the birds and the bees: cf and sexuality paul j. turek, m.d. director, the turek clinic emeritus...
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The Birds and the Bees: CF and Sexuality
Paul J. Turek, M.D.Director, The Turek Clinic
Emeritus Professor, UCSF
Sexuality and CF: Women
•Delayed puberty•Amenorrhea•Reduced libido•?Fertility effects
cervical mucus thinning
Consequence of nutritional issues and not CF
Sexuality and CF: Female Fertility
From mouse models:•Smaller litter size•Reduced number of litters
Consequence of:•Smaller uterine, ovarian size•Abnormal estrous cycles•Fewer ovulations
Hodges et al. Endocrinol. 2008, 149, 2790
Sexuality and CF: Pregnancy
•Respiratory status: hypertension, infection•Lung transplants: organ rejection•Glycemic control•General obstetrical and nutritional fitness
Not terribly well delineated at this point
Whitty JE. Clin Obstet Gynecol. 2010, 53: 369-76
Sexuality and CF: Motherhood
•Sleepness nights•Schoolyard sniffles
The biggest threats to Mom’s life:
I read in one of the papers that couples can get pregnant with sex. Is that true?
A patient, 1998
Sexuality and CF: Men
•Fertility•Erections
History
Basic Male Evaluation
Physical
Medical
Therapy
Surgery
Sperm Processing
ART
Semen Analysis
Hormones
Genetic Tests
Other Tests
Normal Male Anatomy
Physical Examination
“Natural Vasectomy”
CBAVD
Test Answers
CF and male fertility is an easier problem!
Infertility M.D. Provider (Male or Female)
Genetic Counseling
PROGENI
Medical Evaluation
Y microdeletions Karyotype CFTR/5TCFTR/5T
Post-Test Counseling
Treatment Plan
The CFTR Gene
The Gene: CFTR, located 7q31.2 5T IVS8 (5T variant)
Function: Encodes the cyclic adenosine monophosphate-regulated chloride channel in secretory epithelia
Problem: 1500+ mutations described
Mullerian and Wolffian DuctRelationships
•Unilateral: most azoospermic•Bilateral: all azoospermic•Involves any segment of Wolffian duct•Seminal vesicle agenesis, cysts•Can have ejaculatory duct obstruction•Prostatic and utricular cysts•If ipsilateral kidney absent:
No CFTR mutations•If ipsilateral kidney present:
80% have CFTR mutations•“Form fruste” of CF
Congenital Absence of the Vas Deferens (CAVD)
Nonobstructive Azoospermia
(36%)Oligospermia
(49%)
CAVD Idiop.Obstruction
(12%)
Other (3%)Other (3%)
PROGENI The First 500 Patients
Techniques for Obstructive AzoospermiaVasal spermEpididymal sperm
PESAMESA
Testis spermTESATESE
Vasal (MVSA; PVSA)
Epididymal (MESA, PESA)
Testicular (TESA, TESE, MicrodissectionTESE)
Obstructive Azoospermia
Obstructive Azoospermia
Guideline: Least invasive, least damaging, best yield.
Since assisted reproduction (IVF-ICSI) does not have a 100% success rate, it behooves reproductive urologists to develop and use sperm-retrieval techniques that are not only reliable and of low morbidity, but that also have the potential to harvest sufficient sperm to enable multiple IVF-ICSI attempts without repeat surgery
Turek et al. Ass Reprod Rev. 1999, 9: 60-64
Microscopic Epididymal Sperm Microscopic Epididymal Sperm Aspiration (MESA)Aspiration (MESA)
The Mini-MESA
•Local +/- sedation•6-10 million TMC •Freeze-all approach
•Mean 2 pain pills•Recovery 1-2 days•<1% complication•1% need repeating
Testicular Sperm Extraction (TESE)
Window Biopsy Technique
HistoryHistoryPhysical ExamPhysical ExamHormonesHormonesSemenAnalysisSemenAnalysis
Obstructive
Non-obstructive
The Problem of Azoospermia
RepairICSI
ICSI
5% of infertile men
Testicular (TESA, TESE, MicrodissectionTESE)
Nonobstructive Azoospermia
Surgeon beware!!
Bx-FNABx-FNA DiscrepancyDiscrepancy
27% FNA 27% FNA ++
Bx Bx --
IntratestisIntratestis VariabilityVariability
-- ++
25% of testes25% of testes
IntertestisIntertestisVariabilityVariability
-- ++
19% of testes19% of testes
How “Patchy” or “Focal” is Sperm Production?
Office FNA MapOffice FNA Map
Sperm Found?Sperm Found?
YesYes NoNo
IVF/ICSIIVF/ICSI"Directed" TESE"Directed" TESE
Donor SpermDonor SpermAdoptionAdoption
Testis FNA Mapping
Fine Needle Aspiration (FNA) Mapping
Other Sexual Dysfunction:Other Sexual Dysfunction:
Erectile dysfunction
Premature ejaculation
Retrograde ejaculation
Low libido (sex drive)
Massachusetts Male Aging Study:Relationship of Prevalence/Degree of ED to Age
DEGREE OF ED
AGE (yr)
0 0.5 1
70
65
60
55
50
45
40
PROBABILITY
Mechanism of ErectionMechanism of Erection
Arterial Dilation Sinusoids fill Venous compression
History + Physical exam + LabHistory + Physical exam + Lab
Appraise pt’s goal, expectation & profileAppraise pt’s goal, expectation & profileDiscuss diagnostic and treatment optionsDiscuss diagnostic and treatment options
Therapeutic trial Further diagnostic testing
Definitive treatment
Evaluation of Erectile Dysfunction
The penis does not obey the order of its master, who tries to erect or shrink it at will. Instead, the penis erects freely while its master is asleep. The penis must be said to have its own mind, by any stretch of the imagination.
Leonardo da Vinci
Remarkably little evidence of female fertility issues.
Clearly defined evidence of male infertility.
Fertility: Planning is important.
CF and Sexual Dysfunction
www.TheTurekClinic.comwww.TurekonMensHealth.com
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