Tuesday, July 29, 2014

Happy news in HPSland

Today was a great day in HPSland. One of our members had her first baby, a little boy! Another member who has been on the lung transplant list a long time got his new lungs last night! I’ve been on cloud nine all day!

I had intended to work on some HPS writing that needs to get done, but spent most of the afternoon on the phone with members. That is important work to get done as well, but it doesn’t feel like work because I enjoy it so much. They aren't just members - they are all personal friends I treasure!

I don’t know if the people having these great events in their lives truly appreciate how deeply and how much they mean to many of us.

One of the first questions I always get asked by a woman recently diagnosed with HPS is: Will I be able to have children? There are a lot of things women with HPS, as women with any myriad of medical conditions, might want to consider before deciding whether to have children. The concern about whether it is physically possible and safe, however, can easily be taken off the table. 

Knowing you have HPS makes it possible to manage the potential bleeding risks associated with child birth. It is not knowing that can result is scary and life threatening situations. Celebrating the birth of a child in our community is an affirmation of life. It’s a passage of normalicy that we all treasure, besides just treasuring a sweet little new life in the world.

Especially poignant to me right now, however, was Julio getting his lungs. Julio’s older sister died from HPS. His mother and other siblings cared for her in her last years and his family have been through this process now several times. Julio was getting very sick and we were all very worried for him. These lungs came just in time! 

It is our 14th known HPS lung transplant. Every transplant that happens helps to pave the road for all of the rest of us who will need them in the future. The more of them we have, the less powerful the argument that HPS poses too high a bleeding risk for lung transplantation. We’ve now had 14 transplants in three different countries. It is a lot to celebrate!

Still, lung transplants are not a cure. It is more like trading one lung disease for another one. Not everyone can be a lung transplant candidate. We are all still dreaming of the day when we find a treatment that makes lung transplantation unnecessary. Now, that will be one heck of a party!!!!! When that day comes, I’d be happy to do the “I’m too Sexy for my Lungs” dance!!!!

Health Update: Pulmonary Function Tests

Last Friday I had my first repeat of Pulmonary Function Tests (PFTs) since being told I need a lung transplant. You can imagine, I was quite nervous about them. Along the way I am making all kinds of decisions. So many of them seem like gambles, yet they are literally life and death. Luckily my PFTs were a confirmation that, for now, I am making the right decisions. The plan can still change at any time, but for right now, we are just pressing ahead with handling some more immediate issues I have to get under control before I see the transplant center.

As for the results, I must confess, given how much better I’ve been feeling, I was secretly hoping for a miraculous improvement that would wow everyone. What I got was a mixed bag, but the important part is that, for now, I have stabilized.

My forced vital capacity (FVC) fell three points, but my DLCO (the bigger problem right now) went up three points. Machines can vary from hospital to hospital. For some reason, my numbers are always lower at KUMed than at NIH, so the fact that they were about the same was happy news.

Monday, July 28, 2014

Another new HPSer Hundred People Search - HPS

This morning we added a new HPSer to the patient registry. This puts us at 92 to go on this year's Hundred People Search - H.P.S. Remember, the most dangerous thing about having HPS is not knowing it. 

Monday, July 21, 2014

Hundred People Search

I am behind on my e-mails and just found a Hundred People Search new person I had not counted yet. So, this puts us at 93 now. Keep up the great outreach! 

Wednesday, July 16, 2014

If you need a lung transplant, why are you walking so much???

This is a question no one has actually asked me directly, but given a few comments, I think some of you are wondering.

I’ve been working really hard lately at walking as much as I possibly can. I’ve been posting the walks to my facebook page. The encouragement I have gotten from friends and family actually is pretty motivating.

I think some, however, wonder how it is I can walk three or more miles and need to move to get a lung transplant.

Let me clear that up for you! Grin!

For starters, I don’t need the lung transplant tomorrow. The issue is that my lung function has fallen enough that there isn’t as much “wiggle room” before advancement in my disease will cause me to need a lung transplant. As I’ve learned lately, moving is NOT EASY – especially when you haven’t done it in 12 years. Getting listed as a person with Hermansky-Pudlak Syndrome involves not just the many medical issues, but also lots of social support issues and services. If I were sicker, it would be harder to have the energy to move and to get established in my new location. Setting up all of those services etc. just takes time.

The other issue boils down to use it or lose it. I am really hoping that my disease stabilizes again for a few years, as it has done before. I can’t bet on it though. My doctors have told me the best thing I can do is exercise as much as I can so that if my lung disease progresses, I can do more with less, sort of speak.

Lastly, I have to lose weight before I can be listed, even if I needed the transplant tomorrow. I am working on that, but if it were easy I would have done it years ago.

It cracks me up that docs said to me, “You need to start trying to lose weight.” Really? That thought, as a woman in this culture, never occurred to me. I’ve never tried that. Great idea! Geesh.

The good news is my thyroid issues are good right now. My energy levels are better than they’ve been in a while (I’m thinking the change in medications as well as the oxygen.)

