Lisa Tuchman

Cystic fibrosis and transition to adult medical care.

Transition of young adults with cystic fibrosis (CF) from pediatric to adult medical care is an important priority, because many patients are living well into their fourth decade, and by 2010 more than half of all people living with CF will be older than 18 years. Transition to adulthood, a developmental process of skill-building in self-management supported by the health system, is important for the successful transfer to adult CF care. The US Cystic Fibrosis Foundation has been proactive in preparing for increasing numbers of young adults in need of specialized adult-oriented care by creating specialized clinical fellowships for physician providers and mandating establishment of adult CF programs. Despite these initiatives, how to best facilitate transition and to define and measure successful outcomes after transfer to adult care remains unclear. Many adults with CF continue to receive care in the pediatric setting, whereas others transfer before being developmentally prepared. In this state-of-the-art review we provide context for the scope of the challenges associated with designing and evaluating health care transition for adolescents and young adults with CF and implications for all youth with special health care needs.

Sperm Banking for Adolescent and Young Adult Cancer Patients: Sperm Quality, Patient, and Parent Perspectives

Infertility is often a complication for adolescent and young adult males who receive cancer therapy, a problem that might be averted through using cryopreserved sperm. We aim to evaluate feasibility of offering newly diagnosed patients the opportunity to bank sperm and, to determine the beliefs and decision‐making processes of patients and their parents who considered sperm banking.

Provider and Patient Attitudes Regarding Sexual Health in Young Women With Cystic Fibrosis

OBJECTIVE: To explore the attitudes, preferences, and experiences of patients with cystic fibrosis (CF) and CF providers toward sexual and reproductive health (SRH) care for young women with CF. METHODS: Young women with CF aged 18 to 30 years from a US CF care center and pediatric and adult CF program directors from a national sample participated in qualitative interviews investigating their experiences regarding SRH care and their attitudes and preferences toward SRH care provision in the CF setting. Interviews were audio-recorded, transcribed, and coded by using a thematic analysis approach. RESULTS: Twenty-two patient participants and 16 CF program directors were interviewed. Themes shared by both groups included the importance of SRH discussion in the CF care setting, patient and provider discomfort as a barrier to SRH care, and the need for SRH educational resources and provider training to improve SRH care. Providers highlighted the lack of standardization around SRH care in the current CF care model. Patients desired SRH educational resources coupled with early SRH discussions initiated by their CF provider. CONCLUSIONS: Both CF providers and patients agree that the CF provider has a fundamental role in providing CF-specific SRH care. Educational resources coupled with individualized SRH discussions may facilitate improved SRH care for young women with CF. Investigation into the implementation of SRH education and services into pediatric-onset chronic disease care models is needed.

Trends in standard workup performed by pediatric subspecialists for the diagnosis of adolescent polycystic ovary syndrome

OBJECTIVE The purpose of this study is to identify trends in the clinical workup, diagnosis, and treatment of polycystic ovary syndrome by pediatric endocrinologists, pediatric gynecologists, and adolescent medicine specialists. DESIGN Retrospective chart review. SETTING Tertiary care medical center. PARTICIPANTS Females aged 11-18 y who were evaluated for PCOS from June 2009 to October 2011 were included. Any patients with coexisting diagnoses of other primary etiology for amenorrhea were excluded. Patients were identified by ICD-9 codes for PCOS, hypersecretion of ovarian androgens, irregular menses, hirsutism, oligomenorrhea, or amenorrhea. 261 patients were included: 144 from endocrinology, 9 from gynecology, and 108 from adolescent pediatric practices. RESULTS There were no significant differences in the androgen labs ordered by the subspecialties. Gynecologists ordered pelvic ultrasonography for 89% (n = 8) of patients, compared to 9% (n = 10) by adolescent medicine specialists and 24% (n = 34) by endocrinologists (P < .0001). Endocrinologists were most likely to treat patients who met diagnostic criteria for PCOS with metformin (58%, n = 66), compared to gynecologists (14%, n = 1) and adolescent medicine specialists (5%, n = 3) (P < .0001). Gynecologists (43%, n = 3) and adolescent medicine specialists (58%, n = 39) were more likely than endocrinologists (24%, n = 27) to treat patients with oral contraceptive pills (P < .0001). CONCLUSIONS Inconsistent diagnosis and treatment strategies for young women with PCOS are evident among pediatric subspecialties, reflecting lack of standardized care for adolescents. Quantifying outcomes based on diagnostic and therapeutic approaches are important next steps.

