Part IV
Reproductive Health
Specific to Disability
Module 1 - Physical Disabilities
Spinal cord
injury
Cerebral
palsy
Spina
bifida
Multiple
sclerosis
Sub-Module 1
Spinal Cord Injury (SCI)
Objectives
Spinal Cord Injury - SCI
Upon completion of this module, the participant will be
able to:
• Recognize the specific interaction of menses,
sexuality and menopause in women with spinal cord
injury.
• Describe the onset, symptoms and management of
autonomic dysreflexia (ADR)
• Describe considerations involving pregnancy, labor,
delivery and postpartum for women with SCI
• Identify specific resources available for the patient
with SCI and her provider.
Suzanne
About SCI
Neurological
levels –
• sensation tested at each
dermatome
• strength of 10 key muscles
Classification
of injury-
• complete spinal cord injury
• Incomplete spinal cord
injury
Segmental Spinal Cord Level and
Function
Level
C1-C6
Neck flexors
C1-T1
C3-C5
Neck extensors
Supply diaphragm (mostly C4)
C5-C6
Shoulder movement, raise arm, flex
elbow, supinates arm
Extends elbow and wrist, pronates
wrist
Flexes wrist, supply small muscles of
the hand
Intercostals and trunk above waist
Abdominal muscles
Thigh, hip muscles,
Hamstrings and dorsiflexion of foot
Plantar flexion of foot and toe
movement
C6-C7
C7-T1
T1-T6
T7-L1
L1-L4
L4-S1
L4-S2
Source: Wikipedia, 2008
Function
About Autonomic Dysreflexia
• Autonomic dysreflexia (ADR) is the most
important ob/gyn concern for women with
spinal cord lesions
• Spinal cord lesions at or above T6
segment – 50% incidence of ADR
• Causes severe hypertension
• Potentially lethal medical emergency
Precipitating Factors of ADR
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Bladder or bowel distention or irritation
Cutaneous lesions
Menstruation
Sexual activity
Pelvic and rectal exams
Labor
Signs and Symptoms of ADR
• Hypertension
– Baseline BP in SCI 
90/60
– 120/80 may be
abnormally high
– BP can reach 300/220
• Piloerection
• Flushing
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Pounding headache
Sweating
Nasal congestion
Malaise
Skin tingling
Nausea
Blurred vision
Cardiac dysrhythmia
Management of ADR During GYN
Examinations
Prevention is key
Removal of stimulus/cessation of exam
Assist patient to upright position
Frequent BP monitoring
Loosen clothing
Check bladder/rectum
Administer rapid-onset, short-acting
antihypertensive (e.g.nifedipine, nitrate paste)
Menstruation after SCI
• Usually stop menstruation up to 6 months
following injury, most return to cycling
• Increased incidence of prolonged
amenorrhea
• Increased autonomic symptoms during
menses
• Premenstrual dysphoric disorder (PMDD)
symptoms continued after SCI
Sexual Activity
• Women with SCI can have active and enjoyable
sex lives post injury.
• Lubrication is dependent on level and
completeness of injury.
• Orgasm may be independent
of the level of injury
LINK Part 1 Module 2
Pregnancy and Spinal Cord
Injury
Data on Pregnancy and
Spinal Cord Injury (SCI)
• No studies investigating
fertility and pregnancy
after SCI
• Case-reports and expert
opinion inform obstetric
management of
pregnancy
Pregnancy Complications for
Women with SCI
Urinary
tract
infections
Deep vein
thrombosis
Increased
spasticity
Decubitus
ulcers
Alterations in
pulmonary
function
Constipation
Prevention of Pregnancy
Complications
• Monitor ability to transfer
and ambulate
• Monitor weight gain closely
• Leg elevation
• Range of motion exercises
• Monitor need for increased
services
ADR and Pregnancy/Labor
Prevention
– Avoid distended bladder
– Avoid constipation/fecal impaction
– Discuss past episodes of ADR and
triggers
– Discuss measures to relieve ADR
Link to ADR section (slides 8-11)
ADR v. Preeclampsia
ADR
Preeclampsia
Clonus, edema
Clonus, edema
Acute onset
Variable onset
BP increased during
contractions
BP consistently elevated
Bradycardia
Tachycardia
Proteinuria absent
Proteinuria
Normal labs
Abnormal labs
Intense flushing, gooseflesh,
diaphoresis common
Not prominent
Source: Pereira 2003
Labor and Delivery
• Instruct patient in uterine palpation
and unique symptoms of labor
• Judicious use of early labor regional
anesthesia for sympathetic blockade
• Monitor closely for ADR symptoms
• Increased incidence of operative
vaginal delivery
Postpartum Care
Spinal Cord Injury
• Inspect perineum for signs of
infection. Use ice packs with care.
• Watch closely for postural
hypotension
• Assist with breast feeding,
particularly if low nipple sensation
(link to Part III- Mod 3
breastfeeding)
• Monitor for breast health
Osteoporosis and SCI
• Rapid bone loss of 25-50% in lower
extremities occurs immediately post-injury.
• Spasticity with consequent bone tension
leads to additional bone loss.
• Over 90% of postmenopausal women
have osteoporosis on screening exams
• Treatment should be used, but the safety
of bisphophonates in women with
reproductive potential is still unclear.
Link Part 3 – Module 7
Other Medical Risks with SCI
Decubitus ulcers
(Link – Part 2 Module 2)
Rapid weight loss post injury
(Link Part 3-Module 5)
Superior Mesenteric Artery Syndrome
Renal and bladder stones
Cholithiasis
Case Study
•Considerations when prescribing
contraception
•Precautions during pelvic examination
•Other components of well woman care
Summary SCI
• The most important OB/GYN concern of
SCI is ADR
• Temporary amenorhea is common after
acute SCI
• Intrapartum care with SCI should be
managed by a multidiciplinary team.