For quite a while I’ve been worried and puzzled by the fact that when I exercised, I never built up a tolerance for it. It never got easier as it is supposed to do. Now, I am seeing that I am building up some exercise tolerance. A walk that used to make me quite tired is now easy.

I am also tracking my calorie intake and burning quite carefully. I’m a big fan of the apps. MyFitnessPal and MapMyWalk. I think they should make me an advertisement! Grin!!! Well, I guess since I post it to facebook, I already am advertising for them. Grin!

Tuesday, July 15, 2014

Reader comments

Wow, I owe some of you an apology. For the longest time the only comments the blog had been getting were utter spam. Thus, I wasn’t really checking the comments. To my great surprise today when I went in to clean out the spam, I found some lovely comments from many of you.. Thanks so much for all the well wishes! I set the comments free and I’ll start looking more carefully.

Friday, July 11, 2014

Move prep


Third load of stuff given to charity as I work on my move to Virginia. 


Stuff I sent with mom to store. 



Even though my move to Virginia had to be moved back, I’m still very actively working on getting ready to move. Part of me is a little glad for the delay as it gives me time I really need to get ready. When I thought I was moving in July, the anxiety about how to get organized in time was getting a bit much!!!

This past week my mom has been visiting me from Colorado. While I have given away three loads of things to various charities, I do have some things that are sentimental enough to me I hate to get rid of them, even though they are not exactly practical for living in a smaller place in Virginia.

I’ve spent the past few weeks packing things like the nested tea pot Peter gave me when I visited Hong Kong years ago. It is one of those items that just sits on a shelf and looks pretty. Since I won’t have an office when I move, I need the shelf space for more practical things. Still, to get rid of it would have broken my heart. I treasure it. Or, there’s the East German border guard uniform I bought off an East German solder when the Berlin Wall fell…or my yearbooks, journals, photo albums etc.

Mom is taking those sorts of things and will store them for me. I doubt that I will ever see them again. I have no idea how she would ever get them back to me. Yes, I know U-Haul etc. but what people don’t appreciate is that it just isn’t that easy. Colorado is a very long drive from Virginia. Moving these items would be very expensive and I doubt with all my medical bills I will ever be able to afford it. Even if I could, I will never be able to afford a place big enough for all of this stuff in Virginia. It isn’t like I am a college student moving there to start a career. It isn’t like suddenly one day I’m going to make more money and be able to afford a better place etc. Okay, maybe not impossible, but realistically highly unlikely.

Still, it makes me feel a little better to know the items aren’t lost, and that if something happens to me, someone who cares about me has them.

I didn’t cry all week until the morning my mom left. Even then, it wasn’t so much about the stuff as it was about the situation. There have just been so many losses over the years. I have always wanted to live in the DC area, but was never able to get a job there.

If you don’t have health issues you can do things as a young person to take a chance and make things happen. You can live in a little room and eat ramen noodles until you land something. But, when you are a person like me, someone who has had hundreds of dollars of out of pocket medical bills your entire adult life, you just never had that ability to go out on a limb.

I was always terrified of losing my medical insurance. Now, with the Affordable Care Act, insurance cannot hold pre-existing conditions against you. Before, however, if you ever had a gap in coverage, suddenly everything became a pre-existing condition. You either couldn’t get coverage, or if you could, it was VERY, VERY expensive. I could barely afford my medications and medical supplies with insurance. What would I do without it?

The items I needed were not things I could just do without for a while. They were 100 percent necessary to maintain life. It was so scary to live that way.

At any rate, now I am moving to the place I always wanted to live, but it is such different circumstances. It won’t be like living there as a healthy person.

My mom mentioned that taking Finley might mean that I can’t do a lot of things I would like to do when I live there. This is partly true, however, now that I am on oxygen, things are very different. I can’t just get on the metro and be gone for hours. Everything has to be planned. I can’t just go spend the night with friends nearby. I need my “mother ship” oxygen machine for night time. To be able to get a “portable” machine for night means three weeks of advanced planning to get the equipment. It just isn’t the same.

So, my tears were not so much for losing things as they were about just feeling frustrated with the whole circumstances of the situation.

Wednesday, July 02, 2014

How can I pray?

One of the sweetest questions I get asked these days is, “How can I pray for you?” Thank you so much for all of the many, many prayers. Please keep them coming. This journey will likely be years, not months. It’s more of a marathon than a sprint. It will have lots of ups and downs along the way, and I’ll need all the support I can get.

I am surprised by how much things like little comments on facebook mean when you’re really in a dark place. On the whole, I think I’m doing a good job of keeping my spirits up, but that isn’t to say that there aren’t days that are just, well, crappy. On those days it’s the support of all of you that help to keep me focused on moving forward and trying not to dwell on the losses.

There is a lot going on that could use prayer right now.

Please pray for my family. This is a stressful time for them as well as for me. Not knowing exactly when I’ll be moving and when our plans need to be put into action turns my stomach. I know it isn’t easy on Ryan and Sara and my parents either. We all want a plan. We want to know exactly what has to be done, and by when, but we are learning this is a process that runs on its own schedule.

