Pulmonology Update #2

I saw Dr. Ginns, the transplant specialist at Mass General, on November 23rd. I ended up having to go to the appointment by myself since I couldn't find someone willing to go with me. I didn't have any tests this time such as pulmonary function tests, so I was a little less worried that the appointment would influence my ability to drive. I ended up getting there very early and so had to wait to see the doctor. Even once I got into the exam room, I waited a good hour and half to see the fellow working with him. I find this to be one of the most frustrating aspects of working with doctors such as those at Mass General. It's as if your time is not valuable. They ask you to get there at a certain time and should you be late, you'd lose your appointment. They know that at least 90% of their patients are driving a considerable distance for the appointment, it would only seem considerate and respectful to be on time for the appointment. A half hour late is one thing, but close to two hours of waiting in an exam room alone, waiting for a doctor, is a bit absurd. He had no other patients before me, since I was his first patient of the afternoon. It is just frustrating.

The fellow asked a whole bunch of questions and of course we went over my medical history. I explained my history of breathing problems and how it started nearly at birth, and how my first severe asthma attack was at 6 months. I described how my first admission to the PICU for breathing problems was at 13 and that I was intubated for the first time at 15. Since then I have over 25 intubations. Honestly after 25, I stopped counting, as to continue to do so seemed pointless. In fact, I had just been discharged from the hospital 2 days before the appointment. During that hospitalization I had been intubated. I explained my CVID and my history with infections.

We then began to address my most frequent concerns - mainly the decrease in my lung function, increase in o2 demands, and decrease in quality of life. I voiced my concern that with a lung function of 20%, my lung function did not have far to fall. I did not mention transplant to the doctor, but rather stated that I was looking for options for the future. If my lung function continues to fall, I know that something will have to be done and I would prefer to begin to open conversation regarding this rather than get to the point that this is necessary and no discussion has been taken. For a transplant to be possible the patient must be sick enough to need a transplant, but not too sick. I don't want to pass into the "too sick" category. I told them how I was requiring more o2 than ever. That before the most I've ever been on is 2-3 L, and at this point I'm using 5+. The smallest of tasks such as walking to the bathroom leaves me short of breath. I am no longer able to do the things I used to. The most disappointing thing is that a year and a half ago, I was able to exercise. I was doing 2-3 jazzercise classes a day, at least 5 days a week. I cough up blood nearly routinely now. Some days only a little, others up to 2-4 oz. At night, I wake up gasping for breath. Whereas most people have dreams when they have to go to the bathroom that they have to go to the bathroom, then they wake up to find they really do; I have dreams that I can't breath and that I am being suffocated. When I wake up, I realize that indeed I truly am not able to breath.

When Dr. Ginns came in, the fellow reported everything we had talked about. He seemed to take everything in stride, which kind of disappointed me. I guess I expected more of a reaction. After all, I was telling him I was coughing up blood, woke up unable to breath most nights, and how limited I had become because of my declining lung function. Maybe it's just that he sees such severe cases everyday that he is slightly immune to it all. He did take me off the o2 to see "how low I could go." To which I was not a fan. I became so short of breath that I was unable to talk. I was having difficulty even with understanding what he was saying or asking me as my brain could not comprehend what he was saying. I do not know how low my sats eventually fell as I could not see the sat monitor, but finally he allowed me to put the o2 back on saying that he didn't want me "to be uncomfortable." Despite everything, I know he is a competent doctor. He put me on Bactrum as a prophylactic antibiotic. Though I am already on a prophylactic antibiotic, he said the Bactrum is better at preventing respiratory infections. He also ordered a Barium Swallow, to rule out gastric reflux as the cause of my lung problems and a chest CT. I will see him again on January 11th to do a 6-minute walk and more pfts.

I had the Barium swallow and CT scan on December 8th. Neither test was bad. I have obviously had a million ct scans, so no problem there. I had the ct scan in the morning, then the barium swallow. I had never had a barium swallow done before and everyone I talked to told me how disgusting it was to have to drink the contrast, but I did not find it bad at all. It is very similar to the contrast you drink for abdominal ct scans. The hardest part for me was drinking while laying down and having the xray machine so close to my face. I have a hard time laying completely flat anyway with my breathing and am definitely not comfortable drinking while doing so. I am afraid I will choke. When I was trying to drink laying down, they had the xray machine right up against my chest and nearly to my chin. This made me feel very claustrophobic. I was glad it did not last longer than a minute or two. The barium swallow was negative. They saw a small amount of evidence for gastric reflux, but only when I was drinking the water laying down. Since no one drinks laying down, or at least I don't, this doesn't really matter.

