i thought it would be helpful if i would share about my specific physiological experience with what i believe to be a possibly inherited genetic disorder; resembling hfi or some mutated variant of hfi.
i'm going to use a list format, at least initially. i may edited it into more of a prose format later, or not.
1) i had a sense of a pattern of the symptoms by 3rd grade.
2) the symptoms consisted of feeling prematurely hungry, as i would have eaten just 2-3 hours prior.
this hunger would be accompanied by a weakness; as if i hadn't eaten in days, feeling jittery, with increased sweating-clamminess, & a slight cramping in the pit of my abdomen. (i later learned that these were hypoglycemic episodes.) during this time & throughout college, these particular episodes were not accompanied with any gastrointestinal discomfort; which i believe is the reason that i hadn't developed an aversion to sweets or carbs.
3) at this point, i knew i just needed to get something to eat, & i don't recall having nausea or bloating.
4) i also suffered from severe motion sickness; which made me feel like i had stomach flu, mixed with a hangover. focusing my eyes while moving in any vehicle (plane, boat, train, bus, car ) triggered the attacks. reading in a car was out of the question, as was sitting in the back seat of cars & buses. (interestingly, as i have been eliminating fructose/carbs from my diet, the motion sickness is not as severe or constant under those same conditions. there are times when i can actually focus my eyes and read a map or text in a car or on a bus.
5) i also suffered from migraine headaches from a very early age to about the third grade. i find this to be interesting; as the third grade was when i remember noting a pattern for the hfi episodes. i had to be picked up from school on several occasions, up to the third grade. i remember being taking to a doctor, who i believe did some cranial xrays.
6a) i don't recall the hypoglycemic episodes being a major hindrance to my hyper active schedule. from high school, throughout college, i was super active in my academics & intercollegiate athletics. i suspect that exercise & the easy access to food may have been the reason. there is still no concern or connection to sugar at this point. (though i'd lost 2-3 molars to cavities during this time.) i believe i had eventually become accustom to the initial symptoms and would simply get something to eat & that would end the episode.
6b) it is interesting to note in retrospect, that as a young adult, post college, i began fasting for religious reasons. i do recall that during these periods of typically 3 days, & on occasions 7 days, i felt the best; that is, no hypoglycemic episodes. post college & continually, as the episodes increased in severity & more frequently (about 3 hours after eating), the difference with how i felt, when i ate and didn't eat was even greater. i felt much better when i didn't eat, & is probably why i began to miss meals on the regular, when i was busy doing something.
7a) it seems that post college 1984 & prior to my second pregnancy 1994, i was having more severe/debilitating hypoglycemic episodes. these would sometimes progress faster than I could realize that i was in a hypoglycemic state versus just hungry. it would take about 5-7 minutes from the onset of the hunger in the pit of my stomach to a full blown attack that would render me non ambulatory. at some point (1994-1997/33-36 years of age) along with these episodes, i began having other symptoms; profuse nigh sweats, i would be awaken by numbness (progressing over time) of hands, feet, then arms and legs. i'd began having bloating and severe cramping-that i presumed to be menstrual, though i hadn't had such ever before. these cramps lasted about 4 hours, after which i would need to go to the bathroom, pass gas, & be relieved of the cramps. (i later discovered that these particular cramps were induced by a sensitivity to gluten). at some point, someone in the family, was discussing diabetes; i believe that's when i wondered if i was having diabetic episodes.
7b) my first pregnancy was a miscarriage. my second pregnancy, about a year later was a successful birth. during the prenatal stage of that pregnancy i was tested for diabetes; given that sugary drink. i wait the prescribed time, i didn't show any signs of a diabetic response; high blood sugar and i did not feel any adverse effect within their prescribed time limit; so i left the office. sometime later, about 3 hours, i was still out & about driving and i felt an episode coming on. i remember feeling a little bit of panic; i knew how severe the episodes could get and i wouldn't be able to drive. i remember going to the nearest fast food place; a white-castle, & got something to eat. i now suspect that the whole ordeal of being pregnant, 2xs, might have triggered/contributed to the increased intensity of all symptoms, via the increased progression of the underlining condition.
