How Fluffy Died

A week ago Fluffy died after a life that lasted 3 times longer than veterinarians predicted it might. Until a few days before his death he was happy, comfortable, and affectionate. Although he was uncomfortable briefly at the end, he did not suffer fear or pain, as I had long been concerned he might.

When he was first diagnosed with NM, there was no data on how the previous 4 cats known to have the disease had died. Other cats suffering from a similar illness (hereditary myopathy in Devon Rex) died in the main from choking on their food. The veterinarians who diagnosed him suggested, and for many years I feared, this could be how Fluffy would take his leave. Knowing the alarm this sort of death would cause him, I dreaded that eventuality.

Sad as I am to say goodbye to my dear friend, I am happy to report that it was nothing so panic-provoking that took his life.

Years ago, Fluffy's heart was examined for weakness due to an excessively high heart rate. (His pulse leapt by 80 beats a minute from walking about 10 feet.)

His heart proved to be healthy, but as the surrounding affected muscles lost tone and became weaker, the strain on that muscle increased. His vet, Janice Crook of Mosquito Creek Veterinary in North Vancouver, B.C., suggested putting him on Cardio Plus (now called Feline Cardiac Support) by Standard Process. This nutraceutical* supports and strengthen the heart. In the years that he was on it his cardiac related bloodwork dramatically improved. It seems clear that he lived considerably longer due to a daily concoction of 1 crushed pill in a puddle of raw liver. (Yum!) (He was also taking 2 Hepatic Formula--liver-supporting nutraceuticals--after a few years, when his liver began to show strain, as well.)**

In the end, after nearly ten years, the work required of this small muscle became too much. Over a period of a few days his appetite decreased dramatically and he began showing signs of malaise. Most surprisingly, he was not purring (except occasionally when visited by friends, such as Janice Crook, DVM, and Bree from upstairs) and he did not want to lie in his favourite position, cradled baby-style in my arms. This second seemed suddenly to cause an increased workload for his heart, or perhaps his lungs. His breathing became slightly laboured at this time.

Bloodwork and urinalysis showed him to be in excellent health except for a somewhat stressed heart. (Nevertheless, his cardiac blood values were three times better than when first tested.) A physical exam revealed a galloping heartbeat. He was in heart failure.

At this stage we were faced with a limited number of options.

We could fuss over him, taking his blood pressure (which would require shaving his arm and annoying him greatly, knowing his perspective on such things), doing a heart ultrasound, and so on, but Janice and I agreed that these would be of no real use--expensive, distressing, and in the end providing interesting information, perhaps, but not affecting the actual course of treatment.

We could accept that his time had come and euthanize him, or allow him to end his days naturally. (There are those who argue that the right thing to do is always to allow an animal to die naturally, others who always intervene. And there are those who watch and see and try to decide what makes sense in each changing situation. Janice's advice to me years before was that when her animals begin to have 50-50 good and bad days she starts thinking about euthanasia. Fluffy skipped that step and went straight to 100% lousy.)

Or we could try the one medicine she knew of that might help. Vetmedin could perhaps assist his heart to contract strongly enough to allow a comfortable continuation of his life. The drug itself, she said, didn't have the unwelcome side effects of earlier heart medicines. Most cats in heart failure tended to have tough, thickened hearts. This medicine works better with thinned hearts. (Remember, I was grieving as all this information flowed around me. I am giving you a dumbed down version because it's all I could take in.) In Janice's opinion it seemed likely this was the condition of Fluffy's heart, and that he might respond well to the medicine.

I decided to try the Vetmedin. She said he would feel better in a day or two if it was going to help. He was somewhat revived the next day--purring some and cleaning himself and eating more--but the following day he was depressed and uncomfortable again. That day I called his pals to come say goodbye, and we took him to Janice the following day for a last exam and euthanasia.

I have not written as much in this blog as I had initially intended to. Knowing how very few cats have this disease, I didn't worry too much about getting the information out. But I think there is something to learn from the course of Fluffy's illness and the different steps we took to assist him in living well for the time he had.

In support of his heart, I made a daily practice of seeing that he exercised.

In the early days I took the physiotherapist's advice and turned myself inside out trying to get him to stand on his hind legs and walk around, and drove us both crazy bothering him to do so. Also on her advice I tickled his hind legs and spine to stimulate them, ran a vibrating toothbrush over his body (again to stimulate the muscles to contract), and generally made him feel like running and screaming (well...) every time I came near. In the end I didn't feel convinced this was helping, though perhaps it was, but the stress it all created in him, and therefore in me, the perpetrator, seemed not to be worth the hypothetical gain.

