Pink Slip

On Friday the girls and I met with a group of friends for a play date at a ranch. The weather was perfect and the company was great. I had received a call from the pulmonologist's nurse before leaving for outing, informing me that the doctor had received all of my messages from the last 3 weeks and that she would make certain he called me back that day. She told me if I hadn't heard from him by 2:00 pm, I should call her so she could remind him. I am not sure it is my job to babysit this, but I was desperate to talk to him and set an alarm on my phone to remind me to call her so she could remind him. We were enjoy our time at the ranch and it was a good distraction from everything else. Apparently too good of a distraction. You guessed it....after waiting 3 weeks for a return call, I didn't hear my phone ring when he did call. Yep, par for the course. I quickly called the nurse who assured me he would call back. As we were packing up to leave, he did, indeed call again.

The conversation went about as bad as it could have. Well, I guess not AS bad as it could have, I didn't curse once and was able to keep my cool somehow. I'll try to be as basic is I can with it all.
At our last appointment the plan was to:

- Keep doing what we had been doing.
- Repeat the brushing/biopsy if we could get her well for a period of 8 weeks, probably in the spring or summer since there are less viral illnesses then.
- Do a CT scan of her lungs to check for permanent damage.

He started the conversation explaining that she didn't need a repeat brushing since PCD (ciliary issues) are so rare. I don't think that "rare" means there is no chance you have it. I believe that would be described as non-existent. He continued explaining that since Kate has been well for the past few weeks, she's showing that she is getting bigger and healthier. Yeah, maybe. Doesn't change the results from the last test though. Apparently the genetic screening we had done (against his advice because it is so inaccurate), coupled with the fact that she was healthy for a record amount of time, makes him 99% sure she doesn't have a cilia problem. While I'm no math whiz, 30-40% accuracy on a test + a brief period of health = 99% accuracy? There is no reason to redo the brushing since she is now doing better, there is a 99% chance that she doesn't have it and because there is always a risk in having anesthesia or procedures done.

Since she is having a flare up now, he wouldn't want to put her under anyway and even if we did it wouldn't be an accurate reading. I'm by NO means a specialist, but if she had 12 weeks to let the cilia grow back, I don't think that they would ALL die off from one little flare up. I agree that there might not be as many as without that flare up, but I do think it would at least show whether or not the are capable of regrowing. When I pressed him on whether or not we would ever redo the biopsy he said he would be willing to do it, if we really felt it was important, when she was healthy for over 8 weeks. When I explained that we had reached that point and when we called he kept saying no, he explained that it was because it wasn't during the summer. Apparently there are too many viruses that go around the rest of the seasons and that might cause us to have to reschedule if she got one. HUH? I'm sorry but when you have a kid who has been sick for an entire YEAR, you take what you can get! I don't care what season it is. If we have to reschedule 99 times, so be it. On top of that, she was sick the ENTIRE summer last year. We still haven't caught on that this kid follows NO trend? I clarified that this might take a while to get to that point (see: decades!) and he agreed that it would be fine.

His other point was that it isn't going to change the outcome or treatment. Agreed, she still may be sick often. She still may require a lot of medicine, maybe even the same ones she is on now. It does change the fact that we would know what treatment plan to follow (ie. Using percussive therapy, trying different asthma drugs if it is indeed asthma since hers aren't working, etc.). A diagnosis would also open the doors to get involved in research groups which might have new or different treatment plans. It also would give us a clearer picture of what to expect long term. The conversation was fairly long and the majority of it was him repeating the same thing over and over. He went back into the spiel about children who wheeze with viruses. I finally clarified that we would wait until she was healthy for 8 weeks, make sure that it was summer, call his nurse to set up the procedure and hope that she didn't get sick before that date came. Yep, that's the plan.

Needless to say, we will not be going back. I REALLY don't understand. What would the harm have been in just doing the biopsy a few weeks ago when she was well? Or now for that matter? There would be one of two options.

1) She still has no cilia - There would be a very small chance that they could ALL have been killed off by this one tiny flair up, but more than likely this would show they really don't grow back. We assume it is this, even if it means we rebrush her again when she is well again in the future just to be certain.
2) She has some cilia (even if some were killed off by the flare up) and it proves she is able to regrow them and it was just the fact that she was sick so much that gave us the results last time. I wouldn't even care if I got an "I told you so" out of it.

It seems pretty simple to me. I don't understand the hang up. If I had a patient's mother calling me who really wanted me to do a test (that would take 15 minutes) despite the fact I didn't think it was what was troubling her kid, I think I would eventually just do it. If it's what the doc thinks it is, at least it will shut the parent up. I mean really, what's the harm? Am I missing something? And putting off because it's not the right season? Really? What am I not seeing here?

I was SO angry and frustrated when I got off the phone. It hurts me so bad to see Kate sick. The fact that this call fell on the day that Kate started full out wheezing again was a little salt in the wound. It's been just long enough that we've been steroid free that we've gotten to see the real Kate. It makes the monster she is right now seem that much worse. It breaks my heart. We are right back at square one. Steroids, breathing treatments, ineffective antibiotics, horrible diaper rash, raging, manic episodes, sleepless nights and days and still no closer to any answers.

I was talking to my mom and she had some really good points. She wonders if maybe there is an issue of pride on the line. Maybe since we were blown off from the start and the doc had to eat his words when the biopsy came back the way it did, he felt threatened. She thinks he's afraid to do the biopsy again, for fear the results will be the same, showing he was incorrect. So instead of facing that, he just keeps putting us off. I sure hope that this isn't the case, although I can't think of any good reason he would keep putting us off. To let parents be this frustrated and more importantly a baby suffer because of pride? That's pretty low. I don't know if that is what we were dealing with or not, but I am not sticking around to find out. We will not be going back to him period. If it means we don't find out, so be it. We are no worse off than where we are now. Even if we did get an answer out of him, I need a doctor who sees patients more than one day a week, who is compassionate, who sticks to his plan, who returns phone calls in less than 3 weeks time (this was even the case when our pediatrician was trying to speak with him). We are going to have to have a better working relationship than that.

On a positive note, since I couldn't sleep last night (bitter much?) I went back through all the research groups that I found after Kate's initial brushing. I found an email address for one that I hadn't contacted before. At 1:35 am, I sent a message simply asking:

I have a 2 year old daughter that has had a horrible time with her health. We recently had a bronchoscopy with brushing done and the results showed that she had no cilia. I know that cilia can be damaged by illnesses, but can ciliary aplasia be caused by a virus or something (secondary) or is it always genetic (primary)?

In less than 12 hours, despite being on a weekend, I received a detailed, three paragraphed response from the executive director with more information in it than we have ever received. Not only did she explain different possibilities, but she offered to set us up with a PCD research site closest to us. Without knowing any more back story than what I shared above, she stated that if cilia is damaged because of infection, it is important to get to the bottom of why the infections are that extreme. She also made it very clear that biopsy results such as ours should be redone, several times if necessary and that if there has even been talk of PCD with a two year old, we should continue pursuing until we have a definite yes or no.

I felt like it was a gentle reminder that God is still in control and still holds Kate in His palms. This stranger directly answered the main two questions that torment me without me even having to ask them. I am not real sure where we go from here. I've wrote her back with more information and asked for research sites anywhere near us. At this point, I would drive to Maine if I had to (although I would probably wait until she is off the steroids ;) now that would be a rough drive!). While I was very angry and am still very frustrated, I am also hopeful for what might be. Please pray specifically that we are able to get into a research site so that we can have some definite answers.