Re: JC at NIH - What's New?

May 1, 2012

You often talk out "long term cure rate" and "recurrent HTN". Since the specific goal is to eliminate PA, maybe you could address "reoccurrent PA" after surgery. Do you have any informtion on this? This would be a more accurate measure of the cure rate for PA.

Since we all know there are many causes of HTN, is it possible that some of these other causes are entering the picture after ADx? Do they enter the picture if one is on Spironolactone? (If treating with meds do you have to add more meds to the mix? In fact, does renin remain low so your options are limited?)

Do you know of any other doctors currently treating HA that advocate Meds over ADx when an ADx is indicated? Has the knowledge and testing advanced in the last 20 or so years to the point tht you now longer have to resort to trial of Spiro? In fact, has Maggie's and my experience shown you that Spironolactone is not good option and may actully be doing harm?

> > > > > > > > > > >> > > > > > > > > > > A K of 3.7 could be a poor blood draw. If they use a > > > > > > > > tourniquet,, and I have never seen a nurse in an ER or floor NOT use > > > > > > > > a tourniquet except for someone who is very brittle or very > > > > > > > > dehydrated, and usually only after they have tried the tourniquet > > > > > > > > first.ÃƒÆ'Ã¢â‚¬Å¡ And many slap the vein/arm and can be a little rough about > > > > > > > > it, falsely increasing the K. When I ask them now, most act like > > > > > > > > they knew this about elevating K, but you can tell by their look > > > > > > > > they didn't know this nor were taught this.> > > > > > > > > > > ÃƒÆ'Ã¢â‚¬Å¡> > > > > > > > > > > I had the lab a while back draw my blood and I asked her not > > > > > > > > to use a tourniquet due to the potassium - she did anyway saying she > > > > > > > > draws it "fast" and so I let her (because the nurses always know > > > > > > > > more than anyone). ÃƒÆ'Ã¢â‚¬Å¡ It came back 4.0, I didn't take my K for a few > > > > > > > > days and I could tell, absolutely no doubt, that my K was low. So I > > > > > > > > started taking it again and felt 100 times better. The lab was > > > > > > > > wrong. My follow up, after taking the K and with no tourniquet was 3.4> > > > > > > > > > >> > > > > > > > > >> > > > > > > > >> > > > > > > >> > > > > > > >> > > > > > >> > > > > >> > > > > > > > > >> > > >> > >> > > >>

