Butterfly Skin

There are many kind of deseases that affect people in all parts of the world. One of them is Epidermolysis bullosa, a skin disorder that is genetically transmitted and manifests itself at birth or in the first months of life.

The desease

The skin is filled with blisters, ulcers and wounds, especially in the mucous membranes. This happens with the slightest touch or blow. Internal wounds often appear, causing a closure in the esophagus. In return, this causes a loss of weight because they are not being able to digest food.

Fundación Debra © Solkes

The skin of those affected is fragile, weak, extremely sensitive and extremely vulnerable. With the slightest contact the skin is detached, causing wounds and / or blisters that look like a big burn.

Over the past 15-20 years, 13 major genes responsible for the majority of cases of EB have been identified. The genetic errors in EB result in defects in the proteins that adhere the epidermis to the dermis.

Solkes: What exactly is Epidermolysis Bullosa?

Dr. Liliana Consuegra Razzani, director of the Debra Foundation: It is a rare, genetic, non-contagious disease that involves the formation of blisters and wounds from minimal friction, trauma or friction of the skin and mucous membranes. The cause is genetic, incomplete or absent proteins that fix the layers of collagen in the skin and mucous membranes, generating extreme fragility.

Solkes: What is the cure for this illness?

Dr. Liliana Consuegra Razzani, director of the Debra Foundation: Until now there are no curative treatments, there are still inconclusive research projects that seek the cure. At the moment, all we have are “methods” to prevent and treat wounds with very difficult healing throughout life.

The disease affects people all over the world, men and women. In Colombia there are 60 registered patients in different areas of the country and it is believed that there should be more than 250 cases.

The work in Colombia

While Consuegra Razzani volunteered at Hogares Luz y Vida, a place that welcomes abandoned children in special health conditions, she met 2 little sisters with butterfly skin.

So, she wanted to look for alternatives to alleviate the pain, and so she found Debra International and then Debra Chile.

Fundación Debra © Solkes

After a few months more patients with this extremely rare and painful condition came to her, looking for a doctor who could answer questions that the health system could not answer or address.

With this in mind, she had to do a lot of research, training and finding resources to get special dressings that kept clothes from sticking to his skin. After a few years Debra Chile asked her to lead the Debra Colombia project to help relieve Colombian children with this condition and caregivers who were looking for help in Debra from other countries.

And, since this is such a delicate issue, we gave ourselves the task of talking with people who are dedicated to helping those who suffer from this disease.

That’s how we came to the Debra Foundation. The foundation is a non-profit organization affiliated with Debra International that provides interdisciplinary and specialized support to patients with epidermolysis bullosa or butterfly skin.

We had the opportunity to speak with Dr.Liliana Consuegra Razzani who is the director of this foundation in Colombia.

It is important to note that Debra was created in the United Kingdom in 1978 by the mother of a girl with this condition called Debra. Subsequently the meaning is established by the initials of Distrofic Epidermolysis Bullose Rare Asociation.

 

Relieving pain

The purpose of the foundation is clear: to ensure that patients have days with less pain. This is to be achieved through comprehensive specialized and interdisciplinary medical care programs, school-social inclusion projects, psychological and legal support to guarantee their fundamental rights.

Fundación Debra © Solkes

This information left me uneasy because patients not only suffer a terrible illness throughout their lives but are also rejected.

The truth is that they suffer from social exclusion, from the eyes of people, to the indolence of some doctors who do not give the attention they require or the limitations for access to school and work inclusion.

Solkes: What is the importance of the work you do?

Dr. Liliana Consuegra Razzani, director of the Debra Foundation: We make sure health teams, state entities related to health, private companies and individuals become sensitive and support projects that allow days with less pain to patients with a more painful condition that exists and that has no cure. and what is worst tis takes place in a health system that does not know about the treatment of the disease in many aspects (from care to coverage of treatments).

Solkes: What is the most difficult thing you have experienced so far?

Dr. Liliana Consuegra Razzani, director of the Debra Foundation: Finding that patients get complications or die due to lack of attendance from the health system.

Working with people affected by this disease is not an easy task but it is full of challenges, tears, laughter and satisfaction.

Fundación Debra © Solkes

The first thing they do is train patients, caregivers and health teams to prevent and treat wounds properly. Likewise, the medical requirements (specialized healing material – nutritional supplements) are provided and / or we give advice so that the health system of the relevant coverage.

On the other hand, specialized and interdisciplinary medical attention is given in regional meetings, where patients, parents and caregivers share experiences. Finally, a surgical and intra hospital accompaniment is given to patients who require hospital admissions.

The reality

On legislation issues there are many gaps. It is not ideal. A significant amount of changes must take place.

It is a fact that the orphan diseases law exists but has not yet been applied. However, in the last year the Debra Foundation Colombia has achieved joint work with the Health Ministry and the Health Superintendence and little by little we are making progress.

Fundación Debra © Solkes

There are major limitations such as the lack of knowledge of health teams about this condition and its treatments.

On the other hand, the current regulations say that the creams that patients require for treatment are considered “COSMETIC” and therefore the health system does not cover them. Further more, some bandages that patients require are not in the country and therefore some doctores requests are not covered.

Solkes: What are, according to you, some of the biggest problems we have?

Dr. Liliana Consuegra Razzani, director of the Debra Foundation: The indolence and ignorance of some doctors, the gaps in the legislation and the lack of resources to guarantee biopsychosocial well-being of patients with butterfly skin in our country.

 

The results

So far they have been able to support patients with the necessary medical attention, they have provided psychological support and support in school and work inclusion projects.

Likewise, they have sent kits with healing material and nutritional supplementation. Since 2009, more than 80 patients with this condition in Colombia have benefited from these programs since then.

Fundación Debra © Solkes

Although there are many advances, the Foundation is aware that they must do more. And, perhaps what they need most is to make orphan disease legislation effective for patients and caregivers.

People are needed so we can help. People who provide their time, knowledge and experience for the common good of patients and their families.

I conclude this interview with some words that resounded in my heart and mind. The community must avoid judging with looks and having exclusive attitudes that make the pain more intense. Health teams should look for more information about epidermolysis and try to put themselves in the shoes of patients and their families. Last but not least, the orphan disease legislation must be effective. There is no use in a paper if you can not help those who need it. It is necessary to support those who suffer from this disease so that they have days of less pain and can have a decent life. It I all about a decent life for the most vulnerable.

Translated By: Laura Viera A

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