February 1999
Yesterday was a great day. The car was repaired early, taxes were lower than expected, I made a scrapbook with my mother with pictures from recent holidays (a week in Germany and a fun day out in Scheveningen (the kids were amazed at the size of the Kurhaus Grand Hotel)), a good physical therapist and a loving family. My life is going very well. People would call me mad for thinking that, but it’s how I feel. I’m still getting used to it myself.

Saturday, April 3rd
I’ve had a wonderful few days. My birthday was amazingly sunny, so I sat outside eating pastries. My room is a sea of flowers. I visited a forest in Groenendaal with the kids, which went swimmingly. It really was a hot day. The next day I finished my book, sitting outside in the sunshine. On Friday we went shopping with the kids to pick out things for the summer. I loved being there with them. Drinking coffee at the department store, the sun, the smells, the shopping: lovely.

Het bericht Surviving and thriving verscheen eerst op Living with ALS.

Paul, Hein’s brother, installed the head mouse on my desktop computer. It’s much more functional. A much larger screen, a cursor that doesn’t vibrate, a more responsive sensor area for the mouse, not always having to recalibrate my mouse and audible feedback whenever I press a button, which prevents accidental double clicks. Not to mention the programs that I didn’t have on my laptop. In short: I’m content. The advantage of using the head mouse on my own computer is that answering my own mail and using the internet is once again doable. I’m more self-sufficient and I can use my PC again, instead of aimlessly sitting around. I wrote this part in 25 minutes. Not bad. This month’s entire diary is written by using the head mouse. It’s working. I do constantly forget about the dot between my eyes, so whenever I forget to take it off before bed or using the shower, it becomes unusable and ends up in the trash.

My home is my castle

At home, I almost feel normal. Established rituals and habits, trusted caretakers and appliances, surrounded by family eager to talk about their day. A familiar environment where I barely feel out of the ordinary. It couldn’t be any more different when I’m out of the house, especially in larger crowds. I feel abnormal; I’m unintelligible and tend to just sit around in my chair. By now, my group of friends has resolved that issue by doing bridge-drives. They’re fun gatherings during which I don’t just sit around like dead wood, because I hold my own when it comes to playing bridge. We do it once every couple of weeks. I love it.
Besides playing bridge, we celebrated my mother-in-law’s birthday this weekend. Not looking forward to it would be an understatement. Being seen like this by a bunch of people I barely know is awful, I feel especially vulnerable when we enter. Please, let everyone act normal, I pray to myself. Let me just watch from the distance for a bit to prepare myself. No such luck. I was surrounded by people and immediately, every single person there feels the urge to whisper some soothing words to me. I hate it. People, please don’t act like that. Leave your unctuous, intimate consolatory conversation at the door, please. I really can’t take it. Once I was properly seated, things went better. Quietly, I could take part in the festivities too.

A message from the past

I got an email from one of my earliest friends. We were friends since we were three, a friendship that lasted till we were twelve. Since then, we hadn’t spoken for thirty years. Suddenly, she contacted me with the sweetest mail, reminiscing about our time together. She described it so vibrantly, it made me laugh. It made my day. Oh, how I do love email and the internet.

Writer’s school

I started doing a writing course, by email. Every week we receive an assignment. I have to think of characters and describe them. Every assignment has a deadline. It takes some getting used to. Before, I was always free to plan out my own day, but now I’m “forced” to do something by an external force. Some of my fellow course participants also suffer from speech impairments. Eventually, we’ll have to write about communication among our own characters. We even have a real teacher. I’m curious to see what’s next.

Newspaper interview

I’ve busied myself with answering emails from people with questions about my book following an article about it in a newspaper. I had gotten in contact through one of my neighbours, who is an editor for NRC, a newspaper in the Hague. I’ve had my doubts about publicity like that. Is my book too private for strangers’ eyes? Will I be able to handle the public response? Will my kids? Then again, there are 2000 copies of my book and the thought of them disappearing in a shredder is not very appealing. I’ve sold 150 of them so far. In short: I could use some publicity. Thus, I’ve started working on getting as many of them out there, which is a chore in itself. My sister-in-law Monieke is in charge of sending copies to medical libraries and clinics. Any other suggestions for distribution would be most welcome. A photographer came by yesterday. I had a picture taken of me on my own and with the kids. Most of the pictures were of the whole family, but Hein is still on the fence about appearing in the papers. The article will be printed in March. I have full insight in the article and I’m allowed to pull out at any time, in case the article is too personal. I want a positive article about life with ALS, without dramatic captions and such.

Nighttime worries

I’m not getting proper night’s rest. I fall asleep like a log, but after three hours or so the tossing and turning and the leg-stretching start. My right leg becomes rigid and finding a comfortable position for it becomes difficult. That’s when I wake up, often without being able to sleep again that night. Sometimes I panic, legs sweating. Sleeping in has become a distant memory. After 7 or 8 hours, I can’t stand being in bed any longer, even when I’m barely rested. Usually I sleep for an hour later in the day. It must be so frustrating for Hein. He has to work in the morning and can’t really afford all of my nocturnal escapades. During weekend mornings I’m downstairs while Hein gets some well-deserved rest.

