What treatments are used for osteogenesis imperfecta?
Treatments for preventing or correcting symptoms may include:Care of fractures.Surgery.Rodding - a procedure to insert a metal bar the length of a long bone to stabilize it and prevent deformity.Dental procedures.Physical therapy.Assistive devices, such as wheelchairs, braces, and other custom-made equipment.
What are two available treatments for those with OI?
Treatment may include: Braces, splints or casts: An orthopedic specialist may use protective devices to stabilize broken bones during healing or after surgery. Surgery: Your provider may operate to correct curved or misshapen bones. Rodding surgery is a common treatment for children with OI.
What current research is being done to develop a cure for osteogenesis imperfecta?
BPS-804 (setrusumab) is a fully human monoclonal antibody designed to inhibit sclerostin, thereby improving bone strength and therefore reduce fractures in patients with osteogenesis imperfecta (OI) and improve quality of life. The study is international with research sites both in US, Canada and Europe.
What surgical intervention might be recommended for a child with osteogenesis imperfecta?
Intramedullary rodding surgery for children with Osteogenesis Imperfecta (OI) Children and young people with Osteogenesis Imperfecta (OI) are more likely to develop fractures and bony changes such as bowing (bending) and shortening. Rodding surgery may be suggested or recommended by your child's medical team.
What are the current treatments for osteoporosis?
Bisphosphonates are usually the first choice for osteoporosis treatment....These include:Alendronate (Fosamax), a weekly pill.Risedronate (Actonel), a weekly or monthly pill.Ibandronate (Boniva), a monthly pill or quarterly intravenous (IV) infusion.Zoledronic acid (Reclast), an annual IV infusion.
How do bisphosphonates treat osteogenesis imperfecta?
Osteogenesis imperfecta is caused by a genetic defect resulting in an abnormal type I collagen bone matrix which typically results in multiple fractures with little or no trauma. Bisphosphonates are used in an attempt to increase bone mineral density and reduce these fractures in people with osteogenesis imperfecta.
Are there support groups for osteogenesis imperfecta?
The Osteogenesis Imperfecta Foundation (OIF) is a non-profit organization dedicated to improving the quality of life for individuals affected by osteogenesis imperfecta through research, education, awareness, and mutual support.
How does osteogenesis imperfecta affect a person's life?
Milder cases may involve only a few fractures over a person's lifetime. Additional complications can involve hearing loss, heart failure, spine issues, and deformities. Osteogenesis imperfecta can sometimes be life-threatening if it occurs in babies either before or shortly after birth.
What is OI medical condition?
Osteogenesis imperfecta (OI) is an inherited (genetic) bone disorder that is present at birth. It is also known as brittle bone disease. A child born with OI may have soft bones that break (fracture) easily, bones that are not formed normally, and other problems.
What surgical procedure is most commonly used to decrease the incidence of OI fractures?
A common surgical procedure for OI patients, “rodding,” is the placement of metal rods in the long bones of the legs. This strengthens them and helps prevent fractures.
What is a bone infusion for osteogenesis imperfecta?
Pamidronate strengthens the bone by increasing bone density and corrects the imbalance between bone reabsorption and bone formation which occurs in osteogenesis imperfecta (OI). Pamidronate infusions are most effective when they are given every six weeks in small children and three-monthly in older children.
What is an OI in physical therapy?
OI, often referred to as brittle bone disease, is a rare bone disorder characterized by bone fragility (fracturing with little-to-no trauma), short stature, long bone deformities, bone pain, low muscle mass, persistently blue sclera (in some), and hypermobility.
How to diagnose osteogenesis imperfecta?
Specific treatment for osteogenesis imperfecta will be determined by your physician based on: 1 Your age, overall health, and medical history 2 Extent of the disease 3 Your tolerance for specific medications, procedures, or therapies 4 Expectations for the course of the disease 5 Your opinion or preference
What is the management of OI?
Management of the disease includes focusing on preventing or minimizing deformities, and maximizing the individual's functional ability at home and in the community. Management of OI is either non-surgical or surgical.
What is OI in medical terms?
Osteogenesis imperfecta (OI) is a progressive condition that needs life-long management to prevent deformity and complications. The interdisciplinary healthcare team helps the family to improve the functional outcomes and to provide support.
Can osteogenesis imperfecta be treated?
To date, there is no known treatment, medicine, or surgery that will cure osteogenesis imperfecta (OI). The goal of treatment is to prevent deformities and fractures and allow the child to function as independently as possible. Treatments for preventing or correcting symptoms may include: Care of fractures. Surgery.
What is the best treatment for OI?
Surgery remains a pillar of treatment for patients with OI, [ 8] but it should be performed only if it is likely to improve function and only if the treatment goals are clear. Surgical interventions include intramedullary rod placement, surgery to manage basilar impression, and correction of scoliosis.
What is the role of orthotics in OI?
Orthotics play a limited role in management of OI and are used to stabilize lax joints (eg, ankle and subtalar joints with ankle-foot orthoses [AFOs]) and to prevent progressive deformities and fractures.
What is the effect of bisphosphonates on osteoblasts?
As a result, unopposed osteoblastic new bone formation on the periosteal surface results in an increase in cortical thickness.
What is soft tissue surgery?
Soft-tissue surgery is used in specific circumstances ( eg, lower-limb contractures, particularly those of the Achilles tendon). Skilled administration of anesthetics and awareness of the limitations of surgery are essential prerequisites. [ 28] .
Why are rods used in OI?
Because the bone is soft in OI , rods (eg, extendable Sheffield rods or Bailey-Dubow rods), pins (eg, Rush pins), and wires (eg, Kirschner wires [K-wires]) are used rather than solid nails, plates, and screws; the latter are associated with increased fracture risk above and below the device and with poor fixation.
Does bisphosphonate help with bone pain?
Good evidence suggests that bisphosphonate therapy may significantly improve the natural history of type III and type IV disease, particularly by decreasing the rate of fracture, increasing BMD, decreasing bone pain, and significantly increasing height (especially with prolonged cyclic therapy up to 4 years). [ 32] .
Can immunosuppressive agents damage bone?
Furthermore, the use of immunosuppressive agents to prevent graft rejection and graft-versus-host reaction can itself damage bone. Future approaches include the autografting of genetically modified mutant osteoblasts, whereby the mutant collagen gene is inactivated.