I am a 35 year old female who was diagnosed with nesidioblastosis and had 80% of my pancreas removed, but I am still having low blood sugar, I’m nor working because of the hypoglycemia. I saw another doctor who suggested trying a medication called octreotide or removal of the remaining pancreas.

I wonder how my life would be with no pancreas. I understand I will be on insulin and digestive drugs for the rest of my life. Is it possible to live a normal life without a pancreas? Will I be able to work again? Is having this surgery going to shorten my life? Will I have type 1 or type 2 diabetes? Will I lose a lot of weight? I truly need some advice. Is there someone I can speak with has had there pancreas removed to know what there life is like?

Nesidioblastosis in an adult is very uncommon, but if this was confirmed by the histology of the pancreas I would ask your doctor to first of all consider using a calcium channel blocker like Procardia [nifedipine] to control the hyperinsulinism and hypoglycemia, and, if that doesn’t work to consider diazoxide as well. Sandostatin [octreotide] is often effective too, but it has to be given by injection.

There is no reason why pancreatectomy should shorten your life. With insulin and pancreatic enzyme replacement, you should be able to hold a job and lead an active existence. Strictly speaking, you won’t have either type 1 or type 2 diabetes but simply a diabetes due to the absence of beta cells.

Finally, I do think that you will need help from a dietitian in the weeks immediately following any surgery to make sure your nutrition is properly maintained.

DOB
Additional comments from Dr. David Schwartz:

Nesidioblastosis diagnosed at the age of 35 years seems extremely unlikely to me, as this is a disorder of embryologic development of the pancreas and therefore presents as a neonate. So, if I understand your question, you were diagnosed with this as an adult, so I think you may want a second opinion. If, in fact, you were diagnosed at with nesidioblastosis as an infant, and now are having problems again, 35 years later, you may still want a second opinion.

There are several drugs that can be used to try to inhibit insulin production; octreotide is one of them. It is given by injection several times daily, whereas other medications may be taken by mouth. I think pursuing those prior to complete pancreas removal (at which time you will develop diabetes and require daily insulin injections) would really be worth strong consideration.

Based on what you have described, I think I would ask for a referral to another academic medical center and adult endocrinologist to confirm the diagnosis. There are some hereditary conditions that can easily be screened for (e.g. with a blood ammonia level) that can mimic the hyperinsulinism of nesidioblastosis that actually are fairly simple to manage with an oral medication called diazoxide. Please let us know what you learn.

DS
Additional comments from Shirley Goodman, diabetes nurse specialist:

Treatment for nesidioblastosis can include medical therapy (with medication like diazoxide and octreotide) or surgical intervention (pancreatectomy). Medical therapy, even if successful initially, may fail over time and surgical intervention may become necessary.

While the intention of surgical intervention is to remove enough of the pancreas to eliminate the high insulin levels, often such a small amount of the pancreas is left in place that insulin production is insufficient or absent, and the other enzymes and hormones produced in the pancreas are also not being made at normal levels. Insulin and pancreatic enzymes need to be replaced when the production is insufficient or absent. The kind of diabetes that often occurs after pancreas removal is neither type 1 (autoimmune) nor type 2 diabetes, but the treatment is more consistent with type 1 diabetes, since the problem is insulin deficiency. Since glucagon production is impaired also, hypoglycemia is of even higher concern.

SG