Support

For the most part, people have been supportive. A few, however, have not. One such person is one of my close friends, John. Through all of this, I am trying to be compassionate and sensitive to the needs of others. My mom and other people have told me that this is not necessary, that the only person I have to worry about and take care of is myself. But this is not my nature. I am a protector and I take care of others. It’s what I do. It’s who I am. When I tell people, I try to do so as gently and as easily as possible. I know it hurts. I know they’re scared and don’t want me to die, but the fact of the matter is that I am dying and no one can stop this. No one has control over it. Right now, I need people to be there for me and be supportive of me. I need them to put aside their feelings and support mine.

This especially goes for John. We are lung twins. We were introduced by Alysse, who may not do or understand a lot, but we can owe our friendship to her. When she told me her friend, like me, had cvid, I didn’t believe it. CVID is so rare. It seemed highly doubtful that she had a friend in the area who also had CVID, got infusions, saw the same doctors, and had lung issues. It was nearly too good to be true; but it was true, and from there, our friendship grew.

He was the only one that got it when I talked about how much it sucked to be in my 20’s and dealing with this. Like me, he wore a high liter flow of oxygen. He knew what it was like to have to gauge the distance you could travel by how much o2 you had. If I made plans with someone (suppose we went to a movie), and then they wanted to go out to lunch after, but the lunch was not part of the original plans, I couldn’t go because I hadn’t factored that into my o2 usage. We talked about what it was like to be young and unable to work, or go to school. How much it sucked to be o2 dependent. We talked of our hopes of new lungs and running, riding bikes, just breathing easy. He urged me to go to see his doctor at Mass General, head of lung transplant.

Though John has lung issues that present themselves in a similar way as mind do, they are due to a different cause. He has been going through the process of trying to get on the transplant list for new lungs for almost a year. He has had numerous tests, but unfortunately due to the complex nature of the cvid and his lung problems, it is taking them awhile to list him. Each time it seems as if they will tell him yes, and list him, it is postponed, and he is forced to undergo yet another test. This has been very trying for him physically and emotionally. While I was in rehab for my trach, he was in rehab in Boston to recover from getting pneumonia, which he got as a result of a lung biopsy, one of the tests necessary to be put on the transplant list. I worried about telling him about the DNR because I knew it would hit him hard. I am one of the closest friends he has. I get it where others don’t. Most people with cvid don’t have the extent of lung damage that we do. Most people do well with Igg and get few infections after that. But this wasn’t the case for us. Though our lung issues differed greatly – his were a result of venous malformations and mine unknown origin, we both know what it is like to struggle so severely. He hated to see the little support I got from my family, or the neglect I got from my doctors. He wished for me to go to Baystate and Mass General where there are better doctors. It was only when it came to me needing a trach did his friendship falter. He wanted me to get another opinion, go to Mass General, not get the trach. But I already knew that the doctors at Mass General didn’t have any answers. They had specifically looked at me and told me they couldn’t find the reason, they didn’t know why my lungs were so bad. I was not a candidate for transplant. My body was too weak; it would never survive. No, the doctors at Mass General probably would not have put in a trach, but they wouldn’t have had answers other than to say we don’t know why you are as sick as you are. They probably would’ve thrown psycho babble, mumbo jumbo and not solved a thing. At least the trach gave me a chance. I knew that telling him this would hit too close to home because he knows that if he isn’t listed he too, will be facing this process. Without him saying it, I know it scares him. But I did not expect the fierceness of his rejection.

Everyone deals with death and grieving differently. When I told John, he was angry. Though I understand he wasn’t necessarily angry at me, his anger seemed directed at me. As if, how could I allow this to happen. You don’t need to do this. You don’t need to give up” he said. “You need to get to a better hospital in Boston and fight for your life. Get PT and push yourself. Be in pain and let the pain remind you that you are alive” He says, “I know it’s lonely. I know it’s hard. I know it sucks. But life’s a bitch, get a helmet.” “Get a psychiatrist. You have every right to feel sad and want to quit, but you are much stronger than that. You don’t need to do this.” And no words cut me more deeply. Because this isn’t’ a choice. I am not giving up. Honestly, I don’t know anyone who has fought harder against this disease. All those years of pain. I saw specialist after specialist. Went from Boston to New York City. No one had answers. No one had suggestions. All they had were more questions. I fought when I had no one by my side. I got myself to specialists. Made appointments for myself. Advocated for myself. It was lonely all those times in hospitals by myself. Times that my family wasn’t supportive. I had no friends. And though there were moments, I wanted to give up. I didn’t. I fought. I fought and I fought and I fought. I held my head up high and said nothing will stop me. I’m a survivor and I will prevail. This is not depression. I’ve been depressed. Depressed is when the world is black and you want to curl up in a ball In the corner of the room and sleep until it is over. I do not wish to avoid this, but rather want to embrace it, head on. I am sad, yes, but not depressed. I am sad at the people I must say good-bye to. Sad at the people I must leave behind. Things are not bleak and dreary as the world is often described when you are depressed, but bright and vibrant. It is so many colors come together in beauty. I am not choosing this but it is inevitable. It is my moment.

When I talked to Kathy about his, she said, “But they don’t’ have a diagnosis yet.” “They don’t have an organism” Sometimes in medicine, they don’t know “why”. Medicine doesn’t have all the answers. It cannot fix all things. If it is my time to go, I will go. I am not giving up. I am still doing therapy, taking treatments, doing my meds. But it is a losing fight. There is nothing I can do but put it in God’s hands and let His will to be done. It angers me to think someone could say I’m giving up or that I haven’t fought hard enough. Name me one person that has fought harder.

There comes a point at which it’s about quality not just quantity. I didn’t have a quality of life. My life sucks. My lungs aren’t getting better. Without a transplant or something of that nature, I never will have a quality of life. Even if my lungs didn’t get worse; even if they only remained the same, I still would never run, ride a bike, or even walk to the end of the driveway. I would never go anywhere without oxygen & just breathe easy. I do not remember what it is like not to struggle for every single breath, a time when breathing was an unconscious thing I did without thinking about it. I have a trach. I am alive, but I don’t have a quality of life. I have never turned in fear or cowered in the face of pain. I have faced every aspect of this disease head on with dignity. I have held my head up proud and will continue to do so. I have done more than nearly anyone else. John and I discussed it. We discussed that should someone come to us and say we needed a trach, we would say no, and let God’s will be done. But in the end, I didn’t do that. I agreed because I wasn’t ready. I needed more time, and I held the hope that I would get better. I didn’t cower in pain and fear when I went to rehab. I stood there boldly and strong facing the pain and I relearned how to walk, how to talk, how to eat, and how to breathe. I relearned practically everything I had learned through childhood as the trach changed my entire life. Not once did I give up. Not once did I say I can’t or it’s too much. I had my moments, but mostly, I faced each day with determination to get my life back, and to have it on my terms not my diseases. But I can not beat this one. It is not my family holding me back from seeing other doctors and specialists. If I truly wanted to get there, I would. I have gotten places myself many times before without any support. No one can stand in my way if I determine I want something and it’s what is right for me. But everyone has a life and this is my life. It has come that I need to be vented. The doctors haven’t said this, but I know it to be true. I don’t want that. I know I am dying. I refuse to spend the time I have going from doctor to doctor or procedure to procedure to be poked, prodded, and endure pain with no increase in quality of life. No answers, no better options. This is about ME and MY quality of life. I have already been given more time than I was supposed to have. Had it not been for the trach, I would be dead by now. Maybe for some that would be easier. Maybe the trach gave them a false sense of security that I was going to beat this, defy the odds, get better. But we knew it wasn’t a cure. It was a chance. It was a fight of time. I was not ready. It has given me time with my family and friends. Time is so precious. It is something that once lost can’t be gotten back. I did not choose this; however, through this all, I have held my head up high and I will through this too. This is not a choice. I am making, except I am choosing to say no to treatment. However, this treatment won’t cure me, but it will only prolong my suffering. To be realistic, if it is not this infection, it will be the next, or maybe the one after that. But the truth is I am dying and nothing can be done to prevent it. I will embrace death as I have embraced life – head on with my head held up high.

Pulmonology Update #2

I saw Dr. Ginns, the transplant specialist at Mass General, on November 23rd. I ended up having to go to the appointment by myself since I couldn't find someone willing to go with me. I didn't have any tests this time such as pulmonary function tests, so I was a little less worried that the appointment would influence my ability to drive. I ended up getting there very early and so had to wait to see the doctor. Even once I got into the exam room, I waited a good hour and half to see the fellow working with him. I find this to be one of the most frustrating aspects of working with doctors such as those at Mass General. It's as if your time is not valuable. They ask you to get there at a certain time and should you be late, you'd lose your appointment. They know that at least 90% of their patients are driving a considerable distance for the appointment, it would only seem considerate and respectful to be on time for the appointment. A half hour late is one thing, but close to two hours of waiting in an exam room alone, waiting for a doctor, is a bit absurd. He had no other patients before me, since I was his first patient of the afternoon. It is just frustrating.

The fellow asked a whole bunch of questions and of course we went over my medical history. I explained my history of breathing problems and how it started nearly at birth, and how my first severe asthma attack was at 6 months. I described how my first admission to the PICU for breathing problems was at 13 and that I was intubated for the first time at 15. Since then I have over 25 intubations. Honestly after 25, I stopped counting, as to continue to do so seemed pointless. In fact, I had just been discharged from the hospital 2 days before the appointment. During that hospitalization I had been intubated. I explained my CVID and my history with infections.

