Her pulmonologist tested her for Alpha-1.

“I knew about this condition from my mom,” 72-year-old Betty says. “I have cousins who have it. I didn’t know it was hereditary.” In fact, Betty learned that quite a few people in her extended family have Alpha-1.

Action PlanBetty was upset at first when she found out her diagnosis of Alpha-1. “I was pretty depressed for awhile. That’s when I would talk to Aaron [Holderby, Coram’s Patient Advocate

Betty Root: Accepting Alpha-1 and Moving ForwardBy Valerie Hansen | Contributing Writer

4 B E T T Y R O O T: A C C E P T I N G A L P H A - 1 A N D M O V I N G F O RWA R D

Supervisor]. He has always been an inspiration to me. I would call him when I had a problem, and he always helped me out, whatever it was.”

Betty went to respiratory therapy, which helped with her breathing and her emotional adjustment. She didn’t feel as isolated being with other people who had breathing issues. For the last three years, Betty has been on weekly augmentation therapy.

Knowing that Alpha-1 is a hereditary condition made Betty work to not let it blindside any more members of her family. “I went on a crusade and got my family tested,” she says.

Betty knows that it’s important for people with Alpha-1 to stay active. “I probably don’t do as much as I should, but I still go to parks and walk. I

just have to take my oxygen with me. Really, other than my lungs, I’m in pretty good health.”

A Full LifeWhile she doesn’t do yard work anymore, Betty has lots of quality time with her family—four daughters, eight grandchildren and four great grandchildren. “The two great grandchildren who live near here call me ‘Rootster’,” she says with a laugh.

The two differences in her routine are the weekly therapy visits from her Coram nurse and no more yard work. Otherwise, Betty is living her life to the fullest. Alpha-1 gave her a jolt at first, but it has not kept her down. “Rootster” says she plans on being around long enough to watch her great grandchildren grow up.

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Dr. Benjamin Laracuente, a pulmonologist at the Tri-State Pulmonary Medical Practice in Western Pennsylvania, has always been proactive with testing patients he suspects may have Alpha-1. He recently increased his testing because of a free blood test provided by a State of Florida Alpha-1 Detection Program sponsored by the Alpha-1 Foundation. Prior to the free tests being made available, testing for Alpha-1 could cost patients $200 – 300.

Western Pennsylvania is an industrial area with a large population of smokers, people with COPD and workplace exposure to toxins that can cause lung problems. Many of the families are also descendents of immigrants from Northern Europe, a population that has a higher than average incidence of Alpha-1, according to Dr. Laracuente.

Dr. Laracuente tests patients who meet any one of the following criteria:■ Those with emphysema or COPD regardless of

smoking history■ Patients with asthma whose airflow obstruction is

irreversible after bronchodilator therapy■ Those with persistent obstruction and poor

pulmonary function who may have risk factors such as smoking or occupational exposure

■ Siblings of patients with Alpha-1

In addition to his patients, Dr. Laracuente has opened his office to other at-risk people in the community for the opportunity to get the tests

during regional health fairs. “At the mini health fairs people can just walk in and get the test without any commitment to me to be their physician,” Dr. Laracuente says. “We encourage them, however, to follow up with their physician and get a pulmonary physician right away. Our goal is to have alphas live a full and productive life, and we want them to have that kind of life without any issues in the long run.”

Success StoriesIn the first few months of testing both his patients and people from the health fairs, Dr. Laracuente found that 40% of the hundred or so people tested had some form of genotype abnormality.*

“We started finding a lot of MS and MZ* patients in clusters of families,” he says. “We offered this to our community and I think it has opened doors that will be beneficial in the long term to each family we have evaluated.”

Recently one of his patients, an elderly woman he has been treating for some time, brought her 24-year-old grandson with her. The young man had been diagnosed with asthma and had a bad case of pneumonia when he was ten. His grandmother encouraged him to be tested, and the result was a diagnosis of Alpha-1. It turned out that his genotype is MZ.

Proactive Testing for a Healthy FutureBy Valerie Hansen | Contributing Writer

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“I get goose bumps sometimes when discoveries like this happen,” Dr. Laracuente says. “This is a kid who just walked through the door, and he has been walking around with a diagnosis of asthma for years.”

Armed with his diagnosis and the ability to affect his own future, the young man will likely never have to experience the degenerative effects of Alpha-1.

Improving Lives “I pride myself on taking patient care to the highest level,” Dr. Laracuente says. “We want to make sure people are not walking around misdiagnosed or with a condition that a simple test can lead to treatment or types of intervention.”

Once a patient with Alpha-1 has been identified, Dr. Laracuente follows up with additional testing and treatment.

“After the preliminary test, we put the patient through a confirmatory test and arrange for a pulmonary function study. We do other blood tests to confirm their level, their phenotype and genotype. Allergy testing follows to check for immunodeficiency. From here, we provide the treatment that depends on their genotype and level.”

Dr. Laracuente also educates his patients about what having the deficiency implies long-term and short-term. He lets them know educational materials and support is available through the Alpha-1 Foundation and Better Breathers Clubs. “We also add the component of genetic counseling. If the families want it, we tell them about the nursing staff that is available. In severe deficiency, we start them on augmentation therapy.”

Being a pulmonologist familiar with Alpha-1, Dr. Laracuente knows that early diagnosis is the key to a healthy future. In fact, he urges anyone who has a condition involving their breathing to ask their doctor or pulmonologist for the blood test. “With the right diagnosis, treatment and lifestyle adjustments, the lifespan of a patient with Alpha-1 can be increased.”

