{"id":4054,"date":"2020-05-18T15:37:21","date_gmt":"2020-05-18T13:37:21","guid":{"rendered":"https:\/\/www.alpha1-deutschland.org\/?p=4054"},"modified":"2024-05-17T11:25:07","modified_gmt":"2024-05-17T09:25:07","slug":"05-2020-volles-programm-beobachtungsstudie-aus-irland-umfrage-zu-alpha-1-und-vieles-mehr","status":"publish","type":"post","link":"https:\/\/alpha1-deutschland.org\/en\/05-2020-volles-programm-beobachtungsstudie-aus-irland-umfrage-zu-alpha-1-und-vieles-mehr","title":{"rendered":"05\/2020: Full program: Observational study from Ireland, survey on Alpha-1 and much more"},"content":{"rendered":"
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A full program: an observational study from Ireland, a survey on Alpha-1 and Covid-19, answers regarding mandatory mask-wearing, and a YouTube online seminar.<\/span><\/h1>\n<\/div>\n\n\n\n
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Author<\/h4><\/span><\/span>\n<\/div>\n
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Marion Wilkens<\/strong><\/p>\n<\/div>\n\n\n\n

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We already discussed the treatment of Alpha-1 during the Covid-19 pandemic in newsletter 3\/2020. Many aspects were hotly debated, and some questions remain unanswered. However, the intensive research on this new virus continues to yield new insights. Therefore, we are continuing to gather information and would like to share important updates with you.<\/p>\n<\/div>\n\n\n\n

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An observational study from Ireland shows the consequences of abruptly stopping alpha-1 antitrypsin (AAT) substitution therapy:<\/span><\/h2>\n

In February 2017, the Irish Health Authority decided against reimbursing AAT replacement therapy. In the preceding years, some Irish patients with alpha-1 antitrypsin deficiency (AATD) had participated in the RAPID and RAPID-OLE trials and continued to receive AAT treatment beyond the study period (through an extension agreement with the manufacturer). At the beginning of 2017, the manufacturer negotiated with the Irish Health Authority to secure future public funding for these patients' therapy. As no agreement was reached, treatment was discontinued in September 2017 for 19 patients with severe AATD. Up to that point, they had received intravenous AAT at a dose of 60 mg per kg of body weight for an average of 8-10 years.
\nBy mid-December 2017 (approximately 77 days after discontinuing therapy), two of the 19 patients had already died from a severe exacerbation (disease flare-up). Comparing the patients' data \u2013 now without substitution therapy \u2013 to the corresponding period the previous year with substitution therapy, the average exacerbation rate was increased (approximately one more exacerbation per patient). The number of hospitalizations also rose, and various inflammatory markers in the blood were significantly elevated.
\nThe Irish Alpha1 patient organization presented the results of the observational study to the authorities and an agreement was reached regarding cost coverage by the authorities and the manufacturer for the 17 patients.
\nAccording to the ug publication, these observations suggest that abruptly stopping alpha-1 antitrypsin substitution therapy after a prolonged treatment period can have serious health consequences for the patient.
\nWe understand the concerns of every patient who is reluctant to visit a doctor's office for their iodine replacement therapy during the Covid-19 pandemic, but this study shows that a prolonged interruption of treatment is not a sensible alternative. Therefore, we strongly encourage you to discuss with your doctor which form of continued treatment might be appropriate for you. Consider the options of home and self-infusion as well.<\/p>\n

Summary by Marion Wilkens.
\nNote: Unfortunately, this article Oliver J McElvaney et al., N Engl J Med 2020; 382:1478-1480<\/a> Accessible only with an existing subscription to the New England Journal of Medicine.<\/em><\/p>\n

We received a further comment from our advisory board member Prof. Sabina Janciauskiene on this matter:<\/p>\n

Consequences of abrupt discontinuation of alpha-1 antitrypsin replacement therapy \u2013 Reply to the editor:<\/span><\/h3>\n

The goal of augmentation therapy with human plasma alpha-1 antitrypsin (AAT) is to slow the progression of pulmonary emphysema in patients with inherited AAT deficiency. This treatment is continued throughout the patient's life. The letter by McElvaney et al. (April 9 issue) 1) provides important evidence that discontinuing this long-term therapy is associated with poorer health outcomes, including more frequent exacerbations. Lung transplantation (LuTx) is the last treatment option for patients with AAT deficiency who develop end-stage lung disease. In most clinical centers, the use of augmentation after LuTx is not recommended. However, in patients who received augmentation prior to LuTx, withdrawal of this therapy may have adverse effects. Data from the Transplant Center at Hannover Medical School show that 10-year survival rates for patients who received AAT therapy prior to lung transplantation are 50% worse than for patients who were never treated.<sup>2<\/sup> In general, survival rates for lung transplant recipients with AAT deficiency are similar to those for other forms of chronic obstructive pulmonary disease.<sup>3,4<\/sup> The consequences of augmentation withdrawal under different clinical situations must be carefully considered.<\/p>\n

Since the publication of their article, the authors have reported no further potential conflicts of interest.<\/p>\n