• Early regional anesthesia is usually
advised for labor management
• Low bone density is common
References – Spinal Cord Injury
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DeForge D, Blackmer J, Moher D, et al. Sexuality and Reproductive Health Following Spinal Cord Injury.
Summary. Evidence Report/Technology Assessment: Number 109. AHRQ Publication No 05-E003-1, December
2004. Agency for Healthcare Research and Quality. Rockville MD. Downloaded from
http://www.ahrq.gov/clinci/epcsums/sexlspsum.htm. on 8/8/08.
American Spinal Cord Injury Association. Standard Neurological Classification Of Spinal Cord Injury. 2006.
Downloaded from http://www.asia-spinalinjury.org/publications/2006_Classif_worksheet.pdf on 12/5/08
Wikipedia – Spinal cord injury,. Downloaded from http://en.wikipedia.org/wiki/Spinal_cord_injury
on 12/17/08
Campagnolo DI. Autonomic dysreflexia in spinal cord injury. 2006. Downloaded from
http://emedicine.medscape.com/article/322809 on 12/17/08
Jackson AB, Wadley V. A multicenter study of women’s “self-reported reproductive health after spinal cord injury.
Arch Phys Med and Rehab 1999;80:1420-8.
Jackson AB. Medical management of women with spinal cord injury: A review. Topics in Spinal Cord Inj Rehabil
1995;1:11-26.
Research Review, Fall 2000 Published by UAB-RRTC on Secondary Condition of SCI, Birmingham, AL.
Sownloaded from http://www.spinalcord.uab.edu/show.asp?durki=3237 on 8/7/08
Whiple B. Sexual response in women with complete spinal cord injury.. Symposium at INABIS ’98. McMaster
University. Downloaded from http://www.mcmaster.ca/inabis98/komisaruk/whipple0437/tow.html. On 8/11/08
Sipski ML. Sildenafil effects on sexual and cardiovascular responses in women with spinal cord injury. Urology
2000;55(6):812-815
Jackson A, Lindsey L, Llebine P, Poczatek R. Reproductive health for women with spinal cord injury. SCI Nursing
2004;21:88-91.
American College of Obstetrics and Gynecology. Committee Opinion #275 – Obstetric Management of Patients
with Spinal Cord Injuries 2002;ACOG, Washingto n DC.
References SCI
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Pope CS, Markenson GR, Bayer-Zwirello LA, Maissel GS. Pregnancy complicated by chronic spinal cord injury and history
of autonomic hyperreflexia. Obstet. & Gyne. 2002;97:802-3
Pereira L. Obstetric management of the patient with spinal cord injury. Obstetrical and Gynecological Survey 2003;58:67886
Estores IM, Sipski ML. Women’s issues after SCI. Topics in Spinal Cord Injury Rehabilitation 2004;10:107-25
Pentland W, Walker J, Minnes P, Tremblay M, Brouwer B, Gould M. Women with spinal cord injury and the impact of
aging. Spinal Cord 2002;40:374-387
Weiss D. Osteoporosis and spinal cord injury. eMedicine Specialties2008.downloaded from
www.emedicine.com/pmr/topic96.htm. on 8/1/08
Jiang SD, Jiang LS, Dai LY. Management of osteoporosis in spinal cord injury. Clinical Endocrinology 2006;65:555-65.
Vestergaard P, Krogh K, Rejnmark L, Mosekilde L. Fracture rates and risk factors for fractures in patients with spinal
cord injury. Spinal Cord 1998;36:790-6.
Smeltzer, S Zimmerman,V, and Capriotti,T. 2005 Arch Phys Med Rehab 86 (3); 582-6.
Physicians Desk reference (Bisphosphates)
Ornoy A. Wajnberg R. Diav-Citrin O. The outcome of pregnancy following pre-pregnancy or early pregnancy alendronate
treatment. Reproductive Toxicology. 2006;22:578-9
Cowley KC. Psychogenic and pharmacologic induction of the let-down reflex can facilitate breastfeeding by tetraplegic
women: A report of 3 cases. Archives Of Physical Medicine And Rehabilitation 2005;86:1261-4.
Massagli TL, Reyes MR. Hypercalcemia and spinal cord injury. 2008. Downloaded from
http://emedicine.medscape.com/article/322109 on 12/18/09
Moonka R, Stiens SA, Resnick WJ, McDonald JM, Eubank WB, Dominitz, JA, Steizner, MG. The prevalence and natural
history of gallstones in spinal cord injured patients. J AM Coll Surg. 1999;189:274-81
Spina Bifida
Sub-Module 2
Spina Bifida
Objectives
Spina Bifida
• Recognize the specific interaction of
menses, sexuality and menopause in
women with spina bifida
• Describe considerations involving
pregnancy, labor, delivery and
postpartum.
• Identify specific resources available for the
woman with spina bifida and her provider.
Spina Bifida – Case Study
•Desires pregnancy
•Spina bifida lesion at T8
•VP Shunt
•Ileal conduit
Medical Concerns in
Women With Spina Bifida
GI
Orthopedic
Neurologic
Dermatologic
Source: Suzawa, 2006
Urologic
Neurologic Complications
• Hydrocephalus – VP Shunt
• Most have normal intelligence
• Most individuals with SB have strong
verbal skills but have difficulties with
attention and executive functioning.
Urologic and GI Complications
Urologic
• Neurogenic bladder
GI
• Constipation
• Fecal incontinence
• Obesity
Orthopedic Complications
Impaired
mobility
increasing with
age
Overuse
syndromes
Contractures
Osteoarthritis
Spasticity
Spinal
deformities:
kyphosis and
scoliosis
Dermatologic
• Severe latex allergy in 75%
– Unknown etiology
– Latex in many medical,
clothing and household
items
• Pressure ulcers
– Frequent skin examination
(link to Part 2-Mod 2 skin)
– Encourage frequent weight
shifts
Pregnancy Preparation
• Fertility is not impaired
• Genetic Counseling
• Risk of neural tube defect in offspring
– Depends on frequency of occurrence within
the family.
