It has been a tough couple of days. For some reason my pain has been completely out of control. Judy, my palliative care nurse was able to get Dr. Giannini to write for the IV Benadryl. As I've said before, the IV Benadryl helps make the dilaudid more effective and last longer. I was getting IV Benadryl with the IV antibiotics, but since I won't be getting any more IV antibiotics, I needed it to help with my pain. It took many days to try to arrange it. First, for some reason it was difficult to get the order to professional home care. They faxed it multiple times, but for some reason, the pharmacy didn't get it. Then, it was a task to get it and have it delivered. I was in such severe pain that I was crying. I couldn't sleep as I was woken up by the pain shooting through my body. Each breath is pain staking. Even the pain medication doesn't touch the pain. I worry that I'm becoming dependent and that is why it is growing less and less effective, but the pain is getting so much worse. I know that soon we will have to change to either liquid or IV medication. It frustrates and scares me.

I had another episode of bleeding from the trach. I didn't do anything for it because I know there is nothing they can do. I know that I will not bleed to death and it looks worse than it is. You never get used to coughing up blood. My cough is worse as well which proves to me that once again the IV antibiotics didn't kill whatever it is in my lungs. My sats have been low as well. I am on 10L and can't really go any higher. I was in so much pain yesterday that I couldn't go to the movies with mom as we had planned. I know it hurts her to see me in pain.

I met with the hospice team on Thursday. They would like me to meet with Dr. Steingart before stopping the infusions. They talked with my pulmonology team, who of course said that they feel that my condition is not terminal. Even though my condition continues to decline, they refuse to see it. They then called Dr. Steingart whom I haven't seen since I got back from rehab, at which point I wasn't as sick. He hadn't known about my deterioration. Obviously, he was caught off guard since last I was doing better and the trach had helped my breathing. When they asked him about life expectancy if I came off Igg, he was unable to give a fixed amount of time. I know that it's okay because Dr. Giannini is the main person managing my care and she is able to sign the paperwork. The hospice team does want to make sure we have exhausted all options of treatment. I wish that we could find something that would help and make a difference. It is my one true wish. But I don't want to keep suffering. I worry this will keep me off hospice. I don't want to be on hospice if there is hope in something else, but I can't live like this either - with every breath being agonizing. I pray to breathe easy.

Another cyster (person with cystic fibrosis) died last night. Cystic fibrosis is a genetic disease that because of a gene mutation they have no chloride ion channels in their cells. This causes their mucous to be very sticky. They have a lot of lung problems. They get a lot of infections because their mucous is so thick and sticky that they can't cough it up. The many infections damage their lungs, somewhat the same as me. It is almost inevitable at some point they will require a double lung transplant as their lungs go into lung failure. For some, they get too sick to qualify for transplant. Others, for some reason, may not be candidates. In cases such as these, they die at a young age. Hannah was 20. It makes me so sad to think of all those that have died way before their time. I think of many others with different lung diseases that cause them to die young. It's just not fair. This is a world in which I wish we had more answers, more solutions. Medicine has come a long ways over the years. What used to be open heart surgery is now done laparoscopicly. Despite the advances, still many die too young. I grieve for all the mothers and fathers, sisters and brothers, family members and friends, boyfriends and husbands, who are taken from this world too young. They are such wonderful amazing people, whose lives are cut short. How I wish for me and those like me, there were ways to prevent this and give them the opportunity everyone deserves, the opportunity to live life.

Transition to Hospice

I had my IVIg today. I have enough Igg for one more infusion, and then I either have to order more or stop the infusions. This is such a hard decision to make. My goal has always been to make it to Christmas. I was trying to hold off transitioning into hospice until after Christmas because it was very important to me to make it to Christmas and have an enjoyable holiday with my family. I have pretty much reached my goal and think that even if I were to go into hospice now, I'd still be here to enjoy Christmas. The hard part about all of this is deciding when to stop the Igg. I finished my last dose of IV antibiotics today. I have decided that this is my last dose of IV antibiotics. I won't be doing anymore antibiotics. Though this was a difficult decision to make, it is not nearly as difficult as deciding when to stop the Igg. Since September I've been fighting this infection. I have been on countless different IV antibiotics. Despite all the antibiotics, I still have the infection. Nothing seems to be killing it. The infection seems to start to get better when I start on the IV antibiotics, but somewhere in the 14 day course, the antibiotic seems to stop being effective, and the infection once again takes hold and gains the upper hand. I have just come to the point that it doesn't make sense to keep doing different IV antibiotics when they don't seem to be helping. It makes no sense to put these drugs into my body, medications that cost a lot of money, when they don't seem to be effective. There comes a point where you have to say, enough is enough. I am tired. My body is tired. I am the one that does all the medications. I don't have someone who does it for me, and as I grow weaker, as I decline and get sicker, even the simplest of tasks becomes overwhelming.

