A healthy lifestyle is, of course, recommended to anybody who wants to live a full and healthy life. It has been proven that our lifestyle has a considerable influence on our health. The same applies to the person suffering from CPT 2 deficiency, only in his or her case this recommendation is even more important, as a myolytic attack can be devastating and life threatening. Therefore, a lifestyle has to be chosen which minimizes the symptoms of CPT 2 deficiency, and also minimizes the risk in a CPT 2 deficient person.
Before we go into the details of our lifestyle suggestions and preventative
measures, it should be emphasized that the following suggestions should be treated
with respect, but applied wisely. It is counterproductive to become obsessed
(and invariably stressed) with all kinds of draconian measures which may result
in losing one’s joy of life, which in itself is a key ingredient in keeping
a healthy mindset and thereby staying healthy.
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As said elsewhere in this website, the possibility for normal daily exertion and exercise for short duration is not impaired in patients with CPT 2 deficiency, whereas this is usually the case in other diseases of the mitochondria (like carnitine deficiency). This is because under normal circumstances, with no other attack-triggering symptoms present, the mutant CPT 2 enzyme is sufficiently effective in most CPT 2 patients to provide in the energy requirements of the muscle cells. It is only under longer muscular strain, when the fatty acid metabolism is stressed, that the enzyme is most vulnerable, as it can not cope with the large amount of fatty acids that need to be transported through the mitochondrial membrane. The switchover from (short term energy providing) glucose consumption to (long term energy providing) fat consumption by the muscles is called the 'second wind' phenomena, which healthy people experience.
As mentioned above, there are (in general) no early warning signs for CPT 2 deficient patients as to when the exercises have become too strenuous and/or lengthy. The patient gets no indication when the fatty acid metabolism has collapsed, only some time after it has collapsed do the first symptoms surface. Myalgia, cramps, stiffness and muscle weakness usually develop in the course of strenuous, long lasting exercises, such as long distance hiking and biking, mountain climbing, swimming, body building, etc. Strenuous exercise of longer duration should therefore be avoided, as should fasting during these conditions. Also, remember that standing for a long time (outdoor concerts, etc...) uses the leg muscles intensively, and should best be avoided, or at least alternated, with prolonged periods of rest. Under any of the aforementioned conditions, a CPT 2 deficient person should always give his or her muscles the chance to 'catch up' again, by resting and relaxing the muscles, and by eating foods which are rich in carbohydrates.
On the other hand, it should be stressed that people with CPT 2 deficiency should not have a sedentary life. As is true for almost all people, moderate intensity exercises of relatively short duration are beneficial as they strengthen muscles and bones, elevate the mood, relax the body, and also strengthen the immune system.
Being healthy personally, in all senses of the word, is the best defence against
all kinds of diseases. Simple exercises, such as a 15 to 30 minute walk (or
swimming, or biking) each day, are usually enough to increase body resistance
considerably as compared to living a sedentary lifestyle.
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As mentioned elsewhere on this website, a diet high in fatty acids can provoke an attack in CPT 2 deficient patients, as the metabolism cannot cope with the necessary amounts required by the muscle cells. The general recommendation is therefore to avoid too much fat intake in your diet, and to get a large amount of your energy by carbohydrates; in other words a low-fat high-carbohydrate diet. It is also recommended to take frequent meals constituting this mix, as a steady flow of carbohydrates to the muscle cells is advisable.
In general, a truly low-fat diet contains much less fat than the average person is used to eat (but is often recommended). For most people, fat intake exceeds 40% to 50% of caloric intake (the fat content also being mostly saturated). With a diet of 2000-3000 calories this means 100-200 grams fat a day. Generally, health professionals these days recommended (for a healthy living style) in non-CPT deficient patients a fat intake of 25% to 35 %. In the case of CPT 2 deficient people, it would appear advisable to limit fat intake to 10% to 20% of caloric intake (with 60% to 70% of caloric intake coming from carbohydrates, and the remainder from protein). This effectively implies that the maximum daily intake should be only 25-50 grams of fat. This does not seem much but this amount is sufficient to cover the body's need for essential fatty acids. If for some reason this would be too little fat intake, remember that the human body is capable of making fat out of carbohydrates if needed. As a general rule, it is hard for our body to convert carbohydrates into fat, and this is particularly useful in the case of CPT 2 deficiency which is an incapability to handle an overload of fat, and an extra reason to stick to a low-fat high-carbohydrate diet.
What practical implications does this have? 25 to 50 grams of fat per day is not much, compared to what people have become accustomed to with the prevailing western lifestyle. For example, a 100 grams portion of cheese easily contains 30 to 60 grams of fat. One portion of French fries (without mayonnaise) is 15g fat, and a normal sized steak contains 25 grams of fat or more. Most fat is found in (red) meats (unless the meat is lean, or ‘white’ such as chicken or turkey), (fatty) fish and milk products (unless they are low fat varieties). Oils are also (fluid) fat; namely 100 % of their weight! One tablespoon of oil contains 14 grams of fat, therefore beware and take care when cooking your meals! Try to use non-stick pans and the like to reduce the necessary fat and the uptake of it by the food.
Furthermore, because of the good taste, fat is hidden in all kinds of snacks and ‘goodies’ such as cookies, cake, pies, chips, mayonaise, etc. Always look at the amounts on the food labels. As a rule, one should not bake anything in fat (butter) and use nonstick cookware, steamers, woks, etc. If necessary use only a small amount of oil (a teaspoon is often sufficient). Cooking, grilling, roasting, and barbecuing are all acceptable.
