A man who was a champion of the fight to get extra metal drugs to terminally ill patients died last weekend. David Huntley was a professor, and athletes, a husband and a father. He was diagnosed with ALS in 2013. By the time he visited the KPBS Studios last March, he could no longer speak and committed by way of a software program. He wanted to be able to take an unapproved drug called Jim 604 which has shown some comments and slowing the progression of ALS at the FDA did not allow it. We're bringing you his interview again. An interview that included a conversation with his wife Linda Clark and Dr. Harry Hartstein director of the article and translational research Institute at UC San Diego. Dave, why to what the chance to take Jim 604? I have had ALS for two and half years I will pass on the next 6 to 12 months. There no drugs in the pipeline for approval in that window and most of the drugs under investigation treat the symptom and not the disease. Early clinical trial of GM604 resulted in a significant slowing or cessation of the progression in seven of eight treated patients. When GM604 was given to another patient outside the clinical trial, he saw modest measurable improvements and function. If forced to go through a phase 3 trial before approval, it'll take three years and I together with 15,000 ALS patients in the US and three had to thousands worldwide will die. This drug has not passed clinical trials? It doesn't concern you it could be harmful to you? GM604 has been given to 32 healthy volunteers, non-analysts patients, 26 stroke victims and for Parkinson's disease patients with no adverse events. The city has been devastated. Even if there was not such a strong body of evidence for safety, I would want all ALS patients to have access to GM604 because our diseases of hundred percent it'll and a tolerance of potential risk versus reward should be a decision left to the patient in consultation with their doctor. What you think GM604 could do for you ? I believe GM604 has the potential to stop my progression and perhaps restore some of the functionality I have lost. What is your condition now? Before getting analysts I was a university professor and established lecture the groundwater resources Association of California. In the last year I have lost the ability to speak, four years ago I completed both Ironman and the Boston Marathon, now I'm restricted to a wheelchair, and able to run, walk or stand. I cannot sell more than 10 liquids so take my nutrition through a feeding tube into my stomach. I have lost the use of my lips and most of these of my arms so I can no longer hug or kiss my wife, daughter or grandson. How is it affecting you knowing it is a joint out there that may help you but you aren't allowed to take it? It tears me up largely because of my family. Can you imagine the impact on them if two years after I die, results of phase 3 testing confirm the early results that GM604 would have saved my life ? It also frustrates me because I can't see the logic of denying access to a drug to population afflicted with a rapidly progressing disease that it's 100% fatal. Thank you. Thank you for inviting me. Guest host Tom fudge interviewed David Huntley's wife to urge the FDA to allow the jury to be made available to terminally ill patients. What has it been like for you and David's daughter to see him in this condition? Is been devastating. When he contracted that this illness he was in the healthiest condition of his life. He was competing actively in triathlons, we were both involved in skiing and sailing and swimming and cycling adventures. All of that has been taken away. The worst type in the loss of key medication. When he is lying in bed, he can't come me to alleviate his discomfort. There's no way to Connecticut what the problem is. I assume you been told about that ALS is a deadly disease and interval disease? There's no treatment. How did you hear about the drug GM604 My husband, the consummate researcher is always looking at clinical trials around the world of this disease. He participated in one medical child so it's on his radar when this drug released the results of their phase 2 study back in October 2014, we immediately contacted the manufacturer and asked what the next steps were and how we could possibly gain access to this drug. What did your doctor tell you about David's chance to be able to try the drug? Are doctor said he would support us if we could gain access through compassionate use but that is a very lengthy process. We don't know the manufacturer will make it available. That's one alternative called compassionate use. It's very difficult? It takes a very long period of time in this company once already we are in contact with the patient that received the drug. He personally believes he benefited and he has with us on this campaign to get the FDA to grant accelerated approval which is the best way to make it available to patients. If your husband able to take this expand mental drug, what would be your hopes at that point? This is a disease modifying a drug. It's a multi-targeted drug on like a lot of other analysts drugs, it is being shown to slow or even stop the progression of