In an earlier post, I described my experience on alternative diagnosis for patients who have pheo symptoms but without pheo. Most patients cope with their conditions very well and go on with their lives. A small number of patients would see multiple physicians, get numerous tests and imaging studies, and try all kinds of medications, herbs, or behavioral therapies, just to get a definitive diagnosis and to get back to their "perfect" health. As a pheo specialist, I am often the doctor who tells them not only they don't have pheo but a clear diagnosis cannot be established. I explain that further diagnostic work-up is unlikely to yield a definitive diagnosis. I then discuss the skills of coping with a frustrating disease.
First of all, these patients do have a disease. And the disease is not their fault. They just simply have it and they have not done anything to make the disease afflict on them.
Second, medicine has its limits. In spite of the ever-growing progress in medicine, there are many things we don't know in medicine. Sometimes, a definitive diagnosis cannot be reached no matter how hard one may try. We have to wait for the disease to fully reveal itself over time.
Third, look at the brighter side. Although we don't know the diagnosis, we do know that it is not a bad one. It is not cancer, not heart disease, not a disease that will cost a limb or organ, and not deadly if the symptoms have been going on for many years.
Lastly, the most important issue is coping with the disease. What is the purpose of perfect health? What are the important things in life? Can one pursue the goals in life with the disease? What limits one from doing the desired things? The best approach is to focus on functionality rather than on perfect health. If one can do the things one wants to do, that's not too bad even with the disturbing symptoms mimicking pheo. I like to use a car metaphor. Most of us want to have fancy expensive cars but most of us cannot get those cars. If we keep lamenting on the lack of those cars, we lose the purpose of life. Those of us without fancy cars will get a car we can afford with the functions that we think are the most important. Then we drive the car to do the things we want to do. Simply owning a fancy car won't necessarily drive us somewhere.
Dr. Pheo
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Wow-that's tough to read-I got that same lecture from my daughter's neurologist. I suppose If my search for answers were for my 44 year old body I could be okay with it, my search for answers is for my 12 year old. I just can't be okay with her living with medications and almost daily pain and nausea. Being told hypertension in children isn't that unusual and hey she's anxious headaches are expected and let's see in yet another six months if she grows or continues to drop of the growth chart. So here's my story-cyclic vomiting diagnosed at 6, pediatric migrains at 7, sustained hypertension with blunted nocturnal dip diagnosed at 9, trials on 3 or four med have her bp only slightly improved-I begged for a new 24 hour ambulatory monitor when the doc said her BP was good but she was constantly nauseated and daily throbbing headaches were beginning to plague her. Results 77% hypertensive day, blunted nocturnal dip Still! 2 years ago normal plasma and urine catacolamines/ metanephrines. Around the same time we hear she is now under 3% for height and has been at the same weight for 3 years. Now I'm like that Imipramine for her headaches isn't helping I want her off it so we can see what what. The Endrocronologist test for Turner's neg-tells me well let see what happens in 6 months. The neurologist says let's add a 3rd PB med. I say time for 2nd opinion! New Endo says she needs a little synthroid and hey let's get GI in and Genetics and let's repeat the plasma free-well we've seen everybody every body has a test to run finally we get to the plasma free results. plasma limit 202 result 212, Free metanephrine limit 79 result 69, Free normetanephrine 148 result 179. Nephrologist says normal! New Endo says time for 24 hour urine, Genetics says time to add diagnostic referral. So tomorrow we stay home and pee in a jug (not literally)-and hey so I didn't give birth to a Rolls Royce but I'd like her to feel good! Have you heard of Imipramine interferring with result (false negative) I've read it in one place. I'm afraid to even say it to the doctors-I feel like her docs think I'm nuts.
ReplyDeleteDear Anne,
ReplyDeleteI am a doctor for adults so all the posts are only applicable to adult patients.
Imipramine mostly causes false POSITIVE results.
Dr. Pheo
Dr. P.
ReplyDeleteThanx for responding - your info is as always most helpful. Hope you had a wonderful vacation. I did, despite not feeling well - hey, you only have one life to live! given dearth of great expert advice, I did go through with octreo (unexpectedly, much discomfort in thyroid area afterwards for several days- have nodules, biopsied to not be cancerous). Was informed by PA through email today that results are 'anomalous' tho not too 'scary'. S/t seems to have lit up a bit in llq (vague info given by email). I will post again when I know more. However, I understand what you say about other neuroendocrine tumors. Sx are definitely getting worse, at a faster pace. Do you, in your practice, also deal with these? Or, possibly, know s/o in nyc who does? I am an educated person in an allied profession in nyc (without a whole lot of money!), and it has been quite astonishing to me the dearth of knowledge even here regarding this area in most clinicians, even very good ones. Thus my multiple posts to you. Thnx again, Edi.
p.s. I find your above post quite interesting and important. You seem to have a grasp on the emotional oddessy that a pt. must go through. Learning how to negotiate this is an important aspect of getting care. Can't say I've been great at it, but am learning.
