Specialists

One of the many things that is frustrating when living with a chronic illness is that though you may start with one specialist, you soon find that you have many -ologists. In fact, you may have one for each body system: neurologist, pulmonologist, cardiologist, dermatologist, ophthalmologist, endocrinologist, urologist, nephrologist, gastroenterologist, immunologist, hematologist... and the list goes on... and on.... and on... Where as other people collect cars, or coins, we collect medical specialists. 

The problem with acquiring these specialists, as it really isn't a choice as to whether we see them or not, is that when you first need to see them, and this is usually because you have a problem your primary care doctor or one of you other -ologists can't treat, is that you may have to wait up to six months to get an appointment with this specialist. The receptionist scheduling new patient appointments doesn't particularly care if you are dying, well not really, but maybe it is rather urgent that you see the doctor/specialist, the soonest appointment that the doctor has is six months out. You may try pleading your case, telling the receptionist how important it is that you see the doctor urgently, but they are likely to not hear you. They have heard many such stories, maybe even several times today. They are only the receptionist and cannot and will not make exceptions to the doctor's busy schedule. The soonest appointment is in six months, would you like it? Well of course you'll take the appointment, not that you know what exactly you'll be doing in six months, other than obviously seeing this specialist. Then you will kindly ask them to put you on the cancellation list just in case something comes up sooner. In which case, if it does, you will completely rearrange your schedule and anything planned to see the doctor. You may even have to fight with said receptionist because a referral may be necessary to even schedule an appointment, and though your doctor faxed over the referral, their office seems unable to find it. 

Sadly, often times the easiest way to see the specialist is to go to the ER or get admitted to the hospital in which the doctor is associated with. You may not see the exact specialist, but you can get a "follow-up" appointment after you are discharged, made by the doctors in the hospital or social worker. This appointment will most likely be for a week or two weeks after you have been discharged. If you go into the hospital and say that you are a patient of Dr. _____, you may get lucky and actually see Dr. ______, in which case, you most certainly will get a follow-up appointment. Follow-up appointments are much easier to get and you are seen much sooner than new patient visits. 

PFT Scores

Wow, what a week. What many people who don't have a chronic illness don't understand is that having a chronic illness is a full-time job. Going to doctors' appointments and dealing with insurance is a full-time job, one that you never get a break from. 


This past week was one of those weeks in which I had doctors' appointments every day. On Wednesday I had my much awaited pulmonolgy appointment. Now currently, since I've been so sick, I have been seeing the pulmonolgist every week or every two weeks. So a pulmonology appointment was nothing special. The thing that made it special was the fact that I was due to have a full set of PFTs (Pulmonary Function Tests). I was supposed to to do them weeks ago, but right before I was scheduled to do that, I ended up in the hospital. I was a bit nervous about doing them, but also interested in the results. My last set was back in April. I was much healthier then. Before that, I hadn't had PFTs in almost two years. One of the reasons for this was that I was that I was between pulmonologists, but the main reason is that I detest PFTs. Usually I avoid doing them like the plague. I don't mind spirometry, but hate full-set PFTs. Full-set PFTs are different from spirometry. It is more involved. I find them very triggering to my lungs. The last time I had done a full set of PFTs, prior to April, was at Boston Children's Hospital for my cataract surgery. My lungs were so triggered that I not only ended up in the ER, but in the ICU. At that point, my PFTs were good (at 70%). In April, after seeing Dr. Cunningham-Rundles, I bought the bullet and did the full-set PFTs that Dr. R. (my pulmonologist) had wanted me to do since first starting to see him in March. In April, my PFTs were 35%. This shocked me because I honestly felt I was doing well, better than I had when I did them in Boston. This is not horrendous, but it is a big drop from 70%. When Dr. R. looked at the results, he said they were good. Now, I didn't understand this. To me, less than 50% is not good. It means that less than 50% of my lungs are functioning properly. Since Dr. R. was relatively new to my care, I thought that maybe he had nothing to compare it to. Maybe I was just overreacting; however, when I sent the results to Dr. Cunningham-Rundles, she also thought that such results were alarming and that more testing should be done. 


