Social Media and Website Use: The Experiences of Parents and Carers Accessing Care at the Oxford Craniofacial Unit.

INTRODUCTION: Historically, medical professionals have been the providers of specialist information about rare medical conditions. Now, increasingly, patients and the public are using the internet to access and generate information about medical diagnoses. The global nature of the internet allows patients to connect across geographical borders, and to obtain and share information that would have been previously inaccessible to them. This research investigated the use of website and social media by parents of children with craniosynostosis. METHODS: A cross-sectional survey-based design was employed. Participants were parents of children with craniosynostosis attending multidisciplinary craniofacial clinics within the Oxford Craniofacial Unit. A questionnaire was administered which assessed social media and website use adapted from the questionnaire created by Khouri and colleagues (2016) and Huggons and colleagues (2019). The surveys were administered over an 18-month period (November 2020 to May 2022). RESULTS: The final sample comprised 82 parents [70 mothers; 10 fathers; 1 sister/carer and 1 parent (mother/father unspecified)]. The children were aged 11 months-16 years of age (average age 6 y and 3 mo). Children had a variety of diagnoses: 31 sagittal, 19 metopic, 14 syndromic craniosynostosis, 8 unicoronal, 6 multisuture, 3 bicoronal, and 1 unilambdoid.Results showed that 93% (n=76/82) of parents used the internet to find out more about craniosynostosis, with 72% (n=59/82) of parents specifically using social media to find out more about craniosynostosis. The social media platforms used included: Facebook 64% (n=53/82), Instagram 24% (n=20/82), Blogs 12% (n=10/82), Twitter 4% (n= 4/82), Tik Tok 2% (n=2/82), and Snapchat 0.01% (n=1/82).Parents reported that Facebook was the most helpful source of information about craniosynostosis (52%; n=43/82). Parents indicated the key timepoints they used social media included: when their child received a diagnosis (70%; n=58/82), before their child's surgery (34%; n=28/82), before their first craniofacial clinic appointment (83%; n=25/30), and when child was older (17%; n=14/84). Forty percent (n=33/82) of parents said that a diagnosis of craniosynostosis made no difference to their social media use, whereas 34% (n=28/82) of parents used social media more, and 20% (n=16/82) used it less. CONCLUSION: Results highlight that parents use social media and other websites to access information relating to craniosynostosis. Future research should examine whether parental use of social media changes across their child's lifespan and evaluate the quality of this information.

Speech and Language Development, Hearing, and Feeding in Patients With Genetically Confirmed Crouzon Syndrome With Acanthosis Nigricans: A 36-Year Longitudinal Retrospective Review of Patients at the Oxford Craniofacial Unit.

OBJECTIVE: Crouzon syndrome with acanthosis nigricans (CAN) is caused by the specific mutation c.1172C>A (p.Ala391Glu) in the fibroblast growth factor receptor 3 gene, and has an estimated prevalence of 1:1,000,000 births. Most cases occur de novo; however, autosomal dominant inheritance may occur. The clinical presentation typically includes craniosynostosis, midface and maxillary hypoplasia, choanal atresia/stenosis, hydrocephalus, and intracranial hypertension. Patients develop acanthosis nigricans, a hyperkeratotic skin disorder. The authors present the first known study to investigate the speech, language, hearing, and feeding of patients with CAN. METHODS: A retrospective case-note review of patients with a genetically confirmed diagnosis of CAN attending the Oxford Craniofacial Unit during a 36-year period (1987-2023) was undertaken. RESULTS: Participants were 6 patients with genetically-confirmed CAN (5 females, 1 male), all cases arose de novo. All patients had craniosynostosis (n = 5/6 multisuture synostosis, n = 1/6 left unicoronal synostosis). Hydrocephalus was managed through ventriculoperitoneal shunt in 67% (n = 4/6) of patients, and 67% (n = 4/6) had a Chiari 1 malformation. Patients had a complex, multifactorial feeding history complicated by choanal atresia/stenosis (100%; n = 6/6), and significant midface hypoplasia. All patients required airway management through tracheostomy (83%; n = 5/6); and/or continuous positive airway pressure (67%; n = 4/6). All patients underwent adenotonsillectomy (100%; n = 6/6). Initial failure to thrive, low weight, and/or height were seen in 100% (n = 6/6) patients; 80% (n = 4/5) had reflux; 100% (n = 6/6) had nasogastric, or percutaneous endoscopic gastrostomy based feeding during their treatment journey. All patients had hearing loss (100%; n = 6/6). Early communication difficulties were common: receptive language disorder (50%; n = 3/6); expressive language disorder (50%; n = 3/6); and speech sound disorder in 50% (n = 3/6)-necessitating the use of Makaton in 80% of patients (n = 3/5). CONCLUSIONS: Patients with CAN experience significant respiratory, neurological, and structural obstacles to hearing, speech, language, and feeding. The authors present a recommended pathway for management to support patients in these domains.

