Parental experience of hypoglossal nerve stimulator implantation in adolescents with Down Syndrome and obstructive sleep apnea.

PURPOSE: The purpose of this study is to explore the experience of parents in deciding whether to participate in a clinical trial of the insertion of the Hypoglossal Nerve Stimulator (HNS) to treat their adolescent with Down Syndrome (DS) and Obstructive Sleep Apnea (OSA). DESIGN AND METHODS: A qualitative descriptive design with interviews was used to gather parental experiences from those who consented to HNS for their adolescent with DS and OSA. Interviews were conducted, audiotaped, and transcribed. Basic content analysis was followed to interpret the data. Using a process of data debriefing/engagement, codes were generated, and field/reflective notes were used to assure trustworthiness of the data. RESULTS: Parents, 13 mothers/2 fathers, participated. Three themes were identified: Parents experience desperation about acceptance of standard of care for their adolescent with DS and OSA. This desperation led parents to seek information/insights from social media and they came to rely on those sites to explore options, ultimately leading them to HNS clinical trial. Finally, parents had a desire to share experience with HNS implantation. CONCLUSIONS: Parents described being desperate at acceptance of standards of care for OSA. They shifted reliance on experts and parents by searching social media pages to explore options for treatment of OSA. Parents wished to share experiences with the HNS implantation. PRACTICE IMPLICATIONS: Nurses play a role in preparing for the HNS by instructing/educating parents. Nurses can identify supportive social media sites for parents during the HNS decision and suggest ways to measure outcomes of HNS.

Development and initial psychometric properties of the Pulmonary Arterial Hypertension Symptom Scale (PAHSS).

AIMS: The aim of this study is to report the development and psychometric properties of the Pulmonary Arterial Hypertension Symptom Scale (PAHSS). BACKGROUND: Patients with pulmonary arterial hypertension (PAH) experience multiple symptoms such as dyspnea, fatigue and chest pain, yet there is no comprehensive, validated symptom assessment tool to date. METHODS: This study used a cross sectional design. Participants completed: socio-demographic and medical data form, the PAHSS, the Medical Outcomes Study Short Form-36 and the Profile of Mood States short form. RESULTS: The PAHSS contains 17 symptoms measured on a 0 to 10 scale. Principal components analysis demonstrated a three factor solution for the PAHSS: pulmonary, diffuse, and cardiac. Coefficient alphas were good. Statistically significant Pearson coefficients were found between the PAHSS and the Medical Outcomes Study Short Form-36 and the Profile of Mood States short form. CONCLUSION: Findings show that the PAHSS is a promising scale to assess symptom severity.

Health-related quality of life and psychological states in patients with pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a chronic illness that impairs physical function and leads to right-sided heart failure and premature death. There is limited knowledge on health-related quality of life (HRQOL) and psychological states in patients with PAH. OBJECTIVE: The aim of this study was to determine the HRQOL and the psychological states of patients with PAH along with predictors of HRQOL. METHODS: In a cross-sectional design, participants with PAH completed the Medical Outcomes Study Short Form-36 v2 to measure generic HRQOL, the US Cambridge Pulmonary Hypertension Outcome Review to measure disease-specific HRQOL, and the Profile of Mood States to measure the psychological states. Descriptive statistics were used to calculate all sociodemographic and clinical data and were expressed as means and standard deviations for continuous variables and as frequencies and proportions for dichotomous and nominal variables. The statistical significance level was set at P < 0.05. A multiple linear regression analysis was performed to examine the sociodemographic and clinical variables as predictors of HRQOL. A bivariate analysis of the sociodemographic and clinical variables was performed to determine correlates with HRQOL. The variables that correlated with HRQOL at the 0.20 level of significance were included. RESULTS: There were 149 participants, 127 women and 22 men, with a mean age of 53.5 years. The participants demonstrated diminished general health, physical functioning, role physical, and vitality on the Short Form-36 v2. Functional class, education level, oxygen use, years since diagnosis, and calcium channel blocker therapy were predictive of poorer HRQOL. CONCLUSIONS: Patients with PAH are experiencing diminished physical health and HRQOL. Future studies are needed to design and test interventions to improve HRQOL.

Understanding the challenges during transitions of care for children with critical airway conditions.

