Thursday, April 16, 2009

Kaiya's Story

Our daughter Kaiya was born at 40 weeks 1 day gestation in the hospital and it was an uncomplicated birth. She was 7 lbs 9 ounces, 51 cm in length, and had a 34 cm head circumference. She is the second child in our family; her sister Mylie (we named her that long before Miley Cyrus surfaced thinking she would never meet another Mylie in her whole lifetime having such a unique name) was born in 2004 and is a healthy little girl. We knew something was wrong with Kaiya almost from the day she was born. She had strange choking and “blue” events in the hospital and for the next day after we brought her home. The nurses assured us it was just mucous but it was unlike the attempts at clearing the left over mucous from birth that my first daughter had when she was born.

The doctor who delivered Kaiya noticed immediately from birth that she favored turning her head to her left shoulder. She suggested we watch it and try to have her look at toys in the opposite direction and hold her to encourage her to look the opposite direction. We noticed it off and on to different degrees for the next two months. Some days she seemed to have tortipelvis in the opposite direction of her head tilt as well. She looked like a pained banana. We would always notice it upon waking in the morning. Some days it was very prominent other days she seemed absolutely normal. We could manipulate it out of that tilted position, but it was not comfortable for her. We saw two physiotherapists during this time who gave us some suggestions on how to treat “muscular torticollis”. We adhered to the program religiously and it appeared to have no correlation to the days when she was straight or tilted. At 3.5 months she was still unable to hold her head up on her own at all which concerned us so we asked our family doctor about it and he referred us to a paediatrician. During this time she was feeding and growing well. She has always had a voracious appetite. We would spend the next 3 months watching incidents happen with increasing frequency and severity while waiting to see specialists at BC Children’s Hospital in Vancouver British Columbia.

In addition to her tilts at 4 months she was still having around 14 frothy mucous stools each day which started having blood in them. The hospital diagnosed her with a cow’s milk protein allergy that came through my breast milk. I cut out all dairy and her stools normalized immediately. It was at this time that they thought she had “Sandifer’s Syndrome” that was causing the tilts. Sandifer’s Syndrome is severe acid reflux which forces the body to contort into strange positions to relieve the discomfort. So she was put on Ranitidine (zantac). It had no effect.

Like everyone else, were bounced around in the medical system waiting for tests and appointments with specialists until she was 6-7 months old. Until that point, we through our research, were beginning to suspect BPT but had not had it confirmed. She has had EEG’s, an MRI, a fentogram, x-rays, blood tests, genetic tests to date. She has seen neurology and ear nose and throat specialists at our local Children’s Hospital. In the future they will do a cold caloric test (on her ears) to learn more about her vertigo.

We have experienced two distinct types of tilting with Kaiya, but both types always have sometime type of triggering event (cold, teething etc.). They usually come on at the tail end of a sickness not the beginning. For the first 6 months of her life the longer uneventful tilts were more common. As she grew older nearly all the events became the short, eventful violent ones until as of more recently when she has started having some events with just vertigo.. It wasn’t until she started having the violent eventful ones that an absolute diagnosis of BPT could be made. She is now 17 months old and has about 25 events since she was born and has had numerous days of just vertigo. The two types of events we have seen with Kaiya are:

· Long duration (multiple days….up to 9 days long), but uneventful. She will wake up in the morning with a tilt (varying degree and direction and sometimes includes her pelvis in the opposite direction, and sometimes would include limited use of one of her arms) and it will stay for many days until she wakes up one day and it is just gone and she is fine. These are usually preceded by some grumpiness, but not always. Sleep is often disrupted and she appears to be in discomfort when they are happening.
· Short duration (under 12 hours) and eventful. She wakes up with a tilt that usually only involves her neck and not her trunk. When she first started having this type of short eventful tilt there would be a 6-8 hour lag from the onset of the tilt before the vomiting and “seizure like” cycles started but the last few months that lag has become much less usually with only 15-30 minutes’ notice. She often has balance loss. She is always extremely grumpy. She becomes quiet and calm and goes very pale. Her eyes often start rolling. She starts rhythmically vomiting. After she finishes vomiting she is hypotonic, unresponsive, and hesitant to move. If she does not go into a recovery sleep (deep) immediately she will lie with her eyes open staring at the wall and not move…she will be limp. She will repeat this pattern for the next 6-8 hours anywhere from 3-8 times each hour. Essentially “having checked out” for the entire duration. This is what we refer to as an “incident”. These are disturbing to watch because she appears to be having some sort of “seizure activity”, but the EEG’s determined no seizure activity is taking place in her brain when she is behaving this way. With each event she has the number of cycles we are having is increasing. The first few tilts of this nature she only had 4-6 vomiting cycles and the longest one she was up to 23. Dehydration is a concern as she does not eat or drink during the events, she will nurse a tiny bit but only if she is in a deep part of her recovery sleep. She is comforted to be held the whole time and held upright when she is vomiting. She starts to vomit blood after a while if she has had a lot of the cycles which is always alarming for any parent.

Today Kaiya is 17 months old and is a sweet, gentle, smart, lovely girl. She is very tall for her age and has gorgeous curly hair. She is developmentally normal and has a great sense of humour. She began walking a day after her 1st birthday. She might have done it on her birthday but she was in the hospital due to a bad event. She was vomiting copious amounts of blood- much more than usual which really scared us. She is still unable to tolerate cow’s milk protein. I am currently weaning her, but she does not enjoy goat’s milk at all.

We are optimistic that she might “outgrow” BPT. In the last 4 months her incidents are becoming further and further apart and she appears to be having vertigo more often in the place of a “full on event” that we would expect when she gets a cold. We are currently awaiting the results of her MRI and genetic tests.

I would like to say thank you to Richelle for putting this site together so that our families and other people who are touched by BPT have a place to connect. It can be scary especially at first and having other people to share your story with and compare with can help so much.


CMK said...

conscKaiya's story is so close to what has been going on with my Sean I could have written her post myself. Sean is one now and the first 6 months he also had more of the longer duration episodes. Then he gradually began to vomit 2 or 3 times a few hours after the tilt began and then fall asleep. Now the vomiting begins almost immediately. Repeats every 20 minutes or so and lasts about 12 hours. So far we've been lucky to avoid the hospital. He comes out of it right about the time I decide to bring him in for an IV. None of my doctors have ever seen this before. I just keep hoping for hte day that I can say it's over. I've noticed the spells getting further apart. Fingers crossed I'm not jinxing myself by saying that.

Irene said...

HEEEEELP!!! I don't know if you still check this post or this blog! Have you ruled out that Kaiya's BPT was related to oversupply in breastmilk - did she have more than 5 poops a day, and more than 10 diapers a day, if you can remember that far back? I wonder if that results in the child attempting to provide self a diuhrettic for the calcium channels to function better. How about drooling - it can lead to liquid stools, apparently. How about - goat milk still has the same or very similar protein to cow milk in it and may have a slight effect. Any family history of hearing loss? Other allergies?