One Swing Ahead of the Sword

And so these past few days have brought us to a place that is mostly strange, somewhat familiar, frightening beyond all reason, and something that I realized that I have to come to grips with and make room for in our life.

It’s one thing to know that Phil has Duchenne, and the precariousness of this chronic disease. We have made room for that. We have accepted that, just like we accept anything that may be a unique or quirky health issue to any kid. Can’t make any exceptions just because it’s a dread disease and not a simple case of allergies.  But what happens when things go acute on you? Now there’s a question no one wants to ask, much less answer. Truth be told, there is no right, correct, one-size-fits-all answer.

We’ve been fighting flu-like symptoms in the house for the better part of two weeks now, cough, sore throat, runny nose, high fevers, stomach ailments – you name it…if it’s an unwanted symptom, chances are it ran through here. I have not been immune to this onslaught, either, and me and sleep have some serious catching up to do…(like that’s gonna happen…).

Giovanni was the first to succumb to this crap, and spent the first week coughing around the house. He tried to cover is mouth and we talked about universal precautions – but let’s face it, when you are sick, you’re sick, and as much as I would have loved to sanitize the shit out of everything in the house just so Philip wouldn’t catch it, this is just not a part of the picture. We live on a planet of germs, with people who carry and nurture same, and it is what it is.

Then last Friday, Mark and I got hit with it. We managed to get out and about Saturday to attend a friend’s get together, and pick up Philip’s long awaited new puppy, (another blog…) but Sunday dawned in a feverish haze. I guess I knew at this point that Phil being affected was inevitable, and I could almost hear the background music of my life turn ominous.

The last time Philip was sick at all was in May of 2009 – and we ended up enjoying a week-long stay at Children’s Hospital with pneumonia, accompanied by IV antibiotics, chest x-rays, cough-assist machines, nebulizer treatments, a vest that shakes the crap loose from your lungs so you can cough it up and suction it out…fun stuff like that. Thinking on that, I had to come to the realization that the acute conditions that can crop up are an ever-present threat, that can at any moment come slamming through the door and rearrange my dance card, and I needed to be prepared for whatever hell any virus or bacteria floating by might want to inflict on us. And I am terrified.

Talk about feeling helpless. Phil awoke Monday morning with a cough that sounded horrid. The diaphragm is a muscle, too – and Philip cannot cough. Oh, he tries. He feels the tickle and the wiggle of the junk in his chest and throat, but all he can manage is a feeble “cuff”, which just serves to make a gurgle in his chest. You find yourself coughing for him, kind of like the way you move your lips around the imaginary spoon when you are feeding an infant, “Ahh, ummmmm!” as you fly the spoon into their opened mouth and pull it out as they close their lips around it. But you can’t eat for the baby, and you can’t cough for Phil.

So, on the distant upside of the May 2009 hospitalization, we were able to acquire a cough assist machine, a nebulizer and a suction unit for home use – which have sat for the most part in the corner of the bedroom, some very expensive durable medical equipment that sooner or later was going to get a chance to prove it’s actual worth. That time was now.

Tuesday, I called the bus company at 6:00 a.m. and told them Phil would not be going to school today. His fever jumped to 103, and in spite of my own funky constitution, I knew it was time to put on my game face and take on this bug for whatever it was going to dish up.

Strategy…I need a strategy. What am I preparing for? What kind of over-the-counter meds should I give him? What ones can I give him? What kind of prescriptions might we need? At what point will I say “Uncle” and take him in to Children’s? Who do I ask? Whose advice shall I seek? What are the parameters of this?

Like too many times before, the answer to these questions lead you only one place – to the mirror. Tag. You’re IT.

I remembered the 2009 trip to the ER. Phil was triaged right away, and the resident heard no crackles in his lungs in response to his “deep breath”, and was prepared to dismiss us, until I asked if he would humor me with a chest x-ray. It made sense to me, since Phil is not capable of taking a deep breath, which would mean no crackles in your stethoscope, now, wouldn’t it? Sure enough, silly old Mom’s instincts were right, and he was promptly admitted.

Up on the floor, I requested a cough assist – an insufflator/exsufflator. This got me puzzled looks from the nurses, and one of them ran right out and brought back an incentive spirometer for him to use. Um…no. I tried to explain, several times, that this is almost pointless – he cannot adequately inhale or exhale to get the little balls to do much of anything. Could someone please call Respiratory? It took three days to finally get the right person on the job, and got Phil the machine he needed.

