Saturday, December 11, 2004

Reflex Anoxic Seizures

Wednesday morning I had just entered my office before setting out for my 10:00 AM class when the phone rang: Kasia, our 1 year old, had stopped breathing at day care and was being rushed to the hospital.

Mary and I raced to the car; Mary used her cell to call around to various colleagues to arrange coverage for my class, mostly to distract herself while I drove. I had gone into standard male denial mode, so I was okay to drive: Kasia had previously been diagnosed with Cyanotic Breath-Holding, so I assumed this was more of the same, though there was the nagging doubt that the day care workers had dealt with that before and not freaked, so perhaps this was something else.

The Breath-holding incidents were scary enough: something would upset Kasia (a bump or poke; taking something she was trying to put into her mouth away from her; sometimes nothing at all that we could recognize as the initiating stimulus) and her crying would quickly escalate to the point where she would be unable to catch her breath between screams, would hyperventilate, and then suddenly she had stop breathing, turned blue, and passed out. On other occasions she would skip the crying stage altogether, and simply stop breathing, turn blue, and pass out – a process indistinguishable from a child choking to death.

Indeed, we now realize that Kasia's first spell was one we had initially interpreted as a chocking incident one weekend in Costco's food court, in which it appeared that Kasia had only survived thanks to the timely intervention of a nurse seated at the next table. I remember having been appalled at how fast Kasia had started to turn blue – I had just started to say, "I think there is something wrong with Kasia", when a woman at the next table called out, "She's choking!" For the first time ever, Mary froze as Kasia's eyes started to roll up; when a another woman stepped forward, identifying herself as a nurse, Mary thrust Kasia into her arms. The woman whacked Kasia on the back a couple of times as she yelled to her husband to call 911. I had a sinking feeling it was too late to call for an ambulance, as Kasia was already blue, and we were a long way from the hospital. But suddenly Kasia was crying, and the woman was pointing to a piece of chicken that had popped out of Kasia that looked about choking size. In retrospect, it now seems more likely that this was her first full blown Cyanotic spell, rather than choking, but we spend the next month breaking Kasia's food into microscopic crumbs before putting them on her plate…..

The next incident a couple of weeks later sent us rushing to the hospital, where the doctor diagnosed a Cyanotic Breath-holding Spell. The doctor emphasized that this was a genetically determined physiological response, and not willfulness. Mary immediately researched the syndrome through the internet and discovered that these spells occur in a small number of children after 6 months of age, peak at two, and are gone by age 6. Although terrifying for parents and bystanders, they are essentially harmless. After reading through the literature, I came to see it as a kind of "reboot" mechanism -- having reached a level of hysteria from which Kasia could not regain control, the system shuts down, reboots, and returns the child to normal crying.

After our experiences with these initial episodes, we had briefed the day care staff, and a few days later, Kasia had inserted her finger in the mouth of another daycare baby, who had promptly clamped down on Kasia's finger with her new teeth, and Kasia had had a spell. The day care staff told us that they now appreciated what we had been talking about / going through, but basically they had too coped.

Wednesday was different. One of the workers had taken a toy away from Kasia, which Kasia had coped with, if somewhat grumpily. A second staffer, however, had taken a second toy away when she spotted a piece of tinsel wrapped around it. This had proved too much for Kasia. She had become hysterical, held her breath, gone rigid, and passed out. The staffer was prepared for this... except Kasia did not come round. Instead of starting to cry, she remained non-responsive, and did not resume breathing. The worker called for, Jan, the head of the day care, who immediately decided to transport Kasia to hospital. Jan made the conscious decision not to speed and to wait for traffic lights, on the reasonable grounds that an accident would not help. (She did, she said, use the horn.) She drove while the other staffer held Kasia...who by this time was limp, her eyes rolled up into her head, for all appearances dead. Fortunately, the day care is not far from the hospital, and they made it to Emergency in literally 2 minutes.

