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Lifestyle For Health:
Adult Concerns
Health Care for Adults with
Spina Bifida
Mark Merkens, M.D.
Associate Professor of Pediatrics
Director - Spina Bifida Program
Oregon Health & Science University
Portland, Oregon
Thomas S. Webb, MD, MSc
Asst. Professor of Clinical Medicine and Pediatrics
Cincinnati Children's Hospital
Cincinnati, Ohio
Adults with Spina Bifida are
Pioneers
• Very little research or reports on the care of
adults.
• Not many clinics for adults with Spina bifida.
Objectives
• Discuss
bifida.
• Discuss
• Discuss
• Discuss
health care issues of adults with spina
health care skills.
ways to promote healthy living.
Personal Health Care Record.
Changes in your Body
• Changes can occur in all
the body systems.
– Some are directly due
to Spina Bifida.
– Some are caused by
lifestyle choices.
– Some are due to
aging.
NORMAL AGING
• Muscular
– Decreased strength, endurance, flexibility.
• Nervous system
– Deceased sensation, balance, cognitive.
• Skeletal
– Arthritis, osteoporosis, contractures.
• Cardiovascular
– Decreased cardiac output
– Vascular changes.
NORMAL AGING
• Skin
– Thinning, less elastic.
• Metabolism
– Slows, tendency to gain weight.
• Continence
• Pain
– Musculoskeletal, nerve compression or damage.
• Mental Health
– Limited horizons, future.
– Vascular changes of aging.
Aging in Spina Bifida
• Uneven pressure on joints.
– Earlier arthritis and pain.
• Diminished muscle strength & endurance.
– Decreased ambulation, mobility, transfers.
• Diminished nerve function.
– Decreased sensation.
– Decline in circulation in legs & feet.
Aging in Spina Bifida
• Decreased skin tone.
– Increased risk of decubitus ulcers, abrasions.
• Osteoporosis in higher level Spina Bifida
from lack of bone stimulation from walking.
• Risks of recurrent fractures.
• Obesity
– From decreased mobility.
– Causes decreased mobility.
Health Care Problems
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Shunt Failure
Chiari Compression
Tethered Cord
Urinary
Bowel
Orthopedics
Skin
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Heart and lung
Obesity
Exercise
Sexuality
Pain
Mental Health
Hypertension (high
blood pressure)
• Aging
Shunt Failure
• Even if the shunt has
“not been working” for
years.
• Trauma
• Same symptoms
• Adult neurosurgeons
may not think or believe
it.
• Any neurological
problem:
– “It’s the shunt, stupid.”
Chiari-II Brainstem Compression
• Likely to affect apnea and respiratory drive.
• Might be increasingly frequent pneumonias.
• Chronic oxygen requirement, asleep, then
continuous.
• Less likely to be primary event.
– More likely to be recurrence.
• “It’s the shunt, stupid!”
Tethered Spinal Cord
• What is it?
– Spinal cord can’t move
freely because scar
tissue, small cysts or
fatty growths.
• Tethered means “attached
or bound together”
• You can develop a tethered
spinal cord again.
Tethered Cord:
Signs & Symptoms
– Change in urinary pattern
– Change in bowel pattern
– Increased trouble walking
– Pain in the back, neck, leg
– Change in muscle tone of the lower legs
– Change in sensory level
– New / persistent decubitus ulcers
– You develop new or worsening scoliosis
(curvature of the spine)
Spinal Cord Tethering
• Adult providers may not know or think of it.
– A literature report of a 72 year old ambulatory
person, lost ambulation.
• Less likely to be primary event.
– More likely to be recurrence.
• “It’s the shunt, stupid!”
Urinary System
Kidneys remove wastes from the
blood

Flush wastes out in urine
↓
Down the ureter to the bladder
↓
bladder stretches like balloon

Sends message to brain that it’s
full, needs emptying
↓
Brain sends message to open
valves- urethra- and empty
bladder
Kidneys remove wastes from the
blood

Flush wastes out in urine
↓
Down the ureter to the bladder
↓
bladder stretches like balloon

Sends message to brain that it’s
full, needs emptying
↓
Brain sends message to open
valves-urethra- and empty
bladder
With spina bifda, messages
don’t get through
- the bladder may become
rigid,
-valves don’t work very well
- can even be bladder spasms
Reflux
• Sometimes urine
flows back into to the
kidneys from the
bladder.
• Can cause
– Kidney infection
– Kidney damage
Urinary System – Other Problems
• “Bladder augmentation with intestine or
stomach
– Cancer conversion?
– Produces mucous – blocks CIC, causes stone
formation
• Ileal loop problems
– Stones, hydronephrosis
– Cancer?
• CIC long term?
Bowel Problems
• 2/3 of adults have a satisfactory social
continence pattern.
• Regularity is most important.
– Timed evacuation
– Same diet
– Same exercise pattern
• Not related to the level of the spina bifida.