Sexual and reproductive health behaviors and experiences reported by young women with cystic fibrosis.

BACKGROUND The prevalence of general and disease-specific sexual and reproductive health (SRH) concerns is unknown in the United States (U.S.) female CF population. This study aimed to describe and compare the SRH experiences and behaviors of young women with CF with the general U.S. POPULATION METHODS Young women with CF ages 15-24years from five geographically diverse U.S. CF centers participated in a survey investigating SRH. Results were summarized and compared to the U.S. National Survey of Family Growth (NSFG) using logistic regression adjusting for confounders. FINDINGS A total of 188 young women with CF (mean age 19.7±2.7years) completed the survey; data were compared to 1997 NSFG respondents (mean age 19.6±0.10years). Fifty-four percent of women with CF reported having had vaginal sex with a male partner compared to 66% of U.S. women (p=0.55). Women with CF were less likely to have ever used contraception (55% vs. 74%, p=0.0001) or have been tested for sexually transmitted infections in the past year (19% vs. 34%, p=0.001) compared to the general population. Two percent of women with CF reported having ever been pregnant compared to 24% of U.S. women (p<0.0001). One-third of young women with CF reported perceived pubertal delay, 16% urinary incontinence, 16% sexual dysfunction, and 49% yeast infections. INTERPRETATION Young women with CF face significant SRH concerns and appear to be experiencing gaps in SRH care provision. Opportunities exist for intervention development around this aspect of comprehensive CF care. FUNDING CF Foundation (KAZMER15A0); U.S. National Institutes of Health (UL1TR000005).

Cystic fibrosis program directors' attitudes toward sexual and reproductive health in young women with CF.

Advancements in therapy have led to dramatic increases in the life expectancy of patients with cystic fibrosis (CF). As survival improves, young women with CF will have expectations for their sexual and reproductive health (SRH) futures similar to their counterparts without CF. As they face unique CF‐specific SRH concerns, they may rely on CF care providers for disease‐specific SRH care provision. The purpose of this study was to investigate the attitudes of CF providers toward female SRH and perceived barriers in the current CF care model.

Adolescents' Perceptions of Transition Importance, Readiness, and Likelihood of Future Success: The Role of Anticipatory Guidance.

Expert consensus supports anticipatory guidance around health care transition (HCT), but little is known about its impact on adolescents’ perceptions of HCT. This study aimed to evaluate the frequency of HCT anticipatory guidance delivery and the effect it had on participants’ perceptions of HCT. Adolescents (n = 209) with special health care needs were administered National Survey for Children with Special Health Care Needs transition assessment questions, then reported perceptions of transition importance, readiness, and likely future success. Over half of the participants reported no history of discussion about transition to an adult provider (64%) or insurance needs (67%); just under half (43%) had not discussed their changing health care needs. In participants reporting receipt of anticipatory guidance, ratings of transition readiness and future success were significantly higher than those who received no anticipatory guidance, supporting that HCT anticipatory guidance has a significantly positive impact on adolescents’ perceptions of the HCT process.

Psychosocial adjustment in school-age girls with a family history of breast cancer

OBJECTIVE: Understanding how young girls respond to growing up with breast cancer family histories is critical given expansion of genetic testing and breast cancer messaging. We examined the impact of breast cancer family history on psychosocial adjustment and health behaviors among >800 girls in the multicenter LEGACY Girls Study. METHODS: Girls aged 6 to 13 years with a family history of breast cancer or familial BRCA1/2 mutation (BCFH+), peers without a family history (BCFH–), and their biological mothers completed assessments of psychosocial adjustment (maternal report for 6- to 13-year-olds, self-report for 10- to 13-year-olds), breast cancer–specific distress, perceived risk of breast cancer, and health behaviors (10- to 13-year-olds). RESULTS: BCFH+ girls had better general psychosocial adjustment than BCFH– peers by maternal report. Psychosocial adjustment and health behaviors did not differ significantly by self-report among 10- to 13-year-old girls. BCFH+ girls reported higher breast cancer–specific distress (P = .001) and were more likely to report themselves at increased breast cancer risk than BCFH– peers (38.4% vs 13.7%, P < .001), although many girls were unsure of their risk. In multivariable analyses, higher daughter anxiety was associated with higher maternal anxiety and poorer family communication. Higher daughter breast cancer–specific distress was associated with higher maternal breast cancer-specific distress. CONCLUSIONS: Although growing up in a family at risk for breast cancer does not negatively affect general psychosocial adjustment among preadolescent girls, those from breast cancer risk families experience greater breast cancer–specific distress. Interventions to address daughter and mother breast cancer concerns and responses to genetic or familial risk might improve psychosocial outcomes of teen daughters.

