Thursday, June 30, 2011

A Day At The FDA

I was part of the Afamelanotide trial last summer and was fortunate enough to have received the real thing. Unless you have EPP, I cannot explain what a miracle that was. But I did get the chance recently to do just that at the FDA Office of Orphan Diseases. Another fellow EPPer, Matt Johnson, joined me. In my 62 years I had never met anyone with EPP outside of my family (I have two cousins with EPP). That was an experience in itself. Those of us with EPP know that we can never explain to someone without EPP what it is like, but listening to Matt Johnson was like reading my own thoughts. Matt and I spoke a couple of times during telephone conferences before we actually met at the FDA in Silver Spring, Maryland. Although our session was scheduled for 10:30am, we were asked to come early to meet with Dr.Timothy Cote, the head of the Office of Orphan Diseases. I arrived just before 9am and Matt was close behind. Dr. Cote had just returned from Israel two day prior and was sick the day before, so he was not in good spirits, but after a few minutes listening to Matt and me, he was beaming. He told us that his staff, like all bureaucratd, tends to lose site of their mission and was delighted that we were there to remind them that we are out there.

Dr. Bob Desnick, a member of the APF Advisory Board, was also to attend, but his flight from New York delayed him an hour. By the time we were ushered into the conference room over 30 doctors and health professionals were waiting to hear what we had to say. For an hour Matt and I regaled them with the inexplicable world of EPP and the miracle of Afamelanotide during which time we were videotaped. We tried to tell them of the unrelenting pain, the isolation and self-doubt, and ultimate despair. Then we told them about our experience Last Summer and how it had changed our lives. We weren't supposed to directly discuss this, but it just couldn't stay untold - so we did. Dr. Desnick translated what we had to say into meaningful medical speak, but I believe that our words had more impact.

Like most families, we have gone to the beach almost every summer, and every summer after 15 minutes, I am banished to the house and sit with my feet in a lobster pot of ice water for the next 5 days “sipping” copious amounts of adult beverages. Needless to say this is hardly endearing to my wife and makes everyone wonder. But last summer, I spent hours on the beach with virtually no pain – a certified miracle if ever there was one. Like most EPPers, I have never told anyone outside of my immediate family about my condition. My colleagues at the office began referring to me as Mr. Tan without ever knowing why.

But the most compelling thing that day at FDA was a video that Matt had brought. It was a video of the episode that led to his diagnosis. He was 11 at the time and had gone skiing with his family. The video showed the progress of the effect of EPP over the period of about 10 days. As most of EPPers know, even though the pain has past, we are not a pretty site after 10 days. It had a profound effect on our audience.

Drs. Cote and Mueller thanked us for our time and promised that they would advocate our case to the extent they could, but could promise nothing. They explained that there is concern that the drug would be used for “off label” usage and that it would take time and effort to weigh the risks and benefits of a drug such as Afamelanotide. Dr. Mueller told us that she would share the video of our session with the FDA Center for Drug Evaluation and Research. I believe that this was a first and significant step in getting approval for the use of Afamelanotide, but I am not sure that the end is in sight. I live 45 minutes from the FDA campus and with the help of APF will continue to do whatever it takes to gain approval for use of Afamelanotide.

Tuesday, June 21, 2011

Thoughts…Ideas…


       I would like to let you all know how wonderful the Porphyria foundation has helped me.  I always love to help in any way I can and I think most of you are that way to, but have you asked yourself what is your limit?  Do you know you limit?  Do many people pull you in all different directions?  When we feel great or good, we pack everything that needs to be done or want to do all in one day, reminds me of going on vacation to see and do everything in one trip because you may not get back there. You all know what I mean.  Many people that have a disease wear T-shirts, bracelets, raise money, run races and so much more.  I see so many people get depressed and have low spirits because with Porphyria there are different types that affect us in so many ways and does affect our everyday lives.  So if you find yourself in that downward state what can you do to be happy?
·        Find an activity to do, read a book, paint, crafts, write your thoughts down in a journal
·        Exercise, stretch, go for a short walk this you can do inside or out
·        Could you take a day trip, favorite place to shop, visit a museum, go to the zoo
·        Take a vacation give yourself a break even if it’s a stay home vacation
·        Reach out and call a friend or family member, write a letter and mail it  It would touch some ones heart (old fashioned I guess)
·        Do something with your friends inside or outside, grill make a lunch or dinner date
·        Spend quality time with your family
So if you’re down today think of these fun things to do!  The most important thing is to get up and do something and have your life be in balance.  You can always volunteer to spread the message about the APF and send them a letter or note to thank them for all that they do.

