Three years

I made it three years.

It was almost three years ago I woke up. I had to relearn how to walk and how to swallow.  I have a reminder--a six and a half inch scar from a sternotomy--a cut made down the middle of my chest, from the top of the sternum to the bottom. My heart was stopped and I was put on life support while surgeons removed my diseased lungs and replaced them with donor lungs.

I live each day with the statistics of lung transplantation hanging over my head. Many people tell me to ignore the statistics, but I will be honest with you. It's difficult when I deal with the physical and emotional pain of transplant. I have, since transplant lost friends waiting for transplant and I have lost friends who have received their transplant. How quickly one and two years go by and how many friends have died, either of rejection or infection or some other transplant related complication. Lungs are fragile and they don't last forever. Lungs are exposed to the outside air, pollen, pollution, germs, spores, and can suck up whatever is floating around. By one year doctors expect about twenty percent of us transplantees to die off. By five years, about forty to fifty, and by the tenth year, only about twenty-five percent will be going strong. And, all too often we deal with other health problems because of the effects from the medications we take.

Year three has been full of wonderful memories. I've been able to do much more than the second year, even with my limitations. This past year I've logged several health issues, a couple more surgeries, Gamma Knife radiation, several trips to Cleveland for follow-ups, many blood draws, and more. I keep photos of my donor on my shelves at home and can tell you that I sense that I am a whole lot happier than most people I meet on a daily basis, even on my toughest days.

Celebrating two years

My two-year lungiversary went by with little fanfare, much to my dismay. The only ones to acknowledge it were those within my household. But, that is because I have declared it an official holiday, penciling it on the calendar and announcing it days before. For me it is a second birthday. I'm sure it's a feeling that only fellow transplantees can understand. Silly I suppose. But when cards and greetings didn't roll in, I felt a bit... a bit, well, forgotten. Then I had remind myself that almost dying and getting a transplant was a battle that was how long ago? One year? Great. Two years? Come on. Life goes on. No one wants to have to remember to celebrate ME twice a year. Except for ME.

Thankfully, I had less anxiety this year as I reached the date. I think this is because I now have a relationship with my donor's mother. At this time last year, it bothered me greatly that I didn't have anyone to focus that energy on. I had reached out to the family with a letter through LifeBanc and told them how much I thought of them each and every day, and how much their gift meant to me and my family. I wanted them to know that every single breath I take is because they said yes to organ donation. But, by the time I had my first anniversary I had not heard back.

Eventually, I did hear back and since then, we have exchanged several letters. This communication means so much to me. Even though there are no words powerful enough to express the thanks I feel for this gift, at least his mom knows that this wonderful gift is allowing me to spend more time with my family, and is allowing me to selfishly celebrate two birthdays.

Health-wise I am holding in there. I'm working on getting that room in the ER named after me. Yep, I'm back to my old record of going in once a month with the usual--migraine, vomiting, and dehydration. I don't know why it's happening and wish it would stop. Also, I recently found out the pain in my left ankle I have been walking around with for over two months is tendentious. I did not do anything to it... supposedly the medicines can cause it. I guess I have been getting so used to walking around in pain and not realizing what is normal, or rather the new normal. Basically any time I have had aches and pains the doctors usually tell me the medicines can cause it and then they shrug it off. So with this, I just kept shrugging it off until I couldn't take it any longer. But surprise, now I am hobbling around on a walking boot. I'll see the podiatrist again in a couple of weeks to see what my next step is.

Certainly not the fanfare I was looking for, but I hobbled right along and continued my celebrations throughout the weekend. I look forward to celebrating my real birthday soon enough... and perhaps I won't be hobbling around for that one.

Five months

That injection I mentioned last month? I've been having to do them weekly. And my body does not like them at all. The first one put me in the ER. It triggered one of the worst migraines I've ever had. I was crying, writhing in pain, and begging for relief. Of course it came with nauseousness, vomiting, vomiting, and more vomiting. Did I mention the vomiting? I tried to self medicate but the migraine and vomiting got worse and worse until I could do no more for myself. Again, I was hydrated and medicated at the hospital and sent home.

