The Taste of Air

by Richard Schad

  I should have put both my hands up in the air. I was being robbed of my innocence by LAM and lung disease.

  We sat in silence after the diagnosis as several eons passed. I’ll never forget this moment; it’s time-stamped in my memory. Neither of us said a word. Neither of us had to. I looked at my husband and for a moment, I was drawn into his soul. I could see everything; the depth of his sorrow, his anger, his despair. I listened to the doctor in a daze of shocked misery. I had gone to a dark place on a moonless night in some creepy horror movie, and Richard and I are parked on a secluded rood, and then I hear a twig branch break. LAM.

  God, life needs a fast-forward button, because there are days and moments you just don’t want to have to live through, not again, but they keep coming around and I am powerless to stop time or speed it up or do anything to keep from having to face it. How can I continue to put one foot in front of the other when my breath is being taken away? How can I smile and talk and just be, like I am the old me, like nothing has changed? But it all has. Ten years. How do I measure my life in time? Do I now measure it in days, weeks, months, years?

  When I first heard the diagnosis, I wanted to know how long I have, how much of a life I still have ahead of me. When a limit was placed on my life, when all of a sudden life seemed more urgent, I just wanted to know how long. How long do I have? How long?

  I was the 200th woman in the world diagnosed with LAM. My husband was right. I am one of a kind.

  What the heck was this stupid LAM? This LAM; it was like trying to picture a color in a Crayola Box I had never ever seen. Darn it, I don’t won’t to be a case.

  Was a lung transplant, my only hope? C’mon. I believed I would never get to the point of needing a double lung transplant, but by 2001, I went through evaluation for transplant, knowing it was not going to be necessary. But I was proven wrong.

  My ten years are almost up. Now I can barely breathe and I sleep all the time. I have been active on the transplant list at the University of Colorado hospital for almost two and a half years, with no calls, and I am waiting and waiting and I am dying. I have become my own shadow.

  Accepting the death sentence of a lung disease is like falling down a flight of stairs in slow motion—I take it one bruise at a time, a blow, a landing, another short descent. I am not able to run or walk. Bump. I am not able to play or walk with my granddaughter or my dogs. Ouch. Not being able. Black and blue. Thinking about what is gone is the pain. I muttered to myself, “Nothing,” which was odd, because it was everything.

  After diagnosis, I would move for months as if I was underwater. Which is scarier? What is imagined or what is real? Oh my God, how am I going to get through this? It was like being in that horror nightmare and thinking I will wake up, and everything will be fine, but it wasn’t a nightmare, and I did not wake up. Shock, panic, paralysis. I cried and cried and cried some more. I had mini-breakdowns over the next several months where I curled up in bed for days-on-end and retreated from the realities of my world. I was trying to outrun the heartache that was chasing me down. If only. If only. The regrets vibrated to the rhythm of my pounding heart. If only, an anchoring beat. The chorus drummed in my head over and over again, an anchoring beat for the chirping fears and incessant what-ifs that flew through my head like scattered birdsong. Finally, I focused on what I could do, not what I could not do. You know what they say about hitting yourself over the head with a hammer—it feels so good when you stop. I can’t exist sitting in my house on my butt feeling sorry for myself. For the first time in a very long, long time, the LAM was not everything.

  I had gone to a very dark place, and it took a while for my eyes to adjust, but then I saw things, things I never saw before. This isn’t what my life is supposed to be about or is it? We all know death is coming, and we all have the chance to make the most of our time; to choose how we spend our days. There is a limit to our days, it’s not a secret.

  I learned eventually that there is no answer to how long—those predictions and prognoses do not mean everything. The doctors, “they” do not know for sure when my “use by date” will come. Time’s surface is slick as oil, and there is just no way, no matter how hard I try, to hold onto it. What’s most important is my living, not the preparing to die. I remind myself of that each and every day, of the living, no matter how much time I have left.