Pulmonology Update #1

Well since my lung function has declined so much in the past six months, I decided to try to get an appointment at Mass General Hospital in the pulmonology clinic. I have decided to take matters into my own hands and discuss my future, whatever that may mean. One of my friends sees the transplant specialist there, Dr. Ginns. So in August I made an appointment with the general pulmonology clinic for October. I had to make the appointment with the general pulmonology clinic rather than getting straight into seeing Dr. Ginns because I didn't have a pulmonologist to refer me to see Dr. Ginns and you need a referral, whereas anyone can make an appointment in the general pulmonology clinic. It was quite a long day. Went with my aunt and had to go in early to stop by Boston Children's to get some lab work done that can only be done by Dr. Bonilla's lab. I had pfts scheduled for 3:00 and then my appointment with the doctor at 4. These were possibly the most thorough and torturous pfts I've ever done. They did each test nearly 6 times. By the end, I was so tired, I couldn't even complete the test. This was not just simple spirometry, but rather full-set of pfts with diffusion testing ect. For me, they equate to medieval torture methods. Just hearing the words, "Deep breath in, and BLOW, BLOW, BLOW, BLOW!!!!" make me want to run away screaming. The tests left me so short of breath, I was only satting at 92% on 6L. The doctor was running late and I didn't actually get seen by the doctor until after 6pm. The result was what I expected and hoped for. I got a referral to see Dr. Ginns. I basically told them that I was concerned with how much my lung function had declined and needed to begin to discuss my options for the future, especially since I didn't feel that Dr. Rogers was treating me properly and actually endangering my health through his lack of care. While this was the result I was hoping to achieve, part of me was still disappointed, hoping that they would just have a new treatment that would help.

Cardiology Update

I apologize that it has been so long since I updated last. The past several months have been insane with doctors appointments and tests. It has literally been one thing after another, not slowing down for a split second. I have said previously that being sick is a full-time job and it is (See post Full-time Job. My days and weeks are constantly filled with doctors appointments, tests, and other assortment of treatments and procedures. I have had to triage my consults so that I do the most important ones first. So, instead of writing one REALLY long post, I will try to break things up as I update you all.

First of all, back in July when I was in the hospital, I was supposed to follow-up with a cardiologist because while in the hospital they noticed I had severe tachycardia. At rest, my heart rate would be 120-130. By just getting up and going to the bathroom, it would sky rocket up to 160+. Oftentimes my heart rate would get to be so high that it would actually become painful. I'd feel as if my heart would leap right out of my chest. If it happened at night when I was trying to sleep, which is undoubtedly when it would happen most frequently, I wouldn't be able to sleep. I would only be able to lay there, feeling as if my heart was on a runaway train, with me along for the ride. Sometimes I would even feel as if it started and stopped or fluttered in the back of my throat. Rather than just see any cardiologist, I decided to see my family's cardiologist Dr. Doughrety, who has treated my grandfather, grandmother, mom, and uncle. Unfortunately with all the pulmonology issues and immune issues going on, I had to reschedule a few times beofre actually making it to the appointment. I was very impressed. Like the previous doctors in the hospital, he did not believe that my condition was biological but rather due to my failing lungs. As my lung function has declined, my heart has had to work overtime to compensate, trying to push out the same amount of blood and oxygen to my organs despite my decreased oxygenation in my lungs. They did a high-resolution echocardiogam and 21-day heart monitor.

E-Cardio Monitor. Button in center can be pushed when you "sense" an event. So when I felt my heart rate going really fast or just not feeling normal, I would press the button. It would then record the instance and transmit it via cell towers to the company in Texas. They would then call me to make sure I was okay. At times, if they sensed something was not normal, they would automatically record the event. During the time the event was recorded, an EKG was done. 

E-Cardio Monitor clipped to pants pockets for ease of portability. While it was not horrible, could have been much worse, I was extremely grateful not to have to wear it anymore and send it to them after the 21-days was up. Even with the sensitive leads, my skin got very irritated and I started to run out of places to put them. Also it was hard to sleep with. I'd roll on top of it, or the leads would become disconnected as I slept.

Funny story: Within the first 24-hours of wearing it, I was sitting on my bed reading a book, just relaxing. The machine starts squealing and the company calls me. They ask if I'm alright. I respond, "Well, I think so, but shouldn't you know better than me seeing as you can see my heart rate and rhythm." Glad to know they really were monitoring my heart, but it was a bit disconcerting.

The results were as expected. The structure and rhythm of my heart is fine. I do have severe tachycardia, but this is from my heart working so hard to compensate for my deteriorating lungs. They can give me medication for it if absolutely necessary, but at this time, with all the other medication that I am on, we would like to refrain from having to do that. Just another sign showing the decline in my lungs.