I tried to talk to Kathy again today about hospice and discuss with her that I was getting closer and closer to transitioning into hospice. She refuses to acknowledge reality. She wants to bury her head in the sand and live in denial. I know this is hard for her. I know that she has come to care deeply for me. I am not just a client/patient. I am more than that. I am like a daughter and a friend. She does not want to lose me, but denying reality is not going to help things. It won't make it different.

She looks at this as me giving up, throwing in the towel. But I am not giving up. Had I just been diagnosed and refused to even try treatment, that would be one thing, but I have done 6+ years of treatment. I have done IVIG. Most people do infusions once every 3 or 4 weeks, I do them once a week. I have given my life up to this disease. First I gave it my career in nursing, then I gave it my career in research and my Ph.D. I gave it animation and finally my tutoring. I have done the treatments,the tests, the medications. I have gone for 2nd and 3rd opinions and consults. And what have I gotten in return? She wants me to seek another opinion, but I have already done that many times. The only thing the doctors in Boston could offer me is more questions, no answers. If there really was hope that they would figure things out and find a treatment that would help me, I'd be more than happy to do it. I don't want to die. But, there are no answers. She says to me, "But they don't have a terminal diagnosis. They don't have an organism that is causing this severe infection." This is true. I could go to countless more doctors and undergo many more painful and invasive tests in hopes of having a diagnosis or coming up with a cause for my decline, but to me this is not important. It may make my treatment providers feel better. They will have answers and an explanation. They may feel less like a failure, but to me it is not worth it. The diseases and diagnoses they would be looking at are for progrssive terminal illnesses. Is it that important to have a reason why when there is no treatment? It won't change things. The result will still be the same. I will still be dying and there still won't be anything they can do to prevent it. The difference will be that I will have wasted some of the precious time I have left in doctors' offices or hospitals, undergoing painful and invasive medical procedures. For years I have gone from one specialist to another. I have heroically withstood procedure after procedure. I rarely cried or complained. I took the tests and treatments with strength, hoping that it would lead to answers, but it didn't. Now that my time is coming to an end, I dont want to waste the precious time I have doing procedures and tests. I want to spend the time I have with my family and friends, enjoying the time I do have and making memories that will last after I'm gone. It kind of reminds me of last summer when Abby got sick. The vet offered to do an X-ray and blood work and other tests to fully diagnose what was going on. When I asked how this would change her treatment protocol, I was told that it wouldn't. She'd be given the same medication. The difference would be they'd have a concrete clinical diagnosis. I chose not to have the tests done and instead just give her the medication. Why put her through those tests and be charged the extreme fee of having the tests when the treatment would remain the same. Even if I did all the testing, it wouldn't change the treatment. It may make my doctors feel better because they'll have a concrete cause of why this is happening, but it won't change anything for me. I will still be dying.

I understand that this is not a position that doctors are comfortable with. They went into medicine to save people. This is especially true because I'm so young. If I was 70 and had lived a good life, they would not have as hard a time accepting this as they do with someone my age who has not really even been given a chance to live. They feel powerless, and they feel like failures. But I don't view them as such. I am very grateful because they have given me more time than I would've had otherwise. Though the trach wasn't a cure, it gave me more time. Even an extra couple of months is something to be extremely grateful for, but it is important to realize that medicine doesn't have all the answers. It can't cure everything. There are times that things happen that we don't understand why. It may seem unfair. But I hold no anger at the circumstances. I am very grateful to have been given what I have. Medicine doesn't have all the answers and it can't fix everything. There is a time when you have to give it up to the Lord, and trust in Him. We like to think that we have control over all things. In truth, we have very little control over things.