Over the past three months, things have definitely gotten worse and my lungs have made a serious decline. I have gone from needing 2 - 3 liters of oxygen occasionally to needing 4 - 5 liters continuously (more if I'm walking around or active). I can no longer even tolerate pulse oxygen without my sats dropping. As much as I dreaded doing the full-set PFTs, I was interested in seeing the results and comparing them to those in April. I expected them to be somewhat worse; however, the actual results shocked me.


As is normal for full-set PFTs, I started with spirometry. After blowing twice, the nurse left the room to consult the doctor and my previous PFT results. When she came back, she told me they were lower than my previous ones, but again, I had expected this. I didn't really think anything of it. I repeated the test four more times, to make a total of six times. I then attempted the helium testing, but didn't make it through the full test. I got my results, then waited for the doctor. As I looked at my results, I was shocked. Yes, they had gone down, but I had not expected such a significant drop. My PFTs had gone down from 35% to 14%. I looked at the feedback loops to see if maybe I hadn't done them right, though I've been doing PFTs since I was 13. The feedback loops were good. I then looked at the values since I had done them so many times. When doing PFTs, they take the best value. All the values were within 0.02 of each other. Based on this, the results were accurate. Then, Dr. R. came in, looked at my results, and said they were not valid, that there was no way my lung function had declined so much, and that the results couldn't possibly be an accurate representation of my lung function. I was shocked. How could he essentially just throw them out? How could they not be valid? Unfortunately, as shocked as I was with the results and his assessment of the results, I didn't get a chance to ask him why he thought they were invalid. In addition, he was only in the room for approximately five minutes, leaving me to make a follow-up appointment for two weeks. I felt like a deer in the headlights, unable to make heads or tails of the situation.


All the way home, everything bounced around in my head. 14%! Oh my goodness! I felt Dr. R. should not only be concerned, but should be referring me to specialists. Why had my lung function declined so significantly and what were we going to do about it? I'd never heard of invalid PFTs, and I had been doing them for over 15 years. I'd done the test six times, all with the same result. How was this invalid? It did not make sense to my biological and research mind. Yet, I thought maybe I was making a bid deal out of nothing. So now I am stuck with not knowing what to do. People who also have vast experience with this have told me that they have not heard of invalid PFTs, and 14% is an extremely alarming thing, but if my doctor doesn't see a problem, what do I do? I can't fix it myself. I don't even know what's wrong.


Prior to this, I had considered going to Mass General for a consult. If I ever need a transplant, that would be where I'd go. I considered making an appointment there or if I had difficulty, presenting myself at the ER, letting them run tests and figure out what was wrong. As I wasn't in acute distress I didn't feel the ER was an appropriate action. I didn't think they would admit me or transfer me just based on low PFTs. I didn't feel my low values were because I was currently sick. I was at a norm for me, well what has become my norm in the past few months. I wasn't struggling to breathe. As I struggled not knowing what to do and alternately freaking out and trying to stay calm, I somehow got through the night. I decided my best course of action was to try to get an appointment at Mass General. I wasn't in acute distress, so nothing needed to be done immediately. I reasoned that my lungs had not changed from the day before or even two weeks before. The only change was that now I knew the value. The next day after getting the run around of going from department to department, I made an appointment at the general pulmonary clinic. Mass General does not have one pulmonary department like most hospitals, but many different specialized pulmonary departments. I had no idea which one I needed. Unfortunately, the soonest appointment they have is October 25th. I don't know what I'll do until then. I have to hope my lung function holds up and I don't get worse. I guess if I start to get worse, I could try to find a way to get to Mass General and present to their ER rather than one here, but I'd rather not do that. It's very complicated as I have no one to take me there.