*GlossaryGenotype Abnormality: In Alpha-1, a genotype abnormality means that the Serpina1 gene is producing a deficient amount of alpha-1 antitrypsin (AAT) protein.

MS and MZ: Two versions of the Serpina1 gene abnormality that indicate insufficient production of AAT protein.

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“We step in once a patient has worked with an advocate,” Lori explains. “My job, along with the other two team members, is to check in regularly with the patient about how things are going.” She is there to listen to how a patient is feeling, discuss nursing or other services, or solve billing or other financial issues. “Each of us on the team has a slightly different background, which works well,” she says. “If I don’t have the answer for one of my patient’s questions, one of my colleagues usually does. It flows very smoothly.”

Lori can relate especially well to her patients because both she and her daughter received home infusions from Coram for a year and a half. They have a rare genetic lipid storage disease.* Treatment is similar to Alpha-1 in that Lori needed replacement therapy, but with an enzyme instead of a protein.

One example of Lori’s dedication to patients is helping a patient who was getting frustrated with a billing issue. “It wasn’t a large amount of money, but he was having difficulty getting an answer to

As Coram’s newest Patient Relations Specialist (PRS), Lori helps Alpha-1 patients get more comfortable with their home infusion treatments.

P A T I E N T R E L A T I O N S S P E C I A L I S T

LORI WISEBy Valerie Hansen | Contributing Writer

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his question.” Lori contacted the Alpha-1 billing department, and was able to get it resolved quickly. “I was also happy that the Coram insurance people told the patient how to avoid delays in getting an answer in the future.”

Patients FirstLori is a medical laboratory technician with 35 years of experience, including two decades working in hospitals. She is also a volunteer advocate for the Colorado Newborn Screening Advisory Committee, and the Mountain States Genetics Regional Collaborative.

Before coming to Coram, Lori held an IT position at Quest Diagnostics, one of the country’s major laboratory testing facilities. “When I worked in the IT department, I missed helping patients,” Lori says. “In the hospital, I worked with patients so closely — it was awesome. I knew I wanted to go back to working with people.”

That’s when Lori decided to come to Coram. “I was impressed by the service my daughter and I got from Coram. Now I am equally impressed by how much the people at Coram care about our patients,” she says.

Lori feels the most rewarding part of her job is making a difference in someone’s life. “I think that everyone at one time or another on their medical road needs an advocate,” Lori says. “It really does take a village to get everything to go smoothly. There is a lot of satisfaction in being part of that village.”

I think that everyone at one time or another on their medical road needs an advocate.”

– Lori Wise

*GlossaryLipid Storage Disorders: These disorders cause harmful amounts of lipids—or fats—to build up in cells and tissues. People with the disorders don’t produce enough of one of the enzymes necessary to metabolize the lipids, or they produce enzymes that don’t work properly.

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Aaron Holderby Supervisor, Coram Advocacy Programs

Aaron joined Coram’s Alpha-1 Advocacy team in January 2006. He was in-troduced to Alpha-1 in 2003 when his wife was diagnosed. As she began augmentation therapy, Aaron learned what it means to be caregiver to an Alpha-1 patient.

Aaron has always had a passion for helping people, and his wife’s diagnosis brought that passion to the care of other Alpha-1 patients. Aaron supports Coram’s Alpha-1 patients using both his experience as a caregiver to his wife, and his background in case management. He considers it a privilege to serve the Alpha-1 community.

Coram’s Alpha-1 Advocacy Program helps patients manage

the challenges of living with Alpha-1 Antitrypsin Deficiency. Our

program pays special attention to the clinical and social needs of

Alpha-1 patients and their families. Each of our team members has

unique experiences and backgrounds that make them qualified to

support Alpha-1 patients.

Get to Know Coram’sAlpha-1 Advocacy Team

I wanted so desperately to help my wife, and I recognize that there were others who may be in a similar situation. I want to let them know that they are not alone.”

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With the experience I gained working with chronic diseases, I saw first hand the challenges patients and their families encounter. My goal is to provide direction and encouragement.”

When I was diagnosed 15 years ago, there was a limited amount of resources available to help me understand what Alpha-1 was. Now I am able to help others in that situation.”

John Taylor Alpha-1 Advocate, Admissions

Diagnosed with Alpha-1 in 2000, John joined Coram’s Advocacy Team in 2012. He has told the story of his Alpha-1 journey as a speaker for the Al-pha-1 Association and Donate Life Arizona. He has also shared his story through appearances on radio and television.

As an Alpha-1 Advocate, John provides a range of resources for Coram pa-tients. He offers first-hand experience based on his many years managing Alpha-1, including his double lung transplant. John is pleased to be the first Coram contact for newly diagnosed Alpha-1 patients. In this role, he talks with patients about their diagnosis, therapy, insurance, and ongoing care.

Angelia Beards Alpha-1 Advocate, Risk Management

Angelia joined Coram’s Advocacy team in September 2013. She has a back-ground in patient advocacy and a passion for helping others. Angelia is an asset for our patients in managing their Alpha-1 diagnosis, providing guid-ance on financial issues and insurance coverage.

Angelia previously worked as a patient financial counselor and advocate at a cancer center. In this work, she saw the challenges patients with chronic conditions face on a daily basis. She draws from this experience to provide encouragement and resources to our Alpha-1 patients and their families.

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