Glycogen Storage Disease

[Boodicca]

Anyone else on here diagnosed with GSD?

 

[Lamont D]

Hi @Boodicca ,
No, I don't have the condition.
And welcome to the rare metabolic syndrome gang.

I don't know what advice or treatment you have received.
But as someone who has a tendency to look up these rarities, I have noticed that the dietary recommendations is close to what I was getting when first diagnosed.
And of course similar conditions, to do with how your body copes with food.

I found that recommended dietary control, just didn't work, for many reasons.
I suspect you joined the forum because of the low carb approach.
Has it worked?
Can you keep a record of how your symptoms are, wether better on low carb?

I hope someone does know what the best dietary advice is.

With RH and similar conditions, eliminating the worst of all carbs, sugars in my experience of over ten years, is the best control.
It may not be for you, you will have to experiment and find out.

If I can help, I will.
Best wishes.

 

[Melgar]

Anyone else on here diagnosed with GSD?

Hi @Boodicca if you don’t mind me asking, what are your symptoms? I have wondered if I have a glucose storage issue. I have mentioned it a few times in past posts. I go hypo , but not reactive hypos either, just spontaneous ones. Also my liver fails to correct. I see my blood sugars going low, I‘m watching for my liver to correct, but it fails to do so, but not always. I have to correct the low myself. Now the caveat here is I am into fitness and I may just be using up all my reserves.
My Dr thinks I have the early signs of LADA, but we shall see.

 

[Boodicca]

Hi @Boodicca ,
No, I don't have the condition.
And welcome to the rare metabolic syndrome gang.

I don't know what advice or treatment you have received.
But as someone who has a tendency to look up these rarities, I have noticed that the dietary recommendations is close to what I was getting when first diagnosed.
And of course similar conditions, to do with how your body copes with food.

I found that recommended dietary control, just didn't work, for many reasons.
I suspect you joined the forum because of the low carb approach.
Has it worked?
Can you keep a record of how your symptoms are, wether better on low carb?

I hope someone does know what the best dietary advice is.

With RH and similar conditions, eliminating the worst of all carbs, sugars in my experience of over ten years, is the best control.
It may not be for you, you will have to experiment and find out.

If I can help, I will.
Best wishes.

 

[Boodicca]

Hi
Theres no treatment for my type of GSD, just told to drip feed sugars through the day but to be careful as this could cause diabetes, they then started me on Ozempic but it doesn't seem to do much, I'm definitely less exhausted but still feel as if I could sleep 24/7. Interestingly my sugars responded to Ozempic but after 2 years I realised I was becoming more exhausted and gaining back 14k of the 24k I'd lost. Took a routine FBG at 8.2 and knew that whatever else I needed to do that I needed to low carb. The reason for my post is that I'm getting bizzarre readings and my carb consumption is, comparatively, on the floor and my FBG rising, the lower my carbs theres a slow steady rise in my FBG, only rising by .1 a day but I'm a bit twitchy, if this keeps up in a couple of weeks I'll be back at my start FBG!
I'd be grateful for any suggestions, my specialist is the other end of the country and since covid we do telephone calls and my appointment is not until next Friday.

 

[Boodicca]

Hi @Boodicca if you don’t mind me asking, what are your symptoms? I have wondered if I have a glucose storage issue. I have mentioned it a few times in past posts. I go hypo , but not reactive hypos either, just spontaneous ones. Also my liver fails to correct. I see my blood sugars going low, I‘m watching for my liver to correct, but it fails to do so, but not always. I have to correct the low myself. Now the caveat here is I am into fitness and I may just be using up all my reserves.
My Dr thinks I have the early signs of LADA, but we shall see.

