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.

I think one of the hardest things about this is telling people. Knowing that I am hurting them and knowing that it will only be harder and only get worse. I wish I could save them from the pain. I wish I could protect them, but I can't. Not telling them doesn't protect them or save them from hurt. It shortens the time they have with me. I feel I need to give them this time, this opportunity to spend with me and do things, make memories, before I'm no longer here. But it hurts me to hurt them, because I love them so much, and that will, never change. I will never stop loving them, even when I'm not here. I know that this time is a time that is all about me, but I need to make it be about them as well, because they are my support system, my life, and my love. My time here is short, I know that. I can already see it, though others may not. I try to deny it. I try to imagine that I will get better, that I will be able to move into my own apartment and live a life, but realistically, I know this isn't true. I am only able to lie to myself for small periods, and even then, I know it's not true. The periods I am able to deny reality get shorter and shorter. I am sick and I would never wish this existence on anyone. If I do anything one day, I then sleep for two days to make up for it. I sleep all the time. I try to deny the changes, but it's hard when it's right in your face. Denying it, won't help. It won't stop it from happening. It won't give me more time or change circumstances. The facts remain the same - I am dying. It sucks. It's not fair. I try to be patient with my friends and their needs. I know this isn't easy. I've been sick for many years. In some ways that fact makes it harder because people are used to me being sick. They are used to me being in the hospital, even in ICU and on respirators. I always get better. It desensitizes them. We've talked about my death. My close friends have listened when I talked of my fears that I was going to die before I was ready. But I don't really think any of us really wanted to believe these were anything more than fears. We didn't really think this day would come. But it has...

The one thing that really gets me choked up and cry is the idea of all those I love left behind. What it's going to be like when they get the word that I'm officially gone. All those years, that I attempted suicide, not once did I really think about it. I didn't consider death and what it really meant, but that is the difference between suicide and true death. Suicide is done out of anger. It is selfish, where all you're concerned about is yourself. It's a giving up. it's going in the corner, curing up in a ball, and dying. It's black. True death is the opposite. True death is white. It is selfless. It is not giving up or giving in. It is acceptance. I worry about my friends and what it will be like to them to lose a good friend, for some we're so close, we're like sisters. I worry about my siblings. Losing Poppy was devastating and hard, but this... I don't know... Parents shouldn't have to bury children. It hurts me just knowing the hurt they will feel. Yes, I know they'll move on. Life is for the living, and gradually, they'll move on because they'll have to. There is no choice. I know that gradually it'll hurt less, but the hurt will never completely go away. Someday we'll be reunited. Just as I know I'll see Poppy when I go to heaven, I'll be waiting for them.

Random Musings...

You know we have very little control over our life. Sometimes we are on the sea of life and we just have to go with it - go with crests and falls, the storms and sunny weather. There are times it's rocky, other times calm. The reality is that though we like to think we control things, we don't. We just have to go with it; and when we think we aren't strong enough, when we think we can handle only so much, we can be surprised by our strength to survive and persevere. We can handle a lot more than we think we can. Do we really have a choice? Not really.

This is a theme - choices. What choices do we really have? What can we control? We have one choice - to live or to die, and even that I'm not sure how much we control. Even if we are seemingly ready to die, are we really? Are you ever? Are you ever truly ready to leave this world and move on? Do you wonder what it's like? You have to. I have to think that it's part of the human spirit to wonder. My question... Does it hurt? Not the actual dying, no, but the process. Our body doesn't just give up. They talk about death rattles - how people who are dying stop breathing and then start again, stop and start. The body hangs on. It wants life.

My life has not been what I would have thought, nor probably would've chosen. I have been through more than the average person. There is so much left that I want to do, that I always thought I'd have time for, time to do. But how much time do we really have? - Not much... Not much at all... Not nearly enough... I think about things and I hope and pray I leave my footprints on this world. I guess if I were ever to say I had an ultimate goal, it would be to leave this world a better place than when I came into it. I always imagined that I'd do something important. You know... write a book, be a motivational speaker, be known for something important. I remember as a kid having such grand ideas. I wanted to be an astronaut, a famous author, to find a cure for cancer, or solve all the world's problems. I was going to marry Prince Charming and be rich, successful, famous, and happy. I'd have a family - one boy, two girls.

As we get older, we begin to see life in a more practical way. I wanted a career that was satisfying. Maybe I wouldn't be famous, or internationally known, but I would be important in my own little niche. I would meet somebody, fall in love, get married, and have kids. I may not believe in happily ever after, in the fairytale type of way, but I believed in happiness. Nowhere, does it say anything about getting sick and dying. Nowhere did I want to become disabled to the point that I truly couldn't work, need oxygen to breathe, or have a trach put in. This was not in the plans. Sometimes life is unfair. I realize, this is very cliche, but it's true. Why do murderers and child molesters get to live long healthy lives, while I don't? No parent should have to bury her child. I have seen mothers that have had to bury their children, and it's horrible. It just shouldn't have to be done. It's not the way life is supposed to work. Of course, my life didn't work the way it was supposed to work, ever. I think of my mom, and it just makes me cry. She shouldn't have to go through this. No, she has not been perfect. She has made her mistakes. Sometimes I get frustrated, sometimes angry, but she is a good mom and has always done her best. I am especially grateful for the past few months where she was home to help me through this time with the trach.

disAbled

I was in college when I was initially put on oxygen. It was during spring semester of my sophomore year. My breathing had been gradually been declining to the point that I needed oxygen. It was around the time that I was diagnosed with CVID. I had had many boughts of pneumonia, been the hospital quite frequently, and intubated ~10 times in 6 months. I came back to campus after being in the hospital, discharged on home oxygen. Some people in the administration asked if I could go to school. I remember when I was asked this that I just stared at the person asking in a complete state of puzzlement. I could not grasp why they would be asking if I could be at school. My doctor had cleared me physically. I knew no other reason why I couldn't/shouldn't be there. I asked them why they felt this way. They said, "well you're on oxygen" as if this statement explained everything. I looked at them and told them that being on oxygen hadn't changed anything. My lungs didn't work; I had difficulty breathing and need oxygen to help me, but my brain still worked perfectly fine. In fact, It worked better than at least 90% of the other students. I had never really considered myself disabled. I have never considered myself disabled, even through all of the challenges I have had to overcome. I remember starting college as a freshman and receiving a letter in my school mailbox asking me to meet with the school's disability coordinator. I was completely confused. Why did I need a disability coordinator? I wasn't disabled. When she suggested I get accommodations I could use for my classes, such as more time on exams, the ability to turn assignments in late, or take exams late. I got angry at this. I didn't need accommodations. There was nothing wrong with my brain, and I just wanted to be treated as every other student would be. I later learned to take these accommodations because they did help when I was sick and in the hospital. Accepting these accommodations didn't change how I was graded. It didn't mean I was weak, couldn't hack it, or was stupid. I was graded on the same level as every other student. I didn't have to use the accommodations set up if I didn't need them, but if I got sick and was in the hospital, they were there to help me out. It didn't give me an advantage over other students. The same was expected of me as them, but it evened the playing field, as I had extra challenges to overcome as a result of my disease, that they did not have. They were tools to help me be successful in such an environment. Though my lungs didn't work, my brain did.

