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While researchers have made great strides in understanding OI, relatively little progress has been made in terms of actually improving the quality of life for OI patients. On March 6, 40 experts in the field met in Baltimore to discuss strategies to advance clinical investigations and improve standards of care for individuals with OI. The focus of this meeting was clinical care for OI patients. Attendees spent a day and a half working towards the conferences three goals:
  1. identifying the major medical, rehabilitative and psychosocial concerns facing patients with OI.
  2. establishing an agenda for addressing some of these concerns, including proposals for needed research
  3. developing a group of outcome measures to be used across the OI community, including clinicians from all specialties, patients and their advocates.
All groups attemped to establish an environment of inter-institutional cooperation to improve patient care and facilitate development of clinical protocols for OI patients.

Attendees broke into three groups in order to more efficiently tackle the meetings goals. The first group focused on establishing a more consistent method of evaluating and diagnosing OI, as well as choosing a standard set of data to be collected prior to any treatment. The following reflects their initial findings, subject to revision:
  1. Medical histories and physical examinations, including dental & standardized anthropometric measures
  2. Standardized radiographs
  3. Fracture history, focusing on fractures that are disproportionate compared to the impact experienced
  4. Biochemical markers in blood and urine
  5. Bone mineral density, including whole body
  6. Nuclear scan for occult fractures
  7. Peripheral computerized tomography of peripheral bones (pQCT)
  8. Bone biopsy where indicated
  9. Skin biopsy or DNA studies
This group also devised a list of outcome parameters to use when evaluating the indications for or effects of treatment.
  1. Fracture rate
  2. Functional status
  3. Chronic pain problems—general vs. regional
  4. Mobility
  5. Bone quality
  6. pQCT
  7. Bone mineral density
  8. Biochemical parameters—NTX, etc.

Group II, which was devoted to rehabilitation and physical medicine, worked to develop a functional classification system similar to one that exists for patients with cerebral palsy. The group identified essential “domains” to measure as well as appropriate tests to gauge a patient’s functional level in each domain. We’ve listed a number of these instruments and methods under each domain.

Assessment of Pain
Instruments
Smiley Face
VAS 0-10
BPI
Acute vs. Chronic pain syndrome
Prevalence

 Strength
Group members first debated the accuracy and validity of strength testing, then identified appropriate instruments for clinicians to use.
Instruments
MMT
Dynamometry
Biodex
Muscle imaging-ultrasound, MRI

 Evaluation of Self-care Abilities
Instruments
PEDI
CHAQ
AMPS

 Mobility
Instruments
MAS
GMFM
BAMF
FAQ
PEABODY

 Fitness
Instruments
Ergometry
Endurance-(appropriate tests must still be developed)

 Quality of Life
Instruments
CHO
Peds QL
Harter Scales

 Obesity, Metabolic Status & Nutrition
Instruments
BMI

 Aging
Instruments
Function Reach
GET UP and GO
HAP

 Alignment and stability

Gait measurement and efficiency

Metabolic cart and imaging studies (CT)


The third group recommended utilization of specific research priorities based on comprehensive patient evalutation. Project areas and potential studies include:
  1. General muscleskeletal quality of life
    1. How well do existing measures (PODCI, CHQ, SF-36) work for individuals with OI
    2. Development of new measures or subscores
    3. Impact of pamidronate treatment
  2. Spine condition
    1. Scoliosis
      1. Natural history of untreated scoliosis in OI
        1. Does progression continue?
        2. Is curve size related to pain?
        3. Is curve size related to PFT?
        4. Is curve size related to diminished health?
      2. Outcomes of modern scoliosis surgery
        1. Pain
        2. Curve correction
        3. Postoperative loss of correction
        4. Complications
        5. Comparison with natural history group using CHQ, SF-36, Oswestry, SRS and PODCI
      3. Results of bracing
        1. Does it work in your patient group?
        2. Identifying predictors or bracing results
    2. Basilar Invagination
      1. Best methods for determining prevalence
        1. X-rays—are they inconclusive?
        2. MRI—is it too cumbersome?
        3. CTs—do they involve too much radiation?
        4. Neurological exam
        5. OIF-wide survey of identified BI versus age
      2. Long-term outcome of BI
        1. Without surgery
        2. With surgery
    3. Back Pain
      1. How prevalent is it?
      2. How effective is the use of Pamidronate?
      3. How effective is soft bracing?
  3. Extremities
    1. Does function correlate with upper extremity deformity?
      1. Does correction of the deformity change this?
    2. Does function correlate with lower extremity deformity?
      1. Does correction of the deformity change this?
  4. Craniovertebral Development
    1. Does craniovertebral relationship affect the development of mandible and maxilla?
  5. Biomechanics of Tendons and Ligaments in OI
  6. Biomechanics of vertebra

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