Children with a painful bone condition get some much needed help

Their bones snap with little or no cause and they can suffer hundreds of breaks in their lifetime. It's what some people with a painful bone condition can expect, and it can be even more excruciating for kids whose brittle bones are still growing.

Now there's something making those children's lives a lot less painful.

Not one, not five, but ten to 15 bones break every year. That's what Sydney Pardi has averaged since she was one year old.

Sydney Pardi, Suffers from Osteogenesis Imperfecta, explains how painful it can be, "It's kind of hard to do anything when I have a broken bone."

Andrea Pardi, Sydney's Mother, explains how often Sydney would have problems, "We would go into the emergency room at least once a month."

She suffers from a condition that makes her bones very brittle called Osteogenesis Imperfecta. It's believed up to 50,000 people in the US have it.

The breaks happen mostly in Sydney's thighs and upper arms. Doctor Samantha Spencer of Boston Children's Hospital inserted rods in those areas to help reinforce the bones.

Samantha Spencer, MD, Pediatric Orthopedic Surgeon Boston Children's Hospital, explains the issues they have with implanting the rods, "The trouble is the child would grow off the rod and there would be a section of bone that would have no protection."

Now, new telescoping rods grow as Sydney grows.

Spencer, MD, explains how the new rods are inserted, "The rods are inserted through multiple very small incisions; usually each around an inch long."

The stainless steel rod lowers the risk of Sydney needing additional surgeries.

Andrea Pardi explains how much it helps Sydney, "It gives her a chance to build up her muscles and get walking and not have to go through another recovery."

So far, they're working for Sydney.

Sydney Pardi explains how it has made her life better, "I really like having these rods because it heals my bones faster."

Today her breaks are few and far between and a lot less painful all thanks to this growing gadget. Sydney's mom says she's had just one break since January.

Doctor Spencer tells us the telescoping rods are more expensive than the traditional rods. They cost about $1,000, but since they lower the need for additional surgeries, it can save money in the long run.

MEDICAL BREAKTHROUGHS
RESEARCH SUMMARY

TOPIC: SAVING SYDNEY'S BONES
REPORT: MB# 3446

BACKGROUND: Osteogensis Imperfecta (also known as "brittle bone disease") is a genetic condition that is characterized by fragile bones that break easily. People who have OI often experience fractures, muscle weakness, hearing loss, joint laxity, fatigue, curved bones, scoliosis, blue sclerae, dentinogenesis imperfecta (brittle teeth), and short stature. Contrary to the myth that OI is caused by lack of calcium or poor nutrition, OI is actually caused by a mutation on a gene that affects the body's production of the collagen found in bones, and other tissues. There are 8 different types of OI and those who have a mild case can experience a few fractures, but those who have a more severe case can have hundreds of fractures in a lifetime.

FACTS TO KNOW: The number of Americans affected with OI is thought to be 25,000-50,000.
The range is so wide because mild OI often goes undiagnosed. Approximately 35% of children with OI are born into a family with no family history of OI. Most often this is due to a new mutation to a gene and not by anything the parents did before or during pregnancy (Source: www.oif.org).

COMMON SYMPTOMS: All patients with OI have weak bones, which makes them susceptible to fractures. Classic symptoms include blue tint to the white part of the eye, hearing loss, loose joints, flat feet, and multiple bone fractures. Symptoms in patients with a more severe case of OI include Scoliosis (S-curve spine), Kyphosis, and bowed legs and arms.

AVAILABLE TREATMENT: There is not yet a cure for this disease, but there are available therapies to help reduce pain and complications. Bisphosphonates are drugs that have been used to treat osteoporosis. They have proven to be very valuable in the treatment of OI symptoms, particularly in children. These drugs can increase the strength and density of bone in persons with OI. They have been shown to greatly reduce bone pain and fracture rate. Also low impact exercise, like swimming, can help keep muscles strong and maintain strong bones. People who have more severe cases may have to get surgery that places metal rods into the long bones of the legs to strengthen the bone and reduce risk of fracture (Source: www. ncbi.nlm.nih.gov).

TELESCOPING RODS: A problem among children who have a severe case of OI and have to undergo surgery is that they are outgrowing the metal rods that are inserted to reinforce the bone. A new device called telescoping rods grows with the patient. The rod is inserted through multiple small incisions, usually around an inch long. The procedure is recommended for children who have curved bones or who continually break long bones. It does not always prevent fractures, but the rod will provide an internal splint that can reduce the risk of displacement of the bone. The procedure allows the patient to be more active after a break occurs and it prevents prolong periods of inactivity and casting. The Fassier-Duval rod system, approved by the US Food and Drug Administration (FDA) in 2005, is the newest telescopic rod on the market. It was invented by an orthopedist with extensive experience caring for children with OI. It was designed to allow for a less invasive surgery and therefore a quicker recovery (Source: www.oif.org).

FOR MORE INFORMATION, PLEASE CONTACT:

Samantha A. Spencer M.D.
Children's Hospital Boston Department of Orthopedic Surgery
samantha.spencer@childrens.harvard.edu
(617) 355-6808


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