Thursday, November 16, 2017

A three hospital bracelet day



Tuesday turned out to be an exciting day! I collected three hospital bracelets (my jewelry) in one day, and none were allergy bracelets etc. They were for three different admissions! Woohoo!

Bracelet number one

The morning started off like my Tuesday mornings usually start off. I’m up at 4:30 am to get through all of my medications, take Finley out, and have plenty of time for coughing and sitting down to catch my breath to be ready for the cab at 7:15 am. Rehab wasn’t going as well as it had on Friday, but you have good days and bad days. I was on one of the bikes, and it was almost time to go. One of the respiratory therapists came over and said the transplant clinic had been trying to call me and they needed me upstairs right then. Of course my phone was in my purse because I was working out. At first it didn’t hit me why they were looking for me. I thought they might want another sputum sample and I have my paratransit ride to go home after rehab. It is hard to just “run up to clinic” when you never know how long the wait will be, and you’ve got a ride waiting. I felt mildly annoyed.

The therapist said, no, not after rehab – they want you right now. It dawned on me what she was trying to say. Suddenly I felt tingly all over. You wait and wait for the call, and then when it finally comes it is a feeling of shock and being overwhelmed. My mind was racing. This could be it!

One of the therapists came up and waited with me until my sister-in-law Sara could arrive. I hadn’t reached Ryan, but she had and he was on his way. It was faster for him to come by metro than for her to fight downtown Washington traffic during rush hour to pick him up.

After the rush upstairs, we waited, and waited (this would be the theme of the day – waiting!) I think 30 minutes is five hours in the transplant time warp when you are waiting to find out what happens next.

Hospital bracelet two

My transplant coordinator came out and explained that they thought they had lungs (I actually never knew if it was one or two – I actually forgot to ask!) They needed to take a blood sample and have it sent away. She actually apologized that they’d probably have to repoke me later for more blood. I assured her she could poke me as many times as they needed – no big deal!

I had to wait while all the people that were there for morning clinic got their blood drawn, and then they took mine. I went back to the waiting room, and we waited some more. It felt like hours. Ryan and Sara were there, and I think we all felt like we should be distracting ourselves with conversation, but I was coming down from the initial shock, feeling a bit out of breath, and really didn’t have much to say. Happily one of my friends from rehab who got a transplant last January happened to be there for a follow up visit. We got to chat a while and that couldn’t have been more perfect. Sometimes it isn’t the talking that helps. It’s just being present to talk if needed – to know that someone is there if you start to freak out.

My coordinator came by again and said they were admitting me and we were just waiting for the room to be cleaned. Okie Dokie! We waited some more, and some more.

My coordinator came out again and said they’d like to move us to a conference room. Great – we’re going to get the skinny on these lungs! We waited, and we waited.

The doctor arrived and explained the lungs were not going to work out. When they received the CT scan of the donor lungs, they had what are called infiltrates in them. These could be nothing, or they could be a problem. The lungs were too far away to send someone to inspect them in person, decide whether to accept them, and get back in time. Sadly, we learned the donor was a young person.

Sara and I had been talking about how odd it feels to know something horrible is happening to another family, and yet you can’t help but be grinning ear to ear. I felt very guilty about how happy I felt when I knew they must be in the midst of so much grief. I spent the waiting time thinking about how after this hard part, things would start to get better instead of worse. I started thinking about my list of things I want to do post transplant. That’s how I spent much of the waiting time – daydreaming about effortless breathing.

I told the doctor that I had intended to e-mail my coordinator when I got home from rehab. During the past week I felt much more out of breath and needed even more oxygen. So, just for grins, she decided to order a spirometry test.

To do this, it meant a clinic appointment. Since we weren’t on the schedule, yep, more waiting. When they did the spirometry, my FVC had fallen from 45 on Oct. 19th at my last clinic visit, to 30. That is a huge drop. In the last year, that number has fallen five points. So, this was three times that, in about three weeks. Not good! I also had a slight fever of 99.7. I totally didn’t feel feverish.

So, to be sure there wasn’t anything else going on to explain the drop besides disease progression, they wanted to do a CT scan. I was on board with that! Something was definitely wrong.

Ryan, Sara and I went up to the CT scanning place to see if the wait was going to be long or short. We were hungry, and since I wasn’t having surgery that day, I now had permission to eat. The CT receptionist said they could do my scan right away, but my insurance had not approved it yet. It had only been 15 minutes since we were in clinic, so we went to lunch thinking giving it a little time would give the system a chance to catch up with us.

