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The elusive diagnosis

If there ever was a danger that we’d get complacent in this battle with Hermansky-Pudlak Syndrome, there’s always some new story, some new patient’s experience, that comes along, rattles our cage, and reminds us of how much work there is still to do.

Yesterday I received an e-mail from a woman I’ve e-mailed back and forth with on and off for several months now. I can’t get into specifics for privacy reasons – however, this woman could be the poster child for the HPS type of albinism. She doesn’t have just a few of the symptoms – she’s got them all.

I sent her information about how to get tested. While testing for HPS is relatively easy, it’s not a test the average lab can perform. The blood must be sent to Dr. White at the University of Minnesota and reviewed under an electron microscope. Dr. White looks at the platelets with this special, high-powered microscope. If they lack dense bodies and the patient has albinism, then they’ve got HPS.

Dr. White does the test for free. All any doctor would need to do is sign a lab slip enabling a patient to get their blood drawn and shipped.

But, this poor woman was e-mailing to let me know that she’s seen three of her doctors so far, and none were willing to do the test. Each passed the problem off on another specialist.

She’s frustrated, and she has a right to be.

I am dumbfounded. How can a doctor (one of them a pulmonologist by the way) be handed information about HPS, look at this patient, and not do this test? How could they not, at the very least, go do a Google search or crack a text book?

In general I find doctors to be truly amazing people. The amount of information they must carry around in their brains is staggering. I honestly don’t blame doctors when they’ve never heard of HPS. It’s ultra rare, and it’s simply impossible for every doctor to know about every single rare disorder out there.

But this patient has made their job so easy. She’s handing them a potential diagnosis on a silver platter. Since she has every symptom and belongs to a high risk group, why would they not at least do her the courtesy of setting her mind to rest one way or the other? They don’t even need to spend time scrambling around to figure out how to do the test – she’s handing that information to them as well.

Thankfully, this woman has spunk. She’s seeing doctor number four next week. Hopefully this doctor will do the test.

I wish I could say this was the only such situation that has come up – that it was some kind of fluke. But, sadly, it’s not.

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