Losing Brandon

Over the past few years I have for the most part dropped out of the Holoprosencephaly support community.  After setting up and running an email message board for a few years, I have slowly found myself posting and reading less frequently.  As day-to-day care for our HPEkid became more routine and other aspects of our life began to take over I have really lost touch with most the families of children with HPE who I had come to know in the early 2000's. Last night, via Facebook, I learned of the passing of 13-year-old Brandon. Brandon was the first other child with HPE my wife and I had ever met.  We were living in Maryland (this was probably around 1999) and had at that point made email contact with perhaps 3 or 4 other families from across the country.  We learned that Brandon and his mom would be coming to Baltimore to see the HPE experts at Kennedy Krieger institute, so we drove an hour up I-95 after work to meet them.  Brandon was just a baby, definitely under one year of age, and there were some physical differences in how his HPE expressed itself, but it was so uplifting to see another living child when so much of what we were reading about HPE was truly dire.  He shared our HPEkids engaging smile and we instantly felt a connection to Brandon's parents; the camaraderie of knowing we were each going though similar experiences.  We met Brandon and his family two more times, at HPE Conferences held at NIH in Maryland.  I'll always remember him showing up in the hotel lobby one night with his wheelchair tricked out as an army tank.  Now, it has probably been 6 years or more since we've spoken to Brandon's parents and really been up-to-date on his life. So last night, after learning of Brandon's death I went on the HPE group discussion board to post a comment.  I did some searching of the archives and found that another of the very first living HPE children I ever knew (Zuzu Smith, from England whom we never met in person) had also died some years ago.  I had come to grips early on with the fact that children with HPE can die from any number of complications early in life.  But it is sad to learn that some of the "old guard" children, whom we met in the early days, have also passed.  We've become complacent, I suppose, feeling that our HPEkid is stable and will remain this way forever.  This news brings me back to earth to face the fact that HPE and it's attendant complications can always throw surprises our way. 

When she can't tell you what hurts

It's a new year and time to re-start this blog.

Spent much of the day at Children's Hospital trying to figure out what is causing HPEkid's latest bout of discomfort,  Since Friday night she has been in obvious distress. Saturday was the worst; she was uncomfortable in almost any position, but especially upright in her chair with back brace on. Her legs were shaking and tremoring almost constantly, especially on her left, and she was lifting and pumping the left leg (which normally she is pretty much unable to do). Her arms have been pulled back tightly at the shoulders and hands clenched.  Her face has been flushed and she is sweaty and hot, but not like a fever, just seems hot with being so tight and the effort of straining against her brace/chair.  Something feels different to me about putting on her brace, somehow the shape of her chest/ribs/hips feels different.  She sometimes laughs, almost manically, but then goes into a look of real concern/fear.  Little things set her off and seem to startle her.  Sometimes when lying on her back she will clutch at the air with her arms and vocalize, eyes large as though she's afraid of something. Her temperatue has been around 100.

 So today we got in to see Dr. S. who manages her spasticity. One of the first suspects when she is so uncomfortable and tight is that her baclofen pump may be malfunctioning and she could be in baclofen withdrawal.  Dr. S. manipulated HPEkid's legs and while she grimaced a few times, she also laughed often, but was clearly not herself.  Clearly her left leg and side were causing more discomfort than her right.  He ordered a series of x-rays to assess the pump connections and to look at her left hip, knee and leg. And he programmed her pump to deliver a high dose bolus, a quick shot of baclofen so we could watch for some response to further indicate whather the pump is functioning properly.  While waiting for x-rays, I felt it was pretty clear that the baclofen bolus worked - her tremoring diminished and she was sitting much more comfortably for the 2 hours we were there. Everything looked OK on the x-rays.  Pump and all tubing was connected and in place. Hip and pelvis all looked unchanged from other recent scnas she has had.  So we are fairly confident there is no pump or acute orthopedic issue.  The one thing noted on the x-ray was that her colon is quite full and she may be constipated. So for now we are going with the constipation theory. We will go in on Wednesday to her PCP to rule out any other underlying illness or signs of infection.

It is so maddening and frustrating as a parent, when you know your kid is in pain and just "not right" but cannot tell what is wrong.  And it must be doubly frustrating for her to not be able to tell us where it hurts.

HPEKid's first day of school was yesterday. Went fairly well, although we got no communication letter home at the end of the day. For the first time, neither HPEmom nor I went in to school on the first day. Claire really lit up getting on the bus, but was totally exhausted upon getting home.

Summer went pretty well. She did 5 weeks of CYO Daycamp and 4 weeks of ESY (extended school program). We traveled to Maryland for a week to visit family and hang out at Aunt Sarah's pool.

HPEkid also got a ceiling lift and track system installed in her bed room. The track carries her straight into the bathtub. For the most part HPEkid enjoys the ride, although bathing her is still not an 'easy' thing. Haven't got the nurses used to using the lift regularly, and in truth, we don't use it for all transfers to her chair and bed. Pretty much it is just used for bath time. Still, it is there if we need it.

Following a nutritional analysis in June, HPEKid is now on a more appropriate diet, switching from Pediasure to Jevity. Her endocrinologist was concerned about lack of weight gain over the last year.

The other medical development this summer is that HPEkid's scoliosis has progressed significantly in the past several months. We think it may be the cause of much of her discomfort and refusal to sit up in her chair. Her X-ray from last week is here. So she has been fitted for a back brace which should arrive in Spetemeber. The goal at this point is to prevent the curve of her back (currently at 39 degrees) from getting worse. No surgery at this point, thankfully.

Botox Dangers

Saw this article today about a girl with cerebral palsy who died of pneumonia after receiving botox injections for her spastic muscles. Her parents sued Botox maker, Allergan, saying that an overdose of Botox caused weakened the muscles controlling her breathing. I won't debate the legal decision, although I would tend to agree that the drug manufacturer isn't at fault and so many other factors are at play when you have a fragile kid with CP and pneumonia. It just makes me stop and think. When HPEKid gets her botox injections Dr. S. always warns us to watch for labored breathing and makes it very clear that respiratory problems are a risk. With this article however, I am surely going to verify the dose that C. is receiving. Anything over 8 units per kilo of body weight is considered an overdose by the manufacturer. We've been feeling lately that C's botox seems to be wearing off more quickly and have less effect on her tight muscles (she gets Botox in her pectorals and biceps), and have been wondering if we should/could increase the dose. Complicating things for C., is that she also has the implanted baclofen pump delivering a different muscle relaxing drug to her lower extemeties, and labored breathing is a potential concern with balofen overdose as well.