Prominent Alpha-1 researcher Maurizio Luisetti, MD, of Pavia, Italy, passed away on Monday, Oct. 20, after a brief illness.
He was 61 years old. “We have lost a dear friend and pioneer in Alpha-1 research,” said Alpha-1 Foundation President John Walsh. “Maurizio was a true gentleman and the ultimate diplomat who was a constant source of wisdom and encouragement as a member of our scientific leadership.”

An advisor to the Foundation, Luisetti was considered the “father” of the Italian Alpha-1 patient and research community, said Foundation Scientific Director Adam Wanner.

Luisetti was medical director at the Clinic of Respiratory Diseases, IRCCS Policlinico San Matteo for 20 years, and head of the clinic’s Laboratory of Biochemistry and Genetics for nearly 30 years in Pavia, where he coordinated an Alpha-1 research registry, testing program and research network. He was an adjunct professor at the University of Pavia’s School of Respiratory Diseases.

In October 2006, he became a professor at University of Turin and in 2010 was appointed professor of respiratory diseases at University of Pavia. In 2010, Luisetti was appointed director of the clinic’s Pneumology Foundation.

In addition to Luisetti’s research and more than 190 publications, his many achievements included an appointment as international governor of the American College of Chest Physicians in 1996, and being a founding member of the Alpha-1 International Registry. He was head of the Italian Registry for Severe Deficiency of Alpha-1 Antitrypsin’s Coordination Center.

Luisetti served on the editorial boards of American Journal of Respiratory and Critical Care Medicine, Sarcoidosis, International Journal of COPD, Pulmonary Medicine, Monaldi Archives of Chest Disease, and Medicina Thoracica.

Luisetti, who organized many scientific conferences, was to be chair of the second Biennial International Research Conference on Alpha-1, to take place concurrent with the 2015 Alpha-1 International Patient Congress, April 9-11 in Barga, Italy.

He was a pervasive presence at the 2013 International Patient Congress in Barcelona, speaking on topics including “Contributions to the understanding of the natural history and genetic epidemiology of AATD over 50 years,” and “The status of Alpha-1 testing in Europe and the U.S. at the 2013 International Patient Congress.”

“Gordon and Maurizio were such good friends,” said Sally Everett, an Alpha, referring to her husband Gordon Snider, MD, whose important role in Alpha-1 research led the Alpha-1 Foundationto establish a research grant in his name this year. Snider passed away in 2013.

“They shared a passion for research on COPD and Alpha-1,” Everett said. “A truly sad day for his family, colleagues and all of us with Alpha-1 and COPD worldwide.”

Fellow researcher Francesco Bonella, MD, in announcing Luisetti’s passing, said, “Besides his outstanding competence and high international profile, Maurizio was a person with extraordinary humanity and commitment towards his patients and coworkers. He was always friendly and ready to help and cooperate. He strongly believed that progress in research depends on cooperation and networking.

“He will be sadly missed,” said Bonella, senior clinical researcher at Wissenschaftlicher Arzt-Prüfarzt Interstitial and Rare Lung Disease Unit. “We have to continue our work with passion and determination in his memory. Thank you, Maurizio, for all you have done and all you gave us in these years.”

Funeral arrangements are being handled by the Bonizzoni Funeral Home in Via Ciapessoni, Pavia. Luisetti’s funeral will be held at 3 p.m. Wednesday at the Church of San Lanfranco in Pavia.

Source: Alpha-1 Foundation October 20, 2014

Alpha-1 Alliance hosts seminar on Alpha-1 at the Scottish Parliament on 19^th November The Alpha-1 Alliance warmly invites Scottish Alpha-1 patients and their family members to attend our Parliamentary Seminar on Alpha-1 Antitrypsin Deficiency (Alpha-1) on Wednesday,19^th November, in the Scottish Parliament.

The Alliance is holding this event to highlight the unmet medical need experienced by the Scottish Alpha-1 community with politicians and NHS Scotland officials, and to ask for their support in improving the level of care and accessibility of adequate health services for Alpha-1 patients.

The findings of the first ever national survey of Alpha-1 patients are being summarised in a policy report on Alpha-1 that will be published prior to the Parliamentary Seminar. The report will also contain recommendations on how to improve care by establishing an NHS Scotland commissioned specialist service for Alpha-1 that delivers coordinated, multidisciplinary care, based on national standards.

