Lifelong Care Research Articles

Abstract 4137805: Loss to Follow-Up Among Adults with Congenital Heart Defects: A Report from Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE)

Background: Many of the 1.4 million adults with congenital heart disease (CHD) are lost to follow up (LTF). We aimed to identify factors associated with LTF and compare to those who remained in care. Methods: In the Congenital Heart Disease Project to Understand Lifelong Survivor Experience (CHD PULSE), we performed a cross-sectional survey in 2021-2023 of CHD survivors with a history of intervention at 11 centers in the Pediatric Cardiac Care Consortium, a large US-based registry of CHD procedures. Participants reported their cardiac history, general health, education, income, health insurance, healthcare utilization, and marital status. LTF was defined as having seen a cardiologist three or more years ago. Results: Among the 3109 respondents with CHD (median age 33) there were 1157 (37%) LTF and 1952 (63%) in care. Age, sex, marital status, and household income were not risk factors for LTF; but lack of health insurance, lower severity of CHD, and increasing time since last heart surgery were. Notably, respondents who reported being told in adolescence about the lifelong need for a cardiologist were almost three times as likely to report being in care (79% vs 28%, p<0.0001). Among women, most reported no LTF if counseled about concerns with pregnancy or told to avoid pregnancy. The most common reasons for being LTF for 5 years or more (N=857) included feeling well (60%), not thinking there was a need for lifelong care (49%), and a doctor told them they no longer needed to see a cardiologist (47%). Those LTF were more likely to report excellent or very good general health, physical health, and quality of life than those not LTF. There were no differences in self-assessment of mental health between groups. Conclusion: Adults with CHD and LTF were more likely to report less severe heart defects, a younger age at last surgery and a lack of counseling about the need for lifelong care.

Engagement in Healthcare Decision-Making and Associated Factors among Patients with Chronic Non-Communicable Diseases in Southwest Ethiopia, 2023

BackgroundChronic Non-communicable diseases require lifelong care involving patients, families, a team of their care providers, and significant others. Additionally, engaging patients in their healthcare decision-making is crucial for achieving better clinical outcomes. This study aimed to assess patients' engagement in healthcare decision-making and associated factors among adults with chronic non-communicable diseases in Southwest Ethiopia MethodsA cross-sectional study design was used among 422 participants at the public hospital of Jimma town. Study participants were consecutively recruited from May 01 to June 01, 2023. A structured Patient Activation Measure was used to measure patient engagement in healthcare decision-making. Descriptive statistics and binary logistic regression analysis describe the association between dependent and independent variables. ResultA total of 422 patients with chronic diseases participated in the study yielding a response rate of 99.3%. Slightly greater than one-third (35.8% (95% CI: 31.19, 40.41)) of participants in The study area had a high engagement in healthcare decision-making. Residence (Odd ratio 0.27; 95% confidence interval 0.137-0.534), educational level(Odd ratio 0.168; 95% confidence interval 0.064-0.443), access to information materials(Odd ratio 0.226; 95% confidence interval 0.112-0.458), duration(Odd ratio 3.864; 95% confidence interval 2.083-7.169), health literacy(Odd ratio 1.192; 95% confidence interval 1.111-1.28), and preference for autonomy(Odd ratio 1.097; 95% confidence interval 1.058-1.136) were factors statistically associated with patient engagement in healthcare decision-making. ConclusionOne-third of study participants reported high engagement in healthcare decision-making. Thus, healthcare providers should pay attention to patients with low educational levels, who do not have access to information materials, low health literacy levels, and their preference for autonomy to engage them in their healthcare decision-making.

