Last week was relaxing. Bad weather, so most of it was spent inside. My mother is sick and because of her absence on Monday and Tuesday, we had to improvise with our carer schedule. It’s a delicate system and we really ought to have hired a new carer by now. My mother feels like she’s doing me a disservice by falling ill, so she was glad to hear that we had found a replacement.
After three weeks my mother got better. My primary carer Inge and my mother-in-law took her shifts. Hein has found a new carer for Tuesday morning who is also open to filling other gaps in the schedule.

Female discomfort

I asked my neurologist about the possibility of a pill that stops the menstrual cycle but he advised me not to use them. Hormonal shifts may negatively influence the development of ALS. There are women who deteriorate even faster after taking pills. I wouldn’t fancy that. I guess I’ll just struggle through this useless discomfort.

Flu shot

ALS patients are advised to get flu shots to reduce their chance of contracting pneumonia. It goes against every single alternative healing method. I decided to get one anyway. To my delight, a fellow ALS patient told me that his homeopath had actually recommended getting a flu shot. I wouldn’t be able to forgive myself if I died of the flu after refusing to get the vaccine. It was the first time I’d gotten a vaccine since I got diagnosed, but it didn’t have any side effects, luckily.

Head mouse

Typing using my mouse is becoming more difficult by the day. I will have to switch to typing using my head. It’s another big step and it saddens me to lose another trusted faculty. Handling a mouse is becoming impossible because of my tilting hand, my drooping head and the neck ache my posture gives me. I think typing with my head will be more relaxing. I’ll get to sit in a more comfortable chair, for one. I practised for the first time yesterday. It looks like a bindi, but I’m actually equipped with a silver dot between my head, which reflects infrared light to the screen, moving my cursor. It created a lot of curiosity from others. It does look peculiar. The strange thing is, I completely forget about the silver dot between my eyes. I guess others have to get used to it more than I do.
Help! Help! The cursor is flying across the screen. Even when it’s stationary, it trembly. I accidentally click all over the place. Programs start up and shut down, scrolling is impossible and I can’t type a single word. Luckily my hand still works, so when things go awry I can fall back on the old method. It’s all so fiddly my head hurts.
I would like some more explanation on head typing and some alternatives couldn’t hurt either. It’s urgent. I communicate by typing, after all. I’m getting a bit panicky. For now, I’ll just keep using my hand.

Kids Ward and Floor’s age

I’ve always been a bit envious of ALS patients with older kids. “At least they got to see their children grow up”, I’ll think, forgetting that Ward and Floor are my greatest sources of happiness. Afternoons are my favourite time of day. I eagerly await their return home. Like an old-fashioned stay-at-home mom I’ll wait for them (often joined by dad and grandma) with a cup of tea at the ready. Listening to their stories, singing songs, doing homework, going outside, sports, quizzing, having friends over, I’m involved in all of it. I’m in good hands. No reason for envy.
I recently heard Floor say that being a good mother is staying at home for the kids. Before, I would have given her a stern emancipatory talking-to, but not this time. Both because of my lacking communication ability (and maybe because I enjoy being at home with the kids).

One and a half-year-olds

Some friends of ours and Hein’s brother have one and a half year old sons. We went on lots of trips with those friends. Little Thomas and I are both carted around. It gives us a certain bond, even though our developments are essentially opposite. While the boy is becoming more and more self-sufficient, I’m becoming more dependent on others. The little tyke knows that, too. Often he looks at me in wonder. “Why do you still have to be fed?” Even one-year-olds are aware of what is normal and what is not.

Nodding off

My head is getting heavier and heavier and sometimes just rolls to the side. A kiss that is too firm can do the trick. It usually happens when I’m in a chair with a straight headrest. If I can’t keep my head straight, it can droop forward. Ever since Hein adjusted my chair headrest to a backwards position, there has been less stress on my neck and my head tends to stay upright. We’ve been looking for a neck brace for some time. Most of the ones I’ve tried were too tall at the front, making my head tilt back a bit. That’s unfortunate, because I always keep my head slightly skewed forward to prevent aspiration. The braces were just stifling to me. Yesterday we visited a company that makes tailored neck braces. I wasn’t looking forward to having my neck moulded in plaster, but luckily one of the ones they had in storage was a perfect fit for my neck. Content with our purchase, we went home.

6 months of PEG

I got my PEG on May 18th. It’s six months later and I’m glad that using it hasn’t become a necessity yet. I only use it for water. That’s because having to bow forward to drink from a straw is strenuous for my neck. I ask people to hold my cup up to my face when I want to drink. It’s not a fun task for my carers. Hein doesn’t like it much, but Ward and Floor hate it the most. The PEG is a good way to circumvent that.

Sitting in November

I’ve been sitting a lot more lately. I only use the PC in the morning, after which my hand and head are tired out. Afternoons are spent in my comfy chair. I usually listen to music. I’m a huge fan of U2’s latest album. My range is getting smaller and smaller. I don’t think there has been a month in my post-diagnosis life in which I’ve been outside less. The weather has been foul. I’m seeing fewer people in daily life, too. I realize that it’s winter, of course. Fewer people about. Because of my limited daily energy reserves, writing Sinterklaas poems and keeping up with my diary have become my number one priorities. I have completely neglected my email inbox. Oh well, I’ll have time for that some other day.

Het bericht Diary November 2000 verscheen eerst op Living with ALS.

It all began with the NRC interview and the subsequent 50 (70 by now) emails about it, plus an additional few written replies. Many old acquaintances responded, some of them from unexpected people. I love that. Complete strangers send me words of encouragement, poems, reviews, etc. Responding to all of my mail is a lot of work. I decided to publish my March diary early, because my brother-in-law (the webmaster) is expecting a child and he probably doesn’t have time. On Tuesday, my regular help Marja was absent and Hein was home, as well as my mother and father-in-law Kees. My day was messy. To pile more on, my GP diagnosed an ingrown toenail with a lot of fibroma, which had to be removed. On Thursday we left for the hospital, where I was immediately “operated” on, which was a relief. It took 10 minutes at most and the most painful part was the anaesthesia. I won’t be able to wear a shoe for a while, making standing up difficult and using the bathroom quite the adventure. Hein stayed home for two days. He is the only one who, no matter the circumstances, knows how to support me when I stand and, more importantly, catch me when I fall. On Saturday, the bandages came off and I could finally wear my shoe again. The pain is minimal, but I’d much rather it didn’t hurt at all. From 5 o’clock until nighttime on Friday, we were fixed to our television screen, not wanting to miss anything about our Alex and Maxima (the wedding of prince Willem-Alexander and princess Maxima). On Saturday the 31st of March I celebrated my birthday, and my niece Hanna was born. That evening we had a bridge drive with 12 friends. We had a good time. Floor and her friend Maartje waitressed like professionals: “one Sisi and a red wine for table one”. We have three tables, but only played eight games to leave plenty of time for chitchat. The awards ceremony was hilarious as ever, we allowed the winners to claim old Easter junk as their prizes. On Sunday afternoon, the family and a couple of friends came over for drinks. There were fewer people than I had hoped, because I had to postpone the party at the last minute and some people couldn’t reschedule. Despite the thinner company, we still had fun. All I want now is a normal week with good weather, so I can enjoy all of my new garden stuff.