For the same reason, I didn't get a handicap sticker for my car until several years after I was put on oxygen. I never considered myself handicapped or disabled. I had two legs; I could walk. I needed oxygen to be able to get around, but other than that, it didn't limit me too terribly much. There were even times I tried to run the track, with the oxygen attached to my back of course. I put the oxygen in a small backpack and ran the track. My goal was to be on the track team. I wanted to prove that someone with a handicap, like me, could do just as much as an able bodied person, so long as they had the modification to do so, such as wearing oxygen or having a prosthetic leg. One of my friends in college had been born without any legs. This didn't stop her at all. She was on the softball team, an excellent athlete. With her prosthetic legs she was able to do nearly everything anyone else could. I knew of people who were considered able bodied, but were able to do much less. It wasn't until my lungs had declined quite significantly and I was in chronic respiratory failure, that I broke down and got the sticker. By that point, I had no other options. I couldn't walk long distances and could only go anywhere if there were handicap spaces available. Unlike most people who would love to have a handicap sticker because they could park closer, I had fought to not need one. For me it was a defeat, one that symbolized my disease had won. I mourned the day that I got my handicap sticker. It represented the extent of the decline in my health. Then I needed a cane to walk. Now my mobility was affected. I couldn't walk long distances. I certainly couldn't walk long distances without the cane. Each thing was a blatant reminder of the progression of this disease and the losing battle that I was fighting. Now I'm in a wheelchair.

While part of a disability is about the functional loss of ability, there is also the body image, and how you now see yourself. The functional loss is honestly the easiest part to live with. Yes, you have difficulty walking, but you can use a cane, walker, or wheelchair, whatever the case may be to get from point A to point B. That isn't the problem. The real problem comes in how you see yourself. You used to be completely physically able. You could go and do whatever, whenever. Now you are limited and in many ways; you become isolated. It is hard not to lose your sense of self in the process, to remember that you are still a valid and worthwhile human being. Your worth as a person is not based on what you are physically capable of doing, whether you are able to walk, but on who you are as a person and the value you bring to those around just by being yourself.

Before the trach I had planned on tutoring through the summer. I had tutored a student in biology and this year she would be taking chemistry. She struggled academically and had a learning disability. Her biology teacher had suggested taking chemistry in the community, which wasn't a true chemistry course, but a nonsense course. She didn't think she was capable of doing the regular chemistry course. My student wanted to go to college, and colleges would require the regular chemistry course, not the chemistry in the community course, to be considered for admission. I planned on helping her through the summer, introducing her some of the key principles, so that when she saw them in class she would already have a basic understanding. I would continue to tutor her through the class. The trach prevented me from being able to work with her over the summer. I was unable to talk and this made tutoring quite difficult. After doing rehab my speech had improved and I wasn't concerned with communication. Her mother still needed a tutor for her, and though I had a physical difficulty and couldn't yet drive, so long as she came to my house or my mom drove me to meet her, we could still have lessons. My brain hadn't been effected. This is the first time she has come to see me. I was so nervous. I considered attempting to greet her using the walker, not the wheelchair, so she wouldn't see me as physically disabled. I worried that she make judgements and think I was less capable. My mom had to remind me that she wasn't paying for my ability to walk, but for my brain and knowledge, which had not been affected by my illness. While this is true, it is hard not to let a physical disability affect your value and worth as a person. You feel deficient, and this is carried over into other aspects of your sense of self. I have become very self conscious and almost ashamed of my physical disability, not wanting others to see me in the wheelchair. It is important for me to remember that I may have a physical disability, but I am still perfectly ABLE, able to think, able to be a valued person, able to have a purpose. This hasn't affected my worth. There are still many things I am not just capable of doing, but that I excel. My ability to walk hasn't affected this in any manner. My thoughts return to my friend in college born with no legs. This was a strength not a weakness. I am not DISabled, but rather, disABLED, the concentration being on what I am ABLE to do, not what I am not.

The Blame Game

Medicine today is much different than it once was. It used to be that you had one doctor, a general practitioner. They handled all aspects of your care. When you got sick they admitted you to the hospital, and they saw you while you were in the hospital. If they really got stumped and in over their head, only then, they would refer you to a specialist. They did not farm you out at the easiest possible second. They took care of you themselves. Often whole families from grandparents all the way down to grandchildren, all saw the same doctor. The doctor was in many ways, part of the family. This is no longer true. The only type of doctor this still applies to is a pediatrician. The only way you see your doctor while in the hospital is if that doctor is a specialist and he has privileges at that hospital. For the most part, however, you do not see your doctor. Decisions about your care are made by hospitalists. They do not know you or your medical history. If you're lucky, they will have read your chart, but most of the time, they haven't even done this. You will probably have several different hospitalists throughout your stay. You may get one that lasts for a week and then changes. You may have one during the week days and a different one on the weekends. Depending on the hospital, you may even have a different one nearly every day. If you're in a teaching hospital, you most likely will have a team of doctors. The residents will be the ones who actually evaluate you; then they present your case to the attending. You may not know who your doctor is, seeing so many different faces throughout the day, not knowing who is who.

