What Happens When Doctor’s Don’t Know Everything.

Over the last year one thing has become very clear.  Dr’s don’t always know everything.

We recently went to our Dr to ask about the fact that Derek was not feeling great.  After being told several times that he should not be taking extra Hydrocortisone just because he was feeling fatigued, or unwell, or had a ball of gastric wind under his ribs.

Because of this, he stopped taking the extra “stress dosses” of HC and began charting his BP instead.  After 6 weeks of readings at 4 times each day, we went to the Dr.

We found that his BP will go into a clinically Hypertensive range of 154/96 with a 68 heart rate one day and clinically Hypotensive at 94/59 HR 101 the next.  Just the effort of standing, and the change of position could have his pulse rate jump by anything from 40-60bpm (up to a 90% increase), where it should only increase by 20%.

Derek had been trying to get back to a semblance of fitness, so we organised through ACC, to have a schedule of fitness sessions with his Physiotherapist.  The physio was doing the starting assessment, and found out about the problem with his BP and would not touch him without clearance from the Dr.

Armed with the readings from the previous 6 weeks we went along to the Dr to ask if he knew what was happening and why.  The first thing he did (and all kudos to him) was to admit that Derek was the one and only “Addison’s” Suffer he had ever dealt with, and didn’t know that much about Adrenal Infarction and the complications of it.

He looked at Derek’s medical records.  The Endocrinologist had written that at some point, and probably without warning, he would need his fludrocortisone increased.  So it was decided that perhaps a change of Fludro from 0.05 to 0.1mg per day could work to stabilise it.

Home we went, armed with the hopeful knowledge that it was the fludro out of sync that was probably causing the large difference in BP.

We tracked his BP for another week.  There was definitely a change in pressure and heart rate.  It continued going higher during the day, but falling further overnight.  It had also become more erratic.

OK, so 0.1 wasn’t the answer.  So go back to 0.05 but twice a day.  We had seen on various forums where others had had some success with this option.

Another week of tracking and another change in BP (or not).  Still heading upwards during the day and downwards at night and still no real pattern for week days and weekends.

We returned to the Dr with this new information.  He agreed that the extra fludro wasn’t doing anything positive so we should go back to 0.05.

He also had the results of some blood tests.  There was nothing markedly different from last time.  Kidney’s still not at a great level, everything else seemed fine.  Potassium and Sodium all comfortably within range.  Thyroid a little wonky but he has no adrenals so it is not unexpected, and not sounding an alert.

The Dr agreed that it was time to refer him back to his Endocrinologist and to a Cardiologist.  So we now wait for an appointment.

While we wait however, we don’t just sit here doing nothing…..

And the mystery continues.

Along with his BP and HR issues, he also can’t take a fright/shock or be startled now without “crashing”.

We decided we needed to get to the bottom of it.  Thinking it was BP related we went out on Saturday to go for a drive, taking the BP monitor with us.  One of two things was going to happen.  Either nothing would happen to cause him an adrenaline rush, or someone would do something to force me to break quickly and sharply, which would give Derek a fright, and I would get an adrenaline rush.

Thankfully the latter happened.  Derek was on the phone, and didn’t see the car in front of me that started to pull into another lane, and then started driving in both lanes, blocking everyone.  I had begun to speed up as we were going 20-30 km under the speed limit.  A little late, I realised he wasn’t pulling all the way over, and I braked harder than intended.  I got a little adrenaline rush as I thought for a brief instant, that I may hit him.  I felt my heart start to beat a little faster and harder.  I got a tingly feeling around my chest.  I am sure we have all felt it.

I asked Derek how he felt.  I could tell immediately that he hadn’t had an adrenaline rush.  It was obvious that it was going to be another drop.  I continued driving for about 2 minutes, until I could find somewhere safe to pull over.

We took his BP and pulse.  It was perfectly normal.  Almost TOO normal. His pulse was in the low 60’s.  Yet we could see, and he could feel, the usual symptoms.  Headache, feeling slightly ill, couldn’t keep his eyes open, felt weak, his speech went quiet, it slowed down.

