Tuesday, September 25, 2018

Hundred People Search

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! 

Saturday, September 08, 2018

Hundred People Search update

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. 

Wednesday, August 29, 2018

Hundred People Search Update:



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 - it's free! Keep it up! And THANK YOU!

Thursday, August 16, 2018

Just waiting: A continuation of my transplant story…



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 compartmentalizing things. I remember knitting some. I remember watching some cooking show on TV and talking about it with my mom. I remember watching the other patients in the waiting room and wondering who was pre-transplant and who was post-transplant. I hoped the ones with oxygen tanks were pre-transplant.

Finally, a room opened up, and we went upstairs to report to the third floor cardiac telemetry unit nurses’ station. Every other time I’d ever been admitted to the hospital (except the NIH for research) I’d come in because of an emergency situation. It seemed so strange to just show up at the nurses’ station and say, we’re here!

We were shown to my room, and I started to figure out how I wanted to organize my new office. These plans were rather abruptly interrupted by the hospital’s plans for my new office.

This was a telemetry floor, something I’d never experienced in most of my previous hospital stays. It is the heart surgeons who perform lung transplants. Lungs and hearts work closely together – problems with one can lead to big trouble with the other. So, it wasn’t illogical that I was in a cardiac unit. But, my heart is one organ in my body with an excellent track record. The ticker was tick tocking just fine. Even so, the nurse had me change into a hospital gown and wired me up. If you’ve ever had an EKG, this is similar, minus the connections on the legs. The wires all connected to a little box that fit into a special pocket on the gown. The pocket was clever, but still, the box had a habit of falling out anyway. It gets on the nerves quickly.

This meant that the clothes I’d packed and brought were now mostly useless. I’d brought comfortable clothes and was told I’d be able to wear clothes if I wanted. Maybe that was true, but certainly not practical nor encouraged. I was stuck in the hospital gown.

I hate hospital gowns. I understand the need for access for IVs etc. but you’d think with all the wonders of medical technology; we’d have gowns that preserve a little more dignity. I know I’m not the smallest person, but it would be nice to have a gown that didn’t show off my butt so easily. By the time the transplant was over, I’m not sure there was anyone left whom I hadn’t mooned accidently.

Visitors were much appreciated. My friend Julie brought me Lebanese food one night, and it was a very welcome break from the bland hospital food.

After a few days working became harder because of the IV. When I’d bend my arms to type on the computer, it would keep the IV from flowing correctly, and it would get really sore.

After a few days, lungs became available once more. The procedure from Thanksgiving was repeated – clean sheets, a special pre-surgical shower, down to pre-op, lines put in etc. It was easier this time because I’d been through the entire process, right up to the operating room, before. I didn’t feel anxious at all. I even took several naps. It was such a different feeling than at Thanksgiving when Ryan and I had stayed awake all night because we were just too anxious to sleep.

Once again, however, the lungs turned out to be unusable. I’m not sure what was wrong with them. I do know that the surgeon said it would be criminal to try to use them. Everyone was so deflated – not just me and the family, but the surgeon, the nurses, the nurses back on the floor. I know this must get routine for them, so I really appreciate the genuine sincerity everyone seemed to have. I took it pretty well. After all, what good would those lungs have done me if they were in such bad shape? It was the first time, however, back in my room that I started to feel truly anxious about whether a new lung would come through.

I can’t explain that. I know the statistics and I’ve seen many friends go through this and not get a lung. For some reason, however, I just felt it would happen. Was it a coping response? I don’t know, but whatever it was, no matter how illogical it seemed, it was working for me.

A few more days went by. Thankfully my oxygen demands remained about the same. Yet I knew that wasn’t going to last. Then, I got a phone call from Dr. Nathan, head of Inova’s lung transplant program. There was a possible single lung, but it was a high-risk lung. Would I be willing to accept it? I didn’t even have to think about it. Yes!

I had already researched high risk lungs, and what that really meant. I wanted to make that decision ahead of time so I wasn’t in a crisis considering my answer. I knew the docs wouldn’t give me a lung they thought wouldn’t work. High risk had more to do with unknown medical history or a history of things that could make the lung higher risk for infections. I knew lungs were tested for things like HIV or hepatitis C before they would be transplanted. So, yes, knowing another lung or lungs might not come along in time, and knowing if there was anything wrong with the lung they wouldn’t use it for transplant unless they felt the problem was treatable, it was a grateful, and emphatic YES!

To be continued…

Wednesday, July 18, 2018

I want it, and I want it now

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 holding steady, and even trending upwards at a slow crawl. I’ll take that! At least it is moving in the right direction. I haven’t needed oxygen, and the times where I might be on the bubble needing oxygen are fewer and fewer.

