World Hemophilia Day: No cure, lifelong medication? What should people with hemophilia do?
Every April 17th is World Hemophilia Day.
The reason for choosing this date is mainly the birthday of Frank Schonabo, the founder of the World Federation of Hemophilia, in order to awaken the scientific awareness of the disease in the whole society, and to commemorate the Physicians’ contributions to hemophilia.
And this year’s theme is —–“For All – Solidarity, Policy Support, Common Development”.
Haemophilia, an injury that cannot be stopped
The so-called hemophilia is a hereditary and congenital disease mainly due to the lack of substances in the patient’s body that can promote blood coagulation, resulting in the inability to quickly stop bleeding during bleeding.
Suffering from this disease, once the patient suffers from bumps, trauma, surgery, etc., they often bleed continuously, and at the same time, there will be bleeding without any incentive, such as
strong>Bleeding gums, joint redness and swelling, etc.
It usually manifests itself as:
1. Bleeding
The most typical symptom of hemophilia is bleeding, especially in the absence of external factors, but there are signs of congestion in subcutaneous joints and other parts, mostly hemophilia The disease leads to a lack of coagulation factors, causing coagulation disorders.
At this time, it must be taken seriously and treated as soon as possible, otherwise, over time, it may even lead to intestinal bleeding and kidney bleeding.
2. Compression symptoms
The so-called compression symptoms refer to the obvious hematoma performance if the bleeding site is compressed during the process of hemostasis after bleeding.
Prevent hemophilia, preferably with genetic testing before giving birth
Since hemophilia is mostly inherited in families, about 1/3 of patients cannot show a family history, possibly because of the presence of hemophilia in the mother. New mutations.
Therefore, for high-risk groups, it is recommended to carry out genetic testing before pregnancy to determine whether they carry the hemophilia gene.
Even today, hemophilia is no easy task
If adequate and feasible treatment can be obtained, the life expectancy of hemophilia patients is usually the same as that of normal people. The most important thing is that early detection and early treatment are needed.
Unfortunately, due to the lack of awareness of hemophilia in China, the age of diagnosis tends to fall back a lot.
And in the United States, which is an ocean away from us, according to data released by the US Centers for Disease Control and Prevention: The median age of patients with mild hemophilia at diagnosis is 36 months, and the median age of patients with severe hemophilia is 36 months. The median age at diagnosis was 1 month.
Haemophilia is incurable and it is lifelong, which also means suffering fromPatients need lifelong medication, and more than 90% of the cost of hemophilia treatment is on the use of clotting factor drugs.
It’s like a car that leaks oil. If you want to drive smoothly, you need to fill up the car in advance again and again before the oil reaches the bottom.
The same is true for people with hemophilia, who need to constantly supplement the body with clotting factors, which also increase with the patient’s height and weight.
The problem is that due to the high cost of these drugs, only 26.8% of patients take preventive treatment.
According to public treatment: The annual treatment cost of hemophilia patients is about 288,000-312,000 yuan, which is a huge sum for ordinary families. expenses?
Even without this type of treatment, antibody suppression therapy is limited
About 10%-30% of patients often have inhibitory antibodies to coagulation factors. Once antibody therapy fails, it may mean that all coagulation factor treatment methods on the market have been known. There will be an embarrassing situation where there is no cure.
After the antibody appears, the patient cannot continue to use coagulation factors to stop bleeding, which will affect the daily functioning of the body.
Fortunately, since 2004, the national government has also introduced various policies to include products for the treatment of hemophilia into the medical insurance, especially as children’s preventive treatment.
It will also be accompanied by some problems, such as the inconvenience of medical insurance reimbursement.
Especially during the lockdown and management during the epidemic, hemophilia patients could not go to the hospital to buy medicine, and could only be told to go to the pharmacy to buy medicine, but this needs to be paid in advance, which will lead to some financially limited patients facing Risk of discontinuation.
In addition, some patients are faced with the fact that local hospitals cannot prescribe relevant medicines, and some hospitals consider the proportion of medicines required, so they cannot purchase such expensive medicines, and patients can only go to other places to seek medicines.
So this also reflects a problem —–
Medical insurance cannot simply solve the medical burden of patients, but a multi-level medical insurance system needs to be established.
References:
【1】Wu Runhui. (2012). Diagnosis and treatment of hemophilia in children. Clinical Journal of Practical Pediatrics, 27(15), 1214-1216.
【2】Wang Jiankun, Huang Jinxiong. Status and progress of diagnosis and treatment of hemophilia [J]. Chinese and Foreign Medical Research, 2021,19(09):191-194.
[3] Qu Yanji, Yin Huan, Pang Yuanjie, Nie Xiaolu, Dong Peng, Zhan Siyan. A systematic review of the treatment status and economic burden of hemophilia patients in mainland China [J]. Evidence-based in China Medical Journal, 2013, 13(02): 182-189.