Friday 26 December 2008

The Vein Sampling Results

December 2008

I just got my results from Dr. Liddell. He had called last week to give me the cortisol reading. Both adrenals had high cortisol levels which he said basically meant they found the adrenals, that's how they respond when poked with a catheter.

Today he gave me the aldosterone scores. Before the ACTH was added, my right adrenals aldo level was 21. After adding the ACTH, it was 208. This seemed in line with him, he did not believe there was bi-lateral overproduction going on. The left adrenal on the other hand (which is where the tumor is) was way out of line. Before the ACTH, left adrenal read 800, and after receiving it, it shot up to 4000, which is extremely high. The diagnosis was that I have a unilateral tumor on my adrenal gland that is out of control-exactly what we all thought. He highly suggested surgery (which didn't surprise me) saying I was too young and in shape to have to take blood pressure meds for the rest of my life, not to mention the danger factor of having such a condition in the first place. As well, I'd never have to take those darn horse pill potassium supplements again. Next step-- an endocrinologist visit followed by the surgeon.

While I'm not thrilled with the prospect of surgery, I now finally have concrete answers with some bright light up ahead at the end of this very long tunnel. I feel relieved in so many ways.

Sunday 21 December 2008

The Adrenal Vein Sampling Test

December 2008

It was suggested by my endocrinologist that I have an Adrenal Vein Sampling Test (AVS) to determine the aldosterone production values. I have read all sorts of information about this procedure on the web-- how it's often difficult to get the catheter in the right adrenal vein, and that sometimes the adrenals can get damaged. I also read that up to 50% of patients can have an unsuccessful reading. All very scary, risky stuff to me.

Feeling like a lab rat, I was eager to get to the next level of a prognosis, so I agreed to take it. I made a second appointment with my endo to get as many facts as I could on the procedure, and I recommend anyone with Conn's to do the same as this is a serious procedure.

Doctors who perform the AVS are interventional radiologists. The doctor recommended to me was also at Mercy, Dr. Robert Liddell. Since I'm only mentioning in this blog the doctors who I highly recommend, I can say without hesitation both Dr. Sardi (surgeon) and Dr. Liddell are two doctors I feel fortunate to have. Both are well-known experts in their fields, and have staffs that are equally as helpful.

THE AVS PROCEDURE AND WHAT TO EXPECT

Day Before:
There really isn't much to do prior to the surgery. If you are on spiro, you must go off it. After midnight, you cannot eat or drink anything. This includes water. You also can't take your BP meds the day of the AVS, so I suggest taking them right before midnight with a big glass of water. If you are like me, you are probably dehydrated often, so this part was tough, especially in the morning when all you want to do is drink some.

Day of Preparations:
The earlier you take the test the better as you will be parched and low on potassium. My procedure was at 11:00 but there were two hours of prep time.

Things should move fairly quickly once you are admitted. I was in a bed by 10:30 getting a lot of details of the surgery from a nurse. I'll stop here and say I recommend you take someone with you that you can lean on. This is a serious test and it will become a pretty intense day for you quickly, so you really should not go it alone.

The first thing the nurse will do is take your blood pressure. Then you will have your temperature taken. They stuck a weird instrument in my ear to get mine, which I wasn't familiar with.

Next, you'll get a catheter inserted (no, not fun). It is rather unpleasant if you've never had one-- the first half hour is uncomfortable with pressure. Since this is a procedure where another catheter goes into both sides of your groin, you will be shaved down there a bit.

About 20 minutes later, you will receive an I.V. for the anesthesia.

A nurse will take your blood pressure about 3-4 times before they wheel you in, as they want to make sure it's not high.

Soon after, the doctor arrives and he explains how the procedure works, how long it usually takes and what the risks are. Like I had read, the major risks are blood clotting, not being able to find the adrenals, which result in an insufficient reading-something you don't want to occur. I was thrilled when he didn't mention words like death or imploding adrenals.

Surgical Procedure:

About an hour or so after being admitted, you will be wheeled in to the operating room where about 4 nurses are prepping for your surgery. They will clean and sterilize your groin area for a bit and then place a piece of heavy tape or plastic between your legs up to your chest. You will then be administered a sedative and anesthesia- mine was Fentanyl and something else. It takes very little time to get loopy and go under. There will be nurse at your side the entire time to answer any questions, or just hold your hand. The other nurses will be helping the doctor and cleaning/preping instruments. I was surprised to see so many nurses, but like I said, this was a complicated procedure.

