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.