My legs, however, remain a problem. I think they are improving, but it is hard to tell. One day I can walk a mile, and the next it’s a struggle to make it around the block or stand at the sink doing dishes for longer than 10 minutes. I don’t quite get why it can vary so much from day to day.

No one has to sell me on the importance of exercise. I’m sold on the concept. I was never a big fan before, admittedly, but my two years in pulmonary rehab convinced me it is the key, not only to a decent quality of life pre-transplant, but to recovery post-transplant. It seems, however, that my legs never got the memo.

I’m feeling frustrated. Yes, I know, everyone’s recovery is different, but at this point, I feel like I should be able to do more than I can in a more consistent way.

Sometimes I need a reminder about how far I’ve come, even if it isn’t far enough. This morning I was looking for a document in my e-mail from my transplant coordinator. As I searched through the e-mails, there was this record of back and forth about all the problems that have come up along the way. I have to remember I’ve only been out of germ jail for about a month. It wasn’t quite two months ago I was fighting falling white blood cell counts. Even if I can’t walk as well as I should, there has been progress.

Even so, let me be cranky about my lack of progress. Let me complain and moan and be frustrated. It isn’t that I like feeling this way, but be glad that I do. It’s the frustration and the high, maybe unreasonable, expectations that keep me pushing. Be glad I’m not happy to continue to cruise around in my wheelchair (as fun as it is for someone who has never been able to drive.)

Last night as I was lying in bed feeling so frustrated, I was doing leg lifts and talking to my legs. The language I was using to them isn’t family friendly. I was just trying to find a way, something I could do, to make those muscles work. I’m still waiting to hear back from rehab. I can’t wait to hear back from them to work on this problem though. So, I complain, and grumble, and feel frustrated and cranky – and so I move.

Monday, July 09, 2018

Nobody likes rejection


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 had my diary of vitals to show the doctors in a numeric way something was up. It wasn’t just a feeling I had. I had evidence. Not that I couldn’t tell something was up mind you. I went from being able to walk a mile and a half without oxygen, even climb a hill, to hovering at an oxygen saturation of 90 with barely any movement at all. It got a little worse each day over a three-day period. The morning of my transplant clinic appointment I desaturated to 86 just putting my clothes on. I showed up at transplant clinic with an oxygen tank because I was worried about making it from the front door of the hospital to the clinic.



I’ve been fighting infections since the new lung hit my chest cavity. It came with some bacterial hitchhikers and we’ve been working to evict them ever since. So, our first thought was that the last remaining infection was somehow getting the upper hand. I was admitted to Fairfax Inova Hospital for what turned out to be a week stay.



Lots and lots of IV antibiotics were ordered and given for the first few days. A bronchoscopy was ordered to have a look around for any pockets of stubborn infection or other problems. Before the bronchoscopy, I seemed to be making a little progress with how far I could walk without oxygen. But, during this test they flushed a lot of liquid into my lungs so they could suck it out, put it in petri dishes, and wait to see what interesting junk would grow. I think that knocked me back a few days.



Happily, however, the infection side of things looked great – maybe the best it has looked since my transplant. Yippee!! The downside, however, was that meant the most probable cause of the trouble was rejection. The R word. The big R. I don’t care if they say this is the treatable kind of rejection, or that it’s the rejection we are fighting every day anyway…I just can’t get okay with the R- word.



Next came the high dose steroids and all their side effects. I probably didn’t notice the side effects as much right after my transplant because so much was happening with my body. This time, however, I experienced the crazy appetite, being unable to sleep, my body puffing up like a stuffed animal – just to name few. This time, after a few months of starting to feel normal again, I noticed.



It seems the steroids did the trick, so it’s worth it.



I am amazed by how much this recent setback really did kick my butt, to be quite frank. While in the hospital I was so worried about losing ground. I walked around the unit (with oxygen if I needed it). I paced my room. I looked for things about the right weight to try to keep my arms at least as strong as they were before I was admitted.



When I got home, however, even though my oxygen saturations were better, what I could physically do was not. My feet were numb from one of my rejection medications being increased. They are also constantly frozen. My hands shake worse because of the medication. My body was, and is, retaining water, even though lasix makes me pee about every five minutes. My legs looked like the legs of a piano. It was hard to feel real stable on them.



Mother Nature wasn’t helping either. The thing I needed to do most was get out and walk. So, I started getting up super early so I could get out and walk before the heat and the humidity kicked in for the day. Getting up early wasn’t as hard as it usually is (I’m not a morning person) because I wasn’t sleeping anyway. Still, this just doesn’t seem like enough to get back to where I was.