Dr. P. - Received email from dr.s office (a gi) re octreo scan, stating that there was increased uptake on 24 hour spec ct, within the rectosigmoid (where i have had ongoing pain, in fact this is what started me on this oddessy 3 yrs ago now). They recommended scheduling a colonoscopy. I insisted on receiving the report, which stated that the increased uptake was most likely due to bowel content. No uptake noted elsewhere. I am not inclined to have 3rd colonoscopy within three years. I have an appt. with the doctor on Monday, as well as an endocrinologist later that week. Will wait to make a decision. In the meantime, I am getting sicker, at a more alarming pace, now. It would be great to consult with a doctor who specializes in neuroendocrine tumors to r/o, however, this seems to be a discipline that does not take insurance, and it is too hypothetical to spend big bucks at this point. However, my current Q is not related to this. I recently finally bought a smart phone. I love it and find it very convenient. Is there a way to follow your blog on an Iphone?
ReplyDeleteDr. P. - Another Q (preparing for dr. visit - sorry to bother you so much!) My hx. involves sudden onset of llq and ulq pain, quite localized in each area, and diarrhea that never stopped, leading to weight loss. I do drink and smoke, and fats give me worse diarrhea (have an extemely lo fat diet, which I hate. Every time I err, bad consequences.) Actually had mri/mrcp over a year ago, when not so symptomatic, which was unremarkable. However, ulq pain is now main focus for me(radiating to back), quite recently, and more severe than ever before. Along with inreased bp and pulse. Is there a differential between pancreatitis and pheo/endocrine tumor? They both seem to involve labile bp and pulse, diarrhea - but perhaps sx picture is quite different?
ReplyDeleteDear Eddie,
ReplyDeleteFirst of all, I don't know if you can access this blog by iphone. I am quite unsavvy about gadgets.
The octreotide scan result can mean different things. Most likely it is bowel activity, which can be resolved by a delayed imaging (eg at 7 days). It is also possible that you may have some inflammation in the bowel (quite common).
Regarding the pain, you may want to see if you have chronic pancreatitis.
Dr. Pheo
I have what may be a stupid question. Why are pheos/paras NOT considered cancerous until they 'act like cancer?' I know they are slow growing and I know the likelihood of them 'acting malignant' is relatively small.... But if they all have the 'potential...' to act malignant and if the pathology of a 'benign' pheo/para can't be distinguinshed from a 'malignant' one until it pops up as a met. in a non-chromaffin part of the body, then why not call them that?
ReplyDeleteIt just seems like a pheo/para found on one site or multi-focally but not as mets. would be like a 'stage 1' cancer under the traditional rules.... And it seems like the disease would be taken more seriously if the potential nature of these tumors were more readily noted.
I guess I wouldn't want to unnecessarily alarm people. Maybe I am just jaded by my own concern and my (local) physicians' lack of concern about potential developments down the line. I have a Carotid Paraganglioma.
It's entirely possible that I don't have a good understanding of the nature of these tumors or their malignant potential, in which case, I'd still be interested in your response to help me understand better.
One more question more directly relating to my case:
ReplyDeleteAs I said I have a carotid paraganglioma. One of the things that alarmed me as I was researching my condition and pheos and paras early on was that my Mother's Brother died from what was diagnosed as an 'aldosterone producing renal cell carcinoma' located on his adrenal gland and kidney (diagnosed after he'd had a stroke and which took his life only months later).
I'm 29 (and pregnant incidentally) and I know the genetic mutations that would be most suspected given my age and the location of my tumor would likely be the SDH ones, BUT I'm still wondering if there could be any sort of correlation between my uncle's condition and my own para.
Dear Val,
ReplyDeleteYour question on the malignancy part is actually very important. The reasons for not calling pheo malignant in general are actually complicated. Most of them are practical ones. As most patients do not have recurrence or metastasis, it is too much a burden to call a pheo patient having an adrenal cancer (which is adrenocortical carcinoma, a very deadly cancer, in most people's minds). Anothe reason is that patients tend to receive ineffective and harmful treatment such as chemotherapy and radiation if some doctors think they have adrenal cancer. So we deliberately call it "pheo" to avoid the confusion.
Regarding your uncle's disease. I myself am not aware of any renal cell carcinoma producing aldosterone. Renal cell carcinoma may produce renin which stimulates aldosterone production. He can also have an aldosterone-producing adrenal tumor (or cancer) and a renal cell carcinoma. If he indeed has renal cell carcinoma, von Hippel-Lindau disease should be suspected for you as it can present with renal cell carcinoma and pheo. SDH is still possible.
Dr. Pheo
Thank you so much for your responses on both questions!
ReplyDeleteHello Doctor,
ReplyDeleteI know you work with adults but I'm hoping you can offer an opinion. I gave you a brief over view of my daughter above. To recap her pheo labs, Plasma normetanephrine (reference from lab 148 but she is a child so it should be 77) 179. Urine normetanephrine (reference 580, again she is a child so the reference should be around 244) 489. MRI of her chest and abdomen completely normal. Nephrologist confident there is no pheo. Should I just accept that and take his primary hypertension as an explanation? She has headaches 3-4days a week, bouts of nausea, rapid pulse,anxiety, elevated TSH, and growth failure. I think I'm being asked to accept each of these as separate unrelated topics. Any thoughts?
