Metachromatic Leukodystrophy (MLD)

Merry Christmas to all.  This has been a challenging time for our family, and quite honestly, gets a little more complicated as time marches on.  Nonetheless, we are blessed for the support and prayers of our family and friends.  The images below are from Christmas morning.  My brother, Jon, and his wife, Jennifer, came up Christmas morning to spend some time with us.  My father joined us also. 

The first image (left) shows Emily and Jon helping Laura unwrap a present and the image to the right shows Darlene looking inside one of her presents.

And mentioning Darlene - I am amazed at the loving compassion she shows in all she does.  Below is an excerpt of an email she sent to our doctor seeking advise:

Dr. xxxxxx,
Laura has been taking Baclofen ....... This morning she was in pain when my husband came in her room at 6am to give her Baclofen. Yesterday at 1:45 am I woke up to Laura in pain and her left leg was contracted and she couldn't straighten it out on her own. The increased dose at night has helped but she still is having pain.

I have found that I need to allow the medicine 2 hours to take effect before I try to move her and that after 4 hours it has worn off. This leaves a narrow amount of time for activities. In the morning I get her out of bed after 8am and transfer her into a transport wheelchair and into the bathroom on the toilet, ……. transfer into the transport WC to the stair glide, transfer into the stair glide, then transfer her into her custom WC, and feed her breakfast. If I move her or take her to the bathroom after 10 or 11am she is in a lot of pain. She moans and grimaces and both of her legs stiffen straight out and it is very difficult to bend her legs. Sometimes her ….. and that is not good. I have to plan when to take her to appointments and try to plan when I can try to get her to do some exercises without inflicting pain. This is close to impossible. She has a clinic appointment at Hopkins......., which means we will leave our house around 12 when I give her Baclofen. She will be in pain when I transfer her into the car and out of the car. Is there something she can take as needed for pain relief? Should I be giving her Motrin 600-800mg? I don't know if that is a good solution long term but it might be helpful once in a while. What are your thoughts?

Sunday I attended church and was touched by one of the songs, which included the following verse:

Scars and struggles on the way
But with joy our hearts can say
Never once did we ever walk alone
Carried by Your constant grace
Held within Your perfect peace
Never once, no, we never walk alone