– Small risk of affected pregnancy despite folic
acid prophylaxis
Pregnancy Considerations
• Bladder and urinary tract
– Special care after urinary
diversion surgery
– Increased frequency of
UTI
• VP Shunt failure
• Increased risk of back pain
due to spinal abnormalities
(Link -– Mod 1)
Aging and Osteoporosis
• Kyphosis and scoliosis
increase with age
– Compromise respiratory status
– Complicate positioning for
exams and the interpretation of
bone densitometry.
• Osteoporosis can occur in
childhood and persist into
adult years.
Summary
• Impaired executive functioning
• Bladder and bowel incontinence
• VP shunt may affect pregnancy, delivery
and GYN surgery.
• Genetic counseling and folic acid
• Increased risk of osteoporosis due to
onset of immobility at birth
Case Study – Spina Bifida
1. What more information do you need
from Jennifer regarding her history?
2. What considerations are important for
Jennifer prior to her pregnancy?
3. What are 3 issues related to her spina
bifida that may be of concern during
Jennifer’s pregnancy?
4. What considerations are necessary to
prepare for Jennifer’s delivery?
Resources
• Spina Bifida Association – Information and
publications for providers and public. This
includes the books featured below. Access
at www.spinabifidaassociation.org
References
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Hochber L and Stone J. Etiology, prenatal diagnosis, and prevention of neural tube defects. Up to
Date. 2006
American College of Obstetricians and Gynecologists. Neural tube defects. Practice Bulletin #44
ACOG 2003. Washington DC
Bowman RM, McLone DG, Grant JA, Tomita T, Ito JA. Spina bifida outcome: a 25-year
prospective. Pediatric Neurosurg 2001;34:144-20.
Velde SV, Biervliet SV, Renterghem KV, Laecke EV, Hoebeke P, Winckel MV. Achieving Fecal
Continence in Patients With Spina Bifida: A Descriptive Cohort Study. Journal of Urology. 2007
Suzawa, H. Spina Bifida (powerpoint presentation. 2006. Downloaded from
http://www.bcm.edu/medpeds/powerpoints/Spina%20Bifida.pps#257,3,Epidemiology on 8/13/08
Rose BM, Holmbeck GN. Attention and executive functions in adolescents with spina bifida. J of
Ped Psych 2007;32:983-94.
Liptak GSEvidence –based practice in spina bifida: Developing a research agenda. Presentation
at the conference May 2003. Washington DC. Downloaded from
http://www.spinabifidaassociation.org on 8/13/2008.
Klingbeil H, Baer HR, Wilson PE. Aging with a disability. Arch Phys Med Rehabil 2004;85(Suppl
3) S68-73
Singhal B, Mathew KM. Factors affecting mortality and morbidity in adults spina bifida. Eur J
Pediatric Surg. 1999:9(Suppl 1):31-2.
Levy E. Addressing sexuality in spina bifida. Pediatric News in Entrepreneur.com. Downloaded
from http://www.entrepreneur.com/tradejournals/article/print/168434757.html on 8/14/08
Vogel LC, Krajci KA, Anderson CJ. Adults with pediatric-onset spinal cord injury: part 2:
musculoskeletal and neurological complications. J Spinal Cord Med 2002;25:117-23
Arata M, Grover S, Dunne K, Bryan D. Pregnancy outcome and complications in women with
spina bifida. J Reprod Med 2000; 45:743-748.
Sub-Module 3
Multiple Sclerosis
Objectives - Multiple Sclerosis
• Recognize the specific interaction of
menses, sexuality and menopause for
women with MS
• Describe considerations involving
pregnancy, labor, delivery and
postpartum.
• Identify specific resources available for the
woman with MS and their provider.
Case Study –
Multiple Sclerosis (MS)
•38 years old
•Decreased libido
•Fatigue
•Lack of lubrication
•Decreased sensation
Presenting Symptoms of MS
•Optic neuritis
•Extreme fatigue
•Paresthesias
•Spasticity
•Lower extremity weakness
•Loss of coordination
•Pain
•Acute onset of bowel and bladder
dysfunction
GYN Considerations
• Possible worsening of neurologic symptoms
with menses (self-report)
• 40-80% of women report sexual
dysfunction
• Fatigue commonly contributes to sexual
dysfunction
• Depression may be associated with CNS
changes.
• Smoking may increase disease progression
The Effect of Pregnancy on MS
• No change in fertility
• Symptoms of MS may stabilize
or remit during pregnancy with
20-40% of patients having
relapse within 3 months after
delivery.
• No evidence suggests that
pregnancy affects long-term
course of MS
• Increased risk for child having
MS (2.5X)
Drug Therapy Used for MS and
Pregnancy Category
Drug
Cat
Drug
Cat
Interferon Beta-1a
and 1b
Glatiramer acetate
C Cyclophosphamide
D
B Azathioprine
D
Mitoxantrone
D Corticosteroids
Methotrexate
X Baclofen
B-C
C
MS – Labor and Delivery
• Patient may not recognize labor onset
• Epidural anesthesia does not increase
relapse rate and is effective for treating
labor-induced spasticity
• Weakened maternal expulsive effort
may be indication for operative vaginal
delivery
MS- Postpartum
Breastfeeding encouraged, if no
DMA is needed
Watch closely for perinatal and
postpartum depression
Flareup/relapse often occurs within
3 months postpartum
MS -Osteoporosis
• Frequent use of steroids and
immunosuppressants
increases risk
• Low- trauma fracture rate as
high as 22%
• More than half (53.7%) of
postmenopausal women with
MS were found to have low
BMD on screening (Smeltzer 2005)
MS - Aging
• Older individuals diagnosed
have more progressive MS
• Overlapping symptoms of
MS and aging
• More risk of UTI, pneumonia,
septicemia and cellulitis
• Decrease in cognition due to
disease
Summary: Multiple Sclerosis
• Neurological symptoms may worsen in the
premenstrual period
• Fertility and menstruation are not altered
• Pregnancy and MS:
– Symptoms may stabilize or remit during
pregnancy, relapse after delivery is common
– Overall no long term effect on disease course
• Increased risk of steroid related
osteoporosis
Case Study –
Multiple Sclerosis
•Additional Information
needed?