Immunology Update

From my post Consult in Boston, you know that when I saw Dr. Bonilla on September 23rd, he suggested that my IVIG dosage be increased. Although he sent his recommendation to Dr. Steingart, my dosage was not increased. At first the problem was that Dr. Bonilla suggested a dosage increase to 12.5G IVIG weekly. Baystate was not able to get this allotment. To give me 12.5 G, half a bottle of IgG would have to be wasted and it is way too expensive for that. Since it wasn't possible to get the 12.5 G, Dr. Bonilla then suggested an increase of 15 G every 10 days. The problem with this is that it would mess up the infusion cycle. Sometimes my infusion would fall on a weekend in which case it would either be pushed forward or backward. So week after week, I remained at the same dosage. I continued to get sick and fight continous infections. Since the dosage hadn't been increased, no improvement was experienced. This became very frustrating. Finally, after seeing Dr. Steingart on December 3rd, he increased my dosage. I suggested that if we could not get the 12.5 G, why didn't we just increase to 15 G every week instead. It turns out that the pharmacy only gets 5 G bottles in a different brand of IgG. I get Gammaguard. This is the most pure form of IVIg and has the least amount of IgA in it. The other brands cause me to have reactions. So switching brands was not a good idea. Instead, he decided to just increase the dosage to 20 G every week. It is more than I need, but hopefully I will see some improvement. Of course, I expected an immediate improvement and that did not happen. It's hard to remember that IVIg is not a cure and that it will take awhile for my body to experience any improvement. In addition to the dosage increase, Dr. Bonilla put me on prophylactic antibiotics.

Migraines Update

So as you know, I have been suffering from horrible migraines (See post Migraines). Well, back in August, they got to be so bad that they were lasting 6 days or more and I was blacking out. I eventually ended up in the ER. My PCP finally gave me a referral to see a neurologist, but when I called to get an appointment, despite everything that I was going through, especially the blacking out, the soonest appointment they could give me was the end of November. Well things have not improved, but finally being fed up, I decided to try and see if maybe I could get into Baystate neurology. I had seen one of the neurologists there a year and a half ago when I was having problems with severe dizziness. The dizziness was so bad that I could not even walk. Moving at all gave me such vertigo that I would be sick. Thankfully, they were able to figure it out. It was a problem with my inner ear. They sent me for vestibular therapy and it helped tremendously. So, I had hopes that they would be able to help now too. It was worth a shot at least.

I called the office and was told that they did not have any openings until January, but once I explained my symptoms, the receptionist agreed that I needed to be seen as soon as possible. She assured me that she would have someone call me back within the next day or so. True to her word, she called back two days later. She had found an opening in two weeks. She also said that she would put me on a cancellation list, just in case something came up sooner. Boy was I surprised and thankful. A few days later, she called again to say that the doctor I was supposed to see had a cancellation for the next day, did I want to take it. Of course I said yes. The sooner I got to the root of my problem, the better, in my opinion. I hadn't expected an appointment in two weeks, never mind less than a week after initially calling.

It was the same doctor that I saw before. She took a full history of the migraines, their patterns, and things I experienced while having them. I told her not only about the debilitating pain, but the vision disturbances, loss of vision, and blacking out. I was quite impressed that she actually took me seriously. Maybe I'm so used to not being taken seriously that when someone does it amazes me. She started me on Verapamil, which is a calcium-channel blocker. She also put me on a Medrol pack (low-dose Prednisone) and muscle relaxer. She is also having me do an EEG, just to rule out whether these "black-outs" are really seizures. So I have that on Thursday. Her idea is that for some reason the blood flow to my brain is being restricted to the point where I eventually black-out. For these types of migraines, preventative medications, such as calcium-channel blockers or beta blockers, can be used. I cannot take beta blockers because of my lung issues. She also wants to make sure I am not having rebound headaches, which can happen when you take medication like Excedrin or Fioricet to treat the migraine. Though I do not take medication unless things get really bad. Not nearly enough  to have these migraines be rebound headaches, but I want to make sure everything is ruled out properly. So for now, I am trying not to take anything at all. Of course this is not always possible because they get so debilitating. If the EEG doesn't show anything, and I still am blacking-out, then she will do an MRI.

Although the medication has not made a difference as of yet, the migraines are just as severe, I have hope that we will eventually find the right "cocktail" of medications.

A Full-time Job

People don't understand why even though I do not have a job, I am so tired and do not have any time. "But you don't work," they tell me. No, I do not work a "traditional" job. I do not go into the office and work; however, I do work. My office is my house. My most important piece of office equipment, my telephone. What is my job, you ask me? Being a patient. My job is thankless. It has no hours. I do not get compensation, sick time (well it's all sick time isn't it), or vacation time. I do not get a day or week off. This is my life and there is no escaping it. Although most of my work is done between the hours of 8am and 5pm as this is when doctor's offices and insurance companies are open, by no means does this mean my job is done.

