Course Description "Osteogenesis Imperfecta"



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Osteogenesis Imperfecta

Osteogenesis Imperfecta
Goals and Objectives
Course Description

“Osteogenesis Imperfecta” is an online continuing education course for occupational therapists and occupational therapist assistants. This course presents updated information about Osteogenesis Imperfecta including sections on etiology, symptomology, diagnosis, assessment, therapeutic intervention, medical services, and social services.


Course Rationale

The purpose of this course is to present therapists and assistants with current information about Osteogenesis Imperfecta. A greater understanding of Osteogenesis Imperfecta will enable therapists and assistants to provide more effective and efficient rehabilitative care to individuals affected by this condition.


Course Goals and Objectives

Upon completion of this course, the therapist or assistant will be able to:



  1. Identify the symptomology of OI

  2. Differentiate between the different types of OI and identify their causes.

  3. Recognize and understand the tests utilized to diagnose OI.

  4. Define safe OI neonatal and infant care.

  5. Recognize the primary care needs of individuals with OI.

  6. Recognize the precautions associated with providing medical care to individuals with OI.

  7. Identify the special care requirements of OI individuals receiving ER services

  8. Identify the special care requirements of OI individuals receiving surgical services.

  9. Identify the therapeutic needs of individuals with OI.

  10. Identify therapeutic strategies to address the needs of individuals with OI.

  11. Recognize the social services needs of individuals with OI.

  12. Recognize other OI associated health issues.

  13. Identify social, emotional, and family issues often affecting individuals with OI.


Course Provider – Innovative Educational Services
Course Instructor - Michael Niss, DPT
Target Audience - Occupational therapists and occupational therapist assistants
Course Educational Level - This course is applicable for introductory learners.
Course Prerequisites - None

Method of Instruction/Availability – Online text-based course available continuously.

Criteria for Issuance of CE Credits - A score of 70% or greater on the course post-test.

Continuing Education Credits - Four (4) hours of continuing education credit

AOTA - .4 AOTA CEU, Category 1: Domain of OT – Client Factors, Context

NBCOT – 5 PDUs

Osteogenesis Imperfecta

Course Outline

Page(s)

Course Goals & Objectives 1 begin hour 1

Course Outline 2

Overview 3-4

Symptomology 3-4

Prognosis 4

Types of OI 4-6

Etiology 7-8

Diagnosis 8-12

Physical Exam 9

Genetic Testing 9-11

Other Diagnostic Tests 11

Prenatal Testing 11-12

Educating the Family 12 end hour 1

Treatment 12-14 begin hour 2

Neonatal and Nursery Care 14-16

Handling Suggestions 14-15

Feeding 15-16

Bedding 16

Positioning 16

Parent Education 16

Primary Care 17-19

Children & Teens 17

Transition to Adult Care 18

Adults 18-19

Medical Procedures 19-20

Assessing the Patient 19

Taking Blood Pressure 20

CPR 20

Emergency Department Care 20-23

General Guidelines 21

Acute Fracture Care 21-22

Pain 22

Other Related Issues 22-23

Child Abuse allegations 23 end hour 2

Surgical Services 24-25 begin hour 3

Anaesthesia 24

Positioning 24

Intubation 24-25

Rodding 25

Physical & Occupational Therapy 25-38

Role of Therapy 26-28

Safe Handling 28-29

Therapeutic Strategies 29

Protective Positioning 30

Protective Movement 30-31

Progressive Modifications 31-32

Water Therapy 32-33

Adaptive Equipment & Aids to Independence 33-34

Types of Equipment & Considerations 34-35

Self-Care Tasks 35-38 end hour 3

Transportation 38-40 begin hour 4

Car Seats 38-39

Transporting a Casted Child 39

Other Equipment 39-40

Travel by Airplane 40

Public Transportation 40

Social Services 40-41

Nutrition 41-42

Adult Health Issues 42-46

Social, Emotional, & Family Issues 46-47

Myths About OI 47-48

References 49

Post-Test 50-51 end hour 4

Osteogenesis Imperfecta

Overview


Osteogenesis imperfecta (OI), also known as brittle bone disease, is a genetic disorder of connective tissue characterized by bones that fracture easily, often from little or no apparent trauma. It is highly variable in severity from patient to patient, ranging from very mild to lethal. In addition to having fractures, people with OI often have muscle weakness, joint laxity, skeletal malformations, and other connective tissue problems.

The prevalence of OI is approximately 1 in 20,000, including patients diagnosed after birth. OI occurs with equal frequency among males and females and among all racial and ethnic groups. Patients with OI have the full range of intellectual capabilities as seen in the general population. There is nothing inherent in the disorder that affects cognitive abilities. Life expectancy varies according to the underlying severity of the disorder and ranges from very brief (Type II OI) to average. Medical treatment for OI is increasingly understood.

Patients with osteogenesis imperfecta usually have a faulty gene that instructs their bodies to make too little type I collagen or poor quality type I collagen. Type I collagen is the protein "scaffolding" of bone and other connective tissues. Inheritance, in nearly all cases, follows an autosomal dominant pattern, although sporadic cases are common. When there is no family history of OI, the disease is caused by new dominant mutations.

Patients are often knowledgeable about their health status and the problems associated with OI. Accordingly, the opinions, requests, and instructions of adult patients and parents of children with OI should be respected.



Symptomology

Depending on the severity of OI, the following characteristics may be seen:



  • skeletal malformation

  • short stature – Growth impairment is severe in all those individuals with Type II and Type III OI, moderate in those with Type IV, and relatively less in those with Type I.

  • muscle weakness

  • ligamentous laxity

  • smooth, thin skin

  • triangular face

  • dental manifestations – Dentinogenesis imperfecta is present in about 50 percent of patients with OI. Deciduous teeth are usually more severely affected than permanent teeth.

  • blue sclerae – Approximately 50 percent of people with OI have blue, purple, or gray-tinted sclerae.

  • respiratory complications – Lung complications, such as pneumonia, represent a significant cause of death for those with Type II and III OI. Pneumonias are seen in children and adults, and cor pulmonale, a type of heart failure, is seen in adults.

  • cardiac complications – Mitral valve prolapse (laxity) is seen but is not as common as in some other connective tissue disorders.

  • hearing loss – In those with OI, hearing loss is frequent.

  • thermal instability – Those with OI experience slightly higher than normal body temperature, sensitivity to heat and cold, excessive sweating, pseudomalignant hyperthermia after anesthesia.

  • blood vessel fragility – Patients may exhibit easy bruising, frequent nosebleeds, and, in a small number of patients, profuse bleeding when injured.

  • neurologic manifestations – Basilar invagination of the skull, hydrocephalus, and syringohydromyelia of the spinal cord may be seen in patients with the more severe forms of OI.



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