Contraceptive use among women with cystic fibrosis: A pilot study linking reproductive health questions to the Cystic Fibrosis Foundation National Patient Registry

Abstract

Objectives

To examine contraceptive use, pregnancy intention and the association of hormonal contraceptive type with adverse health outcomes among women with cystic fibrosis (CF).

Study design

We recruited 150 women with CF, ages 18–49 from three adult CF programs to complete an online survey regarding their pregnancy and contraceptive use history. Survey findings were merged with retrospective clinical information from the CF Foundation Patient Registry (CFFPR). We used descriptive analyses to report contraceptive method and pregnancy frequencies, and logistic regression to examine the association between contraceptive method type and adverse health outcomes.

Results

Combined hormonal contraceptives were the most commonly used methods (42%), followed by condoms (34%), and long-acting reversible contraceptives methods (27%). Thirty-three percent (n = 50) reported ever being pregnant, half of whom reported having at least one unplanned pregnancy. We found no significant association for mucoid Pseudomonas aeruginosa infection among progestin-only (aOR 1.53, 95% CI 0.07–32.2) and estrogen-containing hormonal contraceptive users (aOR 3.9, 95 % CI 0.20–76.5). Risk of osteoporosis was elevated among women with CF who used depot-medroxyprogesterone acetate compared to non-users (OR 5.36, 95% CI 1.00–29.12).

Conclusions

Both contraceptive use and unplanned pregnancy among women with CF are common. Associations between hormonal contraceptive use and adverse pulmonary or bone outcomes among women with CF are inconclusive due to the study s small sample size. Larger studies are warranted.

Implications

Women with CF should be informed about the risks and benefits of contraceptives in the context of their disease. CFFPR data capturing contraceptive method use may be the most efficient way to elucidate the association of hormonal contraceptives on disease in women with CF.

1. Introduction

Cystic Fibrosis (CF) is a genetic, predominantly Caucasian disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. The number of persons with CF in the U.S. over age 18 has nearly doubled in the last 30 years, from 9000 to 16,000 [1]. Women with CF and the clinicians who care for them are increasingly faced with questions related to the effectiveness and safety of current contraceptive methods.

Given the complexity of this multi-organ disease, treatment for CF is multifaceted and often requires use of multiple daily medications, which may interact with hormonal contraceptive methods [2]. By 2020, CFTR protein modulators (oral medications which modulate the function of the CFTR protein), are expected to be used by approximately 90% of people with CF in the U.S. who are over 12 years of age [3]. Concerns regarding the interaction of one CFTR modulator, lumacaftor, with contraceptive pills exists [4]. Importantly, near puberty, women with CF tend to have more acute exacerbations of their chronic pulmonary disease compared to young men with CF, which are thought to be linked to the presence of female hormones [5]. For example, during ovulation, when serum estrogen levels are highest, data suggests inflammatory markers are elevated, forced expiratory volume in 1 second (FEV1) scores are lower and colonized pulmonary bacteria, Pseudomonas aeruginosa (P. aeruginosa), in particular become more virulent [6,7]. Synthetic hormone impact on pulmonary outcomes and other CF-related co-morbidities has not be evaluated extensively, as indicated by two previously published systematic reviews [8,9].

The United States Medical Eligibility Criteria for Contraceptive Use (USMEC) contains evidence-based recommendations for CF [10]. USMEC does not restrict the use of any of contraceptive methods by women with CF. The USMEC states that Depot medroxyprogesterone acetate (DMPA), use generally outweighs risk (category 2), despite concerns that women with CF have increased risk of osteopenia, osteoporosis, and fragility fractures in the presence of reduced vitamin D and calcium absorption, corticosteroid use, and chronic inflammation [33].

CF is a condition that can be associated with increased risk for adverse health events as a result of pregnancy, yet only about half of reproductive-aged women with CF are using contraceptive methods [11]. Fecundity among women with CF is thought to be somewhat lower than women in the general population because of viscous cervical mucus and decreased cervical mucous pH resulting from abnormal CFTR anion transport gene. However, because CFTR modulators target the underlying protein abnormality, which may improve both cervical mucous viscosity and pH, the numbers at risk for unintended pregnancy may increase as more women with CF take these medications [12].

The Cystic Fibrosis Foundation (CFF) maintains a comprehensive Patient Registry (CFFPR), that captures annual data on over 90% of all persons with CF in the U.S.; it has a low loss to followup annual rate of only 3.4% [13]. The CFFPR currently includes extensive demographic, diagnostic, and clinical data, but lacks detailed information about pregnancies or contraceptive use [1].

