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Dr. Eliza Buyers: Puberty within the Rett Clinic Population
1. Eliza Buyers, MD, FACOG
Adolescent Gynecology
Childrenâs Hospital Colorado
PUBERTY, PERIODS
&
RETT SYNDROME
Rett Clinic Family Symposium
October 1, 2016
2. Topics
ď˛ Puberty (and myths/misperceptions).
ď˛ Menstrual periods; options to manage if needed.
ď˛ Other concerns:
⢠Awareness/preventionof sexualabuse.
⢠HPV vaccine is recommendedto prevent cancer.
⢠Over 21: considerpap smear.
⢠Vulvar/vaginal care.
⢠Time for questionsand discussion.
3. Why, and why me?
ď˛ âItâ is going to happen...
ď§ Puberty is a normal, healthy process
ď˛ Information is powerful
ď§ For prevention
ď§ Know about options to manage any concerns
ď˛ But, why me?
ď§ Because, you matter most!
4. Puberty
⢠Puberty: a process of physical changes where a childâs
body starts to work like an adult body and can be capable
of reproduction.
⢠Starts when the brain (hypothalamus)releases GnRH
⢠GnRH causes pituitary to release LH and FSH
⢠LH/FSH stimulates ovaries to make estrogen.
⢠Estrogencauses breast growth,changes in the vaginal
epithelium,and growthof the uterine lining.
5. Adolescent Development
ďChanges in many aspects of brain and body.
ďDevelopment is not synchronous.
ďAdolescence continues into the 20âs.
ďNot to be feared!
Cognitive Emotional
Behavioral Social
Physical
6. ⢠Onset of puberty (breast bud) average age 10 (range 7-13)
⢠Menarche usually 2.5 years after breast bud - average 12.5.
⢠Delayed if no breast bud by age 13-14, or no menses 3+ yrs after
breast bud.
Pubertal Events in Girls
⢠Growth of pubic hair (and body odor) can occur without puberty starting.
⢠Early puberty (before 7) more common when neurologic conditions. If
concerns, a pediatric endocrinologist can help sort things out!
7. Menstrual Cycle
⢠Estrogen causes the lining (endometrium) of the uterus to
thicken.
⢠First period occurs when the lining gets thick enough to
bleed (usually 3 years after estrogen exposure).
⢠Periods at first may not be ovulatory (or regular), but still
should come every 21-90 days, not last longer than 8 days
in a row.
⢠The physiologic purpose of periods: 1) make an egg
capable of reproduction (ovulate), and 2) prepare
endometrium for implantation of a fertilized egg.
8. ⢠On-line survey; N= 21 caregivers (all moms)
⢠Average age of daughter: 17; average weight: 80 lbs.
⢠All totally dependent on caregivers for menstrual hygiene.
⢠Common Issues: painful periods (dysmenorrhea), PMS
(irritability), and periods > 7 days
⢠62% had tried hormonal methods
ď48% currently using a hormonal method (70%pills, 20% Depo)
ď33% reported ibuprofen âvery effectiveâ to treat cramps.
ď80% reported that hormonal treatment âvery effectiveâ in
addressing the menstrual concern.
Rett Syndrome and Menstruation
Hamilton. Journal of Pediatric and Adolescent Gynecology, 2012
9. Menstrual Issues
Caregiver Survey Data, JPAG 2016
Angelman Syndrome (AS)
⢠N= 51 caregivers.
⢠68% reported at least 1 moderate to severe symptom.
⢠If epilepsy, over half (62%) did not have increased
seizures with menstrual periods.
⢠If seizures were increased (38%), they were well
controlled with either increased seizure medications or
hormonal methods.
ď Overall, 40% using a hormonal method to control
periods.
ď 88% of respondents satisfied with hormonal method
10. Hormonal Options for Managing Menstrual Periods
⢠All methods contain
progesterone which causes the
lining of the uterus to become
thin (therefore, no medical
reason to bleed).
⢠Stopping periods is safe; uterine
lining does not build up when on
hormonal therapy.
⢠Advantages and disadvantages
of each method.
⢠May have unexpected bleeding,
especially in first 6 months.
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11. Awareness
& Preventionof Sexual Abuse
ď Recognizethat sexual abuse does occur.
ď Seek medical attention(report) if concerned that abuse
may have occurred.
Also:
⢠Teach/ talk about acceptable,safe touch and what is not.
⢠Let her know she can tell you anything; no secrets.
⢠Pay attentionto physical and emotionalchanges.
⢠Ask questions,hold others accountable,and trust your
instincts.
12. Talking to Our Children
ď Many, many âtalksâ
â˘Basics of puberty
â˘Reproduction
â˘Label body parts.
â˘Socially appropriate language
and behaviors.
â˘Body boundaries.
â˘Privacy and limits.
Author: Robie Harris
- Books for ages:
⢠â4 and upâ
⢠â7 and up
⢠â10 and up.â
13. Pap Smears & HPV
⢠HPV is very common virus. Most adults have been exposed,
usually through sexual contact.
⢠HPV is the cause of most cervical, oral, and anal cancers.
⢠Current screening recommendation is for all women to
have a pap smear at age 21. (No guidance for women who
have never had sexual contact).
⢠No other need for a pelvic exam.
----------------------------------
⢠HPV vaccine is recommended for all girls & boys ages 9-26.
⢠Safe for girls with Rett Syndrome and Rett like disorders.
⢠The vaccine prevents oral, anal, and cervical cancer.
14. Vulvar Care
⢠Plain water is best.
⢠Baths (if an option) are really,
really helpful to address any
irritation or discharge.
⢠When possible, avoid soaps and
wipes.
⢠Some pads and products may be
more irritating than others.
15. Pediatric and Adolescent
Gynecology Team
At Childrenâs Colorado
720-777-2667
Tricia HugueletStephen Scott Eliza Buyers
Questions & Discussion
Veronica Alaniz