As pheo is a rare disease, it is no surprise that many doctors have little experience on pheo. Ideally patients with suspected pheo should be seen at large centers with extensive experience such as Mayo Clinic or National Institute of Health. On the other hand, most patients with suspected pheo do not actually have pheo and the management of pheo is not that complicated in most cases. Having traveled long distances, many patients with suspected pheo would feel that they probably could have saved the trip if their primary doctors had simply communicated with the experts in large centers about their conditions. A recent paper addresses the above situation somewhat, albeit indirectly.
In this paper, doctors at one center examined whether a "pheo interest group" could improve the diagnosis and treatment of pheo. The pheo interest group is nothing more than a group of doctors with a common interest in this rare disease. A few of the doctors have a large personal experience with pheo. The doctors compared the diagnostic accuracy and management of pheo before and after the formation of the pheo interest group and found that the quality of care improved after the group formation.
It might be expected that quality of care of course would be higher if the patients were seen by pheo specialists. This paper, however, shows that the pheo specialists do not have to see the patients directly. A phone call or email exchange between the primary doctor and a pheo specialist often does the job. The pheo specialist would review the clinical history, lab test, and imaging results (if available) provided by the primary doctor and arrive at a conclusion. The specialist only communicates with the primary doctor, without seeing the patient. The medical responsibility still rests in the primary doctor's hands. The primary doctor chooses to adopt the opinions of the specialist or not. As alluded in this paper, most primary doctors would adopt those opinions after they have had positive experience with the pheo specialists.
The moral: an inexperienced doctor should seek help from pheo specialists for diagnosis and management of pheo. Will the specialists be happy to answer the questions from non-specialists? They should. Take me as an example. I have been approached by doctors in my practice area and from remote places for pheo-related questions. I am happy to answer them. First of all, these questions are not very frequent so do not cost a lot of time. Second, I often learn new things on pheo from my colleagues who have questions in pheo. Last, it is rewarding for me to help my colleagues and patients (indirectly). I always make sure to emphasize that my opinions are "friendly" and non-binding. It is the primary doctor's responsibility to decide what to do.
Dr. Pheo
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I think this is an outstanding idea!!!!
ReplyDeletecould you provide a citation for the article?
ReplyDeleteDiagnosis and management of pheochromocytoma in an academic hospital 3 years after formation of a pheochromocytoma interest group.
ReplyDeleteEndocr Practice. 2011 May-Jun;17(3):356-62.
Are there any pheo specialists who offer a second opinion about diagnosis for a patient for a fee? Cleveland Clinic has "MYCONSULT",an
ReplyDeleteonline, fee-based service for patients to get a second opinion, but I don't know if any pheo specialists are available under "MYCONSULT".
For what it's worth, there are a few Facebook websites out there for Pheo/Para concerns and they are WONDERFUL, but there is also always the concern that laypeople get things wrong often and mis-perceptions can happen easily. I would love to see a Pheo specialist on a Facebook group or messageboard forum of some sort interfacing with patients. I think it would be amazing if there was a single clearinghouse where a medical professional specializing in pheos was an active presence. Dr. Jack West and other docs have done this at www.cancergrace.org (Dr. West's TED talk is outstanding there as well) for other cancers. This disease is so complex and rare that it's isolating. That alone can make it crazy-making for patients who are lost in a wasteland of doctors who aren't sure what to do with them. I so understand and appreciate how busy doctors are, but I wonder about working with doctors training under them (Residents, Interns?) with a principal expert in a supervisory role. Just something I've been thinking about.
ReplyDeleteDear jhardy,
ReplyDeleteI am not aware of any fee-based service on pheo.
Dr. Pheo
Dear Val,
ReplyDeleteIt is a complicated issue. One major concern is whether the opinions from a website expert are binding. Physician-to-physician communication avoids that major concern.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteIt’s a great pleasure to read your blog. I find your post very informative. This is a really great effort since even if Pheo is a rare disease, everyone posses sufficient knowledge to treat or in the most initial stage, identify it. Through this collective effort, the quality of effort will surely improve.
As a reader, I consider your writing to be a great example of a quality and globally competitive output. It would be a great thrill and honor if you could share your genuine ideas and knowledge to our community, Physician Nexus. With this you can gain 1000 physician readers from over 62 countries on Nexus.
We would love for you to visit our community. It's free, takes seconds, and is designed for physicians only - completely free of industry bias and commercial interests.
