Friday, December 12, 2014

The job of chronic illness

I mentioned a few days ago that when I had a few moments, I would blog about my life on hold. People who have never encountered long-term health issues have no understanding or appreciation of just how much work it takes to deal with “medical stuff.” I estimate this week alone I’ve spent seven hours on hold with medical supply companies, insurance, doctors’ offices and pharmacies. This is not unusual. In fact, it is a pretty average week. Some weeks it takes more time than this. It also does not include the time I was at a medical appointment on Tuesday. This is just time on hold on the phone!

If what you need can’t be bought at CVS or Walgreens, it can get complicated. Currently I have three pharmacies and two medical supply companies in my world. It often feels as though none of them have ever heard the term, customer service.

My oxygen supply company is the worst in this regard. If they weren’t bad enough, they don’t have hold music for that 40 plus minutes or more you’re on hold waiting for a human. Instead, they play a recording thanking me for calling and telling me how much they value my time! It just makes me increasingly angrier as I listen to it because clearly they do not value my time. It’s bad enough I’m on hold that long. Don’t rub my nose in it!

When you vent about this, often you get a lot of well-meaning comments suggesting you go to the media or lodge a complaint, but the trouble is the comments often come from people who do not understand how these services work.

Typically, (especially with medical equipment and supplies) you don’t get to choose the company, nor are you really the company’s customer (even if you are.) They get contracts from insurance companies or Medicare to be the provider for your area. Your personal opinion, choice or satisfaction is of little concern.

If a company doesn’t keep enough technicians on hand to reply to problems in a timely way, doesn’t staff customer service phones, and doesn’t stock the most current choices in care, it is pretty easy to have the lowest bid.

I am the real problem. To fight this system is a lot more involved than a well-placed call to someone in the media. It is a systemic problem and needs a systemic fix. (And by this, I’m talking about something way beyond the typical health care reform debate.) But, if you’re the one sick enough to be in need of these services, you often really don’t have the energy or time to take on that kind of fight.

I have so many battles on my plate, it is hard to fight them all.

So, this little blog post is really more of a vent and a way to voice the kinds of day-to-day issues people like me deal with just to have the medicine, equipment and care, we need to seem as normal as possible.

Wednesday, December 10, 2014

H.P.S. Hundred People Search ditto

Murphy's law - just when I post the update about the Hundred People Search - H.P.S. - I get another e-mail that we just added another person today, so that puts us at 85 now! Okay, returning to your regularly scheduled blog....grin! 

Hundred People Search Update H.P.S.

Today we registered a new person to the HPS registry - this puts us at 86 to go on this year's Hundred People Search H.P.S. This is very much behind where we usually are, but we didn't do a trip to Puerto Rico this year either. We usually get around 40 on that trip. So, I'm not too worried about it. Our attention has very much been focused on some of the exciting things happening in research etc. 

Life on hold

 Finley at 11 months old
 Finley at 13 months
Finley on his one-year birthday

It feels like one of the main things I do these days is sit on the telephone on hold! Groan - I'll be blogging about that when I can, but as I sit here listening to bad hold music, I thought I'd post a few pics of Fin (Since I haven't posted many to the blog yet. If you're on facebook, you've seen them! Grin). 

Monday, December 01, 2014

Finley the helper dog

Yesterday I posted some short video clips on facebook of Finley helping me find the telephone. I spend more energy hunting for my phone! It blends in so easily because it's a dark color. If I call it to find it, I don't always find it before it has switched over to the machine. Finley can't bring me the phone because it is too big for him to get his mouth around, but perhaps I'll find a way to overcome that. In the meantime, I've just been teaching him what the word "phone" is by using a handset that has died and putting it in his toy basket with all of his other treasures. I'm a proud mama, but he learned this trick quickly. Granted, he gets it right more often when we are doing obedience training and he knows I have treats in my pocket, but he's getting better at finding it the rest of the time too. I have to remember, however, that if I am not going to get up to put the phone back on it's cradle, that I must leave it low enough for him to find it. (Or to get up on the couch or the chair and see it when he's looking.) If I leave it on the kitchen counter, for example, he won't see it. 