You may be in and out of consciousness during the procedure. I could see my doctor looking at a computer screen while operating the catheters. If you are like me, you may have an odd side effect of the fentanyl by your nose itching like crazy. Towards the end, the doctor woke me and said he was having a hard time finding my right adrenal but didn't want to stop and would it be ok to continue longer- I said yes which put me in there for four hours, a bit on the long side for this procedure.

The Recovery:
You'll return to your bed and will be out of it for about one hour. The nurses will check on you constantly checking for hematomas (which I had) and clotting. You will be asked to keep you legs as straight and still as possible which can be difficult. You will be severely dehydrated but have to wait to drink. Eventually- about 20 minutes or so- they should give you ice chips and then a cup of water. After that you can have juice, soda and some food. I had no appetite at all but ate some crackers. I recommend trying to eat a little something because you'll have a lot of drugs in your system. They also want you to stay flat on your back for an hour and then will raise your head 30 degrees two times after.

The doctor will then come in and tell you how the procedure went. He will not have the results of the hormone levels for at least one week. The discharge instructions are to avoid getting the opening infected and to watch for bleeding.

After you have recovered for 2 hours, they ask you stand to see that you can walk, then you get to go home. Again, make sure someone is driving you- you won't be in the best condition to do this yourself.

Going Home:
When you get home, you will be completely out of it and want to just sleep. You may get ill from the fentanyl, which is still in your system. When you are home, you are advised to drink 16 oz of liquid to flush out the anesthesia. Saltines help too.

Soreness is also expected, and mine began 3 hours later. I felt pain in places I didn't expect- like my arms and lower legs. The pain was overwhelming in my groin area. Big bruises appeared the next day and are expected, but it's important to make sure they are soft, not hard. As well, check your bandage for excessive bleeding. I have no idea why they don't send you home with a day or two worth of painkillers, they really should. I took 600 mg of advil, and slept a lot.

The Dr. said I would be fine in a day--but I was pretty sore for three days. One tip- DO NOT lift anything for the first two days. Seriously, I did this and paid for it...lots of pain. The results come in about 5-6 days.

While it a tough procedure, it will really help you see if surgery is your next step.

I hope this information helps you and wish you luck.

To read more about the AVS procedure with complete medical terminology and explanations, go to:
http://radiographics.rsnajnls.org/cgi/content/full/25/suppl_1/S143

If you live in the Baltimore/DC area and need to have this test, contact:

Dr. Robert Liddell
Interventional Radiology
301 St. Paul Place, 1st Floor Tower
Dept. of Radiology
Baltimore, Maryland 21202
Phone: 410-332-9268 Fax: 410-545-4255

Monday 15 December 2008

The 24 Hour Urine Collection Test

This test is done to see how much aldosterone is released into your urine. It may be one of the last tests your endocrinologist gives you before making a decision on surgery, but it depends on his testing protocol. Mine was supposed to be done right before the AVS, but it got delayed due to my endo's office staff's incompetence.

The test is not, however, as icky as it sounds. But it is a bit inconvenient, especially if you are a busy person who is prone to activities outside of the home. You should try to not leave the house when you do it, or you'll have to carry your little cup with you. And then fly home to refrigerate it, or even worse-refrigerate it at work (gasp!). And you know what they say--sometimes the cuppeth runneth over. And if you have P.A., chances are it will. Often.

So yes, it's best to do this one at home. ALONE. Not much else to say on this other than I'm glad it's over.

How the test is performed

* On day 1, urinate into the toilet when you get up in the morning.
* Afterwards, collect all urine in a special container for the next 24 hours.
* On day 2, urinate into the container when you get up in the morning.
* Cap the container. Keep it in the refrigerator or a cool place during the collection period.
* Label the container with your name, the date, the time of completion, and return it as instructed.

Friday 5 December 2008

The Saline Suppression Test

Taken in October 2008

This was test I had to take before the adrenal vein sampling. It seemed easy enough and I was thrilled I didn't have to do the real salt load test which is done at home. That one sounded hard core with all the drinking and salt tablets, as well as the blood pressure monitoring with no medical staff to check on you. If you can, get your endocrinologist to give you a slip for the saline suppression test. It's done in a hospital where nurses will monitor you often and see that you are ok.

This is a test where spironolactone has to be discontinued for at least 6 weeks. I also had to stop nipedifine, my high blood pressure med, but only that day. Also, if you are a big black licorice eater, you have to stop eating it because it oddly throws off the test.

