Losing Weight With an Enzyme Disorder

I've recently been diagnosed with a deficiency in the CPT2 enzyme. Its a condition which affects the bodies ability process broken down fat for energy, which means when I begin to diet or exercise at a caloric deficit my body is unable to use the burned off fat as a source of fuel for my muscles. This condition became apparent about two years ago and I only got it diagnosed a few months back after having been in the hospital twice for rhabdomylosis. I had always been relatively fit and able to run long distances but since my last bout with rhabdomylosis in the spring I have had issues with my ability to do cardio or endurance training with symptoms coming up after a few minutes of jogging or biking. I have been able to lift weights for the last few months and while this has helped a bit, I am looking at how I can work with what I am able to do currently to lose fat without going back to the hospital. Obviously I will burn fat if I work at a caloric deficit but since I am unable to then use that burned fat as energy I run the risk of damaging my muscles and getting rhabdomylosis for a third time if I'm not careful. If anyone else on here has had this condition or has trained someone who does and has some advice or recommendations please let me know.

I've been meeting with my Doctor about it regularly but the best he has been able to do is tell me to drink a Gatorade before working out and have carbs on standby when I'm done. I'm not looking for legitimate medical advice but rather for suggestions from Veeky Forumsizens in my position I can run by my doctor before trying them out in the gym.

>CPT2 enzyme
This enzyme plays a role in the carnitine pathway of shuttling long chain fatty acids into the mitochondria for energy.

This inability to shuttle energy into the mitochondria forces the cell to look for other energy sources in a starvation manner. And since this is during exercise where glycogen is being eaten up, it will turn to the next energy source: Protein

Understanding this deficiency sheds some light onto approaches you may consider taking in order to work around or offset the limitations of your metabolism.

When exercising ensure that you have sufficient fast acting sugars to supplement what is being consumed. I would avoid gatorade since it's carbohydrates come from sucrose which is 50% fructose. Fructose can only be metabolized by the liver and preferentially into fatty acids. not to mention it's atp trapping nature.

Additionally because cptIi defficiency only effects long chain fatty acids, you may consider supplementation with MCT which process to ketones outside of the mitochondria.

And in addition to these you may consider supplementing with l-carnitine directly to increase carnitine levels to maximize what little efficiency you have in this transport channel. Long chain fatty acids only get through the mitochondrial wall when bound up with carnitine acting as a shuttle. More available shuttles on standby may help mitigate some of the enzyme defficiency.

In light of everything you will also want to consider your diet very carefully. Focus on a diet and schedule that minimizes fat storage and lipogenesis. This includes fructose, and foods that cause large insulin spikes. Choose slow carbs for dietary choices, and reserve faster carbs for workouts, minimize long chain fats and focus on shorter chain fats when used for cooking.

And in terms of exercise, recognize your defficiency in energy metabolism and choose a workout that isn't glycogen depleting along with plenty of intermediate rest to allow for recuperation.

Take a jar of honey wherever you go, especially to the gym. Between each set, take a spoonful of honey to stock up on free sugar and revitalize your muscles' ability to lift. People will buzz about it behind your back but after they see your numbers go up every gym thot will be bringing you flowers.

This all sounds like excellent advice, thank you.

I'm not sure that'll work but thank you for the chuckle

Actual quality service on/fit. I'm impressed.Shouldn't OP thou as well make sure to have good protein supply before training like pre-workout with BCAA or something like that or am I just an idiot.

All of the doctors I have consulted with have recommended I avoid protein supplements as I am already at risk of putting a lot of broken down muscle protein in my system whenever I engage in physical activity. Added protein in my diet post workout has seemed to help but I am trying to be fairly cautious. I'm already worried that my current level of fitness may be the best I can attain and getting rhabdo again will only serve to do me harm.

I don't particularly see how that could be of much help in regards to this metabolic deficiency. And I'd be wary of excess protein and amino acid supplementation on account of OP's history with rhabdo since his kidneys have already taken a couple hard hits in that regard.

I would be of the mind to caution OP against any serious attempts at getting "massive gains" and instead focus on lighter weight training and settling for leaning out instead of trying to make too much strength progress simply because of the risks associated with this shitty energy transport. Playing it low, slow, and cautious would be what I feel comfortable reccomending. Perhaps even going so far as to suggest sticking to strictly calisthenics.