Now, this is not really a reflection of the staff there. In their defense, 1 in every 3500 boys sounds like a lot, but it still represents only the smallest fraction of patients that are run through the hospital every year, and the needs of a Duchenne boy are unique.

Fast forward to his spinal fusion, and the multiple times where I sought the expertise of the staff there, only to be met with questions about my expertise! You just want to scream, “I am NOT an expert! I know nothing about this!! Somebody…anybody? Does anyone out there know what the hell to do with this?”  But you don’t. You just look around and realize that you have just been elected team captain. Any answers are going to come from you and any input will be from parents who have been here before. Thank goodness for the World Wide Web.

Now here I am, at home, with state-of-the-art medical equipment that up to this point I have felt ill-equipped to even use. How much easier this would be if I could pack him up and take him to the Duchenne Hospital, where they know everything, and will take care of him, and give him all the right treatments, at just the right time, and I can leave him in their capable hands, assured that everything that can be done was being done, and now fate is the only thing we are waiting on.

But that place does not exist. I realized with a sinking feeling that that place is here. At this juncture, I know best. I know what Phil’s baseline breathing and coughing abilities are. I know what his mood and disposition are. I know what he sounds like, and what his pain and discomfort thresholds are. Suddenly, I am the expert. And I do not want to be the expert. And the Universe says, “Too bad, toots.”

Phil is miserable, I am miserable, and we are sitting in a pile of plastic tubes, machines, and face masks, trying to get them all to work to the medical benefit that they were designed for. It felt like we were trying to put a complicated Lego set together without the instructions, and no picture except a healthy Philip. 

And so we make up our own little hospital routine. Take his temperature. Mix up a concoction of Guaifenesin and Tylenol for his g-tube. Hook up his feeding tube with an all-day fluid infusion to flush his little system like a toilet. Cough-assist and suction three times a day at settings that were nothing like those they told us to use. Albuterol nebulizer twice a day. An extra can of Ensure at night. Early to bed, late to rise. His desire to sit at the computer or play video games serving as a barometer of his overall feeling of better health.

As of this writing, my self-styled and engineered medical education has been paying off. One call to the cardiologist to ask about the safety of an OTC medication and subsequent recommendation for a substitute was all the intervention necessary – so far. Phil had physical therapy at home this morning, and no fever, so he wanted to go to school – really bad! And so I am taking this half-day to try and catch up on some much needed home maintenance – things like laundry and dishes, and putting a nap off yet again.

And we are not “out of the woods” yet either. In fact, we never will be. The hard truth about the matter is that we LIVE in the proverbial woods. All of the disasters that can befall a person with Duchenne are alive and well in these woods, and no matter where we roam, they are going to happen to us sooner or later. We were told the prognosis a long time ago – the course of the disease – the inevitable outcome.

Phil and I had a very intense conversation about his breathing, as he started to cry in frustration at being unable to work with the cough assist, with it alternately choking him on the inhale and gagging him on the exhale and wanting to quit. I shut the machine down and sat down on his bed, on the verge of tears myself. He calmed down, and I asked him if he wanted to know the hard truth about what could potentially happen if we could not clear his lungs. He thought a minute, and nodded. And I went on to explain about a tracheotomy, and a ventilator, but stopped short of telling him that this was not just a potential thing – that it will inevitably be a reality. Not something I needed to say, when here on the backside of spinal fusion all he was stressed out about was the fact that it involved more surgery.

I reassured him that we could pre-empt all this for the time being – (undisclosed amount of time, there) – if we could get the cough assist to do the job it was designed for, and that between the two of us we had to figure this thing out, and was he willing to tackle that problem with me…together? He nodded, and I wiped his tears since he cannot do so himself, and we started the motor again, testing the levels, tweaking it here, adjusting it there, until he was able to get the crud out of his lungs somewhat effectively. He then identified the nebulizer treatment as something that afforded him some real relief, which was a real grown-up effort on his part, since it’s hard for him not to just be a whiny kid through this sickness. Of course, he is only 12, and analyzing your symptoms and bio-feedback is not something most 12-year-olds are accustomed to. Then again, he’s on the fast-track, having to grow up in too many ways just to cope with a disease that is robbing him of so many things we take for granted.

Knowing that all the milestones of this disease are out there waiting for us is one thing…experiencing them when they come slicing their way into our reality with swiftness and force of a broadsword is quite another. But for today, we are at least one swing ahead of that sword.