Living on the frontlines

In the past couple of days we've added three new HPSers to the registry. This puts us at 72 to go on this year's Hundred People Search! 

We've added two new people with HPS to the registry. This puts us at 75 to go for this year's Hundred People Search. 

Hundred People Search Update: This week we have added two new HPSers to the registry. This puts us at 77 to go on this year's Hundred People Search - H.P.S. For our newer people, the Hundred People Search is a yearly outreach challenge. We've never actually hit 100. It isn't that we want people to have Hermansky-Pudlak Syndrome - it's that if they have HPS, we want them to have an accurate diagnosis and to be able to give them information to help them advocate for their needs. The scary thing isn't having HPS, it's having it and not knowing it. To that end, there was a recent lengthy discussion on one of the general albinism facebook groups about testing for HPS. Bravo on the outreach!!! Several HPS community folks spoke up and helped to educate. I don't know if these two things are related or not, but the office tells me they've had a spike in calls from people seeking information about testing for HPS. Outreach online isn't only effective - i...

Photo: This is me the week before the transplant. The one with the roses was taken on Valentine's Day. My sister-in-law gave the roses Ryan had brought her to me.   To be honest, I don’t remember much about the time I spent in the hospital before my transplant. I’m not sure if that’s because it was pretty boring and routine, so there’s just not much to say – or if it is some kind of self-preservation maneuver my brain has done to shield me somehow. After the big rush to get to the clinic early, we spent several hours in the waiting room with my luggage as if we were waiting for an airplane at the airport. It turns out the big rush was due to a hospital policy that the clinic couldn’t request a bed for me until I was actually there. The hotel Inova Fairfax Medical Center was packed to the gills, and they wanted my name on the waiting list. It was late afternoon before a bed became available. Looking back, I’m amazed at how calm we were sitting there. Perhaps we were compartmentaliz...

Patience may be a virtue, but it is certainly not a skill of mine. I am just about at my five-month lungaversary. They are constantly telling me that everyone’s recovery is different. You can’t compare your recovery to someone else’s. Intellectually, I get that. Really, I do. Emotionally, however, I’m like a toddler. I want it, and I want it now. And why shouldn’t I? This transplant has been hard work. I’m blessed that I got a lung at all. So many of my good friends did everything right, waited and waited, and the lung never came in time. While I’ve beaten the statistics consistently throughout my life, I am well aware of all the post-transplant statistics. I am well aware of all the complications that can happen. I’ve been given a chance. I’ve been given more time. I don’t want to spend it waiting. I’ve got a lot to do with this new lung. Thankfully, (knocking on wood) the new lung is working pretty good right now. It isn’t up to full capacity by any stretch, but my spirometry is hold...

Me posing for a photo with Finley. This is my swollen steroid face. Thankfully, it is starting to go down. Rejection – it’s a word I’m hard pressed to find a good context for – and in transplant land, it certainly isn’t a good thing, but perhaps it’s a constant companion. Once you get an organ that you weren’t born with, your body is intent on seeing this life-saving gift as a foreign invader it must destroy. It’s a battle that is constantly going on inside you, and it’s a battle you and your doctors constantly fight. The only good news is medicine has created a number of weapons to hold rejection at bay. In June I had my first setback because of acute cellular rejection. They tell me it’s the good kind of rejection to have because it is treatable with an onslaught of steroids and medication adjustments. Oh goodie! It was amazing how quickly it came on, and really brought home how important it is to do those vitals and spirometry every day. When I thought I might be in trouble, I ...

Sometime in January I had a transplant clinic appointment. I can’t exactly remember the date now, but I remember the appointment very well. It was the beginning of what could have been the end. I did the usual tests – spirometry, a six-minute walk etc. I was now not just on eight to 10 liters of oxygen. I needed 10 liters of oxygen pretty much all the time; even to walk the maybe 15 to 20 feet to my bathroom. To go for a slow walk required 15 liters. The funny thing about increasing oxygen, especially when it happens over years as it did for me, is that you adjust to it. It just becomes your normal. You might get annoyed by it, but somehow it becomes this thing in your life that just is there. So, although I was clearly very sick at this stage, I didn’t feel sick. I felt tired a lot, but not sick. I didn’t feel pain (thankfully). I didn’t feel weak (even though I needed a lot of help with household activities so clearly I was). I just adjusted. I coped with the waiting for a new lung...