STRENGTHENING BRITTLE BONES
Imagine having bones so weak that you break them by simply coughing or landing too hard when you jump. Between 20,000 and 50,000 Americans have a genetic disorder commonly known as brittle bone disease. There is no treatment, but scientists are testing a drug already approved for another bone disorder.
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Jimmy Foxx was born with legs but by age, 18 he'd broken them 24 times!
Jimmy Foxx
Amputee
"I'd try to play basketball; they'd throw me the ball, and it would land in my lap, and I'd break my femur. And I could see that they were an anchor that I was dragging around."
Having doctors amputate his legs actually gave Jimmy the chance to do normal things.
Jimmy Foxx
"When I was a little boy in physical therapy in the whirlpool, I broke my leg moving my leg against the motion of the water, you know, but within two months of having my legs cut off, I was at the YMCA learning how to swim.
Jimmy's disease is called osteogenesis imperfecta, orOI. It prevents collagen from forming correctly in the body.
Eric Orwoll, MD
Endocrinologist
Oregon Health & Science University
Portland, OR
"It's a disease that results in brittle bones, so fractures happen much more commonly than they should."
In mild cases, the skeleton looks almost normal, but in severe cases, fractures can cause deformities and a shortened stature.
Jan Reeder, MS
Physician Assistant
Oregon Health & Science University
Portland, OR
"There's a large population of adults with OI, and there's no treatment, and so we need something for them."
That something could be a drug called forteo. It's approved for osteoporosis. Jimmy gives himself an injection every day as part of a clinical trial.
Eric Orwoll, MD
"In osteoporosis, Forteo does work by forming new bone and strengthens the bone and reduces fracture."
This model shows a woman's hip before she took forteo -- this is the same hip after treatment. Because his upper body is so strong, Jimmy hasn't had a break in a while. He hopes the new drug will give him even more strength.
So far, Doctor Orwoll has treated three OI patients with forteo and all have shown improvement. The drug did cause bone cancer in rats when given at very high doses. Although humans haven't developed the same cancers, the FDA has limited its use to two years and does not allow children to take it.
BACKGROUND: Osteogenesis imperfecta (OI) is the most common disease causing fractures in childhood. It also causes fractures in adults. OI is a genetic disorder usually resulting from abnormalities of the genes that control the production of a protein known as collagen; this is the main protein in bone and essential for its strength. The fragility of bone in OI is due to the collagen problems; it has nothing to do with the calcium part of bone that is what shows on X-rays.
HOW COMMON ARE FRACTURES? Some OI children are born with fractures that have taken place in the womb. Others have their first fractures soon after birth or several years later. Some people with OI have so few fractures in childhood that the correct diagnosis is not made. Fractures are difficult to predict, especially in childhood. Some occur with normal handling. Others occur with so little trauma that the usual signs of a fracture may not be seen and the fracture is not identified until some weeks or months later when an X-ray is performed for another reason. The bones do not always behave in a brittle way; fractures may fail to occur when expected from an injury, and the reason for these variations is unknown. In men and women, and in almost all types of OI, the fracture rate diminishes during the teenage years and remains low in adult life. The reason for this is also unknown.
CLINICAL PROBLEMS: In addition to the fractures, there may be problems in other parts of the body; most of these are, like the fractures, the result of the defects of collagen. Also:
The joints may be lax, and the whites of the eyes may be blue or gray
The teeth may be discolored and fragile
Deafness may occur
IS OI INHERITED? OI in an individual is present from the time of conception. A mild OI disorder can pass from one generation to another. In some of these people and in most with severe OI, it arises without any family history. In most but not all of these, the cause is a 'new genetic mutation' -- the responsible change in the person's genes arises anew and not because it has been passed on from a parent.
FORTEO: A new drug with the generic name of teriparatide will be marketed under the brand name Forteo. Researchers are hoping Forteo will help patients with OI in a similar way that it helps osteoporosis patients. It treats osteoporosis by slowing or stopping bone loss by blocking the action of osteoclasts -- a type of cell that removes cells from the bone. Forteo stimulates the formation of new bone by increasing the number and action of bone-forming cells.
RISKS: During animal studies, rats were found to have an increased risk of developing osteosarcoma -- a rare but deadly cancer of the bone. No human subjects in the study developed osteosarcoma, but the drug is not recommended for patients who are at increased risk for osteosarcoma, children or growing adults, or those who have had prior radiation therapy involving the skeleton.
Eric Orwoll, MD
Oregon Health & Science University
Portland, OR
orwoll@ohsu.edu
www.brittlebone.org
Copyright © 2006 Ivanhoe Broadcast News, Inc.
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