Mysterious email addresses

I receive a lot of mail from men. At least, it seems that way. After opening, all of these Jeroens, Pauls, Harrys, etc. are actually women, with different names. While I do faithfully correspond with a few men, most people who write me are female. Sometimes, a “male” email address is confusing, because the female writers don’t introduce themselves at the start of the mail, making me doubtful of the author’s gender until the end. Ladies, please get your own email addresses. It’s so easy.

Drinking

By now, all of my drinks are help up for me; if possible, the cups are resting on my stomach at about the same height as my PEG. No problems so far. That is, whenever people actually hold it at a consistent height. All arms tend to fall back down, be it from exhaustion, distraction or gravity. Because of that, my straw no longer rests on the bottom of the cup, causing it to slip out of my mouth. I don’t have any power in my lips. Repeatedly, I gesture “up, up”, which isn’t always understood by my helpers. The biggest obstacle is toward the end of drinking. The cup has to be held askew to get the last drops, but my straw also has to be in the drink too. It’s a difficult game of balance for the helper, to varying degrees of success. To close it off, I burp loudly, like a baby after it had its bottle. Everyone just laughs about these “not done” burps, and so do I. Apparently, I swallow too much air when I drink nowadays.

School visit

Floor had a show at her school this week, which is not wheelchair-accessible. It posed a problem, now that I was in a heavier wheelchair. Hein worried about ways to get me into the school long beforehand, while I thought “it’ll be fine”. It’s the usual division of roles. Just to be sure, we brought my light wheelchair, but I hadn’t used it for 6 months. We arrived at the school well in advance. In my heavy wheelchair, I was carried into the school by four men. Thus, I was able to fulfill my motherly duties and able to hear Floor sing as a soloist, while royally seated at the front. Anyway, the school was very helpful. Glad to know that this can work, because Floor has another show in June. The third one in a short time. Maybe a bit overkill, but she enjoys it nonetheless.

Increase

The World Access server was down for a while, so a the end of the month I didn’t have any data about who was visiting my homepage. By now, I do. In 2001, the amount of monthly hits has doubled, from 1500 to 3000. My book and the interview helped, of course. The book is still selling well. By now, I’ve sold 250 copies. I’ve started selling them myself, mostly through others, because I’m more tempted to hand them out for free. My absolute top salesperson is my mother-in-law with almost 20 books sold, followed by my friend Tineke with 10 copies. My mother rather acted as a library, lending her copy out.

Essay

Ward has to write an essay. He’s chosen the subject ‘Tornados’. I don’t know why, but I had the feeling that Ward didn’t need any help writing it. Purposefully, he collected his stuff and went to work one and a half months beforehand. A polar opposite of Floor, who always starts way too late and whom I’ve always helped out. Because Ward wasn’t making much headway, I offered my help. To my surprise, he immediately accepted. Ward had started by writing the last chapter, which made a lot of what he’d written more relevant in other chapters. After an initial fight about the chapter arrangement, we’ve been pleasantly working on chapters every afternoon. Ward is obviously pleased and we’re writing chronologically now. We’re both satisfied with the results.

Classical Sundays

It’s been a while since our last Sunday trip. The weather is not being cooperative. Thus, we stayed at home, to the delight of the kids, who were tired of hiking and museums. On the past few Sundays, the TV was on all day, on the sports channel. Springtime cycling: the Tour of Flanders and Paris-Roubaix; the Hell of the North. It reminded me of back in the day, not going outside on Sunday, reading the newspaper in front of the TV and spending the day in a stupor. Or even before that; listening to the sports programmes on the radio. I’m getting to like this “doing nothing”, while the rain softly taps the window. I worked on a few History chapters with Floor while Ward finished his essay. Hein constructed a cabinet and put a painting up. Of course, we hunted for easter eggs too.

Anouk

Floor gave me the latest Anouk CD for my birthday. I enjoy it immensely. It’s fairly aggressive music, interspersed by more soft-spoken songs. Before, I would have sang along with the music and dance. Not anymore. I listen to the music like a sack of potatoes, but in my mind, I am free and I dance and scream yell my lungs out. Included in the album was a DVD containing all of her music videos. I can play it on my computer. Hell of a girl, even she could do without those golden teeth.

Driving a car

I don’t enjoy car rides anymore. I’m sat in the back of the car in my wheelchair and bob along to every bump in the road, of which there are a lot. My head flails to and fro, like it’s not even attached to my body. There are even bumps that launch me 10 cm into the air. Those are etched into my mind, so the next time we pass it, I can give ample warnings beforehand. They are not heeded, by the way. After passing the dreaded bump, Hein parks the car and asks me what’s the matter. Tonight, we’re going to see if I can properly sit in a normal car. I can’t in our van, because the backrest of the passenger seat is too straight. It would be ideal for our trip to France. That is, if my head is up to the task. The test drive was successful. My head was fine and the suspension was good. I’ll be going to France, seated in the passenger’s seat of a normal car.

Withdrawal

In the first weeks after the interview I had truckloads of mail to get through. I had to merely switch on the PC and correspondence flooded in. I loved it. This week marks the end of that. There are days on which I don’t receive any mail at all. I didn’t realize that publicity and the attention that comes with it could become so addicting. Luckily, I got some more mail later.

Email craze

Floor recently got her own email address at Hotmail. Immediately after school, she bolts for the computer to see if she got any mail. What makes it more fun is seeing that some of her friends are also online. Many private chats ensue. The fact that she’s just seen them at school, or that they all practically live across the street doesn’t bother her. They just chat, for hours. During all of that, the phone is unavailable. It causes some friction between mother and daughter. It does, however, have a sunny side: we send each other messages. In the emails, we talk about our gripes and make agreements on the amount of hours spent on online chatting. Corresponding with your daughter like that is fun. Floor made one condition very clear: there will be no critisism of spelling and “grammer”. Of course, I couldn’t let that one slide. Moreover, I like to write about things that aren’t said often enough in passing. Especially in my situation. I’ve started mailing Hein more too. Ward is becoming a tad envious and wants an email address too. I’ll allow it. I’m already looking forward to corresponding with my son.

