Monday, October 27, 2014

Meet some of the experts!

I am very excited this year about the Meeting of the Minds taking place at this year's HPS Network Conference. We are expecting 25 doctors and researchers (maybe a few more) to participate. Last year the event was a great success, and this year with more than double the participants, we are hoping that our great minds get together, share notes, knowledge and expertise in the quest for the cure! 

I want to introduce some of our participants, and that will take a while! Grin. 

Meet Dr. Michael Beers! You can learn more about Dr. Beers at: https://www.hpsnetwork.org/en/bios/michael-f-beers-md


Tuesday, October 07, 2014

Hundred People Search

We have added a new member to the patient registry. This now puts us at 88 people to go on this year's Hundred People Search. Yes, that is behind where we usually are, but we didn't have a conference in Puerto Rico this year. We usually pick up a lot of new members there. Keep up the outreach - there are developments coming for which this will be hugely important. 

Wednesday, October 01, 2014

A special kind of bravery

This past Friday the Food and Drug Administration (FDA) hosted a public meeting to discuss the needs of patients with Idiopathic Pulmonary Fibrosis. I have a lot to say about the meeting, but I want to begin by saying that it was one of the toughest medical meetings I’ve ever been a part of. My hat is off to all of those with pulmonary fibrosis, their families and the advocates, who took part on behalf of the thousands more who could not. It was a meeting full of tears and sorrow and frustration. I am told that even one of the doctors from the FDA got teary. While I don’t want to make people cry, hearing that really moved me. It makes you feel like you were heard.

Now, onto the meeting.

The Hermansky-Pudlak Syndrome community has a long way to go in our work with the FDA. The past 22 years (long before I was ever involved) has been a journey of learning new things – medicine, politics, non-profit law etc. Until now, we haven’t had much to do with the FDA because our research hadn’t progressed to the point of having a possible treatment. The NIH has handled the issues involved with the FDA and setting up our drug trials. Now, we are entering a new age, and it is time to tackle understanding the ins and outs of the FDA world.

You’ll notice that the FDA chose to have a public meeting about Idiopathic Pulmonary Fibrosis and not pulmonary fibrosis. I noted that they also had a public meeting about bleeding disorders, and not Hemophilia or Von Wilibrands. Why then is it necessary to only include those with IPF in the pulmonary fibrosis public meeting? By its very name, it excludes people like us. It is the use of an old and increasingly outdated paradigm. As treatments become available there will be some that work better for some patients, and others that work better for other patients.

Take the world of cancer treatments, for example. Years and years ago there were treatments for cancer. Now, they can choose a drug they believe will best work for an individual given the specific type of cancer they have, and even their genetic profile. Someday it could be the same for pulmonary fibrosis. By defining the problem by using the word idiopathic as if the unknown was a disease and not simply a lack of knowledge, the FDA is risking impeding access to life saving treatments to people like us – and someday others. After all, the Sept. 11th responders that have developed pulmonary fibrosis, or the families that know PF runs in their family tree – are they really idiopathic? (Even though most have the IPF diagnosis.)

I think it is a matter of education. The pulmonary fibrosis community in general is only barely understanding and embracing this idea. The FDA, which is a recent player to our world, is really only going off what they’ve been told from the pulmonary fibrosis world. It is a matter of some controversy and debate and will likely require a great deal of advocacy work on our part. That’s okay. Now is the time to make that happen, and this public meeting was the first baby step in what will be an ongoing process for us.

I was so moved by the representatives that spoke on behalf of pulmonary fibrosis patients. I know Donna was very upset that I was listening to this testimony. She was, and rightly so, worried about how upsetting it would be for me given the decline in my health. Family of those who have lost loved ones to pulmonary fibrosis offered extremely graphic descriptions of their relatives’ last days. Others talked about how much pulmonary fibrosis has changed their lives and what a devastating effect it has had, not only on them, but on their entire families. Of course it wasn’t easy to hear. On the other hand, in some ways, it was vindication for some of the things I am starting to notice about my own health.

As I start to notice things that are harder to do now than they used to be, I find I often have a tremendous amount of guilt. I ask myself, is it really my health, or is it in my head? Is there a reason, or am I just feeling lazy? It was affirming to hear others in pretty much the same state of lung disease talk about the same issues and problems.

I want to blog about some of my own answers to the FDA’s questions, but that will have to wait for another time.

The one thought I couldn’t shake through the trauma of the day is this – Imagine having all of these same problems (coupled with legal blindness) and then, when after years and years there is finally a treatment, being left behind?


For now, I just want to thank the FDA for having the public meeting and listening to patients. I want to urge the FDA to make sure that patients with HPS are not left behind suffering and dying when there is a treatment that could help. Lastly, I want to thank Donna and all the other brave advocates who stood up, shared their very souls, and gave us all a voice!

Tuesday, September 16, 2014

Health Update: Nose to the grindstone

I’m a bit late posting a health update, and an update on the transplant journey. I think I completely missed it last week. Please forgive me. 

