Still a Single Road at Times

Aside

In 2013, my second post was very short.  It was entitled “As single road at times”.

My posts back then were very short.  They were a lot about how I was feeling, how we were learning to live with this condition strange new called Primary Adrenal Insufficiency (Addison’s Disease).

It was May 2013 that I began to Blog.  In the last 6 years a lot of things have changed.  But a lot have stayed the same, we have just learned to accept them and adjust to them.

Going to live performances, etc alone has become a new normal.  I buy 2 tickets, and find someone to join me if Derek isn’t up to it.  This could be a big show, or one that our children are performing in.  Adjusting things so that we can go together has also become a “thing”.  Whether that is by buying a matinée performance ticket instead of a night time one, or making sure he has a full day of rest, including a sleep, we will do what we need to, to keep going.

In May last year Derek had a set back.  It was serious but it wasn’t obvious.  A few blood tests went haywire for no obvious reason, he became a lot more tired than normal, and other subtle differences occurred.  Despite this we carried on with life.  We continued planning our big adventure overseas.

We did the trip.  It was extremely tiring on Derek.  We did a lot of walking with him using a walking stick constantly.  We would walk, stop for coffee, walk, stop for a cold drink, walk, stop for a few photos, walk, stop for an ice cream (they make great ice cream in Europe).  You get the picture.

Derek has been more tired than usual since getting home.  It’s now 6 months since the trip and he is still a lot more tired than he was before May last year.  So we have made more adjustments.

We have learned from that trip that you have to choose the fight.  So now, if we are going out and we know it will be stressful, or very tiring on Derek, we use a wheelchair for him.    After borrowing one for an evening we knew would be too hard on him without, he finally agreed that he really did need it.  But not all the time, just for the harder events.  It means that sometimes he can go out, and we can enjoy ourselves together.   I still go out on my own because he is not up to it.  Or we cancel our plans because I don’t want to go alone.

What we thought was just residual tired from the trip appears to not be that at all.  After having more “crazy blood tests” we began to wonder.  We have now looked at his bloods for the last 12 months, what ever is wrong, has been wrong since his various blood levels went haywire in April/May last year.  But that’s ok, there is nothing we can do except keep an eye on his levels so that is what we do.

The reason we know things have not be right since May is that we keep track of all his blood tests.  We don’t need to go the Doctor to ask, we can see.  We know the symptoms, we know the signs and we can see his “normal” is changing and not for the better.

Having said that, in the words of a good friend who writes an amazing blog, he is clearly alive and will remain so.

Advertisements

Dehydration in Adrenal Crisis

When your sodium is normal, but you are dehydrated.

 


Dehydration does cause the serum creatinine to rise and the estimated glomerular filtration rate (eGFR) will, accordingly, fall. The degree of change is generally proportional to the degree of dehydration. Severe dehydration can actually cause acute kidney injury and may lead to a need for dialysis therapy.
National Kidney Foundation.


Water, water, every where,

And all the boards did shrink;

Water, water, every where,

Nor any drop to drink.

The Rime of the Ancient Mariner (text of 1834) Samuel-Taylor-Coleridge


Within the support groups there is always talk about dehydration being a sign of crisis, or impending crisis (or pre-crisis if that’s what you prefer to call it).  To decide if you are in crisis, your electrolytes are checked.  The standard sign is low sodium of <135 and/or high potassium of >5.2.  The problem is when you don’t fit this criteria.

I hear many times, people believe they are in crisis, they feel horrible, they feel sick, they feel dehydrated, but when they present at ED they have their bloods taken, and are told they are “normal”, they aren’t dehydrated and therefore they can’t be in crisis.

What is being tested is sodium and potassium.  I don’t often hear talk about creatinine.

Yet creatinine is the better teller of dehydration.  Derek has had 3 crisis since diagnosis.  The first two I didn’t get the blood test results for.

The 3rd one, because it was 18 months after diagnosis, I knew Derek’s normal blood levels.  I was recording them on OneNote so I had them with me wherever I was, whenever bloods were taken.  So I asked the Dr what Derek’s blood results were.

She insisted they were “all fine”.  I kept pushing requesting very firmly that she told me the levels.  She kept saying she couldn’t remember them, but they were fine.  After pushing more and explaining that I knew what his normal was, so I would like to compare them, she finally told me that one was creatinine.  She remembered this number because it was high, which meant his kidney function was low.

When I pointed out that it was changed and higher than his normal level she actually argued with me that it was better than the one she compared it to.  I asked the date of that one.  It was compared to when he was in hospital suffering  CAPS and his worst and most life threatening Adrenal Crisis, before diagnosis.  She wasn’t interested.

She was a Resident, which means she was learning how to be a Dr, she was a first year resident, so some basic knowledge should still have been in her brain.

Although at that time I knew Derek’s numbers, what I didn’t know was what those numbers meant.