Best,
Janmar Delicana
On behalf of the Physician Nexus Team
www.PhysicianNexus.com
Dr. Pheo: This is off the current topic but I am wondering if there are any long term effects from high catacholomines? Could the autonomic nervous system be effected from them being raised for an extended amount of time?
ReplyDeleteThanks
Kate
Dear Janmar,
ReplyDeleteThank you for the compliments and invitation.
Dr. Pheo
Dear Kate,
ReplyDeleteLong-term exposure to high levels of catecholamines mostly affects the cardiovascular system. The autonomic nervous system is also affected. For example, constipation is common in patients with metastatic pheo.
Dr. Pheo
Thanks for the reply, Dr. Pheo! Could you be more specific in the complications that could arise out of high levels of catecholamines with both the cardiovascular and autonomic system? Particularly, I am concerned about undiagnosed episodes of syncope.
ReplyDeleteKate
Hi Kate:
ReplyDeleteCardiovascular: palpitation, syncope, heart failure, etc.
Autonomic system: sweating, flushing, constipation, etc.
Syncope is a common symptom and could be caused by many diseases. The feelings immediately before the syncope often help suggest causes. For example, palpitation before syncope suggests arrhythmia as likely cause. Headache and sweating before syncope suggest hypertension (as in pheo).
Dr. Pheo
I guess these doctors could have used the advice of an expert. Although they saved the woman's life, they almost killed her in the process. What type of friendly advice would you have given to these doctors. Minimally I would have told them the person's BP needed to be stablized with alpha-blockers for at least a week before the operation.
ReplyDeleteI can see assisting a doctor with the dx, but surgical treatment is a different thing. Especially, when these may have been the best doctors accessible to the person.
http://expressbuzz.com/cities/chennai/a-rare-surgery-performed-at-kmc/300381.html
Dear DJPheo,
ReplyDeleteEven in this case, if the doctors had made a call to an expert, they could have avoided the complications.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeleteit's been a pleasure to read through your blog for somebody who had to fight quite a battle to finally (yesterday) end up with a DOPA PET/CT result showing a 5-fold increase in SUV in my right prostate lobe (lab works had shown up to 5x increase in plasma noradrenaline levels).
As the prostate is seems to be the exclusive domain of urologists (and none of them has real experience in dealing with pheos), do you have any advice about which urologic center to chose for surgery? What questions to ask a potential surgeon?
Many thanks in advance!
awl29
Dear awl29,
ReplyDeletePheo in the prostate is exceedingly rare thus no urologists have real experience. The key thing is multidisciplinary approach. You should have an endocrinologist experienced in pheo in general and an open-minded urologist working together. I would suggest that you go to an academic center having one of the doctors I recommend in an earlier post. They usually have urologist working with them (routine adrenalectomy for pheo is done by both general surgeons and urologists). The key question to ask a urologist is how willing she/he is in working together with an experienced endocrinologist.
Dr. Pheo
I guess it's strange that the expert in my small town is a Urologist. He and his partner trained at SUNY in the kidney transplant program. Luckily our primary care doctor also train d as a surgeon in China and has enough experience to have recognized my husband's symptoms as well. Unfortunately his lab tests were negative, 24 hr urine and blood tests. He went through surgery without a decent prep and his recovery in ICU was frightening. I love your blog and wish I would have kept reading after the negative labs. The last two weeks would have been so much easier.
DeleteDear Alice,
DeleteThanks for sharing.
Dr. Pheo
Dear Dr. Pheo,
ReplyDeletemany thanks for your quick reply! :-)
I now also have the detailed PET/CT findings, where the recommendation is to have a prostate biopsy to confirm the pheo (possibly to exclude a fals-positive PET/CT due to prostatitis (?)).
Wouldn't a biopsy be as dangerous as a surgery in terms of the risk of causing a pheo crisis? Does a biopsy indeed make sense from your point of view? Or would a high-res MRI be of the same diagnostic value?
(As the pheo was only suspected, but not located before DOPA PET, I don't have any prostate MRI so far...)
Thanks a million one more time for your help!
awl29
Dear awl29,
ReplyDeleteI would start with an MRI of the pelvis to clearly delineate the structural relationship between the possible tumor and the prostate. If the tumor is indeed inside the prostate, then a biopsy may be valuable. Of course biopsy of a possible pheo always has a risk of hypertensive crisis. On the other hand, this tumor is so rare in the prostate while other prostate tumors are much more common and could potentially be more aggressive; knowing what we are dealing with before the surgery is important. If a decision of biopsy is made, you should be carefully monitored during the biopsy with anti-hypertensive medications handy to be given.