Psychological conditions of caregivers of adult subjects with Prader-Willi syndrome

BackgroundPrader-Willi syndrome (PWS) is a rare genetic neurodevelopmental disorder. Individuals with PWS face a range of cognitive, behavioral, and emotional challenges that require comprehensive and lifelong care, posing significant demands on their caregivers. The study is not only aimed to assess the psychological conditions of caregivers of adult subjects with PWS focusing on psychological distress and coping, but also to shed light on a crucial yet often overlooked aspect of healthcare. This study aims to compare the psychological well-being of individuals with PWS and their caregivers, providing valuable insights that can potentially improve the quality of care for these individuals. The sample recruited at the Division of Auxology, IRCCS Istituto Auxologico Italiano, was composed of 30 adult subjects with PWS (11 men and 19 women; mean age ± SD: 36.4 ± 10.31 years; mean Body Mass Index (BMI): 35.7 ± 8.92: kg/m2) and their caregivers (10 men and 20 women). To assess the psychological condition of caregivers, the Italian-validated versions of the Depression Anxiety and Stress Scale (DASS-21) and the Coping Orientation to the Problems Experiences (COPE) were used, while to assess the psychological well-being of individuals with PWS and their caregivers, the Italian validated version of the Psychological General Well-Being Index (PGWBI) was used.ResultsDepression (p < 0.001), Stress (p = 0.050), and Total score (p = 0.009) of DASS 21 were higher in the caregivers of subjects with PWS than in the general population. PGWBI scores of caregivers were significantly lower than in individuals with PWS in Positive Well-being (p < 0.001), General Health (p = 0.006), Vitality (p = 0.004), and the total score (p = 0.006). The depression subscale of PGWBI was higher in caregivers than in subjects with PWS. Correlations between the subscales of COPE and the total score of PGWBI in caregivers revealed that the Avoidance subscale of COPE had a negative significant correlation with the total score of PGWBI (p = 0.003).ConclusionsOur results highlighted several critical insights into the profound emotional and psychological challenges faced by the caregivers of individuals with PWS.

Orthodontic treatment of patients with cleft lip and palate from birth through maturity

Cleft lip and palate (CLP) is one of the most common congenital anomalies, requiring lifelong care by a team of craniofacial specialists. A key member of that team is the craniofacial orthodontist, who provides treatment for these patients throughout the lifetime. This care actually begins prenatally, when families first meet with a craniofacial team. The family is provided with resources to prepare for their child's arrival and counseled about future treatment. Presurgical infant orthopedics (PSIO) will usually begin within a week of birth and continues for 3–5 months. PSIO helps approximate the lip and alveolar segments and reshapes the nostrils to facilitate primary surgical repairs. This is followed by a phase of monitoring growth and dental development throughout childhood, as patients with CLP are at increased risk for dental anomalies. Once the maxillary canine roots are one-third developed, the patient will be prepared for secondary alveolar bone grafting. This may include expansion, alignment, and/or maxillary protraction via facemask therapy. A short phase of maxillary dental alignment might follow if functional or esthetic concerns arise. Comprehensive orthodontic treatment is then performed at skeletal maturity, and coordinated with orthognathic surgery when indicated. The orthodontist and surgeon will collaborate closely in planning and managing the surgical correction. It is crucial that each stage of orthodontics is followed by an appropriate retention protocol. Finally, surgical and esthetic finishing procedures take place after surgical orthodontics is complete. This may include surgery to address lip, nose, speech, and breathing concerns, as well as definitive dental restorations.

7816 Implementation And Evaluation Of Clinical Care Structures For People With Differences Of Sex Development (DSD) In Germany