After some more toing and froing we finally got Derek some SoluCortef, but still no fluid because his sodium had come back in the higher end of normal.  I have since learned that his potassium was just above range and higher than normal for him.

The next day when the General Medical Consultant did ward rounds, his first comment was “So, pneumonia and adrenal crisis, and you are dehydrated.”

He had looked very quickly at a vein in Derek’s neck to confirm, but he was also looking at the blood results.

After I got home I started investigating what it was, when sodium was normal, that told him Derek was dehydrated, and why drinking water hadn’t helped.

Here’s what I found out.

Why Sodium and Water may not help. A very simple explanation.

Although Sodium can be a sign of dehydration, when you are on fludrocortisone sodium can be kept within range, but it won’t stop the kidneys from functioning below par.

When creatinine is higher, and therefore eGFR is lower than YOUR normal, it is a commonly known indicator of dehydration.  The lower the eGFR number the more dehydrated you are.

But why don’t sodium and fluids help?

If you are really suffering low cortisol then that same low cortisol stops your body absorbing fluids so you not only need sodium, but you need cortisol, to keep you from becoming dehydrated.  This is where Blood Pressure comes in.  When you are first dehydrated, your body kicks in with a lot of other system mechanisms, to keep your blood volume up, hence you don’t suffer low BP immediately.  It is the dehydration that determines when your blood pressure lowers.

The reason HC works to lower your BP is because it helps your body absorb the fluids it needs to increase blood volume, and thereby reduce the other mechanisms from activating, to try and balance things out.  This won’t necessarily stop the crisis, but it will help you long enough to get more help.

So if you are told you can’t be dehydrated because your sodium isn’t low, ask what your creatinine (or eGFR) is.  If they don’t know, ask them to find out.  If they still argue, ask them what anti-diuretic hormone does, and the effect of that when you have low cortisol.    It is one of a number of the mechanisms in your body to keep your blood volume up when dehydrated.

Generally, a high serum creatinine level means that your kidneys aren’t working well. Your creatinine level may temporarily increase if you’re dehydrated, have a low blood volume, eat a large amount of meat or take certain medications. – Mayo Clinic Website.

 

Back to Derek

When Derek is dehydrated he slips from Stage 2 to Stage 3 CKD.  We have to monitor it regularly.  IF you don’t know what your normal is perhaps you should find out when you are feeling healthy.  This could save you a lot of medical issues when you are sick.  And check out my post on “know your own normal” because sometimes, it too can save you.

One thing I remember is when Derek was in hospital in crisis before diagnosis, after 3 days they had to stop the fluids as his body was swelling up, and wasn’t absorbing the fluids.  His kidney’s were failing, his blood pressure had started going down, and fluids were not working as he was just absorbing into tissue, his blood volume was going down.

A number of people have asked why they get fluids and fee better, before they are in crisis.  these people still appear to have a very small amount of cortisol production.  It may be that it’s enough to help absorb the fluids.  Derek has no production, so at the time his body had nothing, including aldosterone, to help.

 

Blue September

Man Up – Give Prostate Cancer The Finger

It is a few words, with a short reach.

If you go on line and ask for “Breast Cancer Catchphrases” you get hit after hit.  The top ones were:

85 best Breast Cancer catchphrases
71 best Breast Cancer catchphrases
50 top Breast Cancer catchphrases.

Yet when you do the same search for Prostate Cancer you get

Prostate Cancer Humour
Prostate Cancer Quote of the Day
Funny Cancer T-shirts

And the images are worse.  Images of young women with Breast cancer, or “representing” breast cancer.

When you search for images of men with Prostate Cancer, they are all over 65 and the most common quote?  “Lots of men die WITH Prostate Cancer but not OF Prostate Cancer”.

According to information on the New Zealand Prostate Cancer web site:

In New Zealand, prostate cancer is the most common cancer in men, around  3,000 registrations each year and about 600 deaths from prostate cancer each year (based on the statistics from the Ministry of Health 2007 – 2009 which show an average of 3082 registrations and 602 deaths).

Men who develop prostate cancer are mostly over the age of 65. It rarely occurs in men younger than 55. About one in 13 men will develop prostate cancer before the age of 75. In very elderly men, prostate cancer often grows very slowly and may cause no symptoms.

Some men are more at risk of getting prostate cancer than others, but the most important risk factor is ageing. Men with a family history of prostate cancer have a higher risk; that is, if the father, an uncle or a brother has had prostate cancer.

Sounds good doesn’t it?  Only 1 in 13 men develop Prostate Cancer before age 75, it RARELY occurs in men younger than 55.

Just as a comparison, according to the Breast Cancer Foundation of New Zealand, Breast Cancer is the #1 Cancer for women (same as prostate cancer in men), 3,000 new cases of Breast Cancer each year (same as men), 600+ deaths in a year (same as men).  What isn’t the same, is the awareness campaigns.  The only real differences are:
Men get Breast Cancer, Women don’t get Prostate Cancer,
Women are more likely to get it over the age of 50,
men, prostate cancer is more common over the age of 65.