Floor and the tube

Floor feeds water through my tube twice a day. I think she found it an interesting process. One day, she asked me if she could try operating the PEG. She could, of course. Since then, she has busied herself with watering me. It’s a big responsibility for a twelve year old, maybe too big. Either way, she does it carefully and with gusto.

Oleta Adams

My sister-in-law Monieke gifted me a CD by Oleta Adams, signed by the singer and addressed to me. I had never heard of her and it’s not really my style, but I really appreciate the gesture. My new help on Mondays and Tuesdays, Marja, prefers it over U2 and Anouk. Right now I’m very taken with the Buena Vista Club’s latest album and its beautiful female vocals.

Ameland

Going on holiday is anything but stress-free. Before leaving I worry about going to bed, being able to sleep, getting up and cleaning up, hoping the day of departure itself will be fine. It wasn’t. When we arrived in Ameland I felt sick. Was it the journey? My worries about the journey? Had I actually caught something? I wasn’t sure, but decided upon a preventative antibiotics treatment. My general practitioner prescribed me an antibiotics course precisely for travelling. I appreciate that, because I like having assurances. The hotel we stayed at was perfectly suitable for me. The family was most impressed. There was an adjustable bathtub and a very versatile stair lift. Despite the glowing recommendations from my family, I didn’t use the bath. It’s too much. Too… invalid. It doesn’t fit my own self-image, still falling hopelessly behind reality. Going to bed wasn’t too bad. I slept better than I expected, no mishaps for the whole duration of the trip. I only woke Hein up when it was time to wake up. We didn’t have to worry about cooking. Every evening we got three courses, easily mixed with sauces and gravy. I had no problems eating.
What we actually did on holiday? We went on two wonderful hiking trips. Despite the fact that we visited an especially flat part of the country because of my heavy wheelchair, it was still a sweaty ordeal to get me over all of the sand dunes. We went to see the lighthouse by night to see the light sweeping the island. My family made the 58 metre climb to the top of the tower. We went seal spotting on a boat and saw more at least a hundred of them, loafing around on shoals, some of them up close. In the museum we read about how, until recently, rescue boats were pulled into the sea by horses. One time, eight horses drowned trying to get the boat waterborne. We learned a lot about Ameland through museums and chatting with locals. The weather is wintery with both snow and sun. Towards the end of the week, an unpleasant wind picked up. At 4 o’clock on Thursday afternoon I was back home. I can’t complain. I’m back behind my computer. I’m finally able to entertain myself again without having to ask someone to turn a page every minute.

Het bericht Diary February 2001 verscheen eerst op Living with ALS.

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.

CPR

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.

Intimacy

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 circuit

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.

Euthanasia

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.

Thou, France

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.

Hein

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.

Hot

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.

Garden

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.

Food

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.

Saliva

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.

Het bericht Diary May 2001 verscheen eerst op Living with ALS.

I hadn’t taken a tumble in years, but this month I fell to the ground two times when trying to use the bathroom. It’s due to a combination of being tired and badly positioned legs, because of which I sank to my knees. There’s no way to stop it once it’s started. Hein had to pick me up and I could do nothing to make it easier, I was dead weight. The second time I fell, I cracked my head on the floor. I saw spots for a second, but everything seemed fine. Only by the next day did we see what the damage was. While I was drinking, I felt like my jaw had been unhinged, the way my teeth gnashed together. And while I was eating my porridge, my remaining front tooth came loose. I now have three enamel stumps where my front teeth used to be

Hospital

Luckily, we had an appointment the next day as a last-ditch attempt to fix my teeth. I’m split. On the one hand, I hate the way my teeth look, especially when I’m around strangers. On the other hand, I’m dreading another operation and am doubtful that there will ever be a prosthetic strong enough to stay attached when I’m getting my teeth brushed. I’d written another letter for clarification, containing a cost-benefit analysis. “Oh, don’t worry about cost,” a jaw specialist told us. “Your insurance will cover it.” Obviously someone who only thinks in sums of money. “It’s not about the money,” we replied, after which she wrote down the term ‘quality of life’. We met with a surgeon. Seeing as I almost always have my mouth closed, esthetics alone were not enough to warrant the operation. I could have cried. The female doctor suggested I get back in touch with my dentist to see if he couldn’t do anything about it. The week after, he got back to us: negative. I’ll have to live with three broken front teeth.

Out of the house

I’m less and less tempted to get out. Maybe it’s because it’s winter. It’s undoubtedly a factor. Always having to wear a coat or needing to be hauled into the van is a lot of work. And I’m getting sick of going on walks through the same park, especially since I can no longer drink coffee. After all, the tea parlour was always the focal point of our walks. It’s still too cold to sit in the garden, which in itself isn’t exactly ‘leaving the house’ either. Maybe it’s because I’m too nervous in public or social settings. Do I give up too quickly? What can I even do in the outside world, unable to speak, my teeth only making it worse. I’m almost normal at home. But I know that sitting around at home isn’t making me any happier. Because of that, I’m glad I attended parent-teacher conference and went to that birthday party.

