A phenomenological exploration of the mental health experiences of young women with diminished ovarian reserve.

Infertility is a reproductive disease affecting one in six individuals that renders an individual unable to conceive. One cause of infertility is diminished ovarian reserve (DOR), which reduces the quantity and/or quality of a female's oocyte pool. Although typically indicating normal ovarian aging during the late 30s and early 40s, DOR can also impact younger women, increasing their risk for psychological distress from an unexpected diagnosis of infertility. A phenomenological approach examined the mental health experiences and perceptions of infertility-related mental health care of young women with DOR. Women diagnosed with DOR by age 35 in the United States who experienced emotional distress during infertility were recruited from infertility-specific social media and via snowball sampling. Participants completed a demographic survey and semi-structured individual interview that was audio-recorded, transcribed verbatim, and analyzed using a phenomenological approach. Ten women ages 27-41 completed the study. On average, participants were 30 years of age at the time of DOR diagnosis (age range 25-35), primarily Caucasian (90%), and married (90%). Two main themes were found: (1) Young women with DOR feel like a "forgotten community" coping with an invisible disease; and (2) Not all fertility clinics are created equal. Participants perceived their diagnosis as devastating and hopeless and urged others to find a provider with ample experience treating patients with DOR. This study helped to understand how young women with DOR perceive their mental health and identified a significant need for advancing towards more holistic infertility healthcare that encompasses both physical and mental health.

The Lived Experience of African American Persons with Cystic Fibrosis.

Background: Cystic fibrosis (CF) is a rare genetic disease affecting approximately 30,000 people in the United States (US). African American persons with CF are even rarer, comprising approximately 5% of this population. Purpose: The purpose of this study was to explore the lived experiences of African American persons with CF to identify potential disparities in health care. Methods: Descriptive phenomenology was used to explore lived experiences of African American persons with CF over age 18 recruited from CF Foundation-accredited Centers in the US, CF-specific social media, and via snowball sampling. Study data was obtained through telephone interviews that were audio-recorded, transcribed verbatim, and analyzed using Colaizzi's method of thematic analysis. Results: Six men and six women (ages 23-45) completed the study. Interviews revealed three themes: (1) Accepting a Diagnosis of CF; (2) Desiring a Normal Life while Living with an Invisible Disease; and 3) A Slippery Slope of Subtle Racism. Each theme had 2-3 subthemes. Conclusions: It is critical to explore the unique challenges faced by African American persons with CF in order to develop interventions that improve their daily lives and create better futures. Implications for Practice: Findings highlight the unique challenges faced by underrepresented groups with CF and the need to address health inequities to improve care delivery.

A review of the psychometric properties and implications for the use of the fertility quality of life tool.

OBJECTIVES: To analyze and synthesize the reported psychometric properties of the Fertility Quality of Life (FertiQoL) instrument and describe its implications for use in practice and research in men and women with infertility. METHODS: A systematic literature search was performed to identify all articles using the FertiQoL tool. PubMed, CINAHL, and PsycINFO were searched from September 2006 through May 2022. Studies were eligible for inclusion if they reported psychometric data on the original FertiQoL tool using a sample population of individuals with infertility. Sample size, country of origin, and psychometric data were documented for each study. RESULTS: The initial search revealed 153 articles that had utilized the FertiQoL. Following abstract, title, and full-text screenings, 53 articles reported psychometric data and met criteria for inclusion. The FertiQoL is a sound measurement with satisfactory reliability and validity. Studies indicated adequate reliability in the overall scale ([Formula: see text]), as well as the core Emotional, Mind/Body, Social, and Relational scales ([Formula: see text]) and two optional Tolerability and Environment fertility treatment subscales ([Formula: see text]). Although the Relational subscale exhibited slightly lower reliability in several studies, the internal consistency for the measurement as a whole was satisfactory. Results also indicate adequate: 1) face and content validity with extensive professional and patient feedback during development; 2) convergent validity with general quality of life, depression, and anxiety measurements; and 3) structural validity using both confirmatory and exploratory factor analyses. CONCLUSION: The FertiQoL tool is the most commonly used instrument to measure the impact of fertility issues on quality of life in men and women with infertility. Understanding the impact of infertility on quality of life provides valuable insight into the areas of infertility-related care that need to be prioritized, such as mental health or relational stressors. While the instrument has been used in different patient populations with infertility and available in multiple translations, it is necessary to understand the updated psychometric properties and the implications for its use. This review shows that the FertiQoL is reliable and valid for cross-cultural use among individuals with various etiologies of infertility.

Association between unplanned pregnancies and maternal exacerbations in cystic fibrosis.