Het bericht Diary April 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.

Mary Pickford

I found the abovementioned adage while making the new newsletter for the Lawrence LeShan Foundation, a spiritual organisation offering guidance to people with life-threatening diseases. It’s the third newsletter with me as the lead editor. I was very moved by this motto.

Watching the dog

Lieke is watching Buuf’s dog. Buuf [a shorthand for “neighbour” -Ed.] is one of her former, you guessed it, neighbours. Thus, when she cares of me every Thursday, she brings her dog along. She can watch us both. Nervously, the dog entered the room, jumped on me and licked my hands. Horrible. Defenseless, I accepted my fate. Now I remember why I don’t want a dog. This one constantly wants to sit on your lap. Lovely. Whenever Lieke feeds me sandwiches, she squeezed herself onto Lieke’s lap. How cozy, the three of us, huddled together eating a sammich. The dog and I, in a constant battle for our sitter’s attention. Ward came home and the dog bombarded him with love and affection. But of course, walking the dog is wonderful. Along with the neighbourhood kids, a proud Ward gallivanted around with the dog on a leash. She’s fairly obedient. And I, too, warmed up to the dog by the end of the day. Of course, the inevitable question was asked: “Can we get a dog?” Luckily, the idea was quickly nipped in the bud.

The aesthetics of a PEG

Applying a PEG (feeding tube) is no cakewalk. Many ALS patients dread it; because of practical, emotional and aesthetic reasons. Well, I’ve given up a lot of aesthetics already; no more make-up, wide elastic trousers, sweater vests instead of the smart jackets I used to wear to work, no shaving my legs or armpits. The only thing I did was dye my grey hair again. I’m a practical person. That’s easier for my loved ones. Aesthetically, getting a PEG wasn’t a huge hurdle. A dear correspondent of mine doesn’t find a PEG appealing at all. However, in the meantime she is losing a lot of weight, to the point that she’s become tired and lifeless. She’s wearing herself out. She has been admitted to hospital. First, they’re going to try to fatten her up with pureed food and such. If that doesn’t work, she will need a PEG. She’s expecting to remain hospitalized for 5 weeks. We visited her on Wednesday. Lots of sleep, eating for three and being spoiled a bit is her therapy.

After two weeks of hospital, she hasn’t gained an ounce, so she decided to get a PEG, which she pronounces PECH. Because she wasn’t looking forward to having an endoscope in her throat, the PE(CH)G will be installed with a radiographer. It’s an external operation with X-ray and only requires a small tube in the throat in order to make the knot. No gastroenterologist needed. Apparently, this method is frequently used in England on bulbar ALS sufferers, because their bulbar region is so sensitive.

Slightly nervous

Thursday was the first day with our new help, Astrid. I was a bit nervous all morning. Not because of eating or drinking. No, as long as I can sit on a chair, I’ll be fine. My nervousness is for the moment I have to use the bathroom. All morning, I debated “should I go now or should I wait?” Eventually, I decided to make the leap; we went to the toilet. It went well and I spent the rest of the morning feeling relieved. I’m like that with all of my carers who sporadically help me use the bathroom. Would they still be able to adhere to my old routine?

Nose

With ALS, the subject of respiration is unavoidable. Many neurologists are against artificial respiration for ALS patients; the disease is too progressive. Even nasal intubation is advised against (like a correspondent of mine heard from her neurologist), because if ALS is above the neck (bulbar), it won’t be possible to do it nasally, instead needing a tracheostomy (a hole and tube through the throat). It would be possible if the ALS is non-bulbar, but if it’s both, intubation is impossible. One’s facial muscles would be too rigid, or one’s mouth would gape too much to properly install a nasal tube.

I, a bulbar patient, have been going to the Centre for Home Respiration in Utrecht for three years and I have never heard about the impossibility of nasal intubation. Fortunately, a dear correspondent wrote to an American expert on the subject. What a nice fellow, writing back this quickly and elaborately. Last but not least; the answer is reassuring. There’s so much medical incompetence in the Netherlands, or at least there’s too many prejudices and aversions in the medical world.

His answer was as follows

Regarding your question: “…do bulbar patients skip the mask ‘trial’?”`A one word answer is: “No !” . To breathe or not to breathe…. That is the question! It is certainly not true that “a mask is never an option for a bulbar patient.” It is true, however, that tracheostomy needs to be considered as ALS progresses, particularly as bulbar impairment becomes more severe (that is, if long-term survival is desired).

The longer answer is that bulbar impairment with ALS is a continuum from mild to severe. Nasal mask Ventilation (noninvasive) is often successful for people with “mild”, or “mild to moderate” bulbar impairment. The pile up of secretions can become a problem with nasal ventilation, if secretions are pushed down into the back of the throat and airways, thus blocking air exchange and causing gagging or choking. However if one has an effective cough (using the assistive cough techniques) and secretions are not a big problem all the time, then nasal ventilation may work. It is very reasonable (unless bulbar impairment is severe) to arrange a trial of nasal ventilation. If it doesn’t work then go on to the next option (tracheostomy or palliative care).

Edward Anthony Oppenheimer, MD, FCCP
Pulmonary Medicine
Los Angeles, California – USA
Email: Eaopp@UCLA.edu

Trips

I’m glad we went on so many trips and enjoyed the nice October weather. Since we’ve had kids, we’ve participated in the morning and afternoon festivities on October 3rd (The anniversary of the end of the Siege of Leiden). After the parade, the festival became a drunken mess and the atmosphere turned hostile. Thus, we made our way back through Breestraat, watched the water show and watched another parade (like we always do). We’ve been doing it for years, but it looks like it’s coming to an end. Floor couldn’t come this year. She went out with her friends, returning at midnight. After the fireworks, we went to pick her up. Ward did come with us, but he had other plans with his friends at three, so we left early. We ate hotchpotch and that was it, the 3rd of October had passed.

The next night, we were supposed to go see the film Nynke, but seeing as it played on a screen in an upstairs area, we watched La Pianiste instead, not my preferred choice. It’s a hards ruthless story. It did make an impression in me, unlike Bridget Jones’s Diary, so at least it was intriguing. We had drinks afterwards, which was nice. Despite the fact that it was already October, we sat outside on a heated terrace. While I barely drink anymore (I used to drink a lot), I enjoyed a delicious Grand Marnier.