What exactly do I do? Well, I make and go to appointments, and advocate for myself and my care with doctors. At times, this involves yelling, screaming, and hollering, especially when dealing with a particularly difficult and obstinate doctor, who do not want to provide adequate medical care, and refuse to acknowledge that it is my body and that reading a case study in a textbook back in medical school does not qualify as knowing about PIDD or being an expert (see post Living in Pain).

This in itself would be extremely time consuming and exhausting, but my job doesn't end there. The majority of my job entails dealing with my insurance company, getting the proper referrals, authorizations, and pre-authorizations for my various doctors, medical procedures, treatments, and medications. This would be bad enough, but I also must fight with my insurance company for the things that they've refused to pay for which they should have. I must act as a liaison between the medical billing specialists and my insurance company because unless it is billed properly, my insurance company won't pay and then I'll be stuck with a $2,0000 medical bill. I also must deal with the pharmacy to ensure that I get the proper medication. Some may think this is easy - just go and pick up your prescriptions. Not quite. Often I must go and make sure they inputted the insurance correctly, so that once again, I don't end up with huge co-pays. I have become best friends with my pharmacist.

So, no, I do not have a traditional job in which I go to an office and work, but this does not mean that I do not work.

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.

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.

Oxygen Tales

So they decided to switch me to liquid oxygen for when I am out and about. Supposedly the liquid oxygen will last longer and meet my needs better for when I am portable. Though the oxygen company had mentioned they might switch me over three weeks ago, I had not heard anything about it since. So at 10 o'clock when they showed up with a big tank of liquid oxygen, and I do mean a BIG tank, I was a bit surprised. I had had liquid oxygen before, so I knew how to refill the portable tank. Later I had a doctor's appointment so I decided this would be the perfect opportunity to fill the portable and see just exactly how long it would last. They give you an estimate, but I like to know exactly. This is rather important information because how long the tank lasts determines how long you can be away from home. The sheet said it would last approximately 2 1/2 - 3 hours. I was worried, because if this is  true, I would need two portable units. A lot of times, my doctors appointments last longer than this (driving time plus appointment time), and I can't take the chance of running out of oxygen.

BIG oxygen tank

I filled it fine, but when I went to take the portable tank off the top, liquid oxygen kept spurting up. I waited a few minutes, but it wasn't stopping. I had a huge geyser of oxygen erupting out of the top of the tank. Liquid oxygen was going everywhere. My efforts to try to make it stop were unsuccessful, and all I succeeded in doing was burning myself. Liquid oxygen is like dry ice, it is frozen. As the temperature increases, it warms, thaws into a water form, which then thaws further into an air form of oxygen, which is breathed in. Touching the liquid oxygen from the tank will result in a burn very similar to that of touching dry ice or liquid nitrogen. In the year and a half that I had liquid oxygen, I had never experienced anything like this. Even people without any experience with oxygen would know that that much oxygen coming from a tank, was NOT good and that this was NOT supposed to happen.

Filling the portable unit

 

Top part where oxygen just kept spurting up in a geyser of oxygen

Well, two of the three numbers I was given did not work. Finally, I resorted to the number for my oxygen company. The reason why I didn't try this first is that the company that provides me service does not deal with liquid oxygen. They made the arrangements, but the company that does liquid oxygen is completely different. Finally, I got someone on the phone and explained the situation.

After I had explained the story to the specialist, she said, "So you're oxygen is leaking?"
I said, "No, this is not a leak. This is a geyser, a volcanic eruption."
"Oh, well, that's not good," she replies.
"No, no, it's not." (After all, this was the reason that I was calling)
She told me to get a towel, wet it with warm water, and throw it over the top of the tank. She  then told me she will send someone out immediately to replace the tank.

Well, I'm not really sure what the warm towel was supposed to do other than freeze the towel. The towel literally turned into a gigantic block of ice. The oxygen just escaped up and around it. I'm sure the woman was just trying to have me do something and try to be helpful. I was of course, and justifiably, very upset and worried. I doubt there is a protocol for oxygen "geysers," and they probably don't deal with a whole lot of volcanic eruptions of oxygen from tanks. Maybe the warm towel would have helped had it been just a leak, but this was much, much more than just a leak.

Although at the time, it was not at all amusing, I now find it rather amusing, and it does make for a good story.

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.

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.

Primary Immune Deficiency Documentary

Looking for a Cure

Team Hope raising awareness for primary immune deficiencies.

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