Fantastic.  At 50, Derek should not have to worry about Prostate Cancer.  There is no history in his family of breast or prostate cancer, no history of any other form of hormone cancer, he is only early 50’s.  Absolutely NO reason to even consider he would have it.  There is no required routine screening.  There is no “when was your last DRE” at the Dr’s office the way you are asked about your last Smear.  There is no national recall every 2 years the way there is for a Mammogram over the age of 45.

Why would there be, at 50 it is very rare to get Prostate Cancer.  And according to a survey conducted by the Cleveland Clinic:

“It turns out that taller men, and men who have bigger body mass indexes are at higher risk of high grade prostate cancer, and also are at higher risk of dying of prostate cancer,” said Dr. Klein.

Dr. Klein said previous research has shown a connection between obesity and the risk of developing high grade prostate cancer, however the relationship with the height factor has not been noted previously.

Researchers surveyed data from more than 100,000 men and found that for every ten centimetres in height, the risk of developing an aggressive prostate cancer grew by 21 percent.

Not considered taller than average at 5’10”, a healthy weight, under 55, and no family history of Prostate Cancer, Derek is safe.

He might get Prostate Cancer in his 70’s or 80’s but who cares, a lot of men do, and “they don’t die of it, they die with it”.

But all the risk factors are just that, risk factors, not a guarantee that you won’t get prostate cancer.

So why am I blogging about Prostate Cancer on a Blog about Adrenal Insufficiency.

Because, if you haven’t already read the rest of this site, you may not know that Derek has AI because of a medical botch-up when he was having surgery for the prostate cancer that according to all the risk factors, he wasn’t at risk of getting.

We didn’t actually know a lot about Prostate Cancer when Derek was in his 40’s.  So how did he get diagnosed just a couple of years later?

A mixture of things.  At 50 he had he had a problem and went to the Doctor.  He was given DRE (Digital Rectal Exam).  It is far less invasive and embarrassing than a woman having a smear, yet men won’t talk about it let alone have it.  What man wants a stranger to shove his finger up your rectum?  Well get over it guys, you expect us woman to lay there while we have a very cold speculum put up your vagina, followed by a scraping tool, to take cells.  Men simply have 1 finger press down on the prostate, and out, that’s it.  20 seconds at most.  GET OVER YOURSELVES, YOUR RECTUM ISN’T THAT PRESCIOUS!!!!!

It turns out this DRE was actually not completely normal, but Derek wasn’t told that.  He was sent for blood tests to see if there was an infection.  The Dr also requested a PSA.  We didn’t take much notice of blood tests back then, so we didn’t look at what was being asked for, and certainly didn’t worry about the results.

By 52 we had forgotten all about it.  Then he got a soft calf injury that caused a DVT.  He was diagnosed with Antiphospholipid Syndrome (APS).  That’s fine, strange but fine.

By 2011 (at the age of 53) his PSA was rising but hadn’t hit the special number of 4. (It was close)

Then, at the beginning of February of 2012, a month after turning 54 his PSA shot up.  He was fit and healthy, he had APS but that was not cancer so no worries.

In May 2012 Derek had a positive biopsy for Prostate Cancer. Thankfully, because he was having his INR monitored for his Warfarin (because of the APS) he was also having PSA every 6 months.  The Dr had seen in increasing.  So he had had Prostate cancer since BEFORE he suffered a DVT.

After the positive biopsy we looked into the options available.  His Cancer was considered MODERATELY AGGRESSIVE, which meant that, without treatment he would die OF prostate cancer, not WITH prostate cancer.

So, Not overly tall, not over weight, under 55, no family history of hormonal cancer in the family (although his father had dies of leukaemia, but that’s wasn’t a risk factor for prostate cancer) yet he had prostate cancer.

Women are told all the time that you can get breast cancer even without the risk factors.  Men are not told the same.

Why Not?  What is it that makes it so important that a woman is checked for cancer, but a man is not.  Are men any less important?  Or is it because men make the decisions, and their decision is they don’t want that initial FINGER UP TO PROSTATE CANCER?

Some people believe that it is not necessary to have PSA checks, that the chances of prostate cancer killing you is not worth the effects of invasive testing that can be wrong, the worry, the removal of a prostate (and the very high probability of erectile dysfunction) is not worth it.

Well, those people can go elsewhere to comment.  I know that Derek would be terminal now, if not dead, if we hadn’t had the testing, the biopsy, and the surgery.

SEPTEMBER IS PROSTATE CANCER AWARENESS MONTH

Even if you don’t meet ANY of the risk factors for Prostate Cancer, YOU CAN STILL GET IT?

We didn’t know this, until we did!

 

When Different Became Normal?

4 years and 9 months ago I took my husband to hospital for surgery to remove cancer.

