In May, three years ago, I was diagnosed with ALS. The symptoms manifested more than four year ago and last May, my PEG was installed. It wouldn’t be a stretch to call the month of May my ALS jubilee. Statistically, I’m in the critical stage: the wrong side of the statistic; the borrowed time. According to the books, ALS gives you three to five years to live. Whether the countdown starts after the diagnosis or the first manifestation of symptoms is up in the air. As regards respiration, I’m in critical condition too. By the end of April, my lung capacity was one litre. By now, I have 30% of the lung capacity of the average woman my age. Fortunately I’m not suffering from carbon buildup. Secretly, I hope my lung capacity is a bit higher, because I get nervous during breath tests. What is more concrete is the fact I have to accept that I am truly on borrowed time.
Quality of life
Recently, I read a newspaper article that posed the idea that quality of life is determined by two factors: involvement with and equal concern from other people and control of one’s own life, meaning the power to make decisions about one’s own life. I’m not lacking in either of those. I consider myself very fortunate.
I’ve decided long ago that I don’t want artificial respiration. What I do want is nasal intubation, especially at night. Why no artificial respiration? It would mean that I need help breathing 24/7; for suctioning phlegm and other calamities that might arise with respiratory machinery. As ALS develops, one can do less and less.
I don’t feel like I’m the type for artificial respiration.
I’m too pushy, too impatient to live with that. I like to have and stay in control. Seeing as it’s a factor for my quality of life, I don’t feel like giving that up.
To my family, medical ventilation would be a great burden. I’d need care 24 hours a day. It would be a constant in-and-out of caregivers. Gone is the intimacy us four shared. Gone are our relatively normal lives. In short, the involvement of my family would be under duress.
Medical ventilation would mean I’d have to dive head-first into the medical circuit, a world I prefer to give a wide berth. Every 6 weeks, I’d need a cannula change. Not appealing.
There will be a point where one decides enough is enough and consider ending their life. Actively having to make the choice of dying and pulling the plug is not a pleasant prospect.
Can’t I think of any pros that ventilation has? Of course I can, I want to see my children grow up. But at what cost? I think that they and Hein would be able to live happy lives without me. Looking back, since I’ve been sick our lives have been odd, but happy. I think that’s my greatest achievement. The same goes for Hein, without whom that wouldn’t have been possible.
Sat in the passenger seat of a friend’s car (forcing the missus into the back seat, thanks Gisèle), we left for France, to a wheelchair-friendly farmstead, some 200 km southeast of Paris. The Prins boys were in the passenger seat of our van. The drive was amicable. I slumped a bit, so my view outside was mostly take up by the windscreen wipers. Nonetheless, it was so much more comfortable than the van. The farm was beautiful; huge premises, a fantastic swimming pool and a terrace with lanterns. More importantly: there was water in the swimming pool. The water was 13 degrees, but that didn’t keep the kids from swimming. In the stables, the kids found ample material to build a hut, and there was even a fire pit. The place was ideal. So ideal, in fact, that it won’t be available for booking until 2003. The surrounding area is lovely, but not overly interesting. That meant we didn’t feel obligated to go to every single three-star tourist attraction closeby. We visited a pottery village, a wine-tasting where even the kids had a glass (we were near Sancerre), a goat cheese farm, which smelled tremendously and was inhabited by thousands of flies, several of which we took back to the Netherlands, a museum about witchcraft the kids didn’t want to go to, but they’re glad they did, and finally to a medieval castle constructed using only medieval methods, kind of like Archeon. And of course, we paid a visit to one of those amazing huge French supermarchés, where everybody sneakily added their own favourite foods to the big anonymous shopping cart. Visiting a supermarché is an absolute must in France. The house was easily accessible and I slept relatively well. In short, a lovely holiday, although it could have been better.
