True Friends

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

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

Locks of Love

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

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

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

Who am I...

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

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

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

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

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

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

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

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

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

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