Implementation of a Violence Risk Assessment and Interventions Aimed at the Prevention of Patient-Perpetrated Violent Events Across Care Settings.

Healthcare worker violence at the hands of patients is the leading cause of workplace injury with reports demonstrating yearly increases. Literature suggests organizational strategies to reduce workplace injury are necessary to create safe environments for patients to receive care and for healthcare workers to practice, but tactics lack a care continuum focus and largely focus on deescalation and management of violent events. One healthcare system identified that the use of an assessment widely used in Canada, identifying patients at risk for violent behaviors, would supplement already existing efforts at protecting patients. The implementation of this tool across care settings is presented in this article.

Hearing, Speech, Language, and Communicative Participation in Patients With Apert Syndrome: Analysis of Correlation With Fibroblast Growth Factor Receptor 2 Mutation.

Apert syndrome (AS) is caused by the heterozygous presence of 1 of 2 specific missense mutations of the fibroblast growth factor receptor 2 (FGFR2) gene. The 2 adjacent substitutions, designated p.Ser252Trp (S252W) and p.Pro253Arg (P253R), account for more than 98% of cases. Previous research has identified elevated hearing difficulties and incidence of cleft palate in this population. However, the influence of FGFR2 genotype on the speech, language, and communicative participation of children with AS has yet to be examined. METHODS: A retrospective case note analysis was completed for all patients with a genetically-confirmed Apert mutation who attended the Oxford Craniofacial Unit over a 43-year period (1978-2020). Medical records were analyzed for speech, language, hearing, and communication data in detail. The therapy outcome measures, based on the World Health Organization International Classification of Functioning, Disability, and Health was used to classify patient's communicative participation. RESULTS: The authors identified 55 AS patients with genetically-confirmed mutation of the FGFR2 gene. One patient with a S252F mutation was excluded. There were 31 patients with the S252W mutation (male = 14; female = 17), age range of last hearing assessment (1-18 years), 64% (18/28) of patients had a cleft palate (including bifid uvula), 15 patients had conductive hearing loss, 1 patient had mixed hearing loss, 18 had otitis media with effusion (4 of whom had a cleft palate); 88% (21/24) of patients had receptive language difficulties, 88% (22/25) of patients had expressive language difficulties, 96% (27/28) of patients had a speech sound disorder. There were 23 patients with the P253R mutation (male = 13; female = 10); age range of last hearing assessment (1-13 years), 35% (8/23) patients had a cleft palate (including bifid uvula), 14 patients had a conductive hearing loss, 17 had otitis media with effusion (2 of whom had a cleft palate). Results indicated that 85% (17/20) of patients had receptive language difficulties, 80% (16/20) had expressive language difficulties, 100% (21/21) had a speech sound disorder. The S252W mutation was significantly-associated with the presence of cleft palate (including bifid uvula) (P  = 0.05).Data about the cumulative impact of all of these factors for communicative participation using the therapy outcome measures were available for 47 patients: (30 S252W; 17 P253R). Patients with a S252W mutation had significantly more severe difficulties with communicative participation when compared to individuals with a P253R mutation (P  = 0.0005) Cochran-Armitage trend test. CONCLUSIONS: Speech, language, communicative participation, and hearing difficulties are pervasive in patients with AS. The severity and functional impact of these difficulties are magnified in patients with the S252W mutation. Results reinforce the importance of considering patients with AS according to genotype.

