the Florida Hemophilia Association’s annual family meeting. About 330 people
attended, and we presented Pulse on the Road, which I’ll write about in a few
days, once I have the photos back! There was a motivational speaker, our insurance symposium, a men’s rap session,
a woman’s session, and a thorough presentation by Dr. Glenn Pierce of Biogen Idec
on long acting factor and gene therapy, and an energetic kids program.
|Dr. Joanna Davis: excellent
presentation on prophy
morning I attended a very detailed and comprehensive review of prophylaxis. You
know, many families do prophy, and I have read about it, and researched it, and
written about it for years. But today reminded me, you just can’t get enough education on
the subject, and we all need to be reminded of the basics. Dr. Joanna Davis, Medical
Director, Div. of Pediatric Hematology/Oncology, University of Miami Hemophilia
Treatment Center, and local “celebrity” as she was introduced, gave a five star review of hemophilia basics and how to do
prophy the right way.
by boosting factor levels up in the normal range by planned and frequent
infusions, whether a child needs them or not. She reviewed the difference
between different prophy regimens:
course of therapy. It prevents joint damage.
providing coverage during a high-risk situation such as team sports, or after a
surgical procedure. It stabilizes or improves joint function.
of what was infused. For FVIII: about 8-12 hours, for FIX, about 24-48 hours.
infuse on a Monday and you peak at 100%, for example. By Monday evening, a
factor VIII patient may be then at 50% factor activity (which means still in
the normal range). By Tuesday, the factor activity drops to 25%, and by Tuesday
evening, maybe down to 12%. Just before infusing on Wednesday morning, the patient’s
levels may be down to 2%–the trough. So prophy is scheduled according to a
series of the peaks and troughs of factor activity.
third prophy shot of the week. By Monday you’ll be depleted. She reported that
some families do a double dose of factor on Friday, to boost levels high, so the
half-life does not bring the factor activity down to 2% by Monday morning.
veins can’t take all the infusions and needle sticks, so a central venous access
device might be in order. This presents its own risks, such as blood clots. And
sometimes, children may balk at such frequent infusions, and teens can be in
denial and refuse to do their infusions (oh yeah, I can vouch for that one!). And
the big issue is cost. Prophy ain’t cheap.
ridiculously over-scheduled lives often make us want to wait till evening.
Think about it: infuse at night, when things are quiet, and by morning you’ll only
have half the factor working! Best to infuse in the morning, when your child is
most active and when the factor is working strong, at 100%.
of keeping logs about bleeds and presented the ATHNAdvoy, and wow! What a great
presentation too and what a great device. This is a web-based tool that will
help you track your bleeds and product usage, is accessible by your treatment
center, and can produce reports in a heartbeat. This is also available as a
mobile phone app, so you can use your Smartphone to quickly scan in your product,
report your bleed, and then you will always have a constant record, which you
can even print out in colorful reports. THIS COULD COME IN HANDY IN THE NEW
INSURANCE REFORM CLIMATE! Have I said that clearly enough? We should all be
recording every vial of factor we use. I would highly recommend using the
which is astounding. All the data is kept in aggregate and no personal information,
including your name is kept. A bit hard to explain all this in a blog; I recommend
visiting www.ATHN.org to lean more!
(Debbi, aka Wonder Woman, I owe you a dance off)