May 1, 2012

Guess they don't get excited like I do when I find as specific treatable and potentially curable form of HTN. And know the good feeling when a pt almost literally picks up their bed and walks when advanced PA is found and properly treated.CE Grim MDOn Apr 29, 2012, at 11:31 PM, Bingham wrote: Hell, we can barely get the corner doc (and throw in the PA's and NP's too) to even consider hyperaldosteronism most of the time or do the tests right to find it. If the air is electric than I compare it to the Chevy VOLT, where, in a teeny tiny circle it is exciting to some, and because you're there it seems bigger than it is, but it certainly isn't the talk of the town in general. I still say.think one of the biggest reasons is because it, strangely, just isn't "exciting" enough for the average doc and that it just gets perpetuated that way and passed on as rare and not likely - though they all seem to want to find that elusive pheo all the time. I think I have told the story recently of my two friends, nurse practitioners, who wouldn't even TRY spiro in one of their young hypertensives who was resistant to multiple other meds because his serum aldosterone levels weren't off, even though they didn't stop the other meds AND I gave them Dr G's handout AND tried to educate them on how to do it right. They did put him on a new expensive renin blocker (samples to start with) even though his renin levels were okay too. I don't get it. So freakin what? Try the spiro, if it works, like happened in my case (after a hundred years and a hundred other meds), you have it, or at least now have a great clue. But they don't/won't. I really don't get it - but we argued about it in a freinds way and I tried to advocate for the guy. Sadly I am in no position to do it myself for him right now. But they never gave me a reason why they wouldn't write a script for spiro. I have seen this with a lot of patients. I took the liberty of changing the name of this thread. Hope it better describes what we are talking about.Dr. Grim, I would like to respectfully suggest that there is a lot going on in the advancement of diagnosing and treating hyperaldosteronism. I know from spending 2 weeks at NIH that "the air is electric" and the "best Endocrinologists in the World" are working on it, I even had the privilege of talking with a couple of them. I suspect the following, note it was published 2 weeks ago, may be part of it but my Team is not available this week so I have not verified it. We have talked about the "KCNJ5" gene and I understand they are very interested in dissecting my right adrenal! (I'm still waiting for their complete ideas!) They are also very interested in getting DNA from me and my three children! (Three or III is an interesting number isn't it!) Suggest you pubmed KCNJ5 and pay particular attention to the dates of publiction! You can also see where the work is being done!With that information and the following you might begin to see why I say low K may never be an issue for me, have a look:http://www.ncbi.nlm.nih.gov/pubmed/22508439"Advances in WNK Signaling of Salt and Potassium Metabolism: Clinical Implications."AbstractRecent evidence due to the discovery of a family of kinases implicated in arterial hypertension now points to the underlying molecular mechanisms that dictate Na(+), K(+) and water handling in the nephron. These new key players need to be understood in order to fully comprehend the pathophysiology, manifestations, and treatment of common clinical entities such as hypovolemic shock, congestive heart failure, primary hyperaldosteronism, nephrotic syndrome and hypertension. It is through the analysis of the volume status and electrolyte abnormalities that commonly present with these diseases that we can begin to create a link between the abstract concept of a kinase regulation and how a patient will respond to a particular treatment. This review is an attempt to bridge that gap.Copyright © 2012 S. Karger AG, Basel.Maybe it will soon be time to update your evolution article!...> > > > >> > > > > A K of 3.7 could be a poor blood draw. If they use a > > tourniquet,, and I have never seen a nurse in an ER or floor NOT use > > a tourniquet except for someone who is very brittle or very > > dehydrated, and usually only after they have tried the tourniquet > > first.Â And many slap the vein/arm and can be a little rough about > > it, falsely increasing the K. When I ask them now, most act like > > they knew this about elevating K, but you can tell by their look > > they didn't know this nor were taught this.> > > > > Â> > > > > I had the lab a while back draw my blood and I asked her not > > to use a tourniquet due to the potassium - she did anyway saying she > > draws it "fast" and so I let her (because the nurses always know > > more than anyone). Â It came back 4.0, I didn't take my K for a few > > days and I could tell, absolutely no doubt, that my K was low. So I > > started taking it again and felt 100 times better. The lab was > > wrong. My follow up, after taking the K and with no tourniquet was 3.4> > > > >> > > >> > >> >> >>

May 1, 2012

Good keep us posted on the summary studies in others you are meeting there.CE Grim MDOn Apr 30, 2012, at 6:52 AM, wrote: Maybe it is more exciting when you are being considered to be that man (or one of them!) > > > > > > > > > > > > > > > > > > > I have recently read that it was okay to use a > > > > tourniquet to > > > > > > find > > > > > > > > > > the vien and release it for 5 seconds before the > > actual > > > > draw. > > > > > > > (Makes > > > > > > > > > > sense to me that the "damaged" blood would have > > moved on > > > > before > > > > > > > you > > > > > > > > > > captured your sample.) That's how my friendly > > vampire at > > > > NIH did > > > > > > > it > > > > > > > > > > every morning t 5:55 a.m.! I'm waiting for my > > numbers to > > > > come in > > > > > > > > > > from NIH so I can compare to the numbers I get > > locally. > > > > (I do > > > > > > not > > > > > > > > > > expect to find any problems since K was never > > mentioned > > > > and K is > > > > > > > > > > usually not an issue with my style of PA!) > > > > > > > > > > > > > > > > SNIP > > > > > > > > > > > > > > > > > > > > > > > > > > >

May 1, 2012

That's interesting. Dexamethasone is one of those meds I said never again because it made me feel horrible with a capital H! Prednisone I am fine. Dex no way. It's a steroid so may have just been that, but so is prednisone and it doesn't affect me the same.