I knew, as we walked Derek to the hospital ward for admission that things would change.  He was having his prostate out, there were implications, including not knowing if this would put his cancer in remission, or if it was just step one of a long journey.

We had no idea at that time, that things would go so horribly wrong, and that he would forever by living life on the edge of the precipice, waiting for the slightest thing to push him over the edge.

We spent the first 18 months after surgery going from medical appointment to medical appointment.  It wasn’t unusual to be told “wow, and he is alive” like the Dr’s were patting each other on the back for doing such a great job, or shocked that anyone can live through what he suffered (very few do).

Then the appointments they were making started to dry up.  So we began pushing.  Things were still not right.  There were still things that had not been acknowledge, investigated, diagnosed.

After 3 years we felt we knew mostly how the CAPS affected him.  Knowing what was wrong, what we could fix (which wasn’t a lot) and what we had to learn to live with.

We kept using the term “our new normal” when asked about how we coped with everything.  According to the many medical books out there, most people with Adrenal Insufficiency can just take their medication 2-3 times a day and have a normal life.

Except that this isn’t the case for most.

At the 3 year mark we started reading, we started downloading “stuff”, we started learning everything we could about Derek’s medical conditions.  Neither of us has read so many medical studies.  Or chased so many references to find the original source data for all the presumptions.

While learning, life carried on.  We added meds (at our insistence, not the Dr’s), we changed Dereks dosing schedule as we learned that HC didn’t last long

The we realised it.

We are coming up 3 years since Derek’s last Crisis.  We have managed chest infections, urinary tract infections, colds, throat infections, injuries (mostly minor), and frights.  All without emergency medical intervention.

We had woken up one day and we weren’t working on getting used to our “new normal”, life was again just “normal”.

When did that happen?  When did Derek having Primary Adrenal Insufficiency, Dysphagia, constant brain fog, and a frequent need to “up dose” become just NORMAL?

I had to race home earlier this year as Derek was unwell, but I didn’t panic, I didn’t get a ride with anyone, I didn’t even feel an urgent need to go and get a taxi home, I just took the bus and train, then routinely sorted out his medical appointment and treatment.

I wake every morning and wonder “is this they day I find him unresponsive”, is he going to roll over and take his morning cortisol like normal.

For 4 years I would leave home each day worried about what would happen if I got that call.  When I got it, I didn’t panic, I just told Derek what to do, and headed home.

I am never really happy in the morning until I know that he has rolled over and taken his meds.  But quite often I sleep through him doing it.  Whether or not he is awake by the time I am out of the shower is still on my mind, for a fleeting moment, then things turn to normal daily routine.

I get up, I get ready for work, I head out the door, wishing Derek a good day, and work all day.

There was a time when I had to call or txt Derek 2-3 times a day to see how he was, listening intently to his voice to see if I can get any clues on whether or not he is sick.

Now I only call when I need to speak to him about something.  I will still txt him most days, especially if I notice that he seems a little tired the night before.  But it’s not with dread of what the response will be, it’s with a genuine “how are you” as you would ask anyone who was tired.

I don’t know when it happened, but our New Normal, is now just Normal.

The misquote in the medical text which originally said “you can live a normal life span” became, for many Dr’s “You can live a normal life.”   It is a bit like the misquote from Spock, who never actually said “It’s life Jim, but not as we know it”.  It sounds great, but is an urban myth based on some small portion of words.

However, there is some truth in it for anyone who is chronically ill.  As the mother of young children, running around dropping them at different activities, sitting up until they were asleep at night, having them with me in my bed when they were sick was all very normal for the situation, but if you didn’t have children, then it wasn’t normal, it was different, it was hard work, it was tiring.

So too with the chronically ill.  For the outsider, it isn’t normal to feel tired all the time, it isn’t normal to take multiple doses of multiple medications just to function, it isn’t normal to finger prick every day before and after meals.  But for those that do it, at some point, it does become normal.

And that is where we find ourselves.  We are out the other end of the tunnel, and that big light heading towards us wasn’t a freight train on our track, it was on the adjacent track.  It shook our world when it went flying past, but it didn’t stop us in our tracks, it just caused us to take a little detour in our life.

Derek still has to take hydrocortisone, fludrocortisone, DHEA, warfarin, vitamin D, BP meds, and anything else that he needs to function, but that’s normal.

We are lucky.  Our normal is actually OK.  We’ve got this.

How did we get here?  We got educated.  We studied his conditions, and we took control of them and we lived.  We continued to do things daily.  Some days it is a struggle, but we do it, one foot in front of the other, one dose of medication after the other.  Along the way we learned what “normal” meant for Derek, in his blood tests, his BP, his fatigue levels, even his body temperature.  Knowing his “normal” and accepting it, means we can work with it.

Part of accepting the new normal was accepting what you can’t change.  After working around it for a while, it will become normal.  It’s like taking a different road when going to work.  If you take it often enough, it becomes your normal routine.

There is a prayer that many groups use about acceptance.

