Nowadays, patients with EB disease

EB disease is one of the genetic and inherited diseases that occurs with consanguineous marriages. The suffering of patients with this complication is hard and unbearable. The skin of these patients is affected by painful wounds and blisters and constantly needs dressing, care and maintenance. The high costs these families incur are staggering.

“Butterfly” or “EB” is a special and painful disease that is caused by a genetic mutation in humans and will accompany the patient from birth to the end of life.

Epidermolysis bullosa, abbreviated as “EB”, is an inherited disease of the skin tissues that causes blisters on the skin and mucous membranes. It is known as a “butterfly”.
There is no cure for this disease and it will always be on the patient’s body, unless it can be increased and its difficulties reduced in various and costly ways.
The wounds of butterfly patients make life more difficult for these patients day by day, and inevitably other family members are also affected by this disease.
The prevalence of this disease is hereditary and one in 50,000 people and its severity is from mild to fatal, so that it may destroy many sensitive tissues of the body. “EB” or “butterfly” does not belong to any particular ethnicity and occurs in all parts of the world and affects men and women equally.
Many children are born to butterfly parents who have had consanguineous marriages, which is why doctors recommend that those who plan to consanguineously marry have genetic tests.
The skin of EB patients is so sensitive and fragile that the slightest scratch or injury can cause painful blisters and many infections.
The cost of dressing the wounds of an “EB” patient is more than three million tomans per month, and if the patient needs medication or proper nutrition, the cost is more than six million tomans and about 80 million tomans per year, most of which The family cannot afford this amount.

According to the officials of the “EB” house, it is estimated that there are about 800 to one thousand people with this disease in the country, of which 540 patients have been identified and files have been filed so far, and according to the available statistics, 25 “EB” patients He died in the year 95.
Hojjat-ul-Islam and Muslims Seyed Hamid Reza Hashemi Golpayegani, CEO of EB, says about this disease: 99% of patients are licensed Family marriages To prevent the spread of the disease in the country, couples should be consulted and genetically tested before marriage.
He added: “People avoid” EB “patients because of their physical condition and the wounds on their hands, face and body, and this negligence of the people in the community causes them to suffer from mental problems.” And become psychic.
Golpayegani emphasized that this disease is not contagious at all and can not be transmitted to other people under any circumstances. And that is why most of them do not go to school and have to stay at home for the rest of their lives.
He, who is well acquainted with this disease because of his son’s illness, described the many problems that these patients are struggling with, and added: “In terms of recognizing this disease and how to deal with it.” Patients in the country There is a cultural poverty that should be made known to the people through the efforts of the Ministry of Health and the country’s media.
Referring to the establishment of the “EB” house to follow up and solve the problems of these patients and their family members, he said: “So far, cases have been filed against 540 patients with this disease, and unfortunately, 25 of them died last year.”

Golpayegani added: “It is estimated that there are about 800 to one thousand patients with” EB “in the country, and we are trying to file cases for the rest of them in the” EB “house.”
He said: “Thalassemia, dialysis and hemophilia are among the special diseases, but unfortunately EB disease is not yet in the category of special patients and the main demand of all these patients from the President and the Minister of Health is that EB is also among the diseases.” Be specific diseases.
Regarding the treatment costs of these patients, he said: The cost of dressing this disease is three million Tomans per month, because each dressing sheet of this disease, which is the size of an A4 paper, costs 250 thousand Tomans, which must be replaced after 48 hours. The patient needs 20 sheets of these dressings per month.
The CEO of EB House says: “This is just the cost of dressing, and the costs of medicine, nutrition and other materials they need also put a heavy financial burden on families, which without surgery costs about 80 million tomans per year.” Becomes.
He said: “Unfortunately, despite the high costs of this incurable disease, none of the drugs of this disease are among the insured drugs, which provides many problems for patients and their families.”
He pointed out that the eBay house in 1995, with the help of some donors, was able to pay about six million tomans to the families of 540 identified patients, adding: “Last year, we also spent 10 billion tomans to dress patients, most of whom This money was provided by the Ministry of Health.

Doctors say that there is no definitive cure for EB disease, and only daily care of wounds and dressings and prevention of blistering and blistering skin, treatment of infection and treatment of nutritional problems is the only way to cope with this disease.
According to them, the disease appears as a result of a lack of protein in the layers of the skin and begins with a disorder and involvement of the immune system and occurs shortly after birth in infants with this disease in the form of blisters and severe wounds.
During the course of the disease, the body loses the ability to exfoliate, and this complication causes brittleness and excessive dryness and elongation of the skin, which breaks as soon as it comes in contact with a solid object or even a small amount of pressure, and wounds from the surface layers of the skin. From the depths of the body, there are painful and indescribable wounds, so much so that EB patients suffer from pain. Burns They know the third degree is comparable.
The disease affects not only the appearance of patients, but also the internal tissues and mucous membranes of the body from the inside, and the esophagus, trachea, colon and small intestine of these patients are often full of wounds and prevent proper eating and breathing, as well as proper excretion.
As mentioned earlier, EB disease is a genetic disease and Hereditary Therefore, if a person in the first, second and third generations has the disease or has had a consanguineous marriage, there is a possibility of developing the disease.
There are 31 types or types of this disease and it is classified into 4 groups to which 17 genes are related and these genes are considered as the cause of the disease. If the gene type is predominant, the disease is inherited from only one of the parents who had the disease, and the probability of having a child with EB is 50%.

In the recessive form, the disease gene is inherited from both parents, in which case a child from 4 pregnancies is more likely to give birth to a baby with EB characteristics.
“EB” can also be caused by a mutation in a gene that occurs during the formation of a germ cell egg or sperm in one parent.
People who are genetically and congenitally infected with the disease may also have food allergies, while patients should not have food allergies because these patients have major eating problems.
Although doctors believe that there is no definitive cure for this disease, but with gene therapy, this complication can be diagnosed and the disease can be controlled by transplanting brain and bone and protein therapy.
Research also shows that in the near future, fibroblasts can be injected into the wound and gene therapy can reduce the severity and progression of the disease.
In any case, the best help that the government and the relevant officials can give to these patients and their families is to include EB among certain diseases so that they, too, can use government assistance and benefits to tolerate this patient. .
However, it is said that the officials of the Ministry of Health are trying to make EB disease one of the special diseases with the help of the government and the parliament, in order to cure some of the pain of these people.

Source: Iran Economist

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