One of the problems with medicine today is a lessefair mentality. No one wants to take responsibility. If you're a complex patient with many different medical problems, as I am, you have a slew of doctors, each with their own specialty. Often I feel as if I am in the middle of a circle of doctors. I begin spinning around and around, faster and faster, looking toward the circle of doctors that seems to be getting larger and larger as I rotate going faster and faster. I become more and more confused not knowing who to turn to or where to go. Everyone has the "not me" attitude. No one wants to take ownership. No one wants to do the work. Instead, they pawn it off to someone else in a different specialty. You have problems with bleeding - not my job says the immunologist, and he pawns you off to the hematologist. It might be related to a mitochondrial disorder - not my job, see a neuromuscular doctor. It could be alpha-1-antitrypsin deficiency - not me, talk to the pulmonologist. They only do what they absolutely have to, no more. Anything they can pawn off to anyone else, they do. Not my job, they all say

This, however, means that you, the patient, have to be your entire medical team. You are the patient. Yet you also need to be the doctor, knowing what tests to run, what possible diagnosis it is or could be, what effects it has, and what treatment is given. You are the the nurse, taking the blood sample, sputum culture, urine analysis, and getting it evaluated properly. You must call and get the results because you know that the doctor will never call you back on his own. You'll be lost in a slew of reports. Then you have to prescribe the proper treatment because he/she will forget they even put you on treatment. You will be a one-man show. And you must do this all while you are sick. You do not feel well; you are weak and tired. Once you have played doctor, patient, nurse, and lab tech, you also must play insurance company to make sure all the test and procedures are billed correctly, have proper pre-authorization, and get paid for.

I think one of the most frustrating parts of this system is not only do you need a gazillion different specialist because each doctor says it's not my job and just pawns you off to the next person, none of these specialists communicate or work together. They are all cowboys, shooting off from the hip. In today's day and age, there is no excuse for this. We no longer need to wait for the Pony Express, nor do we need to send a telegram or use Morse code. In fact, we don't even have to write a letter, put a stamp on it, and wait for it to arrive in the mail. We can pick up the phone and call the person. Through the use of cell phones they can even be reached day or night, and in just about any location. But even better than this, we can send out an email, that will reach them nearly instantly, and they can reply just as quickly. They don't even have to be at their computer to get it or respond. They can do this from their phone. Yet, many times they can't be bothered. They don't communicate with your other providers. It is becomes up to you to enforce communication and keep everyone up-to-date. Just one more daunting task for you to do so that you get adequate health care when you're sick. It's all extremely frustrating.

Sometimes, one just has to wonder; it shouldn't have to be this difficult. I went to go see Dr. S, my immunologist/hematologist. I haven't seen him since before the trach. Though I didn't call him to specifically notify him that I got a trach, he knew. He had to because the doctors called him to get orders for my Igg. In rehab, I went up to Baystate to get my Igg and he wrote the orders ect. I knew I was going to see him soon, and didn't see the point in calling him just to tell him I had a trach put in. What would be the point? Some asked if I contacted him before getting the trach to ask his opinion. Well no. What was he going to say? It wasn't up to him and really there were no other options. It wasn't an elective procedure. Whether he supported it or not, I needed to have it if I wanted to continue to live. From an infection standpoint, it was a risk. But again, there weren't a whole lot of options. Whether he supported it or not didn't matter. Anyways, I often feel appointments with him are rather pointless. I long ago ceased being a run-of-the-mill patient. I am not your typical patient with CVID. Most patients with CVID start Igg treatments and with these treatment, they are able to live fairly normal lives. Their infections are pretty well managed. Unfortunately, I am a complex patient. Just traditional Igg treatment hasn't been effective. It has improved things. It has kept me alive. I am not constantly in the hospital, or at least I wasn't after I had been diagnosed and started Igg treatment, but I still got frequent infections and was in the hospital a lot. The frequency of infections and hospitalizations have increased, despite an increase in dose. I am currently on quite a significant amount of Igg. I receive 20 grams weekly, a total of 80 grams a month. Most people are on ~40 grams a month. One of the frustrating things is for many patients with immune deficiencies, their immunologist is at the center of their care, directing other aspects of their care and guiding other specialists such as pulmonogy and primary care doctors. They direct all aspects of their care and how it relates to a primary immune disorder. This is important because PIDD effects every organ system. But Dr. S has always had a fairly hands off approach. When it came to pain and CVID, his opinion was that's not my specialty and told me to see someone else, but it is part of the immune deficiency and thus he should've at least referred me to a doctor. Even if he referred me to a pain specialist, he should've been an active participant, consulting with the pain specialist, since my pain was directly related to my immune disorder, not due to some sort of pain syndrome. When it came to my periods and how they were making my CVID worse, it was - I don't know anything about that. I'm not saying he's a bad doctor, but he isn't a free thinker. I was the one to bring journal articles to him. He went with whatever my doctor suggested. I didn't expect this visit to be different. I did want to discuss with him the ideas of Pompe, or a mitochondrial disorder. I wanted to get tested for possibly an Alpha-1-antitrypsin deficiency. Most importantly, was the source of the bleeding issues. That I had such severe bleeding and that it had taken over 2 hours to stop the bleeding was a concern. This visit was especially frustrating because though I was there for over an hour; he was only in the room with me maybe a total of 10 minutes.

Sometimes people make the dumbest comments. You can't help but wonder what were they thinking. Better yet, were they thinking... Probably not. On coming in the room, he asks, "So I see you've got a trach." Powers of astute observation. Yes, it would appear so. "Well how's it working for you?" Just dandy; it's great. Think everyone should get one. Don't have anything better to do with your time? Get a trach. Life certainly won't be boring afterwords. Certainly wasn't how I planned to spend my summer, that's for sure. In fact, I can think of just about a million other things that I would rather do than get a trach. I mean, one good thing, I'm still alive. But definitely poses some challenges. I told him about the bleeding and after much convincing, he agreed to run the test for von Willibrans. Everything else - not my specialty; see someone else for that. This is what I mean. Everyone wants to put it on another doctor, any other doctor really. They just don't want to deal with it themselves. Doctors are not the only ones that fall victim to this. Nearly everyone wants to get away with doing as little work as possible. If they can make it someone else's responsibility so that they don't have to do the work, they will.

