For Medical Professionals

Orthopedics in OI

Good OI orthopedists prefer to treat fractures with short-term immobilization in lightweight casts, splints, or braces to allow some movement as soon as possible after the fracture. This is important to prevent bone loss and high refracturing rate. Limit time in immoblaztion to the shortest period of time.

Many children with OI undergo a surgical procedure known as rodding, in which metal rods are inserted into the long bones to control fractures and improve deformities that interfere with function. There are two basic types of rods. Nonexpandable rods are more versatile but often must be replaced as the child grows. Expandable rods can grow with the bone, but are only appropriate for larger bones (such as the femur) due to their thickness and need to be firmly anchored at both ends.

Progressive, sometimes severe, scoliosis is a problem for many people with OI, and may cause respiratory problems. Bracing is generally not recommended, as the force applied may deform the ribs rather than straighten the spine. Spinal rodding may be appropriate in severe cases, if the bone is strong enough to support the rod.

Physical Therapy and Exercise.

Physical therapy should begin as soon as a child is diagnosed with OI. Especially when it is evident that an infant has muscle weakness or motor skill delay when compared with same-age peers. PT for moderate to severe OI children should not stop when a child reaches basic physical therapy goals. Trunk muscle strengthening is important to help prevent scoliosis as well as pelvic strengthening to try to promote gait stability. The long-term goal for children with OI is independence in all life functions such as self-care, locomotion, recreation, social interaction, and educatio. Adaptive devices can be added as needed. Occupational therapy can help with fine motor skills and adaptive equipment for daily living. As a child with OI grows older and gains more independence, he or she will benefit from continuous physical activity. Adaptive physical education is important to maintain bone and muscle mass. Swimming and water therapy are particularly well-suited for people with OI of all ages. Water therapy should be started soon after birth and will allow independent movement in a safer environment with little risk of fracture. Walking is also excellent exercise for those who are able (with or without mobility aids).

Medications

Bisphosphonates(Pamidronate) have been shown over the last 10 years to increase BMD(bone mineral density), decrease bone remodeling markers, decrease pain and fracture rate in infants and children with OI. It has been shown to increase rate of independent ambulators as well. It has been shown that children with moderate to severe OI should start treatment at the earliest time to see the most positive effects in therapy. Calcium intake should be evaluated and supplemented if needed. Vitamin D supplementaion is necessary to help bind the calcium to the bone.

Experimental Therapies. Various minerals and medications have been tested throughout the years to determine if they strengthen bone in OI. Most of these substances have not been proven effective. Growth hormone and bone marrow transplant are being studied in human subjects. GH with Pamidronate may show positive results. Gene and cell therapies are in the biomedical stage of research at this time.

Healthy Lifestyle

People with OI benefit from a generally healthy lifestyle, including safe exercise and a nutritious diet. Adequate intake of nutrients, such as calcium (to supplement bone density) and Vitamin D (to promote binding of calcium) is important. However, megadoses of these nutrients are not recommended. Evaluation by a physician or registered dietitian will help people with OI determine adequate nutrient intake for their body size and age. It is also recommended that people with OI avoid smoking, excessive alcohol or caffeine consumption, and steroid medications, which may affect bone density.

Advice to Medical Professionals from Parents of OI children.

Most importantly respect the opinions, advice, or instructions provided by parents of OI children and children with OI. They have dealt with dozens of fractures and medical procedures. They often have a good sense of whether a fracture has occurred even before x-rays are taken. They have often learned the best methods to minimize pain and distress (medication, positioning, lifting, etc.) when a fracture occurs.

When a fracture is suspected, minimize handling of the affected limb.

Use caution when inserting IVs, drawing blood, moving a child or performing other medical procedures on children and adults. Pressure on an arm or leg can easily lead to bruising or fractures.

Never pull or push on a limb, or bend it into an awkward position.

Limit Blood Pressure checks to only critical situations and always use a manual cuff at the lowest pressure.

Lift a baby with OI by placing one hand under the buttocks and legs, and the other hand under the shoulders, neck and head.

Do not lift the baby from under the armpits, or lift by the ankles to change a diaper. Be aware of where the baby’s arms and legs are at all times to avoid awkward positions or getting a hand or foot caught.