On Sunday, we visited Dordrecht with some friends. Every first Sunday of the month, a whole route of charity shops and art galleries open their doors. Gorgeous city, Dordrecht, it was a pleasant afternoon. Prince Willem Alexander and Maxima walked the exact same route the day after. The kids, however, are less and less likely to tag along. Floor’s excuse was that she had to do homework and Ward’s neighbourhood football tournament was finally happening. There weren’t as many people as we had hoped, but it was great fun nonetheless. I love the fact that they organise things like that.

Op school stonden ze op het bord geschreven,
het werkwoord hebben en het werkwoord zijn;
hiermee was tijd, was eeuwigheid gegeven,
de ene werkelijkheid, de andere schijn.

Hebben is niets. Is oorlog. Is niet leven.
Is van de wereld en haar goden zijn.
Zijn is, boven die dingen uitgeheven,
vervuld worden van goddelijke pijn.

Hebben is hard. Is lichaam. Is twee borsten.
Is naar de aarde hongeren en dorsten.
Is enkel zinnen, enkel botte plicht.

Zijn is de ziel, is luisteren, is wijken,
is kind worden en naar de sterren kijken,
en daarheen langzaam worden opgelicht.

Ed Hoornik

With this poem, Gerard said farewell to me. The last three lines were especially important to him. Gerard was my correspondent and friend for more than 6 months. He was like my father; he started every email with “Hello, girl”, and he encouraged me to keep publishing my diary. He was an enthusiastic reader. I got an email with this poem one day before he passed. I’m thankful that he thought of me, even in his last moments.

Toenail

Yesterday, my toenail was removed for the second time. My toe refused to heal and kept getting infected and growing fibromas. After half a year of messing with Biotex, silver nitrate and “open-air therapy”, my GP had had enough and referred me to a surgeon once more. Apparently, there is too much pressure on my toe. Yesterday was the day. The injection is the worst part, along with the fact that the bandages can’t be removed for two days. After moving me though, the bandage already unraveled after half a day. Thus, we put a normal bandaid on it and I’m already wearing shoes again because it takes away some of the pain. Let’s hope it heals this time.

Off day

Sunday was one of those off days. It happens every couple of months. I wake up with a headache. Immediately, the anxiety comes in: “I hope it’s not from nocturnal chest tightness that’s common in ALS patients”. I’m cold and I feel nauseous. We cancel on our afternoon guests. I don’t eat anything all day. To make things worse, I got a terrible email. I keep crying about it, which isn’t making my head ache any less. At night, we received the news that Gerard had died. What a day. Luckily, I slept well and woke up the next morning fresh and without a headache.

Passion

On Saturday evening we went to a Hester Macander show, a comedian. The theme of the show was focusing on looking for passion, instead of on the things we do to keep ourselves busy. It resonated with me. I’ve wondered before what I would like to be if reincarnation were real. My conclusion is that I would like to be passionate about one all-overshadowing thing. I would love to make music. I think music is one of our most primal emotions, a language that transcends culture. Making music together is energising. Another possibility is becoming a photographer, a very ambitious one of course, one does have to be unique. Or I’d start a shelter for homeless children in a third-world country. Everyone probably has dreams like that. That deep down, there is a great person within you. The reality is, you’re already doing the best you can, which is a monumental task in itself.

October

October was a good month. Pleasant weather that made going on trips possible, even during autumn break. The new help is doing fine and that’s calming. The renovation of the bathroom and shower is taking forever, but we’re not really working on it that hard. Getting into the one square metre shower stall is still manageable. And I’ve warmed up to the commode. I do miss having a project, like my book last year, or the musical the year before. I’m looking for something new, but I don’t know what. For now, I have plenty of work to do before Sinterklaas.

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

Miscommunication

Multiple people have told me that the piece about the PEG operation I put out last month was about me. It was not. It was written by a friend who was rapidly losing weight and dreading an esophageal PEG operation. I thought it would be informative for ALS patients, because the method described is a unique one. I’m sorry if it caused any confusion. I’ve had a PEG for a year and a half now, installed regularly with a tube through my throat.

Relaxed weekend

I didn’t do anything last weekend. The kids didn’t have plans, either. I can tell they enjoy doing nothing every once in a while. We wanted to go see Harry Potter on Sunday, but every row but the second was taken, which wouldn’t be easy on my neck. Surprisingly, the kids did want to go on a walk with me to the park. Lovely, a stroll in the winter cold, having some tea in the tea parlour on a dim winter’s day. The kids got along famously at first, playing catch. Then suddenly, something happened and they were fighting.

KPN

I have just received KPN’s Christmas hamper. I’ll be opening it with the kids soon. Cozy. Last year, we had the option to forego the hamper in favour of a donation to a charity for muscle disease. I didn’t. That’s bad, isn’t it? Well, it’s too late for my muscles. Moreover, opening gifts with the kids is too fun to pass up, even if the contents are usually disappointing. This will probably be my last Christmas box. I’ll be laid off on January 1st, 2002. They didn’t want to fire me, despite me being on disability welfare for three years. I’m still very connected to KPN. The fact that employees were willing to take a pay cut to save jobs makes me proud. The commotion surrounding KPN’s executive severance packages, however, is appalling. Anyway, many former colleagues of mine have already left KPN. By now, the kids are home. The hamper didn’t disappoint. It’s just wine, chocolate, tea, biscuits, tea lights, a tray and a bag of sweets for the kids. Nothing fancy, exactly as it should be.

Quizzing

While being orally quizzed by me is no longer possible, I still try to lend a hand. I make quizzes on the computer for the subjects that lend themselves to that format, which we go through together. History is my favourite. I once considered getting a History degree. Quizzing languages is a slow process for me. It’s a task better suited for Hein and the grandmas. It’s surprising how much the grandmothers still know from back when, and how much joy they find in showing off. My mother is an especially bad case. When we were quizzing Floor on her French verbs, she constantly wanted to give the answers. Other grandma started spouting French accusatives and ablatives.

My involvement does sometimes lead to conflict. Maybe I meddle too much during Floor’s exam weeks. It’s in my nature. The fact that I don’t have much to do makes me more controlling too. On top of that, Floor isn’t very patient or overly diligent. She’s satisfied very quickly.

Upstairs

Because of the PC problems that were caused in part by the kids’ internet usage, we decided to gift them a computer for Sinterklaas. It’s upstairs in the study, along with the second TV and the Nintendo. The study is looking more and more like a second living room. I don’t like it when they’re upstairs all the time. On the other hand, I don’t get interrupted as much when I’m on my computer. But when I compare the pros and cons, it’s obvious that the cons weigh on me more.