Hi
There are 5 types of GSD so only the specialist could tell you if you have one of them. My basic symptoms start off with me as a supersized child, with continuous weight gain that nothing could control, then this developed into many problems which I initially thought was caused by the weight. Struggling to walk, unable to walk up stairs, used to have to wedge myself in the doorway and then use my abductors to lift my foot sideways onto the step, then realised I couldn't stand without becoming hypo, but weirdly when blood sugars taken theyd be in the 5s.
Unable to lift a small bag of shopping is a classic symptom. Terrible cramps in my neck and shoulders and across my lower back, the cramps will respond to rest and a sugar dose. Because my carbs are so low currently, the choking which is when the muscles in your neck spasm, has become severe when taking my tabs this am I spasmed from my stomach to my oesophagus, mega painful and I'm sipping water cos I've never been scared like i was this am. So I probably should tell you that it's the proximal muscles which dont work, I can only take tiny steps and have to walk with my arms out stretched to try and keep my balance, I can only walk about 3 metres. Since ive been forced into a wheelchair by my disease I do feel more mentally positive, just wish I was still at the walking stage but all diseases are progressive so there you go.
I always think diabetes is a storage disease, we store blood in all our body parts and then we start to sweat it out and when theres nowhere else for your body to store it you're blood sugars raise and if your lucky its found.

 

[Melgar]

Hi
There are 5 types of GSD so only the specialist could tell you if you have one of them. My basic symptoms start off with me as a supersized child, with continuous weight gain that nothing could control, then this developed into many problems which I initially thought was caused by the weight. Struggling to walk, unable to walk up stairs, used to have to wedge myself in the doorway and then use my abductors to lift my foot sideways onto the step, then realised I couldn't stand without becoming hypo, but weirdly when blood sugars taken theyd be in the 5s.
Unable to lift a small bag of shopping is a classic symptom. Terrible cramps in my neck and shoulders and across my lower back, the cramps will respond to rest and a sugar dose. Because my carbs are so low currently, the choking which is when the muscles in your neck spasm, has become severe when taking my tabs this am I spasmed from my stomach to my oesophagus, mega painful and I'm sipping water cos I've never been scared like i was this am. So I probably should tell you that it's the proximal muscles which dont work, I can only take tiny steps and have to walk with my arms out stretched to try and keep my balance, I can only walk about 3 metres. Since ive been forced into a wheelchair by my disease I do feel more mentally positive, just wish I was still at the walking stage but all diseases are progressive so there you go.
I always think diabetes is a storage disease, we store blood in all our body parts and then we start to sweat it out and when theres nowhere else for your body to store it you're blood sugars raise and if your lucky its found.

Thanks for taking the time to explain your symptoms @Boodicca . Very enlightening and of course, horrible for you, sheesh. As a kid and pretty much all my life I have been on the line between very thin and underweight so completely the reverse. I was like, 5’6 and around 100lbs. Thin, likely because I had undiagnosed coeliac disease. I had perfect blood sugars until about 4-5 years ago - they were 4’s and low 5’s. My blood sugar problems started after I had a serious bout of pneumonia, then sepsis. Rushed into hospital, the Resus unit then the ICU. Very scary I thought that was it I’m going to die. I’m still here. A couple of months later, I had my blood sugars tested, perfect, then in the September I was suddenly prediabetic and so that is when my troubles with blood sugars began. Hypos, serious ones. Had the tests for cancers/tumors. I was told I was Type 2, as everyone gets told regardless of symptoms. Nobody can tell me why I get these hypos. I am insulin sensitive. I would hypo after exercise, hypo at night, hypo without food, hypo walking. Having said all that I was told to eat more carbs through the day and this helped with the hypos. My body aches, my muscles hurt when I do activity. It’s like I have run a marathon, that feeling your muscles have been over worked and are full of lactic acid. No idea, but I have read a number if articles on insulin storage disease and often wondered if this is my issue.

 

[Lamont D]

I am always interested in how to treat symptoms, such as I get when I feel a crash coming on.
How should I treat it?
The dietician, the endo, the dsn, any doctor would say treat the low with something that will boost your BG levels, to stop the low and the symptoms.
How many times have you heard that by doin that you feel better...........
And you notice the rollercoaster BG levels, spiking again......
You do for a time until the symptoms coming back.
This is the rebound effect, there is another phrase, but I can't recall it. Most T1s have had this sometime.
With my condition, when I spike again, too high, that triggers the reaction.
But over the first few months, I found, that if I didn't trigger the reaction, I don't get symptoms, and I don't get the spikes the hypos.
It is inconceivable to me now, to deliberately eat what triggers the reaction, carbs an sugars.
What I use to treat it before i go hypo, is a single plain biscuit, and keep testing every fifteen minutes until it has consistently around normal levels.

