Care for Children Who Have OI

Care for children who have OI should be customized to meet the needs of each individual child. No two children with OI are exactly alike: the picture of OI varies greatly, not only between different types, but also within groups. Elements of a care plan will include:

  • Management of skeletal and non-skeletal issues;
  • Rehabilitation to enhance function and encourage development of maximum bone mass;
  • Nutrition counseling for health and weight control;
  • Surgical/hospital care that reflects knowledge of OI and respect for bone and tissue fragility;
  • Referrals to other specialists, including mental health professionals, as needed;
  • Attention to normal childhood diseases and immunizations;
  • Age appropriate information about reproductive health, and sexuality.

Interdisciplinary Care

Children benefit from coordinated interdisciplinary care from physicians familiar with OI. A number of medical centers across the United States and Canada have OI clinics and/or research programs, often as components of genetics or bone dysplasia centers. The OI Foundation maintains a “Clinic Directory” on its website. In other communities the parents and the primary care physician work together to create a network of health care providers for the child with OI. Clear and timely communication between all health care providers is important.

General Pediatric Care

The general health needs of children with OI are the same as other children. Typical childhood illnesses can be expected, but ear infections may occur more frequently. Children with all types of OI have a predisposition to respiratory infections; they may be more serious in children with OI Type III. Immunizations are not contra-indicated and are encouraged. Physicians with experience caring for children with OI suggest:

  • Titrate medication dosage to a child’s weight, not age, even with older children and teens.
  • Monitor the use of non-steroidal anti-inflammatory (NSAIDS) drug. Some have been linked to retarded bone healing after fracture.
  • Minimize the use of drugs that contain steroids because of negative effects on bone metabolism.
  • It is beneficial when PCP office personnel are trained in OI-specific safe handling techniques.

Monitoring

  • Routine screenings are needed for vision, hearing and dental care.
  • Spine checks for scoliosis and kyphosis beginning at age 2 years.
  • Bone density testing is recommended to help monitor changes over time.  A baseline test when a new course of treatment is started is informative along with a test approximately 6-12 months after a change in treatment. 
  • Echocardiogram as a baseline heart valve screening during later teen years or early young adult.
  • Consult with an orthodontist no later than age 7 to assess jaw development, and presence of malocclusions or cross-bite.
  • Perform a baseline quantitative Pulmonary Function Test (PFT) on all children with OI at age 5 or when the child can cooperate, and again at maturity (age 20-25 years). If PFT is normal, repeat every 2 years.

Diet and Nutrition

Children with OI need a balanced diet containing adequate water, fiber, calcium and vitamin D calibrated to their age and size. Nutrition counseling for parent and child may be beneficial.

  • Slow weight gain is seen in infants and may not be failure to thrive.
  • Swallowing difficulties, reported in some toddlers, may require referral to an occupational or speech therapist, or a nutritionist who treats feeding disorders.
  • Small appetite is seen in children of all ages. Inactivity, pain, medications and depression are potential causes.
  • Constipation is seen in children of all ages and with all types of OI and can be recurrent. Short stature, inactivity, pelvic deformity and difficulty with hydration are contributing factors.
  • Weight control is important, as obesity places a strain on the fragile skeleton and can lead to loss of mobility. Hormonal changes related to puberty can contribute to unhealthy weight gain, especially in girls who have OI.

Mental Health

Living with OI presents emotional as well as physical challenges for the child, parents and siblings. Health care providers are encouraged to note signs of depression, substance abuse, and fearfulness, and to make referrals to mental health professionals. Children may experience low self-esteem and anxiety. Children with the milder forms of OI may struggle to cope with having a hidden disorder that can be misunderstood by their peers. Older children may become discouraged by the repeated need to re-learn mobility skills, receive painful therapies and miss out on activities with their peers due to fractures and reduced mobility. Relationships between siblings can be strained.

Development

OI does not affect a child’s ability to think and learn, but children with OI often demonstrate delays in meeting developmental milestones. These delays can be the result of repeated immobilizations after fractures, misalignment of the long bones and joints and the general hypotonia and ligamentous laxity common in OI. Interventions can include physical and occupational therapy, braces, use of adaptive equipment and mobility aides. Small-muscle development, especially in hands and fingers, is likewise affected. To the best of our current knowledge, the incidence of autism, hyperactivity and childhood cancers is believed to be similar to that seen in the unaffected population.

Growth

Mild to significant short stature and a slow growth rate occurs in OI. Hip and back pain due to poor alignment and leg length discrepancies occur in all types of OI and should be evaluated by an orthopedist and/or a gait specialist. Height and weight charts for the child with OI are available.

Other Pediatric Treatment Information

Additional information about treatment for the musculoskeletal issues associated with OI can be found under the Treatment Section of this website. Information managing hearing loss, cardiac, vision, respiratory and other non-skeletal issues associated with OI can be found in the Non-Skeletal Issues section. 

References

Glorieux F. (2007) Guide to Osteogenesis Imperfecta for Pediatricians and Family Practice Physicians. Osteogenesis Imperfecta Foundation. Gaithersburg, MD.

Attribution Thank you to Dr. Francis Glorieux, Canadian Shriners Hospital for Children and McGill University in Montreal, Quebec, for reviewing this document. April 2015.

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