Peds Exam 3 (AD, DMD, SMA, JIA, EBP Interventions, Oncology)

What is the difference b/t low tech, mid tech, & high tech AD?

-Low Tech: inexpensive, easy to learn, readily available, can be “off-the-shelf,” easier to make/maintain/replace (tray w/ yes/no indicators)

-Mid Tech: may cost more, require some training, have special design, may need power source or other support (switch adaptive toys)

-High Tech: higher cost, need specific training to learn, may require on-going support, often customized (augmentative comm device)

what must be considered during the eval for assistive technology?

task, person, environment

What is the framework for making decisions about AT in school setting, including determining if AT is needed, assessment planning, & implementation of AT?

Student, Environment, Task, & Tools (SETT)

What are potential barriers of assistive technology (7)

-Attitudes (“too disabled/impaired to benefit”)

-Thinking AT is all ‘high tech’ (therefore people w/ disabilities can’t use)

-Funding (no one wants to pay)

-Lack of knowledge of what is “out there” to support a person’s functional needs

-Lack of perceived skills by professionally to adequately support AT needs of others

-Thinking of AT as a tool just for people w/ disabilities, not as a tool for all people

-Failure to “consider” AT at all

what are the contraindications to standing programs?

-Orthostatic intolerance
-Impaired skeletal structure
-Severe contractures
-Hip subluxation (consideration)

when is a supine stander indicated/benefits

-medically fragile individuals
-allows gradual progression to upright
-upright interaction
-limited head control needed
-easier transfers

when is a prone stander indicated/benefits?

-upright social interaction
-requires head & trunk control (more than supine) → helps develop further control
-helps minimize extensor tone
-allows for pressure relief from posterior surfaces
-allows gradual increases in WBing
-allows for hip extension & improved alignment

What AT has easy rear access, child typically faces forward in frame, although it may be modified so child can face backwards?

-available supports for chest, pelvis, arms, thighs, ankle prompts

rifton gait trainer

What AT provides posterior & dynamic support that moves w/ child? weight-relieving gait trainer, Open in the front

Mulholland Walkabout Gait Trainer

What is an arrow-shaped frame on 5 castors?

-Single central bar holds chest support & seat w/ pelvic support

-Plate between legs helps prevent scissoring gait

pony walkers

What wc modification has firm base of support, flat seat & back, made of plywood or plastic base?

1-2" of covered foam, adequate for children w/ mild postural needs, no structural deformities

planar seating

What WC seat an off-the-shelf pre-contoured seating adequate for children w/ moderate postural needs or flexible asymmetries?

generically contoured seating

What WC seat is specifically molded to child, appropriate for children w/ significant physical deformities?

-Accommodates for LLD + pelvis & trunk asymmetries

custom contoured seating

What seat is required for <2 y/o? (or until outgrows top height or weight recommendations)

-Weight max up to 30-40 lbs

-Offer best protection in a crash until child outgrows weight or height max

rear-facing seats

What restraint is required for 2-3 y/o?

5-point harness provides extra restraint & support for children w/ behavioral challenges or positioning needs

Semi-reclined position may assist children w/ poor head & neck control who have outgrown limits.

Extra padding & positioning inserts

forward-facing seats

What is seat is required of 4-7 y/o? Requires good head, neck, & trunk control. High backs & lateral sides may provide additional support

Passengers 80-175 lbs

-Harness for positioning support

-Positioning aides: abductor wedges, support pads, foot props

-Used w/ vehicle's shoulder/lap belt system for restraint

belt-positioning booster seats

what conditions/when is a car bed indicated?

Cardio-respiratory conditions
LE casts
Omphaloceles
Midline chest or abdominal defects

What is for children 2 years old or 20-168 lbs?

-Closures in the front or in back

-Need good head, neck, & trunk control

a vest/harness

when is fetal surgical repair of the myelomyelocele sac typically performed

between 23 weeks & 25 weeks 6 days gestation

what is the risk to mothers & children who undergo postnatal repair rather than fetal repair ?