Of course, unless you know what triggers your symptoms. Could it be food or what you drink?
Can you fast?
Have you been tested for food allergies or intolerances?
Have the docs said why you have mobility issues?

With RH, it is the excess insulin because of the abnormal high spike triggering, insulin resistance at first, then second phase insulin response, and my liver can't produce enough glycogen for glucogenisis (liver dump) to prevent hypoglycaemia.
The excessive insulin is something that is an issue for my obesity before diagnosis.
I was over eighteen stone, lost six stone after going without carbs for a few months. I am always wary about putting weight back on.
I just always wonder, if those with metabolic conditions, what is the route cause?
And the majority, but not all, is what goes into our stomach.

Best wishes.

 

[Melgar]

@Lamont D I have got better at managing my fluctuating blood sugars, I try and keep them flattish, no big spikes. My DN at the time along with my GP directed me to have carby snacks, which helped with the hypos. So upping my carbs, helped with the hypos. I can’t fast because that would definitely lead to a hypo. When I do start going low I eat something sugary, but small to stop any yo-yoing. It’s interesting because sometimes my liver kicks in. I see my blood sugars plummeting on my CGM, then whoosh my liver dies its job and I’m at 6. A joyous thing. I wonder why it can’t behave like that all the time. The very low carb diet did nothing for my blood sugars, so that put an end to any low carb diet.
Ed to tag Lamont D.

 

[Boodicca]

Thanks for taking the time to explain your symptoms @Boodicca . Very enlightening and of course, horrible for you, sheesh. As a kid and pretty much all my life I have been on the line between very thin and underweight so completely the reverse. I was like, 5’6 and around 100lbs. Thin, likely because I had undiagnosed coeliac disease. I had perfect blood sugars until about 4-5 years ago - they were 4’s and low 5’s. My blood sugar problems started after I had a serious bout of pneumonia, then sepsis. Rushed into hospital, the Resus unit then the ICU. Very scary I thought that was it I’m going to die. I’m still here. A couple of months later, I had my blood sugars tested, perfect, then in the September I was suddenly prediabetic and so that is when my troubles with blood sugars began. Hypos, serious ones. Had the tests for cancers/tumors. I was told I was Type 2, as everyone gets told regardless of symptoms. Nobody can tell me why I get these hypos. I am insulin sensitive. I would hypo after exercise, hypo at night, hypo without food, hypo walking. Having said all that I was told to eat more carbs through the day and this helped with the hypos. My body aches, my muscles hurt when I do activity. It’s like I have run a marathon, that feeling your muscles have been over worked and are full of lactic acid. No idea, but I have read a number if articles on insulin storage disease and often wondered if this is my issue.

Some of the rare metabolic disorders are just that, part of GSD is we're missing the chemical messenger to activate energy from cells,which is why they put me on Ozempic. But I remember saying to a consultant that my muscles felt theyd been shredded after exercise like someone had used a grater on them and they said well the muscle problems associated with GSD are that when your body can't access glucose from fat stores it breaks down muscles into energy, but I can assure you that they are not being shredded! Well they also said they didn't know why i displayed symptoms of a hypo but my bloods never went below 5! There just aren't enough drs interested in metabolic disorders to do the research, they are massively overloaded and in the waiting room i used to hear people discussing their symptoms and people would wake up 5 stone lighter than when they'd gone to bed, horrible for the patient but harrowing for the dr they're hoping to find a cure for them. I always think I'm lucky not to be in their position.

 

[Melgar]

I find your muscle issues very interesting. I definitely have a muscle issue. I was a competitive runner for much of my life, my muscles were strong and I could run for hours, now I struggle to walk. It’s as if they are not getting enough energy to operate, like I have run them to exhaustion and are now full of lactic acid. That kind of feeling. I can barely walk up the stairs. I power through because I’m pig headed and I’ll just power through despite the aching.

