Glybera is a new gene therapy developed for the treatment of lipoprotein lipase deficiency (LPLD). Lipoprotein lipase deficiency or LPLD is a very rare inherited condition that is associated with increased levels of chylomicrons and chylomicrons are small fat globules composed of protein and lipid (fat).
Symptoms of familial LPL deficiency usually begin in childhood and include abdominal pain, acute and recurrent inflammation of the pancreas (pancreatitis),skin lesions called eruptive cutaneous xanthoma and an enlargement of the liver and spleen (hepatosplenomegaly).
So in laymans terms what exactly is lipoprotein lipase deficiency or familial LPL deficiency? It's extremely high blood fat levels. I mean we are talkin HIGH. Not just a tad over 150 for triglycerides but maybe 2500 - 5000 or more. We are talkin HIGH blood fats. Anyway ...
I remember mentioning somewhere that I didn't even know high blood fats cause acute pancreatitis until I started writing about my experiences, joined a couple support groups (didn't know those existed either) and read a bunch of posts. I didn't know kids got this crap. I didn't know a lot of things except what had happened to me because in 1979 everone looked at me like a bull with a bastard calf and so when I finally got diagnosed I thought I was all by my lonesome. I figured if most doctors couldn't even recognize pancreatitis that it just wasn't to dang common and when I started doing research to find out how to survive the information was so hard to come by that I just figured it was a rare thing - I guess I was wrong. The GOOD news is ...
That those who suffer with pancreatitis due to high blood fats may be able to find relief via gene therapy and Glybera. I still don't know anything about Glybera. It's brand new, just on the market and it may not be available in the United States as yet. In fact according to PRNewswire rollout doesn't begin until sometime towards the second half of next year but ...
If you suffer with pancreatitis due to extremely high triglycerides you may want to poke your doc and get him/her pointed in the direction of Glybera to find out if it may be something you should seriously consider.
Now one important note: A very low fat diet, preferrably vegan, or a modified vegan that includes some egg whites, fish and skinless poultry (not fried) and everything cooked without oil may help as well. In fact John D Brunzell, MD from the University of Washington's School of medicine says "symptoms usually resolve with restriction of total dietary fat to 20 grams/day or less." Read here
IF you have truly tried a very low fat diet for at least 6 months to one year without success you may want to look into Glybera. If you get on Glybera let us all know how you do on it.
Do you ever experience pancreatic pain in the form of a mild stinging/pinching pain in the upper left side of your back?
ReplyDeleteThanks for the new posts,
John
Hey John, you're welcome man. Thanks for reading my blog. Nope can't say that I have ever had a stinging or pinching pain in or near the left side of my back.
DeleteHi there,
ReplyDeleteFirst I just wanted to say that your website has been so uplifting to me. I was wondering if I could briefly share my story with you and you could give me some insight? It would mean the world to me. I'm 26 yrs old and had my first acute (or what they thought was acute?) attack of pancreatitis 1 month ago. I only just now got out of the hospital yesterday. Here's the thing though... my lipase has been jumping from 150-1000 all month long which is why they kept me. It just wouldn't stay low despite being NPO on IV fluids. They even gave me TPN through a picc line and as soon as I'd eat anything, it would jump back up.
Anyway I found your website and told my fiancé, "This guy says to just do juice and avoid meats. Maybe thats why!" The hospital had me eating salmon every time my levels dropped even slightly. So for example, if I ate veggies one day and my lipase went down by 50 points they'd tell me to eat some fish and then it would jump up again by 100 or so. One step forward, three steps back...
I have never drank alcohol in my life. I haven't had any injuries as far as I know. The only risk factor I do have is I take steroids in the form of hydrocortisone because I have adrenal insufficiency. The doctors do not feel this is the cause because I take such a tiny replacement dose and have done so for 2 years. The only other thing that came up was I did take Zithromax, an antibiotic recently. Other than that, they have no idea why this is happening to me which is terrifying to be honest.
I'm in a lot of pain still, home from the hospital armed with Tramadol (which I do not like to take) and just doing liquids for now. Scared to move basically. I have plans to try to see a pancreatic specialist soon, but in the meantime I'm just laying here scared and worried to death.
When I left the hospital my lipase was around 450.. pain has stayed pretty consistent all month long. Just refusing to go away.
Any ideas? Thank you so much again for your site and all of its information.
Hi Kyli - thanks for the kind words about my pancreatitis blog info and thanks for reading it :-)
DeleteI can ONLY tell you what I would do IF it were me who was suffering from pancreatitis and it has been me - OFTEN - until I was able to finally start getting well, actually healing my pancreas. But ...
It took time. Trial and error, hit and miss but I learn slow lol anyway ...
IF I were sick right now I'd stop eating. I'd take 800mgs of Ibuprofen, 200 mgs of grape seed extract and 500 - 1000 mgs of curcumin extract and 1000 mgs of vitamin c. That means I would stop eating anything that was NOT water or the pills I just mentioned until the pain subsides and then I'd continue that course of action for at least 72 hours (3 days). NOTHING but the supplements, Ibuprofen if needed and water. People can live easily for 10 days to two weeks (unless there is some underlying condition like insulin dependent diabetes, screwed up metabolites etc) without food but water is vital.
I do NOT know enough about adreanal insufficiency so that may be why they fed you oily fish which is just asking for problems?
Anyway IF there is NO special diet for you, if you do NOT need to eat like an insulin dependent diabetic does then wait ...
IF I had addisons disease and I was NOT on a special diet I would not eat. I would do what I said above (Ibuprofen, supplements and water) for 3 days. That rests the pancreas. IF my pain and symptoms were gone and I felt hungry (I would NOT eat til I felt hungry) I would try some juice. Vegetable juice (low sodium V8). IF I had a juicer I'd make my own fresh vegetable juice. I'd drink some fresh juice and see how that worked. IF it went well I'd drink more. Small amounts. Maybe 4 - 6 ozs every hour or so all day long for the next 3 days. IF I experienced ANY symptoms it would be back to the water and supplements ONLY for another 3 days however ...
IF everything went fine with the juice and supplements then I'd try a small amount of rice or rice with some spinach. (NO FAT - NO milk, No cheese, NO MEAT, no oil, no butter, no margarine - NO FAT and nothing oily!) If the food went down, stayed down and I experienced no pain, no nausea after a few hours I might try more if hungry or juice.
White, unsweetend grapefruit juice - I would start drinking that as well. I'd actually do that BEFORE trying the food. I'd also be taking a good digestive enzyme supplement. In fact I use Now Foods Super Enzymes. I'd take those with every meal.
Hydrocortisone may indeed be the cause of the AP. Not saying it is, but Corticosteroids are known to cause AP but you have a situation where you are kinda screwed. Since your adreanal glands don't work properly you need the steroids. If it were me I might be looking for an alternative if one even exists but I don't know what I would dig up.
Hang in there Kyli. Never give up girl.
Kyli BEFORE drinking the grapefruit juice I would call my pharmacist and ask him/her if grapefruit juice would interact with the hydrocortisone or anything else you are taking. That is important. Grapefruit juice can be deadly in combination with some drugs - so I would call first!
DeleteHey Kyli I received your last comment but I did not approve it for publication cuz it had your email addy in it. I did respond to you and haven't heard back. I hope you are ok cuz you mentioned you were back in the hospital if I remember correctly.
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