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Pediatrics > Osteogenesis Imperfecta > Flashcards

Osteogenesis Imperfecta Flashcards

1
Q

What is Osteogenesis Imperfecta?

A
  • Inherited disorder of connective tissue
  • “Brittle Bones Disease” or “Fragilitas Ossium”
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2
Q

Osteogenesis Imperfecta Prognosis

A
  • Depends on severity of disease which ranges from mild-severe
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3
Q

Osteogenesis Imperfecta

More deets:

A

Comprises a # of distinct syndromes w/ great variability in its manifestations

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4
Q

Osteogenesis Imperfecta Pathophys.

A
  • Defect in collagen synthesis
    • → abnormality in processing Type 1 collagen
  • Osteoblasts normal BUT collagen fails to mature
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5
Q

Impairments of OI

3 that lead to recurrent fx’s

A

Lax jts

Weak mm’s

Diffuse OP

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6
Q

Impairments of OI

A
  • Lax jts, Weak mm’s, Diffuse OP→ Recurrent fx’s
  • Additional:
    • Blue sclera (eyes), Dentinogenesis imperfecta, deafness, hernias, bruising, hyperhyrdosis (excess sweating), facial deforms (triangle head)
  • Cognition→ usually @ or above avg*
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7
Q

2 Main Classifications of OI

A
  1. Looser Class→ (2 types)
  2. Sillence Class→ (4 types)
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8
Q

Main type of OI: Looser

Looser has ___ types

A

2

*remember this: Looser has 2 O’s, so therefore Looser has 2 types, or just loser spelled w/ 2 O’s

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9
Q

Main types of OI: Sillence

Sillence has ___ types

A

4

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10
Q

Looser System (2 types bc 2 O’s)

2 types in Looser Class.

A
  1. OI Congenita
    1. Most severe
    2. MANY Fx’s @ birth (congenita think congenital (birth)
  2. OI Tarda
    1. Mild (bc opp of congenita)
    2. Fx’s after birth (think Tarda like Tardy, LATE, so AFTER birth)
    3. 2 sub-cats:
      1. OI Tarda Gravis
      2. OI Tarda Levis
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11
Q

OI Tarda Sub-Categories

Gravis vs Levis

A
  • OIT Gravis
    • bowing of LEs
    • amb. w/ orthotics
    • short stature
  • OIT Levis
    • LEAST disabling in Loose Class.
    • amb prognosis EXCELLENT
    • approach avg. ht.
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12
Q

Sillence OI has ____

A

4 types

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13
Q

Sillence and Danks Class.

Based on 4 genetic types of OI:

A

4 genetic types:

Type 1-Type 4

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14
Q

Sillence (4 types) easy to remember bc its just called….

A

Type 1-Type 4

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15
Q

Sillence and Danks OI Classification

All types listed out first then broken down

A
  • Type I→
    • GENERALIZED osteoporosis and bone fragility, jt. hypERlax, hearing loss, gen. short stature
  • Type II→ MOST SEVERE***
    • NOT compatible w/ life; stillborn or die soon after delivery
  • Type III→ RARE, but severe
    • Mod. deformity present from birth and cont’s to deform long bones, skull, spine
    • VERY short stature
  • Type IV→ Rare, more mild
    • Typ GOOD prognosis for ambulation
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16
Q

Sillence and Danks Class.

Type I

A

Gen. osteoporosis and bone fragility, jt hypERlax, hearing loss, gen. short stature

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17
Q

Sillence and Danks Class.

Type II: MOST SEVERE

A

MOST SEVERE

Not compatible w/ life; stillborn or die soon after delivery

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18
Q

Sillence and Danks Class.

Type III

A

Rare, but severe

Moderate deform present from birth and cont’s to deform long bones, skull, spine

VERY short stature**

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19
Q

Sillence and Danks Class.

Type IV:

A

Also rare, mild

Typ. good prognosis for ambulation

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20
Q

Bone Deforms of OI

A

see pics

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21
Q

Medical Mgmt OI

A
  • NO meds to strengthen bone tissue*
  • Tx w/ ortho procedures→ alignment/dec deformity, Resp. care for severe cases
  • MgO2, fluoride, Ca+ supps→ dec Fx (limtd success)
  • Biphosphonates (hormones) w/ menopausal women
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22
Q

Fractures in OI→ The Vicious Cylce

A
  • Once fx occurs→ bone more susceptible to re-fx
    • Already weakened structure predisposes child → limb deforms from bowing of long bones, asymm growth, jt contractures
  • Immobilization to assist setting bone in align.==> Disuse OP; but reqd for healing**
    • → further puts bone @ higher risk for fx*
  • Goal→ LIMIT immob. of extremities as much as possible*
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23
Q

Fx’s in OI:

Best methods:

A
  • Best method for fx→ Stabilization of long bones via internal fixation w/ intramedullary rods
    • Prevent further weakening→ EARLY WB w/ orthotics initiated ASAP
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24
Q

Fx’s in OI

Spinal Deformities?

