Lipoatrophic Diabetes

[Rusty650]

Hi just interested to hear from anyone on the forum who may also have "Lipoatrophic Diabetes" please ? I appreciate this is an extremely rare form and is a result of the rare disease Lipodystrophy. I was mis-diagnosed for 15 years as a Type 1 but now through working with the excellent research team from Addenbrookes hospital, I finally have been given a new classification.

Regards
Si

 

[Rusty650]

There seem to be a number of different types of Lipotrophic Disease and following diabetes and on reading about this I see how many different quite common metabolic situations might lead to lack of diagnoses. From the American National Institute of Health, here is a summary article.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4947059/

Which type do you have and how were you misdiagnosed as type 1 to begin with? For example, a lot of people are diagnosed type 1 as opposed to type 2 from specific blood tests. Were you not given these tests?

What about your new diagnosis and treatments has changed your regimes and lifestyle?

I'm thinking that more folks ought know about this, that it might not be as rare as some people think, and that there ought be at least summary articles here on the diabetes.co.uk forum. I hope you can share your story. On the rareness issue, when I was first diagnosed with Fuch's Endothelial Dystrophy in 2012, it was rare. Now, because of the internet and awareness through the Corneal Dystrophy Foundation (a totally patient run organization), a lot of people have been found to have it and it is no longer considered rare. Looking at the symptoms and scenarios in the article above, I am thinking that your kind of diabetes might also be discovered as no longer rare.

Hi Contralto
I have partial lipodystrophy and the exact gene variant is still being researched as I present with known symptoms from two types that are known to the research team today.

As for sharing my own story , I will try to keep this as short as possible, I experienced problems with glare from headlights when driving at night. It became such an issue for safety that I went for an eyesight test where they found cataracts in both eyes ? The optician referred me to my doctors who after blood tests revealed Hypothyroidism and possible diabetes. So I was sent to the hospital diabetic clinic and was initially diagnosed as having type 1 diabetes by a consultant by the use of the GGT test and the usual known symptoms, thirst, tiredness, excessive urination,etc and on physical appearance ie: extremely lean. I was started on Novorapid and Lantus from day one and had both cataracts removed at the age of 33 around the same time of the initial diagnosis.

The years roll on and I continue to be treated as a Type 1 diabetic. Slowly but surely my insulin requirements creep up. Through the advice of the hospital team I adjust basal rates attend a carb counting course and just keep going. Some years later and i am now on 8 - 10 daily injections taking in about 50 units of basal and about 100 units of bolus to cover food, etc. I always injected in my abdominal area because it was so painful to use my thighs or buttocks mainly because I was so lean. I had to switch from using traditional insulin pens to a syringe and vial as the pain involved in administering the high insulin doses was too much to bear with a spring loaded device. At least with a syringe I could control the pressure applied.

I soon developed hypertrophy on my abdominal area due to the repeated use of bad injection sites. I was then advised that my absorption of insulin was the problem and I was asked to try an insulin pump, however my local hospital at that time did not have agreed funding for such devices. Therefore due to the proximity of where i live I was able to transfer to the next hospital trust where pumps were being used.

So i apparently met all the criteria after being reviewed by two consultants and was started on the Accu-chek Insulin pump using Novorapid. So on I go again as normal. A number of years later I start to fell pretty un-well to a point where i am back for more blood tests where a Pituitary adenoma is found and following the relevant tests, started taking Cabergoline to manage this.

However I still had problems with morning blood sugars being high and generally lethargic and tired. I was told its the dawn phenomena and then started the procession of basal testing to achieve a good pattern that worked to compensate for this. I battled with this for months on end to the fact i just expected this to be the norm and to shut up moaning and just get on with it.

Following a low point in my life I sank into depression mixed with painkillers and alcohol to blot out day to day life issues. I tried to keep this a secret from everybody close to me and this really wasn't the best thing to do. One particularly bad day I disconnected my insulin pump and threw everything to do with diabetes in the bin. I could not take anymore. This constant 356/24hr problem would never go away and I felt so ill i just wanted to runaway from everything.

Well this turned out to be the best thing i ever did, because I didn't have any insulin for three to four days and on awaking from this drink and prescription painkiller frenzy I was shocked to find myself still here.

