Old lady alternate identity...

I aspired to live as long as my great-grandpa did and figured I could be as healthy as he was, too. Unfortunately it looks like that healthy gene skipped me. Either that, or my body rebelled against our toxic environment. Maybe both.

Anyway, this disease creates a lot of needs. I need items I never imagined buying... at least not within the next forty years. Some of these things include a pulse oximeter, an automatic blood pressure cuff, and a shower stool. I had to purchase larger, stretchier socks to accommodate swollen feet, and replace several other items I already own but no longer work for this body.

I cannot even begin to explain how much it bothered me to buy a shower stool. I knew I needed something. I couldn't risk fainting in the shower and busting my head on the tile. But I felt a shower stool is such an old lady thing. And who wants to spend their money on a shower stool rather than on a book or plant, or something more fun?

I explained this to my PH friend and she told me I needed an old lady alternate identity. She has an old lady alternate identity and it helps her acknowledge that these are likely her old lady years and she "may's well have them." She explained that our old lady alternate identities need these things. And, they have free passes to make requests, wear what they wish, speak freely, ignore people who drive them crazy, and take naps whenever and wherever they please.

As silly as it sounds, having an old lady alternate identity helps. These are my old lady years. "May's well have them." Today I'm wearing gray, brown, and two different shades of blue. No coordination but my old lady alternate identity doesn't care. Now please excuse me. I need a nap...

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...

Sifting

Lately, I've been doing a bit of what I call sifting. I take a bookshelf, a box, or a drawer of my things and sift through it. And I ask myself: Will I use this again before I die? Or, will I read this again before I die? And, will my family want this when I am gone?

There are some items I wonder... maybe. Then I ask myself: If I did have some time left to use, to read, to study, to do, would I do THAT? Most things, I think probably not. And then I think up about one hundred other things I'd rather do with my precious time.

Some are things with little connection to my emotions. I can easily let them go. I ask friends if they want this or that, or pop the items in the goodwill box. After all, if I really wanted to, these are things that can be replaced. But then there are the items that hold more meaning and are harder to let go. It's not so much that particular item, but the memories that belong with it. Or, that I had acquired them with the intent on using them more or doing something with them some day. There are items I spent a lot of time, energy, and money on. And, there are items that somewhat defined me. They are my past, my former self, the old me. The new me has limitations and needs to clear out things and streamline her life in order to function more efficiently and focus.

I have collected some pretty interesting things in the past thirty-something years. And, as we all have different tastes, talents, hobbies, and interests, I can see why some items can only be appreciated by certain people. By sifting through and passing along many of my favorites, I have been able to share these things and make people smile. Many of my friends are happily adopting part of my past. They are pleased I have recognized their interests and the items I've passed on will be used, displayed, and treasured now and long after I'm gone.

A friend expressed her concern. She feared that what I am doing is a sign of giving up. But I assured her, I am not giving up. Believe me. I cannot give up. This sifting is part of accepting what I cannot change and preparing myself for battle. One must see these things as they are... just things. Because of my illness, I cannot be burdened by too many extra things. I admit, I need things. I like things. And, there are items that make me smile. Things are great when you are well. Things are a great distraction when you have time. Things are fun when you have the energy. But, I no longer need distractions. I no longer have the time or energy. So now, most things seem like a burden. Things need to be organized, stored, and cleaned. Unfinished projects lurk in corners and mock me. Wonderfully useful items sit unused. These things become constant reminders of a life I was forced to give up. By releasing these things, I not only give them a chance to bring happiness to someone else, I gain energy to focus on my family and health. I am preparing myself--physically and mentally--to fight the battle before me.

Buzzards

The turkey buzzards have returned. That means Spring is arriving and I should be planning my garden. But that planning isn't happening. Instead I'm overwhelmed with everything associated with this illness. Dreary and cold January disappeared behind a whirlwind of doctor visits and a long hospital stay. Had it been a January in the past, you would have found me, most evenings, curled up on the couch with a cup of hot tea and seed catalogs... dreamily flipping pages, circling descriptions and making notes. By now I would have seed packets in hand, sketching out the garden and planning what to plant where.

That was before the illness. This year is different. Now I'm different.

The medicine, I hear, will make me sensitive to the sun and I will burn quickly. I'm supposed to be careful around soil and be cautious of warm, humid days. Dirt and sweat could contaminate the central line that runs directly into my heart. I cannot bend over easily because I can faint and I cannot lift heavy things. I am also tethered to a continuous source of oxygen. Now getting outside just to plant a few seeds is a monumental task.

