Tuesday, April 26, 2016

Hundred People Search Update

Today we added a new HPSer to the patient registry. This puts us at 90 to go on this year's Hundred People Search (H.P.S.). 

Wednesday, April 20, 2016

Hundred People Search

We have added a new HPSer to the patient registry. This puts us at 91 to go on the Hundred People Search (H.P.S.). 

Thursday, April 14, 2016

Hundred People Search Update

We have added a new HPSer to the registry. This puts us at 92 to go on the Hundred People Search (HPS). 

Wednesday, April 13, 2016

Hundred People Search

We have added a new HPSer to the patient registry. This puts us at 93 more to go by next conference for the Hundred People Search. 

Tuesday, April 05, 2016

Hundred People Search off and running

Today we added four new HPSes to the patient registry! This puts us at 94 to go by next conference! 

Monday, April 04, 2016

New Hundred People Search

We are starting a new year in the HPS calendar!!!! So, what is the Hundred People Search? It's an HPS Network challenge to find everyone possible with HPS and help to make sure they have an accurate diagnosis if they need it. H.P.S. - Hundred People Search. 

Last year the Network changed its focus for the year to concentrate on building a better infrastructure for research. We have been working on opening the Young Lung Centers in hopes that someday, should another drug or treatment trial become available, we can fill it faster and thus get treatments to people with HPS faster. 

This year, we are turning our focus back to finding people with HPS. We do not, however, have much budget for this effort. The centers cost money. We are going to need the help of everyone to do outreach and to find others who may need a diagnosis, or who have one, but might not be aware of everything happening in HPS research etc. 

So, we are kicking off the year with two new HPSers added to the patient registry. This puts us at 98 to go on this year's Hundred People Search. 

Wednesday, March 30, 2016

Health Update: A big bump in the road

The last few months have been hard ones for sure! I was going to post a health update about the first six weeks of the year. It was one health issue after another. Now all that history seems minor and hardly worth a mention in comparison to what happened next. Feb. 15th I had a major bump along the road to transplant.



As part of my transplant workup, I had an endoscopy. Because of my GI history, I do not have a colon for a colonoscopy, so this was the way they could check for any active GI disease. I also have a history of reflux, so they wanted to be sure my reflux was not so severe that it would damage the new lungs.



The good news is that the endoscopy was clear. No GI disease. No reflux issues that will get in the way of transplant. And, my thrush had cleared up (one of the problems earlier in the month.) The bad news was the endoscopy landed me in the hospital for five days. Now, I’m still recovering.



I should have more strongly insisted on having this procedure at the hospital. In Kansas I had such a long and wonderful relationship with my doctors that they wouldn’t have batted an eye at doing such a simple and routine procedure at the hospital. I just had to tell them what I wanted, and they made it happen – no questions. They knew I knew a lot about HPS and trusted my judgement. Here, however, my doctors are all new to me. I’m carefully cultivating those relationships and am always mindful of how everything I do could reflect upon me as a potential transplant candidate.



I know that the bleeding of HPS is not an obstacle to a successful transplant. We’ve had 17 transplants so far worldwide. But, there is always a worry about how this issue will be seen by docs not familiar with HPS.



I strongly insisted on precautions for my heart cath. Even though the docs seemed confident there would be no issue, I was not convinced. I insisted on standby platelets and got IV DDAVP. I went to the hospital that day fully prepared to be admitted overnight. I’d made arrangements for Finley, packed my tooth brush and phone charger etc. The endoscopy, however, just didn’t seem like as big a deal. The GI doc was pretty strongly of the opinion it was okay to do at his location. He wasn’t doing biopsies. It was just a quick look around. He had an anesthesiologist on staff and a full suite for that sort of thing. He is the GI doc for lung transplants so they were both quite experienced doing this procedure on people with compromised lungs. In the back of my mind, I was a little worried about making a big deal about the bleeding when I felt the risk was low, and all the other docs I checked with seemed equally confident it would be okay. I don’t want them to be afraid to take my lungs out after all!