I had never seen this before. HTN clearly runs in the family on my Dads side. The men are all gone but Dad and an uncle and every single one - grandpa, great grandpa, 3 great uncles all died of massive strokes related to HTN. Not a single MI in the bunch. Grandma says no low K she ever knew in them. And yet i wonder.................to date, if they them read right, there's nothing but a cyst on a kidney on MRI's. Adrenals seem normal they say.>

> > y'know what's bugging me? This group is supposed to be supportive and helpful to those of us suffering and searching for answers. There are several here who offer up good links to research and some of us give our opinions based on experience, but I would think that if there is concrete evidence out there based on research, that we would want to embrace those data and learn and share current knowledge in this area. When I first was dealing with all of this, a while ago, there were numerous studies that clearly stated that a "trial" of meds is simply not the way to deal with HA. Why is this being told to people over and over again who are new and don't know any better? They deserve better, don't they? We have a poster child, , who shouldn't have been trialed on Spiro. We have me, who should not have been trialed. We have research that says not to. We have docs at NIH that say not to. Looks to me like starting someone with HA labs,

hypertension or whatever on a trial med to diagnose HA is a no no.> > > > BTW, there's been a tragic death in my family (google katie death malibu) and I will be absent for a while due to that and an upcoming NIH stint. See y'all after things get better.> > > > > > > > > > > > > > >> > > > > > > > > > > A K of 3.7 could be a poor blood draw. If they use a > > > > > > > > tourniquet,, and I have never seen a nurse in an ER or floor NOT use > > > > > > > > a tourniquet except for

someone who is very brittle or very > > > > > > > > dehydrated, and usually only after they have tried the tourniquet > > > > > > > > first.ÃƒÆ’Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡ And many slap the vein/arm and can be a little rough about > > > > > > > > it, falsely increasing the K. When I ask them now, most act like > > > > > > > > they knew this about elevating K, but you can tell by their look > > > > > > > > they didn't know this nor were taught this.> > > > > > > > > > > ÃƒÆ’Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡> > > > > > > > > > > I had the lab a while back draw my blood and I asked her not > > > > > > > > to use a tourniquet due to the potassium - she did anyway saying she > > > > > > > > draws it "fast" and so I

let her (because the nurses always know > > > > > > > > more than anyone). ÃƒÆ’Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡ It came back 4.0, I didn't take my K for a few > > > > > > > > days and I could tell, absolutely no doubt, that my K was low. So I > > > > > > > > started taking it again and felt 100 times better. The lab was > > > > > > > > wrong. My follow up, after taking the K and with no tourniquet was 3.4> > > > > > > > > > >> > > > > > > > > >> > > > > > > > >> > > > > > > >> > > > > > > >> > > > > > >> > > > > >> > > > > > > > > >> > > >> > >> > >

>>

May 1, 2012

And it - spiro - likely saved my life and my "trial" should have been years earlier. So who's right and who's wrong? Is my story the right one or is yours? I found my answers here and have worked hard to get better as far as PA goes and the advice is still appreciated - and I am bankrupt, professionally destitute, and me and my family has suffered beyond measure because of years of misdiagnosis and the sequelae from all this mess.

You have an anecdotal report and so do I and they're different and following what you suggest SHOULD HAVE been 's path or your path may have left ME, and others unable to type this now because we could have been dead. , I believe, is in the process, but we haven't concluded he actually has PA with absolute certainty (I may be wrong on that, but that's what I gather from his posts). If he doesn't then was this the right list to begin with? then PA meds were never for him either.

See what you're saying, but the world is much bigger than you, and your case "might" apply to some and not to others. No one could tailor the list to fit only your story, or those who have weight issues, or those who have fructose intolerances, or those who have gluten allergies, or hyperthyroidism, or who live in Europe, or who have depression, or ptsd, or 7 kids or no kids, and on and on.