•Recommendations
Resource
• National Multiple Sclerosis Society http://www.nationalmssociety.org/index.as
px
References - MS
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Benedetto-Anzai MT. Obstetric and gynecological management of women with multiple sclerosis.
Presentation at ACOG Annual Clinical Meeting 2005, San Francisco
Multiple Sclerosis Society. What is Multiple Sclerosis. Downloaded from
http://www.nationalmssociety.org/about-multiple-sclerosis/what-is-ms/index.aspx on 8/13/08
Shabas D, Weinreb H. Preventive healthcare in women with multiple sclerosis. J Women’s Health
& Gender-Based Med 2000;9:389-95
Foley FW. Clinical Bulletin: Assessment and treatmetn of sexual dysfunction in multiple sclerosis.
National Multiple Sclerosis Society 2008. Downloaded from
http://www.nationalmssociety.org/about-multiple-sclerosis/symptoms/sexualdysfunction/index.aspx on 8/14/08
Somers EC. Marder W. Christman GM. Ognenovski V. McCune WJ. Use of a gonadotropinreleasing hormone analog for protection against premature ovarian failure during
cyclophosphamide therapy in women with severe lupus. Arthritis & Rheumatism. 52(9):2761-7
Confavreaux C, Hutchinson M, Hours MM, et al. Rate of pregnancy related relapse in MS. NEJM
1998;339:285-91
Damek D, Shuster E. Pregnancy and MS. Mayo Clinic Proc 1997;72:977-1009
Giesser B. Reproductive issues in persons with multiple sclerosis. Clinical Bulle tin Information for
Health Professionals. National Multiple Sclerosis Society 2003. Downloaded from
www.nationalmssociety.org/PRC.asp. on 8/18/08
Freedman MS. Is spinal anesthesia contraindicated for patients with MS? Medscape: Ask the
experts about Multiple Sclerosis/Neuroimmunology 20002. At http://
www.medscape.com/viewarticle/442083 accessed 1/26/07.
Dilorenzo TA, Halper J, Picone MA. A comparison of older and younger individuals with multiple
sclerosis: A preliminary investigation. Rehabilitation Psychology 2004;49:123-5.
MS- References Con’t.
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DiLorenzo TA. Aging with Multiple Sclerosis Clinical Bulletin.National Multiple Sclerosis Society
2006. Downloaded from http://www.nationalmssociety.org/for-professionals/healthcareprofessionals/publications/clinical-bulletins/download.aspx?id=167 on 8/22/08
Stern, M. Aging with multiple sclerosis. Physical Medicine and Rehabilitation Clinics of North
America 2005:16:219-34
Fleming ST, Blake RL. Patterns of comorbidity in elderly patients with multiple sclerosis. Journal
of Clinical Epidemiology 1994;47:1127-32
Kneebone, II, Dunmore EC, Evans E. Symptoms of depression in older adults with multiple
sclerosis: Comparison with a matched sample of yournger adults. Aging and Mental Health
2003;7:182-5.
Schwid SR, Goodman JE, Puzas, McDermott MP, Mattson DH. Sproadic corticosteroid pulses
and osteoproosis in multiple sclerosis. Archives of Neurology 1996;53:753-7. downloaded from
http://archneur.ama-assn.org/cgi/content/abstract/53/8/753 on 8/21/08
Smeltzer, S Zimmerman,V, and Capriotti,T. Osteoporosis risk and low bone mineral density in
women with physical disabilities .Arch Phys Med Rehab 2005; 86 : 582-6.
Sub-Module 4
Cerebral Palsy
www.ucp.org/uploads/Heather%20Dibblee%2007.jpg
Objectives – Cerebral Palsy
• Recognize the specific interaction of
menses, sexuality and menopause in
women with cerebral palsy
• Describe considerations involving
pregnancy, labor, delivery and
postpartum.
• Identify specific resources available for the
woman with cerebral palsy and their
provider.
Case Study – Cerebral Palsy
• 40 years old
• CP with speech and
cognitive impairment
• Spasticity and some
contractures
Characteristics of
Cerebral Palsy (CP)
• Disorder of motor function arising from
insult or injury to the developing brain
• Characterized by abnormal muscle tone,
deep tendon reflexes, and posture
• Variable degree of cognitive and
communicative impairment
• Seizure disorder present in 30%
• Vision and hearing centers may be
involved
• Growing number of adults with CP
Health Status of Women with CP
Health behaviors, high majority
• Non-smoker, Non-drinker
• Participate in some physical activity
Associated conditions:
•
•
•
•
Cognitive impairment (33%),
Learning disabilities (25%)
Seizure disorders (40%)
Pain ( 84%)
Secondary conditions:
• Pain, hip and back deformities
• Bowel and bladder problems
• Poor dental health, GE reflux
Source: Turk 1997
CP - Reproductive Health Issues
• Increased spasticity and incontinence during
menstruation reported
• Seizure medications may interfere with
contraception methods – (Link Part 3-Mod 1)
• Pain and contractures may affect sexuality
• Contractures and deformities may require
adaptive measures for mammograms (link Part 2Mod 2) and GYN examinations. (link Part 2-Mod 1 )
• Developmentally appropriate sex and
contraception education
CP-Pregnancy
• Expected pregnancy changes:
– mobility, pain
– bladder and bowel control and
function
• Monitoring/adjustment of
medications (Link Part 3 Module 3)
• Influence of contractures and
spasticity on positioning for exams,
labor analgesia, and delivery (Link
Part 2-Module 1)
• Mobilization of parenting supports
• Pregnancy outcomes appear to be
normal
Link Part 3- Module 3
CP –Osteoporosis
• Osteoporosis at early age, due
to non-use of bones
• Increased non-traumatic
fractures
• Use of upright or semi-prone
standing in young adults may
improve some bone mineral
density.