Friday, November 28, 2014

The story of Finley, the Thanksgiving/Christmas dog

For the past year I haven’t blogged about probably one of the most important events in my life. I’ve wanted to hundreds of times, but I’m so emotional about it that every time I start to tell the story, I get teary. Since today is the first anniversary since his adoption, I am determined to get through writing this post!

Little in life has turned out the way I wanted it, or how I thought it would be, or how I planned things. Hermansky-Pudlak Syndrome affects different people differently. Some have little problems their entire lives, while others are profoundly affected throughout their lives.

Chronic illness can impact every part of your life. It can affect personal relationships, comes with great financial costs and can radically alter life goals. Simple things I assumed I’d experience in life such as getting married and having children, having a career, owning a home or having even modest financial security have all been altered or influenced by chronic health issues throughout my life.

On that list of major life goals, since I was about 14 years old, was to have a dog. We had a lab mix growing up named Spad. Spad was my dog sister. She was in every way part of our family. When she died, it was crushing. After some grieving, I resolved that someday, when I was all grown up, I’d have a dog again.

It never worked out. School, work, finances, traveling etc. all made getting a dog seem irresponsible. When I had to go on disability in 2008 and didn’t have a workplace to go to every day, I wanted a dog even more. The problem was I lived (live) in an apartment that forbids dogs.

About three years ago my mom got a dachshund puppy named Gerda. Gerda is like another dog sister. I was at my mom’s house when she brought Gerda home and participated in her first six weeks there. (Turned out to be great experience for raising Finley!) It was such a happy memory and a great bonding experience for my mom and me.

I lived vicariously through my mom, calling every day to hear what little thing Gerda had done that day. Gerda gave my mom such companionship and as time went on, I think my mom wanted me to have that same sort of companionship more and more.

Last fall my mom learned of a dachshund puppy that had been born very small, only two pounds. She thought it would be perfect for apartment living. So, dreading that asking would put the final nail in the coffin of my dream to have a dog, I asked my landlord if I might be allowed to have a dog. After all, I am home all day etc. To my surprise, he easily agreed. He told me he thought I could benefit greatly from a dog. It turned out he’s actually a big dog lover. The condition was that if the neighbors complained about the dog barking, it would have to go.

Thrilled beyond words, I started to do more research. I spoke with my doctors about whether there was any health reason that I should not have a dog. I made sure I wasn’t in any way allergic to dogs. I even called a few transplant coordinators at transplant centers knowing that one day that might be in my future. I didn’t want to get attached to a dog I’d have to give up for a lung transplant. I didn’t want to put a dog through the trauma of being given up. I thought this possibility was still years away.

The more enquiries I made, the more things seemed to fall into place. Everyone seemed to agree that a dog was not only not a problem for my health, but would be a benefit to it.

The last concern (still always a concern) was whether given my precarious financial situation, was it responsible to take on the expense of a dog? I stewed about this question for a few months and still worry about it. In the end, I decided that so many dogs are in need of good homes, that what I could provide would be better than being put to sleep for lack of a home.

And so the search began. I trolled the websites of dog rescues and dog shelters. Soon it was the Thanksgiving season and I had a long trip planned to go to NIH and visit my brother and his family. I decided the dog adoption would have to wait until I got back.

Then, much to my surprise, I got a call from my mom Thanksgiving Day. She and my aunt had been at the Wichita Humane Society the day before because my aunt also wanted a dog. As they were standing in the lobby a woman walked in with a little puppy wrapped in a blanket. He was lying on his back, paws folded forward, and looking up with those sweet brown eyes. The woman was crying. She had adopted him two weeks before, but was unable to keep him.

The next day, Thanksgiving, my mom called to tell me about the dog named Fenway. She asked if I would want this dog if she were able to get it.

Of course I did! If he’d had six legs and two heads I would have wanted him!

We ate our Thanksgiving meal with family but the entire time all I could think about was this puppy and how much I wanted him. That night I pulled up his photo on the shelter’s website and just stared at it for what seemed like hours. We weren’t sure if he would already be adopted by the time my mom and aunts were able to get to the shelter.

