Assessment and Tx of children w/ special health conditions (OI, AMC, TBI, Tumors, Post-near drowning)

Question 1

Which disorder is defined as an inherited disorder of connective tissue?

Answer 1

osteogenesis imperfecta

Question 2

What are three common features in OI?

Answer 2

lax joints
weak muscles
diffuse osteoporosis

Question 3

T/F, children with OI tend to have below average intellectual function

Answer 3

false, they tend to have average or above average intellectual function

Question 4

Which of the following are not common features in children with OI:
Blue sclera
deafness
dentinogenesis imperfect
hernias
decreased sweating and prone to always being cold
easy bruising

Answer 4

decreased sweating and cold-> they tend to have EXCESSIVE sweating and prone to overheating

Question 5

Which types of OI demonstrate a pattern of autosomal dominant inheritance and a defect in Type 1 collagen structure?

Answer 5

Types I, II, III, IV

Question 6

Which types of OI do NOT HAVE a type I collagen defect but do have significant bone fragility with similar presentations to the the other types of OI?

Answer 6

Types V, VI, VII, VIII

Question 7

What are medical interventions that can be used to treat but not cure OI?

Answer 7

bisphosphonates (help increase bone density and reduce fractures)
vitamin D
Calcium

Question 8

T/F, you want to immobilize a child with OI to help prevent any future fractures

Answer 8

false, want to limit it to decrease disuse osteoporosis which can increase risk of fractures as well

Question 9

What are two types of surgical fracture care that can be done in children with OI?

Answer 9

IF with intermedullary rods to help prevent long bones from bowing
telescoping rods that elongate as the child grows (but is still a risky procedure and may or may not work as intended)

Question 10

t/f, we want to avoid PROM when assessing a child with OI

Answer 10

true, we want to do more AROM as PROM or aggressive stretching can cause fractures

Question 11

What is one of the most important things when doing PT management of an infant with OI (in regards to the parents)?

Answer 11

parent education about how to handle and interact with their children and how to position them correctly

Question 12

What is a predictor for ambulation in a child with OI?

Answer 12

if they can sit by 10 months

Question 13

Which standardized assessments can be done on an infant with OI?

Answer 13

PEDI, BSID-III, PDMS-2 (Darr said that she doesn’t think this test can tell you much in the lecture), Brief assessment of motor function

Question 14

What are the two main general principles to remember in handling an infant with OI?

Answer 14

support head and trunk, no forces on long bones

Question 15

What car seats are appropriate for infants with OI?

Answer 15

car beds with padding and rear facing as long as possible

Question 16

t/f, you want to get a child with OI in the pool by the time they are 6 mo

Answer 16

true, pools can be incredibly beneficial and allow for increased and safer movements to occur

Question 17

t/f, you want to limit wb and move a child with OI as much as you can

Answer 17

false, you want to facilitate protected weight bearing and allow a lot of SELF mobility

Question 18

t/f, most children with OI become productive adults

Answer 18

true, many do become productive adults and need to have independent living skills

Question 19

Multiple osteochondromatosis, multiple enchodromatosis, and fibrous dysplasia are all examples of what?

Answer 19

other health conditions that result in bone deformities and activity limitation

Question 20

What is the name of a nonprogressive neuromuscular syndrome present at birth that leads to severe joint contractures and mm weakness and fibrosis?

Answer 20

AMC (arthogryposis)

Question 21

t/f, if there is CNS involvement in AMC, it means it is lethal to the infant

Answer 21

true

Question 22

AMC most likely occurs during the ____ _____

Answer 22

first trimester

Question 23

What are the 2 most common presentations for AMC?

Answer 23

Type 1: Jack Knife
Type 2: Frog leg

Question 24

You receive a referral for a child that is said to have AMC. You are evaluating the child and notice that the child has abnormal muscle tone, webbed joints, decreased cognition, seizures, feeding problems and limited vision. What do you think is happening?