• Treatment considerations (Link
Part 3-Module 7)
CP – Aging
Increase in secondary conditions due to aging
Bladder and bowel dysfunction
Oral motor and GE reflux problems require dietary adjustment
Increased risk for bone, muscle and joint diseases due to
prolonged spasticity
Chronic pain
Decreased ability to ambulate due to fatigue and gait
inefficiency
(Link to Part 3 – Module 7)
Case Study – Cerebral Palsy
• What more
information do you
need?
• What will assist your
examination?
• What preventive
health measures are
needed?
Summary – Cerebral Palsy
• Symptoms of irregular muscle tone and reflexes.
usually associated with spasticity.
• Increased symptoms during menstruation.
• Chronic pain
• Examinations can be facilitated by alternative
positioning and antispasmodics
• Pregnancy planning will likely require medication
adjustment
• Osteoporosis at early age
References – Cerebral Palsy
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Turk, MA, Geremski CA, Rosenbaum PF, Weber RJ. The health status of women
with cerebral palsy. Arch of Physical Medicine and Rehabilitation 1997;78 (Supp 5)
S10-17
Poulos AE, Balandin S, Llewellyn G, Dew AH. Women with cerebral palsy and
breast cancer screening by mammography. Archives of Physical Medicine and
Rehabilitation 2006;87:304-7.
Smith RA, et al. American Cancer Society guidelines for breast cancer screening: update
2003. CA Cancer J Clin 2003;53:141-69
Winch r, Bengtson L, McLaughlin j, et al. Women with Cerebral Palsy: Obstetric experience
and neonatal outcome.Developmental Medicine in Child Neurology.1993;35:974-82
Klingbeil, H. 2004 Arch Phys Med Rehab 85 (3); 68-73.
Caulton, J et al. 2004 Arch Dis Child 89;2;131-5.
Resource: United Cerebral Palsy Association http://www.ucp.org/
Sub-Module 5
Other Physical Disabilities
Osteogenesis Imperfecta
Post-Polio Syndrome
Objectives –
Other Physical Disabilities
Following this sub-module the participant will
be able to:
• Identify some women’s health care
implications of osteogenesis imperfecta and
post-polio syndrome
• Discuss women’s health care management
strategies for those with osteogenesis
imperfecta and post-polio syndrome
• Identify resources and references on these
disabilities for the provider and patient.
OI – Reproductive Health
•
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•
•
May experience heavy bleeding
Fertility rate not influenced
Genetic counseling
Cesarean delivery may be
recommended to reduce pelvic
bone fracture and for diminished
pelvic outlet
• Anesthesia consult
• Hernia prevention with
permanent suture to close
aponeurotic tissues
Post-Polio Syndrome (PPS)
• Affects up to 50% of polio survivors years after
recovery from an initial attack.
• Symptoms:
– increasing muscle weakness
– fatigue
– often pain.
• Slowly progressive.
Post-Polio Syndrome (PPS)
Women’s Health
• Increased risk of osteoporosis
(Link Part 3,
Mod 7)
• Accelerates physiological effects of
menopause
– Compounded muscle weakness leads to poor
balance
– Weakening chest wall leading to decreased
pulmonary function
– Atrophic changes in urinary tract leading to
incontinence (link Part 3, Mod 4)
– Joint stiffness and fatigue leading to sexual
dysfunction (Link Part 1, Mod 2)
Resources/References
Resources
Osteogenesis Imperfecta Foundation –http://www.oif.org
Post-Polio Health International - http://www.post-polio.org
References
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Osteogenesis Imperfecta Foundation. OI Issues: pregnancy. 2007. Accessed at http://oif/org on
1/13/09.
Robetts JM, Solomons CC. Management of pregnancy in Osteogenesis Imperfecta: New
perspectives. Obstet and Gynecol 1975;45:168-70
Vogel TM, Ratner EF, Thomas RC, Chitkara U. Pregnancy complicated by severe osteogenesis
imperfecta: A report of two cases. Anesthesia and Analgesia 2002;94:1315-17.
March of Dimes. Post-Polio Syndrome Quick feference : Fact sheets. Downloaded from
http://www.marchofdimes.com/printable Articles/14332_1284.asp. on 2/10/09
Jubelt B, Agre JC. Characteristics and management of Postpolio Syndrome. JAMA 2000;284:41214
Welner SL, Simon JA, Welner B. Maximizing health in menopausal women with disabilities.
Menopause 2002;9:208-19
Module 2
Intellectual and
Developmental Disabilities
(IDD)
Case Study- IDD
Sexual abuse
Pregnancy
Seizure medications
Sex education
Contraception
Objectives
Intellectual and Developmental Disabilities
After completing this module, the participant will be
able to:
• Identify special considerations in taking a history when
working with women with IDD
• Describe appropriate methods of providing sex
education
• Discuss how to gain cooperation of the woman with IDD
during a GYN examination
• Describe the impact of menstruation and surgical
procedures for women with IDD
• Discuss resolution for issues of informed consent for
women with IDD
Definition - Intellectual and
Developmental Disabilities
Includes at least 3 of the following limitations:
•Self care
•Language
•Learning
•Mobility
•Economic self-sufficiency
•Self direction
•Independent living
Classified according to intelligence quotient
83-52
Mild
51-36
Moderate
Below 36
Severe
Reproductive Care Issues
History taking and education
Physical examination
Menstruation issues
Contraception –(link to Part 3, Module 1)
Pregnancy
Informed consent
Aging and osteoporosis
Issues Seeking Health Care
Resistance to exams due to history of forced
examinations causing
Pain
Anxiety
Hostility
Establishing Communication
• Establish face to face contact with nonhurried manner
• Choose an environment with the fewest
distractions
• Use basic language and establish contact
with the patient
• Assess how the non-verbal patient
communicates
• Assess patient understanding
Taking A GYN History
• Gain information first from patient and
then from other means available.