Black Friday arrived and instead of standing in the early morning cold waiting to get a deal on a TV or iPad, my mom and two aunts braved the cold to get in line at the dog shelter. I must have driven them crazy calling every 20 minutes or so to see how it was going. Was there anyone else in line there to adopt him? Finally, the doors opened and they were able to adopt Finley! You had to be a local resident, so my aunt filled out the paperwork, but Finley was a Christmas gift from my mom. He was the best Christmas gift ever!

I had to wait an entire week before I could get home and mom could bring Finley to me. It was such a long week! It was the closest I’ll ever get to waiting for a baby to arrive.

I spent time at the Petco down the street from where my brother lives planning for my baby. I bought a harness, leash and a few dog toys – anything I thought I might need to get us started. I knew I wanted to change his name (I’m not a big baseball fan) so I spent hours looking at pet and baby name websites.

Although everyone assured me changing his name wouldn’t be traumatic for a dog, I felt like this poor little puppy had already been through too much. He’d been left with a litter of puppies at the shelter, adopted, sent back to the shelter, and now he was bonding with my mom because I wasn’t home yet. It seemed a lot to endure in 12 weeks of life. Finally, I found the perfect name – Finley. It means “little fiery one” and it couldn’t describe him better! He is a high energy puppy! We often shorten the name to Fin, which reminds me of Huck Fin, a rambling orphan with a little more street smarts than an innocent puppy should have. Grin! And, it sounded so close to Fenway that I didn’t feel badly changing the name.

The morning my mom finally came with Finley I was more excited than any kid on Christmas morning. I called a lot of friends just because I was so excited and had to do something. I couldn’t concentrate on work or sit still. I didn’t want to be too excited when Finley arrived to make it easier, but I couldn’t help myself!

I knew at this point in my life I would never have children. This would be the closest I’d ever come to a baby, and thus Fin is, in my mind, very much my baby. I know some people think I’m a crazy dog lady, but honestly, I don’t care. I’ve waited a lifetime for this and I intend to enjoy every second of it the way I want to!

Mom stayed with me for several days as Finley settled in. He needed constant supervision. If left alone for even a second he would chew on furniture, cords or even Gerda’s tail! He weighed just at five pounds when he arrived and slept in a borrowed cat crate. I got up with him every few hours in the night those first few weeks to take him to the bathroom.

When my mom had to leave, Finley and I started bonding. He had very much bonded with her and Gerda in just a week!

I know people were making fun of me, but I put Finley on a schedule like a baby. It’s easier to potty train a puppy if you can predict when he’s going to have to go! And, because he needed to be watched so closely, it was the only way I could get some work done! I needed to know when he was going to be napping! Grin! Mom not only gave me Finley, but set me up with a “dog nursery” too.

She bought a large size dog crate that became Finley’s play pen. I put it under my desk and when it was time for Finley to settle down, I’d put him in the crate with lots of blankets and things to play with and crew on. She also set me up with a starting supply of puppy pads, dog food, dog toys, dog bedding, dog bowls – everything I needed! What a gift! Getting a dog gets expensive!

At night, just like a tired toddler that doesn’t want to go to bed, Finley would have his worst what I termed, “puppy butt runs”. He would run around the house as fast as he could with his little dimpled butt tucked in tight. He would chew and nip at me if I tried to pick him up. Just like a little kid, I’d have to settle him down and put him to bed. I’d catch him with a blanket so his milk teeth wouldn’t hurt me when he bit at me. I’d then sort of swaddle him in the blanket so he couldn’t get away and cradle him in my arms. I’d rock him to sleep, sometimes even singing to him or telling him stories. His little eyes would get heavier and heavier until finally he would drift off to sleep. I’d carry him to the cat crate I kept next to my pillows on my bed and carefully put him inside. I put pillows against one side of the crate to keep the draft away and covered it with a blanket.

Finley’s early weeks were not all easy and cute. The records on his history were very vague, but there was enough there to tell that he had been surrendered with a sickly and malnourished litter to the Humane Society. He had intestinal parasites that gave him diarrhea, not fun, especially for a dog that isn’t house trained yet! It took several rounds of medication and trips to the vet to get him over his initial intestinal problems. Lucky for him, I could relate very well to upset bowels having lived with bowel problems most of my adult life. Grin!