ALS. That is monumental in the field of ALS research. No other drug has been able to do that. So another it's David would get better but he wouldn't get worse? It would buy him time and that would allow the start to be rather refined or other drugs to become available in hopes of neurogenesis in which you regain some function. You're listening to midday edition, I'm speaking to Linda Clark who is an advocate of a right to try legislation. Right to try refers to the ability of people court terminally ill to try expand mental drugs that haven't been approved by the FDA. Linda, why you feel so strongly this legislation is something we should accept? The legislation is the voice of the American people saying we reject this idea that the FDA continues to withhold drugs that show promise of efficacy especially in the case of terminally ill patients. Drug shows it's safe and has real potential for improving a patient's quality of life, extending their life are possibly even saving their life, it makes no sense to withhold that drug. GM604 the drug we're talking about is now days to Charles? There was a meeting between the manufacturer at the conclusion of the phase 2 trial at which time the manufacturer applied for the accelerated approval of Graham. The FDA could forgo phase 3 and make it available in sets conditions. They have a lot of latitude to decide how it should be distributed and what the post marketing surveillance requirements are so we gain valuable information while these patients are trying this drug. To talk more about the legislation. In fact midday producer spoke with California assembly men who introduced the rights to try bill in the lives that your. Explain why things terminally ill patients should have access to experiment with drugs. I think was difficult as legislators, you have your political hat and your public service hat. There's also your human hat. I'm an individual and people think because I'm a legislature the decisions I make and separate from all those decisions when in fact I suffer the same consequences I from decisions I make as anybody else. If it came to one of my family members or myself, I feel like I should have the right to live in a state that allows me -- my greatest time of need to swing the fences and try to save my own life . I think that's what the root of this legislation is. Would be eligible to take experiment till medication should the bill pass . it doesn't require anybody to do anything. It affords people the right to try and expand mental drug without the state coming after you, whether you're the patient, or the drug manufacturer or the doctor. Those that will be eligible for this bill are those that are determined to be terminally ill by the doctor. That's between the doctor and the patient, it's not something we are defining. Essentially somebody who has their doctor tell them you have a terminal illness. That's who will qualify for this bill. Wiseman the current FDA process good enough? Think it's better to have that than nothing. Also takes time to be approved for that program. If you go into the doctor and the doctor says I'm sorry but you have six months to live, three of those months you with the FDA to approve you for compassionate use program, that's your life . I think at the moment the doctor tells you you have it terminal illness and it will result in your death, you should have the right to try and expand mental drug to save your life . it's not like you could try straight up or go you have to exhaust all other options before you can use this option. What other legal drugs are out there, you have to exhaust that but at the same time it doesn't include you -- preclude you from trying these drugs. At this point, it's not something the state allows you to do. Was your response to people who says it might try bills aren't now enough, they could undermine the clinical trial process. I don't think they are wrong. I think the point of the bill is too broad. Where not requiring drug manufacturers to offer their drugs to any patients. That's a choice they have to make on their own. We are a lot of people in this state as well as doctors to prescribe it and allowed those patients to use it. If the manufacturer doesn't want to do, that's their choice. That's not what we're after in this bill. That's California assembly men in Calderon. It will allow terminally ill patients to use expand mental drugs. Joining me in studio is a Linda Clark whose husband suffers from ALS. And Dr. Gary Fierstein. Gary, thank you for coming in. Doctor, I think most people would look at David Headley's situation where he has ALS and say why not? Why not let him try this experimental drug? I think you have concerns about right to try. It really represents a larger societal question of the rights of the individual compared to the needs of the population at large. It's very difficult to argue at the extremes with a clearly terminal disease that there should be some opportunities such as this. On the other hand, I think is very little question that this will have impact on the ability to run and recruit patients into formal ethical trials. Why do you feel it would compromise that process? The way clinical