8) i officially retired from teaching around the year 2000. i actually stopped working at the end of 1997. in the autumn of 1997, i had a fall playing badminton. as i did go to the emergency room a few days later & the xrays were negative, at some point, the situation turned out to be quite critical. as a result, i was in intensive care for almost 2 weeks, had to have back to back surgeries and almost lost the fight. it seems that after this ordeal, the symptoms also increased in number and severity. as our children got older, there were several occasions when i was non-ambulatory & the children had to get me something to eat. perhaps the fall and hospital experience is another potential cause of the symptoms and the disorder becoming more severe and aggressive in their manifestation.
9) the hypoglycemic symptoms at this point were debilitating. i get that premature hunger; which at times resembled typical hunger. sometimes if was busy, i'd get caught unaware that i was in the beginning of a hypoglycemic episode & would put off eating. when the tightness-cramping in the pit of my stomach began i knew i was in the midst of an episode. these episodes would progress too quickly, and within minutes, i would be sweating profusely , shaking, too weak to walk & the cramping in the stomach would feel like someone was twisting my insides with a vice grip. at these times, i was fortunate to have my children at home ( i home schooled) & be old enough to help me out.
10) i strongly suspected some kind of connection to food, as i became aware of all symptoms increasing after eating out and after eating something sweet in the morning; i.e, pancakes & syrup. i initiated eating organic, whole foods, healthy super foods; honey, garlic, organic fruits and vegetables and was working towards becoming a vegetarian; decreasing animal protein and animal fat. ironically, this attempt to eat healthier is another potential candidate for causing an increased in the rate of progression of the underlining condition; increased symptoms and severity.
11) by the year 2009 i had unprecedented (for me) hypertension; 147/93. prior to this, it was constantly 110/80. and come to think about it, that was high for my slight of build, muscle to fat ratio & athletic activities. the episodes and the other symptoms were more frequent and debilitating. i couldn't eat out or eat at home without getting sick. i was getting dizzy and having headaches. I went to a natural-path doctor, who used elimination diets. (i also began seeing a therapist). the natural path doctor helped me to understand that my body was having an adverse reaction to gluten and almost accidentally, helped me to understand that the diabetic like episodes that i had been having for virtually all of my life, were not diabetic. that is, they were not caused by insufficient insulin production or insulin insensitivity. i was in fact having low blood sugar episodes, not high blood sugar episodes. though the symptoms for high blood sugar and low blood sugar are quite similar, apparently, no medical professional was ever concerned or spoke to me about having low blood sugar. she took my blood sugar (meter or serum, i can't recall) and i believe it was in the 60s.
12a) as a result of the elimination diets, i also worked on decreasing gluten in my diet. i first expected that i only had a sensitivity to gluten, therefore i eliminated some major gluten items. as i continued to eliminate items, i was still having the symptoms of a gluten reaction. I couldn't eat foods labeled "non-gluten food". this is when i learned that labeled "non-gluten" doesn't mean absolutely non-gluten. i then had to choose my foods as if i had celiac disease, then the gluten symptoms subsided. at some point, i wondered if the probably with gluten was due to the fructose intolerance going undetected for so long. i wondered if i could tolerate gluten if i eliminated the fructose. it became a moot point, because i eventually realized i couldn't tolerate any plant derived products.
12b) i was also cured from "my addiction" to salt. i now prefer the natural taste of my food over a very salty taste. i began to eliminate added salt from my diet, since i had become hypertension sensitive to salt. at least, that is what i was told by a physician. this proved harmful and dangerous; i began having atypical severe headaches. these severe head pressure-squeezing headaches would begin at the base of my skull; at the occipital lobe, travel up to the parietal lobe, then round to encase the temporal lobes and end its captivity with the siege of the entire frontal lobe-thought my head was going to crack. it is a miracle that i didn't die from the apparent transient stroke episode i suffered in 12/2012 (bp was 204/114). after some research, i realized these were acute encephalitis headaches due to low sodium and an impaired endocrine-electrolyte system. i believe the fructose/carb substrate poison in my body, increased the sodium uptake causing severe hyper vascular constriction, even though my sodium intake was too low and too low in my blood as compared to the other electrolytes; including water. at the same time, i felt thirsty all of the time and my mouth was dry. i was drinking like 8oz of water every hour; and all at once. there was too much water in the blood, as compared to salt. that extra fluid was being sent to body cells-tissue, including brain cells-tissue. in i was poisoning my body with too much water, causing acute encephalitis. i had to learn how to monitor hydration when i was contaminated to avoid adding to the electrolyte regulation problems caused by the poison substrate roaming/flowing throughout in my body.
