There was (maybe still is, I’m not sure) a sports columnist for the local newspaper by the name of Gary Nuhn. Fairly unremarkable, sort of a homeless man’s version of Rick Reilly, he had an occasional column that he called “Notes, Quotes, and Billy Goats”. These columns were more of a series of thoughts than a coherent story. I have no idea why, but that phrase always stuck with me. So, in order to catch you up on the past couple of months, I present to you the Armand’s Hope version of “Notes, Quotes, and Billy Goats”.
• When we last left our hero, he had just begun on his ketogenic diet. Initially, he was on KetoCal 4:1, which means that he’s getting 4g of fat for every 1g of carbohydrates and protein. That seemed to work well to bring his seizures down from 3-4 every day to 1-2 every day. Progress made.
• In December we had a meeting with Armand’s neurologist, Dr. Collins. They wanted to further adjust his formula, taking out part of the KetoCal, and adding in some MCT oil (for added fat) and a small amount of protein powder. This lasted all of one day – Armand’s tummy wasn’t having any of it. A couple of phone calls to the dieticians put him back on the original KetoCal, with the idea that we would try to start occasionally giving him small amounts of protein food by mouth (chicken, turkey, hamburger, eggs, etc.) that was cooked in the MCT oil, or things like avocado. Any carbs he would happen to get by mouth needed to be counterbalanced with even more oil / protein. We’ve had no issues with him getting any carbs, but he has had several tastes of food in very very very very small bites and portions. Progress made.
• Also at this December meeting, we made adjustments to his seizure medications, increasing the dose of Lamictal that he gets while continuing his Onfi. Lamictal can have the side effect of skin rashes, which we haven’t seen (yet). Progress made.
• As a result of some combination of the diet, his seizure meds, and his drug cocktail to manage his GM1, his seizures have dropped down from 1-2 per day to basically 1-2 per week (if that). He actually went from Christmas Day to January 3rd seizure-free, then went from January 5th – 12th seizure free. He did have a small one last night, and another
small one this morning at the airport three today, but even the severity of the seizures has gone way down to the point we even question “Is he having…. I’m not sure…. Well, maybe…. See his legs?…. He’s not making any no– yep, he’s having a seizure.” Progress made.
• With the seizures subsiding a bit, or at least beginning to come under control, his personality is coming back. He’s full of laughs and smiles again much of the day. He kicks his feet again, he wants to work hard on doing things that he used to in order to get some strength back. For a while he would just let his head drop in a car seat, now he’s back to holding it up high looking around out the windows. He giggles for no particular reason and just overall seems content much of the time. Progress made.
• One minor issue we’ve had really over the last couple of days is that he has begun biting his fingers. And I don’t mean small nibbles, I mean that he clamps down good, and then because it hurts he grits his teeth harder and cries instead of opening his mouth to cry. I’ve had to essentially pry his jaws open in order to extricate his finger. Usually he leaves big, deep teeth marks, and even making his fingers swell up. One theory is that his mind regressed to the point that he’s almost neurologically starting over, so like an infant he’s feeling his way and becoming more aware of himself again. Progress made?
• At school, he uses what the kids call his “magic button”. It’s basically a large device that has pre-recorded sayings or messages from his teachers who act as his “voice” to the rest of the class. For instance, they will help him press the button and a message will play “from Armand” saying which song they should sing. It’s a fantastic idea that seems to have helped his classmates acclimate to the fact that Armand can’t communicate very well but still lets him interact with the class in some form. I really can’t say enough good things about his school, his teachers, his therapists, and the teachers’ aides and the level of interaction and care they give him. Progress made.
• Armand is also now a big bus riding boy. Each morning around 12:40 he gets picked up in his Kimba wheelchair and loaded on the bus for school. He’s had no problems, he doesn’t get scared and he gets treated extremely well. Progress made.
• We’ve just started working on getting some of his physical skills back. We do tummy time not only to get him to start rolling over again, but also because he works so hard at lifting his head up. Really, we have to look at him physically like a giant 38lb. 4-month old. BUT, just a few months ago he was really a giant 34lb. noodle. Now he works hard, and you can see on his face that he not only enjoys doing it, but seems to realize he’s doing something well. Progress made.
• We got him a new LeapPad2 for Christmas, and his family provided some great useful apps. We also do music therapy with some therapeutic headphones, the exact same brand they use at his school. He does a lot of listening to classical, especially Mozart, which he really does seem to enjoy – it relaxes him. You can see a change in his whole body language songs come on that he particularly likes. Progress made.
Sometimes it’s hard to look at where he is now as progress, when compared to this time last year when we took a vacation trip to San Diego. He was still walking then, he hadn’t started having seizures, he was feeding himself eating full meals. Now he can barely hold himself up, eats 99.9% of his calories through a feeding tube, and for a time was far less responsive and emotional. But I have to look ahead to 2013 with a different outlook and expectation for him. We do believe that he will start talking again. We do believe that he will start walking again. It may be a long time, but the science backs up that this is a possibility, and we’ve seen a lot of small but significant changes for the better over the last couple of months.
February, we will once again celebrate (is that even the right word?) Rare Disease Day with millions of others around the world. This past year we got an official proclamation from the City of Dayton, as well as a radio interview with Kim Faris of Lite 94.5 FM. We are hoping to expand on this, and ask that you begin thinking of small ways to join us, even if it is simple things like wearing jeans, making a donation to NORD or Armand’s Hope or one of the other organizations that benefit pediatric rare disease patients. We ask that you share Armand’s blog on your facebook or twitter, send out an awareness email, say a prayer for these children, or other things that would just take a few moments of your time. It’s through people sharing information that we’ve been able to touch several lives of parents and family members of children with GM1 or similar disorders. And it’s through this sharing of information that there is some sense of community, that people with rare disorders don’t feel quite so disoriented, alone, and without hope.
I can’t thank you enough for following us.
By the time you read this we should be in Minneapolis again for another round of testing and meetings with doctors. Wish us luck, and I’ll be here all week. Please tip your waitresses.