The Big Fat Reference Guide is a comprehensive guide to all things Alpha-1 including genetic information, treatment options, and lifestyle recommendations. Read on to learn about the origin of this document and find out what changes have been made since its last major update.
What is the Big Fat Reference Guide?
AlphaNet created the first version of the Big Fat Reference Guide (BFRG) in 2004 to provide individuals with alpha-1 antitrypsin deficiency (Alpha-1), their family members, caregivers, and healthcare providers with comprehensive Alpha-1 health management information.
The BFRG was compiled by expert Alpha-1 medical doctors, nurses, researchers, caregivers, and individuals with Alpha-1, and contains all the information needed to gain a basic understanding of this genetic condition including diagnosis, treatment, and risk factors. It also provides lifestyle recommendations for all Alphas – whether or not they have any symptoms associated with their condition. In addition, detailed information is provided concerning illnesses that Alphas may develop.
Why Is It Called the Big Fat Reference Guide?
Before the BFRG was an easy-to-access online publication, it was a massive binder containing documents a newly-diagnosed Alpha and their healthcare team needed to know. Its physical size at the time led to the name – Big Fat Reference Guide. In 2007, AlphaNet developed shorter, topic-focused guides for the most popular subjects related to Alpha-1. Known as the Skinny Little Reference Guides (SLRGs), these resources have been regularly redesigned and updated since their original release to ensure they remain relevant and accurate. They are available within the Living With Alpha-1 category of the BFRG website (bfrg.alphanet.org), which can be found in the top navigation menu of bfrg.alphanet.org.
In the Subscriber Portal BFRG, it is located in the right sidebar.
How Often is the Big Fat Reference Guide Updated?
The Big Fat Reference Guide is a living document and is updated frequently to ensure all the information it contains is as accurate and up-to-date as possible. AlphaNet completed a total overhaul of BFRG content in 2025. Read on below to learn how to access this new version and find out what’s been updated.
How Do I Access the BFRG?
To register for access to the BFRG, please visit bfrg.alphanet.org. If you are enrolled in AlphaNet’s Subscriber Portal you can access the BFRG at subscriber.alphanet.org.
What New Updates Have Been Made to the BFRG?
In 2024, AlphaNet set out to completely update each part of the BFRG. We started by assessing all content in the BFRG, making sure to note repetitive information, necessary medical updates, and opportunities for new chapters. We now have new Quick View Topics, numbered chapters, more information for Alpha-1 children, expanded liver chapters, and updated wellness information. Scroll on to find out where you can find these new updates and more.
Numbered Chapters and Streamlined Content
To make searching for topics a little easier, we’ve organized all updated content into 30 distinct chapters ranging from Alpha-1 genetic information, to lifestyle recommendations, to guidance for Alphas affected by lung and/or liver disease. These chapters are divided into five new Quick View Topics.
New Quick View Topics
- All About Alpha-1: Contains Chapter 1: Alpha 101 which provides important information about Alpha-1 diagnosis and genetics.
- Alpha-1 Lung Disease: Contains Chapters 2-12 and includes information about diagnosis, treatment, risk factors, and monitoring of Alpha-1-related lung disease.
- Alpha-1 Liver Disease: Contains Chapters 13-20 and includes information about diagnosis, treatment, risk factors, and monitoring of Alpha-1-related liver disease.
- Alpha-1 Pediatrics: Contains Chapter 21: Alpha-1 in Children: Lung and Chapter 22: Alpha-1 in Children: Liver which cover risk factors, treatment information, and advice for parents of children with one or more genetic variants of Alpha-1.
- Healthy Living for Alphas: Contains Chapters 23-30 and provides wellness and lifestyle information for all Alphas including how to navigate healthcare provider visits, insurance, relationships, end of life concerns, diet and exercise recommendations from our Medical Team, and more.
Previous Quick View Topics:
New Quick View Topics:
One-Pagers Library Access
AlphaNet One-Pagers are just that — one-page educational documents created by the AlphaNet Medical Team for use by Alphas and their healthcare providers. Each One-Pager focuses on a specific topic important to the Alpha-1 community. Current topics include: sleep apnea, metabolic health, checklists for Alpha-1-related lung and liver disease, bronchiectasis, seasonal respiratory vaccines, and many more. As of June 2025, there are 23 One-Pagers available, but new One-Pagers are created on a regular basis. All AlphaNet One-Pagers can now be accessed directly from the right hand sidebar of the BFRG under the “One-Pagers” title.
New Lung Disease Content
Find updated and expanded lung disease content in new chapters dedicated to exacerbations and medication:
- Chapter 4: Understanding Pulmonary Exacerbations explains how to self-monitor for pulmonary flare-ups, what causes them, and how to avoid them. This chapter also includes updated information regarding pneumonia and how it may affect Alphas with lung disease.
- Chapter 5: Medications for Lung Disease dives into the specific medications that may be prescribed to individuals with respiratory conditions ranging from inhalers and mucolytics to antibiotics and alternative medicines.
New Section for Vaccine Information
In Chapter 10: Staying Healthy with Lung Disease, we have created a new section “Vaccines for Alphas” that contains the latest immunization guidance for Alphas from our AlphaNet Medical Team.
New Health Management Plans
New Alpha-1 health management checklists are available for download in Chapter 12: Health Management Plan for Lung Disease and Chapter 20: Health Management Plan for Liver Disease.
New Liver Content:
New Alpha-1-related liver disease information can be found in Chapters 16-18:
- Chapter 16: Staying Healthy with Liver Disease will help you recognize the signs and symptoms of conditions associated with liver disease including portal hypertension, ascites, and hepatic encephalopathy.
- Chapter 17: Medications for Liver Disease contains updated information about treatment options for liver disease as well as information regarding emerging treatments specifically for those with Alpha-1.
- Chapter 18: Liver Exacerbations explains what liver exacerbations are and how to help avoid them.
Liver chapters can be accessed from the Alpha-1 Liver Disease Quick View Topic category:
Information Exclusively for Alpha-1 Children and Parents
Chapters 21 and 22 contain information exclusively for Alpha-1-affected children and their parents. Chapter 21: Alpha-1 in Children: Lung focuses on how to monitor Alpha-1 children for symptoms of respiratory issues and includes advice for parents regarding the avoidance of lung disease risk factors like smoking and allergens. Chapter 22: Alpha-1 in Children: Liver focuses on the diagnosis of liver disease in Alpha-1 children as well as treatment options, risk factors, and pediatric transplant information. These chapters can be found under the Alpha-1 Pediatrics Quick View Topics.
New Sleep Apnea Information
Find a section dedicated to sleep apnea including the latest diagnostic and treatment information, in Chapter 28: Sleep Disturbances under the Quick View topic Healthy Living for Alphas.
Check Back Often!
We hope our changes to the Big Fat Reference Guide make it easier for all users to find the information they’re looking for. As new Alpha-1 studies are published and potential new treatments become available, AlphaNet will continue to update the BFRG, so make sure to check back often to find the latest Alpha-1 information available anywhere.