This illness is forcing me (and my family) to rethink things, to re-prioritize, and to ultimately give up on some things... even the things I enjoyed doing. Things are different. Remember the spoon theory? What do I really want to spend my spoons on? I have to ask these questions every day, for every task: Is it worth it? Does it make me happy? Do I have a choice? Is there another option? A different way?

A different way?

I plan to plant a few things in the garden but I don't have the energy or time to create something spectacular. It will probably be a bit sparse and more than likely, not as well tended. But, after further thought, the garden doesn't have to be spectacular. That is because just BEING HERE to watch it grow will be spectacular.

Indeed. The turkey buzzards have returned, but Spring will have to be different this year.

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..

Ironic

For the past several years I've been focused on healthy living and have avoided most processed foods, GMOs, and chemical additives. I was doing it to stay healthy and avoid problems with food related maladies like heart disease and type II diabetes. I am not a smoker. Never was. I avoid chemicals like synthetic bug sprays and cleaners in my home. And now I have something so rare and so random that is destroying my lungs. It was not preventable and there is no cure. How ironic.

I am trying to cope with this irony. This disease took away my future plans, my independence, my fast stride, my strength, my savings. It took away my ability to work and the chance for others to depend on me. It keeps me from things I once found pleasurable. And I am holding on, trying to grasp as much extra time modern medicine can provide. But, I'm only hindering the inevitable.

No matter how much they love me, friends and family continue on with their daily lives. They must. And, some are even picking up where I left off, taking over things that were once my responsibility. I'm trying my best to accept all of this, but it's hard. I'm envious. And it's the deepest, most pronounced envy I have ever felt. I feel ashamed and am a bit sheepish admitting this because I know that wanting what others have - health - will make me no better.

This disease brought a lot of things into my life, most of which I did not want. When I can push aside the heavy drapes of anger, of envy, and worry, I can look at the blessings this disease has brought into my life. For one thing, I've grown closer to my mother-in-law. We haven't always agreed on how things should be done, but when I needed her, she was there by my side. I've also observed compassion and appreciate all the help from friends - everything from rides to appointments to surprise goodies at my doorstep. In addition, I have a wonderful husband who continues to show his deepest devotion by helping me daily. And, there's my new friend who has a similar, rare form of this disease.

My friend lives about twenty minutes away and visited me on Friday. I cannot even begin to explain how much that visit meant to me. She's fighting this battle too. We are the same age. We are mothers with a terminal illness. We can openly talk to each other in that "I've been there myself" sort of way. We have a lot in common, obviously. But we also HAD a lot in common. Had we met before this disease struck, we would have been instant friends.

I still have those days--those dark, gloomy days--but I tell myself I must remain focused and strong. I'm fortunate because I can count my blessings... especially blessings that came into my life because of this awful disease.

Sodium in what?

The PVOD has caused right-sided heart failure. And, the right-sided heart failure is causing edema in my lower legs, ankles, and abdomen. I am taking a diuretic, which helps reduce fluid in my body. But, as with any medicine, it creates a viscious cycle. Because of it I have to pee more. And, since it robs my body of potassium I have to take two very large potassium supplement tablets daily. I regularly consume more potassium too... because blood-work reveals I am on the low side.

Anyway, when I mentioned my issues with swelling at my last appointment it was suggested I pay close attention to my liquid intake, in addition to watching my sodium intake. I certainly want to avoid taking additional diuretics if possible so I'm trying. And, I'm proof that someone will not dehydrate on less than 8.6 cups of fluid each day. (And this includes soup, too.)

But this week has been worse. I have cankles. I think it's partly due to my newest "live it up" attitude and my recent additions and exceptions to my once very wholesome and natural diet. Darn these Unreal peanut butter cups.

So, I've been having to keep a closer eye on my sodium intake and have been looking up the sodium content of just about everything. Because of this I've learned some interesting things. For example, one cup of chopped carrots contain 88.3 mg of sodium. That's more sodium than what is in a tablespoon of salted butter. And almost as much sodium as what is found in two Unreal peanut butter cups. One cup grated carrots or two peanut butter cups?

I hobble on over to the kitchen and reach for a peanut butter cup and shrug. Darn carrots.

Let's not tell the nutritionists at Cleveland Clinic about this or they may take the boiled baby carrots off their heart-healthy menu side items.