I went to see Dr. O this week. I had the appointment from before I had the trach. It worked out well though, because I was able to see the doctor with having this infection. Last Thursday I was put on IV Rocephin. First of all, by Monday, I still had not seen any results. In fact, I was still getting sicker. I was more short of breath. My trach was still disgusting, didn't look or smell any better. More than that, the Rocephin made me horrifically itchy. No matter what I did, I felt like I wanted to itch my skin off. Oral Benedryl had no effect. I tried taking a dose of Benedryl before my dose of Rocephin. I slowed down the push from 5 minutes to 10 minutes. Other treatments such as a shower, lotion, even Atarax didn't help. The only thing effective - IV Benedryl. When I brought it up, Dr. O refused to write a script for it. He stated that I would have to call Dr. S and get him to write for the Benedryl. This made no sense to me. The allergic reaction was not due to my immune disorder. It was not related to the IVIg. No doctor is going to write a prescription for a medication to counteract or treat the side effects to a medication they did not prescribe. That would be insane. No doctor would do that. The doctor that had written the prescription for the medication was responsible for any side effect that occurred as a result of the medication. Again, an example of doctors not wanting to take responsibility. Finally, I told him, he had two choice - he could either prescribe a medication to treat the side effect, I had already tried every non prescription method I knew of, or he could change the medication. I could not continue to be in live in such a manner; to do so would cause me to go insane - literally. He decided to stop the Rocephin and switch to IV Doxycyline.

It is so frustrating to have to fight for every little thing. I am sick. I don't feel well. Yet I have to be the patient, the doctor, the nurse, the pharmacist, ect. It's as if it's a one-man show, featuring me. When you are sick, you are tired and weak. You don't feel well. This is not the time that you want such a fight. Being sick is hard enough. But there are no other options. The thing about Rocephin is that I never should've been put on it. It is contraindicated in people allergic to penicillin. Well I am allergic to penicillin. Something that should've been considered, but they don't read the chart or take these things into consideration. The only way they know what drugs to try is by looking it up on PubMed, or in the PDR (physician drug reference), a book with every medication, the diseases they treat, and interactions with other medications. Honestly, it's pathetic and infuriating. The other thing is that I saw him at 2:00, was out of there before 3:00; plenty of time to fax the script to Professional Home Care, the company that manages my IV medications, so I could have my antibiotic when I got home. I called Professional to let them know to expect a fax from the doctor. they had not yet received anything and said they would have to get something by 5 to be able to get the med to me that day. I called the doctor back. The script wasn't faxed until 4:45. They hadn't included the form stating I'd had the med before, and by the time they were able to contact me, it was after 5, at which point the office was closed, and I couldn't get the antibiotic until the next day. Again, you shouldn't have to do all the work. Doctors should communicate amongst themselves without you doing it for them. They should take responsibility and treat you themselves without trying to put it on someone else. Yet somehow they have removed the patient from patient care. How I would love to go back to a time when doctors cared about their patients, when they'd did whatever they had to do to provide good patient care. They had a vested interest in their patients and general practitioners did it all. Unfortunately, those days are gone.

Whose Opinion is it anyway?

Have you ever noticed that everyone has an opinion about everything? This is what you should do; this is what you shouldn't do. This is what you should think, ect. This happens about everything - religion, politics, education, career choices, even medicine - what medicine you should take, what procedures you should have done, which doctors you should see. Granted, the main reason people do this is because they care.

When I had the trach, many people questioned whether this was best decision. It was done proactively rather than reactive. It was planned rather than waiting for an emergency. I remember my mom's initial response when I told her that the doctors wanted me to have a trach "No!!" It was a knee-jerk reaction . She didn't want her daughter trached or on a vent. I don't blame her. As you know, I've always had a "no trach" policy, so it must have come as a shock to her when I was actually considering it. After, people asked me if I had made the right choice. I did; I chose to live rather than die. You soon realize, however, that everyone has an opinion - what they would have done, or would do. In reality, they wouldn't truly know what they would do in the situation until they are actually in that situation. You can make all the plans you want, but reality is much different. One of the things many people said and continue to say is that I should have/should get a second opinion. At the time of the trach, I was in no position to be traveling to Boston for second opinions. I was on bipap continuously, hence the reason they were discussing a trach in the first place. Had I been stable, then yes, a second opinion may have been warranted; however, this wasn't an elective procedure. It wasn't something they did just for the heck of it. They did it because there were no other options. Had there been less invasive options, they would have gone with that. But they had run out of other options, and here I was fighting for my life and losing the battle. A trip to Mass General, just wasn't in the cards. There really wasn't a person to get a second opinion from, unless I wanted to take a chance on getting sicker and not making it. Honestly, even if I got a second opinion, what would it be? Would they say "no trach"? If so, then what would they suggest? Would they have a better plan? Or would they just be like - no trach, but we don't have any other options. This is most likely. We already knew that I couldn't remain on bipap indefinitely. They had tried to ween me, and that didn't work. Most likely, they would have more questions, no answers, and no solutions.

Kathy, my infusion nurse, thinks I should get all my care at Mass General. Though their doctors are supposed to be bigger names and more experience with complex disease, I have never once had a doctor in Boston who had answers and offered solutions. Rather, all I've ever gotten is more questions. "We don't know why this is." "We don't know what is wrong." Since we don't know what is wrong, we don't know what to do. It has always been a frustrating experience. But more than that, how practical is it to have my doctors in Boston. I can no longer drive myself and would have to find someone who could drive me. This entails them taking the day off so that I can see the doctor. If my doctors are in Boston, it will take me 2 1/2 hours to get there. This is not very practical when you're sick. I would have to plan it, and most likely this isn't possible. I can't travel the 2 1/2 hours when I am sick. I can't drive there, and most people can't just stop everything they are doing to drive the distance to get me there. Never mind what happens when I need to come home or if I don't get admitted. If I'm too sick, I'll still end up in the hospital here, and be seen by doctors who don't know me. When you get sick as I do, You go to the closest hospital. I can get sick very quickly. In the morning I could be fine, but a few hours later I am gasping for breath and needing to be intubated. There is no time to drive all the way to Boston. When I first moved home after graduating, I still got all my care at Baystate, which is 45 minutes away. I remember one time I had difficulty breathing and needed to go to the hospital. Mom drove me. I was gasping for breath and mom was trying as best she could to get me there. Finally as we hit Springfield I was too sick to continue and we had to get to the nearest hospital, which was Mercy. I was intubated shortly after getting in the ER. It was a scary experience for both mom and I. Her trying to drive and afraid she was going to have to stop and do CPR. When you crash as quickly as I do, you need to go to whichever hospital is closest to you. It is best if your doctor is on staff there so they are the ones treating you and making the decisions about your care, not someone who doesn't know you. It doesn't really help if you're in the hospital here and they're 2 1/2 hours away. They can consult, but they aren't actually seeing you, and who knows if the doctor actually does what they suggest. Gets to be one big mess.