Sexual and reproductive health care utilization and preferences reported by young women with cystic fibrosis

BACKGROUND This study aimed to describe and compare the sexual and reproductive health (SRH) care utilization among young women with cystic fibrosis (CF) with the general United States (U.S.) population. METHODS Women with CF ages 15-24years from five geographically diverse U.S. CF centers participated in a survey investigating SRH. Results were summarized and compared to a nationally representative sample of young women aged 15-24 who participated in the U.S. National Survey of Family Growth (NSFG) using logistic regression to adjust for confounders. RESULTS A total of 188 women with CF (19.7±2.7years) completed the survey; data were compared to 1997 NSFG respondents (19.6±0.10years). Women with CF had lower lifetime rates of ever obtaining a Pap smear or pelvic exam (26% vs. 57%; p<0.001) and similar rates of HPV vaccination (44% vs. 43%; p=0.64) compared to NSFG respondents. Thirty-seven percent of women with CF reported seeking contraception and <10% reported contraceptive counseling, STI testing/counseling, or pregnancy testing in their lifetime. In the prior 12months, 41% of NSFG respondents reported seeking contraception, 24% received contraceptive counseling, 22% STI testing/counseling, and 23% pregnancy testing. A minority of women with CF received or discussed SRH care in the CF setting, although 66% wanted to discuss SRH with their CF team. CONCLUSIONS Young women with CF report low rates of SRH care utilization and desire SRH discussions in the CF setting. Interventions should target improved SRH care delivery and encourage patient-provider communication around SRH in the CF care setting.

Psychosocial Adjustment and Perceived Risk Among Adolescent Girls From Families With BRCA1/2 or Breast Cancer History

PURPOSE To evaluate the impact of breast cancer family history and maternal BRCA1/2 mutation on the psychosocial adjustment and perceived risk in girls age 11 to 19 years old. MATERIALS AND METHODS Girls age 11 to 19 years old with one or more relatives with breast cancer or a familial BRCA1/2 mutation (breast cancer family history [BCFH] positive, n = 208; n = 69 with BRCA1/2-positive mother), peers (BCFH negative, n = 112), and their mothers completed assessments of psychosocial adjustment, breast cancer-specific distress, and perceived risk of breast cancer. RESULTS General psychosocial adjustment did not differ significantly between BCFH-positive and BCFH-negative girls, either by self-report or mother report, except for higher self-esteem among BCFH-positive girls (P = .01). BCFH-positive girls had higher breast cancer-specific distress than BCFH-negative girls (P < .001), but girls from BRCA1/2-positive families did not differ from other BCFH-positive peers. BCFH-positive girls were more likely to report themselves at increased self-risk for breast cancer in adulthood than BCFH-negative peers (74% v 33%, respectively; P ≤ .001). Girls from BRCA1/2-positive families were more likely than other BCFH-positive and BCFH-negative peers to report themselves at increased risk (P < .001). In all groups, perceived risk of breast cancer was associated with older age. Higher breast cancer-specific distress among adolescent girls was associated with higher self-perceived risk of breast cancer and higher maternal breast cancer-specific distress. CONCLUSION Adolescent girls from BRCA1/2-positive and breast cancer families have higher self-esteem and do not have poorer psychosocial adjustment than peers. However, they do experience greater breast cancer-specific distress and perceived risk of breast cancer, particularly among older girls. Understanding the impact is important to optimize responses to growing up in families at familial and genetic risk for breast cancer, particularly given the debate over the genetic testing of children for cancer susceptibility in adulthood.