A

50% cases

  • Do NOT respond to bracing
    • Sx for severe curves
25
Q

Dx/Prognosis OI

A
  • Mild-Mod OI→ may not be dx’d @ birth but after fx occur from barely any trauma
    • ex. broken humerus getting pulled out of car seat
  • Collagen studies, DEXA, XRay→ R/O other dx’s
  • Prognosis→ depends on type
  • All pts w/ OI→ Fx rate diminishes near or after puberty***
26
Q

OI Differential Dx

A

Leukemia, Idiopathic Juvenile Osteoporosis, Achondroplasia

27
Q

PT Infancy Examination of OI

A
  • Be aware→ medical hx: understand fx hx and immob. tech’s
  • Pain→ FLACC
  • Caregivers handling tech’s→ feeding, transfers, diapering, play
  • ROM:
    • Goni can be used* (size approp.)
    • AROM/Functional ROM
      • Ex. to what extent hands to mouth
    • PROM is CONTRAINDICATED in this pop!!!
28
Q

PROM CONTRAINDICATED IN OI POPULATION!!!

A

!!!!!!!!!!!!!!

29
Q

Good prognosis for ambulation in Infancy w/ OI

A

Sitting by 10mos **** KNOW FOR TEST!!!!

30
Q

PT Infancy Exam: OI

continued: Strength, Gross motor

A
  • Strength→ observe mvmt/motor skills→ NO MMT!!!
  • Gross motor abilities→ Delays often
    • **Sitting by 10mos==> Good prognosis for amb.!!! (on test)
  • Assess approp. of DME w/ child
    • car seats, high chairs, strollers
31
Q

PT Infancy Examination: OI

Standardized Assessments for this pop.

A

PDMS-2, PEDI, Bayley Scales of Infant Dev. II, Brief Assessment of Motor Function (quick descriptive for gross, fine, and oral motor perform.)

32
Q

PT Rx in Infancy for OI

Goals:

A
  • Goal @ this stage→ MINIMIZE:
    • Fx’s, strength defs/weakness, deformities from jt laxity
33
Q

PT Rx in Infancy of OI

NICU*

A

for more severe cases fx’s in utero/or from birth

  • Skull and/or vertebral fx=> Neuro damage
  • Rib Fx’s=> resp/cardiac involvement
  • Decd parent bonding time=> incd parent anxiety
34
Q

PT Rx in Infancy

**Parent Education for ____ and ______

***ON TEST KNOW IT!!!!

A

Handling and positioning

35
Q

PT Rx in Infancy OI

A
  • Parent Education for handling and positioning*** (on test!!!)
    • forces NOT placed across long bones
    • Head/neck fully supported w/ UEs and LEs draped over support arm
    • Pillow to pad infant during carrying
    • Loose clothing and front/side velcro closures to ease dressing
    • Overdressing should be AVOIDED→ reduce overheat/sweating
    • Bathing→ padded/plastic basin w/ adeq. head support
36
Q

Physical Therapy Rx in Infancy

More guidelines

Diapers, Carriers/Car seats, OT imps:

A
  • Diaper changes→
    • rolling tech, NEVER lift by ankles***
  • Carrier/Car seats→ customized for child w/ adeq pad/support
    • may pad strap system→ snug fit w/out stress on bone
    • custom orthotic type inserts→ prevent dangling of LEs while riding
    • Remain rear-facing as long as possible***
  • Work w/ OT for modifications
  • NO percussion w/ CPT******
37
Q

No percussion in CPT w/ OI!!!!

A

*************

38
Q

PT Rx in Infancy

Positioning, S/L, Prone, Supine

A
  • Positioning→ change freq, should not restrict active/spontaneous mvmts
  • S/L→ support w/ towel rolls behind back and thru LEs/arms
  • Prone→ If tolerable, over short roll/wedge under chest
    • allows safe strengthening of UEs via gentle WB
  • Supine→ support @ arms/LEs w/ towel rolls
    • Hips-Neutral rotation
    • Knees→ supported underneath w/ towel roll
39
Q

PT Rx in Infancy

PT and family, Developmental Activities

A
  • PT assists family in ID’ing safe/approp toys and play pos’s
  • Dev. Activities encouraged!→ Rolling, Sitting
    • Rolling→ Infants UE placed alongside head
    • Supported sitting→ seat inserts/corner chairs
    • Once adeq. head control→ short sitting obtained, AVOID rotation forces across LEs
40
Q

Pull→Sit from hands in Infancy OI

A

CONTRAINDICATED!!!

41
Q

Pool Therapy for infants w/ OI

start when?

