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.