Think again.

Back from lunch we were told Aetna said it would be two days before they could get the CT authorized. Are you kidding me? This is not a clinic appointment. This is something emergent. So, back down to transplant clinic we went and asked to see the coordinator again. More waiting.

She came out and said that the regular finance person was out, but that there was a note in my file and that this should be approved. She was going to call Aetna herself.

More waiting.

An hour later she came out again and I think was feeling as frustrated as we were. Aetna was telling her that only one person could sign off on this, and that person wasn’t picking up her phone or answering messages. For all we knew, she was out for the day. So, we had to leave the transplant clinic and report to the Emergency Department.

They offered me a wheel chair, and while I usually turn it down, I was getting very tired and the ER was on the other side of the hospital campus. I gladly accepted.

Hospital bracelet three

Thank God for wheel chairs. We had to wait in line to get signed in. I was really starting to feel tired now. The guy in front of us was in handcuffs and with police. While not being physically difficult, he seemed to be having problems with the paperwork. I started to feel impatient. I looked around the room, and I didn’t see people. I saw germs. If I’m doing worse, the last thing I need is to catch something else in the ER. Get me out of the holding area to someplace a little more sheltered from the coughing and sneezing and such.

When we got to the desk finally, they knew who I was and I got whisked right in. Yeah! Now, however, I’m in the care of the ER and not the transplant clinic. There are protocols that get followed when you show up in respiratory distress, even if that is your normal state of being generally. The CT scan now became an EKG, a chest X-ray and a CT with contrast to look for pulmonary embolism, or blood clots in the lungs. Okay. I can roll with this. I was parked on a gurney in a sort-of room, a choice spot over the many, many people on gurneys in the hallway, parked one after the other like a parking lot.

More waiting. Actually, a lot more waiting.

The ER staff, after a while, started apologizing for how long it was taking. I was only annoyed that because my insurance company couldn’t respond to multiple phone calls in a timely manner, the bill for this day was climbing. Not my bill, but still, just an example of why health care is so expensive. We can’t deal with the obvious. I told the ER staff it was okay. Some family was having a much worse day than me, and in the midst of it, they were willing to give me their loved one’s lungs. I couldn’t really be frustrated because I had to wait. It is relative. This wasn’t nearly as big a deal as that.

They came to put the IV in for the CTA, and had a hard time getting it. I’m not sure, but I could have been getting a bit dehydrated. I’m taking meds to thin out all the mucus I now cough up constantly and it wasn’t the kind of day where I was drinking much. Blood started to run out on the gurney and drip on the floor, but they got it in. Whew!

More waiting.

An hour later a guy showed up to do the chest x-ray. Easy peasy.

Later the nurse reappeared to tell me I had to prove I wasn’t pregnant for the scan. I had asked to go to the restroom earlier, but was told I’d have to use a bed pan, which I hate, and the bed pan never arrived. I actually was well stocked for a sample. I asked if that meant I got to go to the restroom. She said I could use a bedside potty, but didn’t know where one was. I assured her I could use the regular one if she’d let me. She decided it was okay, and quickly the oxygen was plugged into my own tank. I didn’t argue, even though I was trying to save my own oxygen to get home again. I was afraid someone else would show up and veto the maneuver. For someone who couldn’t breathe, I scooted across the ER, holding this little gown barely closed in the back with one hand, and pulling the oxygen with the other. Mission accomplished! Sigh of relief. Given the choice of mooning the ER, or having to use a bed pan, modesty lost out.

Finally, they arrived to get me for the CTA (CT with contrast to look for clots). I got whisked past a line of people on gurneys in the CT area hallway. No telling how long they were all going to have to wait.

I was seeing light at the end of the tunnel. Quick scan, and we’re on our way soon. Nope, think again. Now it has been hours since the IV, which wasn’t doing well from the beginning, was put in and it doesn’t want to work. They fiddle with it, and I felt the warmth of the contrast, but the radiologist wasn’t happy with the pictures.

They needed a new IV. I wonder how many hours that is going to take (to myself.) Another guy showed up on the scene to fiddle with my IV again. He worked with it for about five minutes, and presto! The IV whisperer was successful! Scan number two (trying not to think about what the additional radiation and contrast could do to me for attempt number two) and success!

Back to my little ER bay, past the crowd.

The nurse had steroids to put in my IV. Yippee! Within an hour I was breathing better. The trip to the ER might have been worth it just for the steroids.

