Remarkable developments have been made in the last 10 years that have dramatically changed the quality of life for many
children with Osteogenesis Imperfecta. Doctors at Shriners Hospital for Children in Montreal developed a medical
treatment using a drug called Pamidronate. Pamidronate is a bisphosphonate and acts to slow down the breakdown of bone
while allowing the bone to make new bone. It is a treatment , not a cure, but it the best thing available to these kids
in the history of OI. It is not for all children with OI but a good OI specialist can determine who should be on
it and carefully observe the treatment process to try to prevent adverse effects.
Almost universally, studies
and publications on children with Types III-IV OI show an increase in bone volume, increase in bone density, decreased
perception of pain, increased vertebral height, and a decrease in fracture rates. Children treated with Pamidronate
for four years show at least as much growth as those without pamidronate, and in most cases the growth is improved.
Pamidronate has been shown to increase the level of mobility in moderate to severe OI most probably by alleviating the chronic
bone pain that these children live with daily.
How is the treatment administered?
Pamidronate treatments are given by intravenous infusion. This infusion typically runs 3-4 hours a day for 1 to
3 days depending on the center giving the medicine. This treatment is given roughly every 8 weeks for the first 2 years and
then the time interval increases as the child gets older and bone turnover slows down. Some children remain inpatient
for the entire infusion while other have their IV capped and go home at the end of each day Some lucky children
even have their treatments administered at home through a Home Health Care agency.
Some doctors opt to give the full Montreal dose(9mg/kg/yr) while others give the Omaha dose(4.5mg/kg/yr). There
is no tried and true dosage and the least effective dose should be amount all are trying to use. The hope is that
the medication is given by those knowledgable about the medications and it's potential side effects.
A DEXA scan (bone density scan) is a useful tool to monitor the effectiveness and possible overeffectiveness of this
At What Age Can Treatment Begin?
Pamidronate treatments can begin at any age. For children with moderate to severe OI it has been shown that
the sooner the treatment is started the more impressive the results are. There are doctors willing to put children on
it at birth or shortly thereafter with excellent results.
Cyclic Administration of Pamidronate in Children with Severe Osteogenesis Imperfecta
Link to Zolendronate clinical trial info:
New Hope For Children With Osteogenesis Imperfecta