Recently, in the nephrology ward of the Second Affiliated Hospital of Harbin Medical University (hereinafter referred to as the Second Affiliated Hospital of Harbin Medical University), Mr. Wang (a pseudonym), a 39 year old patient with rare diseases, injected a "life-saving medicine". It is reported that this is the first case of agatase use in Heilongjiang Province α The patients with Fabry disease treated with enzyme replacement therapy are also the first patients in Heilongjiang Province to enjoy the "welfare" of the medical insurance policy of special drugs for rare diseases. Du Xuanyi, director of the Department of Nephrology of the second hospital of Harbin Medical University, said that Fabry's disease is an X-linked genetic disease, which can cause a series of heart, kidney, brain and other organ diseases. If not treated in time, it will be life-threatening. According to foreign reports, the incidence of the disease is only 1/47600-1/117000. Only a few hundred people have been diagnosed with the disease in China. Du Xuanyi said, "in May 2018, 121 rare diseases such as Fabry's disease were included in the first batch of rare diseases catalogue released by the state. With the progress of medicine and the continuous improvement of detection methods, the detection rate of such diseases is increasing year by year." The first-line treatment of Fabry disease recommended in accordance with the consensus of Chinese experts on the diagnosis and treatment of Fabry disease (2021 Edition) can reduce patients' pain and delay or prevent multisystem lesions. However, such treatment drugs are expensive, and patients need 1-1.2 million yuan a year for treatment. China has about 20million patients with various rare diseases, including Fabry disease, and more than 200000 new patients with various rare diseases every year. In the face of an increasing number of patients with rare diseases, the pace of drug security continues to accelerate. In the adjustment of the national medical insurance catalogue in 2021, the "sky high priced drugs" used to treat seven rare diseases, including Fabry's disease, were included in the new medical insurance catalogue, which gave patients with rare diseases hope and attracted more attention. (outlook new era)