Over time I instead taught him to hop up onto my shoulder to be carried around, and would not take him outside or allow him out on his own until he hopped up. This was to keep his hind legs exercised, and because he had a natural inclination to hop up, anyway, he took to this habit well (with the need for mild encouragement nevertheless still often in place). I learned various ways to encourage him to hop up when he wanted me to lift him. One thing that made it easier for him was to settle him on the floor so that he was balanced over his four legs, rather than flopped over on one side. Or to lift his head slightly so that it suggested an upward movement. To tap my shoulder and say, "Up, up, up!" enthusiastically. And so on.

The other main element of his exercise program was to take him to the end of the yard and plop him down and leave him to make his way home. I put him out a couple of times a day for exercise and personal hygiene purposes.

These two practices were much more natural and pleasant for us both than trying to get him to play when he was tired of it or walk when he had nowhere interesting to go. (With NM walking is better exercise than running because different muscle groups are involved in each, and it is the short muscles used in walking that are affected by the disease and therefore in need of assistance.)

The combination of attention to diet (more on that in a future posting), exercise, water intake and crystal buildup (more later), stress reduction, and the use of nutraceuticals had the effect of lengthening his life by many years. (His first brush with death came when he was 8 months old; the weakness of his system was alerting us to its presence for the first time. At that time the examining veterinarian said, when pressed, that there was clearly something very wrong with him, though she had no idea what, and that if he were her cat, she would put him down.)

Giving Fluffy a long, healthy life was done without a lot of expense, but with a great deal of attention to detail, particularly as he aged. Far from being a burden, this requirement to pay attention led to an increase in affection and calm in all parts of my life, and to a greatly rewarding friendship with a self-assured and contented cat. It also encouraged me to to face issues of death and disability in a direct way. Who could complain about any of that?

Thanks, Fluffy. You are truly missed. Yet more than anything I am grateful for the opportunity to know you.

Cheers, kiddo.


*nutraceuticals are extracts of foods demonstrated to have had a physiological benefit or provide protection against a chronic disease. (See American Nutraceutical Association.)

**see Feline Instincts' Twelve Year Study on Additional Feline Organ Support

Fragility with Age

I haven't continued working on this blog in some time. Life, as always, intervenes. However, I am still available to discuss NM in cats with anyone who wishes to leave their contact information on this blog.

In the meantime, Fluffy is coming up to his 10th birthday, if I am reckoning right. He is still happy and mostly well, but is increasingly fragile in an unexpected way. He has had two episodes of urinary tract crystal blockage in the last three months, such that my vet has lent me the apparatus to unblock him at home.

This is a nerve-wracking situation and it is more important than ever to offer him water over the day, to keep him hydrated enough that his urine is dilute and the crystals are washed easily away.

My suspicion is that as this increases a point will come, not too long in the future, where I will have to put him down. His has been a happy life and one of endless discomfort and distress is not how I want it to end. But that decision will not be an easy one.

What to Expect on A Blog Like Fluffy

Coming soon to this blog:

--a list of useful links--Now Available--scroll to bottom of page.
--information about NRM and
--the specific progress of the disease,
--varieties of treatments tried, and
--other interesting bits in the life of one cat.

Your comments and questions are welcome at any time.

If I haven't got what you want here, let me know. I am taking my time as I know the demand is low for this info--but for those who need it, it is important, and I am ready to respond.

Using the Links

Nemaline Rod Myopathy occurs in both humans and other animals. I provide links to human-related NRM sites to increase our understanding of the mechanics of the disease, but I would caution against assuming that the disease and its treatments would be parallel in all cases.

Symptom List

Fluffy appeared normal at eleven weeks of age when we first met him, although within a few days we noticed a "clumsiness"--for example, jumping to a fence and missing, where his twin had no trouble executing accurate leaps. Later we were able to guess that this was not clumsiness at all, but an early sign of weakness. This weakness was apparently not what he was expecting--time and again he attempted things which he should have been able to do, and appeared agitated at his failure. It took a long while for him to stop trying to, for example, climb trees. Over time he showed difficulty while urinating or defecating--not in passing the waste, but in staying squatting while doing so. For that reason, he soon took to lying on his side and slowly emptying his bladder or bowels. It was a bit wrenching to watch, but in fact it didn't distress him and, other than creating the odd occasion when a little cleanup was required of me, it was simply a part of life that caused no particular trouble.