Dear Dr. Pheo
ReplyDeleteMay I ask how i can consult you, where i can send you details about my case. I'm suspecious from 1yr and I want to send you details to get ur guidance pls.
Regards?
Dear Dr.
ReplyDeleteI'm a 29yrs old patient, Suddenly i got hypertension before 1.5 yr, alllab tests gave me good results (kidney function, liver functione, electrolytes, lipid profile,...) Physician asked me to make, renin, aldestrone and catecholeamines. I got normal renin and aldestrone but elevated catecholeamines (normal range max value is 100 but mine was 164) in 24hrs urine collection.
Dr. started to give me carvidilol 25mg but i kept getting high blood pressure of around 150/110, short breathining, high heart rate, flushes,sweating, and extraordinary headach.
I made MIBG and they told me suspecious lesions, ultrasound gave normar result.
I made adrenalina, nor adrenaline, catecholeamines and VMA in urine, Adrenaline(Normal 20, i got 25), Noradrenaline(Normal 70, i got 125), VMA(Normal 16, i got 36) and catecholeamines (Normal 100, i got 143)
Abdom CT scan gave a prominent left adrenal gland with hyperplasia. PET CT and Octereotide scans gave normal results.
Now i take 50mg carvidilol and 12mg cardura daily and my diastolic blood pressure never get less than 90.
Dr. Made clonidine suppression test for me, noradrenaline got reduced from 1025 (normal max=600) to 524... My Dr. Still see all these results equivocal.
I repeated Noradrenaline, adrenaline and metanephrines in urin again, Noradrenaline(normal max=60, I got 110), adrenaline (normal max=20, I got 25), and metanephrines (normal max=100, I got 130).
Is it pheo?
What do you advice? Any other more tests needed? I'm suspecious from 1yr and I want to get ur guidance pls.
Waiting to hear from you very soon
Regards...
Dear Anne,
ReplyDeleteYour daughter certainly has a serious disease. The growth arrest is particularly disturbing. I agree that you should seek advice from a experienced pediatric expert.
Dr. Pheo
Dear HMZ,
ReplyDeleteFrom your description, the chance that you have a pheo is small but still possible. Where is the abnormal MIBG uptake? Have you had a pelvic CT or MRI to see if there is a tumor in the bladder?
You have clear elevation of catecholamine levels. Are you overweight or obese? Do you snore? Do you have congenital heart disease?
You can go to any of the experts I listed before (whichever is closest to you) for advice.
Dr. Pheo
Dr. P. Want to thank you for responding to me. Info helpful as always. I continue to be amazed at how you can do this. My condition may not be related to pheo, and will not post again unless I have more defintive info in this direction (now being referred for Hida scan, but not inclined to do it, as all sx are on left side - think it is a shot in the dark, but will continue to consult with dr.'s and then make a decision). However, I will continue to follow your blog, anyway. I just wanted to give you feedback on how extraordinary this blog is. I am very touched by the stories that I read from the pt.s posts, and what they are going thru, and how much your truly expert advice must mean to them. Clear, concise and accurate. Having been thru the mill of more doctors than I ever imagined or wanted to see, I can't imagine how you have the time for this. I'm not Jewish, and I don't know if you are, but I think the concept might have s/t to do with "mitzva". I am also learning a great deal, which will help me with my pt.s. About the Iphone - I can access the blog as smart phones have internet. However, an app. would be very cool! Thanks again, and keep up the good work - Edie.
ReplyDeleteDr. Doctor-
ReplyDeleteThank you for the response-just to clarify-what kind of pediatric specialist do you suggest? Her growth hormones are normal, TSH is normal with meds, her BP is supposedly now controlled with triampturine and norvasc, the neurologist suggests a beta blocker but last time they tried that she just got weepy. GI wants to do a colonoscopy but I'm not too keen on it. Every discussion ends in we'll see her again in a few months she looks healthy enough.
Dear Edie,
ReplyDeleteThank you for the good words. I wish you the best.
Dr. Pheo
Dear Anne,
ReplyDeleteI would start with the most bothersome symptom. For example, if the nausea is the most bothersome, I would see a pediatric gastroenterologist. If the growth arrest is the most bothersome, I would see a developmental expert. So on and so forth.
Dr. Pheo
This comment has been removed by the author.
ReplyDeleteDear Dr.
ReplyDeleteThanks for responding, in fact my body mass index changes from 24.5-27 (now 25.5), i never sonore, no congenital heart problems, cardiologist told me i've perfect results except small enlargement due to HTN. Pelvic CT was clear even urination rate &quantity increased (in 24hr urin collection it sometimes reaches 6L).
MIBG gave suspecious lesions bilitral without tumors but uptake a bit more in the left which is consistent with CT scan but i made it in last dec.
All these specialist are very far from me.
Heart rate increases very much for any small effort or even eating, walking, raising stairs,...
Is there any further advice?
Is it possible to be from obesity with a bit elevated BMI? My physician didn't say yet its pheo but told me sure not obesity or stress.
One more note, i never got this headach, sweating (especially at night) except from these 1.5 yrs, also i got some inflammation in my skin started in the same period... Is it related?
