On September 9, the National Health Commission and other six departments jointly issued the Second Batch of Rare Disease Catalog, adding 20 new rare diseases. Together with the first list of rare diseases published five years ago, the list now contains a total of 86 rare diseases.

The new inclusion of 86 rare diseases will affect patients? On the evening of September 9, The Paper (www.thepaper.cn) interviewed experts who have been concerned about rare diseases for many years, founders of public welfare foundations, and families of patients with rare neurofibromatosis who have entered the directory.

The patient's family cried with joy

The Second Batch of Rare Diseases List has a total of 86 rare diseases, including achondroplasia, acquired hemophilia, acromegaly, adult Still's disease, Alagille syndrome, gastrointestinal stromal tumor, neurofibromatosis, etc.

More than five years ago, on May 2018, 5, the National Health Commission and other five departments jointly formulated and released the First Rare Disease Catalog, which included a total of 11 rare diseases at that time. Together with the 5 new rare diseases, a total of 121 rare diseases have been included in the rare disease catalog.

Zou Yang is the mother of a small neurofibromatosis patient. Neurofibromatosis (NF) is a large group of autosomal dominant diseases, clinically there are three types, including neurofibromatosis type I (NF1), neurofibromatosis type II (NF2), and schwanyenomasis (SN). Among them, NF1 is the most common, involving multiple systems throughout the body. Since his son was diagnosed with NFI, Zou Yang joined the patient organization "Bubble Home" Neurofibromatosis Care Center in Shenzhen Bubble Home, popularizing neurofibromatosis knowledge and improving public awareness in various public welfare activities.

In the "Second Batch of Rare Disease List" released on September 9, neurofibromatosis was successfully included. That afternoon, Zou Yang, who was in a meeting, cried when he saw the news. "(Excitedly) slapped the table, really crying with joy... This is something we've been looking forward to for a long time... Very pleasantly surprised. ”

Zou Yang said frankly that they have been expecting neurofibromatosis to enter the rare disease catalog; Previously, they also speculated that NF1, with the highest incidence, was the most likely if it could enter the catalog.

"Type 2 and schwannomatosis are too niche. Today it is known that it is the whole neurofibromatosis, there is no subtype, and this disease is in the catalog. Zou Yang said that on the afternoon of the 20th, patient organizations and family groups all told each other the good news.

She also said that there are already drugs for treatment in China, and after the disease enters the catalog, it may be able to improve the accessibility of drugs to a certain extent.

I believe that more batches of catalogs will be released

On the evening of September 9, Wang Yiou, founder of the Pain Challenge Public Welfare Foundation and founder of the Pain Challenge Public Welfare Foundation, told The Paper that in recent years, patients, families and patient organizations have been looking forward to the "Second Batch of Rare Disease Catalog", because the catalog will bring more attention and promotion to the rare disease patients involved. For example, screening, diagnosis and treatment of diseases, unified and perfect diagnosis and treatment guidelines and manuals, training for doctors, etc. In addition, it is also possible to promote diseases with treatment drugs to enter the scope of medical insurance reimbursement faster, and enter the coverage of local security systems, charitable assistance or commercial inclusive insurance.

Wang Yiou said that for more rare diseases that have not yet entered the catalog, he calls on the society to have more strength to give patients other ways of encouragement, support and protection, especially those diseases that do not have effective treatment drugs, hoping not to be ignored because they have not entered the catalog.

Ding Jie, former vice president and professor of Peking University First Hospital, who has been concerned about rare diseases for many years and has served as the chairman of the Rare Disease Branch of the Beijing Medical Association, said frankly that the "First Batch of Rare Disease Catalog" announced in May 2018 is a milestone and a breakthrough from 5 to 0. After several years of discussion, it is also of great significance to announce the second batch of 1 rare diseases, and I believe that more batches of catalogs will be released in the future.

At the same time, Ding Jie also said that the 86 rare diseases announced this time still have a big gap with the number of internationally recognized rare diseases.

She introduced that under the leadership of the Joint Conference of the Chairman of the National Rare Disease Academic Organization, many experts in medical treatment, medicine, medical insurance, medical administration and other fields spent more than a year of discussion, and finally Professor Wang Lin of the National Rare Disease Academic Organization Joint Committee wrote and released the "Research Report on the Definition of Rare Diseases in China". This definition is based on expert discussions and relevant domestic data, and has three dimensions: first, the incidence of newborns is less than 14 in <>,<>; or the prevalence is less than <> in <>,<>; or the number of people affected by a single disease is less than <>,<>. If one of these conditions is met, it is recognized as a rare disease.

Ding Jie said that this definition is recognized by more and more experts. In terms of diagnosis and treatment support and guarantee for rare diseases, some rare diseases that are not included in the catalog may be guaranteed by this definition. (The Paper)