Except in two ways. It became my job to make certain that Fluffy was taking in enough water. He wouldn't go over for a drink across the room unless he was quite thirsty, and with the flaccidity of his bladder urinary crystals could build up more easily than in some cats. If adequate water wasn't taken in, these crystals weren't flushed out, and it was therefore easy for him to become blocked. For most of his life this was easily taken care of by keeping dishes of water on either side of the bed, where he spent most of his time, and I would offer him a sip when I thought of it, and paid attention to signs that he may be thirsty. (Eg, watching intently while I took a drink, etc.) In the last six months to one year of his life it became difficult to leave for more than a day or two as he would not take in enough water on his own, even with cat-sitters offering him water twice a day, to stave off blockage.

The other, much more rare, difficulty related to water, which occurred a few times over his life, but not more in the latter years than in the middle years, was constipation. This was treated in the usual ways, but was best avoided with increased hydration.

Twice in the final year Fluffy appeared to be constipated, due to an apparent mass in his abdomen and a terrible straining to pass. Even the vet thought this was the problem, but on examination there was no impacted fesces. It was, instead, a spasming of his abdominal muscles as he attempted to urinate. He was in fact mildly blocked with crystals, but because of the weakness of the surrounding muscles simply could not push past and ended up twisted and in pain. These increasing incidences of blockage were a result of critical weakening of the muscles and a heightened sensitivity to even a slight decrease in hydration.

Tremours, though initially small and not terribly significant, proved to be the clue that finally led to an accurate diagnosis. There are several diseases similar to NM, such as the muscular dystrophies; tremouring does not occur in them but is a diagnostic of NM. As time went by this tremouring, which happened mostly while he was standing, walking, or otherwise trying to use his supportive muscles, did become quite pronounced. In the latest stages of his illness I could feel tiny tremours and quiverings in the small muscles when he was at rest, but these were subtle and inconsistent. The tremours are not painful and did not appear to affect his comfort or mood.

Heart stress. The heart itself is not directly affected by NM. However, as the affected muscles weaken--the intercostal muscles (between the ribs), and so on--the heart must work increasingly hard to support movement. Over time this causes great stress to the heart.

At some point as a young adult Dr. Crook examined Fluffy and took his pulse while resting and after walking about eight feet. His pulse jumped 80 beats a minute after that brief exertion. We had his heart tested and it was found to be strong but working hard. Dr. Crook recommended a nutraceutical called Cardio Plus (now called Feline Cardiac Support) which he was given daily for several years. Immediately before his death his blood was tested and his heart was shown to be in better shape than it had been before starting on the neutraceutical years before. Nevertheless, the extreme stress on it from the diminishment of other muscle groups led eventually to heart failure. He was euthanized to spare him the possibility of drowning when his lungs filled with fluid as his heart failed.

At around age seven, Fluffy began to show signs of strain on his liver, which Dr. Crook attributed to the NM. He was then put on another nutraceutical, Hepatic Formula for Cats, by the makers of Cardio Plus, Standard Process.

Despite these sometimes saddening, sometimes alarming symptoms, Fluffy maintained a relaxed, happy existence for ten years, with only occasional incursions into his calm world. He was without question, and without bullying, the dominant cat in his neighbourhood, and even when he was very weak much stronger cats would slink away rather than mess with him or his smaller, fitter brother, Sparky. He was deeply affectionate, had an impish sense of humour, and was tolerant of a wide variety of failings in those around him. He would not, however, permit me to sing. This proved very useful. When he was lolling about in the bushes and I couldn't find him to bring him in for the night, I would start quietly singing scales and he would suddenly appear. If I continued, he would run over and hop onto my shoulder. (This was incorporated into his exercise regime, as you can well imagine.)

So do not worry that he was kept alive by heroic means when it would have been kinder to put him down. He was not. The moment it became clear that he was suffering in a way that would not quickly cease, he was gently and lovingly killed by his favourite vet, while his two best friends sat with him. The rotten woman who always held him for drawing blood and other unpleasant things was sent away by his flicking tail and he was allowed to die, as he was allowed to live, in peace with the people he loved.