Assessing Bulbar Function in Spinal Muscular Atrophy Using Patient-Reported Outcomes.

BACKGROUND: Novel Spinal Muscular Atrophy (SMA) treatments have demonstrated improvements on motor measures that are clearly distinct from the natural history of progressive decline. Comparable measures are needed to monitor bulbar function, which is affected in severe SMA. OBJECTIVE: To assess bulbar function with patient-reported outcome measures (PROs) and determine their relationships with clinical characteristics. METHODS: We recruited 47 non-ambulatory participants (mean (SD) age = 29.8 (13.7) years, range = 10.3-73.2) with SMA. PROs including Voice Handicap Index (VHI) and Eating Assessment Tool-10 (EAT-10) were collected alongside clinical characteristics and standardized motor assessments. Associations were assessed using Spearman correlation coefficients and group comparisons were performed using Wilcoxon rank sum tests. RESULTS: A majority of the 47 participants were SMA type 2 (70.2%), non-sitters (78.7%), 3 copies of SMN2 (77.5%), and using respiratory support (66.0%). A majority (94%) reported voice issues primarily in 8/30 VHI questions. Problems included: difficulty understanding me in a noisy room (87.2%); difficult for people to hear me (74.5%); and people ask me to repeat when speaking face-to-face (72.3%). A majority (85.1%) reported swallowing issues primarily in 3/10 EAT-10 questions: swallowing pills (68.1%); food sticks to my throat (66.0%); and swallowing solids (61.7%). The two PROs were moderately associated (rs = 0.66). CONCLUSIONS: Weaker individuals with SMA experience bulbar problems including difficulties with voice and swallowing. Further refinement and assessment of functional bulbar scales will help determine their relevance and responsiveness to changes in SMA. Additional study is needed to quantify bulbar changes caused by SMA and their response to disease-modifying treatments.

Facial Paralysis and Communicative Participation: The Importance of Facial Symmetry at Rest.

OBJECTIVE: There is a paucity of research devoted to understanding the communication restrictions encountered by facial paralysis patients. We aim to explore the relationship between patient-reported restrictions in communicative participation and objective facial paralysis severity using validated scales of facial movement. METHODS: We performed a pilot retrospective study using a consecutive series of adult patients with a diagnosis of unilateral facial paralysis. In addition to baseline demographics, subjects were evaluated using the Communicative Item Participation Bank Short Form (CPIB), Electronic Facial Assessment by Computer Evaluation (eFACE), and Sunnybrook Facial Grading System (SFGS). RESULTS: Twenty patients were included, 10 (50%) of whom were female with a mean age of 61 ± 13 years and mean duration of facial paralysis of 53 ± 82 months. The mean CPIB score was 14.6 ± 10.0 (range 0-29) and was comparable to scores of patients with conditions known to cause significant communicative disability. The mean eFACE scores were 67.4 ± 29.2, 44.2 ± 30.1, and 73.8 ± 30.0 for the static, dynamic, and synkinesis domains, respectively, with a composite smile score of 58.5 ± 16.9. After adjusting for age, gender, and duration of facial paralysis, significant moderate correlations were observed between the CPIB and the static eFACE domain (r = -0.51, P = .03) and smile composite score (r = 0.48, P = 0.0049), in addition to between the CPIB and SFGS synkinesis domain (r = 0.48, P = 0.04). CONCLUSIONS: Patients with unilateral facial paralysis experience significant limitations in communicative participation. These restrictions demonstrate moderate to strong correlations with objective assessments of facial paralysis and quality of life measures. Communicative participation may be a helpful means of tracking response to treatment. LEVEL OF EVIDENCE: IV.

Promoting effective communication for patients receiving mechanical ventilation.

Communicating effectively with ventilator-dependent patients is essential so that various basic physiological and psychological needs can be conveyed and decisions, wishes, and desires about the plan of care and end-of-life decision making can be expressed. Numerous methods can be used to communicate, including gestures, head nods, mouthing of words, writing, use of letter/picture boards and common words or phrases tailored to meet individualized patients' needs. High-tech alternative communication devices are available for more complex cases. Various options for patients with a tracheostomy tube include partial or total cuff deflation and use of a speaking valve. It is important for nurses to assess communication needs; identify appropriate alternative communication strategies; create a customized care plan with the patient, the patient's family, and other team members; ensure that the care plan is visible and accessible to all staff interacting with the patient; and continue to collaborate with colleagues from all disciplines to promote effective communication with nonvocal patients.

Management of cervical esophageal injury after spinal surgery.

BACKGROUND: Esophageal injury is a rare but catastrophic complication of anterior cervical spine surgery. Cases of esophageal perforation may be discovered intraoperatively, or as late as 10 years after surgery. In the current study we aim to review the principles of care and provide an algorithm that can be employed for successful management of this complex problem. METHODS: We performed a retrospective, Institutional Review Board-approved review of esophageal injuries resulting from anterior cervical spine surgery that were managed at our institution between January 1, 2007 and July 31, 2009. We collected demographic information, perioperative data, and final outcomes. Data were analyzed using descriptive statistics. RESULTS: We identified 6 patients who met our criteria. All patients presented with esophageal leaks, neck abscesses, and osteomyelitis. Similarly, all had been treated prior to transfer, without resolution of their leak. After debridement, removal of hardware, long-term antibiotic therapy, maximization of nutrition, and supportive care, 80% of patients resumed oral intake (median time 66.5 days). Mortality was 16.7%. CONCLUSIONS: Neck exploration with removal of hardware, debridement, and open neck wound management are the basic principles of care. Management is often prolonged and requires multiple procedures; however, with persistence, closure is possible in the majority of patients. Our report serves as a guide for the treatment of this devastating problem.

Pharyngostomy tubes for gastric conduit decompression.

OBJECTIVE: This article illustrates our operative technique for pharyngostomy tube placement and describes our clinical experience with pharyngostomy use for gastric conduit decompression after esophagectomy. METHODS: We retrospectively reviewed patients undergoing pharyngostomy tube placement for gastric conduit decompression after esophagectomy from January 2008 to August 2009. Patients were included if they had a pharyngostomy tube placed at esophagectomy (prophylactic placement) or as a means of decompression after postesophagectomy anastomotic leak (therapeutic placement). We collected operative and clinical data and performed a descriptive statistical analysis. RESULTS: We placed 25 pharyngostomy tubes for gastric conduit decompression after esophagectomy. Eleven were placed prophylactically (44%); the remaining 14 were placed therapeutically (56%) after anastomotic leak. Prophylactic pharyngostomy tubes remained in place a median of 8 days (range 4-17 days), whereas therapeutic pharyngostomy tubes were left in place a median of 15 days (range 7-125 days). There were 4 infectious complications (16%) unrelated to length of pharyngostomy use: 2 cases of cellulitis (resolved with antibiotics, tube remaining in place) and 2 superficial abscesses after tube removal requiring bedside débridement. Seventy-two percent of patients underwent swallow evaluation; 22% of these patients had radiographic evidence of aspiration. CONCLUSIONS: Pharyngostomy tube placement for gastric conduit decompression after esophagectomy is simple, and tubes can stay in place for prolonged periods. Our experience suggests that pharyngostomy tubes are a safe alternative to nasogastric drainage.