Just before we left for France, Hein lifted a bag and something in his back cracked. On our way back home, I kept seeing him getting out of the car, hands on his back. What’s going to happen back home. We have a delicate system in which Hein is the main player. Getting me off the toilet was a hard task on the first day, but has improved since then. Pulling me out of bed only works if I put on my shoes in advance. We’re one and a half weeks on now. Hein is still in pain, but he’s still managing to help me. I do realize that Hein needs some time without having to care for me and I can absolutely understand. The greatest obstacles are putting me to bed and showering. I’ve proposed that I’ll sleep in my chair at night, so that problem is taken care of, and I’ve asked Lieke to watch me for a weekend. This weekend, I already tried spending a night in my chair. Apart from the fact that I couldn’t fall asleep at 5:30 and needed some time on the computer to put me to sleep, everything went fine. All we have to do now is pick a weekend.
It only took a week after returning from France for the weather to take a turn. Nothing but sun and it just keeps getting warmer. Before, I used to try to get a tan as quickly as possible, but now I avoid the sun. The shadows of the trees at the tea parlour are delightful and our house is cool too. A family reunion at the Zuiderzee Museum broke me down; too hot and a car trip that was too long. My arms constantly fall to my sides upside down and get stuck between my wheelchair’s armrests. Apart from how warm I get in that pose, the bottoms of my arms got sunburnt. On top of that, I suffer from hay fever and my nose is stuffed, making me short of breath and making me gasp for air like a fish out of water. With all of that doom and gloom, I’m glad the weather’s cooling off again.
I can’t help writing about our garden. She (are gardens female) is such a beauty. We have some new acquisitions: two holly trees, a red pyrus and as the cherry on top, a little apple tree (a classical grafted fruit tree). For Hein and I, the apple tree was love at first sight. I got it for mother’s day, but Ward classifies it as part of his own growing patch, so he considers is his. Other than that, we bought a mirror ball to ward off witches, in accordance with Dutch folklore, as well as a wooden mobile. Ward’s patch is flourishing. We’ve already harvested and devoured the first head of lettuce, it was approved by the tasters. The whole family cares about the garden and we enjoy it immensely. I’m glad that father-in-law Kees works on the garden a few hours a week.
I still have two crustless sandwiches for lunch. The criteria for my sandwiches are becoming more specific, though: they can’t be too wide, preferably long and thin so I can grind them to bits with my front teeth, the bread can’t be too old and dry, it needs a lot of butter and chocolate sprinkles to make eating as smooth as possible, the bits can’t be too small because I need to be able to grip it, and there can’t be too many sprinkles on them, because I tend to make a mess when there are. In short, I’m an awful nitpicker. My other food is mashed and pureed. I’m always stunned when people eat huge chunks of food made of a plethora of different ingredients like it’s nothing. “I used to be able to do that”, I always think, amazed. Recently, I started noticing that my feeders tend to chew with me. Whenever they feed me a bite, their mouths open too. Now that I notice, it’s become hilarious and distracting. Telling them they’re doing it doesn’t help. It’s an instinct, very hard to shake. Try it yourself.
ALS, or at least the bulbar variant, is often associated with loss of saliva through the mouth or, put more crudely: drooling. Whether it’s because of an increase in spittle production or because of weakening lips, it’s a very irritating and degrading phenomenon. It doesn’t happen when I sit normally, but whenever I have to laugh, sneeze or anything else that puts pressure on my lips, I’m in trouble. Whenever I’m forced to keep my head at an angle (on the toilet, for instance) and I’m expected to say something, I’m in trouble. I primarily attribute it to the loss of functionality in my lips. It’s manageable now, but I hope it doesn’t get worse. Sadly, it’s almost hay fever season again.
An ALS encounter
It’s Ascension Day. Just like the rest of the country, we were seated lakeside in Leiden. After three hours of studying the average person from Leiden, we went back home. We stopped at a snackbar for a bit. While I waited, I was addressed by a strange lady. A lady with a somewhat nasal voice. For a second, I confused her with a deaf-mute colleague I used to have, same nasally tone. But then it clicked. It’s Ineke, a fellow ALS patient with whom I’ve corresponded for the past few months, who lives in Leiden. She recognised me from the photo. Hein joined us and we had a pleasant conversation. She has had ALS for three years and apart from her speech, it’s barely noticeable and she is still intelligible. I’m a tad jealous.