The seminar will provide a platform for politicians, patients, clinicians and NHS Scotland officials to discuss the findings and recommendations of the report. The meeting will be a panel discussion, chaired by Aileen McLeod MSP, Parliamentary Health Liaison Officer to the Cabinet Secretary for Health and Wellbeing, Alex Neil MSP, and Member of the Health and Sport Select Committee. Panellists will also include the Alpha-1 Alliance’s Chair, Karen North, and Chair Scotland, Professor William MacNee. The Alliance is currently confirming further speakers for the event.

Professor William MacNee, Chair Scotland of the Alliance, said: “For the first time, the Scottish Alpha-1 community is making their voice heard in Parliament. This event marks an important milestone in the campaign for a national Alpha-1 service in Scotland, and it is imperative that as many patients as possible attend.”

The details for the meeting are:

Wednesday 19^th November, 6-8pm
The Fleming Room
The Scottish Parliament
Edinburgh EH99 1SP

It is important for the success of our campaign that the event is well attended. To support, the Scottish campaign, you should:

• Attend the event.

• Bring a family member or carer along (restricted to 1 guest per patient).

• Invite your MSP by following the instructions on the Alpha-1 Alliance website – just click on

  “3 Easy Steps to email your MP/MSP” or on the “Get Involved” tab.

• Invite your consultant physician to attend the event and to RSVP in advance to info@alpha-1-alliance.org.uk.

To attend the Parliamentary Seminar, you must register your/your guest’s name by emailing info@alpha-1-alliance.org.uk no later than 11^th November 2014. Please also let us know if you have any specific access requirements or if you require personal assistance in the event of an evacuation.

Your support counts! We look forward to hearing from you.

Source: Alpha-1 Alliance 29/09/2014

A Highland mother-of-five with just 24% lung capacity has climbed a steep rock face at Edinburgh Castle to place a huge Yes sign in full view of thousands of people.

Climber Lindsay Jarrett, 42, of Kinlochleven in Lochaber, posted pictures of herself online tightly gripping the famous edifice as she made the gesture.

Her climb was organised by a group called Independence Climber, who timed it to coincide with the busiest weekend of referendum campaigning, prior to the vote.

A spokesman for Independence Climber said: “No damage to the castle or surrounding area – night-time ascent and a successful one.”

Earlier this year Ms Jarrett also voluntary removed her name from a donor waiting list, despite needing a lifesaving lung and liver transplant.

She did it to raise awareness of the genetic condition that is killing her – alpha-1 antitrypsin deficiency, which affects her lungs and liver.

Her lung capacity now stands at 24% of what it should be and she has to be constantly attached to bottled oxygen.

The single mother decided to put her hoped-for treatment on hold and is rock climbing up and down the UK from April to September to highlight the Alpha-1 UK Support Group and to raise money for Climbers Against Cancer.

She is hoping her efforts will inspire her children, Ciara, 25, Jemma, 17, Mia, 10, Rory, 7, and six-year-old Findlay, who are all carriers of the condition. All except Jemma are now suffering the symptoms.

Her latest, and most adventurous, protest, was lauded by supporters on the Independence Climber social network page.

One comment said: “Oxygen on her back, tubes getting up her nose, she climbed 60 metres (200ft) in the dark just to say she was not frightened.

“How can Scotland not succeed with girls like this? Her Yes shone a sparkling light over Edinburgh today.”

Another cause close to her heart is the preservation of Ben Nevis and its environs, which led to her being outraged when a group of students cemented a bench to the summit.

Source: Aberdeen Press & Journal 16/09/2014 (Article)

Source: Andrew R Moore (Photo)

Government committee to consider Alliance application for Alpha-1 NHS national service

The establishment of an NHS national service for the treatment of Alpha-1 could move a step closer this month when a key government committee considers whether the proposal from the Alliance progresses to the next stage.

The committee, called the Prescribed Specialised Services Advisory Group (PSSAG), sits within NHS England and is part of a complicated process that evaluates the funding and development of new services in the NHS.

