Parents' responses to their child's diagnosis of neurofibromatosis 1

Abstract

Eighteen unaffected families with an affected child were interviewed in their homes to chronicle the experiences of parents in receiving their child's diagnosis of neurofibromatosis (NF) 1. Families were recruited through NF support groups and the Genetics Departments of two metropolitan hospitals in Northern California. Characteristics of disclosures were often at variance with suggestions made in recent years for the giving of "bad news." Disclosures typically were made "helter-skelter" during regular examinations. Parents in 16 of the families described shock, upset, and subsequent depression as their responses to the diagnosis. The overriding issues, which dominated in the disclosure, were the uncertainty of the condition, the possibility of many diverse symptoms, and its historic misdiagnosis as "The Elephant Man's Disease." However, physicians' attention to the setting and style of disclosure, imparting appropriate and positive information, allowance of additional time for careful explanation, and rescheduling a follow-up appointment, may be able to more effectively assist parents in receiving and more positively adapting to their child's diagnosis. How would I handle it? How would I deal with it? With help I finally came to realize that I shouldn't have to deal with it. Matt would have to deal with it. And I had to separate those two things. That's what gave me my sanity back, understanding that it is really going to be his problem in life, if it was going to be a problem. And all I could do is guide or support or help, but it really is not anything I could change for him. That's when I got better and started accepting it. It took between 6 and 9 months before I could talk about it without crying. I stopped wearing mascara when this happened."A substantial literature documents the emotional reactions of parents to the diagnosis and disclosure that their child is physically or mentally different [for detailed reviews, see Bristor, 1984;Blacher, 1984;Fortier and Wanlass, 1984;Ablon, 1988; and for cross cultural reactions, see Krauss-Mars and Lachman, 1994]. Concordantly clinicians are giving attention to factors in the setting and process of disclosure of "bad news" and characteristics of the family that may contribute to family response, acceptance of, and adaptation to their child's condition. Current guidelines derived from suggestions of physicians and patients emphasize the importance of parents being told closely following birth; the physician's sensitivity and effectiveness in disclosing the nature of the child's condition by planning the setting; assessing the family's background, knowledge, and experience and choosing strategies suited to the family's particular situation; providing clear and detailed information on the condition in a positive fashion, with information on specialty referrals and community resources included; physicians allowing sufficient time for adequate communication, questions, and ventilation; and arranging for both parents or the parents' support network to be present [Turner...

Communicating With Children and Families: From Everyday Interactions to Skill in Conveying Distressing Information

“…When appropriate information is not provided and this style of communication and relationship does not occur, the bitterness can linger for years. [12][13][14]32,41,42 Physicians who are empathic, well informed, and honest are a source of strength for parents, particularly those struggling to adapt to a difficult situation.…”

“…As an example, although the diagnosis of neurofibromatosis type 1 may not seem to the practitioner to be bad news, the variability of the outcome and the lack of predictability of the disease make this diagnosis very difficult for parents. 13 Similarly, the need for unanticipated blood tests for a needle-phobic child or the disruption of an unexpected overnight hospital stay, the need to take medications for the rest of one's life for a chronic condition, and many other common occurrences are bad news for families. Greater attention to the empathic delivery of bad news will result in improved skills when the stakes escalate, as in terminal conditions.…”

“…[1][2][3][4][5][6][7][8][9][10][11][12][13] The practicing clinician's ability to communicate openly and with compassion is essential for effective and efficient routine health care; this ability becomes a vital lifeline for parents and children confronted with lifealtering and sometimes life-ending conditions. [13][14][15][16] The purpose of this report is to provide research-based and practical guidance to enable effective communication with pediatric patients and their families in a number of common settings and situations.…”

Neurofibromatosis: part 2 – clinical management

“…Since the 1980s, some studies emphasized the need to better understand NF1 and its psychosocial consequences, motivated by its unpredictability, the uncertainties and little control over the natural course of the disease, along with prejudice, all of which could lead to social isolation, anxiety, fear and doubts about a possible professional career. These consequences can affect both patients and their families 37 .…”

“…First, by facilitating both the access and transference of information about the disease between healthcare teams and NF1 affected individuals and their families, monitoring the process through which they assimilate and take into account such information 37 . Second, by helping the development of more efficient strategies to cope with problems, related or not to NF1, encouraging the reduction of negative feelings and experiences in accepting the disease.…”