Amy Chapman- AIP Patient

Monday, June 13, 2011

Searching for a Good Doctor.

I was thinking today, Oh another new Doctor today.  I was not happy nor Sad, but I thought I’ve got to explain Porphyria again….I could do this in my sleep…. I was thinking.  I had to be honest I had a bit of a negative attitude.  I think we have all felt this way at one time or another, how is the new Dr. going to treat me are they going to understand me, believe me, look at 
me weird, think I’m crazy…

I had all my Papers and booklets and Binder ready to go.  I was so nervous and this in turn made me not feel so well, but said to myself and prayed that I could have the strength and confidence that I could get through this.  I got there and waited they did everything so fast {get you in and out}.  Waited for a bit and then it was my time to see the Dr.

The Doctor shook my hand introduced herself to me and said you have a rare disease I was told, what could you possibly have? She asked!

I told her, she looked at me with the crazy look and then excused herself and said she would be right back.  She came back a bit later, she said she had to go look this Disease up because she had heard of it in school but never thought she would see anyone with this condition.

She said she could help me but she really did not want to.  I told her that we hear that sometimes.

But this was # 6 of searching for a new Dr. I was still sick.  My mom happened to be on the phone to find me someone to see.  Ok we may have someone.  All those feelings all over again.  Those of us that go through this so often.  Made an appointment for another new Dr.  Checked In for my Appointment.

I could not clearly understand him, but he had a sincere smile and seemed to be very concerned about me.  He listened to us without questioning us as my mom, sister also have AIP.  He looked at my test results from Dr. Anderson made contact with him, had me meet the Pharmacist and they ordered me what I needed to be well.  I had a total of 12 different nurses 2 Doctors and 2 Pharmacist’s we all learned together and now we have a system.

When you find that special Dr. don’t ever take them for granted tell them how much you appreciate from the small things to the large items, they will help you for a long time.  That brings me to think of how we have Dr.s today for “Protect the Future Program” may we Thank old and new for all their time experience and investment for Porphyria patients everywhere.

Thank You!  Amy Chapman AIP Patient

Wednesday, June 8, 2011

Honesty is the Best Policy

I was a much more private person before I got sick nearly two years ago. I didn’t feel the need to tell too many people that I could get sick with porphyria-- after all, I assumed I wouldn’t. And for what it’s worth, I’m a very lucky person-- when I got sick, I was able to manage it through a number of activities (which I may detail later), and I got my life and health under control, for now.

But boy, was it ever so hard having to explain to friends, acquaintances, and bosses why I was sick. It was even more difficult because I didn’t look sick. Someone once said to me, “You look fine now.” She was suspicious. I was as bubbly as ever, which was mostly an act-- and not everyone is an expert on health by looking at someone. For a brief second I felt badly that I didn’t “look” sick enough, until I realized whether or not people believed me was irrelevant. The only people who needed to believe me were doctors, and that’s another story, another issue for another day.

Yet I don’t regret a single choice I made to actually talk about my sickness. If I was unloading too much information on one of my bosses, then they could deal with feeling awkward; so long as they understood that I was tired and anxious and sick all the time, then they could help make my life better. If friends didn’t want to talk to a sick girl, then they weren’t friends. If I made acquaintances feel uncomfortable by being sick, then they had to deal with their own fear of mortality, not mine.

So, next time someone asks how you’re doing, and you feel utterly terrible and sick, tell the truth. It’s hard. But in the end it’s best if people know your situation, where you’re coming from, and why you write gripey blog posts about honesty being the best policy!

--Miranda Dennis

Glamorous (Nonalcoholic) Drinking

Sometimes I want to be like Audrey Hepburn in Breakfast at Tiffany’s, decked out in Givenchy, throwing wild parties (and singing “Moon River” on my fire escape) and holding a glass of champagne while trying to chat up someone with a name like Rusty Trawler.



Of course, the reality is that 1.) smoking cigarettes (like in the picture), even on long elegant cigarette holders is terrible for everyone’s health, 2.) wild parties sometimes end with cops being called, as the guests often find out, and 3.) drinking alcohol is certainly a huge no-no for someone who has porphyria.

Still, I like to hold wine glasses.