As for everybody, the recommendation is to eat a lot of greens and fruit, because they are plentiful in providing vitamins and antioxidants in their natural combination.
Be prudent with milk products, because of the relatively high fat content that they provide in standard portions. Try to use the low-fat varieties that are available these days.
The same can be said for eggs: the egg yolk is full of fat as well, therefore the use of eggs should be restricted.
It is especially when we dine out that we have to be alert. As everybody knows, fast foods are mostly rich in fat (that is why we like their taste so much). This is so well known these days, that some fast food restaurants now have introduced salad bars for those people who do not want to eat the 'regular' fast food products, which are full of fat.
If you eat in a regular restaurant, look at the menu and discuss the fat content of the meals with the waiter or the chef. They can give you the best idea as to which dish is the right one for you. In general, try to choose lean or white meat dishes (if you want to eat meat that is) and take dishes where it is cooked, steamed, grilled, barbecued or sauteed. Bear in mind that Chinese, French, Italian and Mexican foods tend to be high in fat content. As advisable for everyone, try to avoid fatty sauces or dressings, as they usually consist of pure fat. Back to top...
With regard to Medium Chain Triglycerides (MCT’s), several researchers have
suggested the use of medium chain triglycerides in CPT 2 deficient people. Later
studies have suggested that the role of CPT in MCT transport is considerably
higher than expected and recommend that the MCT dosage should not exceed the
CPT transport capacity. Any dosage of MCT over 0.5g/kg would probably overload
this uptake mechanism (1997, Shaefer et al in J. Neurol Psychiatry 1997, feb;
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As noted before, infectious diseases, and especially viral infections, can be a trigger for a rhabhdomyolytic attack in people with CPT 2 deficiency. They do this through a direct influence on muscle metabolism and fever. Viruses reported to cause rhabdomyolysis include influenza virus, echovirus type 9, adenovirus, Epstein-Barr virus, herpes viruses, and, rarely, coxsackieviruses. A case of rhabdomyolysis associated with coxsackie B virus infection occurred in a patient after mild exercise on a treadmill.
Recent systemic viral infections may predispose patients to significant rhabdomyolysis, even after non-overly strenuous physical activity. It is therefore important to avoid these infections as much as possible.
In the first place, this should be done through a healthy lifestyle to boost
the immune system. Particularly, the dietary measures and regular exercise advised
earlier are important for achieving this goal. Equally important are the measures
recommended to avoid stress and promote relaxation, as stress has been proven
to inhibit the immune system to varying degrees. The consumption of fresh fruit
and green vegetables is always recommended in case of illness. Furthermore,
the use of antioxidants (vitamin C, E, beta-carotene, selenium among others)
are also recommended by some, especially because the lipid peroxidation of cel
membranes seems to play a role in the lysis of muscle cells. Vaccination (e.g.
the anti flu shot) before the epidemic season should be seriously considered.
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Stress in general, and emotional stress in particular, are also mentioned by CPT 2 deficient patients, and also researchers, as possible triggers for an myolytic attack. This is because stress has a negative influence on the immune system, and also causes (continuous) muscle tension. Stress should therefore be avoided as much as possible. A lot can be said about stress, but only a short summary is given, as the management of stress can be a delicate and difficult affair, in which the treatment really needs to be tuned to the requirements of the person involved.
Every person should appraise their personal situation, finding out where (and if possible, why) stress occurs, and conclude which situations (such as work) constitute too much stress, and which do not. When the sources of stress have been identified, a path should be undertaken to a) avoid stress and/or b) reduce the negative effect which the stress causing factor has on the person in question.
For people sensitive to stress, it may be beneficial to learn to relax through methods like (muscle) relaxation exercises, yoga, breathing exercises, walking, outdoor activities (gardening), meditative exercises, listening to music, playing chess, or any other method which may provide the person in question with relief from stress. This is basically dependant on the person in question - finding out whatever method works for them to reduce their stress is very important.
The CPT 2 deficiency association is currently experimenting with a pocket size
biofeedback apparatus which measures muscle tension in certain key muscles,
and gives a signal when this tension becomes too high. This muscle tension is
directly related to the amount of stress that a person experiences, and therefore
this apparatus can aid in identifying which situations cause stress for a particular
person. If you would like more information on this device, please contact us.
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Under the heading Acute Renal Failure (see: CPT Deficiency 101) we wrote: Intracellular iron is an important mediator of tissue damage. As a transition metal it can donate and accept electrons, and its toxicity is due to its ability to catalyze oxygen- and non-oxygen-based free radical reactions. In rhabdomyolysis, the cytotoxic iron effect is derived from heme, a product of myoglobin metabolism. In the renal tubules the iron so derived catalyzes free radical reactions, which are associated with lipid peroxidation. The major mechanism of renal tubular damage in rhabdomyolysis is the mitochondrial free radical production which induces lipid peroxidation.
Procedures which chelate iron, or prevent or reduce the release of free iron,
have been shown to have a protective effect, thus demonstrating the important
role of iron in tubular damage in rhabdomyolysis (with the possible ensuing
ARF). We can add to this that free radical formation and the shift in the reduction-oxidation
potential may also play a significant in the lysis cascade at the muscular cellular
level. It is for these reasons that some recommend the preventative use of antioxidants
(e.g. vit. C, E, beta-carotene, selenium) and the Co-enzyme Q10 (important in
ATP production), the use of which was also advised under the heading "The
treatment and prevention of infectious diseases"..
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|Version||Revision date||Revised by||Comment|
|1.0||14 august 2000||MHN|