My note in ur jan post is more detaild and modified if you can read it plz
Many thanks
Waiting to hear from u Soon
HMZ
Also Chromogranin A, done by ELISA in medicin faculty lab gave me 70ng/ml which is around max but didn't exceed it?
ReplyDeleteMaybe my question isn't clear enough. Do you think we have done enough to rule out pheo? Given her normetanephrines, and one MRI? Thank you.
ReplyDeleteDear Dr Pheo,
ReplyDeleteI am a 42 yr old female, 5'7 115 lb. During my last two pregnancies (age 37,39) I developed what was believed to be gestational hypertension that seemed to normalize after delivery. Also, during these last two pregnancies I had a few panic attacks which woke me from my sleep (tachycardic, hypertensive, shortness of breath, fear of dying).
In April of 2010, I started experiencing palpatations and could hear my bp in my head. Turned out I was hypertensive. Went to doctor who checked my thyroid and then diagnosed me with essential hypertension. I was put on 5 mg lisinopril and cardevil for the tachycardia. I tried taking them, but felt horrible and my bp was dipping down to about 70's/50's. I stopped taking them and called my physician. He said to cut lisinopril in half (which I did, still felt bad from low pressures), then told me to take every other day!?! After monitoring my pressure, most days it is around 115/78, but spikes occasionally to 160-205/95-120. I only took the beta-blocker once, since the tachy is sporadic, as well.
ER docs, family practice,obgyn, etc. all say it is essential hypertension and I need to take my lisinopril, period, or I'll have a stroke! How do you take it when your bp is 115/78? (I don't!)
Finally, seeing an internal med doc who says she doesn't think I have essential hypertension and she ordered a 24 hr urine catecholamines and cortisol & serum epinephrine, norepinepherine, and dopamine levels. They should be back next week, I hope. She wants to rule out a pheo and renal artery stenosis.
Lately, (the past month) the episodes are more frequent. At times my bp is higher in the supine position and I get weird readings with a narrow pulse pressure 140/120 and makes everyone think it is just an inaccurate reading? Also, would irritability, moodiness, and moments of rage have anything to do with a pheo? Sometimes I think this is just all in my head and I must be going crazy!
I have no family history of pheochromocytoma. I don't have profuse sweating (except sometimes at night which I thought might be peri-menopause).
Is there any other testing that should be done or just wait and see how these tests come out? I know you are a pheo specialist, but have you encountered or do you know of anything else that may cause paroxysmal hypertension? I have 12 children and I just want to be as proactive about my health as possible. I already feel they are missing our on their mom @ times because I get so tired at times after my bp has come back down.
Sorry to go on so long! Thank you for this website! I just found it and have been so encouraged already!
Dear HMZ,
ReplyDeleteWith all the information you provide, I think it is unlikely that you have a pheo. I would suggest that you see a cardiologist to work up the palpitation and hypertension.
Dr. Pheo
Dear Anne,
ReplyDeleteI know too little about children's pheo so I cannot answer your question.
Dr. Pheo
Dear Blessedby11,
ReplyDeleteYour symptoms are indeed suggestive of pheo. Let's wait and see the test results first. I usually order plasma metanephrines first but the urine and plasma catecholamines are fine.
Dr. Pheo
Thank you very much, but may i ask if u can suggest any reason that raised these hormones (all of them elevated with different ratios)?
ReplyDeleteI was getting fed up from follow up so u made me relax to consider it as essential HTN.
I repeated the clonidine suppression test but didn't get the new result yet.
Thanks for answering :)
Thank you for your resonse, Dr Pheo!
ReplyDeleteStill waiting on labs above but I was wondering if plasma free metanephrines would be the same as plasma met panel (metanephrines, normetanephrines, and total metanephrines)? If not, I may have to go to a different lab.
~blessedby11
Dear blessedby11,
ReplyDeleteYes, they are the same.
Dr. Pheo
Dear HMZ,
ReplyDeleteIt is not straightforward to figure out what you do have. Please see my post in July 2010.
Dr. Pheo
Thank you very much, In fact I read it before sending you... I'm in MENA region which makes things more difficult... My physician still didn't diagnose me bcs he see most things equivocal but this raise in his point of view isn't accepted.
ReplyDeleteI'll send you the new clonidine suppression test result when I get it (it needs 3 weeks maybe)
Many thanks
Dr. P -
ReplyDeleteOk, said I wouldn't post again unless i had definitive info re pheo which at this point i don't. However, I am still caught up in the actual heading of this particicular 'thread' (I guess?) of your blog. It has for the most part been ignored by posters, which i understand. A pt. who is anxious, confused and suffering needs answers is and is going to ask those q's, and the best way to reach you is thru your most recent post. However, being a psychologist who naturally comes across many with physical ailments both diagnosed and not(and, obviously, my own recent experience)I do think the coping point of view is so important. However,you present s/t quite different from the usual in the literature. Most deal with diagnosed illnesses or conditions, and how to cope. I have not seen much else on how to cope with an as yet undiagnosed condition which leads to significant disruption in a person's entire life, as well as the undersandable obsession with getting a dx in order to organize one's life and get on with it, whatever 'it' may be. It is a learning curve, and i do think there is merit to your online pt's posting here on this topic. Anyway, i appreciate the fact that you are thinking like this - not as common as you might imagine in the medical profession. I dk, maybe a pt. cld start a side blog to share experiences re negotiating the health care aspect, or emotional aspect, e.g. Just food for thought - don't want to take too much time away from pt.'s who have concrete questions. Edie.