After a few weeks at home my legs started to do strange things. Of course they are weaker than they were, but this was different. One day they’d be relatively okay, and the next it was as if they were made of melting butter. It wasn’t that they were tired from working hard, or that I was getting that exercise burn. It was more like my legs just decided they didn’t want to play anymore.



While my spirometry was back to where it was pre-admission, it wasn’t moving out of the range I’d been stuck in for two months now. That is so long without any improvement, it was getting to be a cause of concern for everyone – not just me the patient with no patience.



This Friday, however, I had my first follow up appointment since being in the hospital. I laid this all out for my doctor thinking he’d be concerned and maybe want to move to doing the dreaded biopsies. To my surprise and great relief, he explained all of this was to be expected. The weird leg thing, he says, is a possible side effect after you’ve had a large pulse of high dose steroids. Apparently it can happen weeks after the doses. Thank God! He wasn’t concerned about the other things as he expects them to all improve. They are mostly a result of all the drugs I got in the hospital, and all the medication changes I had when I went home. Best of all, I’ve been referred for more pulmonary rehab. I’m thrilled. When it feels like 105 outside, it is truly hard to get enough meaningful exercise to truly build myself back up in my tiny apartment. I do try, but it isn’t hacking it. Right now, as my friend Mary Ann says, I’m like a hot house flower. In the good old days you just sweated through hot days. Now, walking in the heat makes me dehydrate rapidly. I get to feeling sick to my stomach and faint. Just little o’l delicate me you know…grin. Because of my suppressed immune system the docs vetoed going to a regular gym. So, hopefully my insurance will approve this and I can get enough sessions to at least get my legs more stable again so I feel a little safer doing this on my own.

Tuesday, July 03, 2018

Planning for Armageddon

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 by trying very hard not to think too much about it. The best therapy was playing with Finley, my dog, or working on things for the HPS Network. It all gave life this sort of normalcy in the midst of something that was clearly not normal.



So, I was taken back when the doctor told me I should start thinking about coming into the hospital to wait for my transplant. I knew this often happens, but I had dreaded it. How would I continue to cope sitting in a hospital room I knew I might never leave? Although at this stage I was pretty much stuck at home because my massive oxygen needs made it hard to go many places and not worry about running out of air, I felt I was okay at home. Surely we weren’t there yet?



The doctor and transplant coordinator explained that there was an upside to being in the hospital – it would give my lung allocation score a huge jump. The chances would be better that I’d get another call. I felt like they were trying to make a bad situation sound better. Besides worrying about how I would cope with things in the hospital, I was worried about being able to walk around and exercise. I worked so hard at pulmonary rehab and I didn’t want to lose ground when, if I were at home, I knew I’d be able to exercise and move around. (When you are an inpatient, they don’t let you go to the normal pulmonary rehab classes at the gym. A therapist has to come and see you. So, by definition, the sessions are not as long nor do you have access to all the gym equipment.) I knew I could lose ground quickly and was worried that this would, in the end, not serve me well. I also worried about being in the hospital in the midst of flu season. I had put myself on a kind of voluntary germ lock down, only going to places I had to go and wearing a mask as much as I could. How did it make any sense to go to germ central – a hospital – any sooner than absolutely necessary?



I went home and thought it all over. I didn’t do anything for maybe a week. I just sort of let the idea soak in. Finally, I concluded, the decision was a matter of strategy. Everyone would like to think that the transplant process is one of complete fairness and protocols, but for me, it has been a matter of strategic planning for years. Planning had given me an edge. If I went into the hospital, my score would go up. I had not had another offer since Thanksgiving, and clearly things were getting worse. Perhaps the best strategy was to bite the bullet and go in, even if I felt I wasn’t really ready.



The next few weeks were some of the hardest of the wait. On the plus side, it gave me a chance to prepare to go into the hospital, but on the down side, it gave me a chance to prepare to walk through those mechanical swooshing doors knowing the odds were better I would never walk back out.



My sister-in-law helped me get an attorney to put my “affairs in order.” Not much to do now. I literally own pretty much nothing. After 10 years of being on disability, there is absolutely nothing left. Still, everyone needs things like medical powers of attorney etc. I wrote careful instructions for my brother, who would most likely be in charge of managing my affairs should I be too sick, or too dead, to do so. I wrote out instructions for taking care of Finley that were way more extensive than any of my financial or legal documents. I’m his mama, and I wanted to be sure whoever was caring for him (there were several ways that might go down) knew all of his little quirks, likes and dislikes, favorite toys etc.



I gathered up HPS work I needed to do and knitting I wanted to have with me.



