December 1998

I’m a bit depressed. I’m starting to lose hope. The antioxidants haven’t helped thus far. The talks with my neurologist have proven to be disastrous: “Speech therapy, physical therapy, they’re no use. Maybe a bit of emotional support, but other than that? ALS Digest? A bunch of hysterical Californian hacks.” It’s those small jabs at my emotional supports that make me feel undermined. I haven’t done any speech therapy since. Go on without the things that give me hope? I can’t. I wrote my neurologist a letter about it.

Maart 1999

Doctors aren’t doing me any good. My legs are shaking heavily now. Too much empathy is another thing that I can’t stand. I wish everyone could just act like themselves, like everything is back to normal. Then again, nothing is normal anymore, is it?

Het bericht Doctors 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.

Ward’s school has this bizarre tradition of planning outings in the middle of winter, including an actual night of camping. Two days of walking 30 kilometres using nothing but a compass, sleeping in a tent of three and heating up cans of pea soup. I don’t know what’s the point of all of it, but apparently it’s the fashionable thing to do these days. Ward was lukewarm on the whole thing. His whole outfit was borrowed, the only clothing he actually owned were his gloves. Luckily, his shoes had been broken in already, because Ward’s only preparation was going on a two-hour walk around the woods close to our house, although he did take some supplies with him. What was the trip like, you ask? A lot of walking. It was nice to try something new, but it wasn’t something he’d do again anytime soon.

Ward did winter sports during his spring break. I missed him terribly. He got back on Saturday and had the time of his life. On March 10th, Floor and Hein will go skiing for a weekend.

Scared

This morning I had a nasty accident with the stair lift, probably because my legs were badly positioned. I was still shaking minutes later. I’m always scared of it happening again. Afraid of being afraid. Some of my days are dictated by anxiety. Most normal people can’t fathom what that feels like. “Why wouldn’t you sleep in your arm chair?” Because it impedes my movement and I don’t like lying flat on my back. Nobody seems to think of the little issues. “Just try it for one night.” No thanks. I can’t adjust anything and that powerlessness is accompanied by fear, especially since I can’t talk. All I can hope for is for someone to help me. I prefer writing my fears down beforehand so there’s an emergency plan in case something goes wrong, but even then, I hate being completely dependent on someone else. It’s a matter of trust, something I don’t think normal people fully comprehend.

A good friend and ALS patient described it like this:

”Fear: probably one of the worst parts of having a disease like ALS. I suffer from it too. Fortunately, I don’t have to bother with the patient lift anymore, but when I did, my biggest fear was them leaving me hanging. I used it because my legs couldn’t support my weight anymore. The lift put pressure on my chest, because of which I had trouble breathing.
By now, my greatest fear is my life support. Sometimes, the fluids in the machine have to be replenished, so for a short while, I’m resigned to gasping, fighting for air. It has to be done quickly and efficiently. Last week, one of my nurses managed to put the water through the wrong filter and all I could do was yell incoherently, because I can’t talk without my artificial respiration, my lungs just don’t get enough air.”

My mother

My mother isn’t doing well. She’s in a lot of pain, even with her morphine patches. Last week, she stopped by our place while she was on her way to the hospital. It was the first time I’d seen in a wheelchair. I was shocked. I tried to distract her by talking about the kids, but she just wasn’t all there, slumped in her wheelchair from the pain. She also made a strange mix-up, like she was disoriented. Today, she’ll find out if she can have a back operation to reduce the pain somewhat.

Three weeks on and one hospital stay later. My mother trusts doctors more than anyone, while I try to see as few of them as possible. I select my doctors based on their behaviour and bedside manner (I’ve already got a different GP and, per my request, I always see a doctor I like during my lung inspections in Utrecht). My mother visits several. Of course, that’s easy for me to say. After all, I’m not constantly in pain.

She turned 80 on March 3rd. She’d been admitted to her nursing home the Wednesday prior. A short stay. She left the following day. She hated it there. She celebrated her 80th birthday feeling more chipper than usual in her own home.

Teeth

I’ve completely neglected my teeth since getting ALS. I always thought: “they’ll last longer than I will.” Not so, so now I’m screwed. Last week I had a terrible toothache, I couldn’t eat a sandwich because the crumbs hurt my gums. On Friday they pulled another tooth so I spent the day with bleeding gums. Whatever, the tooth itself had all but broken off completely, so I wasn’t complaining. Almost immediately, something else started hurting again. I’m meeting with my dentist soon to make a full plan. ALS patients, be warned: take good care of your teeth!