We undertook this multi-center pilot study to explore contraceptive use, pregnancy intention and outcomes, and the relationship of contraceptive use with adverse pulmonary outcomes and bone health among women with CF.

2. Materials and methods

2.1. Study design, setting and participants

We conducted a cross-sectional, self-reported online survey of females with CF aged 18–49 years who were current participants of the CFFPR at three CF adult programs. Participants were recruited through email, social media or in clinics from the University of Washington, University of Texas Southwestern, and National Jewish Hospital in Denver, Colorado between October 2017 and April 2018. The institutional boards (IRBs) of the University of Washington (UW) and the two collaborating institutions approved the study protocol. The UW IRB allowed us to verify that survey respondents were current patients at the UW Adult CF Program; the IRBs of the collaborating CF programs did not. Participants provided written informed consent once they signed onto the secured, online survey platform.

2.2. Variables

The lead researcher (EMG) assembled a Registry Development Group (RDG) of 14 experts (including a patient with CF, specialists from CF, family planning, endocrinology, bioinformatics, quality improvement, epidemiology, and a representative from the CFFPR) to help plan this study. The RDG provided input on key outcome, exposure and confounder variables. The RDG recommended categorizing exposure by progestin-only methods, estrogen-containing or combined hormonal contraceptive (CHC) methods and non-hormonal methods because of the uncertainty of synthetic hormones on CF disease. Women with CF who used no contraception were a separate comparison group because of evidence indicating non-contraceptive users consider their disease as worse compared to women with CF who use contraception [11]. Hormonal levonorgestrel IUD (LNG-IUD) was grouped with the systemic progestin only method since there still a low systemic amount of progestin exposure. The RDG also recommended additional exposure and confounding variables, including pregnancy history, and start dates of CFTR modulator therapy, since this information may be imprecisely documented in the CFFPR. The RDG suggested outcome variables from the CFFPR, including diagnosis of osteopenia, osteoporosis or bone fracture. The RDG recommended collecting CFFPR variables that indicated adverse pulmonary outcomes, including percent predicated forced expiratory volume in 1 second (ppFEV1) < 50% predicted, which is an indicator of severe pulmonary obstruction [14]; having 2 or more pulmonary exacerbations in a given year; and presence of mucoid P. aeruginosa phenotype, an organism associated with more rapid decline in lung function [5]. P. aeruginosa phenotype is important in CF because the mucoid phenotype is associated with worse clinical outcomes. Because non-mucoid P. aeruginosa has been shown to convert to the more aggressive phenotype, when exposed to estradiol for as little as 4 weeks [6], we wanted to determine if exposure to estrogen-containing contraception might be associated with mucoid P. aeruginosa status. We collected CFTR mutation class, as those within classes I-III were considered to have a more severe phenotype than those with classes IV-V or other [1]. We collected BMI, which we used as a proxy for nutritional status, with <18.5, 18.5–24.9 and 25.0–29.−29.9 indicating underweight, normal, and overweight, respectively [15].

2.3. Data sources

We collected exposure data through an online, self-respondent survey. To capture the effects of contraception on patients health over time, the survey asked about each contraceptive method used for each year between 2012 and 2016, the most recent year for which CFFPR data were available for analysis. We piloted the survey among 10 women with CF in Seattle and San Diego. Participants entered responses directly into REDCap (Research Electronic Data Capture), a secure, web-based application designed to support data capture for research studies [16]. We downloaded the online survey responses and linked them via a centralized study identification number (ID) to the data imported from CFFPR. Subsequent to CF center staff obtaining informed consent, CFFPR data is compiled based on information entered by individual CF center staff from the electronic medical record (EMR) or center-specific forms completed by patients or families [13].

2.4. Bias

We sought to reduce selection bias by notifying all eligible patients at each CF program about the study, although one site only approached women at their in-person clinical visits to the CF center. We sought to reduce social desirability by having respondents complete the survey directly in REDCap, which was not accessible to staff members at the participating CF programs.

2.5. Study size

We aimed to enroll 290 women with CF and who were also current participants in the CFFPR, which was an estimated 70% of the number of eligible women at the three CF programs combined. Our sample size was not based on statistical power, given that this study was designed as a pilot study.