A

early as 6mos***

42
Q

PT Rx in Infancy OI

Facilitation and other forms of Tx

A
  • Hand placement during facilitation/positioning=> CRITICAL*
    • **Pull→ Sit from hands=> CONTRAINDICATED!!
    • Support behind shoulders as child actively sits
    • Ball work→ PTs hands @ proximal pelvis vs @ legs/feets
  • Walkers, jumpers, exercausers==> CAUTION
  • Pool Therapy→ 6mos***
  • Splint fabrications post-fx
43
Q

PT Exam Preschool Age OI

Essential @ this stage?

A

Eval of modes of mobility and adapted equipment***

44
Q

PT Exam Preschool Age OI

A
  • In add. to infancy info→ eval of modes of mobility/adapted equip. ESSENTIAL @ this stage***
  • Standardized Assess’s:
    • PDMS-2
    • PEDI
    • Brief Assessment of Motor Function (BAMF)
    • Pain→ Wong Baker Faces Scale***
45
Q

Pain→ Preschool→ Wong Baker Faces Scale

A

Pain→Preschool→ Wong Baker

46
Q

PT Rx in Preschool Age and OI

Disuse atrophy and OP

A

Disuse atrophy and OP @ this stage***

  • Structural changes impact participation
    • Dev. motor skills lag due to fx’s
  • Goal of PT @ this stage→ Protected WB and Self-mobility→ Independence
  • Handling/position STILL important→ but now child can become active participant in care
47
Q

PT Rx in Preschool Age OI

Equipment mods, WB, Orthotics

A
  • Equipment mods:
    • IMPORTANT!→ esp car seats as they outgrow infant carriers
  • Protected WB→ W/ bracing, typ not done outside orthotics due to tendency for bowing long bones
    • can be customized
  • Orthotics→
    • HKAFOs may dec bowing w/ decd incidence of fx’s
    • Air splints to support standing→ caution overheating*
48
Q

PT Rx for Preschool Age OI

Exercise!

A
  • Active Exercise:
    • Bolster straddling sit to stand (exactly how it sounds, straddle bolster and bring butt to it and stand→ GREAT IDEA!)
    • Lt weights w/ incremental inc’s
      • ***Watch torque on lg joints*** (on test!!!!)
    • Gym activities→
      • scooters, adapted trike, Simon says/RLGL, overhead reaching (toss w/ lt. ball)
  • Cannot amb→ scooter/power mobility addressed to allow ability to part. w/ peers
49
Q

PT RX in Preschool Age OI

Aquatic progam

A

STILL APPROPRIATE!!!

  • Aids resp. health
  • Warm pool limit→ 20-30mins***
    • *poor thermoreg.
  • Stand in water→ lower lvl pts w/ orthotics in water
  • Good idea to utilize splints in water if recent fx
50
Q

Decd Fx rate in OI when?

A

~Puberty***

51
Q

School Age and Adolescence OI

A
  • Reduced mobility and IND w/ ADLs:
    • Decd social part, social stigma, decd confidence, social iso., decd adjust. to school
  • Overprotective parents
  • Encourage positive attitude and participation!
  • *Spinal deforms increase→ tx w/ spinal fusion
  • DECd fx rate=> ~puberty
52
Q

Spinal deformities of OI increase in this stage

A

School Age and Adolescence

53
Q

WHY does Fx Rate DEC?

A
  • Hormones, improved IND w/ prevention, improved coord/body aware., incd bone strength, amb. w/out bracing less risky @ this age
  • Previously used WC→ MAY be able to achieve household amb w/ supports***
54
Q

IND in Mobility crucial @ this stage:

A

School Age and Adolescence

55
Q

PT RX School Age and Ado.

Continue addressing…

A
  • Endurance, strength, ROM
  • Functional mobility*
    • IND in mobility CRUCIAL @ this stage***
    • Some gain IND to amb in home→ community amb not feasible for most @ this stage 2*
      • short stature, incd energy expend, reduced strength/mm power
56
Q

PT Rx School Age and Ado.

A
  • Strength+Endurance programs w/ child’s goals in mind
    • progressive resistance acts, wts, adapted sports
    • adapted PE gym class
    • emphasize fitness+overall well-being*
  • Encourage to share chore-load @ home
    • self-confidence/worth, improve fam relations
57
Q

PT Implications Transition to Adulthood OI

A
  • Needs continue 2* onset of:
    • incd pain, contd OP, worsening spinal deforms, changing mobility needs as move from edu. setting→ career
58
Q

OI Conclusions:

A
  • OI => sig. deformity and disability
  • PT→ positive impact children w/ OI and families
  • Strength, environ. adapts, parent/caregiver edu=> CRUCIAL
  • EARLY PT intervention=> helps prevent deforms
  • Bc these children are @ or above lvl for cog→ overall focus of tx across all ages→
    • Maximize IND, social interact, edu. so that they may develop into functional adults who can part. normally in day to day life****

Pediatrics (27 decks)

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