Just like research, pick out what you need and make your informed decision. You're going to have 500 opinions and stories to choose from, can't be thinned skin if your on a list with 500 people and 500 stories and ideas to hear about.

From: maggiekat7 <ljurkovic@...>Subject: Re: JC at NIH - What's New?hyperaldosteronism Date: Monday, April 30, 2012, 10:56 PM

y'know what's bugging me? This group is supposed to be supportive and helpful to those of us suffering and searching for answers. There are several here who offer up good links to research and some of us give our opinions based on experience, but I would think that if there is concrete evidence out there based on research, that we would want to embrace those data and learn and share current knowledge in this area. When I first was dealing with all of this, a while ago, there were numerous studies that clearly stated that a "trial" of meds is simply not the way to deal with HA. Why is this being told to people over and over again who are new and don't know any better? They deserve better, don't they? We have a poster child, , who shouldn't have been trialed on Spiro. We have me, who should not have been trialed. We have research that says not to. We have docs at NIH that say not to. Looks to me like starting someone with HA labs, hypertension or

whatever on a trial med to diagnose HA is a no no.BTW, there's been a tragic death in my family (google katie death malibu) and I will be absent for a while due to that and an upcoming NIH stint. See y'all after things get better.> > > > > > > > >> > > > > > > > > A K of 3.7 could be a poor blood draw. If they use a > > > > > > tourniquet,, and I have never seen a nurse in an ER or floor NOT use > > > > > > a tourniquet except for someone who is very brittle or very > > > > > > dehydrated, and usually only after they have tried the tourniquet > > > > > > first.ÃƒÆ’Ã¢â‚¬Å¡ And many slap the vein/arm and can be a little rough about > > > > > > it, falsely increasing the K. When I ask them now, most act like > > > > > > they knew this about elevating K, but you can tell by their look > > > > > > they

didn't know this nor were taught this.> > > > > > > > > ÃƒÆ’Ã¢â‚¬Å¡> > > > > > > > > I had the lab a while back draw my blood and I asked her not > > > > > > to use a tourniquet due to the potassium - she did anyway saying she > > > > > > draws it "fast" and so I let her (because the nurses always know > > > > > > more than anyone). ÃƒÆ’Ã¢â‚¬Å¡ It came back 4.0, I didn't take my K for a few > > > > > > days and I could tell, absolutely no doubt, that my K was low. So I > > > > > > started taking it again and felt 100 times better. The lab was > > > > > > wrong. My follow up, after taking the K and with no tourniquet was 3.4> > > > > > > > >> > > > > > > >> > > > > > >> >

> > > >> > > > > >> > > > >> > > >> > > > > >> >>

May 1, 2012

Want me to include you as one of my kids when/if we start DNA testing?

I believe they are very early in FH III and identifying what is involved. It appears they are looking at mutation of the KNCJ5 gene, here, look at this (you might even understand it!):

Source: http://ghr.nlm.nih.gov/gene/KCNJ5

ã€€How are changes in the KCNJ5 gene related to health conditions?

UniProt provides the following information about the KCNJ5 gene's known or predicted involvement in human disease.

Defects in KCNJ5 are the cause of long QT syndrome type 13 (LQT13)[1]. It is a heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to excercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy.

Note=Somatic mutations in KCNJ5 have been found in aldosterone-producing adrenal adenomas and can be responsible for aldosteronism associated with cell autonomous proliferation. These are typically solitary, well circumscribed tumors diagnosed between ages 30 and 70. They come to medical attention due to new or worsening hypertension, often with hypokalemia. KCNJ5 mutations produce increased sodium conductance and cell depolarization, which in adrenal glomerulosa cells produces calcium entry, the signal for aldosterone production and cell proliferation.

Let me know if you think of another "large" family that needs to be tested! (Probably premature so I hope everyone doesn't "jump on the wagon"! ) I just hope we can move ID'ing and testing step or two closer!