2.6. Statistical methods

We performed descriptive analyses of contraceptive methods used each year between 2012 and 2016, number of unplanned pregnancies, and pregnancy outcomes. Women reporting no contraceptive use in a given year may have included women with a history of hysterectomy, seeking pregnancy, those who had a same-sex partner, or were not sexually active since we did not collect these data from the participants. Respondents may have reported using multiple or different methods, or no method for each year between 2012 and 2016 and thus were counted within multiple contraceptive category types in Table 1. We used a panel data random effects population-averaged logistic model to calculate risk for a particular pulmonary outcome, accounting for between-individual and between-year variation [17]. If a respondent reported using a hormonal and non-hormonal method within the same year, we categorized her has being exposed to the hormonal method. If the respondent reported using a CHC method and progestin-only method in the same year, we dropped that person s data points for that year since we could not imply any kind of relationship when comparing between contraceptive categories. We adjusted these models for CFTR genotype and BMI. We performed logistic regression to assess the odds of outcomes selected a priori related to osteopenia, osteoporosis, or bone fracture in DMPA users compared to non-users, adjusting for BMI. We used Stata IC version 14 for the analysis.

Table 1.

Descriptive characteristics of women with Cystic Fibrosis aged 18–49 years in the CF Foundation Patient Registry who completed contraceptive surveys.^ǂ

	Progestin only1 (N = 35)ǂ Row %	Combined hormonal2 (N = 63)ǂ Row %	Non-hormonal3 (N = 66)ǂ Row %
Demographics
Age (current) (N = 150)
Average age (SD)	30.1 (6.4)	29.6 (6.8)	31.2 (6.4)
20–29 years old	29.2	55.4	44.6
30–39 years old	20.6	30.9	47.1
40–49 years old	11.8	35.3	29.4
Marital Status (N = 142)
Married	16.9	35.4	43.1
Never married/ Single	25.0	48.1	48.1
Cohabitating/ Unmarried, but living with partner	47.1	47.1	47.1
Divorced/Other	12.5	25.0	12.5
Race (N = 148)
White	24.8	40.9	41.6
Non-White	9.1	45.5	72.7
Education (N = 148)
Some high school/ High school diploma/ GED	33.3	27.8	22.2
Some college/ Associates degree	23.5	37.3	43.1
Bachelor s degree	21.6	45.1	49.0
Master s degree/ Professional degree/ Doctorate	21.4	50.0	50.0
Household Income (N = 145)
Less than $15,000	35.7	42.9	32.1
$15,000–49,999	16.1	41.9	54.8
$50,000–74,999	20.0	52.0	32.0
$75,000–99,999	21.7	43.5	52.2
$100,000 or more	23.7	31.6	50.0
Urban/Rural (N = 149)
Rural	25.0	37.5	56.3
Urban	23.3	42.1	42.1
Medical History
CFTR Genotype (N = 145)
Class I/II/III	21.6	42.3	42.3
Class IV/V	42.9	42.9	64.3
Other Class	20.0	35.0	45.0
ppFEV1 % (N = 144)
<50%	26.8	41.5	34.1
50–69%	18.3	33.3	41.7
≥70	23.3	39.5	39.5
BMI (N = 141)
<18.5	16.7	33.3	33.3
18.5–24.9	21.8	37.0	42.9
≥25.0	15.2	33.3	39.4
Lung Transplant (N = 149)	25.0	25.0	33.3
CF-Related Complications
Chronic Renal Insufficiency (N = 67)	38.5	46.2	15.4
Cystic Fibrosis-Related Diabetes (CFRD) (N = 149)	57.1	54.0	53.0
Pseudomonas aeruginosa infection (N = 116)	74.3	76.2	68.2
Mucoid P. aeruginosa (N = 95)	84.6	83.3	86.7
Liver Disease (Cirrhosis or Non-Cirrhosis) (N = 149)	17.4	17.4	26.1
Hemoptysis (N = 145)	20.0	30.0	30.0
Mental Health Disorder (Anxiety and/or Depression) (N = 149)	23.7	37.3	40.7

^ǂNumbers and percentages do not add up to 150 and 100% because respondents could be on multiple types of contraceptive or report varying CF-related complications from year to year (i.e. not every respondent who had hemoptysis, had this complication every year between 2012 and 2016).

¹Hormonal IUD, implant, depot medroxyprogesterone acetate, or progesterone-only pill.

²Oral pill, patch, or ring.

