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.

True Friends

I've said before, that when something like this happens, you quickly learn who your "true" friends are. A disease like this is so isolating. I used to have many friends. I wasn't exceedingly popular, but I enjoyed hanging out with the friends I did have. I had friends I could trust and knew were there for me. Often they would come to the hospital with me. We were close. When I graduated and had to move home, things changed a little bit because I no longer could drive 1 1/2 hours to go to MA to visit. I was replaced by other friends. As I got sicker, I wasn't physically able to drive up to MA all the time. Sad to say, but most people were selfish and self-centered. They couldn't be bothered to make the trip to visit me, or meet part way. When I gained weight due to the prednisone, I stopped going out as much. I was embarrassed by how I looked. In addition to not being physically capable of doing what I did before, I had also gained weight. Though I know that they really wouldn't say something to my face. They wouldn't say that I was weak and fat and out of shape and that if I just had discipline and lost the weight I'd be better. But in my head, that is what they were saying. So I went out less. This just isolated me more. Everyone seemed to be doing normal tings, and here I was locked in my body.

I have been let down a lot. Trach surgery only made things harder, not easier. I haven't seen one of my friends since before I switched to home infusion. When I went to home infusion, I stopped going to MA regularly. I had gotten to the point that I was too sick and too weak to drive that far. Since I didn't drive there, I never saw her because she didn't drive here. In all my friendships, I've been the one to sacrifice things. Yesterday, we were supposed to get together for lunch since she'd be in Manchester visiting her boyfriend. We had planned on lunch. At 2:00 she still hadn't showed. I finally texted her, saying that I was assuming since she hadn't shown up, she wasn't going to. She said that her boyfriend had a church fair and she would try to get together again when she was in Manchester next. I was so angry and hurt. First of all, it would've been nice had she told me this because I had waited for her. I could've done something else or planned something else. I am sick of her broken promises. She says she will visit, then doesn't. I sent her a message back telling her not to bother, that a disease like this showed me who my true friends were. She said she was a true friend. She showed she cared by praying for me but that there hadn't been an opportune time. I got so angry because when it was me traveling the distance, no one ever thought of it. They didn't question whether it was an opportune time or convenient for me. It hasn't been the most opportune time for my other friends to visit, such as my friend John, who is sick and going through the process of being listed for a lung transplant. It isn't convenient or the opportune time for my friend Ducky from New Jersey who has health issues herself to visit, but she does. They do because they care and because they are friends. Friends are there for one another through the good and the bad. They support each other and help each other out. Prayer is good, but I need more than that. I need someone that I can count on during this difficult time. I think out of all the things in this disease, it is the loneliness and isolation that may be the hardest.

Locks of Love

I began growing my hair a little over a year ago. At first, I started growing it because I thought that I was going to have to have a bone marrow transplant. I have been receiving IVIg treatment for my cvid for 5 years now. Typically replacement immunogloblulin therapy is sufficient to keep a patient with cvid healthy. Unfortunately, this has not been true for me. When I was first diagnosed and started receiving IVIg treatment, I received infusions once every 4 weeks. At first this helped. I was in the hospital less than prior to being diagnosed and starting IVIg treatment; however, I was still getting many infections, frequently requiring the need to be hospitalized. My doctor determined that we would increase the frequency of the infusions to every 3 weeks instead of every 4. Initially this helped to decrease the occurrence of infections, but once again, it did not stop it completely. After nearly another year of treatment, it was decided that perhaps a lower dose more frequently would more effectively manage the infections. The new treatment schedule had me receiving 10G IVIg weekly instead of 40G every 3 weeks. The idea was that a lower dose more frequently would eliminate the peaks and troughs, helping me maintain a more steady state of immulglobulins, giving me better protection from disease and infections. This is not how cvid is traditionally managed, but we were willing to try just about anything to get the infections under control. Initially, things seemed to improve greatly. I was able to go 8 - 10 weeks without being in the hospital for an infection. However, this was still not enough to keep me infection free. Not having any other options, my doctor decided to suggest the possibility of a bone marrow transplant. A bone marrow transplant would be a cure for my cvid.

CVID is the result of faulty bone marrow, which makes faulty B cells. In healthy bone marrow, B cells are made and they mature to the point that they can identify, attack, and kill potential pathogens and invaders. In people with cvid, the B cells never mature. Instead, they remain in a naive (baby) state, where they are unable to identify or kill pathogens. Since most people with cvid are able to be kept healthy by receiving Igg infusions, a bone marrow transplant is not even considered. Though a cure, a bone marrow transplant is a very risky procedure. To undergo a bone marrow transplant, the patient must first go through high doses of chemotherapy and radiation to completely destroy the patient's own bone marrow. Once a patient's own bone marrow is completely destroyed, they receive an infusion of a donor bone marrow. The donor bone marrow must be a perfect match. Even under the best of circumstances, it is nearly impossible (except in the case of an identical twin) to get an exact match. The problem is that without an exact match, the body identifies the donor marrow as foreign and attacks it, causing a variety of symptoms called GVD (Graft vs. Host). A person's body can reject the donor marrow completely if it is too dissimilar. Once the person receives the infusion of donor marrow, the donor marrow grows and matures. Once it is fully mature and functional, the person can go home. The donor marrow is free of the genetic mutations causing cvid, and thus the person no longer is considered to have cvid. It is because of the severe risks and complications that a bone marrow transplant is rarely done. The chemotherapy and radiation itself is extremely harsh on the body and a person can die from complications of these medications alone. The chemotherapy causes the person to lose all of their hair.

When the doctors first began discussing this as an option for me, I began growing my hair. I knew that losing my hair would be very traumatic. I love my hair. In fact, it is one of the only physical features of my body that I absolutely love about myself and would never change. Why? Well, first of all, my hair is a unique color. It is made of various colors and tints - browns, blonds, reds... It can't be described as one color, but is often referred to as honey blond. I have never altered my hair color in any way. I am proud of my God-given color. In the summer, the sun causes it to lighten and different highlights of various colors appear. My mom's hair dresser has spent countless hours trying to reproduce the color of my hair for my mom when she gets her hair colored each month. I am proud of my hair and its uniqueness. It is a symbol of who I am. Losing it, even if it is to have a bone marrow transplant and a cure to my disease, would be devastating. This is why I began growing my hair as soon as doctors began even thinking of such an option. If I truly needed a bone marrow transplant, I wanted to be able to cut my hair and turn it into a wig to be worn while I received chemotherapy.

Who am I...

I thought that as I begin, I would explain a little of who I am and how I got to be where I am today. Granted, this is only a small piece of a very big puzzle. I will not say my illness is who I am, however, it is a very big part of who I am. It does not define me, but it most certainly has shaped me. I am neither ashamed nor proud of it. My health is like an arm or a leg, I cannot live without it, but nor do I glorify it's existence. It just is, a part of the larger body.

I was diagnosed with CVID (common variable immune disorder) in 2006. In short, this means that my body does not produce immunoglobulins, which are the cells that help fight infection. This means that I get infections easily, since I have no way to fight them naturally. When I do get infections I often end up in the hospital. There has been damage done to my lungs as a result and at this point I need oxygen continuously. I receive weekly treatments of IVIg (intravenous immunoglobulin) which is supposed to help prevent me from getting infections.

CVID is a primary immune disorder, which means that it is genetic. Despite its name, it is not very common - though it is one of the most common immune disorders.

The road to being diagnosed was very long and difficult. I was always sick as a child with upper respiratory infections sinus infections, and what-not. I was hospitalized for the first time when I was 13. At the time I was diagnosed with severe asthma, though my symptoms seemed to go beyond the scope of traditional asthma. Over the years, my 'asthma' got worse and I had to be intubated several times. I have been intubated, on a respirator, and in the ICU over 30 times to date. I've stopped counting at this point. I got sick more frequently, got pneumonia constantly, was in the hospital several times throughout the year.

In the summer of my sophomore year of college, I got pneumonia and never got better. I was intubated 3 times in one month. Every time I left the hospital, I was back in within a week. I constantly had one infection or another and was on antibiotics and steroids. I required continuous oxygen. I was a nursing student at the time, and told my doctor that I was immunocompromised. If I was in a large room and one person had a cold, I got their cold, except that I ended up in the hospital with pneumonia. My doctor did not believe me and told me that I did not know what I was talking about. But it was my body, and I knew it best. I knew that something was not right. I switched doctors and the new doctor ran a simple blood test - yes that is all it took. When the results came back he confirmed my fears. I was diagnosed with CVID. I was put on monthly infusions of IVIg.

At the time, I had never heard of CVID, never even heard of or thought that I might have an immune disorder. Adjusting to the diagnosis was difficult and took some time. I had to give up my career in nursing since I could not afford to get everything my patients had. I switched to biology with the intents of pursuing a career in research where I could study and perhaps find a cure to this disease.

When I first began the IVIg treatments I expected a quick fix. I expected to go back to how I was before - to not be sick and to not get infections all the time. Though I got slightly less infections, I was still sick and in the hospital with various infections a significant amount of time. The treatments were increased to every 3 weeks. Two years ago, we changed the treatment regimen yet again to once a week in hopes of getting rid of the peaks and troughs that I experienced when the treatments were spaced out to every 3 weeks. When I got them every 3 weeks, I would initially do well right after the infusions, but by the second week would start to struggle with fatigue and other such symptoms. By the 3rd week, just before my infusion, I would be so run down and get sick that it was almost impossible to function.

I struggle with other complications of this disorder. I have chronic pain and abdominal issues. I am often exhausted to the point where getting up and performing even the smallest of tasks, like taking a shower, is an accomplishment. I suffer from severe bone and joint pain to the point that some days I am unable to move. My lungs have deteriorated quite significantly from the numerous infections.

Initially, the weekly infusions helped tremendously. I was healthy and out of the hospital for longer periods of time. I was able to come off the oxygen and live a relatively normal life. I had plans to go onto grad school, but realized that this would not be possible with how sick I am. Currently, I am also not able to work because it is impossible to hold down a job if you are in the hospital and sick every couple of weeks. This has been very difficult for me both physically and emotionally.

The road I've been on is long; the path I continue on is longer. But I have hope that it will get better - somehow.