PURPOSE: The specific aim of this qualitative descriptive study was to gain an understanding of the challenges occurring during transitions of care between two institutions for children with critical airway conditions. METHODS: Qualitative descriptive methodology was utilized to analyze data from two focus groups conducted with nurses from two adjacent institutions. RESULTS: The focus groups were composed of 19 staff nurses, three case managers, one clinical nurse specialist and two nurse directors. Three themes emerged from the nurses' focus groups: Uncertainty with Diagnosis, Communication Between Healthcare Facilities and Family Members, and Parental Acceptance/Readiness to Learn. CONCLUSION: Nurses are in a unique position to improve both the quality and coordination of care to these children and their family members. An effort to improve transfer of care between institutions and nurses could reduce the combined length of stay for patients and reduce avoidable readmissions. Nurses are in a key position to enhance the competence, confidence, and comfort for family members and caretakers to exercise their post-discharge responsibilities. IMPLICATIONS FOR PRACTICE: Consistent, clear communication among health care providers and family members can improve care in this vulnerable population. These data identified need for further education of nurses.

Cyber support: describing concerns of caregivers of people with pulmonary hypertension.

The purpose of this study was to gain an understanding of how caregivers of people with pulmonary hypertension are using an online discussion board. The chronicity and complex medical needs of people with pulmonary hypertension warrant a holistic nursing approach combining the patient and caregiver concerns to adequately address their needs. A qualitative descriptive approach was utilized. A convenience sample over an 18-month period of those caregivers who posted Internet messages to the Pulmonary Hypertension Discussion Board was included. Sociodemographics collected were age and gender of the person with pulmonary hypertension and the relationship of the caregiver to the person with pulmonary hypertension. Clinical variables collected were medications and oxygen use and years since diagnosis. Thematic analysis was used to identify themes. A total of 98 caregivers posted to the discussion board during the 18-month period; 46% of those posting were mothers of children with pulmonary hypertension. Four themes emerged: fear and frustration, questions and concerns, someone to listen to, and moving on with life. These themes characterize how caregivers of people with pulmonary hypertension were using the discussion board. Caregivers of people with pulmonary hypertension may need more information and support from their healthcare providers to adequately care for those with pulmonary hypertension.

Student perceptions of sudden cardiac arrest: a qualitative inquiry.

Sudden cardiac arrest (SCA) is the number one cause of death in young athletes in high school and university settings. Survival and outcomes of SCA is dependent on appropriate recognition of symptoms and immediate cardiopulmonary resuscitation (CPR), along with a shock from an automatic external defibrillator (AED). The three aims of the authors' study presented in this article were: to describe university students' perceptions and beliefs about sudden cardiac arrest, to describe university students' understanding of an AED and their level of preparedness to recognize and respond to a life threatening emergency event, and to identify university students' experiences of responding to handling life-threatening emergency events. Qualitative methodology was employed using semi-structured interviews and thematic analysis. Three major themes emerged from data analysis: confusion, uncertainty, and fear/uncomfortableness. These themes characterised participant's perceptions about SCA. The authors concluded that a lack of understanding of what SCA is and participants' inability to respond to an emergency event was evident.

Symptom Interference Severity and Health-Related Quality of Life in Pulmonary Arterial Hypertension.

CONTEXT: While assessing symptom severity is an important component of evaluating symptoms, understanding those symptoms that interfere with patients' lives is also key. Pulmonary arterial hypertension (PAH) is a chronic disease resulting in right heart failure and increased mortality. Patients with PAH experience multiple symptoms but we do not know which symptoms and to what extent their symptoms interfere with daily life. OBJECTIVES: To: (1) describe the prevalence of those symptoms that interfere with life; (2) describe the severity of symptom interference; and (3) determine those sociodemographic and clinical characteristics and interfering symptoms associated with health-related quality of life (HRQOL) in patients with PAH. METHODS: A convenience sample of 191 patients with PAH completed a sociodemographic form, the Pulmonary Arterial Hypertension Symptom Interference Scale (PAHSIS) and the Medical Outcomes Survey Short Form-36 to measure HRQOL. Hierarchical multiple linear regression was used to analyze demographic and medical characteristics along with symptom interference from the PAHSIS as predictors of HRQOL from the composite mental and physical health summary scores of the Short Form-36. RESULTS: The most interfering symptoms reported were fatigue, shortness of breath with exertion, and difficulty sleeping. Age, gender, functional class, oxygen use, fatigue, dizziness, and Raynaud phenomenon were associated with the HRQOL physical health summary scores. The symptoms fatigue and SOB while lying down were associated with the HRQOL mental health summary scores. CONCLUSION: Patients with PAH are experiencing multiple symptoms that are interfering with their HRQOL and ability to function.

