Antonio López González, Teresa Pérez López, Modesta Mojón Barcia, María Luz Sordo Castro, Manuela Pérez Freire
Diploma in Nursing.
Complexo Hospitalario Universitario A Coruña (Spain)
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A 50-year-old woman diagnosed at birth with Recessive Dystrophic Epidermolysis Bullosa with involvement of two thirds of the skin surface and bullous manifestations since the neonatal period.
At 45 years of age, she was referred to nephrology clinics for follow-up due to progressive increase in blood creatinine levels.
A renal biopsy showed data compatible with tubulointerstitial inflammatory disease with immunocomplex deposits.
Conservative treatment with corticosteroids was established with minimal analytical improvement.
One year later, she was diagnosed with stage V Chronic Renal Failure (Ccr 10 ml/min and Cr 4.5 mg/dl).
Arteriovenous fistula was performed in the right upper limb and included in a chronic hemodialysis program.
Personal Background
• Related to Epidermolysis Bullosa Dystrophic Recessive: Chronic anemia with ferropenic component (follow-up by external hematology clinics to treatment with transfusions, intravenous iron and erythropoiesis piecemeal nutrition),
Probable Zenker's diverticulum).
• Other relevant backgrounds: Recurrent urinary tract infections, parathyroidism and pertrochanteric hip fracture due to accidental fall.
Family History
Padre, a healthy carrier of the gene causing the disease, died at 59 years due to an acute myocardial infarction.
Madre, a healthy carrier of the gene, has neurological involvement caused by a cerebrovascular accident.
Hermana does not have any remarkable history and there are no genetic data submitted to and determined by the presence of the gene that causes Epidermolysis Bullosa because, on request, no studies have been conducted.
Nursing Assessment
The nursing assessment was performed using the "Guidelines for assessment of patients with chronic renal failure undergoing hemodialysis"7.
This guide, developed in our unit, uses tools such as NANDA Taxonomy and Classification of Outcomes (NOC) and is based on Marjory Gordon functional patterns model.
Its objective is to share the use of common nursing language to improve the quality of our care.
The evaluation process was divided into 13 domains, of which we found alterations in the following:
• Domain - Nutrition: compromised nutritional status (M.I.C. 15.23).
Protein-calorie malnutrition as a result of Bullous Epidermolysis and marked by renal failure.
It does not require severe fluid restriction because it maintains residual diuresis of approximately 1.5 L.
• Domain - Intestinal Elimination: decreased frequency and hard stools
• Self-medication regimen: verbal reports of not having a restorative sleep.
Fatigue manifestations which do not represent an inability to perform activities of daily living.
It requires bas as technical help for effective ambulation.
• Domain - Safety/Protection: uses walking aids.
It presents lesions and blisters on the whole body surface and oral mucosa.
• Domain - Comfort: manifests pain related to bullous lesions and controlled with analgesia and physical measures.
Nursing Diagnoses
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Corresponding address: Antonio López González C./ As Xubias no 84 15006 A Coruña E-mail: angonzalez@ser.