 

Even if you are not a person who believes in prayer, the sentiment is the same.  Acceptance of what is, courage to learn how to change what is needed to change, and the understanding that there is a difference.

 

 

 

There are days when Derek isn’t so well, but that’s ok.  We know what to do, and we do it.  The good days outweigh the bad.

Normal, in this new form, is not great, but it is good.  It is doable.  And in the words of a good friend, “He is clearly alive”

I asked Derek his take on this NORMAL

“The new normal is doing less than before, but it is something.  We do what we can, and enjoy it. Failure to accept the situation would lead to depression, and I’m not going there

It’s not what Jo signed up for. But it’s what she has also accepted.  We have all had to accept it, including the kids.  It could never be normal until we all accepted it.”

Thank you to all those who have helped us to get to this point, there are too many to mention here.

 

International Travel with a Chronic Illness – Part 2

Australian Triip – Day 1-3

I had just done all the preparation for our Trip to Australia.

I sat on the train on my way home from work going through things in my head.  Everything that I had done to make sure it the trip went smoothly was ticked off.  Then it came to me.  Derek took DHEA.  It is a steroid.  But what type of steroid?  I grabbed my phone and looked it up.

Well of course it would be an Anabolic steroid, and prohibited for import into Australia.

Great, this meant going on line and filling in an application to import a prohibitied substance.  It also meant a letter from Derek’s Dr confirming it was prescribed for a medical condition.  When I got home that night and pulled out the laptop, found the link to the Australian Customs website and hit apply.

I was amazed that I had a response, with a form, arrive in my inbox within 2 hours.  This meant I could take it to our Dr’s visit the next night, and have the application off by Friday.  I was cutting it fine.  It was supposed to take 20 days to get it approved, and I had 18 working days before we flew out.

We went on the Thursday night to the Dr and discussed everything we needed:

  1. A list of medication for NZ Customs
  2. A letter for Australian Customs regarding the DHEA
  3. Did we need to get an INR while in Australia.
  4. Did we need to carry Clexane incase Derek’s INR dropped too low.

After the discussion the Dr felt that, as it was only a 4 hour flight, and we were in Australia, then an INR check would not be necessary, and if something happened, they could give him clexane anyway.

We then did an audit of all his medication and put enough in containers for a months supply of everything.  If something went wrong and we had to deal with it over there, we wanted enough medication to last for the month at triple dosing, not just a week of normal dosing.

The last thing Derek had to do was go out on the Friday morning and get an INR.  It came back in the afternoon.  It was 2.9.   His preferred level is 3.2.  It wasn’t considered low, was lower than we prefer.  It could be easily managed.

We were ready for our trip.

The night before we flew out Derek took extra hydrocortisone.  We set the alarm for 4am, and I put all the bags in the car.  All we would have to do was get up, get dressed and go.

I woke up before the alarm.  In fact, I woke up half an hour before the alarm, so I went and emptied the dishwasher, and tidied up the kitchen.

At 3:50 I was dressed ready to go and I woke Derek.  All he had to do was put his cloths on, grab his normal daily bag, and get out the door.  He took 20mg of HC (normal morning dose is 10) and we left.

He closed his eyes when we got in the car, and rested.  I drove to our son’s house to pick him up.  Derek was starting to come round as we got to the city so was awake (a very loose description) when I pulled up outside Colin’s home to pick him up.  He was taking the car to his place from the airport.

I told him we would stop at the 5 minute drop off, we would unload, and he could go.

As I took all the bags out of the car I looked around.  There was a baggage trolley outside which I grabbed to put our bags on so Derek didn’t have to carry anything.

As we entered the Departures Checkin I looked around.  Directly in front of Virgin Airlines Check In counter was a wheelchair.  Great, they had it.  Derek needed it.  We went up to check in “Hi, we are here to check in, and is there a wheelchair for Derek?”

“Yes.” The checkin operator looked over to the chair I had seen.  “It’s over there.  Give me your tickets and passport and I will start doing that while you go get it.”

“You can’t take that!”  I stopped to see another check in operator looking directly at me as I was reaching gfor the wheelchair.

“It’s OK.  It’s for my husband”  I pointed to him, leaning against the counter 2 check-ins down.

“Oh, that’s ok.  I thought you were from the Qantas Flight.”  She had looked to where I was pointing.

We finished the check in process, then piled our carryon bags on Derek’s lap and went for breakfast.

Fast Track to a Holiday

I wasn’t sure what would happen when we attempted to go through the Departures Gate.  It was a lot easier than I had though it would be.

As I pushed Derek through the barrier for passengers only I noticed another woman pushing her husband.

We both took the long cattle track as it wasn’t made clear that we could take a short cut.  As we arrived at the security scanning area I unloaded everything to go through the x-ray machine.  I then pushed Derek to the Security Gate.  He was asked if he could get out and walk through.  He did, but the wheelchair was a little too close and it set the alarm off.  He then had to stand and be checked with their hand held metal detector.  He was clear.  I pushed the chair through the side entrance, then went through the scanner myself.  I too was clear.