Symptom Prevalence, Symptom Severity, and Health-Related Quality of Life Among Young, Middle, and Older Adults With Pulmonary Arterial Hypertension.

Pulmonary arterial hypertension (PAH) is a chronic, life threatening illness that affects primarily women. The purpose of this study was to describe the prevalence of PAH symptoms and to determine whether there are differences in symptom severity and HRQOL in PAH symptoms among young, middle, and older adults with PAH. A cross sectional design was utilized. For all the age groups, shortness of breath (SOB) on exertion and fatigue were the two most prevalent symptoms. SOB on exertion had the highest symptom severity scores followed by fatigue for all groups. Symptom severity was significantly different among the groups for palpitations, abdominal swelling and nausea. For components of HRQOL, physical functioning worsened with age. All groups had diminished general health, role physical and vitality levels. There are some differences in symptom prevalence, symptom severity and HRQOL among young, middle and older adults. Awareness of these differences is important for healthcare providers to know and assess overtime. Palliative care should be an integral part of caring for patients with PAH.

Developmental and Initial Validation of the Pulmonary Arterial Hypertension Symptom Inference Scale.

BACKGROUND: Pulmonary arterial hypertension is a chronic illness that produces multiple symptoms and impairs quality of life. PURPOSE: The purpose of this study was to describe the initial psychometric properties of the Pulmonary Arterial Hypertension Symptom Interference Scale (PAHSIS). METHODS: Participants completed a sociodemographic and clinical data form: the PAHSIS and the Medical Outcomes Study Short Form-36 (SF-36). Data analysis included descriptive statistics, principal component analysis (PCA), and Pearson r correlations. RESULTS: PCA revealed a 3-factor solution: Cardiopulmonary, Weary, and Gastric. Correlations between the 3 factors and the SF-36 subscales and composite summary scores ranged from acceptable to high. CONCLUSIONS: These findings support the initial validity and reliability of the PAHSIS. Nursing can use the PAHSIS to assess the impact of patient symptoms to deploy effective, targeted interventions.

Sleep disturbance, symptoms, psychological distress, and health-related quality of life in pulmonary arterial hypertension.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a devastating disease characterized by elevated pulmonary pressures that lead to right heart failure and premature mortality. Patients experience multiple symptoms including dyspnea, fatigue and chest pain, but little is known about sleep disturbance, PAH symptoms, psychological distress and health-related quality of life (HRQOL) in PAH. AIM: The purpose of this study was to describe the occurrence of sleep disturbance and compare PAH symptoms, psychological distress, and HRQOL across severity of sleep disturbance. METHODS: One hundred and ninety-one participants completed a socio-demographic and clinical data form, PAH Symptom Severity Scale, Profile of Mood States (POMS) short form and the Medical Outcomes Short Form-36 (SF-36). Descriptive statistics were used to describe sleep disturbances; analysis of variance models were used to quantify differences in PAH symptoms, psychological distress and HRQOL by sleep disturbance groups. RESULTS: The majority of participants (n=162, 85%) were women with a mean age of 53 years. Sixty-five (34%) reported no sleep disturbance; 54 (28%) mild sleep disturbance; 41 (22%) moderate sleep disturbance; and 31 (16%) severe sleep disturbance. Those reporting higher sleep disturbance severity reported worse PAH symptoms, psychological distress, and HRQOL. CONCLUSIONS: Sleep disturbance is a significant finding in PAH. Increasing levels of sleep disturbance are associated with worse PAH symptoms, psychological states, and health-related quality of life. Interventions that decrease sleep disturbances may improve symptoms and HRQOL.

New pharmacologic treatments for familial hypercholesterolemia.

Familial hypercholesterolemias are a group of genetic disorders that cause high levels of low-density lipoprotein (LDL) cholesterol, which can lead to atherosclerosis and premature coronary heart disease. Heart disease is the leading cause of death in U.S. women. A major goal in prevention of cardiovascular disease is identification and modification of risk factors. Lomitapide and mipomersen are two new pharmacologic options for treatment of familial hypercholesterolemia. Both are indicated as an adjunct for the management of homozygous familial hypercholesterolemia, along with lipid-lowering medications and diet modification.

A virtual community: concerns of patients with pulmonary hypertension.