There were days I looked around my living room and everything seemed so still and normal. The neighborhood noises, the way the light came in through the windows or the way Finley brought me toys when I stopped working – it was all just so crazy normal. Yet there was this count down occurring for that day I’d check myself into the hospital to wait. It was something like being in a crashing airplane plummeting toward the Earth, yet no one seemed to notice.



I had a lot of dark, but to my mind practical, thoughts. Sharing them wasn’t something I felt like I could do much of, however. I felt people would say things like, “Don’t be negative.” or “That isn’t going to happen” or “Have some faith.” I knew if I heard those things I might really lose it.



When my friend Elsie went through the same thing, I remember she had such a strong need to be practical, to be sure all the details were taken care of, but it was hard for the people around her. I’ve seen this a lot with various HPS friends. Not acknowledging what the sick person waiting for a transplant is going through isn’t really helpful. It might feel easier, but it isn’t helpful. We, as the patients, know there aren’t really always answers. Yet, there may be things we need to do or say so that we can relax and cope and play this mental game of chicken until that precious organ arrives.



A week before I was to be admitted, my mother came to help out. She wasn’t thrilled about my luggage situation. I was taking a LOT of stuff to the hospital. My friend Donna Appell had given me some excellent advice. She suggested thinking of being in the hospital as just a change in geography. When I was admitted, I should do what I could to make my hospital room my own. I should establish a routine for the day, just like I was doing at home to cope. So, that is what I did. I packed to turn my hospital room into my office and art studio. The thing I dreaded most was being stuck in there unable to do anything but think of my ever quickening demise. If it took packing three suitcases full of a bunch of stuff it turned out I’d never need to make it possible for me to walk into that hospital, then that is what it would take. Tough luck - HPS Washington DC office was moving to Inova Fairfax Hospital and that was the way it was going to be.



During that last week I had a couple of close calls that brought home my decision was the right one, even if it were a hard one. There were a few times my oxygen dipped so low when I was coughing, that I had to put a high flow cannula in my nose from an oxygen tank at 15 liters, and a mask on my face from the concentrator at 10 liters, just to get my oxygen saturation number to come back up again. It was scary and getting a little dangerous. Several times I wondered if I shouldn’t wait for the next clinic appointment to be admitted. Perhaps I should call the coordinator and go ahead and go?



The day finally arrived for our appointment and admission. The appointment wasn’t until the afternoon, so I’d left several last-minute things to do that morning. My coordinator called, however, and asked if it would be possible for us to come in that morning. I wanted to stomp my foot and say no – this day is hard enough for me already and I’m working with a plan here. Yet, I’m just not that kind of girl. I said we’d be there as soon as we could pull together these last-minute items. It was a blessing that she rushed us. I didn’t have too much of a chance to think about everything. I would have probably been ready by noon and spent the next two hours holding Finley and crying. Now, I didn’t have time. We rushed out the door. I remember looking back at Finley as I shut the front door to lock it. He was looking up at me the way he does every time I leave. Suitcases had come out, so I know he knew something more than the routine was up. But, he looked up at me with his beautiful eyes and all I could think of was how it might be the last time I would ever see him. You can’t explain to a dog what is happening. I was worried he’d think I just abandoned him if I never came home.



The taxi was waiting. The suitcases were being loaded. I couldn’t fall apart. I didn’t want to make it harder on everyone else. It was time to leave.

Friday, June 29, 2018

The blog is back!


I know, I know, it’s been a while. Totally not reliable. Totally not what I had envisioned. I have a good excuse. I’ve been busy getting a lung transplant! Yep, I was one of the very lucky to get a lung! I have many upcoming posts to talk about that.



After all of my desire to record this HPS journey, I’ve left out one of the most dramatic parts. As I got sicker, I just got more tired all the time. There are certain things one has to do in life – fix dinner, work on things that bring in income, go to medical appointments and pulmonary rehab. There simply was less energy to do anything that, while perhaps important and meaningful to me, were not absolutely necessary to my survival.



As you might imagine, recovering from a lung transplant is no small matter either. I am now four months out, have had one major setback, and am still in pain every day and currently struggling to walk further than a block. I had been up to a mile and a half before the most recent hospitalization.



But, while my body is a mess, my brain is back to working relatively normally, at least most of the time. So, I’m hoping to be more back to normal and post some about the transplant journey. And of course, thanks to that new lung, life goes on with lots of things to blog about.



I’m back!

Thursday, January 18, 2018

Hundred People Search Update

We've added six new HPSers to the registry. This puts us at 55 to go on the Hundred People Search. 

Wednesday, January 03, 2018

Hundred People Search update

We've added five new HPSers to the patient registry. This puts us at 61 to go on the Hundred People Search (HPS).