So How Are You Really?

In October 2018 I was diagnosed with adrenocortical carcinoma (ACC). I went through surgery, radiation and chemotherapy. I was off work for about 3 months.  

Since then, people will ask me how I am doing. I don’t feel particularly eloquent or able to answer them. Sometimes I am brief and don’t give much information. Other times I try to express the complexity but midway through the explanation I can sense that I am saying more than what they want to hear. 

I am going to try to come up with the appropriate balance in writing here. I hope this is interesting. I also promise to be completely honest. I have been surprised with this blog that when I am transparent, others tell me it has helped them. 

My scans are NED

I am currently without evidence of cancer. The term that I have learned since my diagnosis is NED (no evident disease). This is a euphemistic way of saying that while the scans show no evidence of cancer the doctors are not willing to say that I am cancer free.  ACC has a tendency to recur. Some cancers have a time frame after which recurrence is very unlikely. With ACC this this time frame is longer. Some have had recurrences even after years of being NED. With each scan I am reassured that there is no cancer evident. As I get further and further out there is increasing confidence that I truly might be free of this cancer. But for now, the cancer experts have insisted on full body CT scans (chest/abdomen/pelvis) every 3 months. 

At 2 ½ years my scans are NED, and I am very thankful for this.

I have primary adrenal insufficiency (PAI). 

I will define a few terms first: 

• The adrenal glands sit on top of the kidneys. “Renal” means kidney so “ad-renal” means the glands next to the kidneys. We are all born with two adrenal glands (one on top of each kidney).
• They are responsible for producing adrenaline (“adrenal”-ine). 
• They also make cortisol hormones (hydrocortisone and fludrocortisone). We refer to the cortisol hormones as steroids (or as corticosteroids). 

I had my cancerous left adrenal gland removed. For a period of time, I took the drug mitotane to further eliminate any stray cancer cells. This drug destroys adrenal tissue and blocks the effect of the body’s corticosteroids.  When I started taking mitotane I also started taking replacement doses of hydrocortisone. It was understood that I was getting by with only one adrenal gland, and that remaining adrenal gland was going to get damaged and suppressed by the mitotane. I would need replacement steroids (hydrocortisone) to compensate.

Once I stopped taking mitotane the hope was that my remaining right adrenal gland would recover (wake up) and start producing hydrocortisone again. In early 2020 I slowly weaned off of the replacement hydrocortisone tablets that I was taking. The plan was for me to reduce the dose by a small amount every 3-4 weeks. Each time I would reduce the dose I would have a heavy dose of PAI (primary adrenal insufficiency) symptoms. After a few weeks I would then reduce the dose further and go through the cycle again. By May of 2020 I was off of hydrocortisone. 

And I was really struggling.

PAI can cause a variety of different symptoms. My symptoms are mostly fatigue, diffuse muscle aches (myalgia), dizziness, and brain fog. 

Others with adrenal insufficiency have not only those symptoms, but will also have muscle weakness, loss of appetite, nausea, abdominal pain and a variety of other symptoms. Some persons with AI produce no hydrocortisone. They are at risk for an adrenal crisis. When this happens, they can have very severe life-threatening symptoms. These can including abrupt episodes of non-stop vomiting and very low blood pressure, and potentially even death. 

I am fortunate in that my remaining adrenal gland does seem to produce some hydrocortisone. It doesn’t make quite enough, but it makes some. For that reason, I am thankful to not have had the really severe episodes of adrenal crisis like other patients have. My remaining adrenal gland does appear to be a bit of a safety net for me. For this fact I am very thankful.

And so, my symptoms are mostly fatigue, myalgia, dizziness, and brain fog. 

The odd thing is that my symptoms are widely variable. The symptoms come and go throughout the day. In any given day (like today) I can go from feeling really crummy to almost feeling normal and then various phases in between. The symptoms can vary from day to day or even week to week in intensity. Stress and workload have a big impact on me. 

The fatigue is a heavy tiredness. It is similar to what I have had after a long call night without sleep. It is the feeling of being so tired that you wonder if you are too tired to sleep. I feel the need to breathe heavily when I lay down to rest. It feels like I am so tired that I am a little bit short of breath. It was a similar sensation to what I have had after an overnight flight to Europe without sleep. It is a heavy fatigue along with feeling like you are walking in a bit of a fog.