We then began to address my most frequent concerns - mainly the decrease in my lung function, increase in o2 demands, and decrease in quality of life. I voiced my concern that with a lung function of 20%, my lung function did not have far to fall. I did not mention transplant to the doctor, but rather stated that I was looking for options for the future. If my lung function continues to fall, I know that something will have to be done and I would prefer to begin to open conversation regarding this rather than get to the point that this is necessary and no discussion has been taken. For a transplant to be possible the patient must be sick enough to need a transplant, but not too sick. I don't want to pass into the "too sick" category. I told them how I was requiring more o2 than ever. That before the most I've ever been on is 2-3 L, and at this point I'm using 5+. The smallest of tasks such as walking to the bathroom leaves me short of breath. I am no longer able to do the things I used to. The most disappointing thing is that a year and a half ago, I was able to exercise. I was doing 2-3 jazzercise classes a day, at least 5 days a week. I cough up blood nearly routinely now. Some days only a little, others up to 2-4 oz. At night, I wake up gasping for breath. Whereas most people have dreams when they have to go to the bathroom that they have to go to the bathroom, then they wake up to find they really do; I have dreams that I can't breath and that I am being suffocated. When I wake up, I realize that indeed I truly am not able to breath.

When Dr. Ginns came in, the fellow reported everything we had talked about. He seemed to take everything in stride, which kind of disappointed me. I guess I expected more of a reaction. After all, I was telling him I was coughing up blood, woke up unable to breath most nights, and how limited I had become because of my declining lung function. Maybe it's just that he sees such severe cases everyday that he is slightly immune to it all. He did take me off the o2 to see "how low I could go." To which I was not a fan. I became so short of breath that I was unable to talk. I was having difficulty even with understanding what he was saying or asking me as my brain could not comprehend what he was saying. I do not know how low my sats eventually fell as I could not see the sat monitor, but finally he allowed me to put the o2 back on saying that he didn't want me "to be uncomfortable." Despite everything, I know he is a competent doctor. He put me on Bactrum as a prophylactic antibiotic. Though I am already on a prophylactic antibiotic, he said the Bactrum is better at preventing respiratory infections. He also ordered a Barium Swallow, to rule out gastric reflux as the cause of my lung problems and a chest CT. I will see him again on January 11th to do a 6-minute walk and more pfts.

I had the Barium swallow and CT scan on December 8th. Neither test was bad. I have obviously had a million ct scans, so no problem there. I had the ct scan in the morning, then the barium swallow. I had never had a barium swallow done before and everyone I talked to told me how disgusting it was to have to drink the contrast, but I did not find it bad at all. It is very similar to the contrast you drink for abdominal ct scans. The hardest part for me was drinking while laying down and having the xray machine so close to my face. I have a hard time laying completely flat anyway with my breathing and am definitely not comfortable drinking while doing so. I am afraid I will choke. When I was trying to drink laying down, they had the xray machine right up against my chest and nearly to my chin. This made me feel very claustrophobic. I was glad it did not last longer than a minute or two. The barium swallow was negative. They saw a small amount of evidence for gastric reflux, but only when I was drinking the water laying down. Since no one drinks laying down, or at least I don't, this doesn't really matter.

Pulmonology Update #1

Well since my lung function has declined so much in the past six months, I decided to try to get an appointment at Mass General Hospital in the pulmonology clinic. I have decided to take matters into my own hands and discuss my future, whatever that may mean. One of my friends sees the transplant specialist there, Dr. Ginns. So in August I made an appointment with the general pulmonology clinic for October. I had to make the appointment with the general pulmonology clinic rather than getting straight into seeing Dr. Ginns because I didn't have a pulmonologist to refer me to see Dr. Ginns and you need a referral, whereas anyone can make an appointment in the general pulmonology clinic. It was quite a long day. Went with my aunt and had to go in early to stop by Boston Children's to get some lab work done that can only be done by Dr. Bonilla's lab. I had pfts scheduled for 3:00 and then my appointment with the doctor at 4. These were possibly the most thorough and torturous pfts I've ever done. They did each test nearly 6 times. By the end, I was so tired, I couldn't even complete the test. This was not just simple spirometry, but rather full-set of pfts with diffusion testing ect. For me, they equate to medieval torture methods. Just hearing the words, "Deep breath in, and BLOW, BLOW, BLOW, BLOW!!!!" make me want to run away screaming. The tests left me so short of breath, I was only satting at 92% on 6L. The doctor was running late and I didn't actually get seen by the doctor until after 6pm. The result was what I expected and hoped for. I got a referral to see Dr. Ginns. I basically told them that I was concerned with how much my lung function had declined and needed to begin to discuss my options for the future, especially since I didn't feel that Dr. Rogers was treating me properly and actually endangering my health through his lack of care. While this was the result I was hoping to achieve, part of me was still disappointed, hoping that they would just have a new treatment that would help.