From Evanina Makow, Manager, Debra Espana
First National Epidermolysis bullosa Day -Hospital Niño Jesús - Madrid
14th of January 2009
The first national Epidermolysis bullosa day was organised by the Hospital Niño Jesus (Madrid) together with the governmental research entity CIEMAT and the patient organisation DEBRA Spain.
The mission was to organise a multi-disciplinary EB service for the first time in Spain. Since the clinical manifestations of EB are very diverse since a number of different forms of the condition exist with varying phenotypes and prognosis, 8 patients were selected on the basis of their genetic diagnosis, all of which having distrophic recessive EB and exactly the same mutation.
Currently there is no cure for any of the forms of EB. However, effective symptomatic and preventative treatment is available, and is best managed, shown by countries such as the United Kingdom, by dedicated, multi-disciplinary specialised EB centres.
The objective was three-fold:
- for the multi-disciplinary team to be able to study the different phenotypes within a same genotype and to offer patients the recommendations on the different management options, and, where appropriate, likely prognosis.
- to allow enough time between all members of the multidisciplinary team and patients, to discuss specific problems or concerns and so that patients could have sufficient time and support to make informed decisions about their treatment.
- to set the guidelines for national standards of practice for multi-disciplinary care of EB as well as a national network of EB professionals
The day was organised in the following way, there were 4 groups of professionals, divided, on the one hand, in the dermatology unit into the groups:
- dermatologists and nurses
- paediatricians, nutritionists and haematologists
and in a separate department, the patients would be seen by a dentist and an ophthalmologist. There were in total, in the morning session, about 30 professionals.
Each group had a lead clinician who was responsible for coordinating the activities of his/her team, ensuring good communication between team members and compiling the conclusions of his/her team on each patient.
In addition, 8 of the dermatologists were assigned a given patient and were asked to prepare, for the afternoon clinical session, a presentation about the different recommendations from the different professionals for that given patient.
From 09:30 to 11:30 4 patients were seen in four separate boxes, and the four groups of professionals rotated between the boxes in order to avoid the patient having to move (they are in each box without clothes nor bandages).
Simultaneously, the other 4 patients were being seen by the ophthalmologist and dentist.
At 11:30 there was a half an hour break for the patients to get dressed and for the other four to get undressed.
From 12:30 to 14:30, the group of 4 patients switched around, allowing all patients to see all professionals.
In each box there was a volunteer (clinician or researcher) who took note of all recommendations on a previously made template with the different disciplines (recommendations from nurses, dermatologists, surgeons, etc). These volunteers did not move from their given box, to avoid doctors having to loose time writing things down.
At 15:00 all professionals as well as patients convened for a light lunch, where patients could talk in a more relaxed atmosphere with their doctors.
At 16:30, an afternoon clinical day, only open to professionals (those of which attended the morning session as well as other professionals interested in assisting) was celebrated in the hospital.
A total of 80 professionals attended the afternoon session which involved seven 20 minute presentations on issues and complications concerning EB, such as diagnostic, treatment, surgery, etc, and then the 8 leading dermatologists presented each of the patients separately, with their different characteristics and the different comments from the professionals, leading to great discussion.
At the end of the session, an internet Spanish network for professionals working with EB was set up and has been used ever since to consult questions, treatments, etc.
All patients were given an evaluation form at the end of the day and were later sent to their home a report with all of the recommendations. From the evaluation form, patients communicated their satisfaction with having had a multi-disciplinary attention as well as having the necessary time dedicated to their questions and doubts.
Ichthyosis Awareness Month 2019 : more than ever needed