The muscle aches are a bit like having the flu. In the morning I often joke with myself that I wonder who beat me all over my body I slept. That is the most common type of muscle aches that I feel.  At times there is a different type especially if I am really low on steroids. This is an odd aching or burning discomfort that I can only describe as something akin to a toothache sensation but in my arms or legs or back. It is a persistently present pulling and burning like sensation. It is strong enough to be distracting but not enough for me to want to take pain medicine. I find myself saying “Ow” under my breath but then still moving on with my day and activities.

The dizziness is a complex and mixed sensation. It is partially the sensation that I might pass out. The medical term for a faint is syncope and therefore the term for a pre-fainting feeling is “presyncope”. When I am tired or low on steroids, I have to be very careful with change of positions. At times I do start to feel my vision graying. I can get the strong sensation that if I don’t stop, I will indeed pass out and fall to the floor. I have had to abruptly drop myself to my knees or lay on the floor a few times to prevent myself from passing out. Each of these times it was usually because I had pushed too hard. Normally I had not had enough water and salt while I was doing something in the heat. 

The dizziness is also at times the sensation that I might fall over. In medical terms I call this “disequilibrium.” This is the sensation that you might drift or fall to one side as you walk. I brace myself carefully to not lose my balance. This dizziness I have learned is most often a sign of fatigue. It improves if I am able to take a nap or rest.

Brain fog is clearly a post-call (or a jet-lag) type of sensation. When I have gone a long period of time without sleep I have learned that I can still function, but it takes a force of the will to do so. In other words, things that are normally easy require effort. For example, if I need to transfer some numbers from one page to another I have to be careful to do so. I can’t just trust myself to hold them accurately in my brain as I am easily distracted. Playing cards with my family the other day I struggled to deal the cards. I had to force myself to focus to do so. I could still logic through strategy and play effectively but the simpler things were a challenge.

The hard part of these symptoms is that they come and go. Often I will notice that I feel kind of normal. Classically this is at 9:40 am. I often look at my watch when I am feeling well and it is uncanny how often for me I see it is 9:40 am. In those times I question myself and chide myself that maybe I am being a bit of a hypochondriac. Maybe I am blowing things out of proportion? I tell myself that I am fine, and I had better just start focusing on the positive. I question whether I am just wanting to slip back into a sick role to avoid the stresses of life (that we all face.) Is my problem purely one of attitude or even just stress or low-level depression?

Later however the symptoms will hit me hard. They are obvious, significant and real. In those moments I want to despair. It is not that the symptoms are so overwhelming, but the frustration of their regular and unending return makes me wonder how long I can endure. I end up not being able to do things that I want to. I end up not wanting to do anything. At times I find myself envying people who have energy. 

In June 2020 I started back on hydrocortisone replacement at low doses. It wasn’t enough. The symptoms were initially better and then became a problem again. Later my doses were increased. 

After one increase in dose, I can remember feeling great hope. For the 1^st time in months, I felt good. It felt like walking out of a fog. I can remember feeling like I could see clearly for the first time in weeks.  Life felt like something to be challenged and enjoyed.  

It didn’t last long. I later had a return of the symptoms despite staying on the same dose.

The goal is to get just enough hydrocortisone replacement but not too much. Too high of doses can cause weight gain, loss of bone mineral density and a variety of other problems. Observational data shows lower life expectancy in patients with PAI and some think that excessive steroid (hydrocortisone) replacement can be a part of this shortened lifespan. I am trying to be responsible and not take too much steroid and yet still figure out how to live my life. 

I have also been put on fludrocortisone replacement. This hormone regulates salt and water retention. When I first went on fludrocortisone I noticed my blood pressure went up a lot (too high). I stopped the fludrocortisone and instead tried to get by with a high salt diet and high fluid intake. I live on Gatorade Zero plus a variety of salt additives to my drinking water. It is ironic for me as a heart failure physician who constantly preaches salt restricted diets that I have to stop halfway through may day to eat or drink a big serving of salt. The high salt diet helped but not completely. I now take ½ of the smallest fludrocortisone dose and still push salt and water intake. This has helped with the presyncope sensation (feeling like I might pass out.) My blood pressure is higher but within acceptable bounds.

I do better when I am not working.  

