On February 25th, an event was held by the ELF Working Group on Mental Health and Lungs Europe to raise awareness of the impact of lung diseases on mental health. Patients, relatives, professionals, and organizations shared personal experiences and discussed research and action needs.

A key topic was the close link between chronic lung diseases and mental health, as well as the need for further research and policy measures. The working group, established in 2024, is committed to raising awareness of this issue and developing concrete strategies.

The three main aspects were:

• How living with a lung disease affects the mental health of patients and their caregivers.
• What gaps exist in mental health research and what priorities are set for the future.
• What activities and priorities the ELF working group on mental health pursues and how these are linked to EU policy.

The webinar is on demand here Available. The language is English.

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Since October 2022, patients with Alpha-1 Antitrypsin Deficiency have had the opportunity to participate in our personalized training and respiratory physiotherapy program PROMISE, and we are very pleased that the program is being extended with another 25 places!

What is changing?

• The program will now nationwide Offered: To also be able to provide care to interested patients who do not live in our catchment area, we have switched PROMISE to a purely digital program. We will, of course, provide support with any technical questions.

What remains the same?

Our goal: Most people know that physical exercise and knowledge of how to manage alpha-1 antitrypsin deficiency and COPD significantly contribute to a good quality of life. But how can you effectively integrate targeted training into your daily routine? What simple tricks can help you control and improve shortness of breath during exertion (and the fear of it)? And which exercises are best suited for you personally?

To optimize disease management outside of drug therapy, we have launched the PROMISE program, which is specifically designed for Alpha-1 Antitrypsin Deficiency patients.

The content:

PROMISE is offered by Pneumo Factory (located in Schönau am Königssee) with financial support from CSL Behring. The program aims to empower you to become a competent manager of your condition by providing non-pharmacological therapy components tailored to your individual needs. A training plan for endurance and strength training is created, which can be performed in your own home without the need for any equipment. Furthermore, essential respiratory physiotherapy techniques, such as pursed-lip breathing, are reviewed and taught, and practical information is provided on topics such as mental health, long-term oxygen therapy, and nutrition.

How long does the program last?

The program initially lasts 8 weeks and includes 6 digital appointments via video conference (using a data protection-compliant platform) with a therapist from Pneumo Factory (physiotherapists, sports scientists). All appointments are spaced approximately 2 weeks apart (by individual arrangement), allowing participants time to independently try out the new training plan in the interim. The long-term goal is for the training to be integrated into daily life and carried out independently and regularly! Furthermore, a digital follow-up session takes place 8 weeks after the last regular appointment (Figure 1).

How can I tell if the program was effective?

At the first appointment, a comprehensive assessment is conducted according to scientific standards, allowing for an evaluation of physical performance, quality of life, and symptom intensity. These assessments are repeated at the fifth appointment to track any changes. To examine the long-term benefits of the program, a final, sixth appointment takes place eight weeks after the fifth, repeating all assessments. Naturally, all participants will receive feedback on their personal progress upon request.

Who is behind PROMISE?

The content was developed by Pneumo Factory (Dr. Tessa Schneeberger [MSc. Sports Physiotherapist, Respiratory Physiotherapist] and Dr. Inga Jarosch [Dipl. Sports Scientist], both research associates and speakers for the Respiratory Physiotherapy Working Group, the Respiratory Therapist Training Program, and the Heart LAG Training Program) based on current scientific findings (www.pneumo-factory.de). Thanks to the generous support of CSL Behring, PROMISE can be offered free of charge to patients.

Who can participate?

Any patient with alpha-1 antitrypsin deficiency who suffers from COPD and feels limited in their daily life by the disease can participate in PROMISE free of charge. To rule out any medical contraindications for training before participating in PROMISE, training approval from your general practitioner or pulmonologist is required (the form is available for download at www.pneumo-factory.de or upon request at info@pneumo-factory.de). To date, 46 participants have successfully completed PROMISE.

What do PROMISE participants say?