Dr Pheo,
ReplyDeleteFor the 24 hour urine, does a preservative necessarily need to be in the urine from the beginning? How much difference would it make in the results? I was given a collection jug without any preservative. I kept it refrigerated, as instructed. I just wanted to know how accurate the results are without the preservative.
Thank you for your time!
blessedby11
Dr Pheo,
ReplyDeleteResults for 24 Hr Urine: I'm not sure if it makes a difference concerning my urine collection (refrigerated/no preservative/shipped to lab refrigerated only... however, catecholamines were frozen). CATECHOLEMINES: Catecholamine Total, Epinephrine, Dopamine = Unable to calculate result since analyte concentration is below detection limit of this method. Norepinephrine = 7 (<80 nml range)
FRAC METANEPHRINES:
Metanephrines Total: 262 (limit <900)
Normetanephrines: 215 (limit <600)
Metanephrines: 48 (limit <300)
Met/Creatinine Ratio: 283 (<600)
Creatinine: .93 (0.63-2.5)
CORTISOL FREE: 17.8 (4.0-50)
CREATININE: 1.13 (0.63-2.5)
ALT Serum Catecholamine Panel
Epinephrine: 50 (limit <50)
Norepinephrine: 687 (112-658)
Dopamine: <10 (limit <10)
TOTAL: 737 (123-671)
Since I was tested, I haven't heard from my doctor. I'm sure that's because it doesn't look like I have pheochromocytoma. My husband did himself order me a plasma free metanephrine test and it should be back at the end of the week. I'm guessing that will only support the other tests indicating no pheo.
I guess it is a bitter-sweet feeling. Now trying to accept the fact that I will have to live like this. Thank you for all you do and I'm glad there is a place for people yet diagnosed to be listened to and advised!
~blessedby11
Dr Pheo,
ReplyDeleteWhat are the chances of having a bichemically silent pheo and could one still cause symptoms that I have (paroxysmal hypertension, occasional left flank pain, and chest pain in particular)?
Thank you, again!
~blessedby11
Dear blessedby11,
ReplyDeleteIf the urine sample is kept refrigerated, the measurements are probably OK. With the normal results in urine and borderline results in blood, the probability of pheo is small.
Biochemically silent pheo does not cause hypertension but can cause abdominal discomfort.
I would now focus on the most significant symptom and give it a fresh look. The most important thing is to follow up with a doctor.
Dr. Pheo
Dear Dr. Pheo-
ReplyDeleteFirst of all you are either at Saint or a masochist. I mean that in the most adoring terms. As I've read through more and more back posts I have come to realize why you posted the above-you have chosen to serve a very frustrated and often mishandled group of people with this blog. So many times you end up getting a pinch of that frustration. I know myself with all I've watched my daughter go through over the last seven years I'm near the end of my rope now that the whole Pheo topic has been brought back up after two years of not believing it was a possible diagnosis. I commend your patience and class dealing with so many frustrated people.
I do have a question I'm hoping you can answer. Do you have any idea why the reference ranges seem to be 148pm/ml on the plasma free normetanehprines from the labs yet the studies done by makers of the test use 112 as the cut off upper reference point? Then there is a study that states that pediatric ranges should be used that take into consideration sex and age for plasma free metanephrines yet the lab doesn't state any? Do you think they are using a different method to measure? or do they "pad" the reference to cut down on false positives? I know you don't do pediatric cases so I apologize for asking but I was reading your post from August 09 and it sounded like you know a little about how this works. We did a second plasma metanephrine on my child and she has come back this time (supine and resting for 20min) with 153, that is either a slight (5) above the lab reference or two times the age appropriate (77pg/ml). Our doctor has told me I'm rude and disrespectful for questioning him. He will see us in 6months to check her blood pressure. I have yet to locate a doctor that is local that has expertise in pheo. We live a few hours from NIH but since we don't have a full body MIBG they won't see us. I've made an appointment at Mayo for November to have her evaluated in their Nephrology department for her hypertention. I'm not sure if this is worthwhile. It would be four days out of school and perhaps upsetting for her. Besides the current medications seem to be working okay-but that is hard to judge. Sorry to babble-anyway do you know what is with the lab references for plasma free mets?
Anne - please keep questioning your doctor. It is not rude nor disrespectful.
ReplyDeleteMy husband was diagnosed with a pheo. His doctor also probably considered me rude and disrespectful (and the feeling was mutual). In fact, it was NOT a pheo - the doctor was wrong. (and my thanks for Dr. Pheo for helping me figure out I needed a real expert, not the doctor we had)
It's your child, and you have every right to question.
I hope you find a different doctor and/or get her evaluated somehwere you trust.
Dear Anne,
ReplyDeleteThank you for your interesting thoughts. The pediatric normal reference range varies by the lab. Can you please post the exact numbers and the lab's name? I can then do some investigations.