  When you are faced with a serious illness, you can let it take over your life, or you can learn to co-exist with it, to make peace with how things are. There is nothing you can do about a situation that is intolerable but tolerate it. I have this horrible dream that I am doing dishes and the phone rings, and my husband answers it, and hands the phone to me. It’s for you, it’s LAM. Maybe, I should have said, I’m busy with life, don’t call back.

  Having LAM made me feel at first like I was a failure, and I was not used to failing at anything. When I finally admitted defeat, and outed myself as a LAMMIE, and put on my oxygen, I felt like I had won. Denial is a wonderful thing, for a short time, but I can’t stay there too long. Denial is not part of my future. Let me be clear diary—acceptance of LAM and liking it are different: I do not have to like it. I do not like it.

  Normal is gone. It’s out the window along with the air. There is no normal after diagnosis. Perhaps there never was. Diary, this does not mean I am abnormal; no one with LAM or a lung disease is abnormal. But in my new normal, I am not giving up. I am just trying to accept something I have to accept.

  I felt like I was being betrayed by my body. I am not LAM but I have become my LAM. It owns me, defines me. What stage? Are you desaturating? Pre—or Post Transplant-? How many pneumos? What’s your FEV1? The dreaded How Longs?

  I wanted a life busy with things other than LAM, but I am on the LAM merry-go-round. I have learned about a new world full of oxygen and pills and PFT tests, not to mention a whole new vocabulary of LAM Speak.

  I know my LAM is here. The holes are in my lungs and the LAM continues to spread. I know the LAM is taking away part of me each day. I know that the ticking-down of LAM is a death sentence, for a crime I did not commit.

  LAM is a specter that lives in my mind just as the cells live in my lungs and lay claim there. The clock is tolling midnight.

  Women, like me, who have LAM can champion the cause and talk of what could be. Women who do not have the disease can champion the cause and talk of what should be. All of us can champion the cause and tell of what must be.

  I’ll write soon,

  Karyn

  LAM—A Breath-Taking Challenge

  Both Sides

  LAM, or lymphangioleiomyomatosis, is a progressive and frequently fatal lung disease, that affects only women, usually in their child-bearing years. Lymphangioleiomyatosis is pronounced limf-an-gee-o-ly-o-my-o-ma-to-sis. “Lymph” refers to the lymph vessels and “angio” refers to the blood vessels. “Leiomy” means smooth muscle, “oma” is a tumor, and “tosis” refers to a disease condition. LAM, the gentle sounding acronym, sounds so nice like Mary has a little. But it is anything but little.

  More than 1500 women with LAM have now been identified (which is virtually nil in the world of disease statistics)—consider then each week about 1500 people die from Alzheimer’s and each day 1500 people die from cancer. Scientists do estimate that approximately 250,000 women with LAM are going misdiagnosed or undiagnosed. LAM is rare affecting only just five women in every million people.

  The diagnosis of LAM can be difficult because many of the early symptoms are similar to those of other lung diseases, such as asthma, emphysema, or bronchitis.

  A normal lung is solid, while in a LAM lung, there are air cysts (“blebs”) or fluid filled cysts, little tiny holes, so the lung looks like Swiss cheese. More and more cysts invade, growing uncontrollably, filling up the lungs until there is no more healthy tissue. The LAM cells destroy the delicate architecture of the lung tissue and replace it with m
ore and more cysts. The cysts and clusters grow throughout the lung and begin to block and choke off airways. LAM prevents air from moving freely out of the lungs, preventing adequate amounts of oxygen from reaching the body’s organs. LAM’s abnormal smooth muscle growth slowly smothers the lungs and closes off the respiratory passages.

  Most commonly, women with LAM will experience shortness of breath after physical exertion, and over time, women may find that they have trouble breathing even at rest. Other symptoms include a bad cough and chest pain, which can be a sign of a collapsed lung (pneumothorax), which a majority of women with LAM will experience, one or more times, in one or both lungs. Karyn’s lung collapsed twice. A lung is at risk of collapsing when air escapes or leaks into the chest cavity. This build-up of air creates pressure, which in turn causes the lung to collapse, preventing it from filling up with air properly. Beyond making it difficult to breathe, the collapse can cause the heart and major blood vessels to be pushed to the unaffected part of the chest, which can dangerously lower blood pressure levels.