Karen North said: “This is a critical moment in the campaign. The Alliance has demonstrated that there is widespread support from MPs in Parliament for a dedicated Alpha-1 service in the NHS and now is the time for the government to listen.”

The application from the Alliance includes our survey on the unmet medical need of Alpha-1 in the NHS and recommendations from leading respiratory clinicians on the structure of a proposed service.

Committee members are being contacted in advance of the meeting by the Alliance to ensure they are fully aware of the urgent needs of the Alpha-1 community.  A dedicated Alpha-1 NHS service hasalready been endorsed by an independent committee of expert physicians who advise NHS England (the Clinical Reference Group).Source: Alpha-1 Alliance 01/09/2014

European Respiratory Society to honor longtime UK Alpha-1 researcher Robert Stockley

The European Respiratory Society will honor Robert Stockley, MD, with its Presidential Award at the 2014 ERS International Congress in Munich, Germany, Sept. 6-10.

Stockley, professor of medicine at the UK’s University Hospital Birmingham and director of the Lung Immunobiochemical Research Group, has focused his research on Alpha-1 lung disease and chronic obstructive pulmonary disease (COPD). This year, he was lead investigator on Kamada’s Phase II/III study of inhaled therapy for Alpha-1.

Stockley organizes the UK’s Alpha-1 Antitrypsin Deficiency database and established the International Registry for Alpha-1. He has been coordinating multi-center trials of augmentation therapy and alveolar regeneration in those patients.

He has published more than 400 peer-reviewed papers, reviews and chapters, edited seven books, supervised 35 higher degree theses in clinical and basic science, and been a member of the editorial board of more than 15 journals. He initiated and established the International meeting on COPD that is held every two years in Birmingham, and is currently finalizing the 2014 meeting program.

Stockley, whose interest in Alpha-1 research began in the 1970s, says it “became clear that the story was far from simple …. I became aware that the presence of [Alpha-1] did not necessarily mean the development of lung disease even in smokers. Nevertheless, the general acceptance of the logical concept of the process of emphysema development resulted in little support for further research funding.”

In the mid-1990s, he was approached by pharmaceutical company Bayer on doing clinical trials on augmentation therapy, which was not available in the UK. Through the collaboration, ADAPT (Antitrypsin Deficiency and Program for Treatment) was established.

“The collaboration  has been highly successful in learning about the clinical variations of what was believed to be a single disease process, the role of other genetic modifiers, the variability in lung function decline, the role of exacerbations, the validation of CT scanning as the most sensitive outcome measure in [Alpha-1] and hence the design and delivery of clinical trials of augmentation therapy, new strategies in gene correction and even studies on the liver,” Stockey said. “This unique combination of basic science and clinical observation leading to understanding of the disease and the design of clinical trials has been central to where we are today.”

Source: Alpha-1 Foundation 28/08/2014

Alpha-1 Global launches website, linking Alphas worldwide

Today marks an exciting milestone for the Alpha-1 community worldwide: The Alpha-1 Global website alpha-1global.org is up and running.

Everyone is encouraged to become a part of Alpha-1 Global:

• Sign up for the discussion platform via the special link on the Welcome page. Create your user name in the format firstname.lastname (for example: linda.smith).
• Even if you aren’t an active participant in discussions, the more people who sign up, the more successful and cohesive the global Alpha-1 community will become.
• Put a link to alpha-1global.org on your website or social media sites to show your support and affiliation.

The website is the culmination of more than a year of planning. In April 2013, Alpha-1 leaders from 23 countries gathered at the 4th International Alpha-1 Patient Congress in Barcelona, Spain. Celebrating 50 years of progress, they agreed the next vital step was to create an organization fostering a strong global community. Alpha-1 Foundation President John Walsh agreed to put Foundation resources behind the initiative, and Alpha-1 Global was formed. Its new director, Gonny Gutierrez, recently answered questions about the organization.

Q. A united resolve to stay connected as a global Alpha-1 community came from the Congress in April 2013. How long afterward was Alpha-1 Global launched?

The initiative launched when I was hired in September 2013, less than five months after the Barcelona Congress. The Alpha-1 Foundation really followed through on its promise to provide leadership and resources to assist in building a cohesive network of organizations and patient groups, as well as building a communications platform for the Alpha‐1 community worldwide.