• „…The therapist was a stroke of luck, because he was not only very personable, but also highly competent and goal-oriented. I quickly realized this, as he identified my shortcomings, weaknesses, and exercise needs in a short time. (…) I was praised, because the targets set at our first meeting were exceeded. I had already noticed this myself, as my shortness of breath when climbing stairs or walking uphill had decreased, my strength had increased, and I felt fitter overall…“
• …“Whether my empyema (…) has improved, I cannot say, but I definitely know that I can breathe much better, that I no longer have panic attacks of shortness of breath, and especially… I am no longer afraid of climbing stairs! The correct technique, pursed-lip breathing, the right pace, the correct inhalation and exhalation, etc., I had previously paid too little attention to all of that…“
• „…The therapist ensured that I could easily integrate my training sessions into my daily routine and also addressed any health setbacks. All in all, my physical performance has noticeably improved, and I'm coping much better with everyday life. Climbing stairs, in particular, is now easier than at the beginning. The program also motivated me to stay committed and continue working on my fitness, nutrition, and self-care. I'm very grateful that I was able to participate in the program and would gladly recommend it…„

Are you also interested in participating in PROMISE or do you have any questions about it? Then please contact us by email: info@pneumo-factory.de. We are happy to answer all your questions about the program!

You can download this information page here:

Promise extension

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At the beginning, Professor Dr. Sabina Janciauskiene from the Department of Pneumology and Infectious Diseases at Hannover Medical School (MHH), as scientific director of the event, and Marion Wilkens, board member of Alpha1-Deutschland e.V., welcomed the large number of guests. They also immediately expressed their gratitude to those who supported and made the event possible: Dr. Annegret Zurawski, clinic manager at MHH, for organizing and conducting the event; Inga Kwapniewska from the German Center for Lung Research (DZL) at MHH for her organizational support; the sponsors Grifols, CSL Behring, and Takeda for their financial support; and Linde for providing the oxygen.

The first presentation was given by Dr. Isabell Pink on the topic of "Bronchiectasis in AATD." Bronchiectasis refers to the widening and dilation of the bronchi, the airways between the trachea and the alveoli. Since AATD itself is a rare disease, its contribution to the overall incidence of bronchiectasis plays only a minor role, as can be seen from analyses of the corresponding European Bronchiectasis Registry EMBARC. However, studies based on the European Alpha-1 Registry EARCO have shown that a large proportion of individuals with the PI*ZZ variant of AATD exhibit bronchiectasis, usually in addition to pulmonary emphysema (27% of all Alphas have both).

Treatment for bronchiectasis initially focuses on the underlying condition, such as asthma or COPD, primarily with inhaled medications. Smoking cessation is also mandatory, and respiratory physiotherapy and measures to loosen secretions (e.g., using nebulizers) are helpful. However, these measures only alleviate symptoms and may prevent inflammation; the tissue changes themselves are irreversible.

The aforementioned deformities of the bronchi lead to the accumulation of mucus, which provides an ideal breeding ground for all kinds of pathogens. This often results in chronic bronchiectasis, a condition for which two of the following three criteria must be met for diagnosis:

• Cough on most days of the week
• Mucus production on most days of the week
• experienced exacerbations (su)

These chronic symptoms can sometimes develop into acute, crisis-like inflammatory processes, known as exacerbations. An exacerbation is suspected if at least three of the six key symptoms occur or worsen for at least 48 hours.

• Cough
• Ejection
• Discoloration in the sputum
• shortness of breath
• fatigue
• Coughing up blood

Fever, inflammation markers, and other clinical abnormalities can also support the diagnosis.

The therapy to be applied depends on the severity of the symptoms; it ranges from physiotherapeutic drainage of secretions to oral antibiotics and intravenous high-dose antibiotics as part of inpatient treatment.

Approaches to reducing the frequency of exacerbations depend on any identified triggers:

• If the self-cleaning function of the airways is impaired, it must be strengthened, e.g. through appropriate medication and targeted respiratory physiotherapy.
• In cases of bacterial infections, the aim is to permanently eliminate the relevant bacterial strains (e.g., Pseudomonas aeruginosa) through targeted antibiotic treatment; incidentally, patients with bronchiectasis and an acute Pseudomonas aeruginosa infection can currently (autumn 2024) apply to participate in a drug trial at [website address missing]. https://www.lungeninformationsdienst.de/klinische-studien/aktuelle-klinische-studien/non-cf-bronchiektasen/eradicate
• In cases of respiratory tract inflammation, therapy is based on the functional cause of the inflammation.