I have been forced to learn lessons about my life. I didn’t want to learn them. I was happy being the guy that could just push through. For years I set a personal goal to get at least 5 hours of sleep per night. Things have changed for me.

I began to realize that my life as an AHF (advanced heart failure) cardiologist involves long hours and a lot of stress. An average day will be from 7 am to 6 pm. A hard day can run from 7 am to 10 or 11 pm and still have the potential for middle of the night phone calls. 

I also had to realize the impact of stress on me. I always prided myself on my ability to handle stress. I remember being 19 years old and working on the ambulance. We transferred a patient who was in the middle of a heart attack to a heart center. The cardiologist met us in the hallway. Everything was moving fast and crazy around us, but he was calm and controlled. By his voice he soothed the patient and his family. He was able to absorb the stress of the moment and not show it. That moment that night had a big impact on me. It molded how I practice medicine to this day.

I have always been proud about my ability to triage and handle the barrage of emergencies all around me. Each day involves life and death decisions and life and death discussions with my patients and their families. Before I just accepted and absorbed all of this stress and dealt with it. Now this stress drains me. By the end of the day, I am often exhausted. Or – as I described above: fatigued, achy, dizzy, and working against brain fog.

The problems that I have learned about are then:

• The long hours
• The emotional burden / stress on me of the type of work that I do.

I tried pushing the steroid doses higher. I hoped that if I could just get to the correct dose I would be ok. Maybe I could get back to “normal.” In the back of my mind, I worried however that I was doing something very wrong. It felt like I was going to destroy or sacrifice my body for the sake of sustaining a bad lifestyle (too much work and stress). If a car only had lower gears so that it couldn’t go 80 mph is it really right to keep revving the engine into the redline zone in order to sustain that speed? 

In the end the extra steroid helped but still didn’t seem to be enough. 

The answer became clear: I need to change my work lifestyle.

That is harder and more complicated than it seems. 

There are external demands and pressures on me. There are however also internal expectations and pressures that I put on myself. Trying to remake myself is not easy. Part of me doesn’t want to change anything. And part of me can’t just “work light”. I have distinct performance expectations for myself. I am not happy if I don’t meet them.

I went to the adrenal insufficiency Facebook groups to read what they had to say. I searched to see how many others with PAI were disabled and how many were able to work full time. What I discovered was wide variability. There are a lot of people who end up on full disability. On the opposite extreme there are a number of people who live pretty intense and full lives. The majority fall somewhere in between. Most have frustrations similar to me. They cannot do all that they want and frequently hit a wall of fatigue and similar symptoms to me. Most end up being forced to learn to live with some limits on their life.

I have spent the past several months soul searching to understand what changes I should make. I continue this process now. I am stepping out of my leadership roles at work. I hope that this will help. I won’t have to carry the stress and burdens of helping to lead the programs. I won’t have the background constant “2^nd job” of leadership that runs at the same time as I am seeing patients. Probably more important is that I won’t have the stress of my own personal expectations for myself in those roles. I hope I will have enough energy to see and care for my patients and still have some energy left at the end of the day. I also am excited to let my younger, talented, and more energetic colleagues have a chance to “put their stamp” on the programs.

There are a variety of other changes coming but the point is that I am seeking to reduce the external demands on me, and at the same time give up roles in which I find myself putting significant internal expectation and demands on myself.

So. How am I?

My CT scans are NED. (No evident cancer.)

I have PAI (Primary adrenal insufficiency).

This means that I don’t have enough energy and in spite of my best efforts to find the right steroid replacement doses, I am going to not have as much energy as I would like to have. I have some energy. I can do things. I am not an invalid. There are times when I feel pretty normal. There are other times when I don’t. 

I am working on making changes for a sustainable future. 

My work (Spectrum Health) and my leadership is being wonderful and supportive of me as I work to figure this all out.

I am ok.

God is good.

I am walking through this all in the best way that I can.

My patient came to the office with his wife. As usual we talked about how he is feeling. He and his wife talked about a few episodes when he didn’t do well. He was pushing his limits too hard. His heart condition would not allow him to continue to live the kind of life that his mind wanted him to live. 

I started talking to him about learning to listen to his body, setting realistic expectations and making smart choices for how he structured his life now.

He didn’t know it but there were two patients in the exam room that day that were thinking through all of those lessons.