Purple Canaries

Joyce Gould with Jill Gould

We dance round in a ring and suppose, But the Secret sits in the middle and knows. – Robert Frost

To all my porph friends—patients, caregivers, their friends and family members,

I apologize for having fallen off the face of the earth for a while though I tend to do that when the porph devil’s dancing in Jill’s liver and overdoing things again.

Because of that, I’ve been back on the exhausting documentation trail—and once again, making progress for her and others though, yes it is truly frustrating. In response to a FB closed forum participant’s request, I had conducted a patient survey (very delayed) and thought I’d put a pretty good report together when the sh#t hit the fan. Jill’s AIP has led me yet again to more (“new” to me discoveries) and while it’s been intensely trying for me (never mind her—the patient!!! and those medicos who want to believe but can’t because 1) the diagnostic information they are fed is erroneous and 2) as Robert Frost said, “We dance round in a ring and suppose, But the Secret sits in the middle and knows.”

If you haven’t heard yet, there are two stories of APF devotees connected to the Alnylam Pharmaceuticals’ Givosiran clinical trials you should know about. One has AIP (“expert-confirmed”) and excretes mega-U-BP during attacks so was readily accepted into the clinical trials where she by all accounts has been doing so well she was elevated to an “esteemed” position—an Alnylam Pharmaceuticals spokesperson. The other hasn’t been so lucky. She, too was an “expert-confirmed,” (HCP) APF devotee—so devoted, she’d actually come to U.S. to “learn” APF’s methods, such as they are. However, when time came to enter clinical trials, her U-BP didn’t cut the mustard. She was denied access to the Givosiran clinical trials. Sad to say, her condition deteriorated and I’ve heard she is dying. Why? Her U-BP isn’t good enough. So, essentially, her expert-confirmed diagnosis was yanked and she’s left to die. Sickening, no?

I have finished the “porph-psyche” paper, titled “Acute Porphyria is not “all in your head.” It’s in your blood and liver….” I’m very far behind in FB messages but will be posting this latest paper on my book website, www.purplecanaries.com. It is sure to raise Cain once again. Oh well. I will share the piece first with the anonymous closed forum participant who so long ago asked me to conduct a closed forum patient survey and who’s waited so patiently for it to be finished. I received some wonderfully brave input. Kudos to all those who responded with information to this intimidating topic. I live for the day when our collective voice is heard—and the nefarious individuals contributed to the aching bodies and hearts for decades will finally reap what they have sown.

I thank patients, caregivers, (Moms & Friends), God, angels (heavenly and earthly) and saints, the universe for the all the inspiration and help that has come my way. Dr./Atty. Philip Reilly wrote in his book, Orphan: The Quest to Save Children with Rare Genetic Disorders, about “concerned parents…who have become disease experts.” He also quoted Sir Archibald Garrod who said, “the first great advance in treating rare human inborn errors of metabolism began because of the persistence of a heartbroken, but dedicated, mother and the curiosity of a young physician-scientist.” I haven’t as yet come across a ‘physician-scientist’ but fully intend to persist in this situation. There are too many unacknowledged porphyria patient like my daughter, not only in the U.S. but throughout to world allow it to continue. The U.S. sad state of affairs concerning the acute porphyrias is akin to “playing with fire.” I for, one, don’t play with fire—except when it comes to shooting flaming arrows at those who have such blatant disregard for human life.



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