Q. What is your background? What interested you about becoming the director?

I have worked for international nonprofits for more than 25 years and have directed a variety of programs and large events for different charitable organizations. My foremost interest is in strategic networking and coalition-building, and I enjoy bringing together people with diverse strengths and cultural backgrounds to accomplish a mission. Becoming the director of A1G promised to merge my professional experience, cultural background and temperament. I believe that language barriers don’t mean communication barriers.

Q. What are your major goals this year, and the path to reach them?

Our overall goal is to establish a global communication platform for Alpha-1 patient groups and organizations. This year I’ve built relationships with leaders from associations around the world and besides meetings face-to-face, I usually connect via Skype. That’s the wonderful thing about our information age: despite busy schedules and time zone differences, Alpha-1 Global can be open for business 24-7.

The Alpha-1 Global website, now be up and running, includes a discussion forum open to anybody who registers. Alpha-1 leaders from different countries will monitor the topics.

The final goal for 2014 is to create a resource library that includes materials from different stakeholders, including industry partners, to build a comprehensive advocacy toolkit that can be adapted by Alpha-1 representatives worldwide. We will categorize countries by augmentation therapy licensure and focus foremost on assistance to countries that have expected licensure of drugs in the next year or two and assist them with mobilization and action steps.

Q. What roadblocks do you see in achieving your goals? 

Since we are dealing with volunteer leadership from different countries and continents, myriad healthcare systems, and a variety of views on treatments for Alpha-1, the community presents an interesting scenario.  It is essential that Alpha-1 Global functions as a central point of contact.

Q. How are countries participating in Alpha-1 Global?

If augmentation therapy is available in a country, we ask leadership to share their knowledge of how they set up their health management system and share action plans that gained them access to care. After obtaining this from different sources, we can provide step plans for associations in countries that are ready to mobilize their communities and campaign for access to care and reimbursement. By providing these resources within a collaborative network, the entire Alpha-1 community will be served. My experience so far is that most leaders are open to participating on this level, as long as we are willing to understand the realities that Alpha-1 communities face on a local and regional level.

Q. Tell us about the Global Steering Committee.

The committee currently consists of seven dedicated members: Shane Fitch (Spain), Gunhil Norhave (Denmark), Karen North (UK), Catarina Pyrrait (Portugal), Steve Knowles (Australia), Frank Willersinn, MD (Belgium) and John Walsh (U.S.). Our first face-to-face meeting took place during the National Education Conference in Kansas City, early June. A second meeting is planned following the European Respiratory Society Congress in Munich (Sept. 6-10). We’ve built a very close team committed to Alpha-1 Global’s mission.

Q. What do you hope to accomplish in the next committee meeting?

Our objectives are to develop a 2015-2017 Strategic Plan and to outline the 2015 Alpha-1 Patient Congress program. We will also create an Alpha-1 Global meeting and conference attendance schedule to ensure we have expert representation at key meetings around the world.

Q. Describe how the website serves the global community.

The site, alpha-1global.org, serves as a hub for global communication. In a year, this site will probably look very different, as people around the world will have had a chance to add their thoughts, share ideas, and evaluate the need for expansion into different languages. We also hope to develop a comprehensive communication structure with our network of association leaders and provide additional awareness and campaign resources.

Q. What is the relationship between Alpha-1 Global and the Alpha-1 Foundation?

Alpha-1 Global is an independent program under the fiduciary umbrella of the Alpha-1 Foundation. The Global Steering Committee approximates a board serving the interests of Alpha-1 Global. We are very intentional about being inclusive of all continents and develop strong ties with local leadership around the world.

Q. Tell us about the agenda for the 2015 Patient Congress and Research Conference, and how they fit with goals of Alpha-1 Global.

The Congress will take place April 9-11, 2015 in Barga, Italy. The hotel facilities can host the entire Congress under one roof. It will be set up as a 2-day/3-night all-inclusive event, with updates on Alpha-1 developments from medical professionals and researchers from around the world. There will also workshops and hands-on training on how to organize Alpha-1 communities to advocate for access to care on a local level. All components will be very intentional in working to build a unified global Alpha-1 community.