Holiday

I’ve been hinting for months that we need to start planning our summer holiday. By now, it’s too late, all of the wheelchair-accessible housing are already taken. Scandinavia didn’t look promising, except for one cottage 400 km deep inside the Arctic Circle. I found something in Ireland but nobody was too excited about it. The trouble is, we’ve seen a lot of handicapped-accessible housing over the years and it’s getting old to go on holiday in Europe.

Time for a family meeting. We had the choice between a cottage in Oxford or one in Zeeland, two places we’d already been once. The kids picked Zeeland because of the sea, the amusement and the possibility of having friends over. We decided to have a few trips to Belgian cities. Oh well, holidays close to home have their charms.

Kneeling on a bed of violets

I read the book with baited breath, despite not understanding the protagonist’s religious convictions at all. It’s a beautifully written book, penned by the oldest son of a family. He was in a unique position as the oldest. He was his parents’ mediator, did not take sides and had a lot of responsibilities, maybe more than a child should have. He reminded of Floor, but maybe that’s the position a lot of older siblings are in. Floor is a great mediator too and always acts as a buffer whenever Hein and I are at odds because of my illness. She’s got a great sense of responsibility too.

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

One wheelchair, one comfortable chair, one commode, one shower seat, one patient lift and one motorised rubber boat. We’re taking three cars, two of which are vans. The only thing that absolutely has to be handicap-accessible is my bed, which it isn’t, it’s too low. Besides not having any doorsteps, the cottage was not built with the handicapped in mind. It’s a good thing we can take most of the things I need with us in the van and there’s plenty of space in the cottage. Although, for privacy reasons, our fellow travellers did need to evacuate the cottage whenever I had to use the bathroom, but they did so without any fuss. It became a routine. Hein’s cousin Adriaan drove the van back to Leiden to collect the last few amenities I needed. I couldn’t thank him enough, my hero.

Everyone brought their kids, something I’m thankful for. The more, the merrier. There were eight of us, nine one the last day, in a cottage made for six. That’s why we brought a tent. We’d told the kids beforehand to plan their own activities, but when we actually got there, no plans had been made yet. Every day, the answer to the question “what should we do today” was “shopping in Berlin!” I bloody hate shopping. To me, shopping means waiting outside or being wheeled around with nobody to talk to because they’re all walking behind me. It makes me grumpy and panicky, especially when it’s hot outside. In Berlin, we spent a large part of the day in a cafe, while the ones among us who wanted to go shopping went into town. Everytime we wanted to leave, another one of us dipped out, meaning we had to wait at the cafe for hours. It wasn’t all bad. We visited Ravensbrück Concentration Camp, which was impactful. We had long walks through Berlin, Schwerin and a few smaller towns. We canoed, read books and played lots of games.

Close one

It happened during the second week of the summer holidays. It was a Tuesday. A bit of egg salad had gone down the wrong pipe and I’d been coughing all afternoon. I thought a car ride would do me some good and maybe dislodge the salad. Wrong. On our way back from grocery shopping, we hit a bump and some mucus got stuck in my throat. I was fighting for air and asked Hein to pull over. Usually, standing upright helps get the phlegm down, but it didn’t this time. I remember trying the “standing up” technique twice, but after that, there’s nothing.

When I regained consciousness, the whole family was standing around me looking freaked out as a paramedic applied an IV. At that moment, the second ambulance arrived, as well as another doctor. Apparently I’d been unconscious for 15 to 20 minutes, purple-faced and eyes rolling. I was surprised and still can’t quite wrap my head around it. I just let them take care of me, except I fiercely resisted their attempts to check me into hospital. My blood pressure was incredibly high, 240/160, but it was decreasing steadily.

I spent the rest of the night feeling awestruck, I still couldn’t believe it. My family was still freaking out. Only the next day did I properly realise the truth: that could’ve been it for me. All of the certainty I had was gone. We spent the rest of the holiday doing as little as possible. I had a blind spot in my field of vision which made reading impossible. Fortunately, it’s passed by now, after two weeks. I would’ve been distraught if I lost the ability to read. Other than that, I have an infected gland but the antibiotics should take care of that. Something I can’t get back is my feeling of certainty. Some nights, I wake up in a cold sweat because I feel like I can’t swallow anymore, but it’s happening less and less.