 

[Melgar]

@Boodicca what are your lipids like? I have low triglycerides. My last lipid panel put my Trigs at 0.54 which would suggest I can’t use fat as an energy source as my liver will not have enough to store it for energy.

 

[Boodicca]

I find your muscle issues very interesting. I definitely have a muscle issue. I was a competitive runner for much of my life, my muscles were strong and I could run for hours, now I struggle to walk. It’s as if they are not getting enough energy to operate, like I have run them to exhaustion and are now full of lactic acid. That kind of feeling. I can barely walk up the stairs. I power through because I’m pig headed and I’ll just power through despite the aching.

Definitely dont give up, there have been times when I wanted to but I know that I went into the chair when there was no other option, and yes pig headed I think of my body in two ways, one is you wont beat me and the other is thank you for keeping me alive! It must think I have a split personality

 

[Boodicca]

@Boodicca what are your lipids like? I have low triglycerides. My last lipid panel put my Trigs at 0.54 which would suggest I can’t use fat as an energy source as my liver will not have enough to store it for energy.

My lipid profile is way too low, I take statins to raise my good cholesterol I think the last time I was tested they were below 0.5, but the consult said it was common in metabolic disorders.

 

[Melgar]

I really appreciate your input @Boodicca. Is the 0.5 your triglycerides?

 

[Boodicca]

I really appreciate your input @Boodicca. Is the 0.5 your triglycerides?

Hi it must be 5 years plus since they last checked my cholesterol and all were extremely low I would say between .34 and .50, cant remember which though.

 

[Lamont D]

@Lamont D I have got better at managing my fluctuating blood sugars, I try and keep them flattish, no big spikes. My DN at the time along with my GP directed me to have carby snacks, which helped with the hypos. So upping my carbs, helped with the hypos. I can’t fast because that would definitely lead to a hypo. When I do start going low I eat something sugary, but small to stop any yo-yoing. It’s interesting because sometimes my liver kicks in. I see my blood sugars plummeting on my CGM, then whoosh my liver dies its job and I’m at 6. A joyous thing. I wonder why it can’t behave like that all the time. The very low carb diet did nothing for my blood sugars, so that put an end to any low carb diet.
Ed to tag Lamont D.

@Melgar
it is the rollercoaster BG levels, abnormal high and rapid drops, that are the symptoms causation.
our body's cannot cope with the extremes of how the hormonal response to even a cold, virus and so on.
Even though our reaction from this is completely different, the treatment is different, the diet is different and more. It is the endocrine system that retaliates to the spikes and the crashes of the rollercoaster BG levels.
Different but predictably the same, in so many ways.
It is up to us to discover how to control our conditions, cos even the specialists, the nboffins, don't have the research to hand. They seem to be guessing or following the medical books, that one size fits all with metabolic issues.
@Boodicca .
You mentioned that some people get symptoms of a hypo, or a false hypo, and the readings are in normal levels.
There is a condition called Idiopathic Postprandial syndrome. Where the patient gets all the symptoms but no hypos at all.
There is another condition that is called flatline hypoglycaemia. As you can guess, regardless of their intake or circumstances, BG levels remained in normal levels.

Is glycogen storage disease (surely condition?) Is a description of what your metabolic condition is, just as RH describes mine? But it is a new one on me!

I will read more about it. But I defo don't like the thought of someone who is prone to weight gain, as you describe, will benefit from constant food all the time.
I got that advice, it was to stop the hypos, but the symptoms didn't stop, and if I didn't eat, I would still go hypo. I had rollercoaster BG levels, which like too much glucose or insulin, is not good. And as with other hyper/ hypo hormonal imbalance issues, the symptoms and poor health will always be there.

 

[LittleGreyCat]

@Boodicca have you had any tests to confirm which kind of GSD you have?
I understand that it can be genetic in many cases and the type can be narrowed down by genetic analysis.