We piled Derek up with everything again, and moved through to the duty free zone.

A sales member came and help us with our purchases after which we went to the gate to wait.  I grabbed a couple of bottles of water, put some electrolytes in Derek’s and settled him with our bags.

Right beside the gate was a coffee shop.  Yay, finally we could have a coffee.

I cued up with everyone to get my early morning coffee while we waited for boarding.  I looked up to see a Virgin Airlines staff member talking to Derek.  She was organising to get him on board.  We only had time to drink half the cup of coffee before we were called to board before everyone else.

I pushed Derek and the staff member took my carry on bag.  We were assisted the whole way.  They settled us in our seats and then turned to the rest of the passengers.

The flight itself was very uneventful.  An hour after taking off he took another 10mg HC (his normal morning dose).   About 2.5 hours into the flight Derek decided he needed to go for a walk, to stretch his legs.  The Business Class Steward saw him get up and offered him the use of the business class toilet, rather than walking to the back of the plane.

As the flight was preparing to land they again approached Derek and informed us that we would have to wait until all other passengers were off before we could disembark as they had to clear the way for the wheelchair, which would be waiting at the door to the aircraft.

After everyone was off Derek got up, and almost fell over.  He couldn’t stand up straight.  His hips hurt, his legs hurt, he was extremely tired (even though he had taken more HC on the flight).  Looking at him, I was thankful I had organised the wheelchair.

Once we disembarked, I was even more thankful.  There was a 600mtr walk from the gate to the immigration hall.  There was no way Derek could have walked that far, and then cued with 300 others (a second flight had just landed).

The beauty of the wheelchair didn’t stop there.  I didn’t have to push Derek at all.  A staff member pushed him.  He also took us through the priority system.  He helped us with our passport clearance, helped us through luggage pick up, and most importantly, helped us through declaring Derek’s drugs.

It was a doddle.  We ticked we had things to declare, they asked us what, we said drugs and food, we handed over the letter from Customs allowing us to import DHEA and we were told “Customs have already cleared you, of you go”.

With that the gentleman pushing Derek lead the way out into the arrivals concourse, took us to a seat, unloaded Derek and left us.

As we were going through baggage claim I had noticed a family (one of the first off the aircraft) waiting for their bags.

I left Derek to sit and rest on a soft seat while I went to pick up a micro sim for my old phone (to give me data while on holiday).  It took a good 15 minutes.

“Guess who I saw only just come out of the arrivals gate.” Derek asked as I arrived back.

“I don’t know.   Who?”

“The guy that was sitting next to us.”  He was also one of the first off the aircraft.  I knew we got through quickly, but not that quickly.

Next job was to get the car I had booked and get on our way up to the Sunshine Coast.

So far everything was going exactly to plan.  This was a good thing.

After Derek had had a rest we went and got the car.  It was a short walk, and he needed to stretch his legs.

We found the car, put our bags in the boot and got in the car.  We were going to stay with Des Rolph and Wendy Lau at Des’ Beach House.  Derek put the address in the phone, and set the system up to navigate.

Let the Games Begin

The trip up went well.  We arrived at the house just after 11.30.  Although I had never met Des or Wendy in person, I had skyped with Wendy a number of times, and had chatted on facebook to Des.

We arrived to discover that Des and Michelle Dalton (another facebook friend) had gone to the supermarket, but Wendy and Chris (Des’ husband) were home to greet us.

It was like meeting up with old friends.  We felt at home with them immediately.  And it was no different when Des and Michelle arrived back.

Lots of chat, some food, a little wine, and what to Derek and I was a late night.  Derek kept double dosing his HC all evening, we had a lovely dinner, and then excused ourselves to go to bed.

des-place

Michelle, Des, Wendy, Jo and Derek. Chris is the photographer.

I woke very early the next morning.  My body was on NZ time and Queensland is 3 hours behind.

I lay in bed watching video’s on my tablet while I waited for Derek to wake.  He finally woke up and took his first dose of HC for the day.  He then rolled over and went back to sleep.

As he slowly woke up he made a great announcement.  “I forgot to take my warfarin last night.”

Bugger.  Friday his INR was 2.9, flying decreased INR, he didn’t take Warfarin last night.

This all equals low INR.  We needed to get it checked quickly, but it was Sunday and we were in a foreign country.

We spoke to Des about what we could do.  There was a lunch function at the house with 6 other Adrenal Insufficient patients and we weren’t going to miss this for the world.  It was exciting to finally get to meet others with AI, and to chat to the spouses to compare notes.

Read about “The Domino Effect” here.

the Domino Effect

The Domino Effect

We had breakfast and started preparing some of the food for lunch (I say we, but mostly Michelle).

Once it was open, Des phoned the local 7 day medical centre to see if we could go in there and organise a script for an INR at thelocal Pathlab.