I went to St. Agnes Hospital of Baltimore to have mine done. If you go to a hospital, the procedures are usually performed in the the infusion centers of the cancer department. There will be people getting blood transfusions and chemo. It can be a bit daunting if you have never seen or experienced that. It was a good experience for me, everybody (including the nurses) was polite and quite positive.

The Test and What to Expect:

1) You will go in early (7:30-9:00 a.m. at the latest) and stay for about 4 hours. You go in early because that's when your aldosterone is at it highest.

2) After you are admitted, you will sit for approx. 20-30 minutes and then a nurse will take samples of your blood. They'll be checking for renin, potassium and aldosterone levels.

3) The nurse will then take your blood pressure.

4) You will get an I.V. put into the opposite wrist or arm from where they took your blood. You will receive a catheter for the saline infusion. You will be expected to sit, not lay down, for the entire time. You can however, sit back if you are in a recliner chair.

5) A nurse will administer 1 of 2 bags of saline (.9% Liter). It will feel really cold going into your veins. The nurse said after a while I wouldn't feel it, but honestly I did the whole time. I was freezing and got some blankets- you may need to do the same. I recommend layering with a sweater and even a scarf, too.

6) You will be allowed to drink juice, water or soda during the procedure, but be careful, they are adding a lot fluid to your system and you'll start to feel like you have to go to the bathroom within a half hour. You are also allowed to eat food, they serve lunch around 11:30-12:00 p.m.

7) You will be there for 4 hours to receive all of the saline from both bags.

8) You will go to the bathroom often after the 1st bag is in your system. I went about 15 times during my visit- no lie. Make sure you have a sense of humor and try to get a seat close to the bathroom... mine was across the room and it was a real pain carrying that I.V. apparatus.

9) After you are finished, the nurse will take you blood again to compare against the earlier sample. She'll check your blood pressure too.

10) You get to go home. When you leave you'll be extremely dehydrated, so make sure to drink plenty of fluids (for as much as they put in you, you emptied out, trust me). Also, you'll have zero electrolytes, so I recommend Gatorade, and take it with your potassium pills. I felt very weak after wards, and my joints hurt. I also had a horrible headache, so I just went home and crashed for a couple of hours. Hopefully, you don't have to go back to work, you'll be very unproductive.

11) Your endo will call you within a week with the results.

Thursday 4 December 2008

The Tests You Will Take

You may already be diagnosed with P.H. If so you will have to go through a very long series of tests before they can give you the proper treatment, which may include surgery. It can take less time if you have a super-endocrinologist who really understands Conn's and wants to help you get better fast. These types of doctors are rare and hard to find. And if you are like me, you will find yourself at the mercy of the doctors offices, not just your doctor, and let me tell you, those medical staff people can make or break you. They have far too much power for so little training. My endocrinologist's office has made numerous errors, including making appointments before having me get blood work or urine collection. And then they make you feel like getting an appointment (after they screwed up, mind you) is a privilege. It's not, and it is your right as a patient. Wasted time can cost you a lot when you are in pain and feel hopelessly off-kilter. You need to become your own advocate (see my below blog on helpful tips with that).

My second endocrinologist (I'm on my 3rd), told me if I didn't follow the order of the tests, it would seriously delay diagnosis and treatment. He was right on that accord. But what he forgot to tell me was what to do in the middle of all the tests. How do I get through my days when I can barely concentrate or remember simple things. Unfortunately, I had to take care of all that on my own, and it's been a very long, sometimes depressing road. If I may be so bold--I recommend going on anti-depressants while you are undergoing all these tests. It can take up to a year to make the right diagnosis and you need to take care of your emotional well-being in the meantime. I've never been a huge fan of them, but recently I feel they may have saved my life. Each patient is different so it's best to talk to your G.P., maybe not your endo because he's only there to deal with your endocrine system, not your head. However, if your endo is an empathetic person, it's certainly worth asking.

In the meantime, here is a list of tests that most endocrinlogists have you take before making a decision about surgery vs. a life-long commitment to medication. The tests can start out in different order with different doctors.

Hyperaldosteronism Tests:

1) Lots of blood work which may be requested weekly or bi-weekly at times.

2) Blood renin tests that are sometimes done in conjunction with the regular blood work.

3)CT scan. This is how they found my tumor, although if your doctor suspects P.A., he will have you take this to be sure.

4) MRI- A surgeon will often request this to see the tumor better (CT's can be rough).