INTRODUCTION: Following availability of the highly effective cystic fibrosis (CF) transmembrane conductance regulator modulator, elexacaftor/tezacaftor/ivacaftor, there was a near doubling of pregnancies reported in the United States (US) in people with CF. We sought to determine health impacts of planned (PP) versus unplanned pregnancies (UP). METHODS: We collected retrospective pregnancy data from January 2010-December 2020 from 11 US CF centers. After adjusting for potential confounding effects, we conducted multivariable, multilevel longitudinal regression analysis using mixed effect modeling to assess whether changes in percent predicted forced expiratory volume in one second (ppFEV1), body mass index (BMI), and pulmonary exacerbations (PEx) 1-year-pre- to 1-year-post-pregnancy were associated with pregnancy planning. RESULTS: Our analysis included 163 people with 226 pregnancies; the cohort had a mean age at conception of 29.6 years, mean pre-pregnancy ppFEV1 of 75.4 and BMI of 22.5 kg/m2. PpFEV1 declined in both PP (adjusted decline of -2.5 (95% CI: -3.8, -1.2)) and UP (adjusted decline of -3.0 (95% CI: -4.6, -1.4)) groups, they did not differ from each other (p = 0.625). We observed a difference in change in the annual number of PEx pre- to post-pregnancy (PP: 0.8 (0.7, 1.1); UP: 1.3 (1.0, 1.7); interaction effect p = 0.029). In a subset of people with available infant data, infants resulting from UP had more preterm births, lower APGAR scores, and more intensive care unit stays. CONCLUSIONS: Following UP, there is an increased trajectory for PEx and potentially for infant complications compared to PP. Clinicians should consider increased surveillance in the setting of UP.

Paths to Motherhood for Women with Cystic Fibrosis.

PURPOSE: Cystic fibrosis (CF) is no longer a disease limited to childhood. With medical advancements, many of those with CF live into adulthood and have similar life goals as their non-CF peers. Most women with CF want to become mothers. However, available options and the related decision-making process is not well understood. The purpose of this study was to explore the decision-making framework of women with CF to better understand the factors they consider when deciding on a path to motherhood. STUDY DESIGN AND METHODS: Qualitative interviews were performed using a grounded theory approach. Inclusion criteria were women with CF who became mothers through biological pregnancy, adoption, or gestational surrogacy. Results: Twenty-five mothers with CF were interviewed. A distinct decision-making process was identified through which women started with a desire for motherhood, assessed several factors, then eventually took the path they felt was right for them and their family. CLINICAL IMPLICATIONS: Our findings provide women with CF a framework that other women with CF have used to assist in making decisions about their reproductive options. Conversations about family planning should occur early and regularly between women with CF and their health care providers. The decision-making process to achieve motherhood for women with a chronic illness, such as CF, includes consideration of unique factors that should be included in clinical conversations.

Fertility preservation in women with cystic fibrosis pre-lung transplantation: A mixed methods study.

AIMS: Explore the knowledge, experiences, preferences, and concerns related to fertility preservation as an option for building a biological family among women with cystic fibrosis. DESIGN: Convergent mixed methods study design. METHODS: We recruited women with cystic fibrosis of childbearing age in the United States through cystic fibrosis centres, snowball sampling, and social media. Participants completed an anonymous survey about fertility and fertility preservation (n = 50). We also conducted audio-recorded, semi-structured interviews with a subset of women to gain a better understanding of their perspectives (n = 20). We transcribed the interviews verbatim and analysed them using thematic analysis. RESULTS: For the quantitative arm, 78% of women indicated that they would like to have a child in the future; however, 74% reported never having had conversations about fertility preservation with their providers. For the qualitative arm, four major themes emerged: (1) Women with cystic fibrosis have inadequate knowledge about fertility and fertility preservation; (2) fertility is a low priority area for the cystic fibrosis care team; (3) women with cystic fibrosis recommend that the cystic fibrosis care team provide specific fertility resources; and (4) providers and literature lack information on fertility and cystic fibrosis. Integrated findings identified that while the majority of women with cystic fibrosis want to become mothers in the future, including post-lung transplantation, they have not received education on fertility preservation, and there is a general lack of knowledge on the topic of fertility in cystic fibrosis. CONCLUSION: Women with cystic fibrosis desire to have children but have little knowledge about fertility preservation, and cystic fibrosis providers do not initiate family planning discussions. IMPACT: Findings from the study support that additional education is needed for women with cystic fibrosis who are considering parenthood. Clinical care models should include early, regular, and thoughtful discussions about reproductive health issues, including fertility preservation.

Predicting Substance Use from Religiosity/Spirituality in Individuals with Cystic Fibrosis.

Religiosity and spirituality predict lower alcohol and other substance use in community samples of adolescents and adults. However, the roles of religiosity and spirituality in substance use have not been examined in individuals with cystic fibrosis (CF). Adults with CF (n = 123) completed measures of spirituality, religiosity, and substance use. Clinical measures of illness severity (e.g., BMI and  %FEV1) were obtained from participants' medical records. Substance use rates for alcohol, tobacco, and marijuana were lower in this sample than those seen in the general population. Of the measured BMMRS subscales, spiritual experiences and religious commitment were significant predictors of lower alcohol use. These results suggest that personal factors of religiosity and spirituality are more important for substance use in adults with CF than participation in religious service and events or adoption of religious practices.