Discharge was in sight!

I was given a script for antibiotics and steroids and wheeled out the door to the onsite pharmacy. Ryan now was in charge of the wheel chair and pushed me up to the counter. The pharmacist looked at the script (no long line – yippee) and says he can fill that right away. Silly me. I thought right away meant he’d be right back. Ryan didn’t move the chair. Other people came and went from the next window, but we stayed put because he said he’d, “be right back.”

You’d think I would have enough hospital experience by now to know no one comes right back, and just a few minutes is code for a few hours. It is just the way things work. I was so ready to be home that I just sort of didn’t think about that.

Finally I asked Ryan to park me to the side, close enough to stare at the pharmacist, but far enough away to not be in the way of others. Nearly an hour later, we had the drugs. My friend Eden was outside to pick us up. We were on our way home! It was around 9:00 pm.

Everyone that has messaged me in the past few days has been so worried about my disappointment. I think I might have been emotional and disappointed if I was in the hospital and the window of opportunity was getting shorter. I’ve been on the list a year now, so actually, just getting a call was a huge boost to my morale. I’ve known so many people who have been through lung transplants that I know this sort of thing usually happens several times. It is just part of it.

I think in a way this was meant to be. It turns out I had a bit of pneumonia in my lungs and didn’t even realize it. It would have probably become much worse or caused additional problems if I’d just been sent home. We learned later in the day that besides the infiltrates, the lungs were not the right size anyway. I think it was meant to be. I didn’t lose the lungs really, and we got to head off a problem that would have become much worse. God is looking out for me!

Thanks everyone for all the many prayers.


Monday, November 13, 2017

Hundred People Search update

We've added four more people to the HPS Network registry. That puts us at 78 to go on this year's Hundred People Search (HPS). 

Tuesday, November 07, 2017

Hundred People Search update

So, I've got some Hundred People Search backlog to get through. We've added four new people to the HPS registry. This puts us at 82 to go for the Hundred People Search (H.P.S.) Some of you have been doing so great with the outreach! Some of that outreach is happening in Puerto Rico, despite no power and poor communications. You all are amazing! Keep up the great work! 

Thursday, October 26, 2017

Transplant appointment update



It has been a week since my transplant appointment, and I’m just now getting around to blogging about it. I have a ton to do, but am making the time to blog today. Grin.

All and all the appointment went well. I did better distance wise on my six-minute walk, but needed 10 liters of oxygen to do it. Even then I spent most of the walk saturating in the 80s. (They stop the walk if you go below 80 now, which I did last time on eight liters.) In practical life, this means I have to make myself walk slower, or crank up the oxygen to 15 liters, which means the tank is gone in minutes. It also means I’m hugely grateful for the wheel chair I have now. I still try to walk Finley around our block, and the park (about a half mile) several times a day. The route is fairly flat. I really can’t do hills anymore. Usually after about a mile I’m getting tired. I can do more, probably another mile more, but the tradeoff is that I won’t have the energy to do much else for several hours. If all I had to do was be a patient, this would be okay. The reality is I need to keep working as many hours as I can to make the money stretch. This has been a long journey, and the longer it drags out, the more expensive it gets.

Nothing developed that would increase my lung allocation score. I was a bit disappointed only because I feel like I’m getting tired even more easily, if that is even possible. But, as I told the docs, how do you tell how much of that is because of the pulmonary fibrosis (which causes fatigue) or the five medications I take daily that have drowsiness listed as a side effect. I feel like I could sleep most of the day, if I allowed myself to do that. Of course, I don’t. I do try to get 10 hours a night. Eight doesn’t seem to be enough unless I can snag a one or two hour nap during the day.

My schedule has been super busy these past few weeks, and will be super busy for the next few weeks. In November it will be a year that I have been on the transplant list. So, it is like going through the whole evaluation all over again. I have to have a bunch of tests repeated, including the right heart cath. The up side is maybe they’ll find something that would add points to my lung allocation score. The down side is there is always the risk they will find something that could create a problem being on the list. The longer you are sick, the more stuff can come up.

But, there we are. It is what it is and I’m plotting forward as best I can. It isn’t like I have a choice really.


I cracked up at the doc and ribbed him a bit. He asked if I would be at the American Thoracic Society meeting in May this coming year. I answered that it depended on whether he gets me a new set of lungs by then. Then, I asked him if he could speed this up…I have this meeting I really want to attend in March! Ha ha ha ha…..