I have found that how a person responds to me and the fact that I'm dying largely depends on how comfortable they are themselves with the topic of death. Death is a natural part of life. We are all dying. From the moment we take our first breath, we have already begun to work towards taking our last. Some of us are dying sooner than others, but the fact remains, we are all dying. It is impossible to escape this. Death is a natural part of life. While many view it as the end, in reality, it is just the beginning. I embrace this time of my life. I have struggled and suffered for many years now. My body has grown weak and no longer has the ability to fight. Instead, it embraces death as a freedom from the pain and suffering I have endured.

Right now life is such a struggle. Every breath hurts. The purpose of the trach was to increase my quality of life. Though I am grateful to have had this time, my time here on this earth is coming to a close. Every person has their line of things they are willing to tolerate, and the point at which they say enough is enough. I have reached that point. I am tired. I am weak. My body is weary and needs a rest. It is tired of fighting to live every day, every moment. I have fought so hard for many years, but now it is my time for peace, time for my suffering to come to an end. I do not have a quality of life. Kathy often says that she would never be able to do the things I do every day. If she would not want this quality of life herself, then how can she expect it for me? Do I not deserve to be free of suffering? I am not giving up. I have done treatment and therapy for many years, but what is the point if it has no benefit and only increases your sufferring? Don't I deserve to be at peace? She asked what terminal diagnosis I have. I don't have something concrete such as cancer. But I know that I am dying. The infusions are no longer working. All the medications I take and therapies I do seem pointless. If they were working, I wouldn't be in this position, and if they truly aren't working, what is the point on continuing them? There comes a point when you have to reach acceptance and give it up to the Lord. I am yours God. Your will be done. I know that I am powerless. I have no control over my life and death. If I am meant to die, nothing on this earth will keep me here. In the same way, if it is not my time, I will not go. Whether I go into hospice or not, will not matter. Going into hospice won't make or prevent me from dying. It will, however, determine my comfort level through my final journey.

The hardest thing about all of this is giving up the Igg. Logically, I know that it is no longer working. If it was, I would not be as sick as I am, but actually stopping the Igg is a difficult step. I don't want to die. If there was another option, if I were to get better, if there was a treatment, I'd be most happy. Since being diagnosed, I have fought for my Igg. To me, Igg is synonymous with life. No Igg is equal to sickness and death. Though I know it isn't working and doing no good, giving up the Igg is a true step of accepting that I will die. I know that when I stop the Igg I will get sicker and my pain will get worse. I am terrified of sufferring, of feeling like I am suffocating to death and not be able to do anything about that. This is what I am afraid of. I do not know what scares me the most, the idea of dying, or the idea of living indefinitely like this. I don't understand how people can say they wouldn't want to live as I do but then judge me and expect md to continue to suffer in this way. I have no quality of life. My body is dying. It is shutting down. It scares me to see how I have deteriorated. I can no longer shower myself. I am too weak. When I came home from rehab my weakness was due to muscles being weak. This is no longer the problem. The problem is not something that can be treated with physical therapy. It is a result of my body shutting down. My lungs are bleeding and falling apart. There is no way to treat this or fix it. Each breath is immensely painful. I can no longer do things for myself. I need help showering and dressing. I am no longer independent. I can't drive. I can't just get up and go somewhere whenever I want. I am reliant on others to get out of the house. I can no loner walk. I can't tutor. I tire so easily and after being awake for more than 2 hours, I am so tired that I must sleep. I fall asleep talking in mid-sentence. Technically I need to be vented, but I really don't want to go that route. I grow weary at having to do everything, care for everything myself. It is a daunting task. I ache. I hurt. I pray for relief. Hospice will give this to me. This is not easy. No person my age should have to be making these decisions. People make this process even harder by judging me. How I wish they could accept me and support me in whatever I decide is right for me.

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.

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.

