February 8, 2004
Question from Saint Joseph, Missouri, USA:
My 10-year-old daughter has been recently diagnosed with hyperinsulinemia and hypoglycemia and is still undergoing more tests and consults for another possible underlying syndrome. She presents with some unusual symptoms, but mainly falls into two categories. She will, on many occasions, awaken early morning hours 4-6 a.m. with cyclic vomiting spells, vomiting sometimes as much as 10+ times occurring sometimes every 10-15 minutes and sometimes only one or two times; severity varies. She will continue to vomit (or wretch) until what we believe is her "insulin" leveling out. She even needed I.V.'s in the past to stop vomiting. She will be so acutely ill and even shock-like one moment, but as soon as the vomiting spells are over and everything "levels" out, she is perfectly fine. It has been suspected that she is hypoglycemic during these "spells". Other times, it is when she goes without eating for 3-4 hours before she will feel "it" coming on and, if she eats soon enough, it may stay off a severe episode or, on the other hand, if she eats too much "sugary foods" without protein, she will go into a spell. And most commonly, it is that she just wakes up early morning hours needing to vomit. She becomes very pale, sometimes will start with a bad headache, feeling of near-fainting or dizzy, lethargic, at times she will violently and seemingly involuntarily thrash her extremities about, and, ultimately, will throw up several times until the spell is over. This is very sporadic in nature, occurring many times in the same week, but sometimes she may go a few weeks without the severe spells, or just "mild" daily spells of "feeling funny (nauseated and dizzy)." Eating something will again stave off the "bad episodes". This just varies and is sporadic. Most recently, she became ill during her 3-hour glucose tolerance test while drinking her glucola. Nausea set in (I think it was just going to be one of those days of spells before the test anyway and because the glucola took 45 minutes to ingest rather than the usual 5 minutes needed for accurate labs; her test was canceled after drawing blood at the end of her drinking the Glucola. Within an hour of finishing the Glucola and, after she was dismissed, she fainted and had a violent vomiting episode still while at the hospital in the cafeteria (she had not yet eaten). Fortunately, a doctor happened to be in the cafe and was available. He said that she appeared to be going into shock, was rigid and sent her to ER. Unfortunately, no labs could be drawn during the episode and were normal in the ER as she was already becoming stable. However, the fasting lab before the glucola showed the insulin to be 19 (2-18 normal range) and a blood sugar of 86, but post-glucola (and within an hour preceding her event in the cafe) the insulin level was 250 with blood sugar being 196. Her daily blood sugars run from the 90's to 120's; she becomes symptomatic when her blood sugars drop to low 70 range or lower. Most recently, she had a CT scan of the pancreas which initially showed a subtle small round area of low attenuation in the pancreatic tail, but was shown to be volume-averaging artifact as it was not visualized on the sagittal or coronal reformations and there was an adjacent air-filled bowel loop in this area in question, presumably normal CT. Normal MRI of the Brain. Documented hyperinsulism and hypoglycemia. She has had recent weight gain. At 4'11", she weighs about 100 pounds, 20 of which she gained within the last year. Normal growth spurt or insulin factor? She follows a pretty good diet and is otherwise healthy, although she does get fatigued easily and sports wear her out. Her maternal grandfather has Type II on oral medication only. The endocrinologist is starting her on Glucophage. They are going to check a creatinine. level next along with neuro and GI consults. The general feeling is that something is going on, but what? I am getting the feeling that this cyclic vomiting pattern in conjunction with hyperinsulism and hypoglycemia is not a common finding and is somewhat baffling. One is directly affecting the other and ARE connected in some fashion. This has been going on for at least the last 3 years, possibly longer, but I could have mistaken her symptoms for other illnesses such as chronic strep throat or flu previous to this. It was when the tonsils came out about 3 years ago and the vomiting cycles continued that I suspected something else was going on as well. Other possible connections from early childhood: sometimes pale complexion, complaints of bad headaches from about age 4-5 on, as well as tingling legs bilaterally. She gets car sick easy. Also has developed occasional mucous diarrhea-like stools within the last 6 months not associated with any other illness. We are not sure if any of these are connected, but worth mentioning. She first was diagnosed with hypoglycemia at age 8; we adjusted her diet to no avail, most recently diagnosed with hyperinsulinism and pre-diabetes. The symptoms are becoming more pronounced within the last year. I would appreciate any of your thoughts on this subject, insight, or articles, etc. It is my understanding the hyperinsulism/hypoglycemia is not a disease by itself, but caused by other conditions; is this correct? Have you heard of others having similar symptoms or know any possible explanations for hyperinsulinism/hypoglycemia causing such cyclic vomiting episodes, etc.? Are there metabolic issues? Neuroendocrine? I am not comfortable with just starting the Glucophage to treat the symptoms and not find out the cause, if the cause can be found that is.
It’s always difficult to be constructive with a complicated story when you you can’t review the chart in detail, see the child, or talk to the other concerned doctors so I hope that you will forgive me if I play the devil’s advocate by saying that on the basis of the figures you give I do not think that your daughter is either Hyperinsulinemic or Hypoglycemic. Of course, if there is other more substantial evidence for this, then thinking of neuroendocrine tumors would be appropriate and so would looking for some of the inborn metabolic errors that can cause hypoglycemia; but not hyperinsulinemia. I don’t understand the role of Glucophage because with a BMI Body Mass Index) of only 20.1 she is only at the upper range of normal and hypoglycemia is hardly a characteristic of Type 2 Diabetes. I was however most impressed by the opening lines of your account and my own first guess would have been that your daughter represents a case of CVS (Cyclical Vomiting Syndrome), sometimes also called Abdominal Migraine and linked to Migraine itself, which is a fairly common disorder in her age group, but for which there is no very specific diagnostic test except perhaps in the response to drugs like rizatriptan. I would suggest asking a pediatric gastroenterologist to look at your daughter because they are the specialists with the best understanding of the condition.