Authors: Sabina Janciauskiene-Wandmarke, Jan Fuge, Sabine Wrenger, Thomas F\u00fchner, Tobias Welte<\/p>\n

References
\n1) McElvaney OJ, Carroll TP, Franciosi AN, Sweeney J, Hobbs BD, Kowlessar V, Gunaratnam C, Reeves EP, McElvaney NG. Consequences of Abrupt Cessation of Alpha1-Antitrypsin Replacement Therapy. N Engl J Med. 2020 Apr 9;382(15):1478-1480. doi: 10.1056\/NEJMc1915484
\n2) Eur Respir J. 2017 Sep 10:50(3). pii: 1700962. doi: 10.1183\/13993003.00962-2017.
\n3) Banga A, Gildea T, Rajeswaran J, Rokadia H, Blackstone EH, Stoller JK. The natural history of lung function after lung transplantation for alpha(1)-antitrypsin deficiency. Am J Respir Crit Care Med. 2014:190(3):274-281.
\n4) Transpl. Int. 2018 Jan;31(1):45-55. doi: 10.1111\/tri.13038<\/em><\/p>\n<\/div>\n\n\n\n\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n<\/div><\/div>\n\t\t

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They are being asked: Alpha-1 and Corona<\/h2>\n

Alpha1 Germany, together with our advisory board member Prof. S. Janciauskiene, aims to gather insights into the connection between our existing medical condition and its influence on infection and the course of a COVID-19 infection. The following anonymous survey is a first step in this direction, and we will gladly make the results available to patient organizations, physicians, and researchers.<\/p>\n

We are all encouraged by the fact that so far only a few people with Alpha syndrome seem to be affected. There is no official reporting center for Alpha syndrome \u2013 we would like to address this to some extent. Doctors are also collecting data across Europe through the EARCO working group, and we naturally compare our findings. Therefore, our earnest request: Join in!<\/strong><\/a><\/p>\n<\/div>\n\n\n\n\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n<\/div><\/div>\n\t\t

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New information on the mask requirement<\/span><\/h2>\n

We also receive many questions about the mask requirement. The patient library prompted the board of the German Society for Pneumology (DGP) and the German Respiratory Society to formulate corresponding recommendations. These can be found here.<\/a>.<\/strong>
\nBut is it really that easy for us to wear masks? Are there ultimate masks that can make life easier for those of us who already find breathing difficult without additional resistance?
\nBased on our own experience, we find medical-grade surgical masks (also known as surgical masks) to be suitable, as the breathing resistance feels lower than with fabric masks (we have not found any supporting evidence for this). Please be sure to try on each mask carefully before you notice, only during exertion, that you are getting short of breath too quickly.<\/p>\n

If you leave the house without a mask (of course with official permission from your doctor), or even go shopping, many people give you strange looks; you feel like an outcast. It's a stigma we don't need \u2013 or what do you think?<\/p>\n

Feel free to send us your experiences at Erfahrungen@alpha1-deutschland.org<\/strong><\/p>\n

Great initiative!<\/span><\/h3>\n

In keeping with the mask theme, we'd like to report on a wonderful initiative: The family of our board member Ronald L\u00fcdemann had a fantastic idea: Marleen (13) and her mother spent a week sewing a total of 250 masks, which they then donated to the company where her father works. The Steinbrecher company expressed their gratitude with a donation of \u20ac1,250 to Alpha1 Germany. We are overwhelmed by this effort and extend our heartfelt thanks to Marleen and Simone.<\/p>\n<\/div>\n\n\n\n\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n<\/div><\/div>\n\t\t

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A personal note<\/span><\/h2>\n

The 2020 information day has now been officially cancelled, and we have avoided any cancellation fees. It was very disappointing for us not to be able to cancel earlier, but from a financial perspective, it was the right course of action for the association, as the state of Lower Saxony had to officially assess whether the event was justifiable or not. In fact, we only received official approval for the free cancellation a few days before the event was due to begin. Of course, we wouldn't have taken any risks with our Alphas, but the very high cancellation fees would have been a significant burden had we cancelled earlier.<\/p>\n

Hopefully, we will all be able to celebrate the next information day with anniversary (April 23-25, 2021) together again.<\/strong><\/p>\n<\/div>\n\n\n\n\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n<\/div><\/div>\n\t\t

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Online seminar on YouTube with Dr. Greulich<\/h2>\n

As a small consolation prize, we got ourselves a Online seminar with Dr. Timm Greulich<\/strong> considered.<\/p>\n

On May 26 <\/strong>It will be uploaded to our YouTube channel \u2013 the premiere will take place at 3 p.m. Tune in and listen to Dr. Greulich's lecture.<\/p>\n

Click here to go to our YouTube channel.<\/strong><\/a><\/p>\n<\/div>\n\n\n\n\n\t\t\t<\/div> \n\t\t<\/div>\n\t<\/div> \n<\/div><\/div>\n\t\t

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Further information<\/span><\/h2>\n
As in previous newsletters, we have compiled some links that we believe contain interesting and useful content on the topic of Corona:<\/div>\n