Hospital Update

Well, I am home from the hospital. I was discharged on Wednesday. Unfortunately, I wasn't discharged in time to make it to go to Boston. I was able to reschedule my appointment for September 22nd (2 weeks from my original appointment). I have been extremely impressed with the people in his office. Not only are they accommodating, but they actually call you back. They put me on a cancellation list, and should anything come up sooner, they will call me. At this point in time, I just want answers. I am so tired of being sick and in the hospital constantly. I DESERVE to have a life, and I can't do that when I'm in the hospital constantly.

Anyway, sorry, back to my hospital update. I was discharged on Wednesday. I can't say I was truly ready to be discharged, but the doctor wanted to discharged me, so discharged I was. See, the way it works is that my doctor rounds in the hospital on the weekend or holidays. During the week another doctor rounds. This doctor doesn't actually see patients. Strange, I know.

Tuesday they tried to allow me to eat. That didn't go so well. Caused a lot of pain and extreme nausea, but I stuck through it. I guess my CT scan showed what looked like a small ovarian cyst. I was sent down for an ultrasound. The GYN that did the ultrasound was amazing. She said that she did not think that it was a cyst, but rather a natural occurrence. It is less than 1 cm, so she said there is no way that something that small is causing my upper quadrant abdominal pain. She did not see evidence of any fluid indicating something had burst or was infected. So good news there.

Dr. S (the doctor that doesn't see patients) came in on Wednesday and was adamant that my pain was being caused by my non-existent (only a few millimeter) cyst. Apparently he knows more than the GYN, in which this is her specialty. She at least sees patients. Then he went on to tell me that women get cysts nearly every month. Okay, that is true in some cases. That these cysts can cause fever and increase WBC. Well, if they get infected, yes. And they are mildly uncomfortable and treated with Tylenol and Advil. Whoa there! I know people who have had ovarian cysts and they can be EXTREMELY painful. He then went on to tell me all about what it was like to be a woman and have a menstrual cycle - because his last menstrual cycle was when?

He then wanted to discharge me without finishing the course of antibiotics. All through the hospital stay, I'd been on IV Cipro. My lungs had mildly improved from when I was admitted, but I was still fairly wheezy and junky. I was able to talk him into prescribing enough oral antibiotics to get me through Saturday, which would be 7 days of antibiotics. I tried to impress upon him that whether or not the exact infection causing my problems was identified didn't matter, but that stopping any course of antibiotics mid-cycle is dangerous and how antibiotic-resistance develops.

This doctor obviously does not understand PIDD. I often think doctors should actually read a patient's chart before going in to see them. I also think that if that patient has a diagnosis that a doctor does not understand or is not knowledgeable about, that he/she should educate him/herself on this disease. Everyone has access to the internet, so there really are no excuses. I often wonder if all doctors acted as med students with the need to impress their attending, if perhaps patients would get better care. I wonder if some doctors just become lazy over the years. I have ceased expecting doctors to do this, but I get so tired of playing both doctor and patient. I do not mind educating people, but it's hard when you feel like you're talking to a brick wall. Well maybe talking to the brick wall would have more of an effect and it would be more responsive.

So, I am home. My lungs are not doing so well. The small improvement I felt in the hospital is slowly being lost. I don't know if the oral antibiotics are just ineffective or what? Maybe my body is not absorbing them properly? It seems that every time I take oral antibiotics, they do nothing. I do not know why this is. I also have horrible thrush. I've tried OTC products, with no luck. My doctor hasn't prescribed anything. It's down my throat and possibly in my stomach. Is this possible? Would that be why I feel so sick?

Again, more questions asked than answered. Feeling a bit discouraged. One and a half weeks until Boston.

"I think I can. I think I can. I think I can." - The Little Engine that Could

Hospital

While there is never exactly a "convenient" time to get sick, as no one ever wants to be sick, but I often seem to get sick at the most inopportune moments. When the last thing I want to do, or can afford to do is get sick, when it's actually extremely important that I stay healthy, that is most often when I do exactly that. During these times, I try to will my body to stay healthy. It's as if I try to bargain with it - just stay healthy until... Unfortunately, my body rarely listens.