Making the case for the collection of a minimal dataset for children with speech sound disorder.

BACKGROUND: NHS case note data are a potential source of practice-based evidence which could be used to investigate the effectiveness of different interventions for individuals with a range of speech, language and communication needs. Consistency in pre- and post-intervention data as well as the collection of relevant variables would need to be demonstrated as a precursor to adopting this approach in future investigations of speech and language therapy intervention. AIMS: To explore whether routine clinical data collection for children with speech sound disorder (SSD) could be a potential source for examining the effectiveness of intervention(s). METHODS & PROCEDURES: We examined case notes from three UK NHS services, reviewing 174 sets of case notes and 234 blocks of therapy provided for school-age children with SSD. MAIN CONTRIBUTION: We found there was significant variation in pre- and post-intervention data and variables collected by the services. The assessment data available in the case notes across all sites were insufficient to be used to compare the effectiveness of different interventions. Specific issues included lack of consistent reporting of pre- and post-intervention data, and use of a variety of both formal and informal assessment tools. CONCLUSIONS & IMPLICATIONS: The case notes reviewed were from three sites and may not represent wider clinical practice, nevertheless the findings suggest the sample explored indicates the need for more consistent and contemporaneous collection of data for children with SSD to facilitate the investigation of different interventions in practice. Researchers should work with the clinical community to determine a minimal dataset that includes a core outcome set and potential variables. This should be feasible to collect in clinical practice and provide a dataset for future investigations of clinically relevant research questions. This would provide an invaluable resource to the clinical academic and research communities enabling research questions to be addressed that have the potential to lead to improved outcomes and more cost-effective services. WHAT THIS PAPER ADDS: What is already known on the subject While there is some evidence for the efficacy of therapy for children with SSD, studies typically focus on very specific populations who meet strict selection criteria and take place in university clinics or laboratory-style settings which do not reflect typical clinical practice in the UK and elsewhere. An alternative approach to investigating the effectiveness of interventions would be to use NHS case note data. It is not clear from the existing literature whether case note data are sufficiently robust to facilitate such an analysis. What this paper adds to existing knowledge This study found that case note data, in particular assessment data, were highly variable across services and would be insufficient to compare different interventions for this population. Agreement on what should be included in a minimal dataset for children with SSD is required to maximize the potential for NHS clinical case notes to become a resource for future research. What are the actual or potential clinical implications of this work? This study indicates that current clinical practice in SLT for children with SSD is inconsistent with regards to the reporting of pre- and post-intervention assessment data and other important variables in case notes. We make the case for agreeing a minimal dataset with a need for clinicians to work with researchers to determine core outcomes and additional relevant data, which can be feasibly collected in clinical practice.

The Sensitivity and Specificity of Parental Report of Concern for Identifying Language Disorder in Children With Craniosynostosis.