Last week was a very busy and a very productive week. We submitted a grant to Walmart asking for funds to help with conference. I don’t know if we have a chance, to be honest, but I felt like our grant application was a good one. Then again, I always feel that way – grin! I sometimes wonder with grants from large corporations like this one, if we stand less of a chance because we are not a huge national non-profit with lots of name recognition. We don’t fit the advertising campaign quite as well. But, we’ll see. If we did get this grant, it would be a HUGE boost to the conference!

We also made progress on another grant we’re working on to help fund Meeting of the Minds – the medical meeting for HPS researchers.

Lastly, we submitted our written testimony for the upcoming FDA hearing on pulmonary fibrosis. It was a week with a lot of deadlines, thus I’m so behind on communicating with friends etc.

As for my health, I was starting to get a bit worried over the last two weeks, but I think everything is okay now. For three days I noticed my oxygen needs were up. I know it is all about the long-term trends, and not just a few bad days – yet I can’t help but get anxious. Once my oxygen needs get over six liters in motion, I think being mobile and independent is going to get a lot more challenging. Thus, an increase had me pretty worked up. Thankfully, after three days things went back to status quo. I think it was an asthma flare up that was quickly and easily treated (thank the Lord!) When you’re on the edge, it doesn’t take much to make a big impact.

I had also started coughing again. I am convinced that the severe cough that no one could figure out how to treat for so long is the reason I experienced such advancement in my lung disease so quickly last winter. Which came first, the chicken or the egg? The fibrosis or the cough? I’m not totally sure, but I don’t think the cough helped at all! Thus, when I start coughing again with that kind of cough that makes your chest vibrate, all I can think about is little blood vessels breaking in my lungs and little traumas egging on the advancing pulmonary fibrosis.

The only thing that seems to work for the cough is Codeine. If you take it all the time, you get so you can tolerate it better and it doesn’t make you as sleepy all the time. I hadn’t needed it in months though, so I had stopped taking it. I don’t want to take a narcotic if I don’t need it. So, when I took it last week again, it knocked me on my butt!

Last weekend I didn’t go anywhere. I didn’t do shopping or go to church or even walk Finley until Sunday evening. I just slept. I’d wake up to go to the bathroom, perhaps try to start working on something, and as soon as I would sit in a chair, I’d just fall asleep right there.

The up side of that is I slept soooo good!!!!! Sleeping is a hard thing for me for a variety of reasons and medical issues. It is rare that I get a complete night of sleep. So, two nights in a row was like heaven!

The other good news is I seem to have adapted to the medication change and am not feeling queasy all the time anymore. I'm hoping to get back on track with my diet and exercise program. It was hard to even eat 800 calories a day when you constantly feel as though as you're going to be hung over the toilet at any moment. You'd think that would be good for a diet, but actually, if this goes on for weeks (as it did) your body goes into starvation mode. I also lost some conditioning because I wasn't able to exercise as much. 

This week I'm sort of starting over. Last week I was feeling better, but also sort of felt like my system was readjusting its self. The good news is I didn't gain any additional weight. The bad news is I also didn't lose any weight last month either. I can't even tell you how frustrating that was and is. It wouldn't be such a big thing if I was working on this for appearance or general health, but in my situation, I'm on a deadline here!!! 

Monday, September 15, 2014

Hundred People Search

We have added two new HPSers to the patient registry. This puts us at 89 to go on this year's Hundred People Search - HPS. 

Wednesday, September 03, 2014

The fish and the loaves

A few weeks ago I was talking to a friend of mine. I was expressing my anxiety about all of the things ahead for the HPS Network, all of the doors opening up for us, but the need to have the funds to walk through those doors. While I can work on promoting funding the HPS Network generally, right now raising money for my medical needs is taking priority on a personal level. How do we help the HPS community understand what it takes to make these things happen?

It’s a bigger challenge than you might think. Sometimes there is so much going on that it’s hard to take time to keep everyone in the loop, let alone help them really understand what it took to make things happen. We’d always rather be doing instead of telling everyone what we’ve done, yet if we don’t get better at explaining the process, how can we expect everyone to understand what it takes to keep the journey to the cure moving forward?

Research doesn’t just happen by magic. It takes money to not only fund the actual research, but the infrastructure to make that research possible.

My friend told me about a homily her priest had given that Sunday about the fish and the loaves of bread to feed the thousands.

I think we’re pretty good about making our money stretch and stretch and stretch. We have accomplished a lot with far fewer resources than it should have taken to come this far. It could be scary, or reassuring (depending on your outlook) to know how much of the quest for the cure for HPS happens on acts of faith. We commit on faith that we will be able to rise to the occasion when the time comes. Somehow, it always works out.

As I thought about the fish and the loaves, however, I couldn’t help but think even Jesus had a few fish and loaves to start with to feed the thousands. The more I thought about it, the more it occurred to me that, perhaps with the exception of creating the world, even God needs some buy in to make miracles happen. He needs some loaves and fish to start with, or some water to turn into wine or some faith to raise the dead.

If even God needs that, then how can we ever expect to find a treatment, and one day a cure, for HPS without making an investment in the process? Lord knows the HPS Network doesn’t have the powers of God!