trials generally operates, there's a test drug or medicine and are typically two groups of patients, one receives a test drug was unknown whether it works or not, and the other receives either the current standard of care that's available or in some cases of placebo, depending on the disease. If there is an alternative mechanism to bypass that, it has the potential for undermining the ability to recruit patients into clinical trials. Most want to receive the test article. The thing to remember, the vast majority of Asians are ineffective in phase 2 studies or even in face three. The side effects typically aren't necessarily seen in six or 12 patients but require sometimes hundreds or thousands of patients in order to get a full appreciation. In essence, if you provide a bypass pathway it has potential downstream effects in terms of more traditional clinical trials. Are you saying if this drug is available to all people who are terminally ill you may not be able to get enough people to volunteer for these trials? We want to be certain if we give an experimental agent to people, we learn something. Heard that one of their proposals would be to collect information on anybody that receives the agent. Still, the quality of information, the ability for most diseases to be able to make an accurate risk benefit assessment on a drug that should be widely debated does typically require larger scale studies. Linda Clark, I'm sure you heard some of these arguments opposed to write to try. What is your response to them? I think right to try is important because it sends a message that we have to look at a new way of approving drugs and accelerating that approval to go right to try can be improved by requiring a post marketing surveillance where you collect data so we dance of the science, but also for consumer protection. We want to know what's working if it has safety concerns. With respect to very rapidly progressing fatal illness with no treatment, we think it deserves a protocol of its own and not the 50-year-old standard which is a double blinded randomized controlled trial judge takes 10 to 15 years, millions of dollars that makes no sense when you have people that will die within a few months to a few years. I've been told you not in a position to say with your four or against right to try, for people who are terminally ill, are there other options? Can we make it broader? That would be a reasonable approach. As mentioned earlier, some of the right to try types of mechanisms would make a lot of sense from the perspective of the individual rights. It would potentially come up with more creative trial designs and move away from long-term studies as important. The other thing to keep in mind, at the extremes, it seems pretty clear. Doctors don't have a crystal ball that allows them to say you have a fatal disease in most cases. What I worry about is what the actual criteria would be for deciding when hopelessness begins. In some cases, it's very clear. In other cases, I think the line is not as clear-cut as most people would like to believe. Linda, how many states have right to try it laws? I believe this about I've 26 other states that are currently proposing legislation. This is something being considered. We're going to continue proposing accelerated approval. We are working nationally with other people. Have a quarter of 1 million signatures on a petition to the FDA. To grant this drug, it fits squarely within their own definition of what should be granted, accelerated approval. Past stayed in efficacy demonstrated in a phase 2 trial. Are going to continue until we get approval. Linda Clark is an advocate for right to try it legislation which last terminally ill patients to use expand mental drugs with the approval of the drug company. Thank you for coming in. Once again& Come -- has been -- Linda's husband suffers from ALS. Professor. David Headley died at last Saturday. Conversations continue between the patients and the FDA to reach agreement on accelerated approval for the use of expand mental drugs. Helping his right to try at Bill who is headed to the Senate Appropriations Committee next Monday.
As many San Diegans spend their weekends around the barbecue, a PBS show helps them avoid the grilling rut by offering ways to flavor food with smoke.
Steven Raichlen is the host of “Project Smoke,” a show where he offers tips and recipes for smoking meat, vegetables and cocktails. The 13-week series aired in June.
Raichlen, who is also the author of 28 books, including “The Barbecue Bible” and “How to Grill,” said smoking is a slow process but ingredients are most important.
“The quality of ingredients matter tremendously,” Raichlen told KPBS Midday Edition on Tuesday. “We use grass-fed beef, organic chicken. They taste better. They are better for the local economy. It’s a win-win situation.”
Chef Jeff Rossman, who owns Terra American Bistro and Bunz restaurants, said there are many components that can make a burger great.
“The classic burger is just simple pickles, mustard, ketchup — but for me, it’s changed,” said Rossman, is author of "Build Your Own Burger: BYOB.” “Everyone wants different flavors, add-ons, crusts and rubs.”