I’m so sorry now that I didn’t blog about those early days. Puppies are much like small children in that every day is a new experience and a new adventure. It is so fascinating to see how they react to things.

After the first few weeks, however, I went through a phase when I’d rock Finley to sleep that I’d just ball crying. I couldn’t explain why because I was so happy to have him! What was wrong with me???

I’m not sure what a psychological expert would say, but loving Finley that much brought up a lot of emotions I had never fully dealt with. Over the years working with others that have Hermansky-Pudlak Syndrome, I’ve watched quite a number of friends die. It’s one thing to have HPS, but it’s another to work with it day in and day out, to watch friends get sick with something you know is in your own future, and then to lose them. Yet, despite lots of advice to back off from HPS for my own emotional health, I was never able to do it. I felt like the benefits of feeling like I was moving research forward outweighed the emotional drawbacks. It made me feel empowered. I never regretted becoming close to friends that later passed as each of them blessed my life and made it richer for having known them. Even so, to be able to cope day to day, I had built up some pretty hefty emotional shielding. I just concentrated on the next task at hand – pretty easy if you live alone.

Suddenly, however, this little creature was tapping into the most tender parts of my heart. It was as if he poked a hole in all this pent up grief, and it came pouring out.

We very quickly bonded, and then fear set in that something would happen and I’d lose him. I’m sure part of that was from the grief I was suddenly processing, but some of it was because of our living situation.

My neighbors initially didn’t seem too pleased that the landlord had allowed me to get a dog. I do understand why. Our apartments are backed up to the yard of a house that had dogs which barked sometimes all night. It drove us crazy. The walls of my apartment are so thin that I can have conversations with my neighbor through them. I can hear his phone answering machine and even his phone messages. It’s like having a roommate you don’t actually live with. So, I can certainly understand the concern about a barking dog.

I was very naive about this potential problem. The dogs I’d had experience with were not barkers. I thought it was something I’d be able to train for since I was getting Finley so young.

The problem was Fin had some separation anxiety. I suppose it is normal for a puppy to be upset about being left. If you live in a house, you put them in a secure area, and if they bark or whine, they soon learn that you always come back. I don’t know if going through so many homes in his first 12 weeks had anything to do with it, but I couldn’t really blame him for getting so upset when I left. How did he know if I was coming back?

In my situation, however, all it would take was one complaint and I’d lose my baby. Getting groceries or going to my church group meeting became really hard because I couldn’t risk Finley getting upset. When I did leave him for very short times, I’d come home to barking, or the neighbors would tell me he’d been howling the whole time I was gone.

I had to start either taking him with me everywhere (and I don’t drive) or taking him to doggy daycare. Here I was worried about how I was going to afford a dog, and now I had to hire babysitters! This went on for months, but I was determined I wasn’t going to lose him. I tried every training trick I could find, but Finley usually outsmarted the process. I couldn’t leave him for short times and just go sit on the stoop because somehow he would know I hadn’t really left. When my dad visited, I went downstairs to do laundry, and my dad reported that Finley went from heating vent to heating vent tracking my movements in the basement. It was really getting to be a problem that had to be solved.

In the spring I went for my check up at NIH and it didn’t go well. I knew my lungs were worse, but I was not prepared to be told that I needed to be on oxygen, and that I needed to relocate immediately for a lung transplant. I guess you’re never prepared for that.

I thought I had been so thorough in my planning for Finley, but now this problem of separation anxiety was even bigger. How could I possibly move, go through hundreds of medical appointments and pulmonary rehab, and somehow not only get my dog to a sitter, but afford one! It seemed that Finley wouldn’t be able to come with me.

This will sound crazy to those who have never been close to a dog, but the idea of losing Finley was more upsetting to me than even needing a lung transplant. It seemed so unfair.

I had missed out on so many normal things in life, so many ambitions and goals. Finally, I had something I really, really wanted and here my heath was going to rob me of even that piece of joy and happiness. I had taken on the responsibility for my little baby, and now I was going to have to send him to live somewhere else.

Finley, thankfully, has never been in danger of going back to the pound. My mom would take him etc. Everyone tried to assure me Finley would be okay and I was thankful for that. But the thing was I was not going to be okay without Finley.