Big mistake!



The actual procedure went very smoothly. When they were removing the scope, however, I coughed very hard. My nose was very dry from the cold I had in January, the meds I took for it, and the oxygen always in my nose. When I coughed, I broke some blood vessels in my nose and had a nose bleed. What happened next I’m not sure if I will ever really know. They were waking me up when this happened, but I think they had the bleeding stopped before I was fully awake. They put in a nasal trumpet to help stop the bleeding. I don’t remember anyone taking it out, so this must have happened when I was still under. The first thing I remember was feeling annoyed that someone was trying to wake me up. Those drugs were great! I was having quite a lovely nap! Then, as I came out of it, I could hear alarms going off and a lot of activity around me. Suddenly I was aware that I couldn’t breathe.



I have sleep apnea. If I fall asleep sometimes without my CPAP on (like during the day, or on an airplane etc.) I have to sit up to kick start my breathing again. I’m not sure if this was in my foggy druggy mind or if it is what really happened, but I felt like I was fighting to sit up.



There really wasn’t a lot of blood on me when I sat up. I was way more concerned that I couldn’t breathe. They had oxygen pumped up high and I heard someone say, “Get the paramedics here.”



For a bit it seemed like I was improving. I was more with it and they were able to lower the oxygen. I heard them cancel the ambulance order. This didn’t last, however. The alarms started going off again and I felt like I couldn’t take a breath. I felt like I was drowning, but without water.



The paramedics were called again. This was a total God thing I am sure. It just so happened that the paramedics were eating lunch at the McDonalds next door. Their rig was already in the parking lot!



They brought in the stretcher and Ryan came with us in the ambulance. He had to sit in the front though. I was in the back of the ambulance concentrating very hard on breathing and trying to use some of the techniques I learned in my pulmonary rehab. class. I started to get better. I thought, oh dear, by the time we get to the hospital I’m going to be fine and this is all so dramatic!



Once again the improvement didn’t last. Suddenly I couldn’t catch a breath again. I felt weird. I heard the paramedic talking to the hospital on the radio. For some reason, I felt I really had to listen to what they were saying. I was thinking, I can’t pass out. I have to be able to talk. I need to know what is going on! I heard them say my blood pressure was 60/40 and my 02 was in the 60s.



We arrived at the hospital very quickly! It was another gift from God. I wonder if this had happened in the hospital, how long it would have taken them to get me to somewhere to handle it anyway. The hospital is very large. When they admitted me the trip up to my room was about 15 minutes. I think we were at the ER in about six minutes (from what I think I remember the paramedics saying about their ETA.)



They unloaded me from the ambulance and took me directly to a treatment room with about six people waiting on us to arrive. A lot started happening quickly and I can’t remember all of it. There was just a lot of activity. They put me on a non-rebreather mask with about 20 liters of oxygen (more than they could give me in the ambulance). I started to get better. They lowered the oxygen to 17 liters, then 15 liters….then they took away the non-rebreather mask and gave me a regular mask. I seemed to be improving. I actually thought if they kept me, it would just be for a night because I was improving.



The improvements didn’t last. They had to put the oxygen back up and put the non-rebreather mask back on. We tried this dance several times, but over the course of I think four hours or so, I just couldn’t maintain improvement.



The really frustrating thing was struggling so hard to be able to say even a word or two. Ryan was on the phone with Carmen and Donna (I think) and I knew he wasn’t giving them the information they needed. But, as I waved my hands, I couldn’t get words out to tell him what to say. Thankfully one of the ER docs came in and I had her talk to Donna on Ryan’s phone. That was a lessons learned moment too. We’ve got to work on reporting skills. People can’t help you ask the right questions if you aren’t accurately feeding them the right information.



I spent five days in the hospital. The first two they wouldn’t let me out of bed, even to use a bedside potty. They had alarms on the bed so that even if I moved too much it would signal I was trying to escape and the alarms would start going off.