It's such a conundrum. I wish someone would tell me what to do. I don't want to harm myself or risk my health if I need immediate medical attention, but I don't know when that is the case. usually you would consult your doctor and have them make these decision, but that isn't possible. My doctor is completely unconcerned. Maybe I'm overreacting, but my gut says I'm not. I do not know what I should do or how I should fix it. I wish there was a guide book for these things, or someone I could consult, but there isn't. I just have to hope and pray things work out as they should.

Warning Signs of PIDD

For most people, the common cold is rarely more than a minor inconvenience; however, for patients with PIDD the common cold can be a life-threatening event. In a person with a normal, healthy immune system, the immune system protects them from infection. In people with PIDD, the immune system is either absent or does not function properly. This causes them to be more susceptible to infection, and when they do get infection, they are not able to fight it off.

How does a person know when it is a typical infection or something more serious such as a primary immune deficiency? According to the Primary Immune Deficiency Foundation, the warning signs of PIDD are:

• Recurrent - the infection keeps coming back
• Persistent - won't clear up, or clears very slowly
• Severe - requires hospitalization or intravenous antibiotics
• Unusual - caused by an uncommon organism
• Runs in the Family - others in your family a similar susceptibility to infection 

If the answer to any of these questions is Yes, then it is important to check with your physician for the possibility of a primary immune deficiency.   

Early diagnosis and treatment is necessary to ensure a good quality of life. With proper diagnosis and treatment, patients can live full and independent lives; however, without this, patients can develop serious and debilitating illnesses.

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....

End of Vacation

Living with a chronic illness is taxing both physically and emotionally. While some people may recognize the physical tolls it takes on your body, few realize the emotional toll it takes as well.Just keeping up with the myriad of medications taken daily to stay alive and maintain some state of health can be exhausting. But then you have to add the endless stream of doctors' visits, specialists and consults, and medical procedures and tests. Every day brings another fight with the insurance company, doctors, and home health care agency to get the treatment you need just to live. When you think it is done, you then look at the pile of ever mounting medical bills, that which insurance didn't pay for. You do all of this day-in and day-out, fighting for your life - literally. Besides the physical exhaustion it brings, it wears on you emotionally. The idea of these never ending tasks are daunting. You become overwhelmed to the point that you just can't do anything.

This is how the past three months have been for me. As I've watched my health slip away, and though I wish it were slowly, in fact it has been more like a spiral. I felt powerless. I did everything I was told to do, took all my medications and treatments, which seemed to increase at every doctor's visit. Yet, I kept getting worse, with no end in sight. There is nothing worse than watching your health run away from you, and feeling there is not a thing you can do about it. I have spent more time in the hospital than out of it, four time in a month and a half. Each time leaving the hospital overwhelmed and depressed, feeling like things will never change and only getting worse despite all the medications and treatments. At times I felt like giving up and just not doing anything. It was knowing that I was going on vacation on August 7th that kept me going and kept me from giving up. My family and I had gone to Myrtle Beach last year, and we were going back this year. It's gorgeous there, a place where I could be at peace. I needed this vacation. To me it was more than just spending time on the beach. It was a break from all the medical appointments and illness. For that week I could just be normal. I had been looking forward to this vacation for over six months.

The Oxygen Fiasco
I began planning for the vacation overt a month before we left. We were planning to fly so I knew that the logistics of traveling with oxygen could potentially be challenging. I met with my pulmonologist, got a letter stating I was okay to fly for the airline, and got a prescription for a portable FAA approved concentrator for the plane. I arranged things with the oxygen company. Two weeks prior to leaving I called the oxygen company and set up the details. They were going to ship down a concentrator so that it would be there when we arrived. They would also give me an FAA approved portable concentrator for the flight, which I would pick up Wednesday afternoon since we were flying on Friday and I had my infusion scheduled for Thursday. I saw my pulmonologist on Tuesday to get official clearance to go since I had been so sick lately and just gotten out of the hospital two weeks prior to this. This was important because my mom was leery of me coming. Basically, I was a liability. Wednesday I went to pick up the portable concentrator at 3:00. I expected to go in, get it, and leave, but things are never that easy.

