Lilly's World: October 2011

Finally something close to home!! Lilly got her first Pamidronate treatment this week. The pediatric floor in Indiana agreed to administer the medication. It was so nice to not have a 1+ drive. We went in Wednesday morning and the nurses were completely ready for her. One of the nurses actually has OI, what a small world. They put a scalp IV in and she did NOT like that. Fussed basically from 12-7, it wasn’t a fun day. They took some labs first and then she got half the dose of PAM over about 4 hours. She slept decent that night but mommy got no sleep. She rolls around so much and I didn’t want her to roll on her IV and it come out. The next morning they started to administer the second half of the medication and found that the IV was blown. So she got stuck again but this time in the hand. She didn’t like that much either but she did better than the day before. After that finished they took a second set of labs and we were out by 2:30. Perfect timing because Early Intervention was coming for an intake at 3. She is going to be getting some therapy through them to ensure she is developmentally doing as well as possible. Well it is now Sunday and Lilly is still not back to normal. She had a fever of 101* for 2 days and has had no appetite since Wednesday afternoon. I miss my constantly happy and smiling baby! This fussy, no sleep baby is not fun. Hopefully she goes back to normal soon.

So a lot of people have been asking me what exactly Pamidronate is. Here is a little explanation:

Pamidronate is one of several drugs known as bisphosphonates. Pamidronate is given through an IV. It inhibits the breakdown of bone. In normal bones new, healthy bone is produced to replace the older bone that is broken down. In OI, imperfect bone is more susceptible to the breaking down. Meanwhile, the new bone is produced--but that bone is also imperfect and abnormal collagen causes this process to become less efficient and produce less bone. This combination of imperfect bone cells, increased bone breakdown, and decreased bone formation leads to the fragile bones that are characteristic of OI.
A study was conducted on 30 children who were given Pamidronate and all of the children had low bone density scores also known as z scores. During treatment, the average bone density increased by 41.9% per year. The incidence of fractures also decreased from 2.3 per year average to 0.6. The results varied among the 30 children, but overall, many of the children gained greater bone density, reported reduced bone pain, gained mobility, and had fewer fractures during the treatment.