Pediatrics

Question 1

Common comorbidities in Down Syndrome

Answer 1

Congenital heart defects (~50%) (most common is ventricular or atrial septal wall defects, most commonly in atrioventricular canal, sometimes blood unable to move between chambers)

GI tract anomalies (~10%)

• Celiac dz (5-7%)
• Duodenal or anorectal stenosis or atresia [no opening when there should have been an opening] (1-5%)
• Hirschsprung disease (1-3%): lack of autonomic innervation to part of colon -> decreased peristalsis, can be life-threatening
• Tracheoesophageal fistula or esophageal atresia (0.4-0.8%)

Thyroid Dysfunction (up to 15% - which is 28-54x higher than in general population! Incidence increases with increasing age)
- Most often hypOthyroidism; thryroxine (hormone supplement) is the current standard of care

Leukemia (1%, but 10-15% more common than in typically developing kids)

• Transient myeloproliferative disorder (TMD) found in ~10% of newborns with Down syndrome (almost EXCLUSIVELY found in kiddos w/Down’s)
• –> rapid growth of abnormal white cells
• –> Usually regresses spontaneously in the first 3 months of life, but some need medical intervention
• –> Group w/TMD are at increased risk for leukemia (10-30%) later in life; need to be monitored closely t/o childhood

Respiratory issues

• All individuals w/Down syndrome are at greater risk for pulmonary problems:
• –> Pulmonary hypoplasia: smaller airways
• —> Hypotonia can lead to easier airway collapse or obstruction
• Increased frequency of upper and lower respiratory infections
• Aspiration: silent in up to 80% of cases
• Obstructive sleep apnea

Skin disorders (up to 87%)

• Eczema
• Palmoplantar hyperkeratosis (thickening of outer layer on palms/soles of feet)
• Xerosis (dry skin)
• Seborrhoeic dermatitis (mainly affects scalp)
• Folliculitis

Hearing and Visual impairments (80% have 1 or both)

Question 2

PT considerations for the pediatric patient with congenital heart dz

Consider…
- Signs/symptoms of heart failure in pediatric patient?

• What should PTs be monitoring /documenting?
• Questions to ask as part of cardiopulm systems review?

Answer 2

• Increased WOB
• Increased time to feed (or possible refusal to eat)
• Become sweaty during feedings
• Poor or decreasing activity tolerance

PTs should document activity tolerance, as our sessions challenge the CVP system! Monitor vitals, document time to fatigue with specific activities. Really watch if vital signs change >10%.

Refer back to cardiopulm MD if we see changes in activity tolerance, presence of signs/symptoms

Always f/u on parental concerns

Cardiopul system review:

• Does your child seem to get out of breath or struggle to breathe?
• How long can your kiddo play or be active before getting tired? (how far into a grocery trip can they make it before asking to be carried?)
• Does your child take a long time to finish a bottle or need frequent rests while being fed?
• Does your child ever turn blue around the lips or look dusky?
• Does your baby strenuously resist tummy time?

Question 3

PT implications post sternotomy in the pediatric patient (consider normal needs and i/s/o Down syndrome)

Answer 3

Pedi sternal precautions = limits on tummy time and UE WB for 4-6 wks post-op (depends on surgeon)

• In pt with Down’s, may exacerbate delay in motor development d/t limited movement opportunities
• Kids tend to have poor activity tolerance and decreased endurance post op

Question 4

PT implications for the pediatric patient with GI track anomalies

Questions to ask for a system review?

Answer 4

• Likely to have poor nutritional status and impaired weight gain - may not be getting the calories they need or absorbing the nutrients
• Will affect energy level and activity tolerance, thus exacerbate motor delays
• Important to understand the anatomical nature of the problem and medical interventions
• Abdominal surgery may prevent or limit tummy time - which can then make kiddo resistant to tummy time later once once they are medically able to do it
• Schedule PT sessions around feedings
• –> 1 hour after feeding may be helpful to maximize energy level, but avoid full stomach

Questions to ask for a system review?
Ask about feeding routine and habits:
- Coughing/choking w/feeding?
- Frequent belching w/eating?
- Avoidance of certain textures?
- Drinking large amounts of fluids between mouthfuls?
- Ability of infant to gain weight?
- Where is the infant on the Down specific growth charts?
- Excess spitting up or diagnosis of reflux?
- Tolerance of tummy time?