-Increased risk of spontaneous rupture of membranes
-Increased risk of oligohydramnios
-Increased risk of preterm delivery

What is defected development of any part of spinal cord? (esp lower segments)

myelodysplasia

what are some early s/s of shunt dysfnx to treat hydrocephalus (12)

-changes in speech
-fever & malaise
-recurring HA
-onset of or ↑ strabismus, spasticity, scoliosis, or seizures
-incontinence begins or worsens
-personality changes (irritability)
-decreased visual acuity, diplopia, visuoperceptual coordination

What are the 5 lvls of Modified Hoffer Scale that classifies ambulatory status of children w/ myelomeningocele?

-Level 1: community ambulation
-Level 2: community ambulation w/ WC use for long distances outdoors
-Level 3: household ambulation
-Level 4: household ambulation w/ WC use indoors & outdoors
-Level 5: non-functional ambulation

what is the likelihood that a carrier mother child will result in normal girl, normal boy, carrier girl, affected boy in DMD

-normal girl: 25%
-normal boy: 25%
-carrier girl: 25%
-affected boy: 25%

What is the pathophysiology of muscular dystrophies?

-Absence or abnormal dystrophin may result in damage to sarcolemma during muscular contraction/relaxation cycles (especially eccentric contractions)
-damaged sarcolemma results in an influx of Ca2+ leading to cell destruction
-these muscles cells that are destroyed are replaced w/ fatty & connective tissues, results in inflammation (pseudohypertrophy) & lack of functioning muscle cells (weakness)

What is the initial clinical presentation & dx of DMD?

Weakness evident 3-5 yrs (dx typically around 5 yrs when no fam hx of DMD is present)
25% do not walk until 2 y/o (~18.3 months)
Pseudohypertrophy of calf
Initial weakness of proximal musculature (hip X, neck flexors, abd) (proximal to distal progressive weakness)
-Clumsiness
-Falling
-Inability to keep up w/ peers when playing

what is the classic red flag associated with DMD

gowers sign (use arms to push on thighs to stand up)

what is the clinical presentation as DMD progresses

-loss of ambulation
-gait changes: toe walking, ↓ arm swing, unable to run/jump
-postural changes (↑ lordosis, scap winging, shoulder retraction)
-cardiac changes: dilated cardiomyopathy, CHF, arrhythmias
-respiratory insufficiency & ineffective cough
-GI issues

what is the mean age of loss of ambulation associated w/ DMD & what can prolong this loss?

9-10 y/o (typically maintain their ability to ambulate for 2 yrs after they are no longer able to independently transfer to standing from floor)
-steroids can prolong this by 3 yrs

what 2 types of outcomes used to examine DMD overtime

-Motor Function Measure
-Vignos Functional Rating Scale for DMD (1= walks & climbs stairs w/ assistance; 10= in bed: can do no ADLs w/o assistance)

what should be avoided intervention wise when treating a pt with DMD

-No aggressive strengthening
-Avoid overexertion
-Avoid immobilization
-high resistance strength training
-eccentric exercises (running/walking downhill)

what are sxs of overuse (4)

-feeling weaker 30 min post exercise or excessive soreness 24-48 hrs following exercise
-severe muscle cramping
-heaviness in extremities
-prolonged shortness of breath

What is the genetic inheritance related to spinal muscle atrophy (SMA)

-Autosomal recessive disorder
-Both parents are a carrier, each child they have has 25% chance of being affected, 50% chance of being a carrier, & 25% chance of not being affected or a carrier

What is caused by a genetic defect on survival motor neuron (SMN) gene (SMN gene → typically helps control & reduce apoptosis)

-SMN defect leads to degeneration of anterior horn cells w/ progressive muscle denervation, ↓ motor neurons, & atrophy

SMA

what is SMA characterized by & sxs that may be present?

-symmetrical, diffuse, & progressive weakness & muscle atrophy
-proximal weakness
-restrictive lung disease
-non-painful, gradual onset
-motor impairments (head/neck control, crawling, walking, swallowing)
-hypermobility distally
-fasciculations (70%)
-tremor of hands
-decreased/absent DTRs

What type of SMA are non-sitters → never achieve ability to sit independently

-poor head control

-become positionally dependent for respiratory support

SMA type 1

what is the onset, disease progression, and life expectancy of SMA type 1

-onset: 0-6 months (usually dx before 6 months of age)
-disease progression: rapidly progressive, severe weakness
-life expectancy: < 2 years

What type of SMA are independent sitters = highest level of fnx? (50% maintain ability to sit independently until 14 y/o)

-Never stands → needs orthotic support to stand

-will need powered mobility (try to get PWC by 9-10 months of age)

SMA type 2

what is the onset, disease progression, & life expectancy associated w/ SMA type 2

-onset: 7-18 months (diagnosed w/in 1st 2 yrs of life)
-progression: delayed motor milestones, rapid progression that stabilizes, moderate to severe weakness
-expectancy: > 2 yrs (can live well into adulthood if respiratory issues are not present)

what is a common secondary impairment that occurs with SMA type 2

hip dislocations

What type of SMA are walkers?

