Girl with congenital deformity must wear bike helmet to protect soft tissue of skull
Portland, Ore., Aug. 20, 1999 - Emily Lang is no stranger to surgeries. She's already had 121 of them in her short 12 years to correct various medical conditions related to deformities she was born with. But the one she will undergo on August 23 is different. This surgery will help get her one step closer to eliminating the need to wear a bright pink bike helmet to school in order to protect the soft tissue of her skull where holes in the bone have formed.
Physicians at Oregon Health Sciences University's Doernbecher Children's Hospital will apply bone morphogenic protein to holes in Emily's skull. BMP is a synthetically created product that initiates molecular and cellular activity that causes the body to grow new bone-forming cells called osteoblasts. These osteoblasts then develop into healthy new bone structures.
In October 1998 Emily had BMP applied to a gap in the right side of her skull. Now, 70 percent of that gap is filled with new bone. In this next surgery, BMP will be applied to several of the gaps in her forehead. Emily is the first child to ever have BMP applied to existing bone and have it successfully grow. BMP has been used to successfully grow bone in animals for many years.
"Emily can't wait till more of her skull has grown in and the day comes when she can go to school without the pink helmet," says Sheri Ficek, Emily's mom. "It will make her feel more like a normal kid and this surgery gets her one step closer to that dream."
"I am hopeful that we will eventually be able to use BMP to replace missing bone wherever it is needed," said Alan Seyfer, M.D., OHSU department of surgery (division chairman of plastic and reconstructive surgery) and chief of plastic surgery at Doernbecher.
Emily was born with several congenital deformities. Her physicians say she probably has Antley-Bixler which caused her elbows and legs to fuse together, craniosynostosis that fused the bones in her skull and Pfeiffer Syndrome which caused her face to recess and her ear canals to close off with skin covering them.
When Emily was six years old pieces of her skull disintegrated as a result of an infection, possibly related to a previous surgery to move the bones in her mid-face forward. The infection left Emily with large gaps in the front section of her skull. Now Emily wears a bike helmet to protect her skull where there is no bone structure.
Jeffery O. Hollinger, D.D.S., Ph.D., OHSU department of surgery (plastic and reconstructive surgery), and Seyfer have collaborated together for more than 15 years to pioneer the use and development of BMP. Hollinger and his team have received three National Institutes of Health grants totaling more than 5 million dollars to study BMP and other tissue-engineered treatments, with additional funding provided by Genetics Institutes, a company that produces and owns the patents for genetically engineered BMP.
Hollinger has pioneered tissue-engineering strategies that involve recombinant technology and polymer matrixes to regenerate bone deficiencies from developmental deformity, traumatic loss and resective procedures.
Physicians hope to use BMPs to restore shattered bones, osteoporotic fractures and cartilage degeneration. Currently, banked bones from cadavers, medical ceramics and artificial materials are used to replace missing or disfigured bones.
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