If you have doubts, you should always politely challenge your doctor's decision. How a doctor handles those challenges tells a lot about the doctor.
Dr. Pheo
Dear Doctor Pheo-
ReplyDeleteThe last labs were drawn at quest, laying down and fasting. They don't require either of these however the work by the doctors at NIHD titled "Utility of plasma free metanephrines in detecting Childhood Pheochromocytoma" does. I had to call to find a draw site that even had a bed. I wrote to customer service to ask about the ranges and got the following reply:"This is in response to your recent email concerning the reference ranges
for testing done through Quest Diagnostics. I had our Referral
department contact the Quest Diagnostics laboratory in Virginia where
the testing is performed to verify the reference range for
Metanephrines, Free, Plasma. There are no pediatric or gender based
reference ranges for this test. Test performance characteristics are
evaluated and validated under routine laboratory conditions. Reference
ranges are established and/or verified as part of this Method
Evaluation/Validation.
I hope this is of assistance."
Collection Date and Time
10/4/2010 10:05 AM
Component
Result
Range
Status
METANEPHRINE
<25
<=57 pg/mL
Fin
NORMETANEPHRINE
153 (H)
<=148 pg/mL
Fin
TOTAL METANEPHRINE
153
<=205 pg/mL
Fin
Needless to say this is not helpful. However on a very hopeful note the NIH has agreed to bring my daughter in for bloodwork and they will evaluate her themselves! I am so relieved. I suppose that Dr. Pacak feels her levels are worth further investigation. Just knowing there maybe a end to this uncertainty is such a relief. I sent my sweet 4ft6inch seventh grader off to school today with a pounding headache unsure of whether to throw up or get on the bus, yet again, with advil in one hand and her backpack in the other. The person at NIH said that one way or another they will help us get to the bottom of this.
I'd still love to know what the labs do with these ranges!
Hi again. I have posted before (several times). I currently have several mets, have done CVD chemo and Sutent. Although my disease is stable for now, I have severe anxiety issues, probably because my cats are always high. My oncologist referred me to a psychiatrist for anxiety meds, who wrote an Rx for propanelol. Oncologist said no to that I think because my BP is normal (115/75). Can you suggest any anxiety medication that can be taken by a multi-para patient with regular BP? The only others meds taken now are pain meds and medicinal marijuana. Thanks.
ReplyDeleteDo you have any experience with Para/Pheo patients who are pregnant? As I mentioned above I have a Carotid Para--non functioning. Cat/Met levels are fine. My BP is actually a little on the lower side of normal (top number is rarely over 115).
ReplyDeleteIt's been suggested that I need to give birth at a hospital that has support capabilities should the stress of labor trigger a hypertensive crisis and it sound like the doctors are thinking of doing a C-section to minimize the time I'm experiencing the 'stress of labor.'
Have you had patients with head and neck paras or other paras/pheos that are non-functioning at least prior to labor and delivery deliver vaginally? Would you recommend a C-section or scheduled induction or might it be ok to let my body do it's thing and bring baby into the world on her own time?
Dear Val,
ReplyDeleteI have taken care of patients with functioning adrenal pheo in pregnancy but not patients with non-functioning paraganglioma in pregnancy. To my knowledge, a non-functioning carotid body tumor should not suddenly release catecholamines. Not infrequently, female patients become pregnant and have uneventful delivery without the knowledge that they have carotid body tumor.
Carotid body tumors sometimes grow larger during pregnancy.
You probably will be OK to have natural delivery but you may choose to be on the safer side.
Dr. Pheo
Dear Pam,
ReplyDeleteHow high are your pheo markers? If the markers are very high, my experience is that you need alpha blockers even if your blood pressure is normal. Once you are on alpha blockers, you can take propranolol.
Dr. Pheo
Dear Anne,
ReplyDeleteI have looked into the pediatric reference range issue. I understand how you get 77 pg/ml as upper limit of normetanephrine for girls. I agree that your daughter's plasma normetanephrine levels are elevated.
The key question is whether the degree of elevation and her clinical symptoms match. If an adult has classical pheo symptoms but normetanephrine levels are lower than 2 fold, the likelihood of pheo is actually lower because most adult patients with classical symptoms have very high levels. I myself do not have a good sense of how severe your daughter's pheo-related symptoms are.
Dr. Pacak is a great doctor. I am sure you will get a clear answer.
Dr. Pheo
Dear Doctor-
ReplyDeleteIt is going to be interesting how this all unfolds. If we use the age/sex appropriate range her levels have been 2-2.5 times. The Imipramine seemed to exacerbate the symptoms but mask the elevation of metanephrines. She has had difficult to control blood pressure for several years with migraines and nausea-but she really has handled it well and hasn't let it impact her functioning for the most part. She has episodes of flushing, persistently rapid pulse etc..My understanding is that with children sustained hypertension is the norm with a pheo. Thank You again. Regardless of the outcome I do hope our experience helps to further research.