12c) i began researching the causes of low blood sugar. this research led me to hereditary fructose intolerance, which eventually led me to research mitochondria disease. during my research of hfi, i came across this new disease/genetic disorder called mitochondria genetic disorder. coincidentally, when i went to go see my g.i. doctor, he also mentioned my mitochondria health, though at that time, i had no idea why? so i decided to look it up. when i'd looked it up, it was astounding to me that the list of symptoms were quite similar to what i was experiencing. as i continued to eliminate all known sources of fructose/carbs, i would have brief periods of feeling better; not exhausted & no muscle fatigue. i actually would be motivated to exercise again, and could do so with out much pain afterward. soon after though, the symptoms would return. this could only mean that i was still being contaminated. i was taking clonidine as prescribed by the emergency room doctor for the 12/2012 visit for severe hypertension. i later added a magnesium oxide supplement to help with bowel movement and to help relax vasoconstriction in the heart. both seem to help initially as intended, but i eventually suspected them and looked up their ingredients. both had plant derived products; some form of carbs. when i stopped their use, i would say that by my next meal i was feeling notably better. one of the drugs had a lactose derivative that was causing substantial allergic reactions on top of all the other symptoms i had to deal with. this was a major victory. the symptoms, returned.
12d) i was eating pork rinds which i thought were safe, because i could eat as much pork and pork fat as i desired. i realized the packaged pork rinds/skin must be crossed contaminated from the factory process where i suspect other carb products are processed. so now, i'm only eating pork and canned salmon, and drinking mineral water, 4-5 times daily, every day. i have periods of no symptoms, yea! unfortunately, another condition arises, due to a certain nutrient deficiency which side tracks my improvement. this is discussed later, in section 14.
12e) in short, from the year of 2009 to the point when i had the transient stroke event in 12/2012 (that got me back into the conventional medical system, which i had discontinued utilizing after my 1997 medical experience) till now, i have researched quite a bit of health science and medical science material. i've become acquainted with several physicians who are actually health advocates. they have come to be health activist who effectively understand the essential approach of integrative health approaches. they have used their websites to inform regular folk of the real science of health and illness and why the current system of medical services for profit is a comprehensive failure in providing real health and prevention of sickness and disease to the public at large. i've read medical abstracts, a book excerpt on genetics, medical articles on the 3 aldolases/enzymes, hfi diagnostic genetic testing procedures, and have visited numerous support groups of hfi sufferers on line. i have also been reintroduced to the kreb cycle/electron transport chain system/the oxidative phosphorylation process, (of the double membrane of the atp producing mighty mitochondria) that i'd first learned about in college.
13a) it is my strong opinion that the current clinical parameters for hfi have been prematurely set and do not sufficiently reflect true science or patients' experiences. my g.i. first statement to me was..."i don't think you have hereditary fructose intolerance". in addition, all of the doctors i saw prior to that and many afterward, had no functional knowledge of the scientific based relationship fructose/carbs had to the symptoms i was having. once i had correctly made the connection to fructose, and tried to communicate this to the emergency room doctor, the colonoscopy doctor, & to the triage doctor of the ambulatory clinic i went to, none of them could hear me. even the physicians who claimed to know something about hfi, kept telling me i didn't have it. all of the above just kept prescribing drugs that contained sugars/carbs and invariably triggered severe adverse reactions. the colonoscopy physician and triage physician, both prescribed drugs that had large amounts of sugar; and this, after i was emphatic that i had severe problems with sugar. on both occasions, after taking the drugs, believing they had taking my concerns seriously, i had severe reactions. i looked up the ingredients for the cocktail of drugs the triage doctor gave me, to "kill the h.pylori bacterium" and sure enough, clear as day, sugar. one of the drugs instructed patients to inform doctor, if you have problems with sugar. during this time the hypoglycemia episodes were most prevalent compared to the other symptoms. at some point though (like during with the colonoscopy) i stopped having the pre-hypoglycemic warning symptoms; shakiness and profuse sweating and stomach cramping; my blood sugar would simply plummet. during the prep-solution phase, i had a home meter readings of 38. it had gone down to 23 when the tech took it at the colonoscopy doctor's office). i showed no apparent physical or cognitive distress. i presume, that my body had had so many hypoglycemic events, that my endocrine system had become desensitized & no longer initiated the pre-hypoglycemic warning response. in addition, i believe my body had begun to utilize the animal fat and protein as my primary fuel sources. perhaps i was in a healthy state of ketosis, which kept my liver, brain and other tissues supplied with sufficient glucose and glycogen.