Please pray for me, that my spirits keep up and I keep motivated and looking forward.

Please pray for Finley and me. I still haven’t blogged about Finley because I get so emotional about him. I need to pick a time to blog about him when I’ve got time to have a good cry.

I want Finley to move with me to Virginia. I feel better about that now than I did a few weeks ago, but it still is another one of those little miracles that has to happen along the way.

Finley is in many ways like my baby. At the same time, he is not a child. I talk to him about things I don’t share with anyone else. I would never do that with a child. 

There are a lot of things I don’t talk to many, if anyone about. I worry not only about my health and the lung transplant process, but I worry about how this is all impacting my family and friends. I worry a lot about my brother’s family and how having someone as ill as I may become around the kids will affect them. I worry about the additional stress it will place on their family. I worry about my parents. I’m also very actively working on the process to try to gain access to a treatment for all HPSers. That alone is a major emotional roller coaster. I worry about all of my other HPS friends who are walking this same path with me. I have lost, at this point, dozens of personal friends to HPS. I know I will lose more. If I talked about these things all the time, even with those closest to me, no one would be able to stand to be around me. Finley, however, just looks at me, tilts his head, jumps in my lap and licks my ears. Sometimes when I’m sad he looks at me and runs to get one of his toys that he promptly drops on my foot. My mom gave me Finley because she wanted me to have something to focus on besides HPS all the time (a challenge when you have HPS, but also work for the HPS Network). Now, when every time I breathe I am thinking about my HPS, it seems like the worst possible time to lose Finley.

Still, for Finley to come with me, I need a support system for him as well. The good news when all this started, Finley was unable to stay by himself without barking. Now, he’s getting much better. I haven’t left him all day yet, but I have left him for up to four hours and he’s done fine. (I check up on him by recording him and checking with the neighbors.) Thus, I think he will be okay for frequent doctor appointments. The tricky part will be when I need to be in the hospital. This will happen from time to time. Sometimes it will be planned, but sometimes I will need to just have someone I can call to go get Finley from my apartment.

I have three families that will take Finley for even a couple of months if necessary, and see to it that he gets sent to my mom if I can’t take him back. This might happen when I actually get my transplant, or get very ill at the end of my life and have to be hospitalized. If I get new lungs, it might be several months before I’m healthy enough and allowed to be around animals. I am very confident that any one of these three families would come through. They are all people I know very well, and have known for years. The catch is none of them live close. They are all at least a few hours drive from Arlington. Thus, I would need a temporary foster parent to get Finley if I get the call and keep him until one of these families can drive down and pick him up. I don’t yet know how all this will come together, but I remain hopeful. I have had some good ideas from a number of people, some leads to follow up on etc. Even my vet here has been on the phone to vets in Arlington trying to get help for “team Finley.”

Please also pray for the entire HPS community. We are on the cusp of having a treatment (not a cure) for the lung disease of HPS. There are, of course, some obstacles that have to be overcome to get access to this treatment. Things are just more complicated and work differently when you are a very, very rare disease. Please pray that things go smoothly in this process so that those who desperately need access to this treatment can get it.

Finally, please pray for my future lung donor and their family. To get new lungs, someone has to pass away. For the lungs to be in good enough shape to transplant, the donor usually dies in some kind of sudden situation. It is usually a traffic accident. They are walking around right now with my future lungs (if I get lungs) and they have no idea that in a few years something terrible is going to happen to them. I know what is coming. As much as it is possible, I can plan. I can say goodbye to people. My family has had time to prepare. You can argue about which scenario is harder. To me, there is nothing productive in comparing pain.

All I know is that the owner of my future lungs has no idea what fate has in store, and they and their family are going to be the ones to make a heroic decision to let me have their lungs in the midst of a sudden and terrible loss. They need our prayers as much as I do.

Tuesday, July 01, 2014

It takes a village






Some of the crowd at the fundraiser Homers Coffeehouse held for me.

I want to once again thank all of the people who have pitched in to help make my upcoming move to Virginia possible so that I might try for a lung transplant. I might not be a child, but it truly takes a village to make lung transplants happen. That’s why transplant centers evaluate your support system as part of the decision to list you for a lung. They want to know if you will have the physical, financial and emotional support to help deal with the roller coaster process of getting, and then maintaining, transplanted lungs. Not only have the fundraiser and the donations helped financially, but they will help demonstrate to the transplant team that I do have a LOT of people out there rooting for me. So, here’s a big THANK YOU again!

Wednesday, June 25, 2014

Living Your Best Life with Lung Disease

This year our HPS team at the American Thoracic Society attended an awesome patient program about the common issues faced by all patients with lung disease, no matter what it is. I loved the approach and found several of the presentations really great. It also gave us some ideas that we hope to bring to the HPS Conference session line up, provided we can find a speaker to deal with them. Rather than explain the sessions, I"ll just send you to the story I wrote for the HPS Network website: - https://www.hpsnetwork.org/en/news/2014-06-25/hpsers-attend-living-your-best-life-with-lung-disease-at-the-american-thoracic