>choose a workout that isn't glycogen depleting
Out of curiosity, what sort of workout can a grown(?) man do to challenge himself that isn't glycogen depleting? Anything that stresses your muscles and requires explosive force or straining against heavy weights will require available glycogen, no?

Not regarding metabolic deficiency, but was thinking there are on the market some supplements with contain BCAA and I-carnitine.

I'm speaking into glycogen depleting as in significantly depleting itramuscular glycogen to the point that the cells are starting to look at protein metabolism as a viable energy source.

Thus the suggestion at keeping fast acting carbs on hand in addition to sufficient rest periods.

I don't see how supplementation of BCAA would help OP when weighed against the associative risks he's facing in regards to this CPT-II defficiency. It would be my worry that throwing in excess amino acids in a system that's already flooding itself with junk from fucked up metabolism would be any benefit.

From my understanding as it is I don't see any benefit. It may be at some point in the future it may be beneficial to him to do such supplementation. But right now he needs to figure out how to minimize the side effects of conditions through targeted and intelligent dietary choices. Once he gets his shit under control with no more bouts of rhabdo he could then look at throwing extra protein in the mix, until then, there's no point adding any confounding factors into the mix.

Currently I'm doing a basic routine of 3 days of lifting with three days of light jogging.

On MWF:
800m jog at 6mph
3x5 front squats at 135lbs
3x5 of back sqauts at 205 lbs
3x5 of Romanian dead lifts at 225lbs
3x5 of dumbbell bench press with 2 60lb dumbells
3x10 of inclined sit ups with a 45lb plate on my chest
3x10 bodyweight only pushups

On TUTHSAT:
2 mile jog broken up into 1 mile segments at 6mph
3x10 inclined situps no weight
3x10 bodyweight only pushups

The running and push ups are the most strenuous on my system by far but I seem to be able to keep my weight lifting up without issue. Its already shown some benefits on tone and simply by tightening up my core and back I have lost about 2 inches along the waistline going from a 38" to a 36". I have tried to up the weight a bit, but this seems to be the extent of what I am able to do without seeing some symptoms like overheating, and dizzyness. Plus since my last case of rhabdo I have had chronic leg pain just above the knees in both my legs. It isn't as bad when the weather is colder or when resting but it is a constant issue. My main concern about my lack of endurance comes from when I attempted a 1.5 mile off treadmill run to test my fitness I was able to maintain my current speed and do it in 14:53 but I found myself feeling and looking like absolute death for about a day and a half with my symptoms persisting long after the run, plus I start sweating like an autist after even the slightest physical activity now, even when its chilly outside. So whenever I ride my bike a mile from my place to campus I look just like I ran a marathon and wont stop sweating for a good half hour.

I am currently 6'1" and 230lbs. When I was in really good shape I was 185 but I doubt I'll be in the condition I was then with my current issues.

Agreed. Btw did you knew this stuff off hand or did you look it up for OP, if you didn't then you defiantly put me to shame. Also are an MD or other health field related?

>Btw did you knew this stuff off hand or did you look it up for OP
I knew of the CPT pathway from studying fasting metabolism, I'm one of the more sensible /fast/ing fags, and I've used it extensively in training fat fucks who want to lose weight. I kew of it and the role it plays in converting fat to fuel but I did read up on some of the points of CPT-II defficiency to make sure none of my suggestions were immediately contraindicated.


>Also are an MD or other health field related?
Not an MD, and I don't work in the health field. Blacksmith, knife maker, and property owner/landlord.

What diet recommendations do you have?

My biggest change so far is that I've stopped buying bread. I do have a rice cooker and often have rice as part of one of my meals a day but I'm trying to cut a lot of calorie heavy items to help with losing weight. I've also cut beer from my diet, stopped buying potatoes, and have worked in a lot of raw vegetables and plant based foods into my diet.

my normal day includes:
Breakfast: 2 poached eggs, a sausage grilled in a gorge foreman grill, 1 cup of greek yogurt, and roughly 1 cup of raw kale, broccoli, or spinach.

Lunch: varies by what I have ready between that week's prep and leftovers, but always has a protein like beef, pork, chicken or red bean soup. A vegetable, either one of the same ones I use for breakfast, some oven roasted green beans or Greek salad (sliced cucumbers, tomatoes, onions, and olives, with feta, no dressing). Then either oven roasted butternut squash, or rice either steamed white or seasoned in the rice cooker to be more fragrant.

