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Saving Sydney's Bones From Breaking

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BOSTON, MA ( Ivanhoe Newswire) - 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 a new device helping make their lives a lot less painful.

Sydney Pardi has averaged ten to 15 bone breaks since she was one year old.

"It's kind of hard to do anything when I have a broken bone," Sydney Pardi told Ivanhoe.

"We would go into the emergency room at least once a month," Andrea Pardi, Sydney's mother, said.

She suffers from a condition that makes her bones very brittle called osteogenesis imperfecta. It's believed up to 50,000 people in the U.S. have it.

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

"The trouble is the child would grow off the rod and there would be a section of bone that would have no protection," Samantha Spencer, M.D., a pediatric orthopedic surgeon at Boston Children's Hospital, said.

Now, new telescoping rods grow as Sydney grows.

"The rods are inserted through multiple very small incisions; usually each around an inch long," Dr. Spencer said.

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

"It gives her a chance to build up her muscles and get walking and not have to go through another recovery," Dr. Spencer said

So far, they're working for Sydney.

"I really like having these rods because it heals my bones faster," Sydney said.

Today her breaks are few and far between and a lot less painful all thanks to this growing gadget. Sydney's mom said she's had just one break since January. Doctor Spencer said the telescoping rods are more expensive than the traditional rods. They cost about one thousand dollars, but since they lower the need for additional surgeries it can save money in the long run.

RESEARCH SUMMARY

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).

INTERVIEW

Samantha A. Spencer M.D., an Instructor in Orthopaedic Surgery and Harvard Medical School Staff Physician at Children's Hospital Boston Department of Orthopaedic Surgery, talks about breaking the cycle of breaking bones.

Tell me about Sydney.

Dr. Spencer: Sydney has a rare disorder called osteogenesis imperfecta, which means that she was born with brittle bones that are fragile and break easily often with no particular injury.

Prior to the rods, would you have been able to help her?

Dr. Spencer: Sydney began breaking as many do at walking age. As soon as she began to stand and try to walk, she broke her bones. She broke her thigh bone and a body cast can be put on, but then often the children rebreak as soon as they have healed and come out of the cast.

What are the rods you used on Sydney?

Dr. Spencer: The rods that Sydney has in both of her thigh bones and her upper arm bones are called Fassier-Duval telescoping rods. The idea of them is that they can grow with the child, so that they do not have to be replaced as the children grow.

How do they work?

Dr. Spencer: When it is first put in, it is screwed in at the top and bottom and then as the child grows from either end of the long bone, the rod telescopes part like an antenna.

What is the procedure?

Dr. Spencer: The procedure to insert the rods is a surgery because this rod has to go inside a long bone which is similar to bamboo. It has a hard outer shell and a spongy inner part. The rods are inserted through various small incisions, usually no more than an inch. Often the bones in these children are bowed, so the bones have to be straightened as the rods are inserted, but through very small incisions. Usually for each rod to go in a long bone it takes 2 to 3 hours, and then the children are in a cast for a month after the surgery until they have enough healing to begin to move and start to walk.

How do you get the rod in?

Dr. Spencer: There are many tools. To put it simply, you have a small guidewire that you thread in first and then you carefully align that down the center of the bone once you have it straight. You can exchange it for the real bigger rod and slide it over the top.

What are some of the difficulties of doing this procedure?

Dr. Spencer: The rodding instrumentation itself is somewhat challenging. I would say it is a tricky system, so it does require some expertise. The bones of these children are very, very fragile, so you have to be very careful as you do it.

Are there people that are better candidates than others?

Dr. Spencer: We generally place these rods in children with more severe types of this osteogenesis imperfecta. There are many different types. The most common form is type 1. More than half of the patients with OI have that. Many of them do not need rods and do not need the bone strengthening medicine we give the more severe children like Sydney.

What is the idea of the rod?

Dr. Spencer: The older technique was merely to take a simple rod, one that did not telescope, and put it in the center of the long bone. The trouble is then the child would grow off the rod and there were be a section of bone below the rod that would then have no protection and would break. The rods would have to be redone every year or two. The hope with these is that you can go quite a long time and the last I looked, Sydney has had hers in since spring 2007.

How long have they been around?

Dr. Spencer: They were FDA approved in the U.S. in 2003. Professor Fassier and Duval invented them in Canada some years before that.

Have there ever been times where you have had to take them out for any reasons?

Dr. Spencer: There have been about a 9 percent revision surgery rate for my series so far of 44 rods; which compared to the historical rate of over 50 percent revision surgery at 2 years for the simple rods, is better. I hope that it will give the children who have this disorder a better quality of life.

What are they made out of?

Dr. Spencer: It's a Fassier-Duval telescopic rod made out of stainless steel. Each end is screwed into what is called the epiphysis so it will cross the growth center without disturbing it, and then as the children grow from either end of their long bone, the rod will be able to telescope.

Is it expensive?

Dr. Spencer: While more costly than the previous simple rodding systems, the initial cost far outweighs the revision surgery cost. Something that will be strong enough and yet it is hollowed out and will telescope like that is much more

When you look at this as a doctor, what do you think?

Dr. Spencer: I am just so happy to make her quality of life a little better, but I know it is still very hard for Sydney and her family. She is a very nervous child and to have to come back and have repetitive surgeries. She has broken with these rods in, but the difference is then the rods hold the bone and they heal very quickly and so they do not need to go into a body cast. The time when they have another crack in the bone that has a rod in it, a fracture, I do not have to do anything except put a light splint on for a few weeks.

FOR MORE INFORMATION, PLEASE CONTACT:

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

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