Abstract Disclosure: S.O. Hiort: None. M. Schnoor: None. U. Doehnert: None. M. Juergensen: None. J. Scherf: None. A. Heidenreich: None. M. Rapp: None. A. Katalinic: None. People with Differences of Sex Development (DSD) pose a challenge for healthcare. Lifelong holistic care requires a broad range of diagnostic approaches, qualified psychosocial, peer, fertility and sexual counselling, and structured therapeutic strategies including gonadal monitoring, hormone or surgical therapy. A quest has been taken to define centers of expertise for DSD and to substantiate and evaluate quality of care. Based on the criteria defined by the European Reference Network (ERN) for Rare Endocrine Conditions, 10 health care providers in Germany qualify for specialized DSD care. Quality indicators (QI) were developed through a Donabedian model to define structure, process and outcome quality mainly based on the German national guideline ”Differences of Sex Development”. Additionally, a consensus was reached though a structured process involving participating centers as well as patient organizations. Structural QIs are collected by an annual survey from the participating centers. Patient-related process and outcome data are recorded in a central registry. Patients and legal guardians receive questionnaires to collect patient-related outcome and experience measures after visits. These are measured by CHC-SUN/ YHC-SUN for treatment satisfaction and KIDDY KINDL, KIDSSCREEN 52, or WHOQOL-BREF for quality of life. An annual evaluation of all data results in a benchmarking of the participating centers. 24 indicators for structural quality were consented, 7 for process and 8 for outcome quality. Indicators of process quality comprise e.g. diagnostic procedures, case conferences, peer counseling or patient education. For structural quality there was an increase of centers fulfilling the QIs in 14 items including e.g. team composition and qualification during the study period from 2020 until 2022. Still very few centers had fixed standard operating procedures e.g. for gonadal monitoring or long-term follow up after surgery. 601 people with DSD were included into the registry from May 2021 until June 2023. In process quality there were considerable differences between centers both in QI compliance and data quality. The proportion of individuals with a genetic diagnosis varied by center from 44% to 96%. Information from questionnaires was available from 445 participants. Satisfaction with care was high. Parents reported in 86% to be very or highly satisfied with the visit, children and adolescents in 76%, and adults in 77%. Our approach allows for detailed evaluation of structural, process, and outcome quality. Health care insurers have secured appropriate funding for continuous evaluation of DSD care and the maintenance of the registry. This will allow for sustainability of the benchmarking and improvement of health care provision for people with DSD. Presentation: 6/1/2024

7984 A Challenging Case Of Hyperphosphatemic Familial Tumoral Calcinosis

Abstract Disclosure: T.G. de Souza: None. T.J. Weber: None. Introduction: Hyperphosphatemic Familial Tumoral Calcinosis (hFTC) is a rare, autosomal recessive metabolic disorder characterized by elevated serum phosphate levels and the accumulation of calcium phosphate deposits in soft tissues. There are fewer that 150 genetically confirmed cases in the literature. There are no consensus guidelines; therapeutic approaches are guided by case reports or series. This case serves to heighten awareness of therapeutic strategies including global health considerations for the disabled adult with a rare bone disorder. Case: A 32-year-old African-American woman presented to establish care for hFTC, for which she takes ibuprofen for disabling bilateral hip pain. hFTC was diagnosed in early childhood and managed at a large children’s hospital. Therapeutic modalities relied on operative resection of skin lesions on eruption. She describes cosanguinous parentage, consignment to foster care as a child and homelessness as an adult. She has not had adult endocrine care for her hFTC. Her medical comorbidities include major depressive disorder, PTSD and suicide attempt. She reports one uncle with hFTC noting that his phenotype was not significantly disabling and apparent recession of his lesions with age. Her parents did not have symptoms of hFTC. Radiographically, she had amorphous calcium deposits over both hips (her primary sites of severe pain), upper back (site of painless spontaneous eruptions), and left elbow without pain or skin penetration, as well as hyperostosis of the ilium bilaterally. She noticed waxing and waning of her eruptions with her menstrual cycle, such that lesions worsen in the luteal phase. Megestrol acetate also induced new eruptions. Examination shows brown staining to all teeth with several missing teeth. She had a marked lower extremity limb length discrepancy with severe restriction of hip abduction. Blood chemistries were grossly normal, including renal function, calcium, uric acid and PTH. Serum phosphorous was elevated at 5.2 (2.3 - 4.5 mg/dL). CT showed bilateral nephrocalcinosis. We instituted treatment with a low phosphorous diet, sevelamer to reduce intestinal phosphate absorption and acetazolamide to increase urinary phosphate excretion. For her pain and functional disability, we recommended continued NSAIDs, physical therapy and social work consultation to facilitate continued access to care. Conclusions: This case uniquely describes hFTC symptom fluctuation with the menstrual cycle, worsening with increased progesterone, suggesting a possible role of gonadal hormones on FGF-23 biology. It also highlights the socioeconomic burdens that can accompany rare bone disease, which become more pronounced as the patient achieves adulthood. Successful transition of care from pediatric to adult endocrinologist, in addition to engaging multi-disciplinary support, is essential in optimizing life-long care in hFTC. Presentation: 6/1/2024

Health and wellbeing of survivors of sexual violence and abuse attending sexual assault referral centres in England: the MESARCH mixed-methods evaluation.

