Fight, Willy, Fight!

I love fall.  Leaves, crisp weather, hoodies, football, hot chocolate, pumpkin everything, hayrides, pneumonia.... Wait, what??  Yep. Pneumonia.  For Willy that is. We've had a good, long run of fantastic health so it was bound to happen. And happen it did. 

Some background. 

Willy's lissencephaly is a regressive disorder.  This means his body's performance peaks, well, really at birth and declines slowly every day thereafter. Mind you, it's a slow regression and we've had a lot of really great years. (Remember? He wasn't supposed to live past 2 and he will be 12 in March!)  Cerebral palsy slowly destroys his muscles which means his ability to walk (he never walked,) talk (he never talked,) roll, (he used to roll all over,) suck, swallow, breath, poop, everything.  Everything you do is controlled by a muscle of some sort. The respiratory muscles are badly damaged in kids with these sorts of disorders and they usually end up succumbing to pneumonia and respiratory distress. (There's a sick special needs family dark humor joke in here somewhere - "if it's not the seizures that will kill them it will be the breathing - or lack thereof.")  

Willy's lungs started going downhill around 2007 or 2008.  We wound up in a cycle of hospitalizations that lasted the better part of three years. Usually it would start with a cold, a sniffle, a cough, and then next thing you know we're in the PICU on 10 litres of oxygen fighting for his life. It wasn't until we put Willy on pediatric home hospice services in the summer of 2013 that he got better. Irony or what? The hospital is so full of germs that once we broke that hospitalization cycle, he actually got better. We put him on hospice, created a DNR (do not resuscitate) order, and settled in for a very healthy and happy two years.

Part of the process of deciding to put him on hospice services was discussing end of life details. What if he gets an infection? Would we do a round of antibiotics? How aggressive would we be with treatments if he were to get sick? Would we go inpatient? If we did decide to go inpatient to fight something, he'd be kicked off hospice.  Luckily, we haven't been tested.  Until now.  This is the first time since hospice that he has been this sick.  In another life I would have been typing this from the PICU (pediatric intensive care unit) at Mott Hospital.  As it stands, with hospice, we have everything we need at home to take care of him.  We have a round of antibiotics, oxygen, albuterol for breathing treatments, morphine, and everything else that a pneumonia kiddo needs. And without the hospital germs. Win-Win? Perhaps. 

The rub is this. Since this is our first "test" of our hospice plan, it's a big deal.  What if this first round of antibiotics doesn't work and he needs another round or a stronger drug? Would we do it? Or would we say it's his body just saying no thanks. Keep in mind, his disorder is terminal. He is already well past his life span. Although we do know many lissencephaly patients who have lived longer than two years old, it is definitely a terminal disorder with shortened life spans. That fact factors into our decision making process. 

When Todd brought him back to me today (along with about a dozen machines and boxes of supplies,) we had "that" discussion again. He said the hospice doctor was out today and they discussed the options.  At some point, we will know Willy is there.  It's not now. Not yet. But it will be someday. Either he won't recover from an illness or his body will stop accepting nutrition. Keeping in mind quality of life, at some point, when his QOL is no longer positive and good, we will have some very difficult decisions to make. I don't think we're there yet. Willy still has good days. Yes, they aren't as plenty as they used to be but there are some. Todd gently reminded me today that Willy's really only awake - truly awake - about 6 hours a day.  And then it takes a lot to get him to crack a smile or engage. 

The takeaway from this should be positive and if it's negative, you're reading it wrong. Sure it's tough and we're all worried. It's also a lot of work. Round the clock treatments and medicine dosages. But I get to do it all at home.  When we switch off, Todd will get to do it at his home. Surrounded by our stuff. Not in the hospital. With the hospice staff coming to the house to check up on him. It's a wonderful thing. The last ten years have given us enough confidence to deal with it all at home. Ourselves. We could call hospice off, go inpatient, treat the hell out of the pneumonia and be as aggressive as we wanted. Maybe he'd respond to treatments, maybe not.  If we did, and wanted to go back on hospice when we were discharged, maybe we wouldn't be approved. Then we've lost the service. You see the rub here? 

We're going with hospice.  We have what we need here. We will take care of it for him, here, at home. We are four adults who love him and who can help take care of him with our opposite schedules. I can sit here and do homework with Gabby in between tending to Willy.  

Hospice isn't the right choice for everyone and I understand that. There is no right or wrong answer.  But for us, comfort measures and quality of life far outweigh life longevity. Hospice gives us the tools to create that world for Willy. 

For now, fingers crossed that the first round of antibiotics works and the pneumonia goes on its merry way.  If it doesn't, well, we'll decide what to do then.