Category: Updates on my health

Updates for my family and friends on my current health and the treatment of my adrenocortical carcinoma.

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Being human Updates on my health

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 1st 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 “2nd 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.

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Being human Reflections on Life, Being Human, and Medicine Reflections on the Christian Life Updates on my health

Climbing the Rock Wall? What Do You Do When You Can’t Anymore? How Do You Deal with Limitations?

There is something that only really my family knows about me.

I was born without my right pectoralis major muscle. 

This is one of the muscles in the upper chest and front of the shoulder that helps you to move your arm. The doctors told my parents that I would have things I would not be able to do. They didn’t think I would be able to throw a ball. My parents opted to treat me like everyone else. I had a happy childhood. I knew that I was missing the muscle, but I still played. I may not have had a strong throwing arm, but I could throw a ball. To be honest I held back a bit on sports knowing that to try to be a pitcher or a quarterback was not likely a good thing for me. I still was able to do a lot of what I wanted to do. 

I applied for the Navy Health Professions Scholarship Program in my senior year of college. This program would pay for my medical school tuition and give me money to live on during medical school. In return, I would serve 5 years as a Navy physician afterwards. It was a great deal and a way to avoid going into the massive debt that a lot of medical students accumulate. 

In the fall of my senior year of college, I filled out the applications and went through the required interviews. I drove to Detroit and spent a day at the MEPS (Military Entrance Processing Station). This was a day of walking around in my underwear with a group of other men getting their physical examinations hoping for entrance to the military. In spite of my missing muscle, I passed the physical. I got accepted into the program. A few weeks later I raised my hand in front of a picture of Ronald Reagan and an American Flag and swore to defend the constitution of the United States. Suddenly I was a commissioned officer in the United States Navy.

Pushups. 

I was unable to do a pushup. The missing pectoralis muscle is key to be able to push your arm forward. I had never been able to do a pushup. The Navy physical fitness standards required me to do pushups. The next year I was going to my 6-week officer training course. There I would be required to pass the physical readiness test (PRT). I anxiously looked up the requirements. In order to pass I needed to: touch my toes (✔️), run a mile and a half in under 10.5 minutes (✔️), do 60 sit ups (✔️), and do 26 pushups (🚫).

Twenty-six pushups. I couldn’t do one. But they had already accepted me. I guess I would figure it out as I went along.

I went through my first year of medical school on the scholarship program (’87-’88). They paid my tuition and gave me $650/month to live on. I registered for my required officer training course for the next summer. In the fall and winter of 87/88, I decided I had better start trying to work out. I had done some basic weight machines before but had never worked to be able to do a pushup. 

My friends, John and Erlund, were an immense help. Erlund had a full set of weights in his apartment. He invited me to come and work out with him. As I did, he encouraged and pushed me. I started lifting more and more weights. Leaving his place after a workout I would feel like I was barely able to drive my home. My arms would feel like jello after the workout he put me through. Eventually I was able to do a pushup. Later I could do a couple of pushups. They were not pretty. They were mostly a pushup that favored my stronger left arm (the arm that actually had the muscles I needed to be able to do a pushup). As time went on I could do more and more. I still couldn’t do 26.

In May 1988 I went to my officer training. I learned how to march, wear a uniform, salute, and run a floor buffer. I also continued to exercise. Every morning I worked to do pushups. 

By the end of the 6 weeks, I did it. I passed. I did twenty-six pushups. They weren’t pretty. But I did them. And interestingly, once I did, I passed every semiannual physical readiness test for the duration of my time in the Navy.

Success.

That is a great disability narrative. It makes for a nice story. The narrative is of a challenge and the ability to overcome through the help of friends, persistence and hard work.  This sort of narrative makes for great inspiring posters. 

Picture a sheer rock face with the person with powder on their hands, 2/3 of the way up straining, sweat beading on their tense muscles. The caption reads, “Never, ever, ever give up. Success is just a little bit further up!” 

It is true. I learned that it is possible to push yourself and overcome limitations.  Sometimes we fail because we believe that we are going to fail. If we try to push through we often can find success on the other side of the barrier or limitation. In this I learned some key skills on how to overcome.