-Stands & walks = highest level of function

-may be able to run (but will not have full gait pattern)

SMA type 3

What is the onset, disease progression, & life expectancy associated w/ SMA type 3?

-onset: > 18 months (usually b/t 2-9 yrs)
-progression: slower but variable progression, mild to moderate weakness, concerns w/ frequent falls & fatigue
-life expectancy: adulthood

what is the prognosis for maintaining ambulation abilities in SMA type 3 if onset of sxs occurs < 2 y/o or > 2 y/o?

onset <2 y/o → 50% walk till 12
onset >2 y/o → 50% walk till 44

What SMA type is adult-onset, typically in their 4th decade?

SMA type 4

What motor scale for SMA was originally designed for individuals w/ type II SMA but now used in individuals w/ type II & III SMA at any age? (20 items)

hammersmith functional motor scale

What is the difference b/t the modified hammersmith functional motor scale, hammersmith functional motor scale expanded, & revised hammersmith scale?

-Modified Hammersmith Functional Motor Scale – same 20 items, but reordered to minimize fatigue

-Hammersmith Functional Motor Scale Expanded – longer version (20 original items + 13 items for people w/ SMA type III who are able to walk; new items include squatting & jumping)

-Revised Hammersmith Scale – 36 items, includes 2 timed tests

what types of SMA is the upper limb scale modules used for

type II and III SMA

what type of SMA is the Childrens hospital of Philadelphias Infant Test of Neuromuscular (CHOP INTEND) used for

type I SMA

what type of SMA is the test of infant motor performance (TIMP) used for

type I SMA

what are survivors of childhood CA at a greater risk for (4)

-Cognitive deficits
-Functional impairments
-Cardiovascular disease/pulmonary disease
-Early onset of frailty

what are 2 most common types of leukemias (functional leukocytes diminish causing body's ability to protect itself to decline also due to defective leukocytes taking over healthy ones)

-Acute lymphoblastic leukemia (ALL): 80% of all pediatric leukemia cases, most frequently b/t ages 2-5, >90% survival rate w/ medical mgmt
-Acute myeloid leukemia (AML): presence of defective granulocytes (bacteria-destroying cells) or monocytes (macrophage-forming cells), 5% of all pediatric cancers, commonly in 1st 2 yrs of life, 63% survival rate

what are common signs and symptoms of leukemias (6)

-enlarged lymph nodes
-enlarged liver or spleen
-fever
-easy bleeding or bruising
-night sweats
-weight loss

What are the 2 most common types of lymphomas found in children? (occurs in B cells & T cells)

-Hodgkin's Lymphoma (HL): less common, most commonly occurring in adolescence, survival rate 97%
-Non-Hodgkin's Lymphoma (NHL): more common, survival rate 87%, most commonly occurring in children >3 y/o

what are common s/s of lymphomas (8)

-painless enlargement of lymph nodes
-night sweats
-persistent fatigue
-fever
-chills
-unexplained weight loss
-anorexia
-pruritus

What are the 3 common categories of CNS tumors?

-Astrocytomas: most common CNS tumors (33%) play a role in brain homeostasis, microarchitecture of brain parenchyma, regulate development of neural cells & provide for brain tissue repair
-Medulloblastomas: occur frequently in children under 10, arise in cerebellum but can spread thru CNS & are highly invasive embryonal tumors
-Ependymomas: arise from glial cells lining ventricular system of brain or central canal of SC, can directly damage brain tissue & also can disrupt normal flow of CSF resulting in ↑ ICP & further CNS damage

what are common signs and symptoms of brain and CNS tumors (7)

-HA
-vomiting (esp in AM)
-vision/speech/hearing changes
-worsening balance
-unsteady gait
-unusual sleepiness
-weakness

What are the 3 types of embryonal tumors?