Wow. A lot of curious people this month. Thanks so much for being here for us. Re: my 10/12 post, latest lab results:
ReplyDeleteOct. 4:
epinephrine: **** not detected (range 10-200 pg/mL)
dopamine: **** ditto
norepinephrine: 2308 (range 80-520 pg/mL)
Sept. 10:
epinephrine: 13 (range 10-200 pg/mL)
dopamine: 31 (range 0-20 pg/mL)
norepinephrine: 3060 (range 80-520 pg/mL)
So here are my questions:
1. An anxiety medication is needed and the psychiatrist and oncologist can't agree. Do you have a recommendation for a patient like this?
2. Excel Diagnostics in Houston is using Indium-111 treatment that was FDA approved 5 years ago. The dr. is Dr. Ebrahim S. Delpassand. They've seen recent records and have extended an invitation to come to Houston for treatment. An octreoscan would be done there to check for uptake. Is this a a viable treatment option for a patient with 10+ small tumors that has done CVD and Sutent? Have you heard of these people?
Thank you.
Sorry that is worded so odd. I was trying to be patient-anonymous.
ReplyDeleteDear Pam,
ReplyDeleteI recommend that you take an alpha blocker. After that, I don't see why anti-anxiety medications cannot be used.
I know that group. MIBG radiotherapy may be better than peptide receptor radiotherapy (the one provided by the Houston group), if you have not tried that yet.
Dr. Pheo
I am not an MIBG candidate, unfortunately. I have been told by a reliable source there are no data to support the efficacy for indium-111 octreotide therapy. Would you send your patient to this place for indium 111? Theoretically, of course.
ReplyDeleteDr Pheo,
ReplyDeleteMy PCP looked over my symptoms:
several 50-70 minute attacks starting with lower abdominal pain, moving to sudden left side ache, pain under left rib cage up to the heart- headache like nothing I ever experienced-feels like my heart is puming toxic blood into me and like I'm going to pass out--legs get tight and numb and left arm is sore like a had a hypo---the doc ordered abdominal CT with some purple dye and iodine in the vein and some eneama that wasn't fun--BUT the doctor never did a /blood plasma test or a 24 hour urine test-she wrote on a paper for me that she thought I possibly might have Pheo.. the CT test is expensive and not a good time---do you understand this order of diagn?
Dear Pam,
ReplyDeleteThe octreotide radiotherapy is considered experimental for pheo. The effects thus far have been not impressive. If you receive it as part of a clinical trial and for free, or if you have the means of covering the cost, you can try it. I usually do not invoke this therapy myself to my patients. If they ask, I say the above to them.
Dr. Pheo
Dear Whitefish,
ReplyDeleteLet me know your age and sex first.
Dr. Pheo
Hello Dr. Pheo,
ReplyDeleteI am 50 yrs old male
Dear whitefish,
ReplyDeleteAbdominal pain is usually not a feature of pheo. Pheo can cause abdominal discomfort when it becomes big.
You doctor probably thinks that an imaging needs to be done first to see what causes the abdominal pain.
Dr. Pheo
This comment has been removed by the author.
ReplyDeletedear dr pheo,
ReplyDeletei cannot begin to express how useful your blog is! and this posting on non-pheos is definitely something we all need!
i was told 7 months ago that i might have a pheo because of my "attacks" , diagnosed as asthma, then as anaphylactic allergy and then as adrenalin surges. 4 months ago, i was told i had a pheo and that my life was in grave danger, because of one very high dopamine reading (4x normal), two increased metanephrine (1.5 and 2x) values and two dopamine readings a touch over the normal range. an mibg scan and a ct scan (without contrast because of allergy) showed absolutely nothing. the diagnosis was a pheo was still too too small to detect.
i am a very healthy 52 year old slim woman and have rather low blood pressure with a bit of dizziness. that runs in the family.
the panic attacks continued regularly - maybe no wonder when doctors have told you that you are living with a time bomb inside your body - and i tried unsuccessfully to find work and get health insurance in my new location, having been told that it was unsafe to live on a remote island as i did. stress was building up badly and last week i attended A&E again during a bad (panic) attack. was this the one where my body would explode, was more or less what i thought.
the A&E doctor observed hyperventilation (after a blood test) and gave me valium. i settled down. the endocrinologist has now told me that i "have a small pheo that is not dangerous". his colleague internist - a man famous for his bluntness - told me i had psychological problems and no pheo. paradoxically, he was the one who told me a few months ago that i could have a brain haemorrhage any day! maybe no wonder i was stressed!
yesterday i discussed the confused diagnosis with my GP. he suggested that sedatives could have a diagnostic value. if they help during an attack, then it could not be a pheo as this would not respond. a few days ago i also emailed my test results to a pheo expert . he was very helpful and thinks i have no pheo. i am very relieved! no time bomb!
so now i am trying to get a grip on my life again, with a little box of sedatives handy in case things get out of hand again!
i am very happy not to have a pheo and rather embarrassed at all the fuss created over some asthma/panic/allergy attacks and your blog posting, with its kind understanding for people like me, is like a warm hug full of encouragement to get on with my healthy life!
thank you very much,
indi
Dear indi,
ReplyDeleteThank you for your encouragement. It is unlikely that you have pheo.