A few days after that ER visit was one of my routine treks to Cleveland. I had the usual tests--labwork, x-rays, spirometry--and doctor visits. I also had a Bravo prob inserted in my esophagus since the Ph probe testing failed at the last visit. I had had a migraine all day and after the sedation wore off it felt like I woke up with a jolt. A jolt from the gut to be exact. I was in such pain and was vomiting. (Nothing new there, eh?) They rushed me to the ER at the clinic where they treated me with what they called a migraine cocktail. That was the day I had my first ambulance ride ever. Ironic, isn't it? Even though I was at the Clinic, the place is so huge that taking the ambulance was faster.

So that brings us to five months post transplant. Five months! I hate to complain knowing full well what the alternative is. But truly, it has not been the easiest thing. The road in these woods is not the smoothest. In fact, it feels more like an uneven goat path than a road. In the four months I have been home I've had three return trips to the clinic, two of which included bronchoscopies, an endoscopy (the Bravo probe results were fine), and a trip to the ER; two additional trips to the ER at a local hospital; routine bloodwork about every week; a follow-up with our family practitioner; several injections; and a visit to the gynecologist, eye doctor, and dentist (like any compliant patient should do post-transplant). Considering all of that and the time it takes to organize my pill box (and the orders), and filling out my daily health log that consists of vitals--blood pressure, heart rate, weight, temperature--and spirometry readings, I spend a lot of time trying to keep these new lungs safe. And every poke, prod, and pain reminds me that I'm living on borrowed time. I admit... I get weary. I'm always plagued with side effects from the medicines and I deal with the anxiety that comes with living a life of impermanence.

I recently completed my letter for the donor family and will take it with me to my next appointment. (It will go through a third-party system until both parties have agreed upon open communication.) It took me several tries to finish it. This is actually my third version, totally revised and edited down to ten sentences. In ten sentences I expressed my gratitude for their gift. And, I clearly spelled out what I hope for... for them to contact me. Initially I wrote long letters. And then I read them and re-read them. I moved paragraphs around and changed words. I deleted stuff and added stuff and cried and re-read and started anew. How do I express a lifetime worth of thanks? In ten sentences. Perhaps less is truly more because honestly, I cannot find a word more meaningful than thanks when I have received such a remarkable gift.

Five months. The surprises, both good... and not so good, continue. I'll check in here once I get a little further down the road. Meanwhile I will continue to do my best and will keep following the goat...

The List

I'm part of a support group of over 1,400 people who are in different stages of lung transplantation. While a handful are caregivers or spouses, most are either waiting for lungs or have had a lung transplant. Last night, after spending some time on the support group page, I realized that in my last post, I may have made it sound like transplantation was an easy decision. I want to clarify... it isn't. It's terrifying. But, when the other option is death, it makes it seem easier. I also want to point out how fortunate I am to have the opportunity to make that decision because it's not offered to everyone who needs a transplant. There are plenty of people on the support group who are struggling to get listed.

Getting on the list is a chore and a privilege. Organs are in great need and approximately eighteen people on the list die each day waiting. These are people who were chosen to be good candidates and have completed all of the tests required to be able to be listed. That doesn't include the people who have been turned down, or are struggling to get listed by changing their lifestyle, their habits, trying to gain weight or lose weight, fix dental problems, etc.

Each transplant center is different. I can only tell you my experience, based on my doctors and my center. My center won't list one until they are close to death. But, they also know they have to list one before they are too ill. They have gotten pretty good at determining this window of opportunity. And, this is my window of opportunity. I'm sick enough to be listed but well enough to survive surgery.