Since being trached, I haven't done much. There were a slew of doctor's appointments I missed and should reschedule, but haven't. Part of it is because I just don't have the energy to go from one appointment to the next. The other, I just don't care. Even if they do have answers, what good is it if they don't also have solutions. So they give you another diagnosis, but they don't have a treatment, just more questions. Really, what good is that? I'm too tired to play the game. People ask, "did you talk to this or that doctor" "Did you get their opinion." No, I did not talk to that doctor. Personally, I was working on fighting to live and getting through the day. I know that chances are, right now, if I saw another doctor they would suggest pulling the trach. They'd probably feel it was unnecessary and overkill as treatment for the problem at hand. Of course, that is easy to say now that I am stable. They weren't there as I gasped for breath or when I was on bipap. Even if they had been, would they have had a better solution? If I was decannulated, what then? How would they treat it? More than likely I'd be back on bipap, unable to breathe, with no options available. They might have to re-trach me, and with all the complications of the first trach, I am certainly not up for a repeat experience. So excuse me for not racing after this doctor or that doctor. The time I have left is too precious to be wasted sitting in doctor's offices. Even if they offered a solution I'm not sure that I'd want to take it. A lung transplant might fix my lungs, but would the new ones be destroyed just as these ones have? Would I even survive? While some may not understand why I don't want to do anything and everything possible, such as having a lung transplant, it is such a gamble. There is no guarantee. It could make me sicker. It may be too much of a fight. I am tired and when my time comes, I want to accept it gracefully. I want to enjoy the time I have with my family and friends and not think about what could have been, but rather what is.

Infection

I have my first infection since getting the trach and it's really difficult. At first everyone wanted to know how I knew that I had an infection. Trust me... I know my own body. But more than that, my trach has changed. It is disgusting. Really thick, yellow mucous, and it stinks - smells like dirty washcloth meets dirty socks. Or maybe a better way to describe it is that it smells like Abby after she's been swimming. Very musty and gross. Now I can't really smell. So for me to smell, means that it is quite bad. Right now, I'm cleaning the trach every few hours. It just gets so thick and mucousy that I have to clean it. I'm also having to suction and I never did before. Also, I've been really short of breath. Sometimes too short of breath to wear the pmv to talk. It's definitely a difference from trach care when I was in rehab. When I would do trach care, it was slightly mucousy, but not overly so, and it certainly didn't smell. This normal and to be expected; nothing like it is now. Besides, I know... just as I knew before the trach, I know now. In fact, I think it is more obvious now because I see it when I clean it. I notice a change. Since the trach comes directly from my lungs, there is no question that that is where the infection lies. While it's good to be able to prove it, to be able to do a sputum culture, it's also harder because it's so "in your face." There's certainly no denying or ignoring it. The hard part about this is though I knew that I'd still get an infection at some point, I hadn't had one in a while, and maybe part of me hoped that maybe I wouldn't get an infection, that the trach had solved all of that. I guess, I also on some level, feel that getting an infection is my fault. I'm the one that cleans it. Yet, I guess I know this isn't true, that it's not my fault, an infection was inevitable, especially since having a trach makes you more susceptible to infection. I do clean it, and am responsible for it, but I take excellent care of it. Just like I take excellent care of my port. When I was in rehab, they'd train the other nurses or have other nurses come and watch me clean it because I did such a good job with it. Just as getting an infection before the trach wasn't my fault, and something I had no control over, neither is this my fault. Still, it is hard.

So Monday, I called the pulmonologist's office to get an appointment, but was told there were no appointments this week. Well first of all, there has to be appointments. There may not be any regular appointments, but there were emergency appointments. There has to be. They have to have sick patient appointments. But instead of arguing, I just asked them to have someone call me back. It wasn't an emergency and I didn't need to be seen. All I really needed was for them to order a sputum culture. I knew they couldn't put me on anything until they had a culture done anyways. Then I called Dr. Constantino's office to see if I could make better headway there. Turns out he's on vacation, and won't be back until next week. Great... Just great... Now I know he isn't really on vacation. He can't; he is the only trach doctor in the hospital. If a patient needs a trach, he has to do it. If a patient has an emergency, he has to be there. There isn't anybody else, and he doesn't have another doctor, nurse practitioner, or PA he works with. When I asked the receptionist what I should do, she told me I had to either see my pcp, or go to the ER. Well the ER would do me no good. The doctors there don't know anything about trachs. They are completely incompetent. The past times since I've gotten the trach that I was in the ER, both times for problems with the trach, they did absolutely nothing. When I had bleeding from the trach I was told that this was normal. When I had difficulty breathing, I was told that I just needed to get used to it. In fact, it turned out that the blood I was coughing up was from my lungs and vessels in my lungs had to be cauterized. The shortness of breath was caused by inflammation, which had caused an obstruction. So I don't have much hope that they'd be of help this time either. Besides, I just spent nearly 3 months in the hospital, I most certainly do not want to go back. Next, I tried Dr. Lafrenier's office because most other trach patients see an ENT to manage their trach. He is my ENT and it would be good to have him as back up in case this happens again, especially since this is the second time Dr. Constantino has been on vacation. However, I struck out here as well. Dr. Lefrenier was also on vacation and wouldn't see me in regards to the trach anyway because he hadn't put it in, which is a load of BS, but nothing I can do until he's in the office anyway. Thankfully, Diane, the PA in Dr. Wasserstein's office called me back and ordered the sputum culture. A sputum culture with a trach is much easier than when you have to just cough it up. My problem in the past had been that I'd be bringing up tons of mucous, but soon as they'd say "sputum culture" all sputum production would cease. At least this way, I had easy access. I had what was on my inner cannula, and if that wasn't enough, I could suction. Having never done a sputum culture before, however, made me nervous. In my opinion, it would have been ideal for me to either go into the office so that someone there could collect the sample or have VNA do it; then I know it'd be done right, and I wouldn't have to worry about somehow not doing it right. But to be honest, the doctor probably didn't know how, and VNA couldn't. So it was up to me. My biggest worry is that I wouldn't have enough for them to culture. Of course when I thought about it, if that happened, I'd just repeat it. It's not like I would be lacking in sputum or that I wouldn't be able to obtain any more. Basically what I did was get the sputum from around and inside inner cannula with a q-tip swab from the trach cleaning kit. I also swabbed inside trach and even coughed into cup. Looked like plenty to me. In the meantime, while waiting for sputum results, I was put on doxycycline and ceftin in case of infection. As I said, there was little doubt that I had an infection. Part of me wanted to put a warning saying "Caution! Do not inhale or sniff." Just unscrewing the lid would knock someone out with the stench alone. Oh, it was disgusting, nauseating. Another reason I knew it had to be an infection was that I had eliminated any other cause. I had completely changed all my equipment and tubing. Anything that couldn't be completely changed was boiled to disinfect. I thought of changing the inner cannula. I have two extra trachs and using the inner cannula from one would be okay, but if it was an infection causing the stench, changing the inner cannula wouldn't help. All that it would do is make the new inner cannula gross too. Some people on the trach board suggest doing a complete trach change, but I've never been taught how to do this. When I saw Dr. Constantino, I asked how often he changes trachs because the box said it should be changed once a month. He said he changes them every six months. Though at most it is an office procedure, many moms and other patients change their or their children's themselves. He said he prefers to do them in surgery at the hospital especially after all the bleeding issues I had, he wants to make sure at least the first time it is changed that he is at the hospital where if something does go wrong or I have a lot of bleeding, he can fix it. Though changing the trach doesn't seem too terribly difficult, it is not something that I just want to do myself without having been taught or shown how to do it.

