A case study of the emotional sequelae of pediatric Drug-Rash-with-Eosinophilia-and-Systemic-Symptoms (DRESS) syndrome on the family
Abstract number :
1.349
Submission category :
18. Case Studies
Year :
2016
Submission ID :
188261
Source :
www.aesnet.org
Presentation date :
12/3/2016 12:00:00 AM
Published date :
Nov 21, 2016, 18:00 PM
Authors :
John J. McGinley, Montefiore Medical Center / Albert Einstein College of Medicine, Valley Cottage, New York; Elissa G. Yozawitz, Montefiore Medical Center / Albert Einstein College of Medicine, Mamaroneck, New York; Sheri M. Escalante, Montefiore Medical
Rationale: DRESS syndrome was introduced by Bocquet et al. (1996) in an attempt to decrease the ambiguity of "hypersensitivity syndrome". Symptoms of DRESS typically include fever, with rash or lymphadenopathy and additional organ system involvement, with eosinophilia often present. Although DRESS has been characterized as "rare", anti-epileptic drugs remain the most implicated. While the impact of DRESS on Post-Traumatic Stress Disorder (PTSD) symptoms has been considered in adults, there are no data on the emotional sequelae of DRESS in children (or their parents). Methods: Case-study of a nine-year old girl with a three-year history of epilepsy who was diagnosed with DRESS syndrome secondary to initiation of carbamazepine (CBZ) four-weeks earlier. She had a widespread pruritic rash without fever. The family's acute worries included concern that the rash would not go away and that that seizures would return. CBZ was discontinued, and she was treated with lorazepam with subsequent initiation of zonisamide once the rash had resolved. She was subsequently referred for neuropsychological assessment eight-weeks later related to a past history of emotional concerns (i.e., selective mutism). Results: On neuropsychological evaluation, cognitive patterns of strengths and weaknesses were not believed to have any association to her history of an immunologically mediated allergy reaction. In contrast, adjustment factors associated with the stresses of a life-threatening condition were very germane. Patient's mother did not endorse her daughter as having any clinical, academic or adaptive concerns on the Behavior Assessment System for Children, 2nd Edition (BASC-2, Parent Form). Likewise, the patient did not endorse any elevated anxiety concerns on the Revised Children's Manifest Anxiety Scale, 2nd Edition (RCMAS-2), although her pattern of "yes"/ "no" responses was consistent with "clinically elevated" Defensiveness (i.e., a reluctance to acknowledge minor faults that most children would concede if they responded in an honest, undefended manner ?" consistent with a "yes" to the item, "I am always right"). Almost a year post-evaluation (and more than a year post-DRESS), her mother became understandably "very concerned" when her daughter developed a "rash" in June of 2016 that was determined to be "heat rash". The residual emotional sequelae of the DRESS diagnosis rekindled fears and worries for DRESS re-occurrence. The manner in which the family may respond to future occurrences of rash will likely continue to be colored by past events. Conclusions: The physical impact of DRESS syndrome tends to overshadow considerations for the undercurrents of emotional factors. The value and importance of the present case study comes through the raised awareness of how emotional residuals associated with DRESS can impact upon families. This included difficulty with the acknowledgement of emotional concerns, as suggested by the child's "clinically elevated" Defensiveness that may have been present pre-morbidly, as well as a predisposition for the parent to be hypersensitive to manifestations that remind her of past emotional trauma (i.e., the possible loss of a child). Funding: None
Case Studies