• Use menstrual and PMS calendars
• Use drawings/photos/anatomic models to
– assess level of body and sexual
knowledge
– assess level of sexual activity and
possibly abuse
– to explain GYN examination
Health Education
• All women benefit
from reproductive
health education
• Excellent resources
are available:
– Women Be Healthy
– Let’s Talk About
Health: What Every
Woman Should Know
Consent and Sexual Relations
No standard screening but the following understanding
should be assessed:
– That sex is an activity that both participants want and engage in
voluntarily.
– That no one can force or threaten you to have sex.
– That you can refuse to engage in sexual activity with someone
even if you have agreed to engage in it before with the same
person, and that it is enough to just say "no" without having to
provide justification for the refusal.
– That sex is usually engaged in private
– That it is not proper to have sex for money or gifts
– That it is not proper to have sex with children, immediate blood
relations or animals
Adapted from : Griffiths (Ed) Ethical Dilemmas: Sexuality and Developmental Disability, 2002
Sexuality and Sex Education
• Have same sexual needs as the general
population
• Parental and society uncomfortable with
sexual needs and expression
• Keep sex education simple and concrete
with repetition and demonstrations.
• Teach avoidance strategies for sexual
abuse.
• See Part 1, Module 2 – Sexuality (link)
Menstruation and Menstrual
Hygiene
• Menarche is often early
• Teach menstrual hygiene in a repetitious
step-by-step manner
• Most women who manage their own
toileting can be taught to self-care for
menses
• Use hormonal intervention only
– After behavioral intervention has failed
– If menses interfere with patient’s quality of life
Cyclical Behavior Changes
• Occurrence –16% menstruating women
with IDD
• Symptoms –temper tantrums, crying,
autistic or self abusive behavior,
seizures
• Diagnosis - Documentation
• Therapy –
– First NSAIDs (behavior may be due to
cramps)
– Then try OCPs, DMPA, SSRIs
Source: Quint 1999
Abnormal Uterine Bleeding
• Menorrhagia common with Down
syndrome, hypothyroidism and obesity
• Antipsychotic medications linked to
hyperprolactinemia
• Menstrual calendars and
pad counts assist diagnosis
• Non-surgical management
See Part 3, Module 2
The GYN Examination
• See Part 2, Module 1 for preparation and
positioning tips (link), particularly avoid the
use of stirrups
• Give options of who will accompany her.
• If the patient can tolerate a speculum, use
a small bladed Huffman or Pedersen
• Use a modified bimanual examination (one
finger) through the vagina or rectum
Cervical Cancer Screening
• Guidelines same as
general population
• Often inadequate past
medical history to identify
HPV risk
• Alternative to speculum
exam is a blind Pap smear
• Resolving institutional
requirements
Ultrasound
• Use for screening vs. medical
indication
• Issues for this population:
– Bladder filling
– Patient cooperation
• Does not reveal condition of the
cervix or vagina including
lesions, discharge or bleeding.
Sedation vs. Anesthesia
Anesthesia only used for indicated examination,
based on complaints
If anesthesia is to be used for another procedure,
plan pelvic exam at that time with other medical
and/or dental providers
No data on utility of sedation
No data on the emotional effects
Surgical Procedures
• Guidelines for hysterectomy and
endometrial ablation same as
general population
• Endometrial ablation should not
be used in the younger
population for menstrual hygiene
• Federal, state and local
regulations
Considerations for Surgical
Procedures
If hospitalized, attempt to arrange
24 hour care of a known caregiver
Provide adequate pain relief,
especially if the woman is nonverbal
Staples for wound closure should
be avoided
Informed Consent
• Assessment of capacity to
consent requires multiple
criteria
• Despite ability to consent –
important to get patient’s assent
• Be alert to coercion and conflict
of interest
• Process often involves state
and jurisdictional statutes
Sterilization
• Legal /ethical issues
• Exhaust other possibilities: i.e. hormonal
preparations and patient education.
• Jurisdictional differences govern guardian
and parental requests
• Patient autonomy is key: “The presence of
a mental disability does not, in itself, justify
either sterilization or its denial”
•
Source: ACOG Committee Opinion #371, 2007
Pregnancy and Parenting
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Fertility
Genetic Counseling
Delay in seeking prenatal care
Anti-seizure medications
Education on impact of pregnancy on
the body
• Concerns for labor and delivery
• Care of the child after delivery
IDD-Osteoporosis
Greater risk for women with IDD due to:
•
Menstrual irregularities
•
Hypothyroidism
•
Use of anticonvulsants
•
Use of steroids
•
Earlier menopause
•
Greater inactivity
•
Vitamin D deficiency
Down Syndrome (DS)
Most common genetic disorder. Secondary
conditions include:
•
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Congenital heart disease – 50%
Altered immune response
Hypothyroidism – 20%
Auditory disorders – 60 to 70%
Obstructive sleep apnea – 50%
Altered vision – 50%
Mental illness – particularly depression and
obsessive compulsive disorder and dementia
DS - Reproduction
Menstruation onset and menstrual
irregularities similar to general
population
Impaired fertility has been noted
Only a few pregnancies have been
described in women with DS with
varying outcomes
DS - Aging
•
•
•
•
Early aging
Frequent early dementia
Early menopause
Osteopenia and
osteoporosis
Intellectual and Developmental
Disabilities Summary
• Assure confidentiality and autonomy to the
maximum the situation allows
• Provide sex and health education that is age
and developmentally appropriate
• Menstruation has impact on the patient and
caregivers.