Children’s neurologist

Floor has been suffering from involuntary muscle spasms (=fasciculations, a possible symptom of ALS). One weekend the panic really got to her, so we went to see a doctor. It didn’t help. The fellow’s answers to Floor’s questions were interspersed by long silences, leading Floor to conclude that there was actually something wrong with her. She was freaked out, despite the doctor’s claims that everything was fine. Nonverbal communication is a powerful tool. I know that Floor is very sensitive about this subject. The GP once asked if she still had spasms. Floor immediately took it as a sign that there was something wrong with her. To nip further panic in the bud, we went to see a specialist. Yesterday, Floor met with a children’s neurologist. It was a lady, to Floor’s relief. She conducted some tests. Everything was alright and she saw no reason to investigate further. She jokingly said that Floor is just a shaky girl and she’s just more cautious because of my condition. Floor is relieved again. I hope those fears are gone. Forever would be great, but I won’t fool myself with that illusion.

Indian food

For his birthday, Hein got a very special present from his Indian friend Lucy: Indian dinner, made by her. Yesterday was the day. Loaded with little pots and pans, partially prepared dishes and chopped vegetables, Lucy and her boyfriend Jan took over our kitchen. Apart from setting the table, Hein didn’t have to do a thing. And Lucy was well-prepared, so it didn’t take long until we were ready to dine. It was delicious. Even Ward, a picky eater, liked it. Sorry if I misspelled anything; Rendang, Gado-Gado, Ork Ark and Atjar were the names of the dishes. Finally, she made nasi for tomorrow. It was a night to remember.

The tree

It’s been a noticeable trend this year; putting a lot of Christmas decorations up outside the house. I’m a sucker for it. It’s my inner child. Our neighbours who moved to China got a potted Christmas tree in the mail. While we already had one, we nicked theirs anyway, for in the garden. It’s pretty, if a bit bare. That’s why I insisted on buying fake red apples. They’re pretty. And when my mother-in-law Lia told me she had a 9 metre long rope light, I was enthused. A string of brilliant lights would beautifully accentuate the coutures of our romantic shed and rosebush and only heighten our Christmas spirit. When the lights got here though, Hein protested. How could I want something so tacky in our garden. I guess he’s right. Our alternative is buying a lot of small lights to dress up our bare Christmas tree. On the holiday evenings we spend at home I suggested we light a torch. And like that, we’re participants of the exterior decoration trend. Of course, we have a wreath on the front door. It matches the neighbourhood. Meanwhile, the kids argue about who gets to have the rope light in their room. What do you mean, tacky?

Christmas letter

This year, I didn’t feel up to it; writing a Christmas letter. During previous years I had a lot to talk about: the diagnosis, drumming up publicity for my homepage, and last year I had my book. But I already write so much and I’m bound to just repeat myself in the letter. However, breaking a tradition is a big deal. I’m a bit superstitious and the Christmas letter feels like a good luck charm, and I’m still here. But I’ve put my foot down. No Christmas letter this year.

Yes or no

I’m slow. Shaking my head isn’t a big deal, I roll my head left to right. Giving an affirmative answer is much harder. I have to lift my head off of my headrest and move it up and down. It’s doable, but it takes its sweet time. Usually, the person who asked a question will have already asked another one. This world is a hasty one. With Ward and Floor, shutting both of my eyes means yes. But even they are sometimes unsure if I really mean yes. Blowing my nose can also take a while, because I need my time to forcefully breathe out. Usually, the handkerchief is already gone when I need it most. I’m slow.

Musts

By now, I’ve seen a lot more ‘must-see movies’; the Discovery of Heaven, Nynke (amazing) and on Sunday we finally went to see Harry Potter with family and friends (fantastic, so atmospheric). Now I just need to see Miss Minoes and I’m still hesitant to go see Lord of the Rings. I did love the books.

Christmas 2001

On Christmas Eve, we played Trivial Pursuit with friends. We’re always in teams, it’s more social and allows the kids to participate. On Christmas Day we did gourmet cooking with my brother. It’s obviously not my thing. We started too late, meaning I was starving. And no, I can’t eat any of the meat. I ate salmon and a lot of garlic sauce, plus a pile of fruit salad. We did have tiramisu for dessert, with homemade whipped cream. Lovely and very filling. My, am I glad I can still eat that. On Boxing Day, we had almost all of my in-laws over. Everyone had prepared a course, so we didn’t have to prepare too much. We focused on setting Christmas-y table: white tablecloth with red beaus, decorations and lots of candles. A beautiful sight and the kids were glad to assist. Our nephew was most impressed with our nativity scene. This time I just had mashed potatoes with veggies and a cranberry port sauce, very well-suited for me. Only both nights dragged on a bit too long for me. Now for some well-earned rest.

New Year’s Eve

The days leading up to it were quiet. Doing some grocery shopping, going for a stroll one time, sleeping in and sitting inside a lot. On the day before, Ward spent the whole day setting off fireworks. He’s got a taste for it now. I don’t know if that’s good, but I know I was obsessed with fireworks back in the day. We spent the evening with friends. It was a late one. We went to bed at 3:30. We played Trivial Pursuit again, Freek watched and set off fireworks for an hour. I kept my composure this year, I didn’t shed nearly as many tears. As opposed to previous years, Floor asked me if I wanted to cry again at 12 o’clock. It has become a sort of ritual, after all, and has lost its shock value. How things have changed. Of course, I happily obliged her wishes.

Eurodisney

Infected by their atmospheric Christmas commercials, we got the idea to go to Eurodisney for two days (one night) just after New Year’s. The weather during the week before Christmas was abysmal. Imagine getting stuck in the snow on our way; not much joy to be had in a snowed-in car. Apparently, Eurodisney is very gracious to the terminally ill; I get to cut in line. Anyway, we left on January 2nd, a wintery day. We’re always too optimistic about our travel times, we arrived at 4 o’clock. Our hotel, Santa Fé, had a Mexican artstyle: terracotta layering interspersed by cacti. After some difficulties regarding a badly booked invalid room, we went on our merry way. The atmosphere was magical. Walking in the dark, alongside the supposed Rio Grande, all of the beautifully lit hotels, a skating rink in front of Hotel New York, Disney Village (so American), Mainstreet (beautifully illuminated), and fireworks around the castle of Fantasyland. But boy was it cold. It was like we were on a skiing holiday. Everyone plundered their closets to stay warm: many different hats, gloves, skiing jackets, winter shoes. We, on the other hand, were woefully prepared. Floor had small ankle socks, Lieke only had one glove and Tim didn’t have any at all. The next day, we were in the park at 11, despite getting up at 7 and having breakfast at 8. I’m responsible for the time loss. Not many attractions are accessible for someone who can’t get out of their wheelchair. I was there, more so for the nostalgic atmosphere, we had been enchanted 6 years prior. The only advantage of me being ‘different’? Indeed, at the McDonald’s, we we got to cut through the crowd to a reserved table, I got priority seating during the parade and I was kissed and patted by Donald Duck and Goofy (to the great envy of some). But on the second day, I felt more like a burden, not being able to go on rides but still needing to be taken care of. And boy, was it cold. It took hours before the car had a liveable temperature. The journey home was smooth. On both trips I sat in the back of our van without it feeling like a rollercoaster.