After a kick up the backside from friends, family and HCP's I once again embraced my diabetes but this time i knew something wasn't quite right. I must be producing insulin ?

So I began my own test with reducing my insulin requirements and over the months try to convince my hospital team that i wasn't a type 1 diabetic. However supportive they all were I was told it's just a honeymoon period and this can happen sometimes as we are all different. No way after like 12 years you must be joking i thought !

Roll the story on again and I attend in the summer on a very hot day a routine diabetes appointment wearing a t-shirt and shorts where my consultant noticed how slim my legs and arms were. She asked to examine me further by which time after several visits outside the room to consulate with other members of her team she said I think you are presenting with Lipodystrophy.

I had never heard of this and she explained further she would be sending me to Addenbrookes hospital in Cambridge for further tests at the National Severe Insulin resistance clinic.

So i finally attend the clinic where following lots of blood tests and research people probing and prodding me they confirmed that i was not a Type 1 diabetic or Type 2 but infact i had the severe insulin resistance syndrome of Lipoatrophic diabetes.

This has been a complete shock to the system. But as of today I am managing this well. I still take about 40 units of Fiasp insulin for basal through a medtronic pump and administer the relevant bolus for food accordingly. I take 10mg of jardiance and was put on a extreme low fat, medium carb (80-125g) and high protein diet as there is no cure for Lipodystrophy at present. My pancreas produces the normal range of insulin following the C-peptide tests and I have no anti-bodies from GAD testing either. My daily blood sugar remains in the 5-6 range and i am just waiting for the results of my latest Hbac1 soon.

I currently cannot exercise much as i have muscle dystrophy in my right leg from the Lipodystrophy and have had ulceration of my left foot due to the weight bearing problem of having zero amount of fat on the soles of my feet.
I have to use a wheel chair currently but i am hoping to eventually get back to being active once again where i can cycle, run and walk more freely as i did prior to all of this.

Its not all still plain sailing and i still have to work at managing my conditions as we all do, but I think I am now finally travelling on the right track to get me home ward bound so to speak.

Sorry for the long story but if this helps anyone on the forum and raises some awareness especially about the importance of C-peptide and GAD testing at early diagnosis then so be it. Any questions please ask....no probs at all

All the best
Si

 

[lindijanice]

Thank you, @Rusty650 for sharing your incredible story with us. It just goes to show how many angles/aspects of diabetes so many of us just don't know about. I'm sure you will always be grateful you wore shorts and a tee that day and had such an observant Nurse! Well done for all you have been through and how you are now coping. Keep up the good fight! Blessings/L

 

[mountaintom]

Thanks for sharing

 

[Rusty650]

Thank you, @Rusty650 for sharing your incredible story with us. It just goes to show how many angles/aspects of diabetes so many of us just don't know about. I'm sure you will always be grateful you wore shorts and a tee that day and had such an observant Nurse! Well done for all you have been through and how you are now coping. Keep up the good fight! Blessings/L

Thankyou for your kind comments@lindijanice yes we don't get many summer days like that anymore so a twist of fate it seems for me that day !!

 

[Rusty650]

Thanks for sharing

No problem in sharing my story @mountaintom, just hope it hasn't bored too many folks !!

 

[Rusty650]

Dear Si,

I found a picture online of someone with lipodystrophy with muscular legs and only a lot of fat distribution on the abdomen.
From the article I read, it can be normal to also have some distribution of fat at part of the back. I can see how the differences in fat distribution might be easily misinterpreted. This is a guy with a skinny butt and legs and a pretty normal abdomen. See below

I've had some muscle wasting in the legs and pain and by process of elimination think it was caused by various drugs, specifically ARBs, a kind of heart medication which can gradually come to cause diarrhea, and HCTZ, a common water pill. So, I take a different drug these days and avoid thiazides and other diuretics. Once I took first one, then the second, off my regimen, I found less debilitating muscle pain and atrophy and gradually regained some muscle. Not a huge amount has grown back, but some. Getting rid of the pain is half the battle.