5)Saline-Suppression Test, or Oral Salt Loading Test (it's usually either/or)

6)24-hour urine collection: The test is done to see how much aldosterone is released into your urine.

7)Fludrocortisone suppression test (FST). After you've followed a high-sodium diet and taken fludrocortisone — which mimics the action of aldosterone — for three days, aldosterone levels in your blood are measured.

8)Adrenal Vein Sampling: This is the most reliable test for determining the cause of primary aldosteronism. A radiologist draws blood from both your right and left adrenal veins and compares the two samples. Aldosterone levels that are significantly higher on one side indicate the presence of an aldosteronoma on that side. Aldosterone levels that are similar on both sides point to overactivity in both glands-(this is not great news).

9) Pheochromocytoma Urine Collection: A pheochromocytoma is a rare catecholamine-secreting tumor. Tumors that form outside the adrenal gland are termed extra-adrenal pheochromocytomas or paragangliomas. Because of excessive catecholamine secretion, pheochromocytomas may precipitate life-threatening hypertension or cardiac arrhythmias. If the diagnosis of a pheochromocytoma is overlooked, the consequences could be disastrous, even fatal; however, if a pheochromocytoma is found, it is potentially curable. You will take a urine test (pee in a jug for a day) to determine this.

There may even be more, but thus far these are the ones I have taken. I'm off to my endo tomorrow for results on the AVS and urine collection. Am nervous but hopeful that this will all be over sooner than later.

Tuesday 2 December 2008

The Effects of Corticosteroids on the Adrenal Glands

When I was 24, I was diagnosed with asthma. I was given a lot of different medications throughout the years, which included a multitude of various short-acting inhalers such as Albuterol, Proventil, Ventolin. Oral bronchodilators: Theo-Dur (theophylline). Long-acting bronchodilators: Serevent. And more recently, another oral inhaler: Advair. Having asthma made me more prone to lung infections and I've had many of those. Bouts of bronchitis and even bronchial pneumonia, were not that uncommon during the dry, cold winter or in times of severe stress. Each time I got really sick, my doctor put me on Prednisone- a drug I hated more than any other I have ever taken in my life. It did crazy things to me then, and now it seems likely that it continues in attacking my endocrine system. Luckily, I am almost asthma free, I rarely get an attack or experience symptoms...I now have another issue to deal with: Conn's Syndrome.

Prednisone is a corticosteroid, as are most of the above mentioned drugs. Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. They are involved in a wide range of physiologic systems such as stress response, carbohydrate metabolism, immune response, regulation of inflammation, protein catabolism, blood electrolyte levels, and behavior. While prednisone can be a great healing drug for many conditions, the side effects can be pretty heinous. The short term effects include: euphoria, insomnia, mood changes, personality changes, psychotic behavior, or severe depression, fluid retention and high blood pressure. The long term effects are: osteoporosis, diabetes, lowered sex drive, glaucoma and cataract formations, and adrenal glands problems such as Cushings Syndrome. Doctors do not recommend stopping the use of prednisone before seven days because it can have bad consequences such as bringing on symptoms of Addison's disease (another adrenal gland condition). Doses are reduced gradually over a few days with short term use and over a few weeks/months with long term use.

While Conn's has not been mentioned as a long term side effect, I still wonder if there is a correlation. There has been stress on my adrenal glands that's for sure. But I'm not a doctor and I don't know of all tricky terminology that would help explain my theory. I plan on asking my endo at our next appt. At this point, all I know is prednisone/corticosteroids help asthmatics like me everyday, and I am thankful for that. But sadly, after years of them being prescribed to me at the drop of a hat, I have to wonder if they are playing any role in my adrenal crisis.

Short Term Effects of Corticosteroids:
* Elevated pressure in the eyes (glaucoma)
* Fluid retention, causing swelling in your lower legs
* Increased blood pressure
* Mood swings
* Weight gain, with fat deposits in your abdomen, face and the back of your neck

Long Term Effects of Corticosteroids:
* Cataracts
* High blood sugar, which can trigger or worsen diabetes
* Increased risk of infections
* Loss of calcium from bones, which can lead to osteoporosis and fractures
* Menstrual irregularities
* Suppressed adrenal gland hormone production-such as Cushings Syndrome
* Thin skin, easy bruising and slower wound healing

http://www.mayoclinic.com/health/steroids/HQ01431

1.20.09 Update:
My G.P. feels there is no relation to corticosteroids and Conn's. I'm not entirely convinced and plan on asking my endo for more info later.