13b) i did see an endocrinologist who seemed empathetic. i also eventually was allowed to see a pediatric geneticist, who eventually placed the order for the genetic diagnostic hfi test. in retrospect i did recall him making a statement that such diagnoses could be made by clinical analysis; patient symptoms-diet, labs, & medical history. it wasn't until much later when i furthered processed this experience, that i realized that perhaps the geneticist was suggesting that he could diagnose me without the genetic testing. unfortunately, i was and continue to suffer from a severe form of anxiety/ptsd, associated with my medical visits. after returning to the conventional medical service in 12/2012, i found that going to my medical appointments, was more arduous than i had anticipated. while in the office, my blood pressure increased significantly and at times i would become so agitated that i couldn't speak or think clearly. my sensory sensitive would go into hyper-drive and i would only be able to process bits and pieces of verbal conversation. it could be days or weeks later before the info got properly processed in my mind. i believe this is what happened when the geneticist was suggesting that i didn't need the genetic test to get officially diagnosed. which is what i was intensely advocating for, in order to receive access to essential financial and other health support for my disabling conditions.
13c) my hereditary fructose/carb intolerance has had a progressive nature. i was seemingly able to metabolized fructose-carbs at birth and through my childhood to some degree. even up to college, the hypoglycemic episodes didn't disable me or cause any apparent gastrointestinal illnesses. it seems that at some point after college, i began to aggressively lose the ability to effectively metabolize fructose/carbs. this condition has progressed to the point where i can no longer tolerate any plant products. i wonder then, if this is in fact the case and based on the research from the boston university (dr tolan) aldolase lab website, is it likely or even possible for all 3 aldolases/metabolic enzymes to have been depleted or have become dysfunctional over time? perhaps the 3% of people who can't be diagnosed by the diagnostic genetic test for hfi that was done on my blood sample, are also of this type or some other mutative variant type?. my condition could be "mimicking" hfi, (a type of mitochondria genetic disorder yet to be discovered/determined). a progressive degenerative genetic disorder that may attack different aspects of an individuals' energy physiology. i have to wonder, since my maternal grandmother was also stricken in her early 40s and progressively lost the use of her musculature and eventually died from pneumonia after suffering 7 years from that unabated condition.
(13d) in either case, the electron transport chain cycle is ultimately affected. whether the direct cause is a specific mitochondria genetic disorder or caused by the bodies inability to produce healthy metabolic enzymes in order to properly metabolize a carb or carbs to the point of producing substrate toxins in the body that in turn hinder/retard the electron transport chain process, causing an insufficient production of atps; the result is in effect, acute mitochondria disease. the consequences subsequently, manifest as a cascading dysfunction of every cell of the body (excluding red blood cells-tissue), of every other tissue of the body, of every system of the body; producing a comprehensive physiological symptoms list. as this seems to be the case for those of us who suffer the consequences of hfi & who never seem to get better, it would seem that all 3 aldolase/metabolic enzymes; a, b & c, are somehow affected, as they are known to be organ-system specific.
13e) the systems that i believe are damaged when i ingest even micro traces of fructose/carbs are:
-my metabolic processes to properly breakdown/metabolize fructose/carbs, to the end of producing a substrate that can not be properly metabolized thus becoming an accumulated poison(s) in my body in every cell (except the red blood cells) of every other tissue of every system. this poison then disrupts my mitochondria of every effected cell...from functioning properly...
-my mitochondria; my electron transport chain system, which regulates atp (adenosine triphosphate) production, thus regulates the production and use of energy to run all physiological systems.
the dysfunction of my mitochondria resulted in the immediate dysfunction of...
-my endocrine system, which regulates my blood, cells, brain and liver sugar levels,
-my sodium uptake thus vascular constriction processes causing hypertension,
-my electrolyte balance affecting internal and external cellular hydration, muscular contraction, temperature, blood pressure and produced severe pain from acute encephalitis etc.
-my immune system causing hyper reaction to other foods and thus causing food and environmental allergies and other foods intolerance; i.e. lactose, gluten & perhaps all carbs, etc.
