A 4-year old patient was endorsed under my care during the 8 hour shift. The patient has been diagnosed with NEMO syndrome with a history of streptococcus pneumonia meningitis at the age of 1. NEMO syndrome has an alternative name of X-linked anhidrotic ectodermal dysplasia with immunodeficiency. It affects multiple organs and presents with vesiculo-bulluos lesions with erythma with linear arrangement on the extremities and lateral aspects of the trunk. These erythematous lesions evolve into verrucous lesions after weeks to month. These lesions are then followed by atrophy or depigmentation.
The immunodeficiency causes the patient to develop pus-forming bacterial infections early in life. As they get older, they have higher risk to become susceptible to mycobacterial infections. The patient has resultant blindness, deafness and severe developmental delay. He was admitted due to the worsening perirectal sore following the hospitalization 13 days ago for inpatient and outpatient treatment with antibiotics. The perirectal open sore is one of the manifestations of NEMO syndrome. It is located on the patient’s left buttock which was first noticed by the patient’s mother.
The patient was observed to be more fussy and possibly having slightly increased seizure activity, mild gingival bleeding and cough. It is noted that the patient exhibits the symptoms of NEMO mutation with immunodeficiency specifically frequent serious infections and poor immunity. Thus, the return of service (ROS) is the open wound located in his left buttock. The patient doesn’t have the ability to perform self-care or self-control activities appropriate for age. There was a verbalization of the patient who wishes there was something to do following his admission to the hospital.
Clinical Log – 2 – Objective The patient has an altered physical growth, negative mood/responses and restlessness. He has signs of immunodefiency as manifested by poor wound healing, scaly skin, and severe developmental delay. Nursing Diagnosis My nursing diagnosis on my patient having NEMO syndrome are as follows: Impaired skin integrity related to perirectal open sore, delayed growth and development related to immunodeficiency, deficient diversional activity related to bedridden and fatigue and bowel incontinence related to medication