Blowing away the pain: a technique for pediatric pain management.

Painful medical procedures in childhood may have long-term negative effects on development and future tolerance of pain. We describe a distraction technique for pediatric pain management that involves "blowing away the pain." This technique may be used for acute pediatric procedures as well as for patients with chronic pain.

Neuropathic pain referrals to a multidisciplinary pediatric cancer pain service.

Neuropathic pain (NP) in children with cancer is not well characterized. In a retrospective review of patient data from a 3.5-year period, we describe the prevalence of NP and the characteristics, duration of follow-up, and interventions provided for NP among patients referred to a pediatric oncology center's pain management service. Fifteen percent (66/439) of all referrals to our pain service were for NP (56/323 patients [17%]; 34 male, 22 female). The NP patient group had 1,401 clinical visits (778 inpatient visits [55.5%] and 623 outpatient visits [44.5%]). Patients with NP had a significantly greater mean number of pain visits per consultation (p = .008) and significantly more days of pain service follow-up (p < .001) than did other patients. The most common cause of NP was cancer treatment rather than the underlying malignancy. Pharmacologic management of NP was complex, often comprising three medications. Nonpharmacologic approaches were used for 57.6% of NP referrals. Neuropathic pain is less frequently encountered than non-NP in children with cancer; nevertheless, it is more difficult to treat, requiring longer follow-up, more clinical visits, complex pharmacologic management, and the frequent addition of nonpharmacologic interventions.

Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa.

BACKGROUND: Inherited epidermolysis bullosa (EB) is a cluster of rare, genetic skin and mucosal fragility disorders with multi-system and secondary effects, in which blistering and erosions occur in response to friction/mechanical trauma. Considering the incurable and potentially life-limiting nature of the condition and the challenges posed by its symptoms, a palliative approach to EB-related care is necessary. However, knowledge and experience related to the provision of EB palliative care is minimal. Evidence-based, best care guidelines are needed to establish a base of knowledge for practitioners to prevent or ease suffering while improving comfort at all stages of the illness, not just the end of life. METHODS: This consensus guideline (CG) was begun at the request of DEBRA International, an international organization dedicated to improvement of care, research, and dissemination of knowledge for EB patients, and represents the work of an international panel of medical experts in palliative care and EB, people living with EB, and people who provide care for individuals living with EB. Following a rigorous, evidence-based guideline development process, the author panel identified six clinical outcomes based on the results of a survey of people living with EB, carers, and medical experts in the field, as well as an exhaustive and systematic evaluation of literature. Recommendations for the best clinical provision of palliative care for people living with EB for each of the outcomes were reached through panel consensus of the available literature. RESULTS: This article presents evidence-based recommendations for the provision of palliative healthcare services that establishes a base of knowledge and practice for an interdisciplinary team approach to ease suffering and improve the quality of life for all people living with EB. Any specific differences in the provision of care between EB subtypes are noted. CONCLUSIONS: Because there is yet no cure for EB, this evidence-based CG is a means of optimizing and standardizing the IDT care needed to reduce suffering while improving comfort and overall quality of life for people living with this rare and often devastating condition.

Intravenous Ketamine Infusion as an Adjunctive Pain Treatment for Erythromelalgia: A Pediatric Case Report.

Erythromelalgia is a rare neurovascular pain condition characterized by erythematous, warm, and painful extremities. Symptoms are exacerbated by heat and relieved by cooling. Treatment is challenging and focuses on symptom control with various medications and therapies targeted toward eliminating destructive cooling behaviors. This pediatric case was notable because the patient's pain dramatically improved after a short-term, low-dose ketamine infusion, allowing her to finally wean off detrimental cooling practices of her extremities. Intravenous ketamine has rarely been described as an adjunctive analgesic strategy for erythromelalgia.

Supporting sexuality for people living with epidermolysis bullosa: clinical practice guidelines.

This article presents evidence-based Clinical Practice Guidelines (CPG) for the provision of healthcare services to address sexuality for people living with epidermolysis bullosa (EB). Currently, a lack of EB-specific research limits these services to sexual health assessment and intervention strategies designed for the general population. Due to the unique challenges of EB, a rare skin-fragility condition causing blistering responses to minor skin trauma and other systemic and secondary complications, condition-specific strategies are needed to support people with EB in achieving valued sexual lifestyles. This CPG represents the work of an international panel comprised of thirteen members including a medical doctor, nurses, psychologists, a social worker, an occupational therapist, and patient population involvement members living with EB. It describes the development of EB-specific recommendations for two primary domains of assessment and intervention related to sexuality: psychosocial and mechanical. Following a rigorous evidence-based guideline development process, this CPG establishes the first internationally actionable clinical practice recommendations for sexuality-related assessment and intervention for this population. Future research priorities are identified. Supplemental materials included provide additional support to clinicians in developing the necessary understanding and skills to promote equity and efficacy in this care domain.

The incidence of intraoperative awareness in children: childhood awareness and recall evaluation.

BACKGROUND: There is a considerable discrepancy between the reported incidences of awareness under anesthesia in children (0.2%-2.7%). In this prospective, observational, cohort study we evaluated 1) the incidence of awareness during general anesthesia in children across three settings, 2) factors contributing to awareness, and 3) short-term psychological effects of awareness. METHODS: Children (aged 5-15 yr) who underwent general anesthesia were included, and all perioperative data including anesthetic drugs were collected prospectively. Children were interviewed three times postoperatively using a semistructured questionnaire. All cases of possible or probable awareness were discussed with the child's care providers to confirm or refute the memories. Internal consensus among investigators across sites was reached, and these cases and a random selection of others were reviewed by three external reviewers. For the purpose of this study, possible/probable awareness was defined as cases with agreement between the internal consensus and at least two of the three external reviewers. RESULTS: One thousand seven hundred eighty-four children completed at least one interview. Thirty-two cases were coded as possible or probable awareness by at least one entity (i.e., either the internal consensus or one of the external reviewers). Fourteen of these cases met the definition for possible/probable awareness, making the incidence of awareness 0.8%. Six of the 14 children with awareness (43%) remembered feeling scared during their surgery and three (21%) reported hurting. Two children in this group (14%) said they would feel worse if they had to have surgery again, which was not significantly different from reports of children with no recall (15%). None of the children with awareness required psychological follow-up. Endoscopic procedures were associated with a higher risk for awareness (relative risk = 4.5 [confidence interval 1.5-13.6]). CONCLUSIONS: Although 0.8% of children experienced possible/probable awareness in this study, none experienced short-term psychological distress.