One million people in England and Wales experience sexual violence and abuse each year, with nearly half experiencing serious sexual offences; around 30,000 survivors access sexual assault referral centres. This research was commissioned by National Institute for Health and Care Research to evaluate access, interventions and care pathways for survivors, especially those provided through sexual assault referral centres. The sexual assault referral centres care pathway was investigated through six sub-studies. There were two Cochrane Reviews (4274 participants). Seventy-two providers and 5 survivors were interviewed at eight sites; the children and young people study involved 12 participants from two sexual assault referral centres. A cohort study involving three-wave data collection over 1 year (21 sites; 2602 service users screened, 337 recruited) used a multilevel modelling framework to explore risk factors for burden of post-traumatic stress disorder symptoms at baseline and change at 1 year. We analysed costs and outcomes and conducted a narrative analysis (41 survivors). We worked closely with survivors and prioritised the safety/welfare of participants and researchers. Cochrane Reviews identified large effects from psychosocial interventions for post-traumatic stress disorder and depression. Sexual assault referral centres delivered a high-quality frontline service for survivors but groups experiencing domestic abuse and some ethnic and cultural minorities were under-represented. The qualitative research emphasised inter-agency collaboration for survivor benefit. The cohort study identified a risk 'triad' of adverse childhood experiences, poor mental health and economic deprivation, which was associated with baseline trauma burden. There were important improvements in trauma symptoms a year later. These improvements were unrelated to different sexual assault referral centre models. Costs and other outcomes were also similar across models. Harmful policing and justice practices/procedures were identified by 25% of participants. In this context, trauma-competent interviewing techniques, regular/timely updates and conveying case decisions with care signalled good practice. The cohort study lacked a comparison group, reducing confidence in the finding that access to sexual assault referral centres explained the reduction observed in post-traumatic stress disorder. Barriers to access call for concerted efforts to implement trauma-informed universal health services. The risk 'triad' underscores the value of holistic approaches to care at sexual assault referral centres and timely follow-on care. Poor mental health was the main barrier to service access beyond sexual assault referral centres. The persistence of trauma symptoms a year after accessing sexual assault referral centres signals urgent need for tackling counselling wait-lists, expanding support options and commitment to lifelong care. Multidisciplinary evaluation of sexual assault referral centres for better health provides a foundation for advancing trauma-informed practices in the context of sexual violence and abuse. This study is registered as ISRCTN30846825 https://doi.org/10.1186/ISRCTN30846825. This award was funded by the National Institute for Health and Care Research (NIHR) Health and Social Care Delivery Research programme (NIHR award ref: 16/117/04) and is published in full in Health and Social Care Delivery Research; Vol. 12, No. 35. See the NIHR Funding and Awards website for further award information.

Tidal Breathing Analysis as a Prognostic Index for Airway Obstruction Trajectory and Asthma in Preterm Infants.

An easy-to-implement and accurate lung function assessment tool for preterm infants is crucial to manage lifelong respiratory morbidities. We aimed to determine which pulmonary function parameters in preterm infants can predict the trajectory of airway obstruction and asthma development after 4years of age. We evaluated 52 preterm infants who had undergone both tidal breathing flow-volume loop (TBFVL) and multiple-breath washout (MBW) analyses in infancy and spirometry after the age of 4years. We evaluated the association between pulmonary function parameters in infancy and childhood and the pulmonary function trajectory until 13years of age and compared the changes in this trajectory according to pulmonary function parameters in infancy. Time to peak expiratory flow/expiratory time (TPEF/TE) in infancy was associated with FEV1, FEF25-75, and dysanapsis ratio in childhood and differed according to level of airway obstruction assessed by FEV1, FEV1/FVC, and FEF25-75, an asthma development. TPEF/TE was a significant predictive factor for airway obstruction and asthma after 4years of age, after adjusting for sex, extreme prematurity, duration of supplementary oxygen and mechanical ventilation, and recurrent wheezing during infancy. In premature infants with lower TPEF/TE, subsequent pulmonary function parameters remained low until 13years of age. In preterm infants, TPEF/TE could be useful to predict airway obstruction and asthma after 4years of age and even a lower pulmonary function trajectory until 13years of age. This information may help clinicians to provide lifelong care for pulmonary morbidity in children and adolescents born preterm.