So, why not be luxe and still invest in those nice glasses, even if you can’t drink the wine? My favorite non-caffeinated drink is usually pure cranberry juice cut with seltzer water. It sparkles brightly, and it leaves me feeling pure and happy. Glamor, like so many things, is a cultural phenomenon constructed from objects and feelings; why not transfer those objects and feelings to a nonalcoholic world where no one ends up dead or in the hospital?

Do yourself a favor: scour Allrecipes.com or FoodNetwork.com or just check Google. Look for nonalcoholic beverages. Or experiment in the kitchen. Invest in sparkling water (or even a machine that carbonates water for you), and live a life that's full of health. And in the credits to the movie adaptation of your life, thank me! I'll be the one in the audience, sipping lemonade from a goblet.

--Miranda Dennis

Om: Yoga For Managing Stress

I was taking a walk in Fort Greene park in Brooklyn when I saw a man doing yoga outside. I stealthily snapped a picture of him, not because it was a bizarre sight (sometimes public yoga can be), but because I thought that was so awesome of him. If one of the best ways to lead is by example, then what an extraordinary leader!



His calm and poise reminded me to go home and unfold my own yoga mat and do stretches:



I'm a very lucky girl. I have gone to Golden Bridge Yoga in Los Angeles and seen Gurmukh herself instruct people on kundalini yoga. When I lived in Amherst, Massachusetts I had an excellent hatha yoga instructor at Yoga Center Amherst. I have great DVDs for my home viewing, and I've a good memory for certain moves and postures. I am able to do my own stretching on a budget! My own meditation on a budget!

The great thing is there's a lot of access to free yoga online (YogaToday.com has a free class each week online). If you have Netflix, request a video! Drop in on a class for a reduced fare. If you learn from books, get a book-- the library will have videos and books. Hurrah! And I really recommend it, especially for people who suffer from any illness where stress is a trigger-- like porphyria.

Whenever I start to feel ill or run-down, I take the time to stretch my body, to meditate, to practice on my breathing, to really try to center myself in my body. A lot of people may scoff and say, "That sounds really New Age and stupid!" But there's a reason people of all faiths, politics, and incomes have started to gravitate towards yoga; it works. The body wants to heal, so let it!

--Miranda Dennis

Sugar My Friend. Sugar My Enemy.

It would be easy-- and probably a lot of fun-- to pretend that having acute intermittent porphyria gives me free reign to eat all the carbs and sugars I want. But interestingly enough, after being officially diagnosed with porphyria, I started to make a lot of lifestyle changes and dropped weight at a slow but steady pace. And I didn't alter my carbohydrate intake a single bit.

I owe much of this to my switch from processed sugars to more natural sweeteners like agave or honey, supplementing my choice to use raw turbinado sugar. Every morning I usually add a spoonful or two of sugar to my French Press coffee pot, to cut the bitter and to leave a light sweet taste on my tongue. The turbinado warms to an almost molasses taste-- you can tell because it's brown, not white, that it'll be closer to caramel than just pure white sugar. Life's good for me. I make tea or bake and use agave nectar, which (according to various sources on the internet, which isn't always right) has one of the lowest glycemic indexes of sweeteners out there.



And now that a heat wave has struck NYC where I live, I have a few ice cream sandwiches tucked away in the freezer. But here's the deal: they're made from soy products and have no cholesterol. They're tiny, too. That's why they're called Cuties:



I'm not saying this as diet advice-- eat a bunch of Cuties soy ice cream sandwiches and put raw sugar in your coffee. I'm saying that I try to focus on various forms of sweeteners, various ways of getting sweets. Not featured, of course, is the best way to get your sugar fix: a banana. Fruits and veggies offer the best sweets available, the kind that does the body good.

Just because we have porphyria doesn't mean we should eat really delicious donuts covered in frosting and delivered by unicorns (okay, every once in a blue moon is okay), but we can have our sweet moments that are good for us without compromising our health. After all, too much unhealthy eating and sugar can lead to type 2 diabetes and obesity, which would further complicate porphyria problems. We must stay vigilant! But sweet!

--Miranda Dennis

Tuesday, June 7, 2011

Facebook!

Recently our Facebook group page got a virus. Please be aware of the malacious viruses that can slow your computers and infect them with Trojans and worms. As a result of the misfortune we have had with FB we have not been able to reach many of our group members. Some were even locked out. These are just a few of the issues we have encountered. Please continue to follow us as we appreciate your support.

Advance Care Planning- Prepare NOW!

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