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.