Dr. Pheo
thank you dr pheo:-))
ReplyDeleteThe frustration of having a "suspected" yet undiagnosed Pheo has not only forced me to drastically change my life, but it has destroyed my trust in doctors. In my experience I have found these road blocks:
ReplyDeleteWrong reference range used for urine analysis
Faulty protocol for urine analysis
When plasma tests were abnormal (high), doctors said numbers weren't "high enough" - (when is high not high enough?)
Wrong analysis or determination of tests by doctors who have no experience with Pheo's
Unwillingness of doctors to look closely at the unique characteristics of the individual
But the absolute worst is -
CONTINUED ACCEPTANCE of previously incorrect or wrong data by new doctors when reviewing old records.
I have suffered with Pheo symptoms ranging from mild to severe (requiring ambulance to ER). I have had multiple CT and MRI's which show: Adrenal Adenoma, multiple cysts in pancreas, spleen, liver, kidney and a hemangioma in liver.
I have had episodes during procedures such as angiogram (clean arteries, good heart), CT's and at the dentist. I have avoided the dentist because of the last episode, but I now have a bad toothache, yet I am afraid to go. I even have a documented episode while at my endocrinologist during a routine visit.
I live my life like a hermit, just to keep stable. I wish someone with knowledge and experience would take a fresh look at my condition but I don't know how to get there. Seems I can't give up - it won't let me.
Dear Granola Girl,
ReplyDeleteI feel your frustration. I highly recommend that you see one of the experts listed on my pheo doctors list.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteI am sure that you have encountered many patients with no clear diagnosis of pheo but with symptoms of high cathecolamine production:
anxiety disorders, hiperkinetic heart, excessive sweating, hypertension and lots of others.
My understanding is that these are of course not caused by a pheo, but by overstimulation of adrenal glands, that produces kind of similar results. So from a physiological point of view nervous overstimulation of adrenal glands and tumor of adrenal medulla though distinct problems, have the same mechanism by which impairment occurs.
My question is : What is your professional opinion on the unilateral/bilateral denervation of adrenal glands ?
From my current understanding this would not affect the cortical hormone production, but would lower adrenaline/noradrenaline blood concentration.
Thank you
Dear Sergiu,
ReplyDeleteYou asked an interesting question. I don't know much about adrenal medullary denervation, except that it is not done in most patients. I am not aware of any clinical indication of this procedure.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteI have symptoms suggestive of pheo : hypertension, headaches , anxiety, eye pain, slightly elevated body temperature, sweating profusely, all occurring in attack-like fashion, alternating with periods when I am ok.
My endocrinologist excluded pheo based on lab tests ( did them only once, metanephrine =80, chromogranin =83 ), sent me to a cardiologist : EKG was ok at that moment, BP high during attacks, normal otherwise, so I ended up with anxiety disorder diagnosis.
Antidepressants did nothing, benzo's did almost nothing, so I stopped taking them.
I was telling the cardiologist that these attacks are precipitated not only by stress (leading to anxiety diagnosis) but also by exercise in exactly the same way.
That is, If I am running, climbing the stairs, I provoke these symptoms after a few minutes of exercise, I get extremely hot, get chest pain, headache, and feel a weird tingling sensation all over my body, so I must stop. But if I manage to continue running, for example, then after a while it stops suddenly and I feel much better, and I will typically have no attack until the next morning. It's a weird anxiety, I must say :)
So I run every morning. My concern though is that I might damage my heart doing this.. it works for now.
Do you think that I might have a pre-pheo condition ? And those are massive epinephrine discharges ?
Thank you. You have a wonderful blog :)
Dear Sergiu,
ReplyDeleteI also saw your question under another post.
The lab results rule out pheo. I suggest that you do a 24-hour ambulatory blood pressure monitoring to get a definitive pattern. All forms of hypertension can be episodic. If you are young, other secondary causes need to be examined as well.
Dr. Pheo
Dear Dr. Pheo
ReplyDeleteI have spells of paroxysmal hypertension, tachycardia, and palpitations (no other symptoms) with varying frequency. Results of initial test of 24 hr Urine Cathecholamines, Total Metanephrines, VMA were normal except for Epinephrine abnormal at 35mcg . A repeat test was normal except for Epi abnormal at 38mcg . A subsequent 24 hr U Fractionated Metanephrines test was normal: Normetanephrine at 189 mcg ; Metanephrine at 223mcg. Thus, there is a negative result for metanephrine in a highly sensitive test and a positive result for epinephrine in a highly specific test. In light of these conflicting results, how can the effects of these tests on the probability of a pheo be assessed?
Can one use a Bayesian analysis to determine the effect of these tests by deriving a joint likelihood ratio by multiplying the positive LR of the cathecholamines test by the negative LR of the metanephrines test? Or does the positive result of the Epi test (specificity of 99.6 % at >35 mcg according to the Oct 2003 article in J Clin Endocrinol Metab by Dr. Young of Mayo and others) warrant scanning without any further biochemical testing.?
An oddity of my case is that if the initial test had been the fractionated metanephrines test the negative result would have been held by some to virtually exclude a pheo, at least in cases of low suspicion (Eishenhofer, Lenders, Pacak).