Before friends used to worry that I must be lonely on my own. I never felt lonely. Yet now, with Finley in my life, I can’t imagine being without him again. Even when he is at the groomers for the day, the house seems so quiet and lonely.

It is a blessing that I worked through so many of my pent up grief issues when I did, because now, needing a lung transplant and having so many cards stacked against me, there is a whole new pile of trauma and emotional baggage to cope with.

At first, I thought I was going to have to move within two months of getting home. As we began to work through various medical issues too complicated to get into here that date moved back.

I think once again God, who brought Finley and my mom together, was working miracles. The extra months have given Finley more time to grow up and mellow out. We’ve been able to conquer the barking and howling problem when I’m gone, although I am still very reluctant to leave him for more than five or six hours. We are using a bark collar that sprays him when he barks – the only thing that has worked. The trouble is it also goes off if he shakes or plays with toys that squeak too loud. It seems cruel to leave him for too long like that.

Much of our future remains up in the air. If I think too much about it the anxiety can be overwhelming. I try to just go on faith that somehow things will work out. Again, crazy dog lady here, but when we say our nightly prayers, Fin and I pray that God will find a way for us to stay together.

I am exercising as much as I can. I’d like to say the necessity of this for transplant is motivation enough, but when that seems too abstract, distant or impossible, it’s Finley that keeps me moving. He needs his walks. I need to do more with less lung function so I can keep caring for him.

Finley truly has been the greatest Christmas gift ever. He’s given me companionship. He’s given me the chance to mother something. He’s helped heal some of the hurts of my heart. He is a happy distraction from work and health issues. Indeed it is impossible to fixate on anything when you’re on the floor playing with Finley. He’s been a cheerful diversion in sometimes intense emotional conversations so that we never part from a conversation at a heavy point. We are both smiling or laughing by the time the conversation is over. He’s been a therapy to me, and I think in a small way, to others. He’s been a point of shared love and bonding with my mom. We talk daily about our days and our dogs as if we were two mothers of toddlers talking about our children. He is even learning to help me with things that now take up carefully budgeted energy. He’s been a gift in ways I can’t even explain, and probably don’t even know about yet. Finley is a Thanksgiving dog that has given me lots to be thankful for, and a Christmas present unlike any other ever.

Tuesday, November 18, 2014

Health Update

Yep, still hugely behind on blogging! I feel badly as I promised all of the people helping me raise money to try for a lung transplant that I would keep them all updated here.

So, here is the update.

A few weeks ago I had PFTs again (pulmonary function tests.) The two numbers we watch pretty closely with Hermansky-Pudlak Syndrome are the FVC and DLCO.

FVC stands for Forced Vital Capacity. It is the volume of air you can force out after taking the deepest breath you can. It is measured in liters, but also in percent predicted. The percent predicted takes into account your sex, age, weight as compared to the general population to compare you with others like you. My forced vital capacity was 74 percent of normal. While not terrific, it actually went up one point (mind you, that’s within the margin of error, but I do love it when things go up.)

The other number that we pay especially close attention to is the DLCO, or diffusion capacity. It measures how well oxygen moves through the tissues of the lung into the bloodstream. Mine was 45 percent of normal. For years doctors have told me that this is the most difficult part of pulmonary function tests to get a really accurate measure on, so not to worry if it goes up and down a bit. Well, easy for them to say! Grin!

When I was at NIH in May, it was at 49 percent of normal, and then in August, it went up to 52 percent of normal. So, I wasn’t terribly happy about this score. I wasn’t expecting an improvement, mind you – but was hoping for about the same. Whether this is in the margin of error seems to depend who I ask and how they think about it. But, it was down and that’s hard for me to shake.

I was disappointed because I felt like the test had gone really well! I am usually so anxious every time I have PFTs done. For years now I’ve been afraid each time I had to do the test that this would be the one that would catch me off guard. This test would be the one that would start the ball rolling to turn my life upside down. This time, however, my life had already been turned upside down. I’ve already been told that I need a lung transplant and that I have to move. I was still walking two to four miles a day on average (with 2 to 3 liters of oxygen). Thus, I really wasn’t expecting a big change.

