Abstract
Introduction: Turner syndrome (TS) is associated with a high risk of primary ovarian insufficiency. Current guidelines recommend early fertility counseling for affected youth and their families. This study assessed clinical providers’ (MD, NP, or PA) fertility counseling practices for girls with TS. Methods: TS providers were invited to complete a survey via the Pediatric Endocrine Society listserv. Descriptive statistics summarized variables of interest. Correlations were used to identify associations between barriers/practice characteristics and fertility preservation (FP) referral. Thematic analysis was used to examine qualitative responses. Results: 119 providers completed the survey. Seventy percent reported discussing fertility implications of TS routinely in pediatric care. Fifty-six percent reported often or always referring patients with spontaneous menarche to FP specialists, whereas only 19% reported often or always referring their patients without spontaneous menarche (p < 0.001). Barriers associated with FP referral frequency included unfamiliarity with FP options, belief that FP is not a possible goal for their patients, and absence of a local reproductive endocrinologist. Qualitatively, four referral barrier themes were identified: (1) questionable utility of referral, (2) lack of perceived interest among patients/families, (3) provider barriers (e.g., lack of knowledge), (4) logistical/structural barriers to accessing fertility-related care. Discussion/Conclusion: Pediatric endocrinology providers report inconsistently discussing fertility implications of TS. The frequency of referral to an FP specialist and factors/barriers affecting the decision to refer remain variable. Future research should focus on expanding provider education, addressing barriers to high-quality fertility counseling and referral for patients with TS, and investigating FP outcomes in TS.
References
1.
McCarthy
K
, Bondy
CA
. Turner syndrome in childhood and adolescence
. Expert Rev Endocrinol Metab
. 2008
;3
(6
):771
–5
. .2.
Oktay
K
, Bedoschi
G
, Berkowitz
K
, Bronson
R
, Kashani
B
, McGovern
P
, Fertility preservation in women with Turner syndrome: a comprehensive review and practical guidelines
. J Pediatr Adolesc Gynecol
. 2016
;29
(5
):409
–16
.3.
Dabrowski
E
, Jensen
R
, Johnson
EK
, Habiby
RL
, Brickman
WJ
, Finlayson
C
. Turner syndrome systematic review: spontaneous thelarche and menarche stratified by karyotype
. Horm Res Paediatr
. 2019
;92
(3
):143
–9
. .4.
Sylven
L
, Magnusson
C
, Hagenfeldt
K
, von Schoultz
B
. Life with Turner’s syndrome: a psychosocial report from 22 middle-aged women
. Acta Endocrinol
. 1993
;129
(3
):188
–94
.5.
Rafique
S
, Sterling
EW
, Nelson
LM
. A new approach to primary ovarian insufficiency
. Obstet Gynecol Clin North Am
. 2012
;39
(4
):567
–86
. .6.
Logan
S
, Perz
J
, Ussher
JM
, Peate
M
, Anazodo
A
. Systematic review of fertility-related psychological distress in cancer patients: informing on an improved model of care
. Psychooncology
. 2019
;28
(1
):22
–30
. .7.
Armuand
GM
, Wettergren
L
, Rodriguez-Wallberg
KA
, Lampic
C
. Desire for children, difficulties achieving a pregnancy, and infertility distress 3 to 7 years after cancer diagnosis
. Support Care Cancer
. 2014
;22
(10
):2805
–12
. .8.
Oosterhuis
BE
, Goodwin
T
, Kiernan
M
, Hudson
MM
, Dahl
GV
. Concerns about infertility risks among pediatric oncology patients and their parents
. Pediatr Blood Cancer
. 2008
;50
(1
):85
–9
. .9.
Letourneau
JM
, Ebbel
EE
, Katz
PP
, Katz
A
, Ai
WZ
, Chien
AJ
, Pretreatment fertility counseling and fertility preservation improve quality of life in reproductive age women with cancer
. Cancer
. 2012
;118
(6
):1710
–7
.10.
Deshpande
NA
, Braun
IM
, Meyer
FL
. Impact of fertility preservation counseling and treatment on psychological outcomes among women with cancer: a systematic review
. Cancer
. 2015
;121
(22
):3938
–47
. .11.
Gravholt
CH
, Andersen
NH
, Conway
GS
, Dekkers
OM
, Geffner
ME
, Klein
KO
, Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner syndrome meeting
. Eur J Endocrinol
. 2017
;177
(3
):G1
–G70
.12.
Nahata
L
, Woodruff
TK
, Quinn
GP
, Meacham
LR
, Chen
D
, Appiah
LC
, Ovarian tissue cryopreservation as standard of care: what does this mean for pediatric populations?
J Assist Reprod Genet
. 2020
;37
(6
):1323
–6
.13.
Blakemore
JK
, Wei
LS
, Quinn
GP
. Addressing practical concerns surrounding fertility preservation in patients with Turner syndrome
. Fertil Steril
. 2019
;112
(4
):651
–2
. .14.
Silberbach
M
, Roos-Hesselink
JW
, Andersen
NH
, Braverman
AC
, Brown
N
, Collins
RT
, Cardiovascular health in Turner syndrome: a scientific statement from the American heart association
. Circ Genom Precis Med
. 2018
;11
(10
):e000048
.15.
