From Chairman Elena Bezbozhnaya

Early 2020 HAE Russia held several events for the members and organized an educational exhibition at the museums of the two biggest cities of the Russian Federation.

In January we conducted a webinar “Effective Health Care: Diagnosis and Treatment of HAE” with members from Novokuznetsk, St. Petersburg, Ulyanovsk, Yekaterinburg, Voronezh, Murmansk and Krasnodar Krai taking part. The speakers at the online meeting were lawyer Ilya Ushankov and myself. The webinar began with a small talk about legal issues. The participants were given recommendations on how to use the right of getting preferential medicines coverage. We also discussed positions of International Classification of Diseases 11th revision (ICD-11) developed by the World Health Organization. ICD-11 has already been adopted in most European countries, and this year it is supposed to be put into effect in Russia. At the end of the meeting, the speakers answered the participants’ questions. Some of them were related to the problem of how patients with HAE can obtain disability status. Since 1 January 2020 Russia has a new procedure for obtaining disability status. The main criterion for getting such status for patients with HAE is the frequency of edema. Still, it is not clear how the patient’s condition will be monitored because the mechanism of fixation of seizures is not described anywhere.

At the beginning of February, a social photo exhibition “The HAE family” was held in The National Pushkin Museum in St. Petersburg. HAE Russia organized the event with the support of the Ministry of Culture of the Russian Federation. The exhibition – dedicated to the International Rare Disease Day on 29 February 2020 – consisted of photographs of children with HAE and their families. At the opening of the event the Director of The National Pushkin Museum Sergey Nekrasov spoke about social responsibility and society’s indifference: “The International Rare Disease Day, the idea of which has already been picked up by 85 countries, is necessary to draw public attention to those poorly known problems that people living among us have. Therefore, I consider it important and proper that such exhibitions now began to appear”.

On behalf of HAE Russia, I thanked the museum staff for their help in organizing the event and told about the current situation with rare diseases in Russia and abroad. A speech by Ekaterina Viktorova, allergist- immunologist at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology in Moscow was dedicated to the features and problems of the diagnosis and treatment of HAE. In the final part of the ceremony, guests of the event were shown a cartoon about children with HAE, created on HAE Russia’s initiative, and the information video “Hereditary angioedema. Life without Borders”, from which visitors could learn how the disease proceeds and what problems people with such a diagnosis face.

After St. Petersburg the exhibition “The HAE family” moved to Moscow. At the end of February, the grand opening took place in the Armory Chamber of the Moscow Kremlin Museums. Dmitry Morozov, professor, MD, Chairman of the State Duma Health Protection Committee, Olga Dmitrieva, Deputy General Director of the Moscow Kremlin Museum, and doctors from the primary federal clinics expressed their gratitude for the opening of the exhibition. All speakers mentioned the social significance of the event and expressed their readiness to expand cooperation with HAE Russia. In response, I thanked the Moscow Kremlin Museums’ staff for their assistance and noted the importance of raising public awareness of the existence of rare diseases. Among the guests of the exhibition were patients with HAE, members of HAE Russia (including children) and representatives of the federal media.

As mentioned, the International Classification of Diseases 11th revision (ICD-11) developed by the World Health Organization will be adopted in Russia soon. In connection with these changes, HAE Russia is concerned about the status of patients diagnosed with HAE of the third type. It is an extremely rare form of the disease. There are only a little more than ten patients with such a disease registered in Russia. In anticipation of the new version of ICD, a discussion about the advisability of including patients with the third type of HAE in the federal register of patients with rare diseases began in the medical and expert community. That is because, unlike the patients of the first two types, they have no defect in the complement system. If codes in the new edition of ICD changed, the third type of HAE would be classified as an allergic disease. This, in turn, would lead to the fact that patients would be left without modern treatment that can relieve life-threatening edema. Representatives of HAE Russia have been actively participating in all stages of this discussion for several months and have sent a petition to the Russian Association of Allergologists and Clinical Immunologists (RAACI) to clarify the situation. In response to the petition, RAACI sent a letter explaining the position of the experts. It states that after an expert discussion, RAACI will recommend the Ministry of Health of the Russian Federation to combine patients with HAE of all three types with the assignment of a single code in the new ICD-11. This is very encouraging news. After assigning a single code to ICD, all patients with HAE would have the right to receive medical coverage from the state budget.

HAE Russia continues its actions to protect the rights of patients and provide them with legal, social and psychological support.