Niko Gian · Osteogenesis Imperfecta

Pamidronate treatment

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Pamidronate //::  in the bisphosphonate family, a class of drugs that prevent the loss of bone density.

“Previous studies of OI bone have suggested that abnormal activity of osteoclasts and osteoblasts (the cells that form new bone) contribute to the bone’s fragility. In normal bone, the osteoblasts are producing new, healthy bone to replace the older bone that is resorbed by the osteoclasts.

This is one of the body’s natural processes to get rid of tissue that no longer functions well. In OI, imperfect bone is more susceptible to the osteoclasts, so OI bone is resorbed more quickly than normal bone. Meanwhile, the osteoblasts are producing new bone–but that bone is also imperfect, and abnormal collagen causes the osteoblasts to become less efficient and produce less bone.

This combination of imperfect bone cells, increased bone resorption, and decreased bone formation leads to the fragile bones that are characteristic of OI.” ~ Read more here: oif.org

“Treatment with Pamidronate is beneficial for the patient with OI, family and society and increased the quality of life. It improved bone density and the clinical symptoms, improved mobility, reduced fracture frequency and prevented severe deformities. A significant improvement in the ability of walking and in the psycho-affective tonus of all patients with OI admitted in our hospital and their families was obtained after treatment with Pamidronate.” – Therapy with pamidronate in children with OI 

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At Niko’s annual clinic visit at Children’s Hospital of Omaha, his bone density increased by 33% in his spine and 97% in his femur. These are the two places taken by a DEXA scan.

His annual dose was decreased by spreading out the intervals from 8 to every 10 weeks.

Niko started Pamidronate treatment when he was 2 months old and will receive treatment for as long as he is growing, or if his bone density reaches a normal range. He will receive an annual evaluation yearly on the same DEXA scan machine to ensure accuracy.

Children’s Hospital of Omaha oversees his treatment and our local endocrinology doctor works with us so Niko can receive his infusions at Children’s Hospital of Pittsburgh.

We work to ensure that the orders are written correctly and the IV team places the IV. For Niko’s first infusion at 2 months old, the team administered a scalp vein IV. For his second infusion, they used an ultrasound machine and were successful in his ankle. Most of the other ones were placed in his arm, and more recently, we have had great success in his hand…. this is the ideal spot and has worked out tremendously.

We haven’t had to use an ultrasound machine the past few times. We do not use a tourniquet and we have been extremely fortunate to hold him down safely during IV placement and blood draws post infusion.

The medication is run over four hours. This last time, in the first 15 minutes, the IV machine started beeping and we learned that the IV came out of his hand. I am thinking this happened because I put Aspercreme on his hands once we got to the hospital. Maybe it didn’t dry all the way, and it was greasy. The IV team quickly came and an IV in the other hand was successful.

I explained to Niko what we were doing, but it doesn’t take away his frustration or pain. Laughter, silliness, distraction, shows and watermelon does ease his frustration! We are extremely fortunate that although it feels like forever, it’s only a short time in an out-patient facility. The nurses are incredible and we do our very best to normalize our time there.

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Niko has been doing so well since his rodding surgery last spring. We are back to 100% regular activity, and then some!

In just a few short months, Niko is standing on new legs and celebrated his second trip around the sun!

Niko is 2

More to come!

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