Tomorrow, I was supposed to go up to Boston for a consult at Boston Children's hospital. So of course, last week, my lungs decide to get crappy. I could feel the mucous building up and getting congested. I got to be so tired and worn out doing simple things, gasping with every breath I took. Instead of decreasing the prednisone, I stayed at 40mg. I needed my body to make it. On Thursday night, I began running a fever. Now I never run  a fever, so when I do, something is seriously wrong. Friday, I called the doctor and was told to go to the ER. I didn't want to go to the ER. I wanted him to give me antibiotics and let me do it at home. I was not at crisis point where I would normally go to the ER. My lungs were bad, but not horrible. I was holding my own. Of course, I knew without antibiotics, this wouldn't last long. I was very frustrated.

Well, I made it through Saturday. Watched a move with my sister and had a good diner. It actually wasn't the lungs that did it this time. They played a part, but the ultimate issue was the fact that I started with severe upper quadrant abdominal pain. It caused me to double over in agony. I took a pain pill, but it didn't even touch it. 2 hours later, I took another. The pain had worsened to the point that the Zophran ODT wasn't even working to counteract the nausea. My breathing, which had been difficult before any of this started, was worse. I couldn't take a deep breath at all. So finally, and very reluctantly, I went to the ER. The ER was insane on a Saturday night of a holiday weekend, but I got right in since my o2 sats were only 81%. I had a temperature, and my blood work showed a WBC (white blood cell count) that was through the roof and an elevated lipase level. Lipase is an enzyme in the pancreas that digest fat. Elevated lipase usually indicates pancreatitis. With the PIDD, my WBC rarely goes up.

The ER doctor was actually very good. He stayed on top of things and consulted my opinion on what worked best for me. This is very rare as usually ER doctors do not understand PIDD at all, have never even heard of PI, and thus, often treatment is horrible. In addition, usually if you are in pain, ER doctors do not listen. If you have been there even once before for a condition requiring pain medication, you can be called "drug-seeking" or an "addict". This is very frustrating. While I understand the need for doctors to be cautious, I have a serious medical condition which can leave me in debilitating pain. If I end up in the ER in pain, it's usually quite severe and caused by another issue such as kidney stones. So it was quite refreshing to be asked what works for me and be taken seriously. Apparently the ER doctor had taken care of me here previously and had also taken care of me at Baystate, which is the hospital I used all throughout college. I have always received excellent care there and because it is where I was diagnosed and being treated for my immune disorder, most doctors there are familiar with my case. Needless to say, though I was hoping to avoid being admitted, I was. I do agree that it was needed, especially with my WBC being so high.

Overall, it's been a fairly good admission. I have had some minor issues with pain management, but overall my pain has been controlled. I am on IV antibiotics. Thus far, I am NPO, meaning I am not allowed to eat anything, to hopefully help the pancreatitis. My biggest concern is that I will have to reschedule my appointment in Boston.

Migraines

Recently, I have struggled with HORRIBLE migraines. In fact, I'm not sure the word "migraine" covers it. It starts above my eyes and works its way to the base of my skull and c-spine. The pressure in my head is enormous. I feel like my head is in a vice-like grip. I've often had headaches associated with my infusion, but these are different fro my infusion headaches. At first, they started 1 - 2 times a week. This was annoying, but I could live with it. I began to be alarmed when I started having visual disturbances with them. I'd get these black spots that would obscure my vision. Sometimes it would be so bad that my vision would go completely black for moments of time. I'd also have problems with positional changes. If I moved my head too quickly (bent down), I would get a stabbing pain through my head and my vision would go black for a few minutes. I consulted my doctor and she gave me Gabapentin. Gradually, this got worse. In stead of 1 - 2 times a week, I was having them 3 - 4 times a week. Last week I was finally able to see my doctor again and told her I needed a referral to a neurologist. Not only had the frequency and severity of these headaches increased, but at times I would black out completely. I'd find myself on the floor, not knowing how I got there. She increased the Gabapentin and gave me a referral to see the neurologist.

