My week began with a day in the skeletal dysplasia clinic. This clinic offers a range of clinical services for individuals with any type of skeletal dysplasia, which are characterized by differences in the size and shape of the limbs, trunk, and/or skull. This week the clinic was only seeing one patient, an individual with osteogenesis imperfecta type IV. Osteogenesis imperfecta (OI) is a condition that causes extremely fragile bones, and is often due to a defect in the gene that produces type I collagen. For those of you that have seen the movie Unbreakable, the character played by Samuel Jackson nicknamed “Mr. Glass” had OI, and Amelie’s neighbor Raymond Dufayel (also known as “the glass man”) was also afflicted. The patient in our clinic was a 36 year old woman, of extremely short stature (approximately 3’5”), who had been a community ambulator until she was hit by a car last year when crossing the street. She is now confined to a wheelchair and is awaiting a custom hip replacement due to a non-union of a fracture of her femoral head after the accident. This clinic takes a team approach, with the physician, the genetic counselor, and the physical therapist performing an initial visit with the patient for almost two hours, followed by a consultation with a nutritionist for another hour. The team then meets to discuss the case as a group and determine what areas of her care and health, and even personal life, are lacking or can be improved. This patient was in fairly good health, although she has near complete hearing loss in the left ear due to her disease, and is probably losing hearing in her right ear as well. She was concerned about going completely deaf, and the physician spoke to her extensively about this issue and what further information and tests she could get from an audiologist to help determine her prognosis. She also has dentinogenesis imperfecta, which causes her teeth to be brittle and she has had almost all of her teeth removed and will be fitted for dentures. I was very interested to learn that she has a child who is now three years old. I would not have thought it would be possible for such a small person with this disease to bear a child. It was a tough pregnancy, and her child is named Miracle because not only did she survive even though she had to be delivered prematurely by C-section, but she also did not inherit OI. I found this clinic experience very different from my other experiences. This patient got a large amount of one-on-one time with several health care professionals and they really examined every aspect of her health and life. This kind of treatment seems to be a good model for a disease like this with such widespread systemic effects.
I also returned to the OR this week, and saw two primary total knee replacements, a revision total knee replacement and a revision total hip replacement with Dr. Bostrom. One of the operating rooms I was in this week was outfitted with a camera, which greatly improved my visualization of the total knee replacement surgery. As in the past, the total hip replacement would have been incredibly difficult for me to see, but I was able to scrub in to the surgery and had a much better view. This revision seemed very difficult, as there was a lot of osteolysis (bone loss) and a lot of scar tissue. The entire surgery took over 2 hours, and in the beginning it was almost entirely dissection of the scar tissue just for the surgeon to be able to dislocate the hip. Even though there was bone loss, the femoral stem took several minutes to remove. The acetabular cup was well fixed, so the surgeon chose to leave the cup in place and just replace the polyethylene liner. The liner was very difficult to remove, and the surgeon ended up slicing it into pieces to get it out. The surgeon then used bone cement to implant the new femoral stem and also cemented a new polyethylene liner into the existing acetabular cup. Seeing this surgery gave me a new appreciation of the impact of a failed implant: the surgery was much longer and more difficult, and the damage to the tissue around the joint was very evident. The tissue was practically black due to the debris and osteolytic response caused by the failed implant. This patient will hopefully have a good outcome, but ideally these implants need to be improved so that they will last the entirety of a patient’s lifetime, so that these difficult revision surgeries can be avoided.
Other aspects of my week included two research meetings with Dr. Bostrom's group, a research meeting in the biomechanics research laboratory, and Adult Reconstruction and Joint Replacement grand rounds. As for research, we are still running RT-PCR on the mouse fracture study, which is going well.
My week ended with a very relaxing outing on Dr. Bostrom's sailboat. Now that’s the way to end a busy week!
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