Livvy’s 1st Seizure

While I wouldn’t normally write a blog about something like this, I felt it was almost my duty as an MLD parent to share our story. Even the upsetting or sad parts. Because MLD is so rare some doctors have never even heard of it. And oftentimes it’s the other MLD parents who I get the best information from. Because they have been there done that and know what works (and what doesn’t). The amount of information on the internet regarding Metachromatic Leukodystrophy is alarmingly small.

So in case there is another parent in my shoes looking for answers, I wanted to share another part of our journey. Today, Livvy had her first seizure. I say first because seizures can be common in MLD children and once they happen they are more likely to reoccur.

This morning when we went to get her out of bed, we found she had thrown up and was actively having a seizure. I rushed to get the Diazepam (Valium) and as our doctors instructed gave her 10mL to stop the seizure. It took anywhere from 15-30 minutes for it to stop. We got her all cleaned up, and stayed by her side talking to her until she was able to fall asleep.

In the meantime, I had also called Hospice of the Valley who had a nurse on their way (aptly named Angel) and called both of our mothers, who rushed over.

Hospice took her vitals, which were normal. Said we did all the right things and the nurse called the doctor to get his thoughts. He initially recommended Attivan but we had tried that with Livvy before simply for anxiety and she did not like it at all. Was very uncomfortable. So we asked to just remain on Diazepam, which they thought was fine.

While they were here we also called the girls’ neurologist Dr. Vinodh Narayanan. He got back to me after Hospice had left and recommended we first get an EEG to check activity before deciding on a plan. He also recommended a nasal spray form of Diazepam (which I can’t remember the name of now but will update the post once I get it). This comes in two bottles, each 5mg. Use one bottle in one nostril and if it doesn’t stop the seizure you use the other bottle. He recommended this since it can be more quick acting than administering Diazepam through her G-Tube.

After we got off the phone I also sent him a video of Livvy mid-seizure. It’s terrible to have to take a video of your child while they’re undergoing something so traumatic but it came in handy and was important for our doctor to fully understand what was happening and decide the best course of action.

From the video, he thought it looked like she was having an epileptic seizure; a complex partial type, rather than a grand mal convulsion. Based on that he recommended we start anti-seizure medication right away but still get the EEG done (which can be done here at home) and use Diazepam when they do occur.

The Hospice doctor suspected that her immune system may have been weakened by a viral infection which lowered her threshold for seizures. Given that we were all sick last week (not from Covid) and she threw up again later in the day we think he was right. We had hoped since it had been a week we had done enough to keep her clear of it but unfortunately not. And as is the case with MLD kiddos, anytime they get sick their disease progresses (another reason we have still been semi-quarantining and taking extra measures to keep the girls safe and healthy).

Since the seizure Livvy can no longer move her left arm or control facial movements. Things that would normally make her smile so big (like talking about her baby sister Keira) don’t get a reaction. And it almost seemed as if she realized she couldn’t do it and got sad and almost started crying. I told her it was OK and that sometimes after seizures we can’t move like we used to but it can come back. As I have now learned post-seizure paralysis can be temporary. So we will see how it goes in the days ahead and keep everyone updated.

Love from the Rileys. ❤

Livvy resting after her seizure.

Enrolling in Hospice

Last week, we enrolled Livvy in Hospice of the Valley. Not because she is drastically and rapidly declining, but more so to have that support system in place for when she does and in order to have physicians come to her rather than putting her in her stroller and driving anywhere (she has always hated carseats and found them very uncomfortable since her diagnosis), or having her anywhere near COVID patients at a local hospital.

Surprisingly, to me, this process was not as difficult or emotionally trying as I thought it would be. Honestly, the hardest part was talking to our palliative care team about end of life scenarios prior to enrolling in Hospice. That is how we came to the decision that it was indeed a good time to enroll Livvy.

And what might come as a surprise to many is that enrolling a child in Hospice is not the same as enrolling an adult. For an adult, Hospice means they have very little time left. For a child, like one with MLD, they could be on Hospice for years (or days…there really is no way of knowing because the disease is so rare). But the point of enrolling a child is not only to ensure end of life scenarios are completed as the patient’s family prefers (at home versus in a hospital with a million tubes connected to them), but also to ensure their comfort until that point arrives.

Livvy’s meds will now be delivered instead of us running back and forth to Walgreens, a Pediatric nurse and social worker will come to the house to check on her every two weeks (or as needed) and a pediatrician will visit every two months (or as needed), and we now have a number to call them 24 hours a day 7 days a week for any medical questions or concerns rather than guessing what’s wrong and having to rush to the ER. Any time, day or night, they will come to her.

For the parents, siblings and patient, this is a game changer. It keeps disruptions and any further trauma to a minimum. We are incredibly grateful to have their support as we contunue to navigate this heartbreaking and unbelievable situation.

For more information on Hospice of the Valley, visit www.hov.org.

One of many lovely photos of Livvy captured by our friend and photographer Kay Eskridge of Images By Kay. ❤