Knowing I'd need a transplant, we started with the prerequisite checklist at a slow pace and worked our way through it... ticking off each test or requirement. One by one--lots and lots of blood-work; regular pulmonary testing; dental clearance; allergy testing; tissue typing; updated vaccines including the entire Hepatitis A and B series; updated and current annual exams; bone density scans; heart cath; and tests like gastric emptying, which involved a series of scans after eating radioactive eggs. (Ick!) They also require a psychological evaluation. The tests are daunting and the older one is, the more tests seem to be required. And, if, like in my case with allergies, if one test has some irregularities, there are additional tests scheduled to rule out other things. And, of course, the checklist includes approval from the insurance or some other guarantee that they will be getting money for all of this.

Once these requirements are met, one's case is discussed by all the doctors, the surgeons, and the social worker. At that point, if it is determined one will be a good candidate, a Lung Allocation Score is assigned and one's information is sent in for the list. All of one's information is entered into a database so that when an organ becomes available, the database is searched for the best matches.

Matches are based on several things--blood type, antigens, antibodies, proximity to regional center, body size and more. There is no guarantee. For some individuals, matches are harder to find. Even if one is first in line for an organ at their center or region, if the organ doesn't match well, it will be offered to the next in line... someone who is a better match. It's a system that assures people will get the best new start with a recycled organ.

It all seems complex, but it's a system that works as well as it can with a shortage of organs. It doesn't have to be this way. If more people were aware of the impact of organ donation, those eighteen people would not be dying each day. Instead, they would be given an opportunity to live longer, to be with their children, their grandchildren, their spouses. A mother and father would cry with happiness, friends would rejoice, and a community would be overjoyed. Someone, someone like me, would breathe again. She would be able to be a mom and wife again. She would be most grateful to her donor for that final gift of love... the last gift any person can give.

Two roads diverged in a yellow wood...

Two roads diverged in a yellow wood and neither will lead me out. One goes straight to the cemetery... so I am taking the other one. I will journey on as long as I can and hope for a successful surgery and a speedy recovery. Transplantation is not a cure. But if all goes well enough, it will give me the opportunity to live for several more years.

A few more years in these woods.

In an earlier post I mentioned the amazingly talented Charity Sunshine Tillemann Dick, who has had two double lung transplants. After her first double lung transplant she did everything asked of her. She took her medicines on time, avoided crowded spaces, and did everything suggested to reduce her risks of rejection and infection. But, when she went for a regular checkup, expecting a clean bill of health, she learned her body was rejecting her new lungs. She was angry. "I feel like I've done my time," she wrote in a blog post, "and I was looking forward to doing some more pleasant time in the the coming years."

Charity explained that even though we do something well or we get through something challenging, it doesn't mean the work ends or that there won't be other challenges. She continued, "While I might not be "out of the woods," there is more beauty and love inside of them than I could have ever anticipated." Charity is right. Even though the road has been pretty rough so far, I have learned so much and have met some wonderful people. And, since I will never be out of these proverbial woods, I won't mind setting up my abode here for the rest of my life. I'll have plenty of visitors and some great neighbors.

Let's talk about organ donation.

As of right now, according to the OPTN's transplant information database there are 118,646 people waiting for organs. There are 1,662 people waiting for lungs. And, I'm not even actively listed yet. (I'm almost done with preliminary testing. I'm about 90% of the way through the checklist.)

Today approximately 18 people will die waiting for a transplant.

If you haven't, I encourage you to register to be an organ donor. And, don't stop there. Discuss it with your family--children and parents--and anyone close to you. Make sure they know what you want them to do and encourage them to carry out your wishes. There are times a donor's wishes are not carried out by the family because they are too distraught at the time of death, or they just didn't know what their loved one wanted.

The Sarah Murnaghan case has brought a lot of attention, both good and bad, to organ donation. It's all over the news and a lot of people have voiced their opinions. I heard a story recently about a mother who went with her 16 year old son to get his drivers license. She wouldn't let them mark organ donor on the card because she didn't want to think about her child ever dying.