We continued home (5 minutes away) and checked his BP when we drove in the drive (before he got out of the car).  It hadn’t moved.  His pulse was the same and his BP was within 2mmgh/l of the first reading for both systolic and diastolic.

We continued checking it every 10-15 minutes for an hour.  I had to wake him to do it as he went to bed and just lay there, unable to talk, or really communicate.  Still there was no change in his BP or pulse.

We are none the wiser.

So we are going to try his glucose levels next.  The problem is, we have to wait to see what happens with a fright.  Unfortunately I can’t just make a very large sudden noise and frighten him myself, as tempting as it might be.

We have done a lot of reading on the topic of the Adrenal Medulla and epinephrine/norepinephrine.  There are definite links to these hormones and stress.  There doesn’t seem to be a lot of research into what the effect of not having an abundance of these hormones does.

Adrenal medulla

The inner part of the adrenal gland is called the adrenal medulla. The adrenal medulla produces hormones called catecholamines such as adrenaline and noradrenaline. Catecholamines play a role in the response to acute or sudden severe stress, for example during life threatening event.

Catecholamines are responsible for the palpitations (racing heart), sweatiness, widening of eyes and shakiness of the hand when faced with sudden fear or other stressful situation.

This information is all over the internet.  What we can’t find is what happens when this process doesn’t happen properly and you don’t get the palpitations, sweatiness, etc with sudden fear or stress.  Does the body skip to the next section (rest and digest), or is it part of the process fails but another part kicks in, and then is not switched off because again, the Medulla has failed it’s job.

It is very hard when you have a rare condition, one that is so rare that little research has been done.

If we could find an answer to what is happening when Derek faces the sudden stress perhaps we could find a way to reduce the impact but the Dr’s don’t know and we don’t know how to get them to investigate.  In the meantime, we are having to do it ourselves.

Dr’s don’t know what to do with Derek.  If anyone has an answer please let us know.

The Holiday Continues

Day 3 dawned clear.  We drove to Hamilton.  A nice quiet, uneventful drive.

Day 4 was a quiet day.  I went for coffee and cake with my girlfriend.  Derek went out with her husband, who was building a deck for his daughters new house.  Robert is not in the best of health and so was working very slowly, making sure he didn’t injure himself.  His pace of work appeared to be the same as Derek, small amounts, resting lots.  They had done work together before, when they were both fit healthy men.  Things had changed dramatically.

Derek managed an hour of very light work in the 3 hours he spent at the house.  He stopped at morning tea time for a long break, then did a little more (holding timber and screwing in bolts).  He took an extra 5mg of HC because he could feel himself using what he had in his system.  By 1 pm, when Roberts son in law came to help out, he bought Derek home.

Derek immediately lay down and had a sleep for 2 hours.  His BP was stable, but he was extremely fatigued from the little work he had done.

His brother was due to come around and see him.  I had insisted that David come to us, as Derek did not have the energy to go and visit him.

I left Derek and his brother to talk.

One thing we have learnt in the last few months is the difference between fatigue (which he suffers from regularly) and the beginnings of a Crisis.

Saturday he decided to help Robert again.  This time he did a lot less work, and lighter work.

The kitchen was due to be finished so once it had gone in, we went round to see how things were going.

When we arrived the three boys were sitting relaxing.  They had been doing so for a while.  Derek was again looking very tired.  He took 5mg of HC again to keep himself going.

Once we had finished chatting, we took Derek home.  He needed to again just lay down and sleep.

We were due to go out to dinner that night so I insisted that Derek have a sleep.  He also took more HC at 4 so he would have the energy to go out that night.  Our table was booked for 6pm.  We were home by 9.15 and Robert and Derek both went straight to bed.

Once upon a time, when we went out with Robert and Adele, we would be out till midnight, then home to have a coffee or wine.  Adele and I were left sitting up alone.  Neither boy was able to stay up.  Derek was absolutely exhausted.