• Acting out behavior is often a response to
physical or psychological conditions
• Premature aging and osteoporosis
IDD
Case Study Discussion
Sexual Abuse
Pregnancy
Seizure Medications
Sex education
Contraception
Resources - Curricula
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Women Be Healthy – Curricula for teaching general and reproductive
health education – University of NC at Chapel Hill – one copy free of
charge. Order at
http://www.fpg.unc.edu/~ncodh/WomensHealth/week2.cfm
Let’s Talk About Health – What Every Woman Should Know – Illustrated
170 pg. workbook, DVD, audio tapes. Author: Caryl Heaton, DO. The ARC
of New Jersey contact Dianne Flynn – dflynn@ARCNJ.org or call 732-2462525 x 28.
Sexuality education videos for persons with developmental disabilities.
Choices, inc. Making Connections and Person to Person www.johncarmody.net/clients/choices/
Through the Looking Glass – has a variety of publications and services to
assist women with disabilities with pregnancy and parenting issues.
http://lookingglass.org/index.php
Management Guidelines: Developmental Disability, Version2, 2005.
Therapeutic Guidelines Ltd. N. Melbourne Aust. www.tg.com.au
References
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Quint EH. Gynecological health care for mentally disabled women. Presentation at NASPaG Meeting, May
1996.
Muram D, Elkins TE. Reproductive health care needs of the developmentally disabled. In: Sanfilippo JS,
ed. Pediatric and Adolescent Gynecology, Philadelphia, PA. WB Saunders Co, 1994:490-498
Bradshaw, KD, Elkins TE, Quint EH. The patient with mental retardation: Issues in gynecologic care.
University of Texas Southwestern Medical Center, Dallas Texas. 1996.
Edwards JP, Elkins TE. Just Between Us: A Social Sexual Training guide for Parents and Professionals
with Concerns for Person with Developmental Disabilities. Austin, TX: Pro_Ed. 1988.
Lennox N, Beange H, Davis R, Duvasula S, Edwards N, Graves P, et al. Management guidelines,
Developmental Disabilities, 2nd ed. Melbourne, Australia: Therapeutic Guidelines, 2005. Downloaded from
www.tg.com.au/index.php?sectionid=100 on 1/6/08
Lee JK, Saw HS. Can human papilloma virus DNA testing substitute for cytology in the detection of highgrade cervical lesions? Arch Pathol Lab Med. 2004;128:298-302.
American College of Obstetricians and Gynecologists. Practice Bulletin #45: Cervical cancer screening.
ACOG 2003
American College of Obstetricians and Gynecologists. Access to Reproductive Health Care for women with
Disabilities in Special Issues in Women’s Health Care. ACOG, Washington DC, 2004.
Kavoussi SK, Smith YR, Ernst SD, Quint EH. Cervical cancer screening with liquid cytology in women with
developmental disabilities. J of Women’s Health 2009;16:115-8.
Quint EH, Elkins TE. Cervical cytology in women with mental retardation. Obstet Gynecol 1997;89:123-6
Rosen DA, Rosen KR, Elkins TE, Anderson HF, McNeeley SG, Song C. Outpatient sedation: An essential
addition to gynecologic care for person with mental retardation. Am J Obstet Gynecol. 1991;164:825-828
Pueschel SM, Jackson IMD, Giesswein P, et al. Thyroid function in Down syndrome. Res Dev Disabil Res
1991;12:287-96.
References , Con’t.
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Down’s Syndrome Medical Interest Group. Medical Series No. 2: Thyroid disorder among people
with Down’s Syndrome: Notes for doctors. Down’s Syndrome Association 2006. Downloaded
from www.dsmig.org.uk. 1 on 0/3/08
Elkins T. gynecologic care. In: Purschel SM, Pueschel JK, eds. Biomedical Concerns in Persons
with Down Syndrome. Baltimore, MD: Paul H. Brookes Publischin gCol, Linc.: 1992: pgs 139-46.
American College of Obstetricians and Gynecologists. Committee Opinion No. 371: Sterilization
of women, including those with mental disabilities. ACOG, 2007, Washington, DC.
In re Montgomery, 311 N.C. 101,316, E.E.2d 246 (1984).
Hayman RL. Presemptions of justice: Law, politics and the mentally retarded parent. Harvard Law
Review. 1990;103.
National Institute on Child Health and Human Development. Facts About Down Syndrome.
Downloaded from
http://www.nichd.nih.gov/publications/pubs/downsyndrome.cfm?renderforprint=1 on 10-6-08.
deHingh YC, van der Vossen PW, Gemen EF, Mulder MB, et al. Intrinsic abnormalities of
lymphocyte counts in children with Down syndrome. J Peds 2005;147:744-7.
Smith DS. Health care for adults with Down syndrome. 2001. Downloaded from
http://healthlink.mcw.edu/article/1001820316.htm. on 10/6/08
National Health Service Direct. Down’s syndrome complications. 2008. Downloaded from :
http://www.nhsdirect.nhs.uk/articles/article.aspx?articleId=136&sectionId=6 on 10/7/08
Elkins TE, Anderson FH. Sterilization of persons with mental retardation. JASH. 1992;17:19-26
References
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Meijboom F, Szatmari A, Utens E, Deckers JW, Roelandt JR, Bos E, Hess J. Long-term follow-up after surgical
closure of ventricualr septak defect in infancy and childhood. J Am Coll Cardiol. 1994;24:1358-64.
Tager-Flusberg, H. Neurodevelopmental Disorders , MIT Press, Cambridge MA. 1999.- Pg 163
Wilkinson JE, Culpepper L, Cerreto M. Screening tests for adults with intellectual disabilities. J Am Board Fam
Med. 2007;20:399-407.