Dinner: Normally the same options but made fresh instead of tupperwared. I may also decide to use some wine and mushrooms to make a reduction or risotto instead of the rice depending on if I chose to cook the meat in a skillet that night. A glass of wine or some bourbon will accompany most dinners as well.

On weekends I will have friends over for dinner with much of the same but in higher quantities. Saturday nights are for drinking but by changing out beer for gin I have been able to remove a calorie dense food group. A large part of my weight gain since April was due to having just turned 21 around that time, being newly single, and having a well paying summer internship I spent a lot of my time drinking.

obviously I will burn fat if I cut calories from my diet but then I have to worry about how my body cant actually take advantage of that broken down fat for energy.

>when exercising ensure that you have sufficient fast acting sugars to supplement what is being consumed. I would avoid gatorade

What other options would you recommend I keep at the ready instead?

>supplementation with MCT in addition to these you may consider supplementing with l-carnitine

This is definitely worth bringing up to my doctor. do you have any recommended brands?

We're stepping out of my comfort territory which is to normally recommend a high fat diet with very low and slow acting carbs. However, your case is unique on account of the fact that you are defficient in a pathway that allows your cells to efficiently utilize fatty acids for energy. I want to point out that you are DEFFICIENT in this pathway, not that it isn't working at all. If it didn't work at all in you, you wouldn't have made it to the crawling stage of growing up.

This deficiency in your case is more of a bottleneck in that your cells struggle to get fat into the mitochondria for energy. This may be an explanation for why you've gotten so fat from alcohol which is preferentially metabolized into long chain fatty acids. If you make to much of this through lipogensis, and your cells end up not using it, it will get shuttled into the fat for storage.

It is from this understanding that my suggestion would be to focus on slow acting carbs and slowing down the carbs you do take in to minimize lipogenesis as much as possible. This can be achieved by ensuring you get plenty and varied fiber. Which you are on track to accomplishing with your kale and veggies. I would encourage you to look at being quite liberal with your leafy greens, and not just one type.

Instead of cooking with animal or vegetable fats, try coconut oil, or even mct oil which have shorter fatty acid chains. See what works for you.

As for the meats, go with leaner meats and fish. Your morning sausage on the george foreman grill is just as fatty as sausage cooked on a skillet. I would feel confident suggesting smoked ham, or seasoned chicken over a fatty sausage in your case.

>you wouldn't have made it to the crawling stage of growing up.
fortunately I have the version of this deficiency that doesn't manifest itself until early adulthood. I was able to process fats without issue for most of my youth and adolescence. IDK why it works like that but its what the doctor has told me.

>This deficiency in your case is more of a bottleneck in that your cells struggle to get fat into the mitochondria for energy. This may be an explanation for why you've gotten so fat from alcohol which is preferentially metabolized into long chain fatty acids. If you make to much of this through lipogensis, and your cells end up not using it, it will get shuttled into the fat for storage.

I think you're right about this one, I've stopped going to bars every week and have cut a lot of alcohol from my diet. Hopefully it will pay off.

>As for the meats, go with leaner meats and fish. Your morning sausage on the george foreman grill is just as fatty as sausage cooked on a skillet. I would feel confident suggesting smoked ham, or seasoned chicken over a fatty sausage in your case.

Not a bad idea ill look into that next time I go grocery shopping.

Honey like the other user suggested might be a good idea. Honey is typically 35% glucose and will go directly to the blood without much in the way of metabolism. In fact the youtube channel James Townsend & Son has a video about an 18th century energy drink which is actually quite refreshing that uses honey, check that out.

>This is definitely worth bringing up to my doctor. do you have any recommended brands?
for the MCT oil I always give my fattys a gallon of Nuvita mct oil. I use that myself. for l-carnitine, you could look into bulk-powders. I don't supplement outside of mct oil myself so...

>We're stepping out of my comfort territory
Fuck you and your sensible, well reasoned and intelligent posts. OP should be taking 5 scoops of protein and working out until he pees blood!!!

jk
Quality posts like these are why I keep coming back to Veeky Forums despite the fags.

>op isn't peeing blood
It's like he's not even trying amiright?

I'd love for OP to post this shit in the /fast/ thread and see all those morons to fast regardless even though it would kill him

I mean it was straight brown when I got Rhabdo the first time. does that count?