Thursday, October 12, 2017

Hundred People Search

We have added a new HPSer to the patient registry. This puts us at 86 to go on this year's Hundred People Search (HPS). Keep up the outreach! 

Wednesday, September 27, 2017

Worried for Puerto Rico



Living with a rare and chronic illness is never easy, but try doing it in the face of an epic natural disaster – no water, no power, no fuel – forget easy access to medical care, medical equipment or medications.

My family connection to Puerto Rico is distant. My great grandfather was from the northwest part of the island. He came to the mainland United States to go to college, and eventually became a professor at the University of Oklahoma. He was a linguist. While I inherited an HPS gene from him, sadly I didn’t get the language gene.

Living in Europe, I learned a lot about the history and culture of the other aspects of my heritage, but I never knew much about Puerto Rico. My grandmother would tell us stories about visiting the family plantation when she was a child, but that was about all I knew of the place.

When I was diagnosed with HPS, one of the blessings (instead of curses) that came with the diagnosis was a sudden intimate connection to the island. Suddenly I knew tons of people from Puerto Rico, and they were more than casual acquaintances – they became family! I was also blessed to get to visit the island several times to attend HPS conferences and work on HPS outreach. That was interesting, but it was also very satisfying to travel through parts of the island I knew my great grandfather and his family must have known. It was a connection to learn more about the culture and the food and the feel of the place.

My family here lost connection with the extended family in Puerto Rico when my grandmother’s last cousin (that she kept in touch with for years and years) finally passed away. I’ve always wanted to find extended family there, and make sure they knew about HPS, but I’ve never been able to do it. Every time I’ve attended an HPS event in Puerto Rico, I look at the crowd and wonder what the odds are that someone there is related to me, albeit distantly.

Unlike my many, many, Puerto Rican friends, I am not waiting to hear about the welfare of a relative. Still, waiting for news and watching for updates has had me very anxious this past week.

I worry for anyone in Puerto Rico who might have HPS and be on oxygen. Their oxygen concentrators won’t be working and I have no idea how they will get fresh oxygen tanks delivered anytime soon! I worry about whether anyone with HPS has been injured and may be bleeding and need medical attention. I worry about the health implications for anyone with HPS going forward as the island tries to recover. Are their lungs being exposed to mildew or mold that could affect their long-term prognosis? Are they out in the sun and unable to get the sunscreen that could prevent skin cancers? Are they getting exposed to bacteria in flood waters that could make them sick and further complicate their medical lives?

On the news I’ve heard about people unable to get kidney dialysis or insulin. When you have a chronic health issue, you are so vulnerable when things go wrong. It is scary!

I’ve felt so frustrated by the scant attention given to the plight of Puerto Rico in the media. I know the challenges of a natural disaster on an island are different than those in an area on the mainland. People can’t load up their cars and drive there to bring supplies etc. Still, I can’t help but feel that if this were happening elsewhere in the US, more would be done faster. It is sad that every news story has to emphasize that Puerto Ricans are American citizens. I guess I’m glad they are making the point, but it says something that they have to make it at all. Puerto Rico has the highest per capita participation rate in the Armed Forces. They have fought and died for the US for generations, yet according to surveys, more than half of Americans don’t even know Puerto Ricans are citizens.

I can’t imagine how all this must frustrate my friends with closer ties to the island.



For now, we are trying to figure out what we can do to help those with HPS recover. What needs might they have that are unique, and what is the most effective way to help?

Thursday, September 21, 2017

Health Update:

It has been a rough few weeks here, but could be much worse. I’ve had a cold for a week and a half. Thankfully, (knocking on wood) it hasn’t impacted the amount of oxygen I need, and so far, hasn’t become a respiratory infection. Yet, just having a cold these days makes me so anxious! I don’t have any reserve at this point. One bad infection, and I’m going to be in the hospital until I get lungs, or I don’t. I just don’t have too far to go before I won’t be able to get enough oxygen at home.

So, you might say, I’m paranoid. I’m paranoid about germs and when I get sick, it’s so much more than just having a little cold. Thankfully, only one day of a fever etc. The trouble is I get better and I think I’m recovered, and then it seems to come back.

The medicine the transplant center gave me to help with the cough is helping. I still cough a lot though. I’m so tired all the time because I rarely sleep well. But, with the medicine, my coughing fits aren’t quite as long. I haven’t coughed so hard I’ve thrown up since starting them, so that’s something. I did cough so hard yesterday I pulled a muscle in my neck…whine, whine, whine…..