A talk

We had a serious talk with the kids, and I think it went well. Hein explained to them that I don’t want artificial respiration. If there are any further incidents like this one, I don’t want anyone to intervene, no more medical interventions. Those close to me have a right to know too. I’m glad it’s out there now.

Back home

Marjan was already waiting for us with a good meal when we got back from holiday. We’d called ahead and given her a shopping list. It took me the whole weekend to get used to being home again. It was Floor’s birthday on Monday and we had a lot of guests. Tuesday was the first normal day I’ve had in awhile. I felt more certain and relaxed again. It’s a bit embarrassing to admit this, but I like a quiet life, a regular one with as few surprises as possible.

Het bericht Diary August 2007 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.

I have three practical worries; my mucus problems, mobility and the accompanying trouble I have using the bathroom using a patient lift. All three of them are easily solvable, but to me the solutions are worse than the problems themselves. There are suction machines to help me clear my throat, but it only works for some people. Others say it only made their issues worse. Which one is preferable? My mobility problem could be fixed by using a sling lift, in which I’d have to sit in something that looks like a big sack. That doesn’t sound appealing to me either. I hate not standing upright and I don’t even think it’s possible to use a sling lift with only one carer, especially since my neck can’t keep my head upright. As for using the toilet, a lot of ALS patients choose a catheter, but that’s a big step, not taken lightly.

My trouble with standing upright made my shower lady ask me if I didn’t think it was time to get a sling lift. She thought Hein’s endless lifting isn’t sensible anymore. Besides, she said a sling lift is easily operable with only one carer. I still have my doubts, although I trust my shower lady more and more. However, she doesn’t see the gravity of the changes she proposes. Hein first talked to me about the possibility of getting a catheter and she backed him up, saying it was just a matter of emptying the bag of pee on my leg every time it’s full. “We could get a catheter,” Hein said, to which I scoffed “We?” Easy for them to say. When the shower lady said that the people delivering the sling could simply take away my standing-up one, all I could do was glare at her.

After a weekend of choking on mucus, we ordered a suction machine and we’ve made an appointment for a demonstration of a sling lift. Two more steps toward complete powerlessness.

P.S.: By now, we’ve tested out a sling lift. It was true, it’s operable for one person, provided that person knows how to use it. I had to endure so many “I told you so”s, not to mention the dreaded comment that the demonstrators could take the old lift with them when they left.
Getting a new sling lift would affect a lot of other parts of my life. It’s impossible to get my trousers and pants down without a way for me to stand up. I already wear special legwear with velcro and a fly on my backside. Despite that, it’s still a whole lot of trouble to get me on the toilet, making a catheter or incontinence aids (Diapers. They’re diapers.) that much more appealing

Oxygen

I’ve quickly grown dependent on oxygen. Three weeks ago, I barely felt stifled, but nowadays I can’t sleep for more than 1.5 hours without having trouble breathing. Luckily, I have oxygen now. Keep in mind, pure oxygen is not the same as artificial respiration. With something like tracheal intubation, a mixture of different gases including oxygen is pumped into the lungs. It requires an extensive hospital stay, usually a week, to find the perfect balance of gases for a person. I’m using pure oxygen. It’s not exactly good for me, because it increases the amount of CO2 in my blood, but it is easier. The oxygen tanks are delivered to my house without me having to stay in hospital. I didn’t even have to consult a doctor besides my first appointment to get a prescription. As little time in hospital as possible, that’s what I like. The oxygen comes in canisters and is applied through nasal intubation. I haven’t heard of too many ALS patients using this method. Of everyone I’ve corresponded with, only one used it too, and only at night. I hope I won’t have to use it by day, although I have noticed that taking afternoon naps is becoming more difficult because I’m breathing more deeply. I’m feeling restless.

Feeding tube

I ingest a litre of food through my feeding tube, which adds up to 1500 calories a day. Besides that, I drink a “Good Morning” for breakfast, eat fruit and Activia yoghurt. An average woman needs about 2000 calories a day, but seeing as I don’t move, I don’t need as much. I’ve been eating a lot more than I did before (I don’t know if ‘eating’ is the right term). Four glasses of PEG food a day, trying to emulate my normal eating pattern. I love how easy it is. Only now have I realised how much energy I spent on eating before. I’ve been gaining weight too. One of my carers said my face has gotten much fuller.