One thing we will say for Lethbridge is that we have always found the Emergency room staff to be very responsive. When the day care staff came through the doors carrying a limp baby, a group of doctors and nurses immediately took her to the back and began undressing her and attaching her to every device known to modern medicine. Total elapsed time since start of the incident: 4 minutes. At which point, Kasia began to cry.

Mary and I arrived about two minutes after that. I dropped Mary at the door, and parked the car. I followed less than a minute later, running into the day care workers, coming out. They stopped and briefed me in detail, so I could tell the doctors later. (They had of course also told the triage nurse everything.) I was immensely impressed with how well they had coped with the emergency. I have the greatest respect for the head of this day care, a professional of the first order, and a take-charge personality if ever there was one. I can think of no one I would rather have in an emergency such as this. Just the fact that she had the presence of mind to note the times at each step so she could tell the doctors, was indicative of her calm and efficient response.

I then went in to join my wife and daughter, having been listening to the reassuring sound of Kasia screaming her lungs out the entire time I was debriefing the day care staff. While Mary reassured me that the doctors had said Kasia would be okay, and my briefing her on what the day care staff had told me, the day care owner's twin sister showed up. I confess I hadn't realized at first that it was the sister, since they are identical twins, but it eventually penetrated that she had left her day care to come and support her sister, Jan, in what everyone had supposed was the death of a child in her care.

Eventually the doctor showed up and after speaking with us, arranging for a follow-up appointment with our pediatrician (no mean feat in a city where there are too few pediatricians for the population), he sent us home with the diagnosis that this had likely been an extended breath-holding incident, and implied that the day care workers had perhaps over-reacted. "You probably wouldn't have brought her in had it happened when she was with you." I personally doubted this, knowing my own levels of terror and the calm professionalism of the day care people, and especially given the strong sense from the staff that this had been something new and different.

As an aside, the doctor mentioned that the blood tests had come back showing Kasia had anemia. When Mary got on line, it became immediately obvious that anemia is highly correlated with both the Cyanotic Spells and with a rarer second Pallid form. The more we read about the Pallid form, the more obvious that was what Kasia had had. Including the inconsolable crying that lasted for hours afterwards....reading the symptoms on these sites was like listening to a replay of the Day care staff debrief....

The next development came when Mary phoned her folks with the news...and they talked about her attacks when she was a child. Of course, in those days people assumed that the spells were the result of willful behavior and sibling rivalry (her sister being born when Mary was 18 months) or, eventually, of epilepsy. Mary's attacks had been frequent enough to label her a "difficult child" but not so frequent that the family ever got used to them… This is somewhat reassuring, since it suggest Kasia's spells might also be relatively infrequent, say, once every couple of months. In contrast, the websites report that some children have Cyanotic spells four or five times a day; and some children with the rarer and far more frightening pallid form suffer episodes as often as twice a day.

When Mary read out the description of pallid form to me, my "oh-well,-they-are-just harmless-spells" attitude collapsed. Apparently, the heart actually stops, the lungs and brain shut down, and it is only when the fail safe mechanism kicks in to restart the heart that the child comes out of it -- to cry for hours and then sleep....

"The heart stops?!" I demand.

"That's what leads to the paleness…there is no blood circulating."

A knot formed in the middle of my stomach that hasn't yet dissolved. I am definitely not okay with my daughter's heart stopping, albeit briefly….

We haven't seen the pediatrician yet, so our self-diagnosis is necessarily tentative --- hopefully, we are not dealing with something worse! But the symptoms match very closely, including the genetic component. And of course, it is all very frightening. The fact that Mary suffered from the same condition as a child and is a here as an adult validates the various websites insistence that the condition is not fatal, poses no threat to, or strain on, heart, brain, or lungs, etc., but such rational considerations do not hold much weight in the face of one's child turning blue or remaining unresponsive for minutes at a time, as you wait to find out if it is just another spell or whether you've just sat there while you child choked to death on a piece of broccoli. The spells may not place any strain on the child's heart, but let me tell you, it sure as hell strains the parents'.

References: tipdergi/eng/03_2002ing/03_01ing..pdf