-the most benign but increasingly not, affect of the fructose-carbs intolerance was the lab work indications of abnormal liver function tests, increased alpha-fetoprotein levels and a lowering gfr (glomerular filtration rate), indicating some possible kidney dysfunction. mri and ultrasounds of my liver were negative, but the lab work fluctuated as my trial & error elimination process proceeded. there is a positive correlation between decreasing ingestion of plant products & lowering-normalizing function of liver enzymatic levels.
13f) since the number of symptoms along with the severity of symptoms, increased over time during the prolonged accumulation of the substrate poison, as i didn't make the connection to sugars/carbs until my age in my late 40s, that seems to indicate a progression of dysfunction of once functional enzymes; or a progressive depletion of functioning enzymes. also, as smaller and smaller amounts of fructose/carb ingestion still produced severe symptoms, though a few less symptoms, this also seems to indicate that an on going depletion of the needed functional enzymes was occurring.
13g) in addition to the direct effect of the poison on my mitochondria, causing grave dysfunction of virtually every system, (acute mitochondrial disease) no matter the amount of contamination, the course of contamination, once i'd stopped ingesting fructose-carbs, seemed to take about10 days for all symptoms to stop/to get the poison(s) out of all of my systems (including excretion via the skin, causing itching, scalp excretion causing a caky-itchy residue on the scalp, some histamine/allergic reactions; running nose, sneezing, increased watering eyes, & sore throat). at the end of about ten days, i miraculously felt significantly better or at least i use to.
13h) the symptoms are overlapping but do have some sequential nature from increasing hypertension, light-headed-dizziness and hypoglycemic indicators, to night & day numbness-increased circulatory problems, lucent dreaming, to hot and cold flashes-accompanied with painful exhaustion episodes, increasing general fatigue, muscular-skeletal fatigue, exercise intolerance due to debilitating muscular-skeletal pain and exhaustion, bloating, diarrhea, my hair becomes very weak-brittle like-thin and falls out, i develop dry eyes, mouth nose and vaginal cavities, pervasive calcium like build up on my teeth, particular the bottom incisors...i've been trying to find an analogy to best describe the overall experience of being in acute mitochondria dysfunction. as i have observed the feeble elderly, that is what comes to mind. i am forced to move (due to muscular-skeletal pain, fuel exhaustion, difficulty breathing, heart palpitations, inadequate blood circulation, a chaotic endocrine system) to seemingly feel & move as a feeble elderly person .
14a) as i believe i have found a sustainable diet of ironically, surprisingly and fortunately conventional pork, canned red sockeye salmon (absent of struvite contamination and including the bones, skin and fat but removing the spinal cord, blood vessel and intestine) and a certain mineral water (san pellegrino (as it has a conducive mineral content to my specific mineral needs) i have experienced a remarkable improvement. all systems seem to improve; including cognition & psychological issues. the improvement is limited to the type of fuel i am able to burn. since i only eat animal fat & animal protein, i am only burning the slower fuel; fat as my primary source of energy. fat does not burn quickly as do carbs, so i literally have to move slower to wait for the energy to "kick in". in other words, no more sprinting for me.
14b) unfortunately, this very encouraging improvement stopped. i have begun to experience mild-moderate symptoms, different from what i had associated with the fructose/carb ingestion. they are stabbing sustained muscle pain, increased neuropathy sensations, some slight bleeding of soft tissue cavities, fatigue, & chills sensations in extremities & a brownish red ting vaginal discharge. throughout my recent medical experiences, my cbc (complete blood counts-lab work) were done numerous of times. they indicated that as i was eliminating fructose/carbs from my diet, my liver function tests and gfr (glomerular. filtration rate) were improving but my platelets were decreasing. the essential trace vitamin b9-folate has become deficient from my diet. as a result, my platelet count and thus clotting capabilities have decreased. other system are being adversely affected due to the deficiency in folate/low platelet count. as i have tried to remedy this problem with meats that have high levels of folate, i.e. beef liver, such attempts have not been successful. these meats happen to also contain levels of fructose/carbs that i am unable to tolerate.
