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.

Locks of Love

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

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

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

Consult in Boston

Last Wednesday I went up to Boston for my consult with Dr. Bonilla at Boston Children's Hospital. A lot was riding on this appointment, mainly the fate of my IgG and whether I'd be allowed to continue treatment, or I'd be taken off.

As my condition has deteriorated over the past year or so, I have seen a few specialists as consults. One suggested that instead of 10g of IVIg every week, I get 20g every 2 weeks. I'm not really sure how he figured his math, or why he thought that would make a difference since it  would cause greater peaks and troughs between infusions, but I never went back to see him and did not take his recommendation.

The next consult I had was with Dr. Alyea at Dana Farber for a possible bone marrow transplant. While he was excellent, he didn't feel that a bone marrow transplant was necessary at the moment. Should it be come necessary, he would definitely help me pursue it.

With the bone marrow transplant off the table, and still getting sick so frequently, I went to see Dr. Cunningham-Rundles at Mount Sinai in New York. She was supposed to be the leading expert in the field. I was especially interested with her work with IL-2 in conjunction with IVIg. Her idea, however, was to take me off the IVIg all together and "see what happens." She also wanted to do this so that she could do some testing, which can't be done on the IVIg. The problem with this is that to do the testing, you have to be completely off IVIg for at least 9 months. The idea that if you just take me off the IgG, I'll get better, is not logical. I have never had a working immune system, not even as a young child. How will I develop something I never had to begin with? In addition, I attempted to come off the IgG many years ago, shortly after I was diagnosed. I was living in Boston and the doctor there wanted a definitive diagnosis. The plan was to take me off for 6 months and do some testing. I made it 2 months. I got extremely sick and spent nearly 2 months in the hospital. Finally, I called it quits and went back to Springfield where I could get my infusions. My body is much sicker now and much less resilient, so the idea of coming off the IgG scared me. Yet, I felt I had no options. Dr. Steingart planned on taking her recommendations, after all, she was the expert. I was able to post-pone having him take me off until we met again in the fall. I wanted to enjoy my summer and did not want to spend it sick. 

Since my appointment, I have talked to other patients who have also seen Dr. Cunningham-Rundles in the past. I was told that this is what she recommends to all her patients. One guy she told that if he just had his gallbladder removed, he'd be fine. She is a researcher and her research cannot be done on IVIg. IgG is a therapy, not a cure. Once you are on it, you will most likely need it for the rest of you life (at least if you are a PIDD patient). What is more, is that if you come off, it is likely that your insurance will refuse to pay for the IgG when you need to go back on it.

Knowing this, I felt trapped. I knew what would happen if I went of the IgG. I'd get extremely sick. If my doctor refused to write for it, what would I do? It was suggested to me that I seek another opinion, but I was tired of doctors and various consults. I had already sought out a second opinion and even a third opinion. I didn't want anymore. I was resigned to accept my fate; however, I was still urged to see Dr. Bonilla at Boston Children's Hospital. Finally after hearing a positive story about Dr. Bonilla from another patient who had had a similar encounter with Dr. Cunningham-Rundles as I had, I made the appointment and decided to give it a try. The worst that could happen is that he agreed with Dr. Cunningham-Rundles that I should come off the IVIg.

Meeting with Dr. Bonilla was extremely different than any other consult I have had. For one, he actually listened to me and what I had to say. He took his time meeting with me and was not in a rush. Also, even though he had a resident with him, she observed. He did the entire evaluation. At most consults, the residents or interns do the evaluation, then the doctor comes in, spends 5 minutes with you, gives their recommendation, and leaves. It was nice to be taken seriously.

Dr. Bonilla definitely did not support coming off the IgG. He said that though some of the testing had not been done, it was too late to do it now. He said that once a person starts IgG, they need it the rest of their life, and coming off would actually be harmful. This was nice to hear. Rather than stopping the IgG, he actually recommended that the dose be increased to see if that would help prevent the infections I have been getting. Unlike the other consults who gave a recommendation and that was it, Dr. Bonilla wants to work with Dr. Steingart to best coordinate my care.

I've talked to Dr. Steingart, but don't know if he will be willing to try this form of treatment. It is hard to feel like the fate of your life and well-being is in the hand of doctors. Thankfully, Dr. Bonilla said that should Dr. Steingart not support his recommendations and continued to try to take me off the IgG, Dr. Bonilla could become my primary immunologist and write the scripts for my IgG. This is a huge relief. Though I hope it doesn't come to that. I love Dr. Steingart, but it is nice knowing I have someone who can help if he refuses to.

Hopefully this is the beginning of good things to come.

What are Primary Immune Deficiencies?

Primary immune deficiencies (PI) are a group of rare genetic diseases that affect the immune system, making a person immunocompromised. The immune system is the part of the body that protects a person from infection. For most people, the common cold is rarely more than a minor inconvenience; however, for patients with PI, the common cold can be a life-threatening event. There is no cure for PI. The treatment is intravenous immunoglobulin (IVIg), which is made from donated plasma. The plasma is collected from people when they donate blood. It takes approximately 300,000 blood donors to make one batch of IVIg. Intravenous immunoglobulin gives patients a transient immune system; it gives them necessary antibodies to help fight infection. A patient with PI will need to receive replacement immunoglobulins (IgG) at least once a month, every month, for the rest of their life.

Since these diseases are so rare, it often takes years before a patient is properly diagnosed. Even once a patient is diagnosed, it can be difficult to get proper medical care because most doctors have never heard of primary immune diseases, never mind know how to treat them. Proper and early diagnosis is essential to a good quality of life and prognosis.

Who am I...

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

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

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

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

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

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

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

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

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

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