2001

The end of the year does call for some sort of conclusion. Personally, the NRC interview was the highlight of the year. Especially people’s reactions to it were heartwarming. I think it was a good experience for the whole family.

Furthermore, I’m thankful to be living such a relatively normal life. The person who makes that possible is Hein. Every day, he gets me in and out of bed and he has for three years, with me only becoming more and more dependent. And always helping me use the bathroom and feeding me. Activities that require both time and diligence. And I’m not always easy to deal with. I’d like to see anyone else try it. I’m so glad he’s there for me. Floor and Ward contribute to my everyday life too, mostly by just being there.
After living with ALS for a few years now, some everyday activities have turned into rituals. I go to movies with Marjolein, once every three weeks I play bridge with friends, my friend group and I play bridge on birthdays, Sundays are for days out with friends, holidays with family and friends, going on walks with my brother Anton, Kees works in the garden and going to the theatre with the kids sometimes. Care has also been routinized. My friends and family help there, too: Marjolein and Tineke have alternated on fridays for three years, my mother, my mother-in-law, Lieke and neighbour Lisette. Inge has been with us for almost three years now. She’s always there for us. This summer break, she has been my tower of strength.

My physical social life has gotten smaller. Apart from friends, we don’t see many people. The concept of acquaintances is almost foreign to me because of my illness. By contrast, I’ve built up a huge virtual network; old acquaintances who mail me, but new people too, friends I’ve made through my homepage. Despite that, I do miss normal physical social interaction, small talk. I count people who are going through the same thing as me towards my friends, too. Sadly, some of them passed away this year.

Enough contemplation and reflection for now.
I wish you all a love-filled 2002.

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

We got back from Zeeland in mid-August, in the nip of time, just before temperatures soared. Hein, the kids and Lieke (my sister-in-law) took 5 days of sailing lessons. The first time they went on the Veere lake, they got caught in a thunderstorm. Luckily, they were quickly dragged to the shore. The following days were more fair and I was able to watch everything from the veranda, including their graduation from sailing school. Lakeside holidays are special; there’s a lot to do and to see, and by nightfall the still lake and the town of Veere behind it make for an extremely tranquil view. And the kids went water skiing, rode the banana (a six-man inflatable vessel in the shape of a banana, pulled by a motorboat at incredible speeds. I’d never heard of it but apparently every self-respecting lake has one) and played table tennis.

The cottage was spacious and wheelchair-accessible. The veranda was roofed and had a nice grassy view. The kids camped out, 100 metres away. That was good for my morning rest. Much less of a full house. I’m usually up early, sitting at the dinner table, washed and ironed as Lieke likes to call it. Lieke and my mother-in-law took the van on an extra trip to Leiden and back so I could be as comfortable as possible; desk chair, shower seat, patient lift.

Of course, we visited some towns as well: Goes, Willemstad, Vlissingen, Middelburg and, of course, Veere. And while my role was that of a spectator, I’d call it a successful holiday. All of this, no more than two hours away.

PEG troubles

Two months ago we stressed out a bit because of a leak in my feeding tube. Hein phoned everyone he could and eventually, Professor Mathuus was able to give us an answer: Hein would be able to fix it himself. Screw off the cap, snip off the tube at the leak and reattach the cap, simple as that. This holiday it happened again. In the middle of the night, I felt water (well, actually it was stomach acid) leaking out of it, accompanied by a lot of bubbling. The rest of my night was filled with fearful dreams of another PEG operation. We knew how to deal with it this time, so the next morning we snipped another length of tube off. But the next day it was leaking again, then again, and even a third time. Turns out that the innermost part of the tube was being eaten away. Again, Professor Mathuus (a most gracious professor who always returns my calls) knew what to do. When a PEG-tube is down to 10 centimetres, a thinner tube is attached below the older, wider tube. Nothing internal. You can imagine my relief. It’s almost 10 cm already!

By now, we’ve visited a doctor, more for additional comfort than anything else. Besides the thinner tube method, I could also choose to have a new PEG installed. The hole is already there, after all. With an inflatable balloon, the PEG is kept in place. The only downside is having to poop out the old cap later. With lighter hearts, we went home; at least I wouldn’t need another tube down my throat.

Surprise

On August 20th, Floor turned 14. Her girlfriends had organised a surprise party. At 12 AM they would be at our door, completely unannounced. Equipped with sleeping bags, of course, to spend the rest of the night chitchatting and keeping us awake. We were aware of the plan. Floor was supposed to already be in bed, but at 12 she was already demanding her birthday presents. At 00:05, there were 5 girls on our doorstep with confetti cannons and serpentine streamers, some of which are still floating around, one week later. It was a successful ambush, it took Floor completely by surprise. The next day at 12 o’clock the girls left, to be back at 6:30 for a rainy barbecue. Floor didn’t want a birthday cake this year.

Camp week

During the last week of summer break, the kids went to camp. Floor to La Roche in Belgium and Ward to Heerde in the Veluwe. They left on Saturday in the streaming rain. It’s so quiet at home without the kids. We had a little holiday of our own. Lots of evening activities and on Tuesday afternoon we went to the botanical gardens, to an exposition about carnivorous plants. We’ve already got one ourselves. Fascinating, but plants with the prefix of “flesh-eating” are a whole lot more terrifying than the average geranium. We’ve put it next to the fruit bowl so the plant can eat all of those pesky fruit flies (I’ve actually seen him eating one before). On Wednesday, after my homeopathy appointment, we went to look at gardens in Nunspeet. They did not live up to my expectations. We spent the evening in our friends’ torchlit garden, lovely. Despite my non-communication I felt like I was part of the conversation. Thursday- and Friday evening were spent on the beach. And during the weekend, Hein biked the Amstel Gold Race, 150 kilometres. He deserved a weekend without having to worry about me. Lieke took care of me that weekend. I slept in my chair in the living room, with Lieke next to me on a stretcher. My neck hurt somewhat, but I felt fine the next day. And the kids? When they returned they were dirty, tired, hoarse but they had a great time.

Het bericht Diary August 2002 verscheen eerst op Living with ALS.

