In hopes of being forgiven for stealing this photo, I'll tell you I lifted it off the BBC Website - which, by the way - is doing a better job of covering the crisis in Puerto Rico than most of the American media. This is a photo (Getty Images) of a recent demonstration march in San Juan.The road to getting the first treatment for the pulmonary fibrosis of Hermansky-Pudlak Syndrome has been a rocky one – and in the past week the newest obstacle is Puerto Rican politics.
When I was diagnosed with Hermansky-Pudlak Syndrome almost four years ago my lung forced vital capacity was 85 percent (it’s gone up to 94 percent, well in the normal range, probably because I didn’t know I had asthma and am now taking asthma medications.) When the National Institutes of Health did a CT scan of my lungs, they saw a number of “nodules.” At the time, they weren’t certain if this was the early stage of lung fibrosis, or simply the markings of a few past illnesses – but the prognosis they offered wasn’t exactly rosy.
In an effort to console me, they assured me that a Phase III trial of a drug they’d been studying to treat the pulmonary fibrosis of HPS would be starting in three months, and that based on my test results, I’d likely be a good candidate for the study.
The trial actually started this past September, three and a half years later.
From the time I was diagnosed, those of us with Hermansky-Pudlak Syndrome have been riding through the strange odyssey of getting a drug approved by the Food and Drug Administration (FDA).
First the rights to the chemical formula for the drug were sold to a new drug company. At first, this company wanted little to do with us. But, thanks to the tenacity of some of our researchers, a dialog ensued that lasted nearly three years. There were legal complications. There were disagreements about just how the trial should be constructed. There were manufacturing issues as the company tooled up to produce enough of the drug for all of its clinical trials. There were statistician considerations, and ethics board approvals to seek.
And then, after all that, the real work began to find the forty people who would participate. That’s no small order for a disorder that’s so exceedingly rare. There are a number of other complicating factors too.
But several people with Hermansky-Pudlak Syndrome have been admitted to the trial and work was underway to find more. Donna Appell and Liz Diaz had planned a trip to Puerto Rico in mid-May to speak to a number of people with Hermansky-Pudlak Syndrome on the island. Kevin, our nurse practitioner at NIH, was gearing up for what we hoped would be an onslaught of patients.
And then political chaos erupted in Puerto Rico eight days ago.
I am four generations removed from my Puerto Rican roots, so I don’t profess to know much about the politics of the island, but my understanding from media reports is this. The government of Puerto Rico is at a stalemate. For the last two years the Governor and the legislature have been unable to agree on a budget, or what to do about the island’s ailing finances.
Eight days ago each side seems to have dug in to stare one another down. Everything except the most essential services, such as police and hospitals, was shut down. More than 95,000 civil servants are out of work. More than 500,000 school kids are out of school because there’s no money to pay the teachers. Even the most basic civil services are getting hard to come by in some of the more rural areas of the island (so I understand.)
Today Puerto Rican bonds were downgraded by Moody’s to just a small step above junk bond status – which won’t help the debt problems.
Currently, it seems, the politicians are arguing over whether, and how, to implement a sales tax in hopes of raising the necessary funds to keep the island functioning.
This is bad news for Puerto Ricans in general, and it’s also bad news for those of us with Hermansky-Pudlak Syndrome.
Our much needed protocol recruitment trip has been postponed, and time isn’t on our side. We’ve already lost three and a half years.
The Hermansky-Pudlak Syndrome Network is currently working on an alternative battle plan. I’m worried that with so many immediate problems, no one will want to learn about their Hermansky-Pudlak Syndrome or care about traveling to the NIH. But, we’ve got a great team of people on the island doing a great job of outreach – and while they may be out of work too, I’m praying that we’ll find an opportunity in this chaos. Perhaps they’ll have more time for meeting with families affected by Hermansky-Pudlak Syndrome.
In the meantime, we just start planning the trip again.
The fact that no one I know without a special interest in Puerto Rico would even know about this chaos, the thousands of people protesting and the thousands of people out of work, is just indicative of so many challenges in the Hermansky-Pudlak Syndrome community. The only coverage I’ve seen, here in the great Midwest, is a story I heard on NPR a single day.
Puerto Rico is a part of the United States – a fact I seem to have to educate the average American about as I do outreach for the Network. It isn’t a foreign country. And yet, none of this has made our news??? No one cares that in a U.S. city there are thousands of people protesting and thousands out of work?
Comments
But, getting to the question of why Puerto Rico isn't getting more coverage here in the mainland U.S. - I've got a few theories. Coverage of pretty much anything that isn't happening right under our nose has been getting worse for years now. It's expensive to cover things in the far corners of the world, and as corporations try to make more money on the news, they cut resources and expect journalists to generate more and more with less and less. As a result, you find yourself covering the easy story, instead of the really good one. Look at how few American news organizations now have international bureaus? But, this is a blog for another night. I'm getting really, really tired and I need to go to bed.
Thanks for reading the blog!!!!