We were told they took walk-in’s so we could turn up at any time.  We decided we were going to leave immediately.  That way we could be back in time for the lunch.

When we checked in at the medical centre I asked if they were able to do plasma INR or if it was just a coagucheck system.

“It’s coagucheck, but that’s fine, its really accurate.”  Came the response.

I was doubtful, as I had heard through the APS forums that, for certain APS patients, it wasn’t that accurate, in fact it was dangerously wrong.

The Dr came out to reception “Derek?”

We got up and followed her into her consulting rooms.

I looked at Derek, who was still extremely tired.  “Derek has Antiphospholipid Syndrome and is on Warfarin.  How much do you know about Antiphospholipid Syndrome?”

“I gives you an increased risk of a blood clot” the Dr said with confidence.  However, she didn’t ask if Derek had ever actually HAD a blood clot.

“It doesn’t just give you an increased risk.  Derek has had clots.  He has actually suffered Catastrophic APS.”

She looked like she didn’t really know what it was, so I began to explain.  Derek chimed in as he could but he was struggling.

We then explained that he had a very high risk of having too low an INR and we needed it checked.

“We can do that here with the coagucheck machine.”

“I don’t believe that is very accurate.  We need a plasma INR.”

“Oh, these machines are very accurate, we have never had a problem with them.”

“They are accurate in atrial fibrillation, and in some patients with certain APS, but Derek has low Platelets as well, so for him it is not accurate.”

“Well let’s check it and see what it is.”

I was beginning to get the “I’m a Dr and I know better than you” feeling.  Not an unfamiliar feeling these days.

We went out the back and had the test.  It came out at 4.3.  this mean a very high risk of bleeding.

“Well that’s not possible.”  I was looking at the result, and then at the Dr.

“INR can be quite unstable when traveling.  It can be caused by heat, by long travel. Lets go back to the office and discuss it.”

Research has shown that Coagucheck INR is not recommended for patients like Derek who has High IGG and low platelets, as they tend to give a higher INR than it actually is.

Derek and I endeavored to explain that with APS flying lowered INR and therefore that result was most likely wrong, but like many Dr’s she was determined she was right.  We also got the feeling, when she mentioned Clexane, she though it would increase INR, where it does not have an impact in INR but does affect coagulation.  It should be used to reduce risk of clots, not reduce INR as was indicated by the Dr.

After some conversation, the Dr was determined we should head straight to the local hospital to have an INR as it was too high and could be dangerous.  She also advised not taking warfarin that night.

I agreed we would take a letter for taking to the Hospital, as long as she also wrote a script for an INR sat the local PathLab on Monday morning.

When we arrived back at the house we told Wendy, Des and Michelle what had happened and that there was no way we were going to the hospital before the lunch.

Wendy said she that her Dr friend was a Heamatologist and she would contact him to see what he thought.  I was still unbelieving of the high INR, and was still concerned that in fact, it would be extremely low.

After a very hectic time over lunch (explained here) Wendy finally got hold of the Dr for us.  I found it strange to be standing on a balcony on the Sunshine Coast, talking to a Chinese Dr in Hong Kong.

He agreed with me that there was no way Derek’s INR was 4.3, and that waiting till Monday would be safe, Derek should just take his Warfarin as normal that night.

Given Derek’s stress levels were very high I wanted nothing to do with Hospitals that day so we would go to the pathlab the next morning before heading south.

The next day we were due to leave.  Everyone in the house was extremely tired, and needed an early night so we went to bed by 8pm (11 NZ time) and slept.

The next morning we woke to pack our bags, get ready, and head to the Path Lab.  It was only 20 minutes down the road.  After we had the blood taken we were heading to Bribie Island to meet up with another friend.  This time someone I met through the Coro St MIRC group I had joined 20 years earlier.

I was excited to be meeting her so was wanting to get the test done.  We had to also organise for them to call us with the results as we would’t be going back to that particular medical centre again.

Medical Incompetence is International

We arrived at the Pathlab and handed over the form for the blood test.  As I handed it over I asked if there was any chance we could have the results phoned directly to us, as we were travelling.

“We will see what we can do.  Is this the correct Medicare Number?”

“What, we are from New Zealand, we don’t have Medicare.”

“So what is this number?”

“I have no idea.”

“Date of birth.  Is it ######”

“No.”

“Phone Number:  Is it ########”

“How did they have a phone number, I have never given them a phone number.  What is the name on that form?”  Derek and I looked at each other.  We couldn’t believe what was happening.

“Dale ####”.

“That’s not Dereks form.”

After a little more discussion we headed out the door to the medical centre to get it sorted.

On the way over we looked at the letter we were supposed to present to the hospital.  It also had the wrong information, but the right medical issues.

Wow, a major mistake.  If the other patient had had to go to hospital, he may have been given clexane because his INR wasn’t at the right level.  It could have killed him.  But worse, if Derek’s INR was wrong, he could also have been incorrectly treated.