This past week, I had the worst migraine/headache I've ever had. After six days I began to get concerned. Each day it got worse, with the pain radiating down my spine. The pain was unbelievable. My skull felt like ti was going to crack open, and honestly, I would have welcomed that. I tried to get into to see the neurologist, but knew there was no way they could get me in for a new-patient visit in the next few days. They hadn't even processed my referral yet. Though my doctor had made the referral, the neurologist's office kept telling me they never received it. Then, they told me they needed notes before they could make me an appointment. Finally, I was unable to take the pain anymore. I was also not able to keep anything down (liquids or solids) even with nausea medication. My friends urged me to do something as a migraine lasing this long was severe and needed to be checked out. So, off to the ER I went. Thank goodness at the ER they were able to give me IV pain and nausea medication that helped. They also did a spinal tap (lumbar puncture). Though they did the LP to see if I had an infection that was causing the pain, the LP helped relieve the pressure in my head. In the past day or so, as my body has replaced the lost spinal fluid, the pressure in my head has begun to build and slowly the headache has come back. I hope this is not going to be a chronic thing. I cannot have an LP every week or so to relieve the pressure in my head each time it builds. The neurologists office finally called back yesterday. The ER sent them a referral, so I can now make an appointment. I hope I can get an appointment soon and get some answers.

Living in Pain

Many patients with PIDD live with chronic pain issues. Pain is often thought of as the fifth vital sign; however, unlike blood pressure, pulse, respiratory rate, and temperature, it is a little more difficult to objectively assess a patient for pain. This can cause pain to go untreated. Untreated or poorly managed pain can lead to other medical complications and problems. Doctors are not sure as to why patients with PIDD suffer from chronic pain issues, and the type of pain PIDD patients suffer from varies widely from patient to patient. Some research suggests that the immune system plays an important role in the development of chronic pain. This may explain why many PIDD patients struggle so much with chronic pain issues.

I have struggled with chronic pain issues for over two years. Mainly it has been my hips and joints that are the problem, though recently it has been much more than that. I have seen a rheumatologist and been told I do not have RA (rheumatoid arthritis). I have had CT scans and MRI's of my hips, neither of which show structural abnormalities. I have done physical therapy, which did not help at all. Basically, once again, I am a mystery. I had been prescribed pain medication by my primary care physician until recently when I had to switch doctors because I moved. My current PCP has told me that she is not comfortable prescribing pain medication.

Now, I have never been very fond of pain medication. I would much rather not take it if at all possible, but when the pain becomes so debilitating that you are nauseous, throwing up, curled in a ball in the middle of the floor, and praying that it just ends soon, there really are no other options. You have to live and be able to function. It is at those times that pain medication is necessary. I do not take pain medication and even though it is a narcotic, I have been at the same dose for those two years. Since my PCP will not prescribe pain medication, when the pain has gotten too bad, I have had to go to the ER. The problem with that is many ER physicians are reluctant to prescribe pain medication due to the bad rap they have gotten from addicts. On several occasions I have been told that I am just drug-seeking. Well my response to that is that the ER physician only ever sees me in pain because who goes to the ER if they are healthy? Second, if I was an addict would I not need more and more of the medication to achieve the same result? If I was an addict would I be able to have 15 pills last me a month - two months, when they are prescribed to be taken every 4 - 6 hours? My pain has never been managed, but rather been treated on a crisis basis. I have been so fearful of running out of pain medication when I desperately need it, that I don't take it when I should. Pain medication should be taken before the pain gets bad to prevent it from doing so. You should not wait until you are a 10 out of 10 on the pain scale to take pain medication. Yet, this is what I do out of necessity.

Finally, in the past year, I have tried to seek out pain management doctors in hopes that they would manage my pain. The first doctor I went to see was supposedly a pain management doctor. When I called and talked to his office, I was assured that he treated pain, not just spinal disorders, and that he did do medication management. Well once I got there, it was a different story. I was told that he did not do medication management. He never prescribed pain medication. In fact, the only thing he did do was spinal surgery. Um.... no thanks.