> > > > > > > > > > > >> > > > > > > > > > > > A K of 3.7 could be a poor blood draw. If they use a > > > > > > > > > tourniquet,, and I have never seen a nurse in an ER or floor NOT use > > > > > > > > > a tourniquet except for someone who is very brittle or very > > > > > > > > > dehydrated, and usually only after they have tried the tourniquet > > > > > > > > > first.ÃƒÆ'Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡ And many slap the vein/arm and can be a little rough about > > > > > > > > > it, falsely increasing the K. When I ask them now, most act like > > > > > > > > > they knew this about elevating K, but you can tell by their look > > > > > > > > > they didn't know this nor were taught this.> > > > > > > > > > > > ÃƒÆ'Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡> > > > > > > > > > > > I had the lab a while back draw my blood and I asked her not > > > > > > > > > to use a tourniquet due to the potassium - she did anyway saying she > > > > > > > > > draws it "fast" and so I let her (because the nurses always know > > > > > > > > > more than anyone). ÃƒÆ'Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡ It came back 4.0, I didn't take my K for a few > > > > > > > > > days and I could tell, absolutely no doubt, that my K was low. So I > > > > > > > > > started taking it again and felt 100 times better. The lab was > > > > > > > > > wrong. My follow up, after taking the K and with no tourniquet was 3.4> > > > > > > > > > > >> > > > > > > > > > >> > > > > > > > > >> > > > > > > > >> > > > > > > > >> > > > > > > >> > > > > > >> > > > > > > > > > > >> > > > >> > > >> > > > > >> >>

May 1, 2012

, if I am the you're referring to, make no mistake - I DO HAVE PA AND IT WAS INITALLY DXed THE SAME WAY YOUR'S WAS! In fact I believe we both even developed gynecomastia, I only developed bumps and needed a mammogram! That scared me enough that I decided I needed to get to the bottom of it. I have and am reporting what I have found and am finding!

I hope and pray you are not masking cortisol!

I hope your case is as simple as only needing a dose of spironolactone, however, If we find a way to ID FH-III would you submit to a cheek swab to see if it might be applicable? Just think, we might save up to 7 families (your children) from going through what you have endured! ie. "and I am bankrupt, professionally destitute, and me and my family has suffered beyond measure because of years of misdiagnosis and the sequelae from all this mess". And if lucky we may even find a way to prevent it!

I am not advocating everyone go through all the testing I have. I am what they consider an "index patient" where they administer a quarter million dollars worth of tests so they can look at all possible effects and hopefully further the research.

I am probably donating at least one adrenal to the project. If I have to pay transportation to do that, I will (but I will "bitch" about it!) $350 is a small price to pay if I can better the lives of my children and grandchildren, and if I can leave the world a little better and more informed, that's a bonus!

BTW, are you depressed? How's your Cortisol?

> > > > > > > > > >> > > > > > > > > > A K of 3.7 could be a poor blood draw. If they use a > > > > > > > tourniquet,, and I have never seen a nurse in an ER or floor NOT use > > > > > > > a tourniquet except for someone who is very brittle or very > > > > > > > dehydrated, and usually only after they have tried the tourniquet > > > > > > > first.ÃƒÆ'Ã¢â‚¬Å¡ And many slap the vein/arm and can be a little rough about > > > > > > > it, falsely increasing the K. When I ask them now, most act like > > > > > > > they knew this about elevating K, but you can tell by their look > > > > > > > they didn't know this nor were taught this.> > > > > > > > > > ÃƒÆ'Ã¢â‚¬Å¡> > > > > > > > > > I had the lab a while back draw my blood and I asked her not > > > > > > > to use a tourniquet due to the potassium - she did anyway saying she > > > > > > > draws it "fast" and so I let her (because the nurses always know > > > > > > > more than anyone). ÃƒÆ'Ã¢â‚¬Å¡ It came back 4.0, I didn't take my K for a few > > > > > > > days and I could tell, absolutely no doubt, that my K was low. So I > > > > > > > started taking it again and felt 100 times better. The lab was > > > > > > > wrong. My follow up, after taking the K and with no tourniquet was 3.4> > > > > > > > > >> > > > > > > > >> > > > > > > >> > > > > > >> > > > > > >> > > > > >> > > > >> > > > > > > >> > >> >>