Now I am on a different side of disability. It is a different story. It requires some different skills that are hard to learn.

I have often wondered when I see my aging patients what it is like to be living during a subtraction phase of life. There seems to be a time of life when things start to be taken away from them. This can include strength, mobility, and even cognition (the ability to think). 

This week I saw a patient who told me that he is not as smart after his cardiac surgeries than he was before. “I used to be really smart. I am not anymore.” I started to talk to him about potentially going through speech (cognitive) therapy. He stopped me, “It is ok, doc. I am retired. I get by just fine. I don’t have to be so smart.”

I saw another patient who was in a wheelchair. At home she uses a walker. She remembered being fit, vigorous, and active. That is gone now. I don’t see that she is ever going to regain that lost strength.  She is having to learn what it means to live and find enjoyment in a life in which much of who she was before has been permanently taken from her.

For me it is more subtle. I can hide it. I can look normal. But in the room with each of those patients I felt very lightheaded, tired and achy. My symptoms are better now that I am on a higher dose of hydrocortisone. But I remain not normal. At the end of that day, I was exhausted. It was not a hard day. It was a normal day. But yet there I was struggling to type my last patient’s note. At the same time, I had an add on end of day virtual meeting. I dialed in but was thankful that I could leave my camera turned off. I could hide and mostly listen. I rallied my energy and chimed in when appropriate. I pretended to not be so tired. I got through the day.

It makes me understand my “subtraction phase of life” patients better now. 

There are a couple of narratives that go along with disability. One is like my 1st story. It is a story of victory and overcoming. These are the stories that we like to read. Another’s disability and then their overcoming can create nice and inspiring stories for us. It makes for great posters.

But there is a second narrative.  

The other narrative is not one that people like so much. It is not as pretty. It is a narrative about having things taken away from you. You feel robbed. You do not have the ability to overcome. 

Sometimes – trying harder doesn’t help.  Sometimes trying harder hurts.

In Norman Jester’s book, “The Phantom Tollbooth”[1] the main character Milo travels to all sorts of mysterious  lands where things are not what you expect. One place he goes is a place where they serve subtraction soup. In that land you do not eat to get full, you eat to get hungry. If you do nothing you eventually will start to feel full. Once you feel full, you eat the subtraction soup to feel less full. “I am so full! I better eat some subtraction soup.”

Milo arrives to this land very hungry. He gladly takes a bowl of soup and eats heartily. Unfortunately, the more he eats, the hungrier he becomes. At the cost of extreme hunger pains, he learns that he must do nothing. “Don’t just do something, stand there,” is the twist on the common expression.

Sometimes great effort is like subtraction soup. Sometimes trying harder and harder does not solve the problem. It only makes it worse.

How do you deal with a subtraction phase of life? How do you deal with loss or when things are taken away from you? How do you live within the narrative where you cannot by force of will or effort overcome your disability or problems?

I see this process play out so frequently with my patients. Over time I see them learning the lessons. Like Milo, the skills that are needed in that phase of life are often very different from what has brought them success earlier in their lives. 

The first skill is being able to get an understanding of the new normal. 

In my mind I have the same abilities and stamina as before. Symptoms always wax and wane. Having good days and bad days is the norm for everyone. On a good day, I imagine that I am back to my normal (and younger) self. When I have a bad day, my hopes are destroyed. The hardest thing is to get a realistic understanding of what I am or am not able to do. 

Getting to an honest assessment of oneself is often very hard. I think many of us tend to fluctuate between a sort of “Walter Mitty” superman view and then a self-denigrating “not good for anything” view of ourselves.  The truth of course is always more measured. Within me there are great things, average things and not so good things. Within my “new normal” the same is true. There is a lot I can do. In my bad days I get frustrated and childishly think that I can’t do much of anything. On my good days I want to slip back into being a full force young and driven man. The real truth: my life isn’t bad. I can do a lot. I can’t do everything. Getting an honest understanding is the first and key step.

The second skill is learning to accept reality and figuring out how to live within it. 

This might involve grieving in order to accept the loss. In other words, the classic Kübler-Ross stages of shock, anger, bargaining, denial and then finally acceptance.[2] The point is that with every type of loss comes a process where we need to work through it and come to a point of acceptance. 