First, Jeff, the guy who needed to give me everything wasn't there, even though we had arranged the time the previous day. Pete, one of the directors of Prompt Care, my oxygen company, started making a fuss over the portable nebulizer I also needed, which had to be straightened out by the home office. Then he didn't want me to have the portable concentrator because it went up to 3 liters continuous flow, not 4. It took an hour and a half to straighten everything out, but finally I was all set up. I signed a liability waiver stating that if anything happened to me, I would not hold them accountable and was on my way. I drove off and 20 minutes later, I received a phone call from the receptions saying that they had called my pulmonologist and he hadn't okayed me to travel. She said they needed to pick up the equipment in the morning. I was devastated and confused. My doctor had cleared me medic ally to go just the day before. How could things change so drastically in less than 24 hours? I tried to call my doctor to get the real story, but he had left for the day.

So now the oxygen company wanted to take their equipment back, which if they did that there was no way I could go. In addition, I had an infusion and would not be home for them to pick it up, hence why I'd done everything on Wednesday. I didn't know what to do. Technically, I had the equipment so I could just not be there for them to pick it up and go on vacation anyway, but I didn't know what the repercussions of this would be; however, this seemed my only way to go on vacation. I certainly couldn't go without oxygen for ten days. I left early in the morning for my infusion, taking the equipment with me, hoping to buy time since they couldn't pick it up if it was with me and I wasn't home. During that time they tried to pick it up. They called me and threatened me several times, saying they had to pick up the equipment. I called my doctor in hopes of straightening things out, and he said that though he had concerns with me flying (wish he had told me that on Tuesday when he saw me), that he felt it was okay for me to go. Frustrated and desperate, I called the representative Karen, who had originally set me up with Prompt Care.

Karen is amazing. She goes above and beyond and is truly dedicated to her job, something you don't see too often. Prior to having Prompt Care as my oxygen provider I had another company. I was fed up with their service. While in the hospital, I told the social worker I was looking to find a new oxygen company. Karen happened to be in the hospital. She met with me and arranged everything so that my transition went smoothly. She was amazing and made a great impression on me. I knew if anyone could help me, she would. Once again, Karen was amazing. She talked to Dr. R (my pulmonologist) and coordinated things between him and the oxygen company. The next morning she came by and delivered a few things that I needed. The equipment they'd given me on Wednesday was having problems so she went back to the office to get me one that worked properly. She did all of this before noon since we had to leave at noon for our flight.

Packing all my meds

Meds for Carry-on Luggage

Meds to be packed




Organization is Key

The flight was definitely a challenge for my lungs. I think my flying days may be over, at least until my lungs get better. Security was hard because I couldn't go through the regular metal detectors but had to be checked and patted down separately by a security officer. I met a nice guy while waiting for the plane and had a great conversation. This meant so much to me because the oxygen and medication make me unattractive. Most guys don't even want to get in a conversation with someone like me. Yet here I was sitting in the airport with two oxygen tanks and wearing a mask, and he was genuinely interested in the things I had to say. We had a lot in common. I was so engrossed in the conversation that I almost missed our flight. My mom came up to me to say that they had already called for handicapped people to board the plane and were boarding the rows. I had not even realized the plane was at the gate. I had to get all the various chargers and equipment in the bags to go on the plane. Then when I got to the desk I didn't have my boarding pass. Somehow as I went through security I lost my boarding pass not even realizing it. Thank goodness my mom had printed off two copies.

Myrtle Beach was exactly what I needed. For some reason my lungs always do so much better in the south, especially by the beach. It was rejuvenating to sit on the beach or by the pool. All too soon it has come to a close. Now it's back to the doctors' appointment ect. Oh, how it ends too quickly, but it was a much needed break.

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.

PIDD Awareness

This is a video of 5-year old Mathew who has a primary immune deficiency (PIDD)




Primary Immune Deficiency Awareness

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.