If the underlying cause is neutrophilic, a drug is now nearing the end of clinical trials: Brensocatib from Insmed. Phase 3, the comparison of efficacy with a placebo, has been successfully completed and largely evaluated: treatment with Brensocatib resulted in significantly fewer exacerbations during the observation period than in the placebo group, and the time to the first exacerbation was significantly prolonged; no serious adverse events were observed. The approval process has been initiated, so availability for patients is expected from 2026 onwards. It is anticipated that AATD patients with the corresponding clinical picture will also benefit from treatment with Brensocatib.

Following this positive outlook, in her subsequent presentation, "Living with AATM," Marion Wilkens, chairwoman of Alpha1-Germany, shared her life story with the audience: as a baby, she suffered from severe jaundice and failure to thrive, which, with today's knowledge, were already early signs of her illness. At 20, she was diagnosed with asthma, and it took another 20 years until she received an AATM diagnosis. Since then, her condition has been managed with regular lung and liver examinations, plenty of exercise, respiratory physiotherapy, and an adapted lifestyle. Among other things, she has been helped in coping with the illness by the wealth of information she has found, some of which she found readily available, and some of which she has gathered herself through her work with the association and presented in her talk. She emphasized once again the value of a large, well-informed, and mutually supportive community like the Alpha1 Germany association as a building block for successfully managing AATM, and then presented its structure and work in all its facets: regular events, self-help groups, information channels online and in print, free pulmonary exercise and memory training, and networking with organizations of doctors and patients (including internationally). All of this serves the goal of enabling those affected to live better lives with AATM.

The third presentation was given by Dr. Hinze on the topic of "Infections and Vaccination in AATD." He based his presentation on the recommendations of the nationwide Standing Committee on Vaccination (STIKO). While the primary goal is to avoid the infections in question and their often very unpleasant side effects, the aspect of preventing additional cardiovascular strain in patients with chronic pre-existing conditions and advanced age also plays a crucial role.

The following vaccinations are recommended:

• Pneumococci to prevent pneumonia (the vaccine is selected by the vaccinating doctor based on the individual's vaccination status)
• Respiratory syncytial virus (RSV) to prevent exacerbations; at the time of the presentation, cost coverage still had to be arranged individually with the respective payer, but in the long term, it is expected that health insurance companies will cover the costs for high-risk patients in late 2024.
• SARS-CoV2 (Covid-19)The vaccination should be boosted annually; after infection, the booster can be omitted for one year.
• Influenza (flu) as a high-dose vaccination for people over 60 years of age, also with regard to preventing the exacerbation of cardiovascular crises (heart attacks!).
• Herpes zoster (shingles) as an inactivated vaccine; even if there is a noticeable reaction to the first vaccination, the second vaccination should definitely be carried out after 2 to 6 months.

Dr. Mark Greer then gave a presentation on the topic of "Lung Transplantation in AATD." This procedure should be considered when lung function is largely lost and no further treatment options are available. However, transplantation is subject to a number of other prerequisites, including:

• Age
• body weight
• Compliance with the smoking ban
• no cancers
• no diseases of other vital organs
• psychological stability
• Ability/willingness to undergo rehabilitation

In addition to these objective criteria, assessments by the treating physicians also play a role, e.g.:

• a mortality risk above 50% within the next 2 years of life
• A survival probability of over 80% for the next 5 years of life after transplantation

If all these conditions are met, a patient can be considered for a lung transplant. This will then take place as soon as a suitable donor organ is available that is not needed for a patient with an even greater need for a transplant.

In the vast majority of cases, the patients' quality of life is significantly improved by the transplant, even though life afterwards differs significantly from life before: for example, medication must be taken regularly for life to prevent rejection of the new lung, and close monitoring by the transplant center remains necessary for years to come.