14c) since i am restricted to eating the same meal/diet every day for every meal, i can't afford to have carb-toxins accumulating in and among my physiological systems-it's simply too debilitating. i tried a supplement that supposedly had no ingredients (active or inactive) that would breakdown into digestible carbs. it claim to provide 250 % of daily recommended folic acid, for a 2000 calorie diet. i decided to take it every other day. by the 10th day, thus 5 tablets, i was having the reoccurring fructose/carb symptoms. i called the company, and it was discovered that wood pulp and palm oil were used as sources for cellulose and stearic acid. i have long known that i am unable to tolerate any oils from plants; olive, corn, vegetable and certainly not manufactured poly unsaturated fats. i seemingly cannot tolerate any product derived from plants. note: i did receive an infusion of dextrose/glucose when i had a colonoscopy. the prep solution for the colonoscopy was laced with sugar, even though i had told the physician i had a real problem with with sugar; probably hfi. after taking the solution, my blood sugar per my home meter was 38. it was taken at the physicians office, and it had dropped to 23. they got it up with an infusion of dextrose. that said, i don't know if i still had the other subsequent symptoms after that...so perhaps, i only can't eat plant foods or plant derived products because they all contain trace amounts of fructose/carbs that have to go through the metabolic process, utilizing enzymes that i longer have or that are dysfunctional?
14d) i couldn't tolerate the pain that seemed to be getting worst or risk becoming a spontaneous bleeder. since i knew it took about 10 days for the fructose/carb toxins to get out of all of my systems, i thought i would try taking the folate pill once every 11 days. i wanted to see how i might balance the lower platelet count-increased risk of bleeding with decreased fructose/carb intoxication and its consequences. could i take the folic acid, once every 11 days to produce enough platelets but to prevent debilitating accumulation of the fructose/carb toxins in my body? since the low platelet symptoms seem to return within days of stopping the folate (about 4 days) i decided to try 1/2 pill of the foic acid every 4th day. so far this is proving to be manageable. i did request that my pcp order regular cbc labs to monitor all levels but particularly liver function and kidney indicators and as well, foate and platelet levels.
(15) it would be quite beneficial to all, i think, if clinicians had significantly more education with regards to the current and actual medical problems of their patients. common sense and science, agree that nutrition, diet and lifestyle are the pimary influences for our health status. science is strongly demonstrating that our lack of healthy activity and our addictions to sweets, salt, and processed carbs are killing people prematurely. carb intolerance is a growing health problem as is mitochondria dysfunction/disease and genetic disorders. with the reality of the internet & greater access to support groups & medical information, physicians should ask, "why do you think you have thus & such, instead of accusingly implying and assuming that patients are ignorant & are hypohondriacal. in effect, dismissing their complaints by stating "i don't think you have thus and such".
(16) in summary, i do have some form of or a condition that mimics hfi. my hfi has been progressive since before the third grade. my hfi may be caused by another genetic disorder that may be linked to my maternal grandmother's premature death. the progressive nature of my hfi experience and the fact that i didn't notice any gastrointestinal problems when i was young & therefore did not develop an aversion to sweets, suggest that i had healthy gastrointestinal enzymes at that time. i also had healthy amounts of muscle enzymes or else i would not have been able to be a 3 sport athlete in college. my body's apparent lost of its ability to properly metabolize fructose/carbs has seemingly resulted in acute mitochondria disease. in the presence of fructose/carbs, my mitochondria is no longer able to produce sufficient atps to fuel my physiological systems, including the burning of animal fat & animal protein; every system apparently becomes dysfunctional (except the red blood cells) in addition, my body's desperate attempt to rid itself of the poisons, causes in allergic reactions & residue build up. the toxin also aggravates; makes worst any other health issues i may also have. my hfi was not able to be diagnosed by the diagnostic genetic test that was done for me. all of my physiological systems are affected by my condition (not being able to properly metabolize ingested fructose/carbs). apparently, this includes oils, acids, & wood pulp from plants. perhaps, suggesting some dysfunction of all 3 aldolase/enzymes. as there seems to be a range of severity among hfi sufferers, perhaps the amount of dysfunctional aldolase in each organ-system is indicated by the particular symptoms, onset, and severity per individual sufferer? perhaps all hfi sufferers won't manifest an aversion to sweets at an early age, perhaps the hfi disease is not static but dynamic-progressive in some hfi sufferers. perhaps, adults can have an onset of the disease with no prior history of gastrointestinal problems? consequently, it seems the clinical parameters for hfi should be reviewed and updated to reflect actual science and patient experiences.