May 1, 2012

Most with GRA HAVE Normal adrenal scansMay your pressure be low!CE Grim MS, MDSpecializing in DifficultHypertensionOn May 1, 2012, at 11:46, Bingham <jlkbbk2003@...> wrote:

That's interesting. Dexamethasone is one of those meds I said never again because it made me feel horrible with a capital H! Prednisone I am fine. Dex no way. It's a steroid so may have just been that, but so is prednisone and it doesn't affect me the same.

I had never seen this before. HTN clearly runs in the family on my Dads side. The men are all gone but Dad and an uncle and every single one - grandpa, great grandpa, 3 great uncles all died of massive strokes related to HTN. Not a single MI in the bunch. Grandma says no low K she ever knew in them. And yet i wonder.................to date, if they them read right, there's nothing but a cyst on a kidney on MRI's. Adrenals seem normal they say.>

> > y'know what's bugging me? This group is supposed to be supportive and helpful to those of us suffering and searching for answers. There are several here who offer up good links to research and some of us give our opinions based on experience, but I would think that if there is concrete evidence out there based on research, that we would want to embrace those data and learn and share current knowledge in this area. When I first was dealing with all of this, a while ago, there were numerous studies that clearly stated that a "trial" of meds is simply not the way to deal with HA. Why is this being told to people over and over again who are new and don't know any better? They deserve better, don't they? We have a poster child, , who shouldn't have been trialed on Spiro. We have me, who should not have been trialed. We have research that says not to. We have docs at NIH that say not to. Looks to me like starting someone with HA labs,

hypertension or whatever on a trial med to diagnose HA is a no no.> > > > BTW, there's been a tragic death in my family (google katie death malibu) and I will be absent for a while due to that and an upcoming NIH stint. See y'all after things get better.> > > > > > > > > > > > > > >> > > > > > > > > > > A K of 3.7 could be a poor blood draw. If they use a > > > > > > > > tourniquet,, and I have never seen a nurse in an ER or floor NOT use > > > > > > > > a tourniquet except for

someone who is very brittle or very > > > > > > > > dehydrated, and usually only after they have tried the tourniquet > > > > > > > > first.ÃƒÆ’Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡ And many slap the vein/arm and can be a little rough about > > > > > > > > it, falsely increasing the K. When I ask them now, most act like > > > > > > > > they knew this about elevating K, but you can tell by their look > > > > > > > > they didn't know this nor were taught this.> > > > > > > > > > > ÃƒÆ’Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡> > > > > > > > > > > I had the lab a while back draw my blood and I asked her not > > > > > > > > to use a tourniquet due to the potassium - she did anyway saying she > > > > > > > > draws it "fast" and so I

let her (because the nurses always know > > > > > > > > more than anyone). ÃƒÆ’Ã†'ÃƒÂ¢Ã¢â€šÂ¬Ã…Â¡ It came back 4.0, I didn't take my K for a few > > > > > > > > days and I could tell, absolutely no doubt, that my K was low. So I > > > > > > > > started taking it again and felt 100 times better. The lab was > > > > > > > > wrong. My follow up, after taking the K and with no tourniquet was 3.4> > > > > > > > > > >> > > > > > > > > >> > > > > > > > >> > > > > > > >> > > > > > > >> > > > > > >> > > > > >> > > > > > > > > >> > > >> > >> > >

>>

May 1, 2012

I would argue should have been on Spiro and DASH much sooner. Anyone with drug resistant Htn and OSA should have Spiro added IMHO AND taught to DASH. only when we have good data on several hundred seen here can we make better recommendations based on data. Ultimately we will need trials to test which is the best way to go with who. May your pressure be low!CE Grim MS, MDSpecializing in DifficultHypertensionOn May 1, 2012, at 12:12, Bingham <jlkbbk2003@...> wrote: