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Osteogenesis Imperfecta Clinic

The Osteogenesis Imperfecta Clinic at Children's Hospital & Medical Center is recognized worldwide for its expertise and leadership in treating children with brittle bone disease, also known as osteogenesis imperfecta (OI). In years past, many children with OI faced a future filled with pain, bone fractures, surgeries, casts and immobility. However, today, thanks to advanced techniques like those used at the OI Clinic at Children's, we offer many children the opportunity to live a more active lifestyle and have hope for a brighter future. As a regional center for OI treatment, approximately 80 percent of patients travel significant distances to Omaha to seek treatment. Our goal is to increase the quality of life for these children through compassionate care, advanced medical options and continued research so that we can find a cure for this genetic disorder.

Our staff includes a team of multidisciplinary health care professionals who are trained and experienced in treating and managing children with OI. Led by medical director Richard Lutz, M.D., fellowship-trained in pediatric genetics, endocrinology and metabolism, and Paul Esposito, M.D., fellowship-trained in pediatric orthopaedic surgery, the team also includes physical and occupational therapists, an audiologist, dietitian, nurses, nurse practitioners and radiology technologists, all working in one location to make the care process more convenient and comfortable for you.

What Is Osteogenesis Imperfecta?

Osteogenesis imperfecta (OI) is a genetic disorder that primarily affects the body's ability to produce collagen found in bones and other tissues. As a result, bones are brittle and break easily. Other possible symptoms of OI include curved bones, scoliosis, muscle weakness, joint laxity, fragile skin, blue sclera, brittle teeth, breathing problems and shortness of stature. Hearing loss is also common, especially in adulthood. The symptoms and severity of OI vary greatly from mild cases that may go undetected; to moderate cases with multiple symptoms and more fragile bones; to acute cases where life expectancy may be decreased, largely as a result of respiratory failure. OI occurs in males, females and all ethnicities. Although it can be inherited, OI frequently appears in families with no previous history of the disorder. This is due to gene mutation rather than any action taken by either parent before or during pregnancy.

Treatment

Each child with OI is unique, not only as an individual but also in diagnosis. Children's OI Clinic takes a highly personalized approach to patient care. Individual treatment protocols frequently are adjusted based on pain and fracture potential, age, growth and other key factors. Physical and occupational therapies progress along with your child's development. We listen to feedback from you and your child and seek minimal treatment for maximum benefit. Since the majority of our patients travel to Omaha from many states and several countries, we interface with primary care providers and health agencies in your hometown to safely administer medication and other therapies. We also work with insurance companies to stress the role that early preventive care plays in lessening the cost of care for osteogenesis imperfecta.

At Children's OI Clinic, we strongly believe in treating significantly involved infants early to decrease bone fractures. This involves using intravenous bisphosphonate medications like pamidronate, which improves the density of bone and reduces the pain suffered by your child. (This practice differs from many practitioners who don't begin treatment until a child reaches the toddler stage.) However, research is showing that early bisphosphonate treatment leads to a better prognosis for decreased fractures and pain and improved psychomotor development, leading to increased mobility, comfort and confidence. We also have found that the earlier your child is treated, the fewer number of surgeries he or she will need and the more normal his or her growth and development will be.

Your child's bone density is measured and monitored throughout the treatment process with a Dual Energy X-ray Absortiometry, or DEXA scanning. Unlike scanners that measure bone density in adults, the high-tech DEXA scanner is gauged specifically for children, including babies and toddlers. Our radiology technologists have extensive experience in working with children and osteogenesis imperfecta. We expose the children to the least amount of radiation to obtain the maximum benefit.

Advanced Treatment

For children with moderate to severe OI, the combination of bisphosphonate treatments that strengthen bones followed by the surgical insertion of growing rods offers the greatest promise for improved comfort, growth and development. The most commonly used rods are the Fassier-Duval Telescopic Rods, which are inserted through relatively small incisions often without postoperative casting.

The Children's OI Clinic embraced the procedure early on after Dr. Esposito received training from co-inventor of the rods, Francois Fassier, M.D., of Montreal. We have since built one of the most active programs in the nation. Because of Dr. Esposito's interest in the care of these children, he is often consulted by other pediatric orthopaedic surgeons across the country.

Research

With the support of the OI Foundation, Dr. Lutz and Dr. Esposito are collaborating with other OI centers, including the Kennedy Krieger Institute at Johns Hopkins and the Shriners Hospitals for Children in Chicago and Montreal, in an effort to find a cure for OI through advanced research. While a cure is not imminent, other research being conducted at our clinic will have a more immediate impact upon treatment protocols for the disorder. Current studies involve data collected from numerous growing rod surgeries, spinal deformity studies and information regarding hearing loss in OI patients. Dr. Lutz and Dr. Esposito also are widely published and are in frequent demand as lecturers on the topic of OI. The goal of the team is to ensure that all children with OI, regardless of where they live, receive optimal care.

© Children's Hospital & Medical Center | In Affiliation with University of Nebraska Medical Center College of Medicine