No one likes to think about it but it happens. But maybe we should think about it. Maybe it would make people get up in the morning singing praises rather than grumbling. Maybe people would be more kind on the road because they want fewer accidents. Maybe we'd have more terminally ill patients being able to make amends at the end of their life...

Maybe.

Talking about death in this society has become somewhat of a taboo. I've discovered a lot of people prefer not to talk about. Some ignore it. They fear discussing it will somehow summon or hasten death. I've heard stories of families who avoided talking about a family member's illness. Somehow they went on believing everything would work out and avoided telling the kids until the last moment. And then, when death came, everyone was bitter because they didn't make those last days count. They didn't prepare. They didn't know what the deceased would have liked.

They didn't talk about it.

Talk about it NOW because we will all face death. We just don't know when our time will be up. Tomorrow is not guaranteed. Someone, somewhere, right now, is faced with a loss and a decision that could bring hope to so many families. One deceased donor can save up to eight lives through organ donation. They can also provide up to 100 people with corneas, skin, bones, veins, tendons, ligaments, and more through tissue donation.

In just the time it took to write this blog post, eight people disappeared off the transplant list, three of them were waiting for lungs. Did they die or did they get their lungs? Did someone, dealing with a devastating loss, say yes or no?

A system...

I think everyone has heard about the ten year old girl, Sarah Murnaghan, in Pennsylvania, who needs a lung transplant. Yesterday a judge ordered the Organ Procurement and Transplantation Network System to suspend their under-12 rule for Sarah.

This case has brought a lot of attention to organ donation and how organs are distributed. And over the past few days I've seen numerous comments from people who are not very well educated about the subject of organ donation.

This needs to change.

I need a double-lung transplant and I am learning much more than most anyone needs to know. I don't know what the ramifications of this case will be concerning the future of organ transplantation. But I do know that the system was overhauled in 2005 to increase the effectiveness of allocating organs and I'm told it has been successful at procuring more organs and reducing the amount of people dying waiting for organs. Perhaps this will be a good time for the organization to re-evaluate things and make sure the system is still running smoothly.

Perhaps this is also a good time to talk about an underlying issue here. And that is, despite children under twelve dying every day who could potentially be donors, their families are not agreeing to organ donation. Only 20 deceased lung donors in 2012 were under the age of 10.

Statistically no child should die waiting for an organ.

Lung Recipients

It's all very surreal to think that one day I will be receiving lungs from another person--a person who has died and has chosen to give me another chance at life in their absence.

Doctors assure me the surgeries usually go relatively well. It's the rejection and potential complications that cause concern. I have blogged about the statistics before. I have approximately a 50 percent chance of making it to five years on a lung transplant. I have a chance. I have hope. And, thanks to the power of the internet, I'm discovering inspiring stories of lung recipients who are on their 3rd, 17th, and 21st year. 

What some of these lung recipients are doing with their new lungs is amazing. There's Rowan Jimenez who got back into his music, biking, and rock-climbing; Alex Parker, a Jazz singer who wrote a song called Breathe In for the Ontario Lung Association; Charity Sunshine Tillemann Dick, who continues to wow audiences with her amazing singing and spirit; and Hélène Cambell who started Give2Live, a program to help transplant patients at the Toronto General Hospital.

Most of all, they have embraced life. They are powerful because they truly know what it is like to face death. And now that they can breathe, they are going to share with the world.

A healing heart...

I returned Thursday night after two days of testing at the Cleveland Clinic. I'm still gong through the necessary steps for the lung transplant listing, and had several tests and blood-work done. The poking was never-ending. Every time a nurse, therapist, or doctor turned the corner, she/he was ready with some form of needle. I had blood draws from both arms, a blood draw from the wrist, a small series of injections on my upper arm for allergy testing, and the Hepatitis A and B vaccine in the other upper arm. I can be thankful though that everyone I encountered was pretty good with their aim. And, I got my favorite phlebotomist for the main blood-draw that required several vials. My husband saw her in action and commented later that it was like watching a magic trick. One minute she had the needle in her hand and the next it was in my arm and blood was coming down into the tube. This girl is good.