So, on to the next pain specialist. First of all, I must say that there aren't really genuine pain specialists in my area. There are doctors that treat spine disorders and doctors that do spinal surgery, but no one that deals with pain management. So my next step was to find a pain clinic. I set up an appointment down towards the other end of the state with a pain clinic. Again, I had talked to someone in the office and was assured the do pain and medication management. I had been referred by my PCP who had other patients who had been to this pain clinic with positive results. Well, guess I wasn't so lucky. I met with the doctor, whom didn't even examine me. I sat on one chair, he in another, while he asked why I was there. Well, because I am in pain and need it managed. Hello? Why else did he think I went to a pain management clinic? It was not because I have a sore throat. I stated the obvious and told him my story. He said he had seen the MRI and CT scan reports and neither had revealed any structural abnormalities. Again, something I already knew. I told him the source of my pain was likely as a result of my immune disorder. I, then, went on to tell him about PIDD. I asked him if he has heard about primary immune disorders. To which he responded, "Yes." Okay, so now I feel hopeful, but he still has that puzzled look on his face as if to say he is not sure how this is relevant. So then I ask him if he had any PIDD patients. "No." Well, maybe he just didn't have any current patients. One can have hope, right? So then I ask if he's ever treated any patients with PIDD. "Well, no." Okay this is not a good sign. My good feeling is quickly fading. So then I ask if he knows about primary immune deficiencies and their complications. "Yes." Now I'm confused. He has heard of them, he supposedly knows about them, he does not have any patients, nor has he ever treated a patient with PIDD, and he has that completely mystified look as if to say, "why are you wasting my time with all of this." So then I ask, how he knows about primary immune disorders. To which he says, "I read about it in a case study in some textbook back in medical school." Um... not exactly the same, and I would not classify as "knowing" about them or being an expert in them. I attempted to explain PIDD to him, but with no amount of success.

In the end, he refused to write a prescription for pain medication, nor even discuss pain management. I did not have a structural abnormality and thus, in his opinion, no logical cause for my pain. He went on to tell me that it was "beneath him" and a "waste of his resources" to prescribe pain medication. In his words, "any monkey can prescribe pain medication." He then suggested I get my PCP to write for it. Well, that is why I was there, because my PCP would not write for it because she was not comfortable doing so. He said that she should have no problem writing such a little amount (15 - 30 pills every month - 2 months). Yes, I agree, but that is not the case. He then suggested that if I couldn't get my PCP to write for it, I should seek a new PCP. Easier said than done. Would he like to call the 100+ PCPs on the list my insurance company has and find one that will prescribe pain medication. Should I ask that question of them before I get the appointment, or does he really expect me to go in for 100+ new patient visits to find one that will prescribe? And why again, can he not prescribe? Is this not his specialty?

So I left, in pain, and quite discouraged. Here I had hoped that this would be my answer, and yet I am still in the same place I was before. I go home and then decide to call a few of the other pain clinics in the state and even one out of state, but within traveling distance. Who minds traveling if you get good treatment. In total I talked to four other pain clinics. I had now seen four pain clinics in the past year, two of which were spine centers because I did not yet know the distinction between a legitimate pain center and a spine center that dealt with pain, and talked to an addition six clinics - a grand total of ten clinics/doctors. Out of the phone calls I made, I got similar responses from everyone. Yes, they deal with pain and medication management. No, they do not just treat spine disorders, but other types of pain. No, they do not prescribe pain medication. Okay, how can you say you treat pain, deal with medication management, but do not prescribe medication? Finally, I asked the obvious question, well if you don't prescribe medication, what do you do? I was told they all do interventional pain management. Now, what is interventional pain management? Spinal blocks, nerve stimulators, and surgery. All of which, to me, seem to have some sort of invasiveness. They certainly seem more invasive than prescribing a pill. You would rather stick a needle in my spine, or even cut into my spine and insert a machine, or do some other type of surgery, then prescribe me a little pill? Does this make any sense? Maybe they make money based on how many spines they operate on or stick needles in, therefore prescribing a medication is just not lucrative enough. Then, again, if my pain gets any worse, I might be inclined to let them stick things in my spine or operate on it. If they screw up, I won't be in pain anymore. I may not be able to walk or feel anything else either, but I certainly won't be in pain. Not really sure how any of this would help if I had cancer, and there are certainly many types of chronic pain that are not related to the spine. Wonder also how these spinal therapies would help widespread chronic pain, such as mine, except by doing just as I fear, cutting off the feeling in my entire body. I guess if you gave me a nerve block in my cervical spine, the rest of my body wouldn't hurt. Of course, I wouldn't feel anything and I probably wouldn't be able to breathe because it would block the nerves to my diaphragm and breathing, but I wouldn't be in pain....