I get very frustrated because I get tired so easy. I have so much to do, but I can never keep up with it all. I have so many friends I want to stay in better touch with, but by the time I do what I have to do medically every day, and work a few hours, I’m so tired it is hard to call people and just chit chat. It makes me cough more, and it is hard to be very conversational when you are so tired.



I hope everyone can forgive me for not being in touch better.

Wednesday, September 06, 2017

Hundred People Search - H.P.S.

We added another new HPSer to the HPS patient registry. This puts us at 87 to go on the Hundred People Search - H.P.S. Keep up the outreach! 

Tuesday, September 05, 2017

Hundred People Search

We have added a new member to the HPS registry. This puts us at 88 to go on this year's Hundred People Search - H.P.S. 

Friday, August 18, 2017

A Rude Awakening

This past Monday I was on the HPS Conference Planning Committee video conference and my phone rang. It was Inova Fairfax Hospital. Every time I see that number my heart falls into my feet. It isn’t that I don’t want a transplant. It isn’t that I wouldn’t be thrilled to get the call. But, when that call comes, I know we will be embarking on a whole new medical saga and the road can be rocky. Surgery isn’t the only scary thing. Getting a transplant isn’t a cure. It’s trading one lung disease for another in hopes that, at least for a while, you get to be healthy enough to have a somewhat normal life. But, we are not replacing auto parts here. It isn’t a sure thing. It might not work. So, getting that call is exciting, but super scary too.

When you first get listed, you may know it is unlikely to get a call, but still, you are on constant alert for the ring of the phone. Nine months in, life goes on. You do your day to day things and try not to think about it too much. At any moment this call could come that will turn your life upside down, but you sort of adjust into this weird normal until it does.

So, when I saw who it was on the caller ID, I immediately picked up the phone. It was my transplant coordinator and one of the doctors. My stomach did a flip flop. But, it wasn’t the call. It was just as much of a stomach turner though.

They were calling to tell me that the transplant committee had met and discussed my case. They think it is time to list for a single or a double lung transplant, and not just a double. They think my disease progression is speeding up, and by doing this, I might get more chances. There are pros and cons, and whether for me a single or a double would be better, is a debate. A single might not last as long as a double. But, the surgery isn’t as hard on you, and if you manage not to get cancer or develop some other health problem from the anti-rejection drugs, it gives you some options if you should have to go for a second try at a transplant. Can you imagine that? We might have to go through all of this again! No wonder I don’t have any finger nails left!

The call caught me totally off guard. At every transplant appointment we’ve had this discussion about whether it is time to list for a single or a double, and so far, we’ve held to sticking it out for a double. While I’ve had progression, it’s been a kind of creeping progression. The call was jarring both because I had to consider listing for a single, and because it is a sign that I’m not doing as well as I thought.

That might seem crazy to someone else, but it’s my way. The day I was admitted to the hospital with severe bowel disease, a hemoglobin of 6 and ended up having an emergency ileostomy days later, I’d had no idea how ill I really was. Sure, I was sick. No doubt about it. I was chronically anemic from internal bleeding. I was living on Ensure, jello, ramen and baby food because I couldn’t eat. I had lost tons and tons of weight. My stomach cramped a lot. I went to the bathroom 15 to 20 times a day and it was very painful every time. But, I felt I was managing a chronic condition for which there was nothing else to do. I just had to get through it. So, I did. That very day I had taken a midterm exam that I blew out of the water, babysat for one of my professors and tutored football players in English. The idea that the doctor’s office wouldn’t even let me walk across the street to be admitted to the hospital seemed utterly crazy to me that day.

Perhaps it is the same way now. I’ve got things to do. I can’t focus on how bad things are. I have to focus on how to get the job done. Learning that my docs had met about me, and were concerned enough about a changing picture to want to change the plan was just as jarring as the whole one vs. two debate.

It took me 24 hours to process things. I talked to my friends in the HPS community, family etc. I looked through and reread journal articles. Ultimately the decision wasn’t as much about science as it was about faith. What decision would I regret more? What decision would give me the best chance of accomplishing some of the things that make this fight worth doing? How much control over this do I really have anyway? So, I decided to leave it up to God. If the plan is to get two lungs, then I will still get two lungs. But, if that is not the plan, then this makes the way for other plans.

It’s been a tough week to be honest. My anxiety levels have been super high, even after making this decision.