ABSTRACT: Many factors that may co-occur with craniosynostosis, such as oral structural anomalies, hearing impairment, visual impairment, cognitive difficulties and psychosocial factors, may predispose this population to communication difficulties. At the Oxford Craniofacial Unit, children's speech, language and communication are regularly monitored in accordance with a systematic developmental screening protocol developed by the Speech and Language Therapists in the 4 United Kingdom (UK) Highly Specialized Craniofacial Centers. In addition to routine assessments, when parents attend routine multidisciplinary clinic appointments, they are asked about their child's communication development, and whether they have any concerns.A retrospective review was undertaken of parental concerns about hearing, speech development, behavior, physical development, concentration, school and friendships as indicated by parents on the Oxford Craniofacial Unit Pre-Clinic Questionnaire. The areas of concern were then correlated with the results of a standardized, guided parent questionnaire about children's language development, (Children's Communication Checklist - 2 (CCC-2)), to determine whether parental concern alone is a reliable way of identifying whether patients require further assessment for Language Disorder associated with Craniosynostosis.Participants were parents of 89 monolingual English-speaking children with craniosynostosis (62 male; 27 female), age range four to 13 years (mean age = 8 years 7 months), receiving active care at the Oxford Craniofacial Unit (June 2017-July 2018). Results of the pre-clinic questionnaire indicated that 6% of parents had concerns about their child's communication development. Results of the CCC-2 indicated that 29/89 (32.6%) of children required further assessment for Language Disorder associated with Craniosynostosis. When language difficulties were identified on the CCC-2, only 14% (n = 4/29) parents indicated concern on the pre clinic questionnaire. Results indicated that parental concern about behavior was the most important factor in identifying language disorder (P = 0.023).Results reinforce that the pre-clinic questionnaire is useful for identifying areas of parental concern. Results also indicate that parental concern alone is not sufficient to identify language disorder, and that further, detailed assessment is warranted. The results are consistent with previously reported links between behavior and language in the general population.

Feeding, Communication, Hydrocephalus, and Intracranial Hypertension in Patients With Severe FGFR2-Associated Pfeiffer Syndrome.

BACKGROUND: Pfeiffer syndrome is associated with a genetic mutation of the FGFR2 (or more rarely, FGFR1) gene, and features the combination of craniosynostosis, midface hypoplasia, broad thumbs and broad great toes. Previous research has identified a wide spectrum of clinical phenotypes in patients with Pfeiffer syndrome. This study aimed to investigate the multifactorial considerations for speech, language, hearing and feeding development in patients with severe genetically-confirmed Pfeiffer syndrome. METHODS: A 23-year retrospective case-note review of patients attending the Oxford Craniofacial Unit was undertaken. Patients were categorized according to genotype. Patients with mutations located in FGFR1, or outside the FGFR2 IgIII domain-hotspot, or representing known Crouzon/Pfeiffer overlap substitutions were excluded. Twelve patients with severe FGFR2-associated Pfeiffer syndrome were identified. RESULTS: Patients most commonly had pansynostosis (n = 8) followed by bicoronal (n = 3), and bicoronal and sagittal synostosis (n = 1). Seven patients had a Chiari I malformation. Four patients had a diagnosis of epilepsy. Ten patients had with hydrocephalus necessitating ventriculoperitoneal shunt insertion.Feeding difficulties were common (n = 10/12) and multifactorial. In 5/12 cases, they were associated with pansynostosis, hydrocephalus, tracheostomy and tube feeding in infancy.Hearing data were available for 10 patients, of whom 9 had conductive hearing loss, and 8 required hearing aids. Results indicated that 3/4 patients had expressive language difficulties, 3/4 had appropriate receptive language skills. 6/12 patients had a speech sound disorder and abnormal resonance. CONCLUSION: This study has identified important speech, language, hearing and feeding issues in patients with severe FGFR2-associated Pfeiffer syndrome. Results indicate that a high rate of motor-based oral stage feeding difficulties, and pharyngeal stage swallowing difficulties necessitating regular review by specialist craniofacial speech and language therapists.

Pharmacologic Management of Persistent Pain in Cancer Survivors.