Sore throat

On Thursday my throat started to hurt a bit and the next day, I couldn’t talk anymore. The only thing that escaped my lips was a pathetic rasping. I obviously had strep throat, which I’d probably caught from one of my family members, all of whom were ill as well. It took two weeks to pass. Two weeks of not being able to clear my throat as mucus just kept building up. Two weeks of fighting for air. It was unbelievably tiring, all of the coughing and the intermittent panic attacks. I repeatedly asked my company to leave so I could concentrate on clearing my throat. I was afraid of showering. I woke up multiple times a night to try to get some of the mucus out. My throat didn’t even hurt anymore and, for awhile, I was scared that this would be the new status quo for me. Clearing one’s throat, something that takes about a second for normal people, usually costs me hours. Fortunately, it did pass. I haven’t coughed in two days. Yesterday, after three weeks spent indoors, I tentatively went outside. Not too far, mind you. Today, I visited the Anthropological Museum in Leiden, still feeling a bit apprehensive. On Thursday, I’m going to a musical and the fundraiser is planned on the day after. I’m excited, if a bit nervous.

Hein

For more than ten years, Hein has been my most trusted companion as my illness progresses. He was there with me, in the doctor’s offices, during homeopathy appointments, during the PEG operation, but also at the hairdresser’s, driving me around, going on holiday with me and even falling asleep. It’s not been easy for him, with the past few months being especially challenging. Every time I have to get out of bed to spend the rest of the night sleeping downstairs, he carries his mattress downstairs and sleeps next to me. I can’t be alone anymore. Lieke has been here to help me sleep for a night or two a week, and Floor does the same. Still, the lion’s share is Hein’s. He’s had my back from day one and is constantly vigilant. He may not be a paragon of patience, but I understand why he isn’t, even if it may not seem like I do in the heat of the moment.

Sometimes, I get mad when he doesn’t understand my needs, when he thinks I’m being too nitpicky about my body’s positioning or when he’s not in my field of vision while he’s talking to me. The same goes for him, he doesn’t always put up with my barrage of little tasks. It’s not uncommon for ALS couples to have these kinds of tensions. Still, we manage just fine, largely thanks to Hein.

Fundraiser

It’s finally upon us. The night before the fundraiser I was pelted with emails, especially about the Italian delicacies. At the last minute, it turned out that we weren’t supposed to make the Italian food ourselves as it was against the law to do so without a permit. In the end, we found a caterer. I considered the musical on the night beforehand a sort of dress rehearsal for the fundraiser. For the first time in a while, I was put into the car, alongside a canister of oxygen. There was no trouble and I fared well. So, the next day we arrived in Alphen, equipped with my most comfortable chair and oxygen. The theatre looked enchanting and the interior was very nice too. When I came in, I was the centre of attention, a position I don’t do well in, I had to acclimatise first and find a nice quiet place to settle down. I was seated by the entrance, able to watch the crowds come in. Hein and Floor were by my side, while we had a good view of the cloakroom, where Ward was busy handling people’s coats. A picture was taken of me and the organisational team before the show began. It was a successful night. Monieke gave a speech, the show was fun with a lot of audience interaction. Monieke, as the person who had set the whole thing in motion, was lauded by the ALS foundation and the final yield was almost 4000 euros. During the intermission I had a picture taken with all of my carers, who had never met each other before that night. We went home at half past twelve at night and we hit the hay at two. I was still tired the next morning, but it was worth it. I had a marvellous night. Thanks, everyone!

During our post-fundraiser meeting, we drew a few conclusions, which are as follows. Generally, we were happy with how the event went. The Italian delicacies set us back a bit because the catering cost us some money, but it did look very festive. In total, we’ve made a net 3000 euros from donations and ticket sales, so combined with the LEGO sorting nights, we’ve made 4300 for the ALS Foundation. We didn’t know 60 out of the roughly 200 people that showed up, so we must have drummed up some publicity as well. My website was visited 2500 times last month by 1800 unique users. That’s about 600 more than my average.

Relieved

Yesterday, we met with my occupational therapist. It turned out that I wouldn’t need to alter my wardrobe any further in order to use the sling lift. You can imagine my relief. Besides that, she believed that the sling lift wouldn’t impede my bathroom usage, so a catheter wouldn’t be necessary. Another reason for celebration. She talked to me specifically and not just to my carer. I think I’d like to see her more often.

Sinterklaas

This will be the first year we celebrate Sinterklaas with just the kids. Travelling all the way down to Alphen to celebrate with fifteen people is becoming a bit too straining for me. I’ll be spending the rest of the month writing Sinterklaas poems, which is why I’m posting this diary early.

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