Levels of IL-18 and IL-28 in thalassemia patients with viral infections

Thalassemia is an inherited hemoglobin disorder that requires lifelong medical care. Patients with thalassemia are very prone to infections by viruses, which may exacerbate their condition, leading to serious complications. Knowledge of the immune response in these patients could be very helpful in the effective management of their disease. The levels of IL-18 and IL-28 were evaluated in thalassemia patients with different viral infections to assess their potential as biomarkers and therapeutic targets. This was a retrospective analysis of clinical data on thalassemia patients with viral infections. Demographic, clinical, laboratory, IL-18, and IL-28 data at baseline were included in the study. In the present study, the authors assessed the correlations between interleukin levels, viral infection, treatment response, and overall survival. The study enrolled 250 patients with thalassemia and different viral infections. In these patients, the levels of IL-18 and IL-28 were found to be elevated with a significant correlation to viral load. Thus, a higher baseline interleukin level predicted a better treatment response and overall survival. Elevations in IL-18 and IL-28 in thalassemia patients with associated viral infections likely indicate immune system activation. Their correlation with viral load and treatment outcomes suggests their potential as biomarkers and targets for therapy. These findings further underline the protective role of IL-18 and IL-28, hence contributing to enhanced survival rates of the patients. Further studies for translation of these insights into practice may help modify clinical care for better patient outcomes.

Effect of Silicon Nitride Coating on Titanium Surface: Biocompatibility and Antibacterial Properties.

In recent years, with the advent of a super-aged society, lifelong dental care has gained increasing emphasis, and implant therapy for patients with an edentulous jaw has become a significant option. However, for implant therapy to be suitable for elderly patients with reduced regenerative and immunological capabilities, higher osteoconductive and antimicrobial properties are required on the implant surfaces. Silicon nitride, a non-oxide ceramic known for its excellent mechanical properties and biocompatibility, has demonstrated high potential for inducing hard tissue differentiation and exhibiting antibacterial properties. In this study, silicon nitride was deposited on pure titanium metal surfaces and evaluated for its biocompatibility and antibacterial properties. The findings indicate that silicon nitride improves the hydrophilicity of the material surface, enhancing the initial adhesion of rat bone marrow cells and promoting hard tissue differentiation. Additionally, the antibacterial properties were assessed using Staphylococcus aureus, revealing that the silicon nitride-coated surfaces exhibited significant antibacterial activity. Importantly, no cytotoxicity was observed, suggesting that silicon nitride-coated titanium could serve as a novel implant material.

Patient and carer perceptions of video, telephone and in-person clinics for Phenylketonuria (PKU)

BackgroundIn phenylketonuria (PKU), attending multidisciplinary clinic reviews is an important aspect of life-long care. Since the COVID-19 pandemic, video and telephone clinics are used as alternative methods for people with PKU to have contact with their care team. There is limited research concerning patient preference, experience and perceptions of alternative types of clinic review. Individuals from the UK with PKU and their caregivers were invited to complete an online questionnaire, hosted on the National Society for PKU (NSPKU) website and social media platform.ResultsData was available from 203 respondents. Forty one per cent of respondents (n = 49/119) preferred in-person clinics; 41% (n = 49) a hybrid of in-person, video and telephone clinics; 9% (n = 11) video clinics only, 6% (n = 7) telephone only and 3% (n = 3) were unsure. The main respondent obstacles to in-person clinics were costs, travel and time, but this was balanced by the benefits of a physical examination and better patient engagement/motivation. Twenty one per cent (n = 36/169) of respondents were uncomfortable with the number of healthcare professionals (HCPs) in a clinic room. Patients were less likely to consult with a doctor on video (64%, n = 91/143) or phone (50%, n = 59/119) reviews compared to in-person (80%, n = 146/183). Issues with video and telephone reviews included the shorter time length of review, distractions, technical issues and poor patient engagement.ConclusionsOnline video and telephone clinic platforms were effective in overcoming the challenging circumstances in management, monitoring and treatment of patients with PKU during the COVID-19 pandemic. However, in-person clinics remain the preferred respondent option. It is important that HCPs are flexible, enabling people with PKU a choice of clinic options according to their individual clinical need and circumstances.