The two different blood draws at the same time is something that was implemented after an error in blood typing took place at Duke University about ten years ago. So now, in order to double-check and confirm a recipient's blood type, they have two different people draw one's blood. Two people armed with needles and two different pokes. Thanks a lot Duke University. I've had my blood typing done a couple times before and I am happy to report it has not changed. I'm still the same type I was a couple months ago... and the same type I was years ago. No surprise there. So, there should be no mistakes in that department, right?

The heart can be a resilient organ. And, if healthy otherwise, and treated well, it can bounce back from stress and trauma. And my heart, I'm happy to announce, showed a tiny bit of improvement. And, even though it was a tiny bit, I was happy. I have been told that, in my condition, as long as things are not getting worse, it's good. The heart is still working hard, still has a bit of fluid around it, and the the right side is still dilated. But testing reveals it's not working as hard as it was.

The diseased lungs appear to have responded well enough to the Flolan treatment. The Flolan dilates blood vessels and is very risky. It's an extremely powerful drug that is administered through IV tubing that runs to a port in my chest--a direct line to my heart. It is dispensed through a pump that I wear around my waist and the medicine cartridge must be kept cold--sandwiched between ice packs--at all times.

But drugs are stupid. Flolan doesn't just dilate blood vessels in the lungs. It dilates blood vessels everywhere. So, some of the side effects I have to live with include leg pain, jaw pain, and headaches. But, it's keeping me stable and that's the important thing.

I am still at a fairly low dose of Flolan, so we are going to try raising it again each week in very small increments now that my body has had some time to get used to it. When we attempted to raise it after returning from the hospital I didn't do well. The side effects overwhelmed me and my blood pressure dropped significantly. I was miserable for days. The doctor had me drop back down to the previous dose and I felt better. It's not unusual to feel awful the first couple days after increasing Flolan, but side effects should always return to a tolerable level and one should not experience a significant drop in oxygen saturation, blood pressure, or experience trouble breathing.

The lungs are not so resilient and a transplant is in my future. We know that things can change at any time. So, the social worker says it's time to think about fund-raising because we will need help. The transplant surgery is extremely expensive, even with insurance. And, the medications needed afterwards are expensive. There are other costs, too. There's the hospital stay, the after transplant care, lodging, travel expenses, food, etc. It feels uncomfortable to put this need out there but we cannot get through this alone.

There are non-profit organizations that hold donated funds for transplant patients and the social worker gave us information for a couple organizations to consider. I struggle with this. I never intended to be a financial burden on my family. We had always hoped to pay off the house and the student loans and help our daughter get through college when the time comes. We've always lived within our means and budgeted trips and larger purchases, avoiding the all powerful credit card trap. But this illness has ruined all of that planning. We now owe thousands in medical care. We are still paying on medical care I received last year. And, the bills continue to roll in, adding to the ever mounting debt and stress. So, even though I struggle with this, I know I must, for the sake of my family, be open to this type of help.

Speaking of help... April is National Donate Life Month. If you want to learn more about organ and tissue donation, visit the Donate Life website. Donate Life is also on Facebook.

What's this PVOD thing again?

A lot of people have been asking specific questions about PVOD, or Pulmonary Veno Occlusive Disease. So, I'm going to do my best at explaining my version of what happens with PVOD.

PVOD is a rare condition that causes Pulmonary Hypertension and symptoms can include dyspnea, dizziness (and fainting), fatigue, edema, and a dry cough. In PVOD the small pulmonary veins in the lungs are affected. Pulmonary Hypertension should not to be confused with Hypertension, or High Blood Pressure. They are two different monsters and are very different.