³Non-hormonal IUD, permanent sterilization of self or partner, condoms, diaphragm, cervical cap, fertility awareness, natural family planning, vaginal gel, or exclusive breastfeeding for first 6-months of infant’s life postpartum.

3. Results

Enrollment of study participants was more complicated than initially expected because the survey was inadvertently published on Facebook, resulting in its completion by persons who were not in the CFFPR (Fig. 1). Three hundred and sixty-eight persons completed the survey initially. Of these, 153 were excluded because of incomplete data, male names, or no match with the CFFPR database. We launched a second phase of enrollment. After excluding respondents who were not participants in the CFFPR, we successfully linked 150 contraceptive survey respondents to their clinical data in the CFFPR.

Fig. 1.

Study flow diagram of women with Cystic Fibrosis aged 18–49 years in the CF Foundation Patient Registry who completed contraceptive surveys.

Table 1 describes demographics, contraceptive method use, and characteristics of women using contraception between 2012 and 2016. Most progestin-only users used the LNG-IUD (n = 21/35, 60.0%) and most non-hormonal users used condoms (n = 40/66, 60.1%). There were 36 women who were never on any type of birth control (not shown). Respondents were mostly married (45.8%), white (92.6%), had some college or Bachelor s degree (69.0%), and lived in an urban area (based on zip code) (89.3%). The majority had Class I-III mutations (76.6%), FEV1 70% or greater (59.7%), and BMI of 18.5–24.9 (84.4%). Compared to non-responders of the same age range from the same participating CF programs, the study participants were younger by two years on average, more likely to be married, college-educated, have a higher BMI, and FEV1 of 70% or greater; they were less likely to be of white race (not shown).

Women with ppFEV1 <50% and chronic renal insufficiency used CHC with greatest frequency (41.5% and 46.2%, respectively) (Table 1). Women with CF-related complications, such as a history of hemoptysis (30.0%), CF-related liver disease (26.1%), and associated medical conditions, such as a history of a mental health disorder (40.7%) or lung transplant (8.1%), used non-hormonal methods most frequently (Table 1). Women with CF-related diabetes used progestin-only methods (57.1%) more frequently than CHC or non-hormonal method (54% and 53%, respectively). Most respondents (77.3%) had P. aeruginosa infection, of whom 81.9% had the mucoid phenotype. Between 2012 and 2016, women with CF most commonly used CHC (42.0%), followed by barrier methods (34.0%), long-acting reversible contraception (LARC) (26.7%, of which 23.3% were IUD users and 4.0% were implant users), no method use (24%), DMPA (7.3%), other methods (6.0%), and permanent methods (3.3%) (Fig. 2).

Fig. 2.

Contraceptive method type used 2012–2016 among women with Cystic Fibrosis, aged 18–49 years in the CF Foundation Patient Registry (N = 150).¹ Tubal ligation, Essure, or vasectomy. ² Intrauterine device (IUD), hormonal or non-hormonal.³ Implant. ⁴ (Combined hormonal contraceptive) Oral pill, progesterone-only pill, patch, or ring. ⁵ Depo-Provera injection/shot. ⁶ Condoms, diaphragm, or cervical cap.⁷ Fertility awareness, natural family planning, vaginal gel, or exclusive breastfeeding for first 6 months of infant s life postpartum.

One-third (33.3%) of the respondents had at least one pregnancy in their lifetime, half of whom had at least one unplanned pregnancy (Table 2). Among women who had a pregnancy between 2012 and 2016 (n = 26), 50% had at least one unplanned pregnancy, half of whom were using CHC or condoms in the same year that they reported an unplanned pregnancy (data not shown). The most common pregnancy outcome was live birth (78.6%), followed by stillbirths (defined as fetal loss >20 weeks gestation) at 14.3%, and miscarriage (defined as fetal loss ≤20 week) at 4.8%. Among the 36 women who took CFTR modulators between 2012 and 2016, only two became pregnant.

Table 2.

Proportion of women with Cystic Fibrosis aged 18–49 years in the CF Foundation Patient Registry who completed contraceptive surveys reporting at least one unplanned pregnancy (N = 150).