If we, as people touched by HPS, can’t invest in our future, how then can we expect the greater world to just take care of it? It just doesn’t work that way.

For now, I’m trying to have faith that more of us will take on funding the cure however we can. I don’t like praying to God as though he is some sort of wish-granting genie, but I am praying for guidance and wisdom for our community to help us reach the challenges ahead of us.

Tuesday, September 02, 2014

Health update:

Just a quick update! Things are plugging right along here. I’ve had a lot of stomach upset the last two weeks, especially last week. Yesterday and today it was much better, so hopefully we are over that little speed bump. Besides that, there isn’t much to report. Yeah! Yep, that’s my goal – to have the most boring medical life possible!!! Grin!

Thursday, August 21, 2014

Health Update: Plugging along

I haven’t posted an update in a bit. It’s been super busy and honestly, I just have days where I can barely keep my head above water and do what absolutely must get done that day.

All and all I am doing well.

I started a new medication and am hopeful it will help with some of this. It does take a bit for your body to adjust to it though. Some people have no issues, while others get side-effect symptoms. I’ve had some of those issues, but really can’t say for sure if they are related to the medication. For example, I’ve had stomach upset and some nausea, but then again with my gastrointestinal issues, I go through times when I have that already. It’s hard to say if it is related or just bad timing. Either way, it doesn’t matter. It isn’t so bad that I can’t cope with it. The biggest issue isn’t even so much the stomach upset as it is the getting up every 40 minutes or so all night to go to the bathroom. A few nights of that really wears a person out! Grin! Today I’m having some mild tummy issues, but the bigger problem is I’m just tired. It makes it hard to focus and concentrate well. I find the fatigue also makes me forgetful.

Thankfully, I go through a few days of this with each increase in the medication, and then things seem to level out. That makes me think it might be related to the medication. If so, I just have one more increase to go and then hopefully, we’ll be over this little speed bump and on to the next one.

Thanks again to all of you who are offering support and who have helped with my fundraising for the move and the transplant. I started holding off on some of my thank you notes because I wanted to be able to give an update about the plan when I was thanking people. Now, I can get back to that. Grin!

Saturday, August 09, 2014

Health Update: The news is in – a plan is forming

I heard the news I was waiting for and now I can begin to target a move schedule! Yippee!!! I still have to sit down with my team and look at the calendar and make sure we are all on the same page, but I think it is looking like I’ll be free to leave the Kansas City area in February. The catch is this is a horrible time of year to try to find a low-rent, ground floor or elevator, and hopefully dog friendly apartment. That’s a really tall order even during the best time of the year for the apartment market.

So, we will see where we are at in February. If things are unchanged, then we don’t have to rush. If things start to change quickly again, then the plan is scrapped and we do whatever we have to do. If things seem to continue to change, but at a slow rate, then perhaps some of the wish list for the apartment will have to go to the wayside just to get myself there.

I am a planner. I don’t like having things up in the air. These last few months have been so, so, so stressful!!! Not just for me, but also for my family!

Last night my body literally ached – I think just from finally relaxing a bit and letting some of the tension go. I have a zillion things I need to do, but Finley and I had a little celebration. I got a people treat (been on a diet to lose weight for the lung transplant) and Finley got a special dog treat. We watched movies together until time for bed. It was so nice!

This morning we slept in, and then went for a four-mile walk. I need to clean house and I have a ton of network stuff to do, but it feels so good not to have this panic just under the surface that at any moment I’m going to have to pick up in a rush and move.

I’ve done a lot of packing already and am sort of at the point where I couldn’t pack much more without knowing when the move was going to happen. After all, if I keep working here for several more months, then packing up my office isn’t really helpful just now.

I’m going to miss having a dedicated office. There is no way I can afford a two-bedroom apartment in Arlington. I’ll be lucky if I can find a one-bedroom I can afford with housing help. I’ve loved having a room with a door I could close to separate my work life from my personal life. I’ve loved having a place where I could leave my painting materials etc. set up all the time so I could paint or work on crafts whenever l liked. Work and crafts can look so messy that having them all in one space with a door you can close has been a welcome luxury. It’s a luxury that’s days are numbered! Grin!

Still, I’m feeling so grateful right now that it seems like a small thing. I’m so grateful for recent medical reports that are not worse than those in May. I’m grateful for all the family and friends who have done so much to help me do what I need to do to make this all happen.

So, for now, I’m still in the Kansas City area for a few more months.

Thursday, August 07, 2014

Health Update: What is the plan?

This is just a short update. I know that everyone in my life, and all of the generous people who have helped to raise money for my lung transplant, are wondering – what is the plan? So, the update is there is no new news yet. I am waiting on some answers that could greatly impact the move schedule. I thought we would have heard back by now, but it is taking longer than expected.

This journey is such a roller coaster. I get anxious about all that is on my mind and not being able to answer everyone’s questions about when I will move. You’d think after years of living with HPS and waiting on test results that could dramatically alter your life over and over again, I’d be good at this waiting thing. I’m not so good. I think I’m doing a bit better, but it’s more exhaustion over being anxious than true master coping skills – grin!!!!

So, for now, we just wait.