Widespread service fragmentation for patients and families with tuberous sclerosis complex (TSC) in the Republic of Ireland

BackgroundTuberous sclerosis complex (TSC) is a rare approximate 1:6000 birth incidence, a genetic disease with a wide variability of physical and neuropsychiatric symptoms. Patients require lifelong care from multiple healthcare specialities, for which International and United Kingdom (UK) TSC consensus recommendations exist. Personalised care delivered by a centralised coordinated team of TSC experts is recommended. There is no such service for the estimated 600 TSC patients in the Republic of Ireland (ROI) and there is a paucity of information regarding the healthcare of this group.PurposeEvaluate the baseline care of patients with TSC attending epilepsy services in the Republic of Ireland (ROI) against UK TSC consensus recommendations.MethodsPatients with a diagnosis of TSC attending 12 adult and paediatric epilepsy centres in the ROI were identified. Clinical audits measured the baseline care of a subset of these patients against UK, TSC clinical recommendations. Data was anonymised and analysed at Trinity College Dublin.ResultsOne hundred thirty-five TSC patients attending twelve epilepsy centres were identified. Adults (n = 67) paediatric (n = 68). The care of 83 patients was audited (n = 63 ≥ 18 years) and (n = 20 < 18 years). Many baseline tests were completed, however, they required intra or interhospital referral. Care appears fragmented and there was no evidence of formal disease surveillance plans.ConclusionsThe number of TSC patients attending epilepsy services is lower than expected (n = 135). Specialist services and treatments for TSC are available through informal referral pathways. Although UK, TSC consensus baseline recommendations are roughly adhered to, care is fragmented. Increased coordination of care could benefit disease management.

The Role of Patient Organisations-Patients' and Parents' Views and Experience of Hirschsprung's Disease.

In many countries, patient organisations offer advice and the exchange of experiences to Hirschsprung's disease patients and their families. Professional treatment by experienced health care providers and the availability of life-long multidisciplinary follow-up care are essential. However, outside the clinic, patients and their families have to manage life on a day-to-day basis at home, which often brings up uncertainties and questions: Parents go through different stages during the diagnosis and treatment of their child, the affected children themselves go through many different stages of development, and even through the course of adulthood, new questions regarding the chronic disease may arise. Patient organisations can support the patients and their families at all stages of life by listening, offering information in an understandable way, connecting people, and sharing others' experiences. This enables families and patients to develop a better understanding of the rare disease and promotes their management strategies and confidence. The holistic approach of patient organisations aims to complement the medical treatment. Therefore, the referral of all patients and their families to patient organisations should be part of the medical advice in the treatment of Hirschsprung's disease.

Aftercare of Childhood Cancer Survivors in Switzerland-The General Practitioner Model.

Purpose: Childhood cancer survivors (CCS) represent a growing population worldwide, and lifelong follow-up care is recommended for most. Once CCS become adults, the transition to adult care is emerging. Today, there is no transition or long-term follow-up care model in the adult setting that clearly outweighs others. We therefore aimed to evaluate the transition to physicians outside the hospital. Methods: In this single-center, cross-sectional, questionnaire-based study, we assessed in 2022 the current follow-up care situation of CCS who already transitioned to physicians outside the hospital (family physicians, pediatricians). We asked CCS about cancer knowledge, worries, self-management skills, and expectations and physicians about their experience with CCS and their needs when caring for CCS. We included physicians where a CCS was transitioned to. We compared the results with CCS transitioned in a hospital setting and used descriptive statistics. Results: Twenty-three CCS responded to the questionnaire (median age at questionnaire of 22 years, median 14 years since diagnosis). Nearly two-thirds reported not being in follow-up care anymore. The cancer knowledge was good, and cancer worries were low. Twenty-eight physicians responded with 21 reporting that they care for CCS. Half of them see CCS for acute problems only. Physicians are open to care for CCS but request the necessary recommendations and would also be available for respective training. Conclusion: Transition to physicians might be an option for selected CCS. However, education and empowerment of CCS early on and education of physicians is urgently needed to prevent loss to follow-up, which may lead to lifelong nonengagement and incorrect perceptions about future health.

Challenges in Management of the Adult Vascular Anomalies Population: A Case Report and Review of the Literature.

Vascular anomalies (VAs) are a group of rare diseases affecting 0.01 to 0.02% of the population. VA may be associated with complex disorders affecting multiple organ systems, requiring a multidisciplinary approach to treatment. Multidisciplinary clinics are most frequently affiliated with pediatric centers. Multidisciplinary clinics in the adult setting are rare. Many adult VA patients find it difficult to establish care with individual providers, let alone a multidisciplinary clinic, leading to barriers to lifelong comprehensive care.

Transition mentorship for spina bifida patients with the JUMP program: a pilot study.

The transition from pediatric to adult care is challenging for patients and families with spina bifida (SB). Lifelong care relationships yield to new care environments that are typically larger, less personal, and less engaged with the nuances of SB care. Adolescence and young adulthood are often characterized by personal and psychological stresses due to factors independent of illness or chronic medical complexity. Surveys have demonstrated that transition is associated with uncertainty, anxiety, and elevated risk of adverse events for many SB patients. To help mitigate this, the authors developed a trial mentorship program between teen patients with SB and undergraduate/medical students. This study analyzes and presents the initial outcomes from this program. The authors created the Join, Unite, Motivate, and Prepare (JUMP) program to improve readiness for the transition process. The mentee target population was patients aged 13-19 years receiving care at the authors' SB clinic. Mentors were screened/approved undergraduate/medical students who volunteered to participate and successfully completed online training in mentorship. Upon enrollment, each patient set a combination of clinical, self, and parent/guardian goals using the individualized transition plan. These goals were shared with the mentor, mentee, parent/guardian, and physician. To monitor success, the SB program director routinely met with each mentor to discuss progress made and areas of growth. These included continuous quantitative and qualitative goal setting and failures that needed to be addressed for each agenda. Thirteen mentor-mentee matches were created over 9 months. Of the 13 matches, 6 had more than 5 communications after the initial meeting, and 1 mentor-mentee match is still in contact today. Noted success in the program has been through mentees gaining employment, applying for scholarships, starting college, and connecting with others who are going through similar circumstances. Challenges have arisen through failure to follow-up after the initial office visit, risk with using the virtual platform, and wide geographic dispersion of both mentors and mentees across the authors' state. Transition from pediatric to adult care for adolescents with SB has proven to be a large hurdle. Easing this process through well-thought-out, interactive processes has the potential to improve readiness, increase patient autonomy, and provide exposure to the adult healthcare community. However, the mentorship model, in the SB setting, has not proven to be the remedy.

Evaluation of emergency skin decontamination protocols in response to an acid attack (vitreolage)

The incidence of “acid attacks” (vitreolage) is a global concern, with those affected often receiving lifelong medical care due to physical and psychological damage. The purpose of this study was to evaluate the effectiveness of several emergency skin decontamination approaches against concentrated (>99 %) sulphuric acid and to identify the effective window of opportunity for decontamination. The effects of four decontamination methods (dry, wet, combined dry & wet and cotton cloth) were assessed using an in vitro diffusion cell system containing dermatomed porcine skin. Sulphuric acid (H2SO4) was applied to the skin with decontamination protocols performed at 10 s, 30 s, 8 min, and 30 min post exposure. Skin damage was quantified by tritiated water (3H2O) penetration, receptor fluid pH and photometric stereo imaging (PSI), with quantification of residual sulphur (by SEM-EDS) to determine overall decontamination efficiency. Skin translucency (quantified by PSI) demonstrated a time-dependent loss of dermal tissue integrity from 10 s. Quantification of dermal sulphur content confirmed the rapid (exponential) decrease in decontamination efficiency with time. The pH of the water effluent indicated complete neutralisation of acid from the skin surface after 90 s of irrigation. Wet decontamination (either alone or immediately following dry decontamination) was the most effective intervention evaluated, although no decontamination technique was statistically effective after 30 s exposure to the acid. These data demonstrate the time-critical consequences of dermal exposure to concentrated sulphuric acid: we find no practical window of opportunity for acid decontamination, as physical damage is virtually instantaneous.