• Megacolon
• Unknown impact of chronic enemas, rectal
bulbs.
Orthopaedic Problems
• >50% found to have poor fitting braces.
• Premature arthritis.
– Shoulders
– Knees in ambulators who don’t use crutches / braces.
• Scoliosis progression if unfused.
• Osteomyelitis (bone infection) from decubitus .
Skin Problems
• This is where Pediatricians have the least
experience.
• Decubitus ulcers from pressure.
– inadequate chair pads.
– ill fitting braces, shoes
• Decubitus from incontinence wetness.
Skin Problems
• Decubitus from abrasion
– Walking without covering
– Feet protruding from
wheel chair
• Decubitus from tethering
– Changes in foot position /
deformity
– Changes in gait
Skin Problems
• Decubitus from poor circulation.
• Dependent edema causes poor circulation and
pressure.
– Circulation is modulated by nervous system.
– What is the impact of chronically purple or cold feet?
• Fungal infections / In-grown toe nails.
Latex Allergy and Spina Bifida
• 50% of people latex sensitivity.
• Latex allergy can get worse.
• Recommend:
– Avoid exposure to latex in hospital
and home.
– Take copy of latex guide with you
to doctor/hospital/work.
– Carry non-latex gloves with you to
doctor/dentist.
– Medic-alert bracelet.
– Epi-pen
Hypertension
(High Blood Pressure)
• 140/90
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“White coat hypertension”
Renal hypertension
Essential hypertension
Rule out Spina Bifida causes
– Renal status—ultrasound,
chemistries, imaging, scanning
Obesity
• Major issue related to prevention – a health care
problem for all in US.
– Slower metabolism with aging
– Poor diet / not enough exercise
• Contributes to high blood pressure, heart
disease, diabetes, sleep apnea.
• Obesity more likely in Spina bifida.
– Decreased mobility / decreased exercise
Heart Disease and Spina Bifida
• Adults with SB at high risk for coronary artery
disease and high blood pressure.
– May occur at earlier ages in SB.
• Causes:
– Obesity, poor diet, lack of exercise.
– ? other factors
• Treatment:
– Diet, exercise, medications for blood pressure.
Lung Disease
• Interventions
– Talk with your doctor if you get short of breath, have
frequent or severe snoring, restless sleep.
– Regular evaluation of scoliosis.
– Exercise .
– Yearly influenza vaccine; pneumococcal vaccine.
– May require more in-depth testing (sleep studies).
Exercise
• Individuals with SB are
less likely to exercise:
– More difficult to find
exercise to do - need for
special programs or special
equipment.
– Decreased opportunities.
– Lack of motivation.
Sexuality
• Deep personal relationships important to overall
health and quality of life.
– Mental health
– Longevity (how long we live)
• Making responsible decisions about sexuality.
– Knowledge of avoidance of STD’s.
– Avoid condoms containing latex.
Sexuality
• Pregnancy - ability to become pregnant is not
decreased for women with spina bifida.
• Pregnancy may contribute to SB related problems.
– Neurologic- shunt malfunction, tether symptoms.
– Urologic - decreased continence, infections.
– Orthopedic - gait changes, decreased mobility, pain,
muscle strain .
• Men – many can achieve erection / ejaculation.
– Low level spina bifida the better.
– Fertility often decreased (but isn’t zero).
Pain
• May be one of the most common concerns
among people with spina bifida as they get
older.
– Joint stress / muscle strain
– Earlier onset of arthritis
– Other causes
• Important to listen to signals your body is giving
you.
Mental Health
• Substance Abuse
– Including smoking, alcohol and illicit drug use.
– Individuals with disabilities at increased risk;
incidence in adults with SB not known.
– Limits ability for relationships, work, independence.
– Contributes to other health related problems.
Mental Health
• Individuals with SB at increased risk for limited
socialization.
– Limited experiences and practice.
– Contribution of Non-verbal Learning Disabilities
(NVLD).
• Depression an important mental health issue.
• Interventions - social interactions based on
interests, counseling, medications.
Finding a Health Care Provider
• Ask your pediatrician or
specialist.
• Talk to others with a
similar disability and health
care needs.
• Talk with your MRDD
service facilitator, case
manager, advocacy
organization, disability
agency.
Finding a Health Care Provider
• Find out if the office is physically accessible.
– Parking
– Exam table
– Restrooms
• Visit new provider; decide if he/she is a good
match.
Talking with your health care
provider
• Before the visit, write down
your questions and concerns.
• Ask your provider questions.
• If you are worried, say so.
• If you don’t understand, say
so or ask:
“Could you please repeat that?”
• Bring a friend or family
member with you to help ask
questions or make you feel
more comfortable.
Health Records
• Ask your pediatrician, specialist to transfer your
medical records to your new provider.
• Keep your own record of your medical history-if
your parents have already started it, add to it.
• Take these records with you to your .
• appointment.
Making a Portable Medical Record
• Start collecting history now.
• Transferring the medical record at 18 or 21 will
not work.
• Begin a notebook that includes:
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Surgeries (ask for reports)
Radiology procedures (ask for reports)
Illnesses needing ER or hospitalizations
Medications and any reactions
Equipment (dates of purchase/repairs and providers)
Immunizations
Useful Websites for
Portable Medical Records
• Some websites have health history
notebooks to download
– http://www.cincinnatichildrens.org/svc/alpha/c/specia
l-needs/resources/default.htm
– http://depts.washington.edu/healthtr/healthhistory/de
fault.html
– http://www.medicalhomeinfo.org/tools/assess.html
Health Care Skills Checklist
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I
I
I
I
I
I
have a good understanding of spina bifida.
keep records about my medical history.
understand and practice healthy habits.
manage my own medications.
know how to refill prescriptions.
make my own medical appointments.
http://depts.washington.edu/healthtr/healthhistory/default.html
Health Care Skills Checklist
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Make Health Care Appointments.
Talking with Health Care Providers.
Know how to get the doctor’s office.
Keep a calendar of medical appointments.
Know medications.
Wear a medical alert bracelet.
Know allergies.
http://depts.washington.edu/healthtr/healthhistory/default.html
Health Care Skills Checklist
• I order my own medical supplies.
• I understand my medical insurance coverage.
• I am able to arrange transportation to medical
appointments.
• I communicate my health needs to my doctor.
• I carry emergency numbers with me in my
wallet.
http://depts.washington.edu/healthtr/healthhistory/default.html
Medications
Name
_____
Dose
____
Frequency
_________
Reason
______
_____
____
_________
______
_____
____
_________
______
_____
____
_________
______
• Know your Medications, dosages, and frequency
and carry a card in your wallet.
Sometimes it’s hard to remember
to take your medicine
• Take care of your own menstrual needs.
• Keep a record of monthly periods.
Recommendations
• Go to a Primary Care Provider who knows about
coordination of complex conditions.
– Internist / Med-Peds physician
– Family Practitioner
– PM&R
Recommendations
• Medical screening:
– Blood pressure, height & weight periodically 18 years
and older.
– Cholesterol testing:
• Men - regularly 35 years and older.
• Women – regularly 45 years and older.
– Screening for colon cancer after 50.
– Tetanus shot every 10 years.
Recommendations: Women
Gynecologic exam and Pap
smears at least by 21. (earlier
if sexually active)
Breast self exam:
-once a month 1 week
after period or on your birth
day.
Mammograms by age 40
.(earlier if family history of
breast cancer)
Men
-Learn how to do a
testicular self
exam.
1. Once a month.
2. Do after a warm
shower or bath.
– prostate testing
beginning 40 - 45.
Recommendations
• Identify subspecialty care.
– Physicians with experience in chronic spinal
neurological conditions
– Neurosurgery
– Urology
– Orthopaedics?
Guidelines for Spina Bifida
Health Care Services
• Stick to the guidelines for annual evaluation.
– Annual U/A, Renal Ultrasound, renal chemistries
– Spine x-ray every 5 years.
• Baseline Head CT scan when shunt stable,
working.
– Large syrinx need frequent MRIs.
• Annual check of braces, crutches, wheel
chair, shoes .
Guidelines for Spina Bifida
Health Care Services
• Daily skin checks.
• Use the crutches Rx’d.
• Wear pressure stockings if feet chronically
blue or cold.
• Monitor BP.
• Annual exam by a podiatrist .
• Ongoing eye exams (MD physician).
• ECHOcardiogram perhaps every decade?
Recommendations
• Avoid smoking/drugs; don’t abuse alcohol.
• Find work you enjoy or do volunteer work.
• Develop a circle of strong social supports.
– neighbors, church, hobbies, work.
• Seek help if feeling sad or depressed.
• Get adequate sleep – notify your doctor if not
sleeping well or chronically tired.
Recommendations
• Find exercise you can do, and do it.
• Proper diet
– Watch calories / fat intake.
– Appropriate vitamins and minerals.
– ? Multivitamin / extra vitamins A, B (including folic
acid), E, zinc and selenium.
• Practice stress management.
Recommendations
• Pain
– Low impact exercise, rest, anti-inflammatory agents.
– Meditation, bio-feedback and Acupuncture may help
with chronic pain.
• Osteoporosis
– Adequate calcium, Vitamin D.
– New medications being tried - ? Use in SB.
Health Guide for Adults Living
with Spina Bifida
– Provides health information and resources to help you
manage your health and prevent further
complications.
– Edited by the SBA Editorial Review Board.
– $4.99/CD $14.99/Print
We Are All Responsible For:
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Taking care of our health.
Seeking advice when needed.
Sharing our knowledge and experience.
Making our own decisions and acting on them.
Living with the results of our decisions.