In PVOD, I imagine the lungs as a tree with big branches and small branches coming off the big branch... and even smaller ones coming out from the small branches. Then I imagine someone snapping off those smaller branches until there is nothing left but the bigger branches. Basically, that has happened to my lungs. PVOD has destroyed all those small branches. And unfortunately, those branches are not expected to regenerate.

No one knows why a person gets PVOD. There are so few of us with PVOD they cannot make any connections. Researchers have a general idea of what we've been exposed to and there are suggestions as to what it could be linked to, but the statistics are just not there. In fact, if I've done my math correctly, a total of 32-63 people are diagnosed with PVOD in the United States each year. Compare that to over 232,000 cases of breast cancer diagnosed each year in the United States. Chances of being diagnosed with PVOD any given year is approximately 1 in six-million. I have a greater risk of being struck by lightning: 1 in 700,000, in any given year, in the United States.

I wonder if I should I buy a lottery ticket.

Anyway, there is no cure for PVOD. It is somewhat similar to stage 4 cancer. I know it's going to take me down, but I just don't know exactly when. And, at this point, treatment options are extremely limited.

A double-lung transplant is an option for some. But, it's not a cure. Double-lung transplants are risky and the recipient has about a 50% chance of surviving into the 5th year on the new lungs. As doctors explain, when a person has a double-lung transplant, they give up one problem for another.

The current treatment for PVOD is to use a variety of medications that dilate the blood vessels and target the symptoms of the PVOD. Other medications are prescribed to help counter pain and side effects of the drugs. It's a vicious cycle. This treatment can include the use of oxygen and additional heart medications when the heart is exhibiting signs of failure. None of these medications cure PVOD. Medication may keep a patient stable or make them a little stronger. But, medications can also do nothing, and in some cases, make things worse.

There is mention of stem cell research eventually providing treatment for Pulmonary Hypertension. Researchers are hoping to find something that will regress the disease. And, perhaps they will find something eventually but it may take many years to research and test, and then gain approval for such treatment. More than likely it won't happen in my lifetime.

I'm thinking a lottery ticket is sounding pretty promising after all. Perhaps it would increase the odds of being able to pay off my medical bills...

Lung Transplantation

The first human lung transplant took place in 1963 and the recipient of the transplant lived only eighteen days.   For the fifteen years that followed there were multiple attempts at performing lung transplants and they failed because of rejection and healing difficulties. However, in the 1980s, a powerful immunosuppressant called cyclosporin, was introduced and other techniques to aid in healing were devised. This enabled the first successful single lung transplant to take place in 1986 at the University of Toronto.

What took twenty-three years to devise has evolved into a procedure that is done about 1,800 times annually in the United States. Statistics of survival rates up to the third year vary depending on the transplant center. For example, the two closest centers to me are the Cleveland Clinic and University of Michigan. The University of Michigan performs about twenty to forty lung transplants a year and have a 90-percent survival rate for the first year while the Cleveland Clinic did 108 lung transplants in 2011 and have a little over an 80-percent survival rate for the first year. However, the Cleveland Clinic is considered an "aggressive, high-risk center" and will take patients over the age of 65, while the University of Michigan will not. So, these conditions must be considered when looking at the numbers.

The median survival rate for double-lung recipients is 6.6 years. Approximately six and a half years. Yes, there are double-lung recipients who live ten years, and some live longer, but it's rare. "Nationwide, only a third of patients live 10 years."

A little over two years is what singer Charity Sunshine Tillemann-Dick got out of her first set of donated lungs. She was on intravenous medicines as early as 2006 for idiopathic pulmonary hypertension and received a double-lung transplant at the Cleveland Clinic in September 2009. By November 2011 she was bed-ridden with an infection and by late December she was back at the Cleveland Clinic. She slipped in and out of consciousness until January 24th, when she was matched with her second set of donated lungs. A year after her first transplant she told her compelling story of being a double-lung recipient on TED talk. She exhibits such a strong spirit and I hope the new lungs work well for her..