If the tumor is actually separate from the prostate, a biopsy is probably not needed. A frozen section during surgery may be enough.
Dr. Pheo
Dear Dr. Pheo:
ReplyDeleteIn an earlier post (4/30/2011), I indicated the positive results of two 24 hr catecholamine tests (during spells) of epi 35; 2nd test epi 38 and negative results of a later 24 hr fractionated metanephrines test: normetanephrine: 189 ug/24 hr; metanephrine 223 ug/24 hr .(These were taken in June/ July 2010)
I’ve just had a plasma free metanephrines test with: normetanephrine at 90 pg./mL; metanephrine at 146 pg/mL. The tests were done in a non-fasting state, in a seated position, with venipuncture. Is the upper reference level < 100 pg/mL for metanephrine with the above procedure? I also had an anxiety attack, estimated pulse 100 or over and presumably hypertension, during the procedure.
What is the effect of the elevated result of plasma metanephrine on the probability of a pheo? Should I have another fractionated urine test done at this point? I am a 71 year old male. All my kidney function tests (including a good alb/cr ratio) are normal.
I am so glad to have found this blog!
ReplyDeleteMy consultant obstetrician queried a pheo with me 7 days postpartum, we have run three 24 hour urine collections, all of which have come back with elevated levels of dopamine (7501 nmol/24hr (ref <2500) and metabolites - 3 Ortho methyldopamine at 2926 nmol/24hr (ref 200-1400). All results generally around those levels.. Also had elevated noradrenaline/normetanephrine but not by as much (670nmol/24hr (ref <570) and 2307nmol/24hr (ref 300-220) respectively). CT adrenals NAD, as was MRI (without contrast) of thorax and abdomen. Awaiting MRI head and +/- pelvis. MIBG (I-123) on wednesday.
My husband and I are reasonably terrified at the moment given the credible evidence that dopamine, while not being a given for malignancy, is definitely a marker. It is comforting to know that the imaging so far is NAD but they haven't scanned the whole of the sympathetic chain yet to completely exclude.
Is there ANYTHING else that this could be? We have both read and read and read and read and just can't find anything that would elevate dopamine levels this high?
Also, I would be interested to know your views on tyramine foods with elevated dopamine levels (+/- a pheo) as I've noticed a definite worsening in how I'm feeling when I eat foods with tyramine in..
My endocrinologist has started me on doxazosin and, 2 weeks later, added in bisporolol as well. BP now steady at 140/85-90 (from 190/130) and pulse now 60-80 (from 130-140).
I would be grateful for any thoughts, thank you..
Helen
Dear Dr Pheo,
ReplyDeleteHaving read your blog further I thought you might appreciate some further information. I am a 33 year old female. I had no problems with blood pressure in my first pregnancy but was pre-eclamptic with my second. At 5 weeks postpartum I was readmitted with a bp of 190/130, pulse 130, headaches, sweating etc etc. This was diagnosed as essential hypertension and I was started on adalat, 20mg bd. Nil improvement in bp was noted either through adalat or losing 56lbs. I became pregnant again when my second baby was 5 months old and that was only when my bp returned to normal. BP was unremarkable until 35 weeks when I stopped taking aspirin, it then became labile and headaches increased, as did my pulse. I was admitted at 37 weeks and given 2 steroid shots in the evening at 38 weeks and 1 day and 38 weeks and 2 days. By the following morning my bp was 190/130, I had the worst thunderclap headache I have ever experienced and I also had central chest pain radiating to my neck and left arm. I also had severe flushing (I looked sunburnt) for 12 hours and then went very very pale, to the extent where each new member of staff caring for me would immediately look up my Hb (11.7 1/7 post section, rising to 14.5 by 6/7 post section, nil iron supplementation given). My endocrinologist thinks it likely this episode prior to delivery was a hypertensive crisis - could this have been provoked by the steroids?
Also, the urine results I had done - the last two collections have been around the 2.5 litre mark but only because I only had one collection pot for each collection so I was hugely limiting the amount of fluids consumed. The first collection I drank normally and my output was around 4.5 litres. (When I was admitted for pre-eclampsia with my second pregnancy they ran a 24 hour urine collection for total protein and my urine output was of a similar magnitude then.)
