As founder and guitarist for Twisted Sister, Jay Jay French is used to making a lot of noise onstage, but now he is raising his voice to scream “We’re Not Gonna Take It” for another reason: to promote awareness of uveitis. His 17-year-old daughter, Samantha, has been battling the ocular disease since she was first diagnosed with it at age six. Uveitis creates swelling and irritation of the uvea, the middle layer of the eye that provides most of the blood supply to the retina, and this inflammation causes white blood cells to attack the problem, mistaking part of its body for something foreign; if unchecked, uveitis can lead to glaucoma and ultimately blindness. There is only one specialist in the country that has a research facility devoted to finding a cure, and French wants to help change that. On top of a benefit concert being performed by Twisted Sister at the Best Buy Theater in NYC on April 29, a special guitar and amplifier auction is being held on May 1 in Boston. (More information can be found at PinkbustProject.org.)
“There are a couple of things that I’ve learned,” French declares about the emotionally exhausting process he and Samantha have gone through. “Although the disease is the leading cause of blindness among American girls, out of the 30,000 ophthalmologists in the United States only about 100 really know anything about uveitis. And out of the 100, three would be considered specialists, and out of those three, one has a research institute.” That lone individual is Dr. Stephen Foster of the Massachusetts Eye Research and Surgery Institution (MERSI) in Cambridge, Massachusetts.
“If your kid is seen by a doctor in Alabama or a state that has no specialist, the ophthalmologist [might] see that there is some sort of inflammation and do some rudimentary research about what to do under these circumstances—write a prescription for steroid [eye]drops, take them until the [white blood] cells go away, that kind of thing—without understanding the underlying nature of the disease. It’s an immune disease and topical drops are not the long-term answer.”
French’s daughter Samantha has gone back and forth over the years between trying new drugs, weaning off of them to see if her body can deal on its own, then going back to using eyedrops—which, ironically, if used too much can lead to blindness—then trying a new drug. He says the key to uveitis treatment is to have it diagnosed, to use the eyedrops in the short term to get the cells down and come up with the right drug cocktail to turn off the body’s mechanism for creating the disease.
“Every step on the ladder with her has been reached because Dr. Foster feels that the previous one didn’t work correctly,” explains French. “And since he is more or less the Yoda of this, you come to the conclusion that this drug doesn’t work or this drug plus another one doesn’t work or the next one works but is problematic because it lowers your immune system so much you get sick. And so on and so forth. So she’s on her sixth cycle of drugs at the age of 17, and the current cycle is with a drug called Humira, which is advertised for rheumatoid arthritis. It’s got all these nightmare [side effects] scenarios like cancer or death. It’s injected every two weeks, and so far she’s gone into remission on this drug. However, it’s an immune lowering drug. You’re susceptible to infections and more intense colds. She was on a drug called Remicade for two years that is an injected/infused drug. It took four to five hours in a cancer ward and had to be administered every 6 to 12 weeks depending upon the cycles. The cost of the drug to the insurance company was almost $20,000 per dose, and it was the first drug that substantially reduced the cell damage in her eyes.”
Since going on Humira, Samantha has been in remission since last August. French says that the rule of thumb is if the remission goes for two years, then doctors will wean the patient off of it to see if their body can stand alone. “There’s no cure for this disease,” stresses French. “The disease has a strange pathology. For reasons they do not know, it goes into permanent remission with a certain percent of girls after—which is good news—except there’s no way of knowing if Sam is one of them. And the only way you know is to stop taking the drug. What’s the danger of not taking the drug? The cells come back. Then you start your cycle all over again [with] the drops and whatever medications. This is what we’ve been living with for 12 years. As a parent your heart breaks every time you have a doctor’s appointment. She hates taking drops; they sting in her eyes. She always wonders if she’s doing it correctly or not. She gets depressed. Add to that a normal adolescent girl and you have these issues. So I’m out here fighting a fight I never thought I’d have to fight.”
For French, his daughter and her mother, the journey has been an emotional rollercoaster ride. After uveitis goes through remission and then re-emerges, that renewed period of activity can be depressing and “crushing emotionally, and you have to spend a lot of concentrated time to get a handle on it again before the disease does permanent damage to the eyes,” says French. More doctors need to join the fight against uveitis, but in the meantime, parents need to be aware of its existence and inherent dangers.
“Here’s the bottom line: if you took every six-year-old girl in New York and screened them for uveitis, you’d probably find in a population of 8,000,000 about 1,000 undiagnosed cases,” states French. “If those kids were treated right away, the chances of losing their sight would be greatly diminished. If a kid has uveitis but doesn’t know it—for the first couple of years you can’t see the damage and don’t feel anything weird—those two or three or four years of non-treatment leads to damage that cannot be brought back. It’s mostly girls that get this. If your kid is six years old and hasn’t been to a doctor, make sure they get an eye screening. It’s a real simple thing. [Samantha] was seen by an ophthalmologist, a young kid out of medical school, at her public school. What’s criminal is that ophthalmology students don’t know how to recognize uveitis.”
After getting the school doctor’s note about inflammation, then consulting a specialist in New York who treated uveitis, French, his daughter and her mother went online and found Dr. Foster, who was at Massachusetts Eye and Ear at the time. “He was very purposeful and very direct,” recalls French. “He didn’t think the eyedrops were effective, but [looked to] systemic drugs, which scared me and her mom. You don’t want to hear about chemotherapy. And beyond being checked for uveitis, you have to be tested for JRA [juvenile rheumatoid arthritis] every six months because it could transform and go to the rest of the body, in the joints, and many girls with JRA have to be screened for uveitis because there is a much higher percentage of girls with JRA who have uveitis than go the other way around. Very few people who have uveitis first go over, but some do. You also learn about JRA and have to have a battery specialists [for that].”
To help raise money funding to fight uveitis, Jay Jay French gained the support of his bandmates to do a benefit show and also started his Pinkburst Project three years ago. It is a unique, groundbreaking collaboration between guitar and amplifier manufacturers to fight the good fight, and the Twisted axeman got different guitar makers to replicate his famous pink guitar. “I had all these famous guitar companies copying a Gibson guitar, which is like asking Honda to make them look like a Cadillac,” he quips. “If you’re a car collector, you’re going to want that Honda because it will be the only one that ever looks like a Cadillac. So I had Martin, Grestch, Paul Reed Smith, Epiphone, Roukangas and Fender all try to look like my Gibson Les Paul because I knew that guitar players would love how crazy that idea is. So I got them to do matching paint and created a collection of 13 guitars that you’ll never see again. You never will. The trapezoid inlays, the bound fret boards [with] the company names—they’re all one-of-a-kind guitars of the most famous models.”
Further, French created guitar pairings with specific custom amps from the likes of Marshall, Fender, Vox, Mesa, Orange, Hartke and Diamond, and he proclaims that it is the only time in history these amplifiers companies agreed to be covered with the same color tolex “because the tolex is a propietary thing. A Fender covering is not a Marshall covering or a Mesa covering. I have matching [pink] guitars and amps. If you want to find a Fender telecaster Pinkburst with a Fender pink amp to match, it’s the only one in history. That’s it. It’s the only game in town. Hopefully that kind of stuff drives up the prices in an auction. These are the tools that touch the soul of many people. Hopefully a rich collector will want every one of them. I’d love to see the whole collection bought by one person and put on display, but however it goes it doesn’t matter. These are tools to earn money to go to research for uveitis, so some day my daughter doesn’t have to have this damn disease.”
An evening with Twisted Sister & Friends will take place at the Best Buy Theater in NYC on April 29. Proceeds will benefit the Ocular Immunology And Uveitis Foundation.