Source: alpha-1 GLOBAL 27/08/2014

Minister gives update on review of Specialised Service Commissioning

In April, NHS England established a specialised task force to make some immediate improvements to the commissioning of specialised services after a budget overspend of £400 million. Originally scoped for three months, the task force has now been extended to run for a further three months, resulting in a delay of the publication of NHS England’s five-year strategy for specialised services.

Peter Aldous MP met with the Alpha-1 Alliance in May where we briefed him about the unmet medical need of Alpha-1 patients in England and our policy suggestions to improve access to services. Following the meeting, Mr Aldous tabled a Parliamentary Question to the Secretary of State for Health asking for clarification on the next steps in the commissioning process for a highly specialised Alpha-1 Service.  Along with several other MP’s, Mr Aldous asked for an update on the review into specialised service commissioning.

The Conservative Public Health Minister, Jane Ellison MP, responded by providing information on the extension of the review and also the scope. The taskforce is expected to call on around 50 experts from different disciplines to give their opinions on how to improve the commissioning process for specialised services.

Source: Alpha-1 Alliance 27/07/2014

Alpha-1 Alliance’s application for highly specialised Alpha-1 service endorsed by Clinical Reference Group

The Alpha-1 Alliance is delighted to announce that the Clinical Reference Group (CRG) for Specialised Respiratory Diseases has formally endorsed the Alpha-1 Alliance’s application for the establishment a highly specialised service for Alpha-1 Antitrypsin Deficiency in England. The CRG for Specialised Respiratory Disease is a committee of senior respiratory clinicians, who advise NHS England on the commissioning of new specialised services.  Receiving the CRG’s endorsement for our application is an important milestone in establishing the proposed specialised service. The Alliance’s application will now enter the next steps within NHS England’s formal commissioning review with strong support of the wider clinical community.

Karen North, Trustee of the Alpha-1 UK Support Group and Executive Member of the Alpha-1 Alliance, commented: “Receiving the CRG’s endorsement means that the Alliance’s application has now crossed the first big hurdle on the path to a national specialised service for Alpha-1.We will continue to engage with NHS England officials in the coming months to support the review of our application by the different commissioning committees.”

The Alpha-1 Alliance is currently in the process of establishing a permanent Clinical Advisory Committee of senior expert clinicians with specific expertise in Alpha-1 to advise the Alliance throughout the further development of the specifications of the proposed Alpha-1 service and their implementation. The Committee will provide ongoing clinical advice to the Alliance’s Executive Members and support the Alliance’s formal engagement with NHS England’s commissioning process.  The Committee will also ensure that the views of the wider Alpha-1 clinical community are comprehensively reflected within the Alliance’s campaign.

Source: Alpha-1 Alliance 17/07/2014

Alpha-1 Alliance secures wide clinical expert support for a national service at Advisory Board

Yesterday, the Alpha-1 Alliance brought together clinical experts specialising in Alpha-1 and Alpha-1 patient group representatives to discuss the desired service specifications for a future national highly specialised service for patients with Alpha-1 Antitrypsin Deficiency (Alpha-1) in England. The objective of the meeting was to collate the views of expert NHS clinicians in Alpha-1 and patient representatives on how access to specialist, coordinated and multidisciplinary care can be improved and made more equitable across the country.

Wide patient and clinical consensus was reached on the details of what comprises such care and how it can be made available to affected patients in England in a cost-effective manner. The outcome of the meeting informed the Alliance’s formal application for a highly specialised Alpha-1 service in England, which will be submitted to NHS England within the next few days, together with a consensus paper signed by all of the expert clinicians who attended the Advisory Board meeting, expressing their full support and endorsement of the Alliance’s application. Both documents will then be reviewed by the Clinical Reference Group for Specialised Respiratory Disease, a committee of senior respiratory clinicians who advise NHS England on the commissioning of new specialised services.

Source: Alpha-1 Alliance 27/06/2014

Minister answers Parliamentary Question on Alpha-1 Antitrypsin Deficiency – In May, the Alpha-1 Alliance met with Peter Aldous, Conservative MP for Waveney, to discuss the unmet medical need of patients affected by Alpha-1 Antitrypsin Deficiency (Alpha-1) and the Alliance’s campaign for the establishment of nationally commissioned highly specialised service for Alpha-1 in England.