Wednesday, June 14, 2017

My new set of wheels





I am so blessed to have such awesome friends. There is no way I’d have the resources to get through this medical drama without all the angels in my life coming alongside and helping me – sometimes even with things I don’t even realize I need help with.


I had never thought about getting a wheel chair. My legs are one thing that is working quite fine. During the past three years that my lung disease has been pretty bad, I have used wheel chairs or scooters for short-term things, like attending a conference that involves a lot of walking. But why I didn’t even think of how it could improve my current quality of life, I honestly don’t know. My focus has been on walking, walking, walking – as much as I can stand and still function, and as much as the oxygen company will allow me (a blog for another day.)


Lucky for me, my dear friend Donna thought of it for me. She had a family member pass away and leave behind an electric wheel chair. Other friends joined in and transported the wheel chair a day and a half drive to get it to me. I feel like I have been liberated! As my lung disease gets worse (and unless I get a transplant before this happens) this stage isn’t going to last forever, but for now, and for however long it lasts, I can take advantage of living in a place with so much to see and do, and with public transit! I can run to the pharmacy and not have it cost me the time to go to the pharmacy, plus the hour or more it takes me to recover from the workout of the Arlington hills on the way to and from.


Ironically, my Fitbit steps have gone up since getting the wheel chair. I am actually walking more! I think this is for a few reasons. First, if I go a few days without doing a certain amount of walking, I lose ground quickly. My breathing gets worse. I feel worse overall. Second, although I lost enough weight to get listed, I have to continue that journey, and not moving won’t help. Third, I can do things I have to do, like to go the pharmacy, and have more energy and oxygen left to do the things I enjoy doing, like walking Finley. Fourth, I love Finley like he is my child. I would do anything for him, and he needs to be walked. I love watching him when we are walking. It looks like he is smiling. His tail curls and wags and he literally prances. I call our walks, Fin Therapy!


Driving the wheel chair can sometimes be exciting when you are legally blind. I don’t think I’ve ever driven it at top speed. The steering is way more sensitive the higher the speed, and the swivel wheels tend to do what their name implies when you hit bumps or uneven ground. Not racing around like I’m driving a car seems a little safer for everyone. I also have to remember that people and traffic are not aware that I might not see them (another reason for going slow on the sidewalks - so I don’t run over people because I didn’t see them in time to react.) Interestingly, however, at least in my neighborhood, the traffic in general seems more cautious of the wheel chair than they were of my cane. That is in the category of, things that make you go….hmmmmm…..


My friend Eden and I recently tried out the wheel chair access of the public bus system, and I was very pleased with how easy it is. Now, if I wanted, to say, go to one of the Smithsonians on a whim, I could. I don’t usually, but knowing I could is a great psychological boon!


Thank you friends – and look out Washington. Blind driver on the loose!


Saturday, June 03, 2017

Update from the last transplant appointment

The day before the American Thoracic Society meeting, I had a transplant clinic appointment. I'm not sure how to characterize it - good, bad or neutral. Basically, my lung function fell, my six-minute walk covered less distance and the doctor reminded me (as if I needed to be reminded somehow) that although the differences are small, they've all had a downward trend for the past two years. 

Sometimes I keep myself calm about things by looking for the small upsides in larger bad news. For example, the upside of getting sicker is that my lung allocation score (where I am on the transplant list) goes up. This time, however, the numbers, while not positive, did not impact my lung allocation score. So, no upside to being reminded I'm getting worse. 

It was a depressing appointment. 

I'm so grateful that the day after ATS started, and I got to spend time with all of my HPS friends as well as keep busy. 

So, still waiting, but no change in my position on the list. I guess that is good news, in a weird sort of way? 

Thursday, June 01, 2017

What HPS research can offer the greater pulmonary fibrosis community

While working on going through everything I collected, wrote down etc. from the recent American Thoracic Society meeting, I found this abstract about potential biomarkers in Hermansky-Pudlak Syndrome Pulmonary Fibrosis. This is not a done deal yet, sort of speak, but to me, it's an exciting development. 

For HPS, finding meaningful biomarkers that directly tie to lung fibrosis progression, and not just HPS in general, could help our efforts for better treatments, and someday, a cure. 