Religious coping and psychosocial adjustment in patients with cystic fibrosis.

The role of religious coping (RC) in psychosocial outcomes and health-related quality of life (HRQoL) in adults with cystic fibrosis has not been addressed. Multivariate regressions evaluated the effects of baseline RC on depressive and anxiety symptoms and HRQoL at 3-month follow-up in 123 adult cystic fibrosis patients. Higher positive RC attenuated the effects of perceived stress on greater depressive and anxiety symptoms. Negative RC predicted less vitality and social HRQoL, as well as more digestion symptoms. Positive RC may buffer the impact of stress on patients' psychological distress, whereas negative RC may contribute to lower health-related quality of life.

Retropharyngeal abscess in a post-lung transplant cystic fibrosis patient with prior cervical fusion: a case study.

BACKGROUND: Cystic fibrosis (CF) is a chronic, genetic, incurable disease that affects primarily the respiratory and gastrointestinal systems. End-stage lung disease is the leading cause of death in people with CF, and lung transplant is required to preserve life. Anti-rejection medications are necessary post-transplant; however, these medications lower immune response and increase susceptibility to bacterial infections. Complications from infections post lung-transplant account for approximately 30% of CF-related deaths. Retropharyngeal abscess (RPA) is a rare deep neck infection that occurs most commonly in children. This is the case of a 45-year-old Caucasian male with CF who developed a retropharyngeal abscess post wisdom teeth extraction that seeded into hardware from a previous cervical disc fusion. CASE PRESENTATION: The patient presented to the emergency department with severe neck and shoulder pain, limited range of motion in his arm and neck, and dysphonia. He reported feeling pain for 10 days and suspected the pain was caused by a weightlifting injury. The patient reported low-grade fever 5 days prior, which responded to acetaminophen. He was afebrile upon admission and in no respiratory distress. Diagnostic labs revealed WBC 22,000/uL and CRP 211 mg/L. The CT scan showed a large abscess in the retropharyngeal space between C2-C7. The immediate concern was airway obstruction and need for possible intubation or tracheostomy. The patient was transferred to ENT service with neurosurgery and transplant consults. The RPA was drained and lavaged. The cervical hardware was discovered to be infected and was removed. The source of the RPA infection was determined to be from the patient's wisdom teeth extraction 6 months prior to RPA. The patient received 8 weeks of intravenous ceftriaxone for Streptococcus pneumoniae bacteremia and underwent revision of his cervical fusion 3 months after hardware removal. CONCLUSIONS: Clinicians should consider prophylactic antimicrobial therapy for immunocompromised patients when they are at increased risk for transient bacteremia such as following invasive procedures (e.g., tooth extraction). Prophylactic antimicrobial therapy could prevent potentially life-threatening infections such as RPA in immunocompromised patients.

Two Unanticipated Pregnancies While on Cystic Fibrosis Gene-Specific Drug Therapy.

Women with cystic fibrosis (CF) desire to become pregnant and accomplish the same life goals as women without CF. The underlying pathology of CF and medications used to treat this genetically transmitted disease can affect women's reproductive potential. An interview with Ana (pseudonym), who became pregnant twice while taking the medication lumacaftor/ivacaftor (LUMA/IVA), was analyzed using thematic analysis. She described her experiences related to "Fertility and Pregnancy Surrounding LUMA/IVA," the major theme that emerged from her narrative. While there are anecdotal reports of infants conceived by women on LUMA/IVA and other CF precision medications, pregnancy rates and outcomes are not systematically tracked. Education about risks and benefits of these medications should be provided as part of comprehensive clinical care.

"If We Would Have Known": A Couple's Regret Over a Missed Opportunity to Have a Biological Child After Lung Transplantation.

Cystic fibrosis (CF) is a genetic, chronic disease that results in thickened secretions in the respiratory, gastrointestinal, and reproductive tracts. Over 95% of males with CF have congenital bilateral absence of the vas deferens causing infertility. This is a case study of a 29-year-old male who underwent a lung transplant after 8 months of oxygen dependency secondary to poor lung function. Approximately 1 year posttransplant, he and his wife decided that they wanted to start a family and consulted a fertility specialist who advised them to utilize donor sperm due to the teratogenic effects of posttransplant medications. Taken by surprise with this news, they expressed regret about the missed opportunity for pretransplant sperm aspiration and cryopreservation to conceive a biological child. He reported, "If we would have known, we would have made sure I [banked] my own [sperm]." This case study highlights a critical gap in CF comprehensive clinical care.