Pulmonary Hypertension (PH) is a complex disorder characterized by elevated pressures in the pulmonary artery that cause right heart failure and eventually leads to death. Treatment regimens can be complex and mortality is high. The purpose of this study was to determine how people with PH are using an online Discussion Board. Qualitative descriptive methodology was used to analyze a convenience sample of self-identified patients with PH. Internet posts to an online Discussion Board from January 1, 2010 to December 31, 2010 were analyzed for common themes. Five hundred forty-nine individuals with PH posted to the Discussion Board. Four themes emerged: Uncertainty and Concern, Guidance and Validation, Support, and Refocusing Life. People with PH are using the Internet to answer questions about PH and seeking support. Future research needs to be conducted to further explore the needs and concerns of people with PH to provide tailored interventions.

Cluster analysis of symptoms in pulmonary arterial hypertension: a pilot study.

BACKGROUND: Pulmonary arterial hypertension (PAH) is characterized by elevated pulmonary artery pressures leading to right heart failure and death. AIMS: The aim of this study was to use cluster analysis to describe the symptom profile in PAH and differences in the health outcomes of health status, health-related quality of life (HRQoL) and psychological states in the cluster groups. METHODS: A cross-sectional descriptive design was used. A convenience sample completed a socio-demographic and clinical data form, a PAH Symptom Severity and Interference Scale, the Medical Outcomes Study Short Form (SF-36), the United States Cambridge Pulmonary Hypertension Outcome Review (US CAMPHOR) and the Short Form of the Profile of Mood States (POMS). RESULTS: Of the 151 participants, the mean age was 53.5 ± 15.1 with the majority female (n = 128, 85%). Fifty-eight (41%) were disabled and 67 (44%) were Functional Class IV. The most prevalent symptoms were shortness of breath with exertion (n = 149, 99%) and fatigue (n = 144, 93%). Three clusters emerged: Cluster 1 diffuse symptoms (n = 93), Cluster 2 severe cardiopulmonary symptoms (n = 32) and Cluster 3 moderate cardiopulmonary symptoms (n = 26). Overall, on the SF-36 the participants had poor general health, reduced physical function, role physical, vitality, and a low composite score for physical health. On the POMS the sample had limited vigor and increased fatigue. Cluster 2 Severe Cardiopulmonary Symptoms had worse SF-36, US CAMPHOR and POMS scores than the other cluster groups. CONCLUSIONS: Patients with PAH are experiencing a constellation of symptoms that are interfering with their life and emerging clusters were present.

Symptom experience of pulmonary arterial hypertension patients.

PURPOSE: To describe symptoms experienced by pulmonary arterial hypertension (PAH) patients and the impact these symptoms have had on their lives. METHOD: Qualitative descriptive methodology was chosen incorporating semistructured interviews to investigate the symptom experience of ten patients with PAH. RESULTS: All patients were diagnosed with WHO Group 1 PAH and 70% were WHO functional Class III. Two overarching themes emerged: holding back and redefining life. Holding back was represented by three subthemes: fear, anticipation of worsening symptoms, and treatment effects. Redefining life was characterized by uncertainty, activity restrictions, and making the best of it. CONCLUSION: This study revealed specific concerns related to the symptom experience of PAH patients and how they redefined their lives to accommodate these symptoms. These findings provide a basis for larger, quantitative studies to examine the extent of symptom impact as well as a framework for development of self management interventions to improve the symptom experience and QOL.

The experiences and concerns of young adults (18-40 years) living with an implanted cardioverter defibrillator (ICD).

BACKGROUND: Although the implanted cardioverter defibrillator (ICD) is a widely accepted life-saving technology, adjusting to life after ICD implantation may vary according to the recipient's age. Few studies have investigated how young adults manage life with an ICD and there are no studies describing their concerns or experiences of living with an ICD. AIM: The aim of the study was to describe the experiences and concerns of young adults (18-40 years) living with an ICD. METHODS: Qualitative descriptive methodology was chosen incorporating interviews and written narrative responses to investigate twenty young adult's experiences of living with an ICD. RESULTS: There were four descriptive categories: psychosocial, developmental, physical, and economical. Themes in the psychosocial category were: returning to normal, mood disturbances, and body image concerns. Themes in the developmental category were unique to young adults: childbearing and childrearing, while themes in the physical category were expectation regarding physical activity, and the economical category, the issue of financial security. CONCLUSIONS: These data reveal that young adults with ICDs experience the unique concerns of childbearing, childrearing, and are worried about their financial security. These findings provide insight for the development of age-appropriate interventions for young adults before and after ICD implantation.