Because people with HPS can know they will develop lung fibrosis way, way, before it ever happens, we offer researchers a unique opportunity to uncover early warning signs for pulmonary fibrosis. I can't think of another pulmonary fibrosis population that 100 percent knows they will get pulmonary fibrosis, sometimes decades before it happens. There may be one out there, but I'm not aware of it. 

For people with HPS, or someday others at higher risk for PF due to genetics or exposures, these biomarkers may someday help doctors know the perfect time to start therapies that could preserve lung function. 

These biomarkers could also greatly help prove whether drugs work in future drug trials. 

It's pretty exciting stuff! I wanted to post it here, however, for a few reasons. 

First, as a very, very rare disorder we can often be overlooked. Our funding can be harder to get. Why, after all, spend money on such a rare disease instead of on something that affects more people. This research is a great example of how the rare can be a good investment for more common disorders. 

Second, this research would never be possible without the patients with Hermansky-Pudlak Syndrome who take the time out of their busy lives to participate in research. 

It isn't easy. Many of us have kids, jobs or other responsibilities that make it hard to take days out of our lives to travel, often across the country, to participate in research. It isn't convenient for any of us. Yet, without these willing people there would be no biomarkers to look for - no reason for researchers to keep looking for better treatments - in short, no reason to find a cure. 

So, thank you to all the HPSers who travel to NIH, or who are participating in the Young Lung centers. We know it is a sacrifice, but what you are buying us all is hope. 

Here is the research that got me excited today: 
http://www.atsjournals.org/doi/abs/10.1164/ajrccm-conference.2017.195.1_MeetingAbstracts.A6799

Tuesday, May 30, 2017

Hundred People Search Update

We have added another HPSer to the patient registry. This puts us at 93 to go in this year's Hundred People Search. Keep up the great outreach!!!! 

Thursday, May 04, 2017

Hundred People Search Update

We have added a new member to the HPS registry. This means that we have 94 more to go on this year's Hundred People Search. Member networking was key to finding this family, so please, keep up the outreach! 

Monday, April 10, 2017

Hundred People Search

We have a Hundred People Search update! There are three new people on the HPS Network registry, one from overseas. I'm always especially happy to see those as HPS is so rare, we can't afford to be divided by international borders. Keep up the outreach!

Tuesday, April 04, 2017

Celebrating 100 years


Recently my grandmother (grandma) Meribel Cockerill passed away. She was born on July 11, 1917 and passed away on March 23, 2017. I can’t even imagine living that long!

Friends are telling me they are sorry for my loss. I am sorry, but celebrating. She was not well for many years and honestly, she was miserable on this earth.

Most of my life I never lived near either set of my grandparents. I did, however, live with my grandma Cockerill for six months after my ostomy surgery. It was one of the blessings that came out of a pretty horrible year.

My grandfather had just passed away, and my mom had to return to her job in Germany. I was a LOT better than I had been during the nearly two months I spent in the hospital – but I wasn’t well enough to go back to college or be on my own. Often staying at my grandparents could be stressful. I couldn’t drive, so I couldn’t get out to go to the mall or grab a hamburger. My grandparents didn’t have 18 plus years of experience raising a child with a visual disability, so they were constantly worried about me. Even just going to walk around the building to get fresh air could create anxiety for them and result in tension between us.

When I came to live with my grandma after my surgery, however, walking to the elevator from her apartment was an accomplishment. Day by day I would go a little further and she was okay with it. We got along perhaps the best that we ever did. We really needed each other. I wish I had appreciated more at the time how much I wish I could have asked her now. Her mind was still together then, and it would have been such an opportunity to learn things I wish I knew now.

My grandma Cockerill’s maiden name was Hernandez. Her father, Jose Hernandez, had come to the mainland United States to go to college from Puerto Rico. I didn’t know I had HPS then, or how much I’d wish in later life that I had a way of contacting distant relatives in Puerto Rico. At that time she was in touch with a living cousin who had been a school psychologist.

Sometimes she would tell me stories about visiting her grandma in Puerto Rico as a kid. It wasn’t a simple flight like it is now. They would take a ship – often from New York – and then spend months on the island. Considering that they lived in Oklahoma, that really was quite a trip!

My grandma was the first person who tried to teach me to knit. My parents had gone on a trip for a week, and my grandma and grandpa had come to take care of Ryan and I. At that point in my life I wanted to be Laura Ingels Wilder when I grew up. Knitting seemed like a pioneer kind of thing to do. We went to the store and she bought me some yarn and knitting needles. I never finished the project since, after a week, no one was around to help me. What I knitted was supposed to be a scarf, but it sort of turned into a triangle with a bunch of holes in it. Yet, decades later, when I made my second attempt to learn to knit in college, I did still remember how to knit and purl. Grin!