On Tuesday night my PEG gave out completely. Right now I have a 4cm tube sticking out of my stomach and the cap no longer fits. The contents of my stomach were spewing out. After closing the tube off with a clamp, my PEG spent the whole night dripping fluids. It’s not a good time, let me tell you. The next day, Floor discovered that the clip we use to reseal bags of peanuts was the perfect way to seal the tube; no leakage. Too bad it’s so huge. Every time Hein put me on a chair, there was a chance of it coming loose. My greatest fear was, and is, the tube retracting inward, meaning I would need a new endoscopy.

On Wednesday we visited the AMC. Just as I needed him the most, it turned out our professor wasn’t available that day and nobody else was trained to install a new PEG. Thus, we had to improvise. In the end, I went home with a clamp used for umbilical cords (my idea) and a bandaid to keep the tube from going inward. I can’t use my PEG anymore, but that’s not much of an issue. I’m perfectly fine without it. The average lifespan of a PEG is 2 years, I’ve had mine for 2.5. I’ll hopefully be getting a new one on Monday.

By now, I’ve got it. It was a cakewalk. Despite that, I’m always incredibly nervous in moments like this one. My mouth cramps up and my teeth chatter. Good luck talking to doctors, who are likely already low on time and patience. The worst part was the laxative treatment to get the old cap out of my system as quickly as possible. I took 2 litres of laxatives in 2 hours. Luckily it was relatively manageable, no incidents. My only question: did I actually excrete the cap? I hope this marks the end of my “troubles”.

Aesthetically, my new PEG leaves much to be desired. The cap is bulky because it has three valves; one for water, one for medicine and one to fill the balloon in my stomach with water. As a consequence, it keeps tumbling around under my bra. When I look down I can see it bulging out. Right now it’s still concealed by winter vests, but come summer it’ll be very visible. All of that for a thing I barely use. For that reason I’ll probably have a word with my doctor. These troubles aren’t over yet.

Power outage

It’s a thought all of us have sometimes; what if the power were to go out right now? It happened on Sunday. The light had been flickering for a while beforehand, which is when the thought first crossed my mind. I calmed myself though. The lift was still working, so I’d just go upstairs. Barely a minute after I got off the lift, everything went pitch black. What if I’d been on the stairlift when the power went out? Luckily, Floor and Hein were quick to find some torches. Apparently, our whole street was out of electricity. For a while, I thought “luckily I’m already upstairs, I can still go to bed. But I forgot that my adjustable bed is always upright to make getting in and out easier. That position makes sleeping in it impossible. For a lack of better alternatives, I decided to sleep in a garden chair. In the middle of the night, the lights went on again. Anxiety came over me again. I don’t like surprises.

Adventurer

I’ve never been much of an adventurer, but I have never needed as much predictability in my life as I do right now. I had barely recovered from my PEG and electricity hijinks when my shower chair got stuck one morning. While I’m in an upright position I can’t hold my head up straight, meaning I can’t talk and everytime I pass a threshold I slump, go rigid and spasm. That means I’m in my chair, stiff as a board, slowly sliding down. Time to think of a temporary alternative again. Another disruption, as if I needed any more.

Art route

There was an art route in Oegstgeest on Sunday. Artists opened their doors and a few schools had expositions of art made in Oegstgeest. We had good walking weather. Because of limited accessibility, we decided to focus on the schools. Two times, I was parked outside, in front of the stairs. Schools from the 30s liked their stairs. I couldn’t see anything. By a stroke of luck, we found a gallery close to the mall, where I bought a pendant. That saved the day. And, like I said, it was a good day for a stroll.

Flags

Every email I have to answer (and I usually answer every single one), I flag. That way, I know how much work is still ahead of me. Sometimes I get disheartened when I see how many flags are still left. Other times, they invigorate me and I make quick work of them, leaving me content. But the following day, I find I already have a backlog. My happiness was fleeting.

Crap weekend

Everyone has an empty weekend every once in a while; not a thing to do. Sometimes, it’s a good time. Not this weekend. My standards for weekends aren’t exactly high; I want to at least have seen someone or have gone outside, preferably both. This weekend, I didn’t get to do either. On Saturday I was overwhelmed by self-pity. That rarely happens. I see my situation as a given, but right then I was extremely upset about being stuck in my chair, I felt bad for myself. I couldn’t even look at my computer, my eight-thousandth game of bridge couldn’t hold my interest (I still haven’t gotten any better). On Sunday there was a storm so we couldn’t go into town. In short, a crap weekend.

Hein 50

Hein will be 50 on November 6th. We’ll be celebrating it that Saturday in small company. I asked friends and family to write some things about Hein so I can compile it. You see, I have a computer program for making newspaper layouts. They always come out looking lovely. I have some work ahead of me these coming weeks.

Het bericht Diary October 2002 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.

Things are going well between my shower lady and I. We’ve finally figured each other out. She’s still a bit rough when she’s dressing me, but she takes the time to shower me and blow-dry my hair. She heats up my towel on the heater, which I think is on way too much. I actually like showering now, which became even more clear when she went on holiday in May and I was stuck with a substitute.

Hans Warren

I got a copy of Hans Warren’s final diary for my birthday. I specifically asked for it because I wanted to know what the writing of a professional diarist looks like. Besides, I wanted to know what was going through his head when he and his boyfriend Mario went through a rough patch in his last year. It was rough. I know writing must have been therapeutic for him, but it doesn’t take long for the reader to spot certain repetitions in the text, like him being lukewarm on the trips Mario took him on, not liking eating out, buying African art and having difficulty sleeping and pooping, but also Mario’s suspicions that Hans Warren was being difficult for the sake of being difficult. I skipped a lot of pages while I was reading it. I immediately bought the book written by Mario Molengraaf as a homage to Hans. I also did something I’d never done before, and had the book thrown in the bin. Obviously, that struck a chord with Hein, who immediately took it out of the trash.

Family holiday

In early May, we had a family holiday in Steenwijk. There were a trampoline and a ping pong table. I’d gladly visit it again. It had a sunroom, a huge living room, a big hearth, a verandah connected to a private lawn, a great handicapped bathroom and a giant kitchen. We loved it from day one. The weather was great too. We only spent the nights inside, the rest of the time we were outside. There were more kids than there were adults, but we did just fine. The trampoline was used by old and young people alike and we had plenty of table tennis competitions over the weekend. Lieke won in the end, beating her own son, Tim. It’s too bad that I felt sick on Saturday, so I spent most of that day inside. On Sunday we went on a boat ride through Giethoorn and hiked in Kalenberg, the exact same thing we did seven years ago.