Morgan
TL
, Kapa
HM
, Crerand
CE
, Kremen
J
, Tishelman
A
, Davis
S
, Fertility counseling and preservation discussions for females with Turner syndrome in pediatric centers: practice patterns and predictors
. Fertil Steril
. 2019
;112
(4
):740
–8
.16.
Glaser
BG
. The constant comparative method of qualitative analysis*
. Soc Probl
. 2014
;12
(4
):436
–45
.17.
Ellis
SJ
, Wakefield
CE
, McLoone
JK
, Robertson
EG
, Cohn
RJ
. Fertility concerns among child and adolescent cancer survivors and their parents: a qualitative analysis
. J Psychosoc Oncol
. 2016
;34
(5
):347
–62
. .18.
Nilsson
J
, Jervaeus
A
, Lampic
C
, Eriksson
LE
, Widmark
C
, Armuand
GM
, “Will I be able to have a baby?” Results from online focus group discussions with childhood cancer survivors in Sweden
. Hum Reprod
. 2014
;29
(12
):2704
–11
.19.
Stein
DM
, Victorson
DE
, Choy
JT
, Waimey
KE
, Pearman
TP
, Smith
K
, Fertility preservation preferences and perspectives among adult male survivors of pediatric cancer and their parents
. J Adolesc Young Adult Oncol
. 2014
;3
(2
):75
–82
.20.
Bernard
V
, Donadille
B
, Zenaty
D
, Courtillot
C
, Salenave
S
, Brac de la Perriere
A
, Spontaneous fertility and pregnancy outcomes amongst 480 women with Turner syndrome
. Hum Reprod
. 2016
;31
(4
):782
–8
.21.
Azem
F
, Brener
A
, Malinger
G
, Reches
A
, Many
A
, Yogev
Y
, Bypassing physiological puberty, a novel procedure of oocyte cryopreservation at age 7: a case report and review of the literature
. Fertil Steril
. 2020
;114
(2
):374
–8
.22.
Lampic
C
, Wettergren
L
. Oncologists’ and pediatric oncologists’ perspectives and challenges for fertility preservation
. Acta Obstet Gynecol Scand
. 2019
;98
(5
):598
–603
. .23.
Quinn
GP
, Bowman Curci
M
, Reich
RR
, Gwede
CK
, Meade
CD
; ENRICH/ECHO Working Group
, Impact of a web-based reproductive health training program: ENRICH (Educating Nurses about Reproductive Issues in Cancer Healthcare)
. Psychooncology
. 2019
;28
(5
):1096
–101
.24.
Johnson
EK
, Rosoklija
I
, Shurba
A
, D’Oro
A
, Gordon
EJ
, Chen
D
, Future fertility for individuals with differences of sex development: parent attitudes and perspectives about decision-making
. J Pediatr Urol
. 2017
;13
(4
):402
–13
.25.
Quinn
GP
, Vadaparampil
ST
, King
L
, Miree
CA
, Wilson
C
, Raj
O
, Impact of physicians’ personal discomfort and patient prognosis on discussion of fertility preservation with young cancer patients
. Patient Educ Couns
. 2009
;77
(3
):338
–43
.26.
Nahata
L
, Ziniel
SI
, Garvey
KC
, Yu
RN
, Cohen
LE
. Fertility and sexual function: a gap in training in pediatric endocrinology
. J Pediatr Endocrinol Metab
. 2017
;30
(1
):3
–10
. .27.
Yetman
AT
, Katz
R
, Cramer
J
. Outcome of patients with Turner syndrome living in a rural versus urban setting
. J Am Coll Cardiol
. 2018
;71
(11 Supplement
):A616
–A
. .28.
Besharati
M
, Woodruff
T
, Victorson
D
. Young adults’ access to fertility preservation services at national cancer institute community oncology research program minority/underserved community sites: a qualitative study
. J Adolesc Young Adult Oncol
. 2016
;5
(2
):187
–200
. .29.
Zwingerman
R
, Melenchuk
K
, McMahon
E
, Liu
KE
, Siren
A
, Laferriere
N
, Expanding urgent oncofertility services for reproductive age women remote from a tertiary level fertility centre by use of telemedicine and an on-site nurse navigator
. J Cancer Educ
. 2020
;35
(3
):515
–21
.30.
Marcin
JP
, Shaikh
U
, Steinhorn
RH
. Addressing health disparities in rural communities using telehealth
. Pediatr Res
. 2016
;79
(1–2
):169
–76
. .31.
Daly
C
, Micic
S
, Facey
M
, Speller
B
, Yee
S
, Kennedy
ED
, A review of factors affecting patient fertility preservation discussions & decision-making from the perspectives of patients and providers
. Eur J Cancer Care
. 2019
;28
(1
):e12945
.32.
Zhu
J
, Feldman
HA
, Eugster
EA
, Fechner
PY
, Nahata
L
, Thornton
PS
, Practice variation in the management of girls and boys with delayed puberty
. Endocr Pract
. 2020
;26
(3
):267
–84
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