A science lesson and medicines to keep Oliver comfortable

His breathing is completely back to normal yesterday and today! We are so happy! Just another crazy MLD symptom apparently. This would be a great time to have a team of doctors who understood the disease, or a book to read on the topic, or a website that tells us more than just the definition of metachromatic leukodystrophy. There is no information out there, the disease is so rare and every case is so different. It's frustrating, lonely and very scary. We never know when or what to expect. We've asked his doctors. They don't have any answers.

We do know that his brain sends his body all kinds of crazy messages as the myelin sheath continues to deteriorate. The myelin sheath insulates nerve cells as they send electric signals throughout the brain and to other parts of the body. Think of the myelin as the insulation around an electrical wire, without it the wires get very hot, break down, short circuit and stop working. This is what happens with Ollie. His brain send signals, or tries to send signals to different muscles and systems in his body, but as the nerve cells get damaged without the protective myelin, the signals misfire, repeat themselves, or never make it to their destination. All of this causes painful muscles spasms, seizures, paralysis and the inability to coordinate his muscles to do meaningful actions, such as walk, talk, eat and apparently can cause strange breathing patterns.

To keep Oliver comfortable he is now on 7 medications.

Baclofen for muscle spasms and stiffness
Keppra for seizures
Neurotin for nerve pain through out his body
Ranitidine for acid reflux
Diazepam for seizures, muscle spasms, anxiety and as a rescue drug
Ibuprofen for pain
Morphine for intense pain and to regulate breathing
(And he gets Botox injections for muscle stiffness every 3 -4 months)
He also gets miralax and a multivitamin 1x a day

Most of these meds are 3x/day, some are 2x/day, and some are every 6 hours (so 4x/day), so we have to set an alarm and get up in the middle of the night to administer them. Some have the same dose throughout the day, while others have different doses for morning, noon and night. Sometimes he gets additional diazepam or morphine based on his need. It can all get confusing and overwhelming, but we've worked out a system. At all times we have an emergency medicine tray on the table. It has his seizure and pain medicines measured out in their syringes and ready to go. That way, in the event of a seizure or screaming pain we don't have to do it under stress and pressure, trying with shaky hands to hold a tiny glass bottle and carefully measure out 1.25ml, 5ml, or was it .5ml?!? We also set up his morning medicines the night before and put them in the fridge. After we administer the morning meds, we set up the lunch meds, after the lunch meds we set up the dinner meds. The trays are always ready and waiting so no medicines are late. Timing is important for keeping seizures at bay and pain away. Diazepam at 5 and 11am and 5 and 11pm. Raditidine at 7:30am and 7:30pm. Keppra 3ml breakfast, 4ml lunch, 3ml dinner. Or was it 4,3,4?

It can make your mind spin.

A typical dinner medicine tray looks like this...




In some ways it is easier to give him his medicines nowadays since everything is through the gtube, but in early August when he could still swallow he was only on 3 medications. 2 months later and he's now at 7 and the doses are constantly being adjusted. Crazy!

I think I'm going to take a nap, even writing this made me tired.