The reference range I quoted earlier for normetanephrine should have read 300-2200! My levels of dopamine had lowered a little on the second and third collections but not to less than double the maximum range. Likewise my levels of noradrenaline/normetanephrine had lowered too but were still out of normal range. 3 Ortho methyldopamine remained elevated to levels quoted previously on all samples. The collections were done at 1 week, 5 weeks and 6 weeks postpartum. (Currently 7+/52 postpartum) I am not taking any of the medications that can cause false positive, luckily because I am asthmatic they were cautious with labetalol use. Only medication of note was MST - I was taking 165mg daily for severe pelvic pain but had spent the last 2 and a bit months of pregnancy weaning down by 15mg every 4 days. I was therefore on 15mg a day from 35 weeks and dropped to 15mg every other day from 36 weeks, stopping completely at 37 weeks and 5 days, steroids at 38+1 and 38+2, delivering at 38 weeks and 3 days. Minimal withdrawal noted to the baby after delivery to morphine.
We've had an advanced thyroid profile done, that returned as normal. Equally cortisol levels are normal too. I do have some anolomies on my U+Es, I can give details if you wish.
Many thanks,
Helen
Dear jhardy,
ReplyDeleteElevation in metanephrine or epinephrine (versus normetanephrine or norepinephrine) is always more concerning. The persistent elevation in your case raises the probability of pheo. You can either monitor the levels periodically or go ahead with imaging.
Dr. Pheo
Dear Helen,
ReplyDeleteMy suspicion of pheo is not high. There are rare dopamine-secreting pheos but they usually do not cause hypertension. Dopamine in urine is mostly from the kidney rather than from pheo. I recommend that you do plasma metanephrines and catecholamines. I also await the additional imaging and MIBG results. I suspect that they will be negative.
Dr. Pheo
Dear Dr Pheo,
ReplyDeleteThank you for your reply, that is reassuring to hear! I don't think we can get plasma dopamine/metaboblites over here (UK) but I am seeing my endocrinologist (well, his reg as he's on holiday) on thursday so I will ask then.
Are the levels of dopamine consistent with what you would expect for someone with pre-eclampsia in pregnancy? Everyone always commented that my pre-eclampsia was quite atypical which was what made the obstetrician query the pheo.. Likewise, would you consider the levels of dopamine to be of concern?
Lastly, I understand that the MIBG scan needs noradrenaline to bind to so that the tumours can be lit up on scan. My noradrenaline levels, while elevated above the normal range, are not significantly elevated - would therefore the scan be able to pick a pheo up were there to be one? Would a PET be indicated if the MIBG comes back negative or can we breathe a sigh of relief if the MIBG comes back negative and start looking elsewhere for why the dopamine levels are high? I'm also waiting on the results of a CgA blood test, if that is negative can we absolutely rule out a pheo? Both my husband and I were saying that if it's ruled out, for peace of mind, it would be nice if it were "you definitely don't have one because of a, b and c" rather than "you don't have one because we can't see it on scan" - the former seems more diagnostic!
Many thanks again for your reply
Helen
Dear Helen,
ReplyDeleteUrine dopamine levels are indeed elevated in women with preeclampsia.
As I explained in my earlier posts, pheo diagnosis is based on integration of clinical findings, lab tests, and imaging. We should not base our diagnosis on a single test. There is no gold standard to cover all situations.
Dr. Pheo
Dear Dr Pheo,
ReplyDeleteThank you for your reply.
I guess time will tell then when I get the scan results back!
Best wishes,
Helen
Dear Dr Pheo,
ReplyDeleteMIBG showed elevated uptake (slight) in left adrenal gland.. Also (from MRI) there was thickening to the thymus. Awaiting consultant's return from leave on monday to make a plan of care..
What is the significance of the thickening to the thymus?
Regards,
Helen
Dear Helen,
ReplyDeleteMild MIBG uptake in the adrenal gland is not uncommon in people without pheo. Thymus thickening is not specific. Paraganglioma in the chest is usually in the posterior rather than anterior (like in the thymus). Overall, these findings do not suggest that you have pheo.
Dr. Pheo
Dr Pheo,
ReplyDeleteA noted pheo expert recently stated to peers that rules should be established instead of recommendations. One was that only a few surgeons should be allowed to operate. The context was within a discussion of para, but I thought it also applied to pheo. This doctor is known to challenge peers to make a point. What your thoughts about standards for surgeons operating on pheos?
Dear DJPheo,
ReplyDeleteI suspect that you are the ISP 2011 as well (see my new post). I don't think it is good idea to regulate what surgeons can or cannot do.
Dr. Pheo
7 1/2 years ago dx malignant pheo left adrenal, now normetaneprhine 24h UR- 5261. Where should I go?
ReplyDeleteDear Anonymous,
ReplyDeletePlease go to a nearby pheo specialist. See the post "pheo doctors".
Dr. Pheo