Question 5

PT implications for the pediatric patient with Thyroid dysfunction

Answer 5

• Less energy for activity or therapy sessions
• Diminished performance secondary to weakness, fatigue, or muscle/joint pain
• Decreased motivation to participate in physical activity
• Untreated or under-treated hypothyroidism can slow down motor development even more than expected
• Especially notable if change is seen relative to baseline fxn

Question 6

Signs and symptoms of leukemia?

Answer 6

• Abnormal labs w/blood count
• Anemia
• Fatigue
• Poor endurance
• Organ enlargement (liver, spleen, lymph nodes)
• Easy bruising

Medical interventions

• Chemo, BMT
• Similar rates of complete remission in kids w/Down’s syndrome as in typically developing kids

Question 7

PT implications for the pediatric patient with leukemia

Answer 7

• Fatigue and poor activity tolerance; may require adjustments to PT schedule and intensity, especially during medical treatment
• Immunosuppression: MUST have good hand hygiene and cleaning of toys/equipment
• Referral if see change in baseline performance along w/other signs and symptoms

Question 8

Common respiratory issues in kiddos w/Down’s Syndrome

Answer 8

ALL individuals w/Down syndrome are at greater risk for pulmonary problems:

• –> Pulmonary hypoplasia: smaller airways
• —> Hypotonia can lead to easier airway collapse or obstruction
• Increased frequency of upper and lower respiratory infections
• Aspiration: silent in up to 80% of cases
• –> Hypotonia may affect swallowing
• –> Undersized oral cavity with normal sized tongue
• –> Hypoplastic palate: high and short
• –> Hypoplastic maxilla

Obstructive sleep apnea

• Abnormal sleep study in 100% of pts w/symptoms and 60% in asymptomatic pts!
• Removing tonsils and adenoids improved sleep studies, but failed to normalize in most
• Signs/symptoms of OSA:
• –> Irritability and behavior changes
• –> Fatigue, excess sleepiness during daytime hrs
• –> Snoring
• –> Frequent awakening at night
• –> Abnormal sleep positions

Question 9

Common congenital heart defects in kids w/Down Syndrome

• > Interventions?
• > Implications in this populations?

Answer 9

Most common is ventricular or atrial septal wall defects, most commonly in atrioventricular canal, sometimes blood unable to move between chambers.

• > Requires open heart surgery via sternotmy to correct defects
• > Kids w/Down’s tend to have longer hospitalizations and increased risk of respiratory complications post op d/t pulmonary hypoplasia that accompanies Down Syndrome

Question 10

Common GI tract anomalies in kids w/Down Syndrome

Answer 10

GI tract anomalies (~10%)
- Celiac dz (5-7%): autoimmune response to gluten -> constipation, bloating, diarrhea, poor growth. Can have celiac dz, but may or may not be symptomatic.

• Duodenal or anorectal stenosis or atresia [no opening when there should have been an opening] (1-5%)
• Hirschsprung disease (1-3%): lack of autonomic innervation to part of colon -> decreased peristalsis, can be life-threatening
• Tracheoesophageal fistula [opening where there shouldn’t be one] or esophageal atresia [NO opening when there should have been one] (0.4-0.8%) - holes or no holes, these likely require surgical intervention

Question 11

Thyroid dysfunction in kids w/Down Syndrome typically presents as [hypo / hyper] thyroidism. Gold standard medical intervention is ___. Signs and symptoms of [hypo / hyper] thyroidism in this population include…

Answer 11

Thyroid Dysfunction (up to 15% - which is 28-54x higher than in general population! Incidence increases with increasing age)
- Most often hypOthyroidism

THRYROXINE (hormone supplement) is the current standard of care

Signs and symptoms:

• -> Fatigue, poor endurance
• -> Muscle and joint aches
• -> Increased sensitivity to cold
• -> Depression
• -> dry skin

Question 12

PT implications for the pediatric patient with respiratory issues

Answer 12

• Poor participation d/t fatigue or sleepiness
• Behavior changes and irritability: resistance to PT activities that exceeds that which is typically expected for cognitive development
• Good hand hygiene and cleaning of equipment/toys is necessary to decrease risk for infection
• Monitor vital signs especially RR, WOB, and breathing pattern
• Use caution with offering snacks or drinks d/t swallowing difficulties

Question 13

PT implications for the pediatric patient with skin disorders

System review ?s

Answer 13

• Discomfort w/handling, textures of clothing
• Discomfort or poor tolerance of orthoses
• Good hygiene is important
• Clean those toys & PT equipment!!

System Review

• Ask about and examine for rashes, bruises, broken or irritated skin
• Does your child seem to bruise easily and/or often?
• Does your child have unexplained bruises? (a kiddo w/downs may fall a lot d/t poor postural control too, but those would be explained bruises!)
• Does your child seem overly sensitive to textures or heat/cold?

Question 14

Typical hearing and visual impairments seen in children w/Down Syndrome include:

Frequent ear __ - small ear ___ are a contributing factor

Hearing loss which is [ conductive / sensorineural / mixed ]

• Visual impairment: most often the result of refractive errors (up to 75%) and include…

Answer 14

Typical hearing and visual impairments seen in children w/Down Syndrome (up to 80%!) include:

Frequent ear INFECTIONS - small ear CANALS are a contributing factor

Hearing loss which can be conductive, sensorineural, OR mixed!

• Visual impairment: most often the result of refractive errors (up to 75%) and include…
• –> Strabismus (25-50%)
• —> Nystagmus (up to 29%)
• –> Congenital cataracts 4-7%

Question 15

PT implications for the pediatric patient with hearing or visual impairment

System review ?s?

Answer 15

• Make sure you have child’s attention (eye contact) before giving commands
• Minimize background noise when feasible
• Incorporate sign language and visual cues
• Seek parents’ assistance re: communication needs, visual field, and strategies
• Encourage use of correction lens and hearing aids as appropriate
• Speak slowly, use fewer words

System Review:

• Do you have any reason to suspect child isn’t hearing or seeing well? Response to loud noises? Ability to access environment and toys not related to motor skills?
• Does kid avoid certain textures/tastes?
• Does your child seem to avoid or seek sensory input (e.g. flopping onto furniture, jumping into things? Avoids or seeks out spinning or rocking? May suggest sensory integration dysfxn)
• Does your child run into things or seem to not know where his body is in space?

Question 16

Atlantoaxial instability (AAI) is present in up to __% of individuals with Down’s Syndrome, but only 2% are symptomatic. There is some debate over screening for this routinely, but [what are the current recommendations re: screening for atlantoaxial instability?]

Answer 16

Atlantoaxial instability (AAI) is present in up to 15% of individuals with Down’s Syndrome, but only 2% are symptomatic. There is some debate over screening:

• Current screening isn’t specific or cost effective, and doesn’t reliabily determine which individuals w/AAI are at risk for subluxation and cord compression.
• Screen kids age 3-5yo, but also obtain neural canal width measurements

AAP recommends:

• If kid is symptomatic: do plain neck XR in neural position. If abnormalities are found -> refer to neurosurg to stabilize upper C-spine. If NO abnormalities on neutral XR, repeat XR in cervical flex/ ext.
• If ASYMPTOMATIC, AAP does not recommend routine XR (as it isn’t specific, not worth the XR exposure)
• Insufficient bone mineralization and epiphyseal development to get an accurate radiographic exam <3yo
• Counsel parents annually re: risks w/activities that puts pt in extreme C-flex or ext. E.g. with anesthesia (often requires extreme C-extension to intubate) and rigorous activity (contact sports, diving into a pool, therapeutic horseback riding / hippotherapy, jumping on trampolene) review symptoms

Question 17

Symptoms of atlantoaxial instability?

Answer 17

• Significant neck pain
• Radicular pain down the arm
• Weakness: especially new onset or change
• Spasticity or change in mm tone
• Change in gait or change in use of arms and legs
• HYPERreflexia (recall: in down syndrome, typically hypOreflexive)
• Change in bowel or bladder fxn

Question 18

What health things do you need to look out for annually for kids w/Down’s syndrome as they age (>5yo)?

Answer 18

• Individual ear audiology eval
• Blood work: thyroid fxn (TSH) and hemoglobin
• Review symptoms fo celiac dz (if pt eats a glutenous diet)
• Assess for symptoms of atlantoaxial instability, review precautions for surgery/anesthesiology (avoiding hyperext) and radiography w/parents
• Review symptoms of obstructive sleep apnea
• Monitor for behavior problems
• Discuss healthy diet, exercise

Opthamologist eval (q2yrs from 5-13yo, q3 yrs from 13-21yo)

Additional things for kids ages 13-21:

• Derm referrals as needed as skin issues become more problematic in adolescence (folliculitis in up to 50-60%)
• Cardiology referrals: can develop mitral and aortic valve dz in up to 50% of adolescents without a hx of congenital heart dz! Refer for TTE if changes on auscultation (new murmur or gallop) or with hx of increasing fatigue, SOB, or DOE
• Celiac dz: screen adolescents q3yrs; ~50% biopsy-proven celiac dz are asymptomatic!
• Physicians must discuss sexual development, healthy relationships; should advocate for least invasive and least permanent method of birth control; physical response to puberty is the SAME in individuals with Down’s, but these may be confusing for these pts i/s/o cognitive deficits
• Monitor/eval for psych disorders (more prevalent in adolescence and adulthood)

Question 19

What factors contribute to risk for obesity in individuals w/Down’s Syndrome?

Answer 19

• Lower resting metabolic rate
• Tendency to be less active (d/t hypotonia and physical activity make physical activity more difficult)

This really hits in teenage years (between 13-18yo)

Question 20

Orthopedic things to be on the lookout for in adolescents with Down Syndrome?

PT goals?

Answer 20

Hypotonia and ligamentous laxity increase risk for joint pain

FOOT conditions are the most common, have greatest impact on daily life (e.g. pes planus, hallux valgus, toe deformities)

KNEE PAIN is also a biggie.

• Patellar instability
• Genu valgum

HIP PAIN

• Subluxation
• Slipped capital femoral epiphysis (needs surgical correction)

PT goals:

• balance mm strength between antagonist and agonist across involved joints
• Improve alignment with orthoses as tolerated (particularly feet, ankles) - lack the muscle tone to stabilize at a joint

Question 21

Health surveillance for adults with Down’s syndrome should include…
(given details for each category and what this area of screening is looking for/what pts are at higher risk of)…

Cardiology

Otolaryngology

Thyroid Function

Audiology

Ophthalmalogy

Tooth/gums

Mental illness

Bone health/Osteoporosis

Cognitive changes later on?

Musculoskeletal considerations?

Answer 21

Cardiology

• Can develop valve dysfxn, even with (-) prior hx (mitral valve prolapse in up to 57%, valvular regurgitation)
• Screen w/careful auscultation
• Refer for TTE if changes noted
• Other symptoms: fatigue, irritability, weight gain, DOE

Otolaryngology

• Obstructive sleep apnea in up to 50% of individuals w/Down’s
• CPAP is not always well tolerated - may not have the cognitive ability to understand why they need to wear the mask, may be uncomfortable
• Mouth guards are being used more recently

Thyroid Function - screen TSH levels annually

Audiology - eval q2 yrs

• Conductive and sensorinural hearing loss in up to 70% of adults w/Down’s
• May manifest as behavior changes, not responding to commands
• Communication deficits may impair ability tell caregiver about hearing loss

Ophthalmalogy - exam q2 yrs

Gingivitis and peridontal dz: more common in individuals w/Down’s.

• Cavities are LESS common, but they might have more tooth loss d/t the peridontal dz!
• Dental visits 2x/yr

Mental illness: in up to 30%

• Depression
• OCD
• Conduct disorder

Osteoporosis
- Higher risk and earlier onset (mean age onset = 35yo!!)
- Long bone fractures and compression fractures are common in adults w/Down’s
Risk factors for osteoporosis include: short stature, hypotonia, decreased physical activity, early menopause, decreased muscle strength, thyroid disease

HIGH prevalence and EARLY ONSET of Alzheimer’s Dz
- 30s: 0-10%
- 40s: 10-25%
- 50s: 28-55%
- 60s 30-75%
Symptoms include change in behavior, loss or decline in function, memory loss, seizures, incontinence

Musculoskel:

• Early onset degenerative joint dz from years of hypotonia and ligamentous laxity - ESPECIALLY in FEET, KNEES, and HIPS
• Osteoporosis
• Challenging for families to find appropriate care - outpt clinics are less comfortable w/Down Syndrome; pedi therapists don’t want to treat adults; significantly underserved population!

Need safe, community -based fitness opportunities and strengthening for health and weight management!
- Aim for NON competitive, and with a social component to get best participation

Question 22

Physiological changes as we age

Answer 22

• Less collagen in muscle tissue
• Collagen: becomes more cross linked, becomes more abundant in skeletal muscle (in lieu of mm itself) -> decreased mm flexibility
• –> Collagen is influenced by activity! Decreased activity -> increased collagen turnover and replacement in mm tissue –> decreased flexibility
• Muscle mass:
• –>Peaks (d/t hypertrophy/size) ~16-20yo F, vs 18-25yo
• -> Max strength achieved in 30s and maintained until the 50s!
• –> Amount of strength needed to accomplish ADLs is unchanged throughout life, but person’s max strength gradually decreases w/age

Decreased mm strength d/t

• Loss of mm mass, increased subcutaneous fat
• Decr Type I and II fibers
• Decreased fast twitch (type II) fibers perhaps d/t decreased fast twitch motor neurons (lose ~10% mm fibers per decade after age 50!)
• Decr cross sectional area of mm
• Training/exercise cannot prevent mm loss with age, but it slows it and makes it not as bad!

Question 23

ACSM recommendations re: how to increase flexibility…

Answer 23

General stretching program to all major extremity and trunk mm groups
2-3x/week
Hold each mm group 10-30 sec; 4 reps per mm

Question 24

Osteoporosis is the most common metabolic bone dz, characterized by BMD >= ___ standard deviations below the mean. We see a loss of bone mass and micro architectural deterioration which leads to bone weakness and fracture.

Osteopenia is characterized by BMD __- ___ standard deviations below the mean.

Answer 24

Osteoporosis is the most common metabolic bone dz, characterized by BMD >= 2.5 standard deviations below the mean. We see a loss of bone mass and micro architectural deterioration which leads to bone weakness and fracture.

Osteopenia is characterized by BMD 1-2.5 standard deviations below the mean.

• Chronic mm weakness contributes to this!
• Osteoporosis is a “geriatric disease with pediatric roots” - femals es achieve peak bone mas by age 30, bone building prior to age 18 is very impioratnt!
• Need adequate Ca2+ intake during adolsecence

Question 25

Risk Factors for osteoporosis include…

Answer 25

• Female
• Thin / small frame
• Family hx
• Post- menopause/ early menoapuse
• Amenorrhea
• Eating disorders (anorexia or bulemia)
• Medications: AEDs, corticosteroids
• Low Ca2+ intake, especially during adolescent growth spurt
• Cigarette smoking
• Sedentary lifestyle
• Excess EtOH or caffiene use
• White or Asian race

Question 26

Adults with developmental delay likely are:

• Overweight, more so in those [who live at home/ who live in institutions]
• Decreased __
• Poor __
• Poorer health related behaviors in those who [live in the community / live in institutions]
• Increased incidence of neuro and psych illness compared to general population
• Social isolation, lack of experience interacting w/others

Answer 26

Adults with developmental delay likely are:

• Overweight, more so in those WHO LIVE AT HOME vs those living in instutitions (largely d/t strict dietary control in institutional settings)
• Decreased PHYSICAL ACTIVITY
• Poor NUTRITION (at times reflective of behaviors, parents giving kids what they want to eat to avoid behaviors)
• Poorer health related behaviors in those who LIVE IN THE COMMUNITY
• Increased incidence of neuro and psych illness compared to general population
• Social isolation, lack of experience interacting w/others

Question 27

Common medical comorbidities/problems in adults with developmental disorders?

Specifically in individuals:
>40 yo: increased rates of ___
>50yo: increased __, __, and __

Relationship between epilepsy and cognitive impairments - given these, at risk for anything else?

Answer 27

• High cholesterol
• HTN
• Hearing impairment
• Epilepsy
• Endocrine problems (especially thyroid dz)
• Skin conditions
• High rates of dental/oral disease (often d/t poor dental care in childhood, these kids may be quite fearful of dentist)
• Vision impairment
• Decreased BMD
• Mental health impairments
• Coronary artery dz
• Stroke
• Obesity: individuals with mild and moderate CI have higher rates of obesity than individuals with profound CI (who are likely dependent on others to feed them)
• Diminished work capacity associated w/decreased peak O2 consumption

> 40 yo: increased rates of DEMENTIA
50yo: increased CVD, diabetes, and OA

Epilepsy + cognitive impairment = increased risk for major DEPRESSION or PSYCHOSIS

In kids: more severe impairments and functional limitations -> increased incidence and intensity of non-accidental pain (e.g. from trauma from others or self- injourous behaviors)

More severe disability in Down Syndrome -> higher rates of health problems

Diet, fitness/exercise are key!!

Question 28

Relationship between IQ and incidence of psychiatric, neurological, and seizure disorders?

Answer 28

IQ <45: more neurological illness, higher incidence of seizure disorders
IQ 45-70: more psychiatric illness

Individuals w/developmental disorders may be at risk for increased medication side effects too - perhaps because they may respond differently to meds than the general population, vs maybe not taking meds appropriately

Question 29

Average life expectancy for individuals with developmental disorders (DD)? Timing of aging?

What is considered “elderly?”

When do we start to see muscle changes typical of aging in individuals w/DD? What are these changes?

Average age of dx of osteoporosis?

Answer 29

Individuals with DD are living longer! Average life expectancy now 70-74yo.

Aging process appears to occur earlier in these individuals, including onset of Alzheimer’s Dz and osteoporosis (average age of osteoporosis dx = 35yo).

> =55yo is considered “elderly”

Muscle changes typical of aging (sarcopenia, decreased flexibility, decreased force production) begin as early as 50yo in individuals w/DD (and occur faster in this population!)

Question 30

Common health conditions in adults w/Down Syndrome

Answer 30

• Cardiac abnormalities
• Higher risk for health problems than other individuals w/DD
• *Overweight/ Obesity: d/t sedentary lifestyles (weaker, decreased balance), less opportunities for social interactions
• Thyroid abnormalities (typically HypO)thyroidism
• Cataracts
• mid-Cervical Arthritis
• Sleep apnea
• Atypical seizures
• Leukemia (especially as they get older): enlarged lymph nodes, swollen gums, bruises
• Osteoporosis
• Alzheimer’s Disease (higher risk d/t early menopause [<46yo], history of Hypothyroidism, Trisomy 21 - puts you at higher risk for plaque development)

Question 31

Symptoms of alzheimer’s dz in individuals with Down Syndrome?

Answer 31

• Memory loss
• Weight loss
• Decreased skills w/ADLs
• Personality changes
• Late onset seizures
• Loss of conversation skills

Question 32

Effects of exercise training in individuals with Down Syndrome?

• Impact on VO2 or HR max?
• Impact on behaviors?

Answer 32

(1) Walk/run study: 65-75% peak HR, 10 wks, 3x/wk 30 mins
(2) Rowing study: 55-75% peak VO2, 16 wks, worked up to 25 mins rowing gradually
(3) Jog walk training w/ECG monitoring for target HR 65-75% HR max; 12 wks, group training w/edible, verbal, visual reinforcements/ motivation -> DID improve exercise/physiologic parameters compared to controls! But low HRmax and VO2 max t/o

(4) Cardio: 12 wks, 3x/wk, 30-45 mins (bike, TM, elliptical, or recumbent stepper; pt’s choice) PLUS 15-20 mins strength. By week 5, all pts were tolerating 30 mins at 50-75% peak VO2 (w/close supervision to achieve this).
- >Strength via (bench, seated leg press, seated leg curl, tricep push down, seated row, shoulder press, lat pull down, bicep curl) at 70% 1RM, 1 set of 10-20 reps. Incresaed resistance by 10% if able to do 20 reps for 2x sessions.
- > And IT WORKED! Improved CV fxn and strength, and decreased body weight; no changes for controls.

Both (1) and (2) found improved exercise endurance (able to walk further/row longer) BUT NO change in VO2 max relative to control group

• -> DS pts may not respond to training in conventional matter OR might not have been intense enough (if pts were at low end of the above target HR/VO2 ranges)
• –> Motivation could impact training (given HRs at low end of prescribed range)
• –> Needs to be WELL PLANNED OUT and SUPERVISED to make changes
• –> Low peak VO2 overall (controls vs not) may cause problems as individuals age, w/difficulties even w/light ADLs

Why lower HR Max? Maybe d/t circulating catecholamines or reduced sensitivity to catecholamines? *So, they may not be able to reach target HR physiologically!

Why lower VO2 peak? Maybe d/t increased body fat and lack of consistent physical activity?

Exercise also DECREASES maladaptive behaviors!

• ~40% of individuals with intellectual disability show significant psychopathology: aggression, impulsivity, self stim, SIB
• Self stim decreased by 33% after 8-10 mins of jogging!
• More on-task behavior noted after structured exercise class

Question 33

Discuss changes in strength in individuals with intellectual disability (ID)?

Answer 33

• Kids and adults w/ID have 30% lower levels of mm strength and endurance, with significantly lower peak torque and power on isokinetic testing compared to sedentary controls
• Hypotonic muscles (e.g. in Down Syndrome) are weak mm!

Question 34

Cerebral palsy is a [progressive / non-progressive] lesion of the brain which occurs early in life and results in primary [ cognitive / motor / sensory] impairments. Recent studies have reported a gradual deterioration in functional skills in adults with CP.

Answer 34

Cerebral palsy is a NON progressive lesion of the brain which occurs early in life and results in primary MOTOR impairments. *But people tend to lose FUNCTION musculoskeletally as they age - Recent studies have reported a gradual deterioration in functional skills in adults with CP.

Question 35

Impact of aging on muscles in CP relative to normal aging?

Answer 35

Normal aging:

• Decreased Type II muscle fibers
• Decreased BMD
• Decreased flexibility

CP:

• Selective atrophy of Type II muscle fibers with spastic muscles
• Likely to have been born pre-mature -> decreased BMD compared to full-term babies
• Decreased flexibility, increased joint contractures

Question 36

Musculoskeletal pain impacts __-__% of adults w/CP

18% <30yo report chronic pain, vs 40% in those >60yo! Location of pain related to type of CP and individual’s age.

Role of physical activity in fatigue and chronic pain?

Answer 36

Musculoskeletal pain impacts 67-84% of adults w/CP

18% <30yo report chronic pain, vs 40% in those >60yo!

Location of pain related to type of CP and individual’s age.

Physical activity can improve fatigue and chronic pain!

Question 37

Where do we tend to see PAIN for the following groups of individuals w/CP:

• Generally?
• w/Dyskinesia:
• w/Hemiplegia
• w/Spastic diplegia:
• w/Spastic quadriplegia:
• Women vs men?
• Impact of age?

Answer 37

Tend to seen pain…

• w/Dyskinesia: Neck, shoulder, HA
• Generally: back, hip, and arm pain
• w/Hemiplegia: least likely to report back pain (maybe because they’re more active than other groups?)
• w/Spastic diplegia: Foot/ankle
• w/Spastic quadriplegia: Knees
• Women vs men? Women tend to report more HA, back pain, and hip pain relative to men w/CP

Impact of Age:

• Neck, shoulder, and back pain peaks between 30-40yo (maybe as they are most active/ambulatory in this range?)
• Increased HA w/age
• Decreased leg pain w/age - maybe d/t increased use of w/c’s?
• More pain w/those w/decreased motion and w/contractures (which occur in 91% of pts who were non-ambulatory)

Question 38

Scoliosis in CP

• Impact on function?
• Expected progression?

Answer 38

• Scoliosis can lead to back pain!
• Curves >50deg at skeletal maturity show continued progression of 1.4 deg /yr (skeletal maturity will NOT decrease scoliosis!)
• Progression can lead to difficulties w/seating and positioning, cardiopulmonary compromise

Question 39

Examples of pain behaviors in children (and adults!) w/severe cognitive impairment?

Answer 39

• Eating less, not interested in food
• Increase or decrease in sleep patterns
• Cranky, irritable, unhappy
• Withdrawn or seeks comfort of closeness
• Change in eyes: squinching, open wide, frowning
• Grinding or clenching teeth, tongue thrusting, chewing tongue
• Less active/quiet than usual; agitation or fidgety
• Move body part so it cannot be touched
• Shivering, sweating, tears, sharp intake of breath, change in color

Question 40

Efficiency in gait in CP vs non-CP?

Describe changes from childhood to adulthood

Describe specifics about what is different w/CP gait pattern and why that impacts efficiency

Answer 40

• Typically developing: gait becomes more efficient as children mature
• Bilateral CP (Spastic diplegia): as kids mature, require INCREASED (3-5x!) energy expenditure relative to those w/out CP
  –> Flexed knee gait/crouched gait in CP results in DECREASED speed and INCREASED energy expenditure.
  Takes more energy to stabilize hip, knee, ankle (and they’re already weak!)
• Decreased knee flexion during gait -> 23% increase in O2 consumption!

*Later onset walking (e.g. at 4yo instead of 2yo) -> will likely lose walking abilities earlier!

Question 41

Fatigue is a big thing in kids w/CP!

Associated w/more [mild / moderate / severe] forms of CP - why?

Fatigue is also associated with ___, deterioration of ___, limitations in physical and emotional role function, and [ QoL?]

Answer 41

Fatigue is a big thing in kids w/CP!

Associated w/more MODERATE CP than mild or severe forms
–> it’s a result of workload vs work capacity. People w/ moderate involvement require MORE energy to keep up with peers. Individuals w/severe involvement usually don’t have the motor abilities to become fatigued.

Lack of physical activity is also associated w/fatigue! - so stay ACTIVE!

Fatigue is also associated with PAIN, deterioration of FUNCTIONAL SKILSS, limitations in physical and emotional role function, and LOW LIFE SATISFACTION

Question 42

Osteoporosis and CP

• Big factors that influence bone strength?
• More severe CP = [greater / less] risk for osteoporosis
• Thoughts on standing programs and bone health?
• Impact on PT interventions in childhood?

Answer 42

Osteoporosis and CP

Big factors that influence bone strength?

• Height, weight have significant influence on bone strength.
• Most bone growth occurs during growth spurt during childhood and adolescence
• Exercise during growth can enhance periosteal bone growth and strength
• Static loads DO NOT initiate osteogenesis - the largest bone loads and bone strains come from the MUSCLES not body weight! People who are strong (muscularly) tend to have stronger bones (consider men vs F) and why bone strength tends to accompany muscle strength improvements as kids are growing
• Whole bone strength adapts mainly to maximally force muscle contractions, NOT low force contractions

More severe CP = GREATER risk for osteoporosis
- AED use is also a risk factor, but non-ambulatory status is a bigger risk factor

Thoughts on standing programs and bone health?

• DYNAMIC not static WB appears to have more potential for maintaining bone strength in adults - maybe a rationale for maintaining gait training programs!
• Endurance activities increase muscle endurance, but NOT whole bone strength
• Max force muscle contractions place larger loads on bone, thus increase bone strength

Impact on PT interventions?

• WB should be emphasized (especially dynamic) during the growth years, may benefit from continuing to encourage walking even if not functional!
• In older adults, vigorous exercise CAN prevent further bone LOSS, but will NOT cause large enough strain to increase bone strength. Muscle strengthening should be emphasized, as strong mm appear to correlate w/strong bones
• Standing studies w/kids w/CP need to be controlled for:
• Prematurity (lower BMD)
• Sunlight exposure (more severe = not outside as much)
• Need to define functional status (GMFCS helps)
• AED use
• Nutritional status (may be lacking vitamins for bone growth, even w/tube feeding)
• Height, weight

Question 43

In typically-developing populations, the level of physical function is stable until ~__ yo. In CP, physical function starts to decline at ~__yo.

Tips for managing aging w/CP:

• Sleep recommendations?
• Pacing?
• Impact of caffiene?
• Body weight?
• Exercise recs?

Answer 43

In typically-developing populations, the level of physical function is stable until ~60 yo. In CP, physical function starts to decline at ~30yo. 75% of those w/CP who will stop walking stop walking by age 25!!

Tips for managing aging w/CP:

• > =7 HOURS sleep/night to combat fatigue
• Take BREAKS when tired
• AVOID caffeine: caffeine has been reported to increase spasticity
• Avoid getting overweight
• REGULAR involvement in LOW IMPACT exercises and WEIGHT training

Question 44

Specific exercise interventions / recs on functional mobility in adults w/CP

• Role of video games?
• Role of rhythmic auditory training?
• Role of strength training?

Strength training specifics?
General activity recs?

Answer 44

Specific exercise interventions on functional mobility in adults w/CP

• Video gaming for BALANCE activities -> improved balance in adults w/CP
• Rhythmic auditory training: improved comfortable GAIT SPEED

Strength training:

• more improvement in body image and self efficacy
• Aim for a FAMILIARIZATION period: very low dosage 2x/wk for 2-4 wks! (going in too hard too fast -> muscle pain, decreased adherence)
• Gradually increase dosage to accommodate to improved strength, endurance, function
• 1-4 sets, 6-15 reps; minimum 12-16 wks

General recs:

• Replace sedentary time with light-intensity activities: short periods of activity to break up sedentary periods
• Increase time in WB activities (e.g. standing frames)
• Consider: is it realistic for very sedentary individuals w/CP to participate in <2hrs/day sedentary behaviors (e.g. TV, computer time) especially if GMFCS IV-V?
• –> consider aquatic therapy, PWB TT, hippotherapy, exercise w/exoskeleton
• Research needed on how childhood activity levels impact adult activity levels