I am 24y old, f., from Germany. I have a long and complex medical history. I have been diagnosed with chronic intestinal pseudoobstruction (diffuse intestinal motility disorder). CIP causes constipation and/or diarrhea, abdominal pain/spasms, nausea, vomiting , inability to eat, malabsorbtion and several other symptoms. It is generally a progressing disease with no cure and limited treatment options.
In my case it affects my small bowel, stomach and former colon and urinary bladder with varying degree of severity in these different parts. I have no colon any more and a permanent ileostomy. As I can’t empty my bladder by my own I have a suprapubical catheter as well. I also have a Hickman catheter which I use no longer due to my high risk of sepsis and significantly decreased caloric needs.
Within the last 6 months I have been experiencing new and fast progressing endocrine/metabolic issues which cannot be adequately explained by an adaption process or general effect secondary to a severe chronic disease and make me suffer from additional issues.
According to the current results and my symptoms (which occurred abruptly and which I have never experienced before), there might be an ongoing excessive hormone production that is either caused by the adrenal glands or by an ectopic source. At least, a significantly increased free cortisol and DHEA level were measured and a reversed diurnal rhythm was stated. Other hormones were not measured.
I found out that CIP is occasionally associated with neuro-endocrine tumors (sometimes also of the gastro-intestinal tract).
In several endoscopies of my stomach/duodenum slightly elevated reddish nodules were observed which could not be explained but may stem from diffuse lymphoid hyperplasia in retrospective. At the same time they found several significantly enlarged lymph nodesalong the ieal wall via sonography but I did not present with acute infection/inflammation that time. Thus, there was some confusion as to where the enlarged lymph nodes were coming from.
In the full thickness samples of the gut tissue including a very little part of the terminal ileum lymphoid hyperplasia was definitely noted but no other small bowel parts were examined.
In addition, white lesions were also found during endoscopies which once raised suspicion of intestinal lymphangiectasia. However, these endoscopies were not goal-oriented and done for other purposes (insertion of a nasal-duodenal tube etc) and so these incidental findings were not clarified or further confirmed since there was no medical interest in doing so. However, celiac disease was ruled out many years ago when I started to search for the cause of my severe and progressing digestive problems. Once, the reddish nodules in the duodenom/stomach were observed as well but there was no inflammation.
I also have low IgA-levels.
In addition, an enlarged spleen, dilated liver veins and a mild tricuspid and mitral insufficiency were stated. I also have Raynauds with no obvious reason. I also remember a MRT report telling about a mild thickening of ther term. ileum.
My medical history seems to repeat itself. Again, I am not taken seriously by the endocrinologists although they have mostly never dealt with such a case before and often do not have any knowledge about CIP and its possible connection to (neuro-)endocrine defects. They all dismiss me and say my symptoms are just due to an adaption process and do not require any clarification/further diagnosis. As I don’t have the typical outward appearance for someone with endocrine/metabolic disturbances and particularly with cortisol excess and as I am still considered as underweight despite significant improvement of weight and nutritional status due to parenteral feedings they just neglect my symptoms and forget that my underlying condition and intestinal malabsorbtion may blur the typical clinical picture of these conditions.
However, I have numerous new symptoms which did occur abruptly and simultaneously and which all can be related to (neuro-)endocrine tumors (of the gastro-intestinal tract) and their hormone excess.
There are several other new symptoms which could be attributed to excessive hormone production (of cortisol /serotonin) by an neuro-endocrine tumor including: decreased metabolism/very low caloric needs (parenteral nutrition has been stopped); extreme sleeping disorder; excessive thirst (at night as well) and increased urine output, easy dehydration; increasing muscle weakness and muscle atrophy despite of the best nutritional status I have ever had during the last 5 years thanks to parenteral nutrition; high frequency of(catheter-related) infections including sepsis/suppressed immune-system (4 infection with sepsis within 5 months); flank pain; abruptly changing of stomal output (previously little thick output, now massive and liquid output); altered body composition but steady weight (50kg, 170cm)despite little oral intake and intestinal malabsorbtion, redistribution of fat tissue and accumulation in the abdominal area, loss of muscle tissue; extensive fatigue; irreversible osteoponia; increased body hair, especially in the belly area and face; sudden attacks of sweating, especially while spleeping; episodes of intensive heart beating, especially wile going upstairs etc)
By the way there is no actual disease of the thyroid gland. Cortisol and DHEA levels are significantly elevated (up to 11 times than normal)
I have never experienced any of these symptoms before even with a weight of 37kg and in my 4 year history of marked underweight
Could these symptoms be related to a carcinoid tumor? I am at my wit's end and I can't afford more problems than I already have. I have not had any testing for carcinoid yet. I would be grateful for any comment.