  Women with LAM are at risk of lymph fluid leaking into their chests, and enlarged lymph nodes. In addition, because LAM surfaces mostly in women, doctors think that estrogen plays a role, and in some cases, women are advised not to become pregnant. Karyn’s second lung collapse came at the same time she became pregnant.

  LAM women are also at risk of developing benign kidney tumors (angiomyolipomas). Karyn had a benign tumor removed from her kidney in 1991.

  The rate of progression and the incidence of resulting symptoms can and does vary considerably among patients.

  All races are affected and women with LAM have been identified in over sixty countries. The average age of women at the time of diagnosis is approximately forty-eight, though, at the time of diagnosis, the reported age varies from six to seventy-five years of age. (Karyn was 49). Also, most women with LAM have had symptoms for three to five years before ultimately being diagnosed. (Karyn’s first lung collapse was in 1980).

  LAM is a progressive disease that at this time cannot be cured, is caused by a genetic mutation, sometimes inherited, sometimes random, which eventually consumes the lungs. Lung transplantation is often considered as a last resort. While many women with LAM add several years to their lives through transplantation, it is not a cure. Karyn was transplanted May 17, 2009.

  Dear Diary,

  With LAM, my lungs plain suck at being lungs. Breathing with LAM is like trying to pull air through swollen tubes. One day I was fine. Then I was sucking air through a straw and now . . . Have you ever tried sucking air through a cocktail straw? LAM is not like cancer with stages marking the decline. But if it was, the fourth stage has left the depot.

  Karyn

  January 7, 2009

  Am I My Genes? LAM Came Uninvited, Unwanted

  This Side

  Dear Diary,

  You just take some things for granted, like the love of your husband and your children, the sun rising tomorrow, and the breathing of fresh air from your lungs.

  Once I crossed over into LAM world in 1999, I became a stranger in a strange world. Nothing, not anything, no longer, is taken for granted. What to expect, what to hope for, what to fear—none of those are clear. I knew the day LAM came in uninvited and unwanted, my world was going to change. I had crossed a line and there was no going back. Life back on Planet Earth will never ever be the same.

  I remember one day walking, weighted down by an anchor masquerading as an oxygen tank, and kids chirping at me, that I shouldn’t have smoked. I wanted to yell back, smoking never caused this. Smoking in no way causes LAM, but, rather than argue with those pointing fingers at me, I would point back and say, rather calmly and sternly, “And don’t you start!” There can be some good from the bad LAM.

  I remember how the simplest task became difficult and complicated. For us Lammies, we become no longer self-sufficient. I can no longer do whatever I want, and how I want. My arms and legs are missing that little oomph that will put me on my feet. I sit back down, gather my strength, and go for it again. Scary because I know sooner or later, I may not be able to make that second try. I am becoming a turtle on its back. I am becoming inert, as unmoving as a portrait.

  I cannot change reality. My illness is here to stay. My pills and medications are here to stay. My lungs will fight me every day. Cultivating well-being is an ongoing process of learning to accept my limitations. I am tired of being tired.

  Stairs used to lead to somewhere interesting, or important. Now, the stairs go nowhere. Slight inclines have become hills. I climb stairs and my chest moves up and down as if I have climbed a Fourteener. I think twice, three times, before going outside to retrieve the letters from the mail box. Groceries are no longer carried. My LAM belongs to me alone and separates me from the people, the healthy ones, who do not even notice they are healthy.

  When you have LAM, you should get a pass on life’s other problems, but, of course, it does not work that way. Life goes on and I must participate. The same old problems exist, along with new ones I could never imagine. And the problems from the past just do not go away. It’s hard to sort through it all, but I really have no choice. Maybe having LAM means you just have to work harder to get things done.