-Neuroblastomas: found in developing SNS, begins more frequently in adrenal gland, affects children under 5
-Retinoblastomas: originate in retina, affects children younger than 4
-Wilms' Tumor or Nephroblastoma: most common form of kidney cancer in children younger than 5

What are the 3 most common bone & soft tissue tumors (sarcomas) found in peds?

-Osteosarcoma: tumors found in bone typically found at distal femur or proximal tibia w/ next most common site being proximal humerus, pathologic fx often 1st presenting sign
-Ewing's Sarcoma: found in both bone & soft tissue, pain at site of tumor is often 1st sign, 25% metastasize (lung, bone, or bone marrow)
-Rhabdomyosarcoma: soft tissue sarcoma arise from mesenchymal cells become striated muscle cells, typically occur in head/neck region, urinary & reproductive organs

what are common signs and symptoms of sarcomas (5)

-intermittent pain that often worsens at night
-swelling
-decreased ROM
-altered gait
-swelling

what precautions should be taken when treating a pt after anesthesia administered?

Decreased balance & postural control; Falls

what are acute side effects of radiation therapy (8)

-n/v/d
-hair loss
-mucositis
-fatigue
-skin changes (redness, blistering, dry skin)
-pain
-myelosuppression (↓ bone marrow activity causing fewer RBCs, WBCs & platelets)
-cognitive deficits

what are chronic side effects of radiation therapy (8)

-fibrosis & tissue injury (↓ joint ROM & ↑ risk of osteoporosis)
-Obesity
-Cardiac & vascular disease
-Restrictive lung disease
-Cognitive deficits (cranial radiation)
-Pain
-Infertility
-Kidney dysfunction

what provides a good indicator of an individuals risk for developing an infection & what values indicates pt has ↑ risk of infection?

absolute neutrophil count (ANC) of 500 cells/mm3 or lower

what considerations should be made when tx a pt post-chemo in regards to risk for osteonecrosis, CIPN, CRFL, & heart failure

-Risk for osteonecrosis: avoid excessive high-impact activities during tx sessions
-Risk of CIPN: tx loss of ROM, foot drop, motor weakness of impaired leg
Risk of CRFL ensure complete systems screen & make appropriate referrals to other healthcare providers; establish communications w/ child to determine fatigue lvl for therapy & tailor your sessions to their needs
Risk of heart failure: monitor vitals, eval cardiac fnx/endurance

What is the conditioning phase, infusion phase, & enfragment period associated w/ bone marrow transplant/stem cells to tx pediatric cancers?

-Conditioning phase: elimination of nearly all blood cells & stem cells in pt → leaves pt immunocompromised & w/ myelosuppression
-Infusion phase: pt receives stem cells via infusion → transplanted stem cells can be collected from bone marrow, peripheral blood, cord blood
-Enfragment period: stem cells migrate to bone marrow & do their job

what is an important milestone for a pt in enfragment period of bone marrow transplant?

when ANC count is >500 cells/ul for at least 2 days in a row

What can occur when transplanted stem cells give rise to blood cells, which recognize tissue of pt as foreign & reject those tissues?

graft versus host disease (GVHD)

what are acute sxs of GVHD (8)

-Rash
-Itchy skin
-Skin discoloration
-Dry mouth
-Mouth ulcers
-Diarrhea
-Weight loss
-Joint contractures
-Malabsorption

what are the 5 "F" words associated with pediatric PT exams

-fitness (body structure/function)
-functioning (activity)
-friends (participation)
-family (environment)
-fun (personal factors)

What measures CIPN in children w/ non-CNS cancers?

-Scores correlate w/ functional measures of balance & manual dexterity

-Most sensitive tool for assessing CIPN

-assesses sensory, functional, autonomic sxs in addition to light touch, pin sensibility, vibration sensibility, strength & DTRs

Pediatric modified Total Neuropathy Score (peds-mTNS)

What is a tool used to identify functional abilities of children following reconstructive surgery 2ndary to a tumor resection? (osteosarcoma, Ewing's sarcoma)

-examines factors pertaining to pain, physical function, emotional acceptance, use of supports, walking ability, quality of gait

musculoskeletal tumor society (MSTS)

who is the functional mobility assessment (FMA) designed for & what does it assess?