What scares me is how quickly we seem to adapt to the new normal. I question whether I’d really know if there was a huge drop because it isn’t the sort of thing that changes from one day to the next. Perhaps living as a legally blind person all these years and constantly having to adapt, create solutions to get things done when one didn’t already exist etc….I just do it without thinking.

Last week my mom was here helping me work on the move, and suddenly I needed more oxygen than I had been. For two days after she left I was back on at least 2 liters 24/7. I also became more aware of little things I’m doing to adjust that I wasn’t even aware I was doing until someone else was here. The past few days, however, I’m back to using about as much oxygen as I was before the PFT and about what has been normal for me since starting oxygen in May. I’m thinking maybe the stress and all the dust etc. from packing was causing a little more stress on my lungs. These days it doesn’t seem to take much.

In the end, the change was not so huge and drastic that it altered the plans for the move one way or the other.

Maybe a crappy DLCO will keep me from getting too comfortable about all the things I need to get accomplished.

So much more to say, but not enough time. I will try to elaborate on this more later.

Friday, November 14, 2014

The first treatment has arrived!

This has been a historic fall for those with Hermansky-Pudlak Syndrome! I’ve been so busy helping to handle all the opportunities flooding our direction, and keep things on track, that I’m very behind on news stories, and even more behind on blogging!

How could I possibly have missed blogging about one of the biggest milestones in HPS history in the United States? Just think, there are things in the works so important that even this got put to the side for me!!!! That’s how exciting things are right now in HPSland!

In October the Food and Drug Administration (FDA) approved not one, but two, therapies for idiopathic pulmonary fibrosis! The first one, Esbriet (Pirfenidone) is the same drug the HPS community participated in clinical trials to use, so we do have some history with it. I was in one of those trials.

The day it happened, I felt so happy I could have exploded with joy! When I first was officially diagnosed with HPS in 2002 (long story), there were no treatments. Lung transplant centers thus far had refused to even try to transplant someone with HPS for fear of the bleeding complications.

Now my health is steadily getting worse and I am trying to get a lung transplant, yet I’m so thrilled and grateful that I lived long enough to see the day when we had 14 HPS lung transplants under our belt, and there was a legal treatment for pulmonary fibrosis in the United States. It has been such a long road of hard, hard work!

I know most people who have the privledge of taking a medication every day to help them never appreciate what it took from the researchers, the patients, the pharma company or the advocates to make that a reality. It is something I will never take for granted.

I worry, however, that my enthusiasm for this moment in history will oversell the drug to other HPSers. Let me be very clear. This is not a cure. We still have a long way to go. While there are a few anecdotal cases of some improvement, success with this drug is slowing the rate of progression – not improvement. I’ll be pretty happy if it just helps to stabilize my condition after the last year of being in a kind of free fall of progression. It also has some side effects. Some have no trouble with them, but others have a lot of trouble with the side effects. We are not taking a Tylenol here – this is a serious medication after all.

Perhaps it is the side effect of hope that is the best one for me though. Just when things looked so bleak, there is hope. I’m hoping I can surf this bleeding edge of medical science to make it to the next drug. There will be others. Ultimately, it will likely prove to be a drug cocktail of several mediations.

Now, we must keep advocating. We must always be the voice reminding the researchers, the government and the pharma companies that time is one of the most precious resources for us. It can never be replaced. The FDA had a chance to approve Esbriet three years ago. It was being used elsewhere in the world and even their own internal panel recommended it. Yet, they required a second phase III study. I wonder if I’d still be on oxygen if I’d had access to this drug a year ago? I wonder if some of those friends we’ve lost in the last three years would be here now if the drug had been approved? There are other drugs in the pipeline and every month that passes not deciding which drug we try next is lost time we simply don’t have.

So, we take a moment to celebrate this victory, yet we’re already on to the next advancement!

Tuesday, November 11, 2014

Hundred People Search Update

Today we added a new HPSer to the patient registry. This puts us at 87 to go on this year's Hundred People Search. We are way behind where we usually are, but we usually pick up 40 new people or so when we go to Puerto Rico. Since that didn't happen this year, we are a bit behind where we usually are. Now that there is a therapy available for the lung disease, it is just another reason why getting HPSers diagnosed and into the patient registry is so important. 

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: