So much has happened in the past four days that it might just be impossible to jot it all down. We’ve been to the ER, been in the OR, and were readmitted to our old hood, unit 5D. The hardest thing to figure out right now is where to start. I guess it’s best to talk about the relationship between the epstein barr virus, ptld and lymphoma. I made reference recently to a serious complication that can arise from the epstein barr virus. After all the research I did about transplant in general, ptld, or post transplant lymphoproliferative disease, really took me by surprise. The epstein barr virus is really just a type of cell within your immune system that proliferates through your body and causes infectious mono. In a person with a normal immune system, the symptoms can be a pain, they may take a while to get over, but eventually your T cells (suppressor and killer cells) get control of your B cells and you overcome the virus.
Unfortunately, those, like Rafi, who have just had a transplant, take an immunosuppressant to keep those T cells in check. T cells are really responsible for transplant rejection so you don’t want those T cells to be to numerous at first. Problem is those T cells are needed to keep those B cells in line. If the B cells are allowed to proliferate, they can mutate into a rather virulent form of cancer or lymphoma. In the case of allogeneic stem cell transplant recipients, the type Rafi had, the outcomes once you get to non-hodgkins lymphoma are not good at all. So last week, we learned that Rafi’s epstein barr virus had come back with a vengeance. She had a viral load of 2400, then 17000 then 33600. This called for a full body CT Scan to see if she had any enlarged lymph nodes. Sure enough, she had some pretty large nodes in her neck and in her armpits. This was enough to send our blood pressure through the roof. Due to the CT scan findings, we had to schedule a lymph node biopsy. Rafi would have to go into the operating room, go under anesthesia and have some cells taken from one or two lymph nodes to see if the inflamed lymph nodes were caused by the epstein barr virus or if it had morphed into ptld or lymphoma.
One of the only things that kept me sane, I can’t speak for Jackie on this one, is I knew Rafi had a good T cell profile at 100 days post transplant. Plus, the first line of defense against the virus, ptld and lymphoma for that matter, is to get off the immunosuppressant so that the T cells can come into the picture. It’s takes a while to get off the drug, Rafi’s taper down schedule was almost 3 months. She was in the middle of getting off the cyclosporine when the virus hit plus we then accelerated her taper to best allow her T cells to come in. Still none of this is a guarantee of anything.
On Sunday, Rafi spiked a fever. Normally that earns you at least a two day ticket into the hospital. The attending physician was quite logical and figured it was due to the virus, all of our numbers looked good, so he said we should just go to the ER, get some blood taken so they could run some tests just to make sure there was no bacterial infection, take a broad spectrum antibiotic and then go home. The next morning we were due into the hospital at 5:30am for surgery so it wouldn’t be a big deal. Jump to 6:30am on Monday, we are in preop, still waiting on anesthesia to come to the room to tell us who would be intubating Rafi. Jackie and I told surgery that we would only work with one of two anesthesiologists or we would cancel the surgery at the last minute. There was no way we were going to witness another traumatic intubation that would lead to enough inflammation to block her airway again. It just wasn’t going to happen. We made this perfectly clear to anyone who would listen, and to one or two others who didn’t want to hear it.
At close to 7am, after us pushing the nursing staff, in comes a representative who tells us that neither of our choice doctors were available to work with Rafi and someone else was scheduled. Jackie and I both looked at this woman and said, OK we are leaving right now. We made this perfectly clear and the surgery is off. Five minutes later, one of our choice anesthesiologists, walks in to our little preop room and tells us he would be our physician for the procedure. Jackie and I both let out a sigh of relief. Rafi had to have this procedure, and we just think this man is great. His name is Dr. Sarpal and he is the nicest guy who generally cares about his patients and does his best to make you, the parent, feel comfortable with what he is going to do and how he is going to do it. Knowing that I was going to be in the OR with him, he tells Jackie and I that he is not going to intubate Rafi, and instead use propofol to keep her sedated. He knew we didn’t want to have her intubated, he knew her history, had already intubated her twice, and felt that she could tolerate the procedure without the breathing tube. We really love this guy.
A few hours and two lymph nodes biopsied later, we were in postop and Rafi spikes a fever. We were due to be discharged in 40 minutes, when I got on the phone with the attending physician at the blood and marrow transplant clinic. She knows Rafi, Jackie and I well, and knows we would fight her on getting admitted. Dr. Margi MacMillan made some good points, so logic and safety won the day. We would be heading back to unit 5D just to be sure the fevers were not caused by anything else except the epstein barr virus.
There was one advantageous aspect to being the hospital today. Rafi was due to get her first dose of Rituximab today and there can be some pretty serious infusion reactions while you are getting the drug. It takes about 8 hours for the drug to be administered as well. Rituximab is a drug that targets B cells. While not technically chemotherapy it generally does the same thing without the toxicity, but infusion reactions can be fatal. Sure enough, Rafi had a reaction. She doesn’t complain about anything. I saw this kid go through hell with a smile on her face. Today she looks at me and says, “Daddy pick me up!” But not in a good way. As sh eis in my arms, I feel the trembling become more severe. I can feel her heart racing and she is complaining. So we slowed down the administration of the rituximab and gave her some demerol. That and some good time in daddy’s arms as she looked at mommy calmed her down. She stopped complaining and actually took a very short map. She will need three more doses of the rituximab, but we are told the first dose is the worst.
We’ve been on edge since Monday morning waiting for the biopsy results. We are admitted to the hospital, planning on leaving tomorrow, and in walks the attending who we have never met. The biopsy results are in, they came back faster than we anticipated and I literally stop breathing. Then I hear those words:
“There is no evidence of lymphoma”
I swear a tear just dropped off my face. The inflamed lymph nodes were caused by the virus and nothing else.
There really is nothing more to say.