Some patients are amazing at how they can do this. Others really struggle with it. The harder they try to become what they were before the worse they get. We are trained to tell patients in chronic pain that their focus should not be on trying to be completely pain free. Instead, they should focus on trying to regain function in their lives in spite their pain. The experts tell us that in doing this their pain often gets better. But if they focus on their pain, especially on trying to be completely free of pain, it often gets worse.

Sometimes they eat the subtraction soup.  The harder and harder they try to make themselves better, the worse they get. It is a not an easy lesson for them. It is often something that you can’t tell them. Over time they have to learn to accept their situation and stop trying so hard. They learn to shift their focus onto trying to make the best of where life has put them.

I saw another patient who has significant ongoing symptoms[3]. He told me he has 2 good days, 2 bad days (= stuck in bed) and 3 in between days each week. He has amazingly learned how to live within this illness and find happiness in his life. It doesn’t mean that he doesn’t have limitations or severe symptoms. It doesn’t mean that he has given up on seeking better treatments for his condition. It just means that he has worked through the process of understanding and accepting his situation. Having done that, he can enjoy the good days and figure out how to cope with the bad. 

The third skill is having the strength and insight to set up a structure for your life around the disability or limitation. 

This means finding a way to have a sustainable life and one that makes sense within what you are able to do. This too is a hard task. It means saying, “no” to things. It means setting priorities. I tell my patients about energy conservation. I tell them to intentionally choose what they want to do with their energy. If they can’t do everything, what do they want to do? I often joke that young men exist to do yard work so they don’t have to.

They can instead go out to breakfast with friends. Or they can be there to go shopping with their spouse.

I continue to struggle with this. If I can’t do everything I did before, how do I structure my life now? It is a lot easier to say “yes” than it is to prioritize and selectively say “yes” and “no.”

Those reading this who know me may want to know – what is my current reality? 

In early 2020 I worked to wean off of my supplemental steroids. Starting in May/June 2020, I really have struggled with adrenal insufficiency. The symptoms are a combination of fatigue, diffuse muscle aches, lightheadedness, disturbed sleep, and a variety of other symptoms. If I try to just push through the symptoms, I get worse. Instead, like Milo, I found that I had entered a strange new land in which the rules were different. Attempts to push through end up being subtraction soup and make me worse. Over time, with the help of increased hydrocortisone doses, a carefully regulated schedule, and some structured exercise, I have improved some. 

Getting COVID was hard. Fortunately, I made it through that. I was absolutely exhausted. I am back now to my usual adrenal insufficiency symptoms. 

Milo eats and then eats more of the subtraction soup. All it does is make him hungrier. Eventually he learns the secret is not to eat, but to not eat.  Milo has to learn to do something that is not easy. It is to do nothing. It is to accept his circumstances and wait.

I am not good at that. I am impressed by my patients and friends who can do it. They can somehow find peace and joy in spite of the limitations that have been put on them. The apostle Paul said that is the secret:[4] Learning to be content in the circumstances in which  you are. 

Maybe that is worthy of a big poster on our walls? I can see it now. Big sheer rock face with ropes and gear and people with powder on their hands struggling up the wall. Seated at the bottom on a folding camp chair is another person. This is a person who can’t climb the wall to the top. But this person is quite happily camping on the bottom. Maybe they are even roasting a marshmallow or two. “Be content with such things as you have,[5]” is written in big print along the bottom.

So how am I? 

A little confused still thank you. Trying to figure out how I am and what my limitations are and what I can do to make them better. When do I push and when do I rest? In the process I am trying to learn to accept, be content and enjoy and appreciate my life. 

Care to join me for a toasted marshmallow and a camp chair?

[1] The Phantom Tollbooth, by Norman Jester, Random House, 1961.

[2] On Death and Dying by Elisabeth Kübler-Ross, Scribner, 1997.

[3] For patient privacy I am intentionally vague about the condition.

[4] Philippians 4:11-13

[5] “Let your conversation be without covetousness; and be content with such things as ye have: for he hath said, I will never leave thee, nor forsake thee.” Heb 13:5, KJV