Peace at last

The consult with Dr. C last week seemed finally like the answer to our prayers. It didn't answer all of our questions, but at least we finally feel like we talked directly to someone who knew what he was talking about.

We don't have a different answer than before, but I now feel peace about it. I am not in a state of desperate panic about not seeking preventative treatment for this pregnancy. I don't feel quite so much like a ticking time bomb. I do still have some anxiety about whether to pursue a cordocentesis in the 8th month, and whether to plan a C-section, but that isn't a decision that needs to be made now, so there really is nothing more to do, and I can put it from my mind a bit. That is a good feeling.

A recap of our conversation with Dr. C (quotations are not exact but based on my notes):

How is the antibody testing done?
Jennifer's serum is mixed with a random platelet sample. The lab looks for platelet aggregation. It won't show up if the antibodies are present at a low level or a low intensity, which is what I am calling the non-specific immune response, as I suspect in your case. If the blood has antibodies against very common platelet antigens then you are much more likely to have trouble in this or any subsequent pregnancy.

What was John's actual history?
His platelets counts were

• 40-60,000 at birth on 6/26.
• 30,000 when readmitted to the hospital on 7/3
• 13,000 by 7/5
• He had IVIg on 7/4 and 7/5 (plus steroids)
• 84,000 on 7/6 when he was discharged.

At what platelet level do you consider the switch to be from "low" to "dangerous?"
Right around 50,000

What else could cause low platelet count in an infant besides NAIT (or NATP, the alternate acronym for the same condition that Dr. C preferred to use)?

• infection (unlikely for John - no other clinical signs, which is why they stopped an IV antibiotic treatment on 7/4)
• stress at birth ... low oxygen levels during birth lead to decreased blood pH and acidosis (typically would be seen in first 3 days after birth, so again, unlikely in John's case)

Some doctors have indicated that John's records are inconsistent with NAIT, because his levels continued to drop after his birth for 10 days, instead of improving upon birth. Do you have thoughts about whether he really had NAIT?
NAIT involves a highly specific or high intensity antibody. I believe that John's case was  an immune-mediated thrombocytopenia, but not NAIT (the alloimmune variety).  I believe John's case was a non-specific immune-mediated response.

With NAIT, the high-intensity, specific antibodies cross the placenta during pregnancy (which leads to the high prenatal risk to the baby). The non-specific antibodies generally don't cause severe problems prenatally, either because they are present at too low of a level or too low of an intensity. It is believed that newborns may get this non-specific antibody from a direct mixing of mom's and baby's blood, and thus a higher exposure to mom's blood, in the birthing process. Thus, the baby's condition can worsen after birth (the most significant antibody exposure) rather than improve quickly (because no longer getting large doses across the placenta, as with NAIT).

In addition, John's condition may have been exacerbated (though unlikely to have been caused) by the tegretol Jennifer was taking. Low platelet count is a potential side-effect of that medication.

What are your recommendations for this current pregnancy?
First, a non-specific immune response, as I suspect John's case was, means that it is not likely to get worse with each pregnancy (as it would be with NAIT).

Second, probably not useful to keep testing for platelet antibodies in Jennifer's blood during this pregnancy. You didn't have them with John's case (meaning either that they are something that we don't know how to test for, or they weren't there, or they were non-specific enough that they didn't show up in our current screening protocols. Thus, they are unlikely to show up again in this pregnancy, and we have confirmed that already with one screening at 20 weeks. Platelet antibodies last about 6 months to a year, so any that you had from John would be gone by now.

An early delivery will probably not be necessary, unless there were a known event that would cause significant mixing of mom's and baby's blood (like placenta previa).

I concur with recommendation of Dr. Berkowitz, that you either plan a C-section to be safe, or else a cordocentesis to check the baby's platelet levels at 36-37 weeks. If the count is below 50,000, then I would recommend proceeding to an early C-section, probably after allowing about 48 hours on the medication to mature the baby's lungs. If the count is above 100,000, you could plan a normal vaginal delivery.

[Of course, as Jennifer's regular OB, Dr. R, pointed out with her typical good humor at the next visit, "Jennifer, of course you will get a count in between 50,000 and 100,000 and then we'll have to figure out what to do with that."]

Still waiting...

We have yet another medical consult tomorrow morning. That makes one every Thursday for the past month and they all involve at least half the day (there goes my "non-teaching day" for research time). Argh. But, I am still glad to have this appointment. I have been waiting anxiously for it.

Tomorrow, we meet with Dr. C, the pediatric hematologist who treated John at his birth 3 years ago. This is the partner of the doctor who told me that "without a doubt, John has NAIT, even though we can't confirm that with the lab tests." Since all the maternal-fetal medicine docs now are claiming that John didn't have NAIT (or that it is at best a good theory), I would like to discuss the case again with Dr. C.

And, it will be nice to talk directly to Dr. C this time. One thing that has been very frustrating through all of this is that each person we talk to only seems to have a fraction of the knowledge about our case, and only a fraction of the knowledge about NAIT in general. So, each is giving us an action plan based on incomplete data input. This frustrates the researcher in me. Get your data collected, sorted, and straight first. Then draw your conclusions.

My dad suggested that we go to a place like Mayo Clinic, because he has heard that they practice a "team-approach," getting all the various practictioners together in one room with the patient to discuss the case. That sounds very much like what I want (need?) right now. Unfortunately, Mayo doesn't have anyone on staff who specializes in NAIT. And, in any case, my doctors here are not sending me to another clinic for an on-site, complete consult. They just want to do the quick phone conversation route. Without me involved, apparently.

So, I feel that it is up to us to make our own "conference room." We will gather all the info we can from Dr. C. tomorrow. Then, we need to evaluate that and see if we can make a case for enough new or conflicting information to initiate our own call/email to Dr. Berkowitz in NYC. And then, I guess I am at the end of the line. At that point, I will exhausted all avenues and I will have to learn to accept the recommendations that I can get from the doctors that I have. As always, back where I started. This is all up to God. If I can just get myself out of the way.