Answer 24

think that the child has another diagnosis other than AMC, all of those are characteristics that a child with AMC WON’T HAVE

Question 25

t/f, a child with AMC tends to have normal intelligence and speech

Answer 25

true

Question 26

T/F, a child with AMC tends to have an imbalance of agonist and antagonist muscle activation

Answer 26

true, will have an imbalance which may be symmetrical due to weakness of both agonist and antagonists

Question 27

t/f, it is rare for a child with AMC to have mm weakness

Answer 27

false, tend to have weakness. due to the decreased motor unit function

Question 28

What are some general developmental skills you would expect to see in a child with Type I AMC?

Answer 28

roll or scoot rather than creep
usually sit by 15 months using trunk flex and rot
stand PRIOR to PTS
walk with AD and orthotics around 18 mos with assist and independently between 2-3

Question 29

What are developmental skills you would expect to see in a child with Type II AMC?

Answer 29

have greater difficulty with rolling than Type I
faster with other skills
may creep or choose to booty scoot
may NEVER transition to stand or stand independently
may start walking during second year but require significant bracing and support

Question 30

t/f, when working on standing with a child with AMC, you want to use a prone stander

Answer 30

false, you want to avoid prone standers when helping them stand 2hrs per day by age 1

Question 31

t/f, you can get ROM back in a child with AMC who has not been having consistent stretching done

Answer 31

false, if they lose ROM, it is gone, have to emphasis parent education on the importance of following stretching HEP

Question 32

What are the three mechanisms for primary brain damage?

Answer 32

acceleration-deceleration
crush/contusion
penetration

Question 33

What are examples of secondary brain damage in children (4)?

Answer 33

extracranial- hypoxemia, hypotension
intracranial- hemorrhage, swelling

Question 34

What is the main difference between a pediatric and adult BI?

Answer 34

early edema after TBI

Question 35

T/F, near drowning incidents tend to have a very poor prognosis

Answer 35

true, 1/3 survivors have significant neurological damage due to HIE

Question 36

t/f, benign brain tumors in children do not require intervention

Answer 36

false, they still need intervention as the tumor may still damage the surrounding tissue and cause increased ICP, edema, herniation, etc

Question 37

t/f, young children tend to have a high incidence of cerebellar and BS tumors

Answer 37

true

Question 38

Which pediatric brain tumors are most common throughout childhood and primarily occur in the cerebellum and have increased ICP sx and cerebellar signs (ataxia) (2)?

Answer 38

astrocytoma, medulloblastoma

Question 39

Which pediatric brain tumor is in the posterior fossa and CH and produces increased ICP, HA, seizures, and focal cerebellar deficits?

Answer 39

ependymoma

Question 40

Which pediatric brain tumor is benign and occurs in the midbrain and tends to produce visual disturbances, HA, vomiting, and endocrine disturbances?

Answer 40

craniopharyngiomas

Question 41

Which pediatric brain tumor is a has progressive CN dysfunction and gait disorders?

Answer 41

brain stem gliomas

Question 42

t/f, pediatric brain tumor long term survivors tend to have minimal neuropsych and cognitive deficits

Answer 42

false, tend to have major deficits (better prognosis for astrocytoma and medulloblastoma)

Question 43

What are the range of levels in Rancho levels of cognitive function in pediatrics?

Answer 43

from V (no response to stimuli), to I (oriented)

Question 44

At what age should you use the adult scale of Rancho cognitive function?

Answer 44

kids greater than 12

Question 45

How should PT treatment change when treating a non-response- early response stage, then agitation/confused stages, to higher-level response stage in ABI?

Answer 45

first RLA I-III; Peds scale III-V: prevent MSK complications
second RLA IV and V; Peds level II: follow simple commands w/ impaired judgement and problem solving, manage/prevent secondary impairments, fxnal task trainin/parent education
last RLA VI-VIII; peds scale I: practice progressively challenging tasks, reduce environmental restrictions, increases physical conditioning