Finally the results came back. As expected, it did grow bacteria. Honestly, had they said it was negative, I would've had them do another. All I had to do would was take out my trach to show them - the look and smell alone made it obvious that there was an infection. When I called the doctor to get the results, she said she was going to call in an order for ceftin. I get really frustrated by doctors. They write orders to treat you without having looked at your chart first. I'd been on ceftin for 5 days. At the time of the culture, I had been on it for 3. Obviously, this strain of bacteria was resistant because if it was going to work, it would have already begun to do so. In addition, she had been the one to call in the antibiotics previously; she should've known I was already on cefin. I then told her how oral antibiotics weren't effective because I didn't absorb them properly, and IV antibiotics are best. Since I have a port, there is no reason to not get them at home. No reason to have me get so sick that I need to be hospitalized. It is better to be proactive, start the IV antibiotics, and prevent a hospitalization, especially since I am more at risk of getting an inflection in the hospital. With the trach, my lungs are more stable. Thankfully she agreed.

Though I love Kathy, my nurse, she can be quite frustrating. She has strong opinions, some of which I don't agree with. She had screwed up the IV Benedryl and my chance of using it because of her belief that it could cause a potential side effect in the possible future. When I told her about the IV antibiotics, she was not supportive and in fact she suggested they do a second culture because mine may have been contaminated. Her argument was that it should've been done at the doctors office or by the VNA. Personally, I was insulted by this. Originally, I had wanted the doctor's office or VNA to collect the sample, but neither would do it. The only reason I wanted them to do it was that I had never done it before, and they had more experience. But because they wouldn't do it, I had to. I was a biology major and had worked with cell culture before. Plus, it's not exactly rocket science. I swabbed the inner cannula both outside and in. I swabbed inside the trach, using sterile technique, and coughed into the cup. Everything I had was clean and sterile, so there was no chance of contamination. Anything that was cultured was from my own body. If a doctor or nurse had done it, there would be the chance that they could contaminate it with their own germs, by breathing on it. So I was kind of insulted to have her suggest that I had not done it right. Had a second culture needed to be done, it would have postponed treatment. By the time it had been done, sent to the lab, and results obtained, it would've been Monday, and I would've been sick for over a week. In my opinion, prompt treatment was important and the sooner I got on the IV antibiotics, the better. I was really trying to avoid the hospital. Thankfully, she didn't call my doctor. My doctor was able to get it set up through my oxygen company. This was best because they were able to get it to me that night, whereas had they used BioRx, though I love them and am happy with the IVIg, I wouldn't have been able to start until it had been mailed to me. I am proud of myself for advocating for my care and getting what I needed. I am very glad I did because my lungs have gotten worse. I am short of breath and not really able to use the pmv. In addition, I began running a fever. So while Kathy can question whether I had an infection, it was obvious I did. I know my body, and a change in my trach indicates an infection. I know when I have an infection and when I do not. It's more obvious with a trach because the mucous coming up is directly from my lungs. So any indication of an infection is from your lungs. The frustrating part is that I've only been home two weeks and already have an infection. I didn't when I was in rehab. One of the tenets is coughing a lot, so I am concerned I got it from him, but who knows. Just hope this won't be a trend. I know I'm at greater risk since I now have the trach and because I'm in contact with many more people and all the potential germs they carry. In rehab I was fairly secluded and protected. But I can't exactly live in an institution.

Not Traditional Treatment

Well, I finally made it to the pain clinic in hopes to resolve some of my pain issues. I mentioned to Linda what I had been getting in rehab in regards to pain control and how it had been helping. We discussed that though non-conventional, IV Benedryl had the greatest effect. Linda was open to the idea. I basically told her how frustrated I was to go from nearly absolute pain control back to complete out of control pain again. Had I not had the time in rehab and had not had my pain effectively managed, that would be one thing. Prior, I hadn't known pain management. The Fentanyl patch is an improvement over the oxycotin, however, the lowest my pain level has been since coming home has been a 7. In rehab, I was able to maintain a 4 or at most maybe a 6, if severe. I used 4mg po dilaudid ~ 2-3 time/day, and some days didn't need breakthrough medication at all. To me, it is unacceptable to have to live this way. She did not want to switch to 4mg tabs of dilauded citing that she wasn't comfortable with such a huge dose increase. Apparently dilaudid even po is much stronger than percacet. But she was willing to write for 2mg po dilaudid 4x/day. I know that this will probably not be sufficient, but it is a start and a move in the right direction. One of the things I have learned when dealing with the pain clinic is try to be conservative with changes, be willing to accept smaller changes than you suggested in hopes of working towards your desired outcome, make suggestions as if someone else (VVNA, doctor, other person of noted authority other than you) had the idea and thought it would be helpful, back it up with evidence as to why it may be helpful, appear gracious and understanding when agree to change even if it it's not exactly what you were hoping for, and be willing to compromise. Yes, the ideal would have been 4mg but in seeing what I had taken while in rehab, she was willing to switch from Percacet to Dilaudid, which is the important thing. One step at a time. Often this is frustrating. It shouldn't seem necessary to play such games. It shouldn't be necessary to take each step and not just go right to what is known to be effective, as I am the one that suffers in the meantime, but it is the way of it. On the plus side of things, she was open to IV Benedryl as a help for my pain and was willing to write for it, so long as she talked to Dr. Steingart because he basically maintains my port and she wanted to make sure that he would watch things - didn't want me to get an infection, or clot, or whatever and has no knowledge of ports. This seemed reasonable and understandable. So I left there full of hope.

Well my hope came crashing down a few hours later when she called and told me the Benedryl would not be possible. She had talked to Dr. Fejos, who her license is basically under, and he wouldn't let her write for it because it is not "traditional" treatment for pain management. Well honestly, it doesn't get any more frustrating than this. Here is a non-narcotic treatment that seems to help manage my pain, which in my book is a good thing for two reasons - it helps manage my pain, and it is non-narcotic. I hate taking narcotics and being dependent on medication. Then, despite having a valid prescription wouldn't get it because of a possible side effect in the possible future. Then, the pain management specialist was willing to write for it, but was vetoed by her boss because it's not "traditional" treatment. It's all one roller coaster ride - up, down; hope, despair. Of course, we have to remember that I nearly left the pain clinic because of Dr. Fejos.

Interesting to think that I first went there just about 1 year ago. I initially went there because the pain in my bones and joints had gotten extremely sever and none of my doctors would write for pain medication. I was living from ER script to ER script. I had seen a couple of "pain specialists" who were not truly pain specialists. Most were spine specialists. One guy had me in spine surgery within less than 5 minutes of walking in the office. Shook my hand and was like, "so when are we scheduling your surgery?" My surgery? What? Um.... no thanks. No one is touching my spine, thank you. Interestingly soon after that I ended up at Bristol spine and Pain Center. The problem with finding a pain clinic is that most centers aren't truly pain management clinics. They deal with spine problems, or back injuries or accidents. They don't do true pain management and not from a chronic condition such as a primary immune disorder. If you can find a cancer center, sometimes that is best, but some only take cancer patients. Others don't do medication management. How is this possible, you ask. They one do
"interventional" pain. This means spinal shots, never blocks, physical therapy, even biofeedback - no medication. It was so frustrating. The other problem they don't deal with generalized body pain, and my pain affects just about every area of my body. Once you do find a pain clinic, you can't just make an appointment. You have to get your doctor to call in a referral, ect. When you're in massive amounts of pain, all of this is difficult.

Finally, I got into see Dr. Fejos. The first thing that was important to me was that he did medicaiton management - Yes. Okay, good. Then I asked if he had heard about primary immune deficiencies - yes. Another gold star. Had he had any experience with primary immune deficiencies - yes. Maybe two gold stars for this one. Some doctors had heard about them, but few had any experience. Did he have any current patients with PIDD - No. Okay, but there's nothing against that. We are a rare breed. How many patient had he treated in the past. Well none. Wait a minute, I thought you said you had experience. Well, what experience do you have? Well, I read a case study in a textbook back in medial school. The ultimate let down. That's not experience! I went on to talk about my pain. How it was in nearly all my bones and joints and due to my immune disorder. Explained that I'd had to stop doing jazzercise and been in the ER multiple times, and that my pcp was uncomfortable writing for pain medication. He asked what was helpful, and told him 4mg po dilaudid. He then said that it was beneath him to write such a low dose. That had I needed 15mg, that was worth pain management, but 4mg certainly not. He said, that any monkey could write a script and that could be the job of any med student, not someone of his caliber. When I once again explained that my pcp would not write, he said that he would talk to her, but that he wouldn't write such a low dose. Ah .... again, back to no option. I was so frustrated, and in my opinion, case closed. Wasn't going back there again.

Again was living off ER pain meds. I would ration them and when I ran out i would have to go back to get another script. It was not a good system, but I had no other choice. I looked for other specialist but couldn't find any. Finally, I went back to Bristol to try again. This time, I got an appointment with Tammy, the PA. She was more helpful. She ordered an MRI of my hips and spine; my pain originated in my hips. Then, gave me low-dose percacet. The MRI was a disaster. I didn't know how claustrophobic I was until I got in and freaked out; ended up crying hysterically. The MRI didn't show anything earth shattering - some arthritis in my spine, but not much more than that. The arthritis had been expected, and wasn't severe enough to cause the degree of pain I was experiencing. I didn't expect to have ligaments, fractures, or anything like that. I hadn't had a back injury or accident. The problem was deep within my bones and joints. The problem was the chronic, debilitating pain all over. In December, the VNA got involved. They were concerned with the extreme pain level and I was put on oxycotin - a long-acting pain med in addition to the short-term med. Then, I was also switched to seeing Linda, the nurse practitioner. Seeing Linda changed everything. Of course, it also helped that I was able to show her the article that was written about me and lung problems. After reading the article, she seemed to "get it."

It's just so frustrating because in rehab my pain was managed and now it's not. It seems so easy but because it's not "traditional" treatment, they won't order it. It could be standing on my head, and so long as it worked. I'd do it. I just don't want to be in agony all of the time.

Unless a person has lived in pain, they cannot understand the devastating effects it can have. One of my friends termed it, "pain brain." All you can think about is the pain. Sometimes, the pain takes over my body. It's as if I can feel the nerves firing in my body, down my spine. It is unbelievable. Every part of me hurts. It over takes all my thoughts and I think that surely, if this continues, I will go insane. It's unbearable. My thoughts become muddled so that the only thought that continues to exist is the extreme pain. I forget things because the pain takes over everything. All I want is to curl up in a ball and sleep, but I cannot sleep when my body is in such deep pain. I do not want to move because doing so just makes it worse. I fear to even breathe. It takes over my life. I cannot stretch or do pt. It effects your emotions. You get depressed. Everything becomes tainted by it.

When I saw Linda, it was as if she expected the trach to have fixed everything. Before I had severe lung pain. As my lungs have gotten worse and begun to fail, I suffered with severe lung pain. She expected this to be gone. But though the trach has helped my breathing, it has not fixed things. It is not a cure. It will not stop the deterioration or progression in my lungs; however, it will make it easier to breathe. It will give me more time. But my lungs are still failing. They still hurt. The area around my trach hurts, and though she expects that things are all better, this isn't the case. She thought that since being home, my pain would be less than it was in rehab; however, this is not true. At first I didn't understand. I, too, expected it to be less since I wasn't getting as intensive therapy. But it is not, it is more. When I thought abut this, it does make sense. Though I am not doing as much therapy, I am in fact doing more. In rehab, you do therapy, but when you're not in therapy, you are either sitting or laying in bed. Here, I do things all the time. I am in the wheelchair most of the time, but I am constantly doing things. I wheel myself from the bedroom to the kitchen, get my drinks, prepare my food, put dishes away, ect. I am constantly doing things and pushing myself from one place to another. By doing more, this is going to cause more pain. Granted this doesn't change the fact that only a certain amount of medicaiton is prescribed. It is frustrating how others don't understand that. the trach was not a cure. Things are not all better. I just wish what anyone else wishes, to be free of pain.

What a Pain

Since I've been home, I've been in so much pain. It's been absolutely horrendous. I am in agony all the time. My bones and joints hurt. My lungs hurt. My trach hurts. I just can't win. I am taking pain meds non stop. I take them every 4 hours, as frequently as possible. Often it seems as if I am looking at the clock, waiting for it to be time for me to be able to take pain medication. The lowest my pain has been since coming home is a 7.

Before going to rehab, this was somewhat acceptable. Since I never seemed to get complete relief from the medication, any relief was okay and acceptable. I just figured that for whatever reason, I was a person whose pain couldn't be adequately managed. I had pretty much given up on pain management. As long as my pain wasn't a 10, I was okay. It was when it got above this, that I couldn't deal with it. All of this changed when I went into rehab. In rehab I had adequate pain management. I was getting IV Benadryl around the clock. I was also getting oral Dilaudid as breakthrough medication. Though my typical pain management schedule was for Percocet as breakthrough medication, the Benadryl and Dilaudid combination was more effective. With the Benedryl, I needed less medication to effectively manage my pain. It helped the narcotic pain medication work more effectively. With it, I was able to need less narcotic pain medication. I was only needing 2-3 doses of the Dilaudid per day. Some days I didn't need any. My pain was well managed. This was a miracle to me. I had never had adequate pain management. I had pretty much given up hope that it would exist.

When I was discharged from rehab, they wrote a prescription for me to get the IV Benedryl. Before going to rehab, I had been taking oral Benedryl several times a day to control the itching attacks that I get. On an average day, I was taking 6+ doses of breakthrough narcotics and at least 5-6 doses of oral Benadryl. In the hospital, I was only needing ~2 doses of breakthrough narcotics and 2-3 doses of IV Benadryl. Once home, however, Kathy, my infusion nurse, didn't want me to use the IV Benadryl. She said she was concerned because it carried the risk of possibly developing a dyskenesia, or abnormal muscle movement. She said with the challenges I was already experiencing, this was the last thing I needed. Now while I love Kathy and she is an excellent nurse, she is also very opinionated. When she has an opinion about something, she gets stuck on it. Well this was one of those things and she contacted Dr. Giannini about the fact that she strongly felt that the IV Benadryl was not a good idea. I understand her concern, and if I was a typical patient, it would be valid. Of course, if I was a typical patient we wouldn't be discussing it because traditional methods would be successful, but I am not a typical patient. In my opinion, 2-3 doses of IV Benadryl is much better than 6+ doses of oral. Using IV Benadryl, which seems to make the pain medication more effective and thus make it so I require less narcotic pain medicaiton and help control my allergic reactions, seems better than needing 6+ doses of narcotic pain medicaiton. I also don't understand why IV would have this potential side effect if oral did not. Wouldn't you develop it much faster with the 6+ doses/day than 1-2 doses/day? Isn't it better to use non-narcotic medication vs. narcotic pain medication? I also have a hard time understanding why we should veto using IV Benadryl and its benefits for a potential side effect that could occur in the future. This is not even something that will happen, but something that "may" occur as a "potential" side effect. Let's face facts, I'm not going to live forever. My life expectancy just with the CVID alone is 20 years after diagnosis. I have been diagnosed for 5 years. I will be lucky if I get another 5 years out of this situation. During this time I want to be as comfortable as possible. I have a hard time justifying not using a treatment based on potential side effects in the future. I am more concerned with right here, right now. I want to be comfortable. I don't want to be in agony constantly. Being in this much pain is not fair to me. I can't do the things I should and need to do. I can't do as much physical therapy as I should or need to because I'm in so much pain. Pain makes physical therapy and exercising near impossible. The pain completely takes over my body and my life. It's all I can think about. I can't do the things I enjoy because all I can think about is the pain. It is constant, never letting up or giving me a break. Sometimes the pain is so severe that I can't endure it, and worry that I will go crazy from it. No one should have to live this way. It's not fair. It's not a quality of life. I hurt all over. It's depressing. It dampens your mood, and it makes it hard to concentrate on things. Thoughts of pain consume every aspect of my life. The most frustrating thing is that it doesn't have to be this way. The pain can be managed. I have proof of this because it was well managed the entire time that I was in rehab. There is no reason for me to have to suffer. It wasn't difficult to treat. IV Benedryl is a benign medication. It's not difficult to obtain, nor is it a highly regulated medication like many narcotics. It is a simple antihistamine. In addition, it is easy to administer, something I'm capable of doing without a problem. There is no reason for me to be in so much pain when the solution seems so easy. Of course, since Kathy didn't support it, she called Dr. Giannini, my pcp. My pcp listened to her because she is a nurse and obviously knows what she is talking about. When the VNA later called Dr. Giannini to try to get it set up for me to administer the IV Benadryl at home, Dr. Giannini refused to accept the script in support of it. Therefore, I am not able to get it as a medication. So, though the medication helps manage my pain effectively, and with it, I need less narcotic medicaiton and my pain is managed effectively, I can't get it. Instead, I must suffer in agony. It's horrendous to have something so simple, so available without complication, and yet unable to get it. I wish sometimes that they would live in my shoes and see what it's like. Maybe then they would be more understanding, and stop limiting potential treatments by thinking outside the box to improve my quality of life.