  I remember back to the spring of 2008 when I was no longer able to take showers, another little loss of self-sufficiency and control. A loss of freedom that the LAM has caused and I hated it. It scared me also, how far will IT go? How much of my strength will the LAM steal? I know women who could not get off a couch. Will I end up bed-ridden?

  So it gets harder and harder to do the same things. And I would say, “I think I can do it, work a little harder to do them, and make the LAM work harder to try and stop me.” Like the late night commercials, “But, wait there’s more.” The worst? When I realized I could no longer care for my granddaughter, Avah. All I could do was place her on the floor surrounded by her toys. I could no longer pick her up and hold her. Her little arms would reach up towards me. And all my lungs could do was cry.

  My world had shrunk to the size of my withered lungs. The only thing expanding in my life was my medical file, which looked like the Los Angeles telephone directory. In my shrunken life, all I could do was sit, eat, and sleep.

  If you were not there at the bad moments, if you were not there to agonize over another sign of deterioration, then you could not see how I had changed, but I could. You get used to a stage of illness and then suddenly it gets worse and your world shrinks further.

  Everyone needs to feel that they have some control over their own life. We all do. I hate feeling helpless; getting sicker. My lungs feel like a sack of cement. I hate that I am suffocating. Please someone cauterize the ache of loss. LAM turns off the lights, one by one, and it was getting darker.

  LAM turned my world upside down, shook it hard, and then looked to see what was left. LAM came knocking, I didn’t want it, but it barged in, uninvited, unwelcome. I wish I could make the disease go away; maybe I could chase LAM into a corner, push it off a cliff a long distance away, or just get it out of my consciousness and allow me something else to think about, but I never forget that I have LAM. I cannot feel them but I know there are holes in my lungs.

  It is easier to have LAM than to pretend I do not have LAM. When I admitted defeat, I felt like I had won. Denial is not shoes I walk in for long—life is way too short to spend a lot of time there.

  LAM is a part of my life now. I never forget that I have LAM. It will always be a part of me. It’s a part of who I am. LAM is only a small part of who and what I am; never the sum total. I can live with that. My LAM has humbled me. I have learned so much, been given so much; to forget that I have LAM would be to forget part of who I am. I am a LAMMIE.

  I am not my LAM but none of us are who we are before we got sick and sometimes that’s so hard for me to accept since it is not a change I welcomed. LAM may b
e another adjective to describe me, but it is not who I am. It is one of the things I have, but it is not who I am. It is not who I am. It is not. LAM’s trying to kill me. I’m trying to stop it from doing that. Most days that seems to be enough to say about LAM. The world is full of far more interesting things.

  Don’t they always say in movies you should never show fear? I do not want the LAM to know that I am worried about it. I want the LAM to be worried about what I am going to do to it; for all that the disease has done to me. I hope that LAM lies awake at night worrying about what I am going to do to it next. That only seems fair.

  I know LAM is there. I know it is taking away part of me each day. Some days, I want it just to go away. Some days, I want it just to be quiet. But LAM is never quiet, always trying to find a place to cause trouble.

  I want to scare LAM like a vampire caught out at sunrise running like hell toward the safety of a hidden coffin in a windowless basement. LAM, I hope you are getting tired and frustrated. I hope LAM is thinking about all the bad things it has done, because I am going to make it work to get me.

  Lung disease can be unrelenting. Boxers get breaks between rounds. Football players catch their breath in a huddle. A few seconds can make all the difference in the world, between victory and defeat. I just need a timeout, but there are no timeouts with LAM.

  Diary, it’s not the pain. It’s not the fear, the uncertainty, or the anger. It’s the stress that’s the toughest to deal with sometimes. I can take the medication for the pain or discomfort. I talk to my friends about the fear; I remind myself I am not alone. When the anger flares, I remind myself that there is no one to be angry at. It doesn’t mean that those feelings are not valid and oh so strong; they are, but what they add up to is stress—the pressure of coping with everything that LAM brings with it can feel like a vise, and sadly, there’s not an easy answer as to how to deal with the stress. Everyone around me, my husband, the caregivers, my family, my friends, my doctors—they all fall victim to it.