Finals

We’re not too concerned about Floor’s final exams. She’s been drinking a lot of cola so she doesn’t fall asleep on top of her books. She gave us a list of rules so we know what we can and can’t say during her exams. At least we’re still allowed to pick what we’re having for dinner. We’ve been corresponding with a lot of people who are in the same situation. Tips on foods that give you a lot of energy, for instance. It turns out that bananas and glucose tablets seem to be the best options, so we’ve bought a lot of those. She’ll get the results on June 15th.
After she finished her last exam, Floor wasn’t as sure of herself as she was at the start. One week of Greece with her friends will surely calm her down.

24

I got the first season of 24 for christmas. I bought series 2, 3 and 4 online. I downloaded the final series, the fifth one, online. That’s it for 24 for now. 5 x 24 x 45 minutes = 90 hours of watching TV with Ward. I’ll miss it. I downloaded and watched Prison Break and Lost with Floor. There are only three episodes left, then we’ll have to wait for the series to start again. It’s one of the few things we’re able to do together.
Just when I’d decided to download a few more films, I discovered that the Swedish police shut down The Pirate Bay.

Eight years since my diagnosis

I was diagnosed with ALS eight years ago, which means I’ve had it for nine years. It’s become the new normal, even to the people close to me. I’ve had a PEG (stomach tube) for six years now, too, but I still eat regular food. Yeah, I’d say May has been an important month in my life.

Emailing

Writing emails has become difficult, more so than writing in my diary. Roeline and Lieke have been helping me get through my emails, diligently typing out the letters that are coming out of my nigh-unintelligible mouth. It’s such a great help. I was starting to dread email correspondence for a while.

P.S. I hope I don’t have to remind you about June 21st, World ALS Day.

Het bericht Diary May 2006 verscheen eerst op Living with ALS.

I’m eating more carefully than ever, but I still manage to choke on my food often, resulting in hours-long coughing fits. It’s especially terrible when I’ve almost gotten the mucus out of my trachea but it gets stuck. I always feel like I’m burning up when that happens. I usually have someone open the door, remove my blanket, adjust my chair and leave the room before I spent a long time concentrating on getting the mucus out. It happens about once every three days. The worst times were the ones during which I wasn’t at home. It’s happened two times so far, one of them in a supermarket and one of them during my weekly walk on Saturday. I’m developing a fear of being in public because of it. Maybe I should give up eating altogether and fully rely on my PEG. Porridge is the worst offender and usually results in a whole morning spent coughing and heaving. I’ve started eating less and my belly is getting smaller. But eating through a feeding tube feels like such a final nail in the coffin and I’m not ready for that just yet.

Sleep has gotten worse too. Whenever I’m in bed, I create so much saliva that I lose the ability to swallow, which wakes me up and sends me into a panic. But whenever I decide to spend the rest of the night in my chair downstairs, I wake up feeling short of breath, gasping for air.

Faith? I don’t have much left. I’ve become more tense, which lead to more spastic tremors in my legs. Now I understand how people develop phobias. I’ve started becoming afraid of being afraid.

Scenario

I’ve been getting a lot of comments on my story, about how badly things could have turned out. One fellow ALS patient wrote on her blog:

”That night, everything went wrong. I woke up and immediately had difficulty breathing. My night time carer cracked a window but it didn’t help. I asked her to get my neighbour, Eveliena. The last thing I remember was spelling the word “dying” a few times, after which I lost consciousness. I was completely bewildered when I heard someone say “Irma, you’re in hospital and you’re on artificial respiration.” What? I thought I was dead.
Suddenly, I was faced with the impossible choice between a tracheostomy and death.”

She picked the tracheostomy and spent four months in hospital before being relocated to a nursing home. That’s a scenario I want to avoid at any cost.

Ward

Now that Floor has moved out, it’s up to Ward to keep our spirits high. He’s such a treat. By day, he’s usually in his room, but at 20:30 he goes back downstairs to watch TV with me. Together, we decide on what to watch. The only times he misses TV night is on days when he has football training. And watching is a strong word, he usually intermittently checks his MSN on his PC. Ward feeds me bits of chocolate and adjusts my blanket and backrest whenever I ask him to. Hein does the heavy lifting when putting me to bed, but Ward always comes by later to fix the minute details. Positioning the pillow underneath my head is a matter of finesse. Ward is very patient.
He’s also a great cook. His favourite dish is tortellini, one he perfected when he was only thirteen. The same goes for salads. Normally, kids don’t learn how to cook when they’re thirteen. Then again, normalcy is few and far between in our family.

Twenty years

Next month, we’ll have lived in this house for twenty years. I’ve been sick for roughly half of that. It’s an odd thought. In those two decades, we’ve had three fireplaces, replacing the old-fashioned stone one with granite, which in turn was replaced by a romantic one adorned with a mirror. Optimistically, we ordered a lot of firewood. Optimistic, because we’d never actually used the fireplace before. Before we knew it, the whole living room was engulfed in smoke. Once, and never again we thought. We had some specialists look at the chimney, but apparently they thought it was a lost cause. That is, until we had one over two weeks ago who told us that it was simply a matter of making the chimney a bit wider, something he managed to get done within a week. It only took him about an hour. Hopeful, we put the wood into the fireplace and before we knew it, we had a perfect roaring hearth. We feel stupid about not having the thing fixed earlier. Ward has a penchant for firestarting. Thus far, we’ve spent two Saturday evenings gathered around the fireplace with a rented movie playing in the background.

The fundraiser

By now, we’ve had three meetings. The first one was the most fantastical, the second one was the most practical and by the time the third one rolled around, we’d already put the plans we’d made during the second one into action. The only things we still have to figure out are the Italian delicacies. Tickets are already on sale and we’ve reached out to local newspapers too. We’ve managed to land an interview in one of them and an article in another. We’re hoping to fill the whole theatre, so come one, come all!

Changes

After I got back from holiday, I made a few changes to my usual routines. When I’m using the lift, my carers know to support my ankle with their foot so I don’t sprain it. It makes using the bathroom a whole lot less stressful, as well as making it easier for me to position myself properly before sitting down on the toilet.
I also decided to drop porridge from my breakfast, replacing it with something called ‘Good Morning’, a nice nutritious fluid breakfast. I eat one glass with a spoon and one-and-a-half glasses of it with my feeding tube. As a result, I don’t cough as much anymore. Besides, I read on a Belgian ALS website that Activia yoghurt doesn’t thicken one’s saliva as much as normal dairy does, so I’ve decided eat that instead of my usual sour cream.

The last change has nothing to do with my illness, but with my age; I’m having difficulty reading newspapers. These days I only scan the headlines and need to keep my books at an arm’s length before I’m able to read them. I just so happen to hate glasses of any shape or form, so this might be an issue.

Het bericht Diary September 2007 verscheen eerst op Living with ALS.