I am curious what was the difference in the testing that they gave you over at the Addenbrookes hospital in Cambridge or what were the further tests at the National Severe Insulin resistance clinic? Maybe there are a bunch of lipodystrophy folks here in Forum land hidden under wrong diagnoses??

I am also curious what you actually eat. Maybe a week or two on your diet may be helpful to some of us type 2s. I just got around to listening to Dr. Bersteins's monthly lecture tonight. He spent quite a bit of time railing against reducing proteins for all types of diabetics and is concerned about ultra high fat diets. And certain type 2s and type 1s are expected to have low fat for heart, kidney and liver conditions. I wonder if the kind of carb matters.

As for the feet, I have come to appreciate memory foam as a great cushion that fits in shoes when cut to size. Thin mattress toppers of the stuff can be purchased cheaply and provide a huge amount for padding this and that. Not so great for summer as it is warming but there are also gel filled insoles and heel cushions. Memory foam is not washable, so if you are going to sweat, putting something that can soak up the sweat between you and the foam is practical, just like wearing white cotton athletic socks is practical and leads to fewer reactions to color and less mold and fungus when you sweat.

View attachment 25913

Hi Contralto
Yes the picture depicts lipodystrophy well. For me I always thought I had a very defined muscular tone to my arms and legs even though I had never really used resistance training. Just lots of cardio trying to get my blood sugar down, but now i know different when it was actually the lack of adipose tissue from the Lipodystrophy which was the cause. I am also agree with you that there are probably more individuals out there mis-diagnosed or waiting to be diagnosed with similar problems.

For me regarding tests, I had a whole host of blood tests done, too many to list here but a couple of things from this that i noted was the early requirement for C-peptide and GAD testing. I strongly believe this should be brought right back to the initial diagnostic stages of anyone presenting with possible diabetes symptoms. Im thinking GP level.

Although I was extremely lean, I was tested for Leptin levels as this sometimes can be associated with some variants of Lipodystrophy. Again I know from my experience at the clinic Leptin deficiency can have a serious effect on obese patients and you would like to think more exposure and awareness is made of this again in the earlier stages of diagnosis. I think there must be a lot of obese people struggling to lose weight when realistically they have a leptin deficiency which is the root cause of this.

Professor Stephen 'O' Rahilly who leads the research team at Addenbrookes SIR clinic has done remarkable work in this field of Leptin and obesity. Well worth a "google" to find out more about him and his research findings.

Cushing syndromes featured heavily in the testing. Again not much awareness of this serious disease but I think more and more people i helping to bring this to the wider public.

As for what i eat, through the advice of Addenbrookes nutritional team, my focus has to be low fat, medium carb and high protein based. I also try to adopt my own personal strategy of "fueling the car". That is if I'm only going on a short journey in the car, i put in a short amount of fuel. But if Im going on a long trip then Im filling up the tank. So if i have a very lazy day its the bare minimum to keep me going ( metabolic base rate). But if it's a busy day with lots of activity, etc then i would increase the protein and carbs to such a balance to suit.

So a typical day would be eggs for breakfast, nothing till lunchtime which would consist of a sandwich of lean meat with Hovis Lo-carb bread (2 slices) and slices of bell pepper. Nothing till dinner which would normally be a lean meat (chicken) with broccoli , carrots, runner beans or spinach or kale or whatever veg is too hand and one or two small potatoes or small roast potatoes. Followed by a serving of Skyr yoghurt or Arla protein "Quark" based dessert.

If i did require a snack then this would probably be something like a Ryvita or anything which was about 10g carb or less and lo-fat. Or some other protein based snack.

I found the protein keeps me fuller for longer periods and I don't experience the sugar rush, so I tend to only now eat the three meals a day. I did initially try the lo-carb and high fat diet but this was no good for me as this can be quite dangerous for Lipodystrophy, hence the lo-fat option. The medium carb level also gives me the sustained energy levels. If I was lo-carb i just would not function at all. I have tried this and suffered terribly in doing that, so having between 80g -125g per day seems to work for me.

As for the feet, yes I agree the memory foam insoles do work the best presently. I have had various orthotic insoles made which simply do not work and therefore resorted to wearing thick padded socks and memory insoles which are not the best option for Summer but I just have to manage the best I can for now until I find a better un-alternative.

All the best Si