Everyone tried to assure me that what had happened was an injury, not an illness. It was like breaking your leg – it would heal and I’d be myself (such as it is on oxygen) again. This was true, but I couldn’t help but think of all the friends I’ve had over the years in the hospital on huge amounts of oxygen. Even though it is probably very different to have it happen so suddenly, not being able to breathe is not being able to breathe. I feel like I got a glimpse of what is to come, and that has been almost harder to cope with than the actual event its self.



What they think happened seemed to change over several days and depended on which doctor I was speaking with. But, what I’m pretty sure happened is that I aspirated blood from the nose bleed into my lungs. When I arrived at the ER, they cut off the hospital gown I had on from the doctor’s office, so only the ER docs saw it. I saw it, and it really didn’t have a lot of blood on it. Everyone kept asking me how bloody it was because they were trying to figure out how bad the nose bleed was – but I think it wasn’t bloody because most of the nose bleed blood went down my airway and not out onto my clothes.



Finally I got to go home. I was on 15 liters of oxygen to walk, and on 10 liters at rest. Walking to my bathroom was a project. The day before I’d walked Finley two miles. I was on nothing at rest, and three to four liters walking on level ground.



My dad and stepmom came and stayed for a few days. That was a huge help. I thought when I left that I might be able to manage on my own. But, when the docs heard family could come, they were pushing that idea. Thank God! I tend to sometimes think I can do things I can’t really do.



Since then I have improved, although I had a week of a sinus infection. My temp went up to 103 and my oxygen needs went up by about three liters.



Now, I am finally getting back to where I was. My stamina isn’t the same, but it is improving. I’m on no oxygen at rest. I’m on four liters to walk on level ground (a bit more than before). I either have to use more oxygen, or walk super slow with lots of stops to get up hills.



I am back at pulmonary rehabilitation and although not back to what I could do before – I am doing better every time. I actually didn’t lose any ground on the weights I could do. Where I really lost ground was the aerobic exercise. Two weeks back, however, and I’ve doubled what I can do on the treadmill and I’m back to the same amount of oxygen as before. I still have a ways to go in terms of how long I can be on the treadmill.



I have another transplant appointment coming up next week, so we will see where we are I guess.




Thursday, March 17, 2016

New HPS gene discovered

A new gene type of HPS was reported in the January 7th edition of the journal Blood, a journal of the American Society of Hematology. HPS type 10 involves albinism, early onset seizures, neurodevelopmental delay, infection susceptibility and neutropenia. Bleeding was not studied in the patient; however, this new HPS gene does match a mouse model gene with the same platelet issues as all patients with HPS. Previously, there was not a human match for this mouse model. This type of HPS also involves the same protein complex involved with HPS type 2. HPS type 2, however, is not known to cause neurological complications.



Sunday, February 14, 2016

What to say, and when to say it



There is something I turn over and over in my mind. It holds me back from blogging sometimes because I never reach a clear guideline about it. How much do we say about living with HPS, and when do we say it?

My background is as a journalist. Telling the story is just in my DNA (sort of like HPS – grin). Is there ever a down side to telling the story? Most of the time I don’t think so, but there are times I question myself.

What motivation does anyone have to find a cure if they do not truly understand the consequences of not finding a cure? Unfortunately, by the time we HPSers start to more fully understand the consequences of not having a cure, we are often too ill to do much about it. We need those who are not struggling with lung disease, bowel disease, or bad bleeds, to be our alleys and help us do the work it takes to find that cure.

It takes a lot of work. Few people, even people very involved in the HPS community, truly appreciate how much work it takes.

The world is full of causes, however, that take a lot of work. So, how do we compete for the hearts and minds of supporters both from within, and from outside of, the HPS family?