The success rate of lung transplants is constantly improving: half of all lung transplant recipients now survive the operation for more than 6.5 years.

Following this presentation, Prof. Dr. Sabina Janciauskiene addressed the question "Substitution – yes or no?", illustrating her points with reference to real-life case studies. She began by describing the mechanism of action of substitution therapy, which aims to compensate for the unbalanced activity of neutrophil elastase that leads to the breakdown of lung tissue. Alternatives to the common intravenous administration of AAT are currently under development: inhalation, transdermal administration, and the use of recombinant (i.e., biochemically produced) AATs. The latter is currently being tested in a clinical trial: this variant is metabolized more slowly by the body, potentially allowing for significantly longer treatment intervals, but it is also associated with a number of side effects.

With conventional replacement therapy using a weight-dependent dose, the treatment success for individual patients is sometimes difficult to predict, as examples from clinical practice demonstrate. Determining the dose based on the existing AAT level and other clinical parameters may offer advantages. Patients with poorer lung function than currently anticipated could also likely benefit from replacement therapy. However, no improvements have been observed in patients with bronchiectasis or asthma. The continuation of therapy after lung transplantation, on the other hand, remains controversial.

It is often overlooked that AATD can trigger a host of skin problems, often indirectly via AATD-related intestinal issues. These include, for example, acne, eczema, psoriasis, rosacea, urticaria, and dermatitis herpetiformis.

The final presentation of the day was given by Dr. Katja Deterding on the topic of "AATD from a Hepatologist's Perspective – New Treatment Options." She began by explaining that AATD involves an imbalance between AAT and neutrophilic elastase (NE), which can lead to the enzymatic destruction of lung tissue, resulting in pulmonary emphysema. This imbalance does not arise from insufficient AAT production in the liver, but rather from AAT being misfolded, causing it to polymerize into clumps or strands so large that they cannot leave the liver cells that produce them. This can lead to liver fibrosis, cirrhosis, or even liver cancer. While this process is particularly pronounced in individuals with the homozygous PI*ZZ variant, those with the heterozygous PI*MZ variant have more AAT entering the bloodstream, thus largely protecting the lungs, at least in non-smokers. However, in these patients, the risk of liver complications is significantly increased if chronic liver diseases are also present.

To detect and classify liver fibrosis, a liver tissue biopsy is the most reliable method, but also the one with the highest risk of adverse side effects. In contrast, measuring liver elasticity using FibroScan is free of side effects and therefore very suitable for monitoring disease progression. For monitoring alphas with the ZZ classification, the following are recommended:

• Annual liver function tests, possibly more frequent if taking potentially liver-damaging medications.
• Fibroscan every 3 years if the results are normal, otherwise more frequently (alternatively, the APRI value can be calculated from measured liver values; see also the link to an APRI calculator on the Alpha1 Germany website at [link to APRI calculator]). https://alpha1-deutschland.org/alpha-1-und-die-leber)
• Liver function test in advanced disease
• Cancer monitoring in advanced cirrhosis
• Even though liver and lung problems only very rarely occur together in ZZ patients, the lungs should be checked regularly even if abnormal liver findings are present.

Novel therapeutic approaches for AATM look like this:

• The intake of chemical agents is intended to correct the misfolding of AAT so that it passes from the liver into the blood in sufficient quantities to avoid burdening the liver with polymers and to adequately protect the lungs.
• By intervening in the genetic material (DNA) or in the copies of individual DNA segments necessary for the formation of proteins (i.e., based on RNA), the liver is to be made to produce "normal" AAT.
• Similarly, RNA-based interventions can downregulate the production of AAT, thereby allowing a liver burdened by AAT clumping to recover; a clinical trial of this approach has been underway in Germany for some time and is currently being expanded significantly, for which further study participants are being sought; interested parties can find further information, including the inclusion and exclusion criteria for participation, online at [website address]. https://global.theredwoodliverstudy.com/de-ch

This presentation concluded the second MHH patient seminar on "Alpha-1 Antitrypsin Deficiency." The large number of participants, the numerous questions and lively discussions with the speakers, and the intensive exchange of opinions among the attendees give them hope for further events in this seminar series.

