Update

Back in January I posted about the strange episodes of discomfort HPEkid was having and our struggle to determine the cause. Through January and February we really didn't come up with any answers. Her spells of distress, leg tremors, and extreme tightness in her arms and upper body came and went several times, although never as powerful as they were in early January. However, blood work from those January doctor visits did show elevated serum sodium levels (153), so Dr. R (endocrinology) increased her DDAVP dose. It sort of made sense that perhaps HPEkid had just outgrown her DDAVP dose; she had been on the same dose for probably 5 years. But a week later her sodiums were still high. We increased DDAVP again (to double her prior dose) and they went down to 148, still high but going in the right direction. We tried to rule out a urinary tract infection and an initial urine test indicated high white blood cells, but further tests came back normal. One odd feature is that when HPEkid is having her 'episodes' she seems to hold in her urine - doesn't void for 18-24 hours, then lets it all loose in a real soaker. A couple of times the docs were unable to get urine samples even with a catheter.  This pattern doesn't really make sense to me in relation to her DI and sodium levels. If her DDAVP dose was too low she should be voiding heavily and frequently, flushing water from her system and causing the elevated sodiums. But if anything she was doing the opposite (holding on to water) and her sodiums were still spiking high. I still haven't heard a good explanation of this. 

Anyway, in March, after a few days without any episodes, her shaking and pain returned. HPEkid went in to see Dr. J (orthopedics) to check on her back brace, but also because it her left leg was clearly causing pain again. This time the x-ray revealed a fracture in her left tibia up near the knee. Dr. J said it was already about 80% healed so it was not a 'new' fracture. He made a splint for the leg to limit movement, mostly for her comfort. So now we have to fight with a scoliosis brace and a toe-to-thigh half cast on her leg! I guess it is pretty common for non-weight bearing kids to develop soft brittle bones that can break easily from simple every-day actions, so we are going in next week for bone density scans and have started calcium and vitamin D supplements. We hope the leg is the root cause of all HPEkid's recent discomfort. She is still having days where she seems uncomfortable and will have leg shaking and tightness, but lately it has not been to the extreme that it was earlier in the year.  But I am not entirely convinced that we have found the real culprit.  

These days HPEkid seems to cycle between three states: 1) significant discomfort and pain, leg tremors and pumping or lifting of the left leg, and generalized muscle tightness 2) when this passes, she goes absolutely limp, totally exhausted, sleeps deeply for a long time barely waking even to change diapers or dress, and 3) what we call 'normal' where she is happy and alert but moderately tight.  Oddly, through all of this, HPEkid's reflux and vomitting is not really a factor.  That is, it's not any better or worse than normal. Sometimes we've seen some brown fluid in her residual, possibly blood, but not enough to say it always coincides with her 'episodes'.

On top of it all, I have just started a new job and suddenly have less time to run her around to her different doctors.

Watch the tone

Today, it feels like C's trunk and upper body body are a bit more loose and floppy than usual. Sitting in my lap, she felt like a wet noodle, with absolutely no tone in her back/upper body. Having just increased her baclofen dose to 145mcg on Tuesday, we need to watch this. It could be that we have reached the limit to how high we can take the dose. Or it could be she was just tired this evening. Will have to alert her teachers and therapists and monitor over the next few weeks.

Baclofen

Took C. to the neuro-developmental center for a doctor's appointment to refill her baclofen pump and adjust the dose. We were cutting things pretty close, with the alarm date on the pump only two days away. Her last dose adjustment was in October at 130mcg/day. Today we went up to 145mcg/day. We continue to increase the dose gradually, trying to achieve maximum reduction of tone in C.'s legs without compromising trunk stability or head control. While overall, her high-tone is much improved since last year, she continues to show intermittent extension in the legs and occassional bouts of tremoring, especially when uncomfortable. Head control and alertness seems fine so we continue to up the dose.

Baclofen

A bit of background:
Baclofen is a muscle relaxant. It can be given orally to reduce high muscle tone and spasticity, but in oral form, the drug causes drowsiness and affects tone throughout the body. We tried oral baclofen for a while but a) never really saw much change in tone, and b) did want to do anything to compromise her few strengths - head control and sociability. In the Summer of 2007, C. was scheduled for surgery to her right hip (another post) and we decided to have a baclofen pump inserted at the same time. The pump is a device that delivers the baclofen directly to the spine - which means you can give much smaller doses and, depending on where you place the catheter, target only those muscles below the insertion point. In C's case we are targeting only her legs. I will save discussion of surgery and the hospital stay for another day - suffice to say C had the pump implanted in July 2007, went home 10 days later, but developed dramatic swelling around the surgical site, which eventually had to be drained via syringe. Some time after this, the pump actually flipped over in it's cavity beneath the skin which twisted the catheter to such a degree that the baclofen was no longer flowing. The pump had to be surgically revised in March 2008. Since then, she's been soing fine. Slowly increasing the dose and delivery rate of the baclofen. We have to say that in C's case the results are not particularly dramatic. Her legs and hips are significantly looser than before, making it easier to change diapers and get dressed. But at times her tone still kicks in, espacially if she's sick, in discomfort, constipated, or just plain mad. Baclofen is not, for us, a miracle drug - she's not suddenly able to sit or walk or anything (and we did not ever hope for that). It's simply another tool we use to manage her symptoms and try to make her life (and ours) a bit easier.