Improvements in screening, diagnosis and treatment of cancer has seen cancer mortality substantially diminish in the past three decades. It is estimated there are almost 20 million cancer survivors in the USA alone, but some 40% live with chronic pain after completing treatment. While a broad definition of survivorship that includes all people living with, through and beyond a cancer diagnosis-including those with active cancer-is often used, this narrative review primarily focuses on the management of pain in people who are disease-free after completing primary cancer treatment as adults. Chronic pain in this population needs a different approach to that used for people with a limited prognosis. After describing the common chronic pain syndromes caused by cancer treatment, and the pathophysiologic mechanisms involved, the pharmacologic management of entities such as post-surgical pain, chemotherapy-induced neuropathy, aromatase inhibitor musculoskeletal syndrome and checkpoint inhibitor-related pain are described. The challenges  associated with opioid prescribing in this population are given special attention. Expert guidelines on pain management in cancer survivors now recommend a combination of pharmacologic and non-pharmacologic modalities, and these are also briefly covered.

Transition from acute to chronic pain: where cells, systems and society meet.

Current treatments for chronic pain are often ineffective. At the same as searching for novel therapeutics, there is growing interest in preventing acute pain becoming chronic. While the field is still in its infancy, its knowledge base is increasingly expanding. Certainly, biomedical factors, for example, the type of tissue damage, are important but they are often not modifiable. Psychosocial risk factors (e.g., thoughts and beliefs about pain, mood, social support, workplace problems) are modifiable. There is an increasing body of research that cognitive behavioral therapy can prevent transition. Internet-based delivery of cognitive behavioral therapy improves access. Clinicians need to be aware that they may inadvertently promote pain chronification in their patients by what they say and do.

Long-term outcomes from training in self-management of chronic pain in an elderly population: a randomized controlled trial.

This study compares the outcomes, from pretreatment to 1-year follow-up, of an outpatient, CBT-based pain self-management program (PSM) that included exercises, pain education, and pain coping strategies, with a control condition (exercise-attention control, EAC) that included exercises and a control for the attention of the treatment team. We previously reported short-term results (to 1-month follow-up) from the same study. This new paper considers the important issue of maintenance of treatment-related gains. The participants (n = 141) were a heterogeneous sample of ambulant, community-dwelling older adult patients with chronic pain (mean age: 73.90 [6.5] years [range: 65-87 years]). The long-term results indicate the pain self-management program group achieved and maintained significantly better results than the exercise-attention control group on the primary outcome, pain-related disability, as well as on usual pain, pain distress, depression, and fear-avoidance beliefs. The mean effect size for these gains by the pain self-management program group over the exercise-attention control group was 0.37 (range: 0.29-0.45), which is in the small effect size range. While statistically and clinically meaningful, these findings do indicate some weakening in effects over time but not to a significant degree. The study has implications for the provision of pain management interventions for community-dwelling older adults with chronic pain.

Self-management intervention for chronic pain in older adults: a randomised controlled trial.

This study compared an outpatient pain self-management (PSM) program, using cognitive-behavioural therapy and exercises, with 2 control conditions in 141 chronic pain patients aged > 65 years. Results immediately posttreatment indicated that relative to the Exercise-Attention Control (EAC) group, the PSM group was significantly improved on measures of pain distress, disability, mood, unhelpful pain beliefs, and functional reach. The mean effect size for these gains was 0.52 (range: 0.44-0.68). By 1-month follow-up, relative to the EAC group, the PSM group remained better on most measures. At the 1-month follow-up, relative to a Waiting List (usual care) (WL) group, the PSM group was significantly improved on measures of pain distress, disability, and unhelpful pain beliefs. The mean effect size for these variables was 0.69 (range: 0.56-0.83). Relative to the WL group, the EAC group made no significant gains on any of the measured variables. At 1-month follow-up, the mean proportion of reliably improved cases (across outcome variables) was 41% (range: 16-60%) for the PSM group, twice that of those who met this criterion in the 2 control conditions (and this difference was statistically significant). Similarly, significantly more (44%) of the PSM group (vs 22% and 20% for the control groups) achieved a clinically significant improvement on pain disability. In the short term at least, cognitive-behavioural therapy-based PSM was more effective than exercises and usual care.