Drinking

I can still drink, but I can’t do more than a few gulps. Not only can I not keep my head upright because of my neck, but I can’t create suction as well as I could before. I drink for a bit, but if it isn’t going well, I’ll give up. The rest of it’ll just go in my feeding tube. I use it more and more, even in public. Bystanders don’t know what to do. They look away, but are also intrigued by this less than common phenomenon. Only a few people are brave enough to ask me about it. “How does that feel?” I only feel it if the food is extraordinarily cold or hot, otherwise it barely registers. I don’t mind using my PEG in public, provided that it’s done decently. I hope it doesn’t bother all of you, either.

Photo

Lia, Hein’s mother, turned 80. There were so many photographers present. This picture was taken by Adriaan. Of course, Floor is the looker in this one. Her kind smile, youthful skin and beautiful eyebrows are a huge contrast to mine. But the biggest difference is how carefree she looks, whereas I look almost frightened. I specifically asked for a picture of the two of us, but apparently when it came to it, I was too apprehensive about the result. And, the most important thing for me was keeping my mouth shut.

Organising

It’s only now that I realise how much the construction of our sun room impacted me. I could write about nothing else, got writer’s block and lost the drive to organise other things. By now, I’m over it and back to organising. This week I got three confirmations of things I’d planned. One of them was for our holiday in Scotland. They have some room for us after all!
Afterwards, we’ll be going on holiday to South Limburg. To our surprise, there were still vacancies. We’ll be going with family instead of friends this time.
The third confirmation pertained to my birthday party on April 2nd in the botanical garden, a beautiful location. Here’s a link if you feel like visiting it too.
I’ve had a great week.

Grocery shopping

I haven’t been inside a supermarket for over 6 months now. The weather was so bad on Saturday that, instead going on my normal walk through the park with my sister-in-law Netty, we went to the Albert Heijn. I vowed to get all of the things Hein tends to forget: tiramisu, croissants, a quiche, stroopwafels, etc. I longed for all of the things on the shelves that I can no longer eat. It’s a good thing I don’t go grocery shopping anymore. Back home, I always make shopping lists and usually decide what’s for dinner. But I usually focus on functional groceries only. We only get sweets and treats when the kids remember put them on the list.

Euromast

I’d been planning a visit to the Euromast for some time [The Euromast is the highest building in the Netherlands. -Ed.]. The last time we planned on visiting it, the weather was too good to spend the day inside, so we went to the forest in Amsterdam instead, but this time the weather is so bad that the Euromast seems ideal. The top floor was roped off because of high wind and the barriers obstructed my view, but the restaurant with floor-to-ceiling windows had a great panoramic view. We’d love to go back there when it’s dark outside.

Head cold

I hoped to avoid getting sick this winter. Winter was almost over and nobody had gotten sick yet. But then, one by one, my carer, Floor and my other carer got a cold. And now I have it too. The evening news called it an epidemic. I’ve had a sore throat and a runny nose for the past few days and I’ve barely written anything because of it. I’ve started taking doxycycline again. We always have some in the house to prevent pneumonia. Luckily, one of my carers was able to fill in for the other as she recovered at home. I can’t wait for spring to start.

ALS patient union

ALS patients don’t write history. I’m convinced that, if ALS patients had longer lifespans, the disease would get more recognition. Patients are the driving force behind initiatives that spread awareness. Vincent Straatman was the impetus behind the Valscherm Foundation. That lead to the establishment of the ALS Centre. But after that, interest in ALS waned and the information was neatly divided between the UMCU and the AMC. I haven’t heard from the ALS Centre in a while, besides a Christmas card. Vincent was looking for a way to get ALS into the public consciousness with him as the figurehead, and now Ardi Bouter is trying to do the same, without knowing about his predecessor. How would he? We don’t leave much of an impact. And what about the VSN? ALS is the most represented illness within the VSN. That may just be because MS has its own union, with Maartje van Weegen as its figurehead. But the VSN doesn’t actually have any patient involvement and is more medical than social. I’m afraid it’ll remain that way. ALS doesn’t, and will never have, widespread public awareness.*

Portraits

There’s an empty white wall in our sunroom. What better way to dress it up than a painting or two? We’ve wanted to get portraits of the kids for years. After a series of events, we found ourselves in an art studio in Haarlem one Saturday. Myriad portraits were shown to us; realistic, impressionist, with or without a backdrop, old and young people. By now, I can no longer see the wood for the trees. I don’t care too much about the style, as long as the portraits look good.

Holiday

Ward is so glad he doesn’t have school. “I don’t have to do anything right now,” he’ll often say as he’s lazing around. He celebrated the first day of the holidays by keeping his pajamas on all day. It’s snowing outside. I’ll be working on my scrapbook with Lieke later.

*if you’re actually interested, check out Stichting ALS

Het bericht Diary February 2005 verscheen eerst op Living with ALS.