When my grandma was sick this time, out of curiosity I looked up news headlines from about the time of her birth. She was born weeks before the first draft card was pulled to send troops to World War I. Aviation was just getting off the ground – pun totally intended. Now, we have rovers on Mars sending back photos and videos from the planet’s surface. I’ve seen the stars (via the rover camera) from the vantage point of standing on Mars.

When she was born, no one listened to radios. Today, we have the internet and I regularly communicate with friends around the world.



Although we will greatly miss her, I know she’s happy and whole with her friends in family in heaven. I just hope I don’t join her there anytime soon. While I doubt I’ll walk the Earth 100 years, I still have a lot to do here!

Wednesday, March 29, 2017

Hundred People Search

It is that time of year again - the time of year when the Hundred People Search (H.P.S) starts over again. We are kicking off the year with two new members on the HPS patient registry. That means we only have 98 to go by next year's conference! It's a big task, and honestly, we've never hit 100. Perhaps this is the year that can change that.

What is the Hundred People Search? It is an outreach challenge to the community of those impacted by Hermansky-Pudlak Syndrome or HPS. We dream of a year when there is no one to find, but sadly, that just isn't in the cards. Together, as a community, we dream of better treatments, and someday, a cure - but that can't happen unless the HPS community comes together. We have to find one another, all over the world and not just in the United States, to show researchers we are here, we are willing and we need a cure!

We are limited by being rare, and by not having millions to spend. So, it is up to us and our supporters! Many are involved in the albinism community and many others are involved in the Puerto Rican community (most affected by HPS). They are best placed for grass roots outreach to find others with HPS.

It is up to everyone whether they want to be more deeply involved in the community, or just sign up so that they can learn about research, meet others in the same shoes and join in to help us in the journey to find a cure. Everyone copes differently. It is vital for all of us, however, that we find those impacted by HPS around the world so that someday, we can find better treatments, and a cure, for all of us.

Thursday, February 16, 2017

Hundred People Search

We have added a new HPSer to the patient registry. This puts us at 70 to go for this year's Hundred People Search initiative. We only have three weeks to go for the year. If you know an HPSer who is not registered with the HPS Network, please ask them to register with us - no matter where in the world they live. It is free and it helps promote a cure for all of us!

Tuesday, February 14, 2017

National Donor Day – a special kind of Valentine

Today is National Donor Day – a day to highlight the very special gift that those who agree to become organ donors give to the 120,000 of us currently waiting for new organs in the United States. It is the kind of gift that words fall short of. Hallmark doesn’t make a card for this. How could they? 

I’ve noticed from the experience of some friends who have received their lungs that the time frame before you are allowed to contact (through UNOS) your donor family has changed. They told me in my transplant education that while I was still in the hospital, I’d be given a card to send to my donor family. It is, of course, up to them whether we ever make contact. I know if they are willing, I will want to do it. 
I can’t imagine writing that kind of thank you note, especially while still in the hospital – but I could never not do it! I’ve been thinking of writing a note and having it in my things ready to go so that it is well thought out, and ready to go. 

What do I say? I’ve spent a lot of time thinking about it lately. Do I tell them about my anti-bucket list? (It’s the list of things I’m looking forward to doing when my lung function is normal again.). Do I tell them about my life before the transplant? How does that not sound like a resume for my lungs? I want them to feel that their loved one’s lungs have found a good home, and that I am somehow worthy of such a gift. Just thinking about it makes me teary. 
A few weeks ago I was watching a medical drama. I used to love those kinds of shows, but have been watching them less frequently these days. This particular episode (fiction) followed a family as they decided to donate their loved one’s organs. They went from hope to resignation. I know better to believe that this dramatization was anything like what families donating a loved one’s organs might experience. Spending a lot of my time in the medical world, I know these shows make sacrifices of fact and reality for the needs of an hour-long drama all the time. Still, even so, I was balling. I think I cried for 40 minutes. 
I think about this family whose path will cross with mine someday a lot. 

So, on this National Donor Day, let me just say THANK YOU! to the live donors and donor families out there. What a gift you give us. It isn’t just the gift of life, it’s the gift of hope.