-designed for use w/ children & adolescents w/ LE sarcoma
-assess 6 domains including: pain, function using TUDS & TUG, supports/AD, satisfaction w/ walking, participation in school/sports, & endurance (9 min walk test)

what are the normal range values for WBC and their purpose

-norm: 4,000-11,000 ul
-purpose: fight infection

what are the normal range values and purpose for neutrophil (ANC)

-norm: 1,500-8,000 cells/ml
-purpose: fight infection

what are the normal range values and and purpose of RBC's

-norm: 3.8-6 million cells/ul
-purpose: transport of O2 & nutrients

what are the normal range values and purpose of hemoglobin

-norm: 10-13 g/100ml
-purpose: transport of CO2 & O2

what are the normal range values and purpose of platelets (thrombocytes)

-norm: 150,000-400,000 cells/mm3
-purpose: helps blood to clot

at what WBC levels should NO aerobic exercise, light aerobic exercise, & resistance exercise be performed

-NO aerobic exercise: <5,000 cells/mm3 & fever present
-light aerobic exercise: >5,000 cells/mm3
-resistance exercise: >5,000 cells/mm3

at what hemoglobin levels should NO aerobic exercise, light aerobic exercise, and resistance exercise be performed

-NO aerobic exercise: < 8 g/dl
-light aerobic exercise: 8-10 g/dl
-resistance exercise: > 8g/dl

What is not a single disease, but a term that encompasses all forms of arthritis that begin before 16, persist for > 6 wks, & are of unknown cause?

Juvenile idiopathic Arthritis (JIA)

what are common primary sxs of JIA (10)

-joint inflammation characterized by swelling, end-range stress pain, stiffness, & loss of full ROM
-morning stiffness or inactivity stiffness namely upon walking
-muscle atrophy
-weakness/poor muscle endurance
-acute or chronic iridocyclitis (most common in oligoarticular JIA)
-systemic manifestations (severe in systemic JIA, mod in polyarticular JIA)
-gait deviations (primary or secondary)
-bony overgrowth or pannus
-ligament laxity
-flares

what should be created in collaboration w/ PT, parents, & child w/ JIA regarding child's tx plan?

joint health & self-mgmt program that promotes a balance of rest & safe exercise/activities & max fnx & participation

what are the common s/s associated w/ systemic JIA (7)

-spiking fever (102+) that occurs 1x or 2x daily for at least 2 wks w/ rapid return to normal b/t spikes
-evanescent rash (discrete, erythematous macules) most often on trunk or limbs
-may also include pleuritis, pericarditis, myocarditis, hepatosplenomegaly, & lymphadenopathy

what are the 3 types of JIA and which is most common

-Systemic
-Polyarticular
-Oligoarticular (most common)

what are common s/s associated w/ oligoarthritis JIA (4)

-low grade inflammation in 4 or fewer joints (most common in knee, then ankle & elbows)
-joint is swollen & may be warm, not always painful
-30% develop iridocyclisitis that may lead to functional blindness
-systemic sxs are rare

what are common s/s associated w/ polyarticular JIA (7)

-arthritis in 5 or more joints
-insidious & progressive onset
-symmetric
-affects large & small joints & may include C-spine & TMJ
-joint is swollen & warm but rarely red
-mild systemic sxs
19% develop iridocyclitis

when performing strength testing on a pt w/JIA, what should be done if pt is in pain & is inflamed versus what should be done if pt has no inflammation?

-Painful & inflamed: perform isometric strength testing if child is in pain, avoid "break" method, as child may give way due to pain
-No joint inflammation: 6-15 RM is sufficient for establishing a baseline

what should be considered when tx a pt w/ JIA when performing interventions/modalities to manage their joint health?

-NO heat, ultrasound, or diathermy
-Include cold, exercise, & occasional splinting
-Active ROM preferred over passive ROM

what should aerobic exercise prescription be when tx a pt w/ JIA?

Train at least 2x per week
Moderate to vigorous intensity
45-60 min per session
6-12 weeks

what should anaerobic exercise rx be when treating a pt w/ JIA?

15 high-intensity cycling sprints (15-30s all out)
-Each sprint followed by 1-2 min of active rest
3 sets of sprints, 5 minutes b/t sets