Backlog journals - First PAM treatment

The geneticist had finally approved Pam drug treatment after E's heavy spinal compression fractures. They initially used the term "Possible OI", and now they can confirmed it is a "real OI". At least we dont have to travel overseas for this treatment like what we have planned earlier. So Evelyn received her first pam drug treatment at 19 months old.

Pamidronate is given by a drip (infusion) into a vein through a fine tube called a cannula.  The infusion is given over 3 to 4 hours. After the infusion, the doctor will monitor and check if E has any side effects such as "flu like" symptoms, or fever. Usually baby will get these side effects on their first treatment. We were lucky that Evelyn had been doing great during & after several hours of the infusion. However, we still need to stay overnight for close monitoring. Evelyn discharged the next day without any side effects still. Woo hoo….



Evelyn sleep throughout the 4 hours pamidronate infusion



There are 2 tests to be monitored yearly along with the pam drug treatment:-

1) DEXA scan to check her bone mineral density. This is to monitor the progression of her bone density after the treatment received.

2) Kidney ultrasound to check if her kidneys are normal, because a long term drug treatment will cause to kidney stones.

The researchers proved that children who received this treatment have made a remarkable improvement in quality of life. Many of the children gained greater bone density, reported reduced bone pain, gained mobility, and had fewer fractures during the treatment.

Evelyn can bear weight at 20m - still shaking her legs

We just love the pam treatment. Evelyn had gained so much strength after her first treatment. She can bear weight at 20mo. Her appetite had improved gradually too. Her weight stop at 6.2kg over the past 6 months (15mo to 21mo), she gained 0.5kg to 6.8kg at 22mo. she was still far behind her peers. We cant force-feed her. Severe OI children have tiny and fragile bones and poor muscle tones so the expectation on their weight & height are totally different to the normal growth chart. In OI world, they would not look at the "normal" chart, instead they said "Let them grow at their own pace".

"No matter what you're going through, there's a light at the end of the tunnel and it may seem hard to get to it but you can do it and just keep working towards it and you'll find the positive side of things". - Demi Lovato

Evelyn weighed at 22 months old : 6.8kg, 74cm