Other health-related charities do it with stories. It isn’t exactly a new concept. Think about the commercials we see on TV for children’s hospitals or for other more common disorders. The commercials are not full of people who are doing just fine and appear to need nothing. They are full of images that pull heart strings, and thus, pocket books, wide open. They are personal stories.

It is no accident that our members’ transplant funds are far more successful fundraisers than the fundraisers of average members not in a crisis. We are glad of that. I’m glad of that as I am one of those needing the help of a lung transplant fundraising effort. But, I can’t help but think it is a shame that we as individuals, and our surrounding circles, don’t feel a sense of urgency about HPS until it has come to a life and death crisis. Wouldn’t it be better to never reach that point? Wouldn’t a treatment, and someday, a cure, be so much better than trying to figure out how to fund a lung transplant? That isn’t possible, however, without involvement before we are so ill.

There is a flip side. When I’m deciding what to share here with the entire world, I never know who is going to read it. Sometimes it is someone newly diagnosed and frightened. Will they read something here that pushes them to avoid the HPS community instead of embrace it? Would they fear the HPS community is just too depressing to be a part of, and thus never get to know us? Sometimes it might be a young person with HPS. Parents like to think they can control how much their youth affected by HPS know about the disease, and when they know it. The reality is our teens with HPS are not stupid. They, like millions of other teens around the world, are very adept at using the internet to get whatever information they wish. If they want to know, and they don’t feel they are getting questions answered, they go searching for those answers. What if they happen upon my page when I’m having a bad day? What if they gain such a sense of dread or certainty about life expectancy or likelihood of illness that it affects important decisions in their early lives? Would they truly appreciate that their futures can look very different than my present – especially if they get involved and help make that new and better future?

I can’t tell you how many hours I’ve spent thinking about these things, and talking about them with a few of my HPS friends.

In the end I think we have to tell the story – all of the story. The good. The bad. The ugly. And, even, the gory and horrible. It must, however, be done with a respectful understanding of the above mentioned concerns.



We have to tell the HPS story mindfully. As any writer will tell you, it isn’t just the story; it is the way it is told. I hope that I can temper some of the scary aspects of HPS with all of the hope that is out there for the future. I hope I can temper sadness with humor. Most importantly, I hope that I can loosen up enough to start telling this story again.

P.S. I honestly would appreciate feedback on this balancing act. Any thoughts? Please share. 

Saturday, February 13, 2016

New Year Resolutions in February

Evidently timeliness is not one of my New Year’s resolutions since it is nearly half way through February and I’m just now posting this blog entry! Perhaps a better resolution for me is to not beat myself up so much about all of the things I can’t do.

That’s getting to be a big one!

Honestly, 2016 didn’t come in with a bang. Rather it came in with a sniffle, cough, headache etc. I’ll blog more about that when I do a health update.

I didn’t do so great with my 2015 resolutions. My goal was to paint 12 paintings. Well, I did three.

Part of the problem is that my stamina just isn’t what it once was. I feel okay, but the number of productive hours in the average day is shrinking. I do the most urgent things first, and there isn’t always anything left for the things I enjoy or the things that just make me happy.

That is not a complaint, however. We have to make choices about how to spend our time, and while I’d love to do the things I just purely enjoy, I do feel like the work I am doing instead is important. It gives me meaning, not just pleasure. So, I’m content with the choice.

That being said, this year’s resolution is to blog more often. I’d like to blog at least once a week. I’d really like to blog every day, but that’s probably a bit too ambitious. Unlike painting, it doesn’t take a lot of set up to blog. If I just make myself do it, then it would only take maybe 10 minutes a day.

When I was still working, I’d sometimes blog while eating my lunch. It was just a few minutes, but it made my work day so much more productive. It was like a lunch time brain dump of all the stuff rattling around in my noggin. Once the decks were cleared, I concentrated so much better through the afternoon.



Right now in HPSland there is so much going on that there is just a lot of stuff floating around in the old noggin. Perhaps cleaning house up there a little more often wouldn’t just be enjoyable, but practical too.