Dr.-Ing. Heinz Stutzenberger

08.09.24

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This webinar took place for the first time in 2024, but certainly not for the last time.

The most important sessions covered:

The basics of Alpha-1 (AATD)

Living with Alpha-1

The latest research + patient participation in clinical trials

Treatment and therapy options

Did you miss the conference? – No problem, because the patient conference is here Available on demand.

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October 28, 2024 – Autumn Time

Whether it's a cold, bronchiectasis, or the effects of Covid-19, many people are familiar with the problem of struggling with mucus in the body, and especially in the lungs.

Our pulmonary exercise trainer, Peter Kukry (Peddar), himself an Alpha-1 lung disease patient, therefore set to work and recorded the following video for us. He is supported by Dr. Heike Isensee, an active participant in the pulmonary exercise program and group leader of the Stuttgart self-help group. The video is rounded out with expert advice from Dr. Alexander Rupp of the Im Zentrum Stuttgart pulmonology practice.

Sometimes simple techniques and exercises can make everyday life significantly easier, try them out!

Video link: (4) Coughing, coughing techniques and expectorant – Helpful exercises for home! – YouTube

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In spring 2024, an app-based registry for children and adolescents with alpha-1 antitrypsin deficiency was launched. Families themselves submit their information to the registry. The development team from the University Hospital Bonn hopes this will result in the highest possible participation rate, as only sufficiently large datasets can provide helpful information about the disease.

To participate, the app can be downloaded from the App Store (alpha 1 kids). Alternatively, registration is also possible via a web version (alpha1kids.de). After an initial registration step, families will receive a QR code by mail, which they can then use to activate the app.

Children can be registered using an initial registration form. It is also possible to register multiple children. The length of the questionnaire adjusts depending on the severity of the illness. Of great importance to the team at the University Hospital Bonn are the progress reports, which can be completed whenever there is new information to report. Laboratory results can be photographed, uploaded, or entered manually. The data entered up to that point can then be displayed in a table or graph and saved or printed.

The purpose of this registry is to gain a better understanding of the disease, so that in the future it may be possible to identify earlier which patients are at high risk of developing a serious liver disease and which are not.

A better understanding of the natural course of the disease is of great importance! Please help and register your child in the Alpha1-KIDS registry.

Thank you.

If you have any questions, please contact us at alpha1kinderzentrum@ukbonn.de Please contact the team at the Alpha1 Children's Center in Bonn directly, which manages the registry.

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Burden = Bronchiectasis Uunderstanding and Rresearch on Ddaily Eexperiences and Nseeds

They are also known as non-cystic fibrosis bronchiectasis, non-CF bronchiectasis, or simply bronchiectasis. Bronchiectasis is a chronic, often progressive lung disease characterized by irreversible, abnormal dilation of the bronchi and chronic symptoms.

Further information and registration can be found here here

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Fungal infections Oral thrush is an undesirable side effect of inhaled corticosteroid medications used to treat lung diseases. This side effect (familiar to some patients with alpha-1 corticosteroids) could easily be avoided, as such fungal infections are more likely to occur when drug particles settle on the oral mucosa and weaken the immune system. To prevent this, rinse your mouth with water after inhaling and, if necessary, gargle or inhale immediately before a meal. Using an inhaler spacer can also help.

Oral thrush usually develops when the immune system is not strong enough to prevent the spread of the fungi.

Symptoms of oral thrush:

• White coating (not removable) on tongue and inside of cheeks
• Pain when eating and drinking
• Bad breath

If you suspect you have oral thrush, you should see your doctor. Oral thrush should always be treated, as the infection can spread.

Oral thrush can usually be quickly controlled through timely and consistent treatment with antifungal medications (containing active ingredients such as nystatin or clotrimazole).

These medications, called antifungals, inhibit the growth of fungi or kill them. Treatment should always be discussed with a doctor. Self-treatment with home remedies is not recommended, as this can worsen oral thrush.

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