We used to live in Hamilton, so Sunday we stayed in bed late then went to catch up with some more friends.  They invited us to go to a restaurant with them for lunch.  It was great food and great company.  Derek’s main comment afterwards was how tired he was.  He found it very hard to focus on conversation and to keep himself functioning.  We got home and again he needed to lay down and sleep.  Sitting in a restaurant for lunch, not even a noisy one, was hard work.

What we had learnt over the last few days was that our social life, as it had been, was no longer.  It was like Derek had suddenly become an 80 yr old over-night.  Late out of bed, short visits, sleeps in the afternoon, to bed by 8.30 at night even with extra medication.

Adele and Robert had their daughter and son-in-law around on Sunday night for dinner.  Derek slept that afternoon, once we came home from lunch.  Dinner was set for 6.30.  That was good, because no sooner was Dinner over, Derek went to bed.  He was up till 8.00 and he could hardly keep his eyes open.

Monday of the second week was a nice sunny morning.  It was cool, but bright.  The weather was on our side.  We sat around relaxing till 9 am, then decided to just go for a walk.  It was a slow, flat walk, chatting, looking at houses.  Total distance was around 3km (nowhere near the 5-6 we try to do once a week).

As we got to the 2.5km mark Derek started burping.  This was not his normal, drink water, burp bubbles up.  This was his “I have gastric pain under my ribs and I need to release pressure” burps.  Those burps he does when he seems to be going into crisis.  He also started walking a lot slower, and reduced the amount he was talking.

He had taken his HC later than normal today, so was only 2 hours into his meds (the best time normally for him).

When we got home Derek just wanted to sit down.   He did this for 10 minutes, then took his blood pressure.

It wasn’t good.  106/72, hr of 75bpm.  He then decided, because it was so low, to do a standup bp.  It dropped to 99/68, hr was 90.  He had been using an electrolyte mixture to drink over the last few days, and had not over drunk on the walk but with the onset of reduced brain power (brain fog), gastric pain, and low BP this was definitely the start of a crisis.

We didn’t do any extra medication, he had some salty food, and more electrolyte to see what would happen.

He also went and lay down for the rest of the day.

We went out for dinner with more friends that night (again a 6 pm dinner).  It was again a home by 9.00 pm evening.

Tuesday was a day to stop, do nothing, totally relax.  We went for a coffee but the rest of the day was rest for Derek.  We had done nothing compared to what we would normally do when visiting Hamilton, but it seemed to take it’s toll on Derek.  We had the drive back to contend with the next day and I was a little worried that even though he hadn’t done anything it would be too much.

Wednesday morning was a damp day.  There was a storm brewing at home (rough gale force winds, heavy rain etc).

We decided to just drive as far as I could drive.  We stopped for lunch in Taupo, then carried on south.

We stopped again for coffee in Taihape as we didn’t stop there on the way up.

The trip home was incident free but I was getting tired.  We arrived in Levin and I decided to pull over and find a Motel for the night.  The storm was coming up country to meet us, and I was too tired to want to drive through it.  We were only 1.5 hours from home but when I was the only one driving, it was 1.5 hours too far, especially when it was going to be so wet and windy.

It turned out to be a good idea.  We picked up some groceries from the nearby supermarket, and then just relaxed in the unit.  The weather that night was very loud, windy and extremely wet.

Thursday morning we woke to rain, wind, and lots of traffic.  We drove a short distance, to another small town, and stopped for a morning drink while we waited for the business traffic to end.  Then we drove the rest of the way home (well I drove, Derek just sat there).

Again, the drive was incident free and we were home an hour later.

It was good to be home because it meant Derek could basically go to bed and get over the trip.

It was nice for him to get away and pretend (for a short time anyway) that he was “normal” although it didn’t last long because he couldn’t really do anything.  For me however, it was very much a “busman’s holiday”.

I spent most of my time making sure he didn’t overdo it, and then looking after him when he did.

Oh for want of a holiday.

The one thing we confirmed is that driving is not a good idea for Derek.  If there had been anything happen when he was driving, we could have been in serious trouble if he couldn’t pull over immediately.  This is another lesson learned.