An interdisciplinary team approach, in which the treatment can be individualized to each patient and his or her family and tailored to the severity of the disease, is most beneficial to the patient with epidermolysis bullosa (EB). In the Netherlands, the Center for Blistering Diseases in Groningen provides a large interdisciplinary EB team that performs at a high level to provide the best EB-patient care for both children and adults. The possibility of rapid diagnosis, the efficient carrousel-like clinics, and the continuity in care are typical elements of the Dutch method of delivering interdisciplinary EB care.
Epidermolysis bullosa (EB) is an umbrella term for a group of rare genetic blistering skin disorders characterized by blister formation, from birth on, in response to minimal trauma or friction. Fragility can also involve the internal mucosa and the eyes in some forms of EB. The symptoms experienced by patients with EB vary, but blistering and pain are common symptoms to all types of the disease. This chronic skin blistering affects the physical, personal, emotional, and socioeconomic aspects of patients’ life; it is no wonder that EB has a severe impact on the quality of life and impairs the health status in most patients. EB is one of the most devastating chronic diseases known to mankind. Because of the myriad of medical and nonmedical complications that patients with EB endure, all patients and their families should be evaluated in a specialized center familiar with the many complications of EB. Obviously, medical services for people with EB demand a multidisciplinary approach.
Aims of the Dutch interdisciplinary EB team
In many countries medical services have a large multidisciplinary EB team, headed by a dermatologist. The Dutch EB service in the Center for Blistering Diseases in the University Medical Center, Groningen was established in 1990 and services both children and adults. The EB service, including all EB team activities and the nurse coordinator’s salary, is funded by the university medical center from its standard academic care budget because it is recognized as a top reference service. Not all expert centers experience a comparably funded, convenient situation.
In addition, Dystrophic Epidermolysis Bullosa Research Association (DEBRA) Netherlands funds an outreach service with an EB social worker. Home outreach nurses are lacking in this program; the hospital-based nurse specialist will visit a patient at home on occasion. Thus, the EB service in this small country is hospital based.
The aim of the interdisciplinary EB team is to provide excellent, comprehensive care and treatment for patients with EB, to help in the process of managing the condition and enhancing comfort and patient autonomy. One of the activities of the EB team is to make early and precise diagnosis and thus be able to inform the patients in the first weeks about prognosis and treatment. An important part of the Dutch EB center is its diagnostic and research laboratory that provides routine diagnostics, including immunofluorescence antigen mapping, electron microscopy, and mutation analysis. In addition, the EB team aims to provide state-of-the-art medical advice and treatment, both during hospital admissions and EB-clinic visits, and in the community. Finally, one of the goals is to conduct scientific research.
Vision of the interdisciplinary team
The vision of the team is to work patient-centered, that is, to acknowledge the situation of the patient and to tailor the treatment (age appropriate) to both the severity of the condition and the symptoms and problems experienced. When undertaking treatments the EB professional negotiates with the patient over what is acceptable to him or her and what the professional feels able to undertake within the bounds of professional accountability, always remembering that the patient is also an expert in his or her own condition. All professionals involved in the treatment of EB can participate in the International EB forum ( http://www.internationalebforum.org ), to consult, to educate, or to inform medical professionals worldwide about the best possible care for patients with EB. There are good links with other EB centers in Europe and other continents. This international communication and cooperation is very helpful in successful delivery of care.
Vision of the interdisciplinary team
The vision of the team is to work patient-centered, that is, to acknowledge the situation of the patient and to tailor the treatment (age appropriate) to both the severity of the condition and the symptoms and problems experienced. When undertaking treatments the EB professional negotiates with the patient over what is acceptable to him or her and what the professional feels able to undertake within the bounds of professional accountability, always remembering that the patient is also an expert in his or her own condition. All professionals involved in the treatment of EB can participate in the International EB forum ( http://www.internationalebforum.org ), to consult, to educate, or to inform medical professionals worldwide about the best possible care for patients with EB. There are good links with other EB centers in Europe and other continents. This international communication and cooperation is very helpful in successful delivery of care.
Rapid diagnosis
Newborns suspected of having EB are immediately referred to the children’s hospital of the University Medical Center Groningen and admitted to the neonatal ward or intensive care unit. Diagnosis is obtained by writing down the family history, physical examination, taking skin biopsies, collecting blood samples of the newborn and parents for DNA testing, and physical examination, taking medical photographs. Two punch biopsies (2 and 4 mm) are taken from a fresh blister (<24 hours); secondly, another 2 biopsies are taken from the intact skin of the inner aspect of the upper arm. The 2-mm biopsies are fixed in 2% glutaraldehyde and analyzed by electron microscopy; the 4-mm biopsies are snap frozen in liquid nitrogen to conduct rapid IF antigen mapping. Within 24 to 48 hours, diagnosis is obtained and informed to the parents. During the hospital admission, parents are provided with accommodation and learn, step by step, to perform dressing changes and to care for their child. In the meantime the specialist nurse organizes all the care at home, which is provided by a community nurse, with or without the parents. On average, after 1 week of support, instruction, training, and information about diagnosis, prognosis, and care, the parents and their child are discharged.
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