	N	%
Women with Cystic Fibrosis
Women who were ever pregnant	50	33.3
Women who had at least one unplanned pregnancy, ever	25	50.0
Women who were pregnant between 2012–2016	26	17.3
Women who had at least one unplanned pregnancy, 2012–2016	13	50.0
Pregnant while on a CFTR modulator, 2012–2016	2	7.7
Pregnancy Outcomes
Pregnancy outcomes, ever (N = 84)
Live births	66	78.6
Miscarriages	4	4.8
Still births	12	14.3
Abortions	2	2.4
Pregnancy outcomes, 2012–2016 (N = 35)
Live births	28	80.0
Miscarriages	1	2.9
Still births	6	17.1
Abortions	0	0.0

Table 3 shows the unadjusted and adjusted odds ratios of adverse pulmonary outcomes by contraceptive method type (progestin-only, CHC and non-hormonal), with non-use of contraceptives as the reference category. When adjusted for BMI and CFTR genotype, CHC use was associated with 3.89 greater odds (95% CI 0.20–76.46) of mucoid P. aeruginosa infection compared to no contraceptive use. Progestin-only methods had slightly higher odds of ppFEV1 < 50% (aOR = 1.38, 95% CI 0.04–50.91), having two or more pulmonary exacerbations in a given year (aOR = 1.24, 95% CI 0.31–4.88), and having a mucoid P. aeruginosa infection (aOR = 1.53, 95% CI 0.07–32.22) when compared to no contraceptive use. The adjusted point estimates among non-hormonal contraceptive users indicated lower risk for adverse pulmonary outcomes compared to non-users (aOR 0.38, 95% CI 0.02–9.29; aOR 0.46, 95% CI 0.17–1.75; aOR 0.61, 95% CI 0.05–7.13 for ppFEV1 < 50%, two or more pulmonary exacerbations, and mucoid P. aeruginosa infection, respectively).

Table 3.

Risk for adverse pulmonary outcome (FEV1 < 50%, 2 or more pulmonary exacerbations, or Mucoid P. Aeruginosa infection) by contraceptive method type among women with Cystic Fibrosis, age 18–49 years who used contraception between 2012 and 2016.

Type of contraceptive	FEV1 < 50%		2 + Pulmonary Exacerbations		Mucoid P. Aeruginosa Infection
	Crude OR (95% CI) (N = 135)	aOR (95% CI)* (N = 120)	Crude OR (95% CI) (N = 140)	aOR (95% CI)* (N = 124)	Crude OR (95% CI) (N = 104)	aOR (95% CI)* (N = 91)
None	REF	REF	REF	REF	REF	REF
Progestin-only1	0.73 (0.06–8.57)	1.38 (0.04–50.91)	1.35 (0.41–4.47)	1.24 (0.31–4.88)	7.14 (0.63–81.45)	1.53 (0.07–32.22)
Combined hormonal2	2.00 (0.23–17.15)	0.67 (0.02–21.08)	0.60 (0.21–1.75)	0.47 (0.13–1.63)	4.09 (0.58–28.81)	3.89 (0.20–76.46)
Non-hormonal methods3	2.62 (0.34–20.24)	0.38 (0.02–9.29)	0.36 (0.12–1.08)	0.46 (0.17–1.75)	2.07 (0.32–13.23)	0.61 (0.05–7.13)

^*Adjusted for BMI and CFTR class.

¹Hormonal IUD, implant, depot medroxyprogesterone acetate, or progesterone-only pill.

²Oral pill, patch, or ring.

³Non-hormonal IUD, permanent sterilization of self or partner, condoms, diaphragm, cervical cap, fertility awareness, natural family planning, vaginal gel, or exclusive breastfeeding for first 6-months of infant’s life postpartum.

We also evaluated the risk for bone disease among women with CF who ever used DMPA between 2012 and 2016 (table 4). Adjusting for BMI, the risk for either osteopenia or osteoporosis and DMPA use demonstrated no significant association (aOR = 1.17, 95% CI 0.31–4.41). When we separated osteopenia from osteoporosis, women with CF who used DMPA had a higher risk of osteoporosis (aOR 5.36, 95% CI 1.00–29.12) compared to those who never used DMPA.

Table 4.

Risk for bone disease with 3-month DMPA injectable contraceptive use during years 2012–2016 among women with Cystic Fibrosis, ages 18–49 years in the CF Foundation Patient Registry who completed contraceptive surveys.

