Factor, prophylaxis and…complacency

For parents of boys with hemophilia anti-hemophilic factor products are heaven-sent. Combined with a prophylactic regimen the results are amazing. Although bleeding activity and severity can vary widely we have found that with prophylaxis Jack has few, if any, breakthrough bleeds and the restriction on his activities (within reason) are next to nothing. And this is wonderful. I’m sure there are untold numbers of people who see this active, go-for-it boy who would be shocked to learn he has a genetic condition that in the not too distant past would have likely resulted in debilitating joint damage or, even worse, an increased risk of death.

Jack has been on prophylaxis for nearly five years now, following a year of immune tolerance to defeat inhibitors. In that time we’ve transitioned from a port to peripheral sticks and with each step and a greater awareness (both Jack’s and ours) of recognizing and treating bleeds the results have been liberating.

But there’s a flip side. At times we can be lulled into complacency. A missed infusion here, a missed infusion there and nothing goes wrong. No harm, no foul; right? Busy schedules, few bleeds and we think we can skip a day and get back on schedule tomorrow. But it doesn’t always work out so well.

We were caught missing an infusion last week. At a time when we should have been hyper-vigilant we let our guard down. Our normal prophylaxis is M-W-F but sometimes we will purposefully alter the schedule to match up with games, events or activities to ensure that Jack has factor on board.

Now, Jack is finishing baseball and starting swim team practices. So last week he had swim practice on Wednesday, a baseball game Thursday, swim again Friday and baseball again (playoffs) Saturday. He had his normal infusion Wednesday morning but we missed his Friday infusion. On top of all this activity Jack planned to spend the night Friday at a friend’s house. So of course we got a call around 10:00 pm from Jack saying his leg was hurting and it was painful to walk. After all his activities it's no wonder. I collected him within 5 minuets, came home and Allison infused and Jack was off to bed. We suspect it was a soft-tissue bleed about mid-thigh. The next morning Jack couldn’t walk from the bedroom to the couch. He had a 9:00 am game so we infused again about 8:00 am. I dreaded the phone call to his coach.

I told Jack that if he couldn’t play he would still put on his uniform and go cheer his teammates. He said he wanted to give it a go, so we wrapped his thigh in an ace bandage and went to the ball field. I’m not sure the ace bandage really did anything save boost his confidence, but by the time we got to the field he could walk fine and after warming up he was jogging and running without pain.

He played and played well helping his team win. I’m proud of him for calling us when his leg was hurting and getting out and trying when he was unsure if his leg would hurt. He handled the situation like a pro. It was his parents who dropped the ball, letting distractions get in the way of the proper infusion schedule.

Of course we use this as a ‘teachable moment,’ using our mistake to show how important it is to take care of yourself and follow the proper schedule so as to avoid injuries like this.

Undoubtedly this won’t be the last missed infusion (try as we might, it’ll happen again) but it goes to illustrate that all the factor products in the world do you no good if not used effectively and how easily we can be lulled into a false sense of security when what is really required is constant vigilance.