Management Guidelines: Developmental disability Version 2Therapeutic Guidelines Ltd. N. Melbourne Australia.
2005, Pg. 230
Schupf N, Pang D, Patel BN, Silverman W, Schubert R, Lai F, Kline JK, Stern Y, Ferin M, Tycko B, Mayeus R.
Onset of dementia is associated with age at menopause in women with Down’s syndrome. Ann Neurol
2003;54:433-8
Ranganath R, Rajangam S. Menstrual history in women with Down syndrome – A review.. Indian J of Human
Genetics 2004;10:18-21
Bovicelli L, Orsini LF, Rizzo N, Montacuti V, Bacchetta M. Reproduction in Down syndrome. Obstet Gynecol
1992;59(Supple)13S-17S.
Van Dyke DC, McBrien DM, Sherbondy A. Issues of sexuality in Down syndrome. Down Syndrome Research and
Practice. 1995;3;65-69
Schrager S. Epidemiology of osteoporosis in women with cognitive impairment. Mental Retardation 2006;44:20311.
Dinerstein RD, Herr SS, O’Sullivan . A guide to Consent. 1999, Washington DC. ;American Association on mental
retardation.
Module 6
Sensory Disabilities
Hard of Hearing
Low Vision
Case Study –
Sensory Disabilities
Previous adverse experience
with pelvic exam
Communicates via sign
language but also does some lip
reading.
Acute symptoms requiring
evaluation.
Objectives –
Sensory Disabilities
At the completion of this module the
participant will be able to:
• Describe unique strategies in health care
delivery for women who are hard of
hearing
• Describe unique strategies in health care
delivery for women who have visual
impairment.
Hearing Impairment Overview
• 9% of US Population (Ries, 1994)
• The patient’s age, place of birth, the timing and
amount of hearing loss and preferred
communication modality determines
communication strategies:
– Hard of hearing
– Deaf who communicate orally
– Deaf who communicate using sign language
Communication Barriers for the Deaf or
Hard-of-Hearing
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•
•
Lip reading
Masks
Note writing
Reading
comprehension
• Family as interpreters
• Automated telephone
systems
Communication Facilitators for the Deaf
and Hard-of-Hearing
• Free telephone relay
services
• Text messaging
• Discounted
communications
equipment
• Tax incentives for
providing
accommodation
Suggestions for Communication
• ASK patient how she prefers to
communicate
• Prepare written easy to read instructions
for basic office procedures
• Minimize back ground noise
• Have good lighting
• Be discrete in public setting – No raised
voices
• Inform patient before touching or moving
Hard of Hearing
ADA Requirements
• Provision of a qualified interpreter
• Flexibility in policy, procedure and
practice
Hard of Hearing OB/GYN
Health Issues
• May have decreased family history
information
• Face masks during delivery and surgical
procedures inhibit lip reading
• Assure means for follow up
communication
Communication Suggestions for
the Blind and Low Vision
• Don’t make assumptions about functional effects
of visual acuity
• Do not touch or remove mobility aids
• Describe procedures before performing them
• All written forms and documents should be read
aloud in a private setting
• Reading aloud may not provide effective
communication for some patients
• Use preferred techniques when handling money
or credit cards
Health Information Considerations for
the Visually Impaired
• Assure patient information is in a form
patient can utilize
• Formats include : braille, large-print texts,
audiotape recordings, videotapes or DVDs
with oral descriptions and computer
diskettes (Iezzoni 2006)
• See resources section to access large
type, braille and recorded patient
information.
Pregnancy, Labor and Delivery
• Assure that an appropriate means of
communication is established in each
setting
• Cues for anticipated needs
• Contact procedures for off hour emergencies
• Determining the presence of bleeding or
discharge for pregnant women with low
vision
• Establish a plan for labor and delivery
Working with Service Animals
Service animals should
remain with their owner
whenever possible
Case Study –
Sensory Disabilities
• Communicates with
ASL, no interpreter
available
• Fearful due to past
experience
• Current pelvic disorder,
requiring evaluation
Summary – Sensory Disabilities
• Assuring accurate communication is key to
patient care
• ASK the patient what will help in the situation
• Utilize technology available
• ADA requirement to provide qualified interpreter
and other office accommodations.
References –
Sensory Impairment
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Ries PW. Prevalence and characteristics of persons with hearing trouble: United States, 199091. Vital Health Stat. 10. 1997
Barnett S. Communication with deaf and hard-of-hearing people: A guide for medical education.
Academic Medicine2002;77:694-700
National Institutes of Health. Healthy People 2010: #28, Vision and Hearing. Downloaded from
http://www.healthypeople.gov/Document/HTML/Volume2/28Vision.htm on 6/6/08
Steinburg AG, Sullivan VJ, Montoya LA. Lipreading the stirrups: An investigation of deaf women’s
perspectives of their health, health care, and providers. Paper presented at National Health
Service Corps 25th Anniversary Meeting in Washington, DC 1998.
Iezzoni LI, O’Day BL. More than Ramps: A Guide to Improving Health Care Quality and Access
for People with Disabilities. Oxford University Press, New York, NY 2006.
Price,S. Legal Briefings: Service animals under the ADA. ADA & IT Technical Assistance Centers
2006. Downloaded from http://www.equipforequality.org/resourcecenter/ada_serviceanimals.pdf
on 10/20/08
Meador HE, Zazove P. Health care interactions with deaf culture. J American Board of Family
Medicine 2005;18:218-22
Resources
American Council of the Blind
http://www.acb.org/resources/index.html
American Foundation for the Blind:
National Association of the Deaf –http//: www.nad.org
Pregnancy information DVD with Signing for the Hard of Hearing –
“Your pregnancy and what to expect” contact Harris
Communications www.harriscomm.com click on consumer
education.