	Osteopenia (N = 62)		Osteoporosis (N = 14)		Osteopenia or Osteoporosis (N = 66)
	N (%)	Adjusted OR* (95% CI)	N (%)	Adjusted OR* (95% CI)	N (%)	Adjusted OR* (95% CI)
Never used DMPA (N = 57)**	23 (40.4)	REF	4 (7.0)	REF	23 (40.4)	REF
Used DMPA (N = 11)	4 (36.4)	0.79 (0.20–3.11)	3 (27.3)	5.36 (1.00–29.12)	5 (45.5)	1.17 (0.31–4.41)

^*Adjusted for binary average BMI (<19, ≥19) from 2012 to 2016.

^**Respondents who used LARC or CHC (N = 81) were removed from the 138 who never used DMPA from 2012 to 2016.

4. Discussion

By linking survey data to a national CF registry, we found that similar to prior studies about contraception and CF, the most common contraceptive methods used by our respondents were CHCs and condoms [11,18]. We also found that the percentage of women with CF using LARC between 2012 and 2016 was more than two times that of women in the general female population of similar age and race (26.7% vs 10.6% nationally), while permanent sterilization use, including vasectomy, was about seven times less (3.3% vs 22.7% nationally) and non-contraceptive use rates were slightly lower (24.0% vs 35.1% nationally) [19].

LARC use may be influenced by the fact that many persons with CF enroll in clinical trials in which women with CF are required to use contraception due to the unknown effects of trial medications on fetal outcomes [20]. Women with CF are also often advised by their clinicians to avoid pregnancy due to their disease status [21]. The proportion of women with CF reporting unplanned pregnancy in our cohort was 50%, which is similar to that of Non-Hispanic white women, ages 15–44 years nationally (45%) [22]. Similar to the general US population, the majority of pregnancies for women with CF resulted in live birth. The number of women who reported stillbirths was much higher in our cohort compared to numbers reported nationally and in an earlier report using CFFPR data [23,24]. Because our sample was not representative of a national sample of women with CF, further study of pregnancy outcomes among a more representative sample of women with CF is warranted.

There were inherent weaknesses in this study and thus, our findings should be interpreted with caution. The observational nature of this study and the small number of women surveyed requires that we carefully consider confounding and potential for bias. Baseline pulmonary function likely impacts contraceptive use and bone health. Prior research suggests contraceptive users are more likely to report having milder CF disease than non-contraceptive users [11]. Other important factors that affect pulmonary health include age, treatment adherence, exercise, allergen exposures, socioeconomic and insurance status, and co-morbid health conditions [25–27]. Since the current median age at death among those with CF is 30.6 years, women older than 31 years are likely healthier than those of the average CF population [13]. In interpreting results, we also considered the development timeline for disease complications. Conditions such as osteoporosis develop over time, so contraceptive use for a single year before diagnosis may not accurately reflect the risk of disease development from prolonged contraceptive exposure. Study participants reported having used contraception for an average of 3.5 years out of the total five years for which the survey asked about contraceptive use. About a quarter of respondents already had a diagnosis of osteopenia or osteoporosis in 2012. Likewise, because we did not exclude women who may have had mucoid P, aeruginosa as a diagnosis in 2012, associations between hormonal contraception exposure and mucoid P. aeruginosa are inconclusive.

Additional limitations that threaten our study s internal validity are relevant. Our survey used self-reported information about contraceptive use, which is often inaccurate because of recall bias or social desirability bias [28,29]. Exposure data would have been strengthened if we had been able to verify contraceptive prescriptions or provision with medical chart or pharmacy data, although electronic medical record contraceptive data is variable [30,31]. We did not implement our recruitment strategy per protocol, as the survey was inadvertently available publicly for a short time during the data collection period. Publicly available surveys increase the potential for fraud, including duplicate responses, and collection of false information [32]. Importantly, one of this study s strengths is that we were ultimately able to confirm that each survey respondent was a current participant in the CFFPR.

Despite these limitations, we successfully linked self-reported contraceptive information with CFF national registry data to assess contraceptive use with important CF-related clinical outcomes. Because of the limited numbers of women of reproductive age with complex diseases, registry data may be the most efficient way to evaluate trends in contraceptive use and to assess their impact on disease status. Our study highlights that women with CF choose types of contraception at different rates than adult women within the general population. Our study is also one of the first to inquire about pregnancy intention and outcomes among women with CF. While the absolute number of women reporting prior pregnancies was small, the surprising proportion of unplanned pregnancies in this population raises the need to validate this finding with larger studies. While no definite conclusions can be made regarding hormonal contraceptive use on pulmonary or bone health among women with CF, our preliminary findings suggest a larger trial that adequately assesses risk while accounting for potential confounders is essential.