The German cochlear implant registry: one year experience and first results on demographic data

PurposeClinical registries have great potential for quality control of medical procedures regarding the indications, therapeutic processes and results, including their possible complications. This is particularly true when providing patients with severe hearing loss or deafness with a cochlear implant (CI). This treatment represents a lifelong care process that requires continuous quality control over time. On the initiative of the Executive Committee of the German Society of Otorhinolaryngology (Deutsche Gesellschaft für Hals-Nasen-Ohren-Heilkunde, Kopf- und Hals-Chirurgie e.V., DGHNO-KHC), a national German CI registry (Deutsches Cochlear Implant Register, DCIR) was established in January 2022. This article focuses on the first demographic and baseline data of the DCIR.MethodsThe DCIR covers the complete therapeutic process from indication, surgery, fitting and lifelong aftercare in CI therapy. By the end of 2022, 75 hospitals in Germany had agreed to contribute to the DCIR.ResultsDuring the year 2022, 63 hospitals actively contributed data to the DCIR. Pseudonymized data from 2,292 CI implantations (2,176 primary implantations, 99 explantations with immediate re-implantations and 17 re-implantations following an earlier explantation) in 2,108 patients were documented. Cochlear implantation was accomplished in 1,807 adults (≥ 18 years) and 301 children (< 18 years). Fourty patients (1,9%) were children < 1 year of age and 55 (2,6%) were patients > 85 years. From the total of 2,292 implantations, 226 (9.9%) were performed as simultaneous bilateral implantations (CI implantation in both ears of 113 patients on the same day of surgery) and 412 implantations (19.1% of 2,162 implantations with data provided on the contralateral ear’s hearing status) were in patients with single sided deafness (normal hearing in the contralateral ear). In addition, the reported complications in 2022 were also evaluated. Seven reports (0.4%) of mild to moderate severe facial nerve dysfunctions were documented. No reports of severe or total facial nerve dysfunction (House-Brackmann grade V/VI), meningitis or death related to CI therapy were documented.ConclusionAlthough still in the start-up phase, these initial DCIR data already provide an interesting first insight into the demographic structure and baseline data of CI therapy in Germany. The successful implementation of the DCIR represents an important step towards continuous quality control of CI care.

Sustained Release Therapies with the Prostaglandin Analogues Intracameral Implants.

The travoprost intracameral implant was recently approved by the US Food and Drug Administration for sustained release medical treatment of open-angle glaucoma in the USA. The approval represents a substantial and progressive step forward in the area of sustained-release glaucoma therapy. Topical intraocular pressure-lowering medications for the treatment of glaucoma are faced with a host of challenges for long-term and usually lifelong care. A changing paradigm in glaucoma management involves first-line interventions with laser modalities, micro-invasive surgeries, and sustained-release treatment platforms. Future needs in the area of sustained-release therapy include a non-prostaglandin drug delivery platform and longer-term treatments that do not require surgical reintervention.

Ayurvedic management of Schizophrenia (Unmada) – A case report

Schizophrenia is a chronic, severe mental disorder with symptoms such as hallucinations, delusions, and lack of emotional expression requiring lifelong care and treatment to help people manage their symptoms. Treatment usually involves medications, psychotherapy, and social support. Antipsychotics are the management of choice. In Ayurveda, symptoms of schizophrenia resemble with those explained under Unmada, which is manifested due to derangement of the mind caused by imbalance or vitiation of Doshas. Yuktivyapashraya chikitsa along with Satvavajaya plays an important role in improving the symptoms and thereby the quality of life of patients. This report deals with a case of Schizophrenia, which was diagnosed as Kaphaja pittanubandha unmada according to the diagnostic principles of Ayurveda. A 36-year-old male patient presented with increased anxiety, decreased sleep, and mingling with others and was assessed using the Positive and Negative Syndrome Scale (PANSS). Panchakarma procedures including Virechana, Nasya, and Basti along with internal Ayurvedic medicines were given and improvement was noted in symptoms. Scores in the PANSS scale before and after the treatment were 79 and 59, respectively, inferring the importance of Ayurveda management approaches in addressing manifestations like Schizophrenia.