We got back to the medical centre to explain what had happened and request a new form.

The Receptionist went to speak to a Dr.  She came back a few minutes later.  “The Dr discovered her mistake yesterday after you left so she faxed the correct details to the hospital.”

That was great, except there were two public hospitals, she had no idea which we would have gone to.

We got the correct Pathlab request and went back for the blood draw.  Derek was treated by a Kiwi phlebotomist.

We then headed to Bribie Island to see Jubbly (Jacqualine Russell).

It was a 1.5 hour drive so we let Jubbly knew we would be late.  We were also meeting Elaine Miles, another person I had known for 20 years without meeting.  I was excited.  Yesterday was Derek’s day, today was mine.

jubblyelainejo

We finally arrived just on 12.00 noon.  As we got out of the car to walk to where we were meeting them Derek asked if we had the chargers, tablets, and cables for our technology.

Bugger, no, and my phone was running out of battery.

I went back to the car to check, sure enough, in our haste to get to the Pathlab, and the stress of the previous day, we had left them all behind.

I would have to think about what we could do.  But that was later.  Right now we were focusing on Jubbly and Elaine.joexercisejoderek

After chatting for a short time we headed to the fish and chip shop to get lunch.  All the way Jubbly kept poking me, just to make sure I was real.  It was like on the Sunshine coast, like meeting a long lost friend.walking

We had a great time.  We talked, and talked, we laughed, we ate fish and chips by the beach, we walked along the boardwalk.jubblyjoselfie

 

 

 

 

 

 

After the stress of the previous day, it was great to relax.  Derek was starting to show his tiredness, but he also looked like he had started to relax a bit.

Then we had to organise getting the stuff we had left behind.

This meant we had to drive the 1.5 hours back north before we drove south.  So far this holiday had not gone exactly to plan.  It was a major learning curve and we were only at day 3.

After saying a gre

at goodbye to Elaine and Jubbly we got in the car and headed back north.

Watch out for the next leg of our “holiday”.

A Presumptive Diagnosis

Aside

The marrying of Evidence-based medicine and Experience-based medicine.

Have you ever sat in a Dr’s surgery, told the Dr everything about your signs and symptoms, and then you hear those fated word:

“WELL IN MY EXPERIENCE”

Many years ago, we trusted Dr’s to use their experience to know what was wrong with us.  These days, we can now have blood tests, scans, x-rays and more, to back up that experience.

Some Dr’s are single (they only use Evidence, or they only use Experience.  Some Dr’s have successfully married the two.  Others are in a relationship, but is very shaky and they have to work hard to keep the relationship on an even keel.

If your Dr is a “in my experience” type Dr you need to remind them that actually Doc, it’s not just about your experience, it’s also about your skills to interpret results of blood tests, scans, images, or any other investigation, to build a full picture.  Its about your ability to keep an open mind, your realisation that not every blocked nose is a cold, that every high BP is from poor diet and lack of exercise.

You might have had Jane Doe in here with a blocked nose that was hay fever last week.  But I don’t get hay fever, my nose is not normally blocked like this, and it is giving me a major headache.

If ever a Dr says to you “Well in my experience” and they have never met you before, then there experience could mean diddlysquat, if it not balanced with correct interpretation from clinical investigations, blood tests, x-rays, scans, or what ever else needs to be done before a confirmed diagnosis is made..

Dr’s must learn to park their experience at the door when they first come into the room to see a new patient because their experience, however vast, can mean a missed diagnosis, a wrong presumption, the brushing aside of what appears a “minor abnormality” which can be a major cause, or the vital clue to the true condition.

 Let me give you an example.

As those who have read my blog know, my husband Derek has Antiphospholipid Syndrome.  It means his blood is more susceptible to clotting.  To prevent this happening he takes Warfarin.

In May 2012 the Dr’s stopped his warfarin for 5 days without any anticoagulation cover.  We, at this time, trusted Dr’s to know what they were doing.

The day of the biopsy he felt “unwell”, the day after his biopsy he was seriously sick.

Enter the Presumptive Dr.  Experience based diagnosis and treatment began.  In the experience of all the Drs we dealt with, on balance of presumption, he had Sepsis.

Immediately treating that sepsis was the right option.  However, they still had to make sure it was sepsis, not just use their experience and accept it.

After giving the high dose anitbiotics, what they did was ignore the facts as presented.  When we spoke to each Dr we told them:

  • Derek had not had warfarin for 5 days (and was still not taking it)
  • Derek had a blood clotting disorder which, without warfarin, meant he had a high risk of clotting
  • He felt unwell at the time of the biopsy

The Evidence Based Medicine did not support a diagnosis of sepsis, especially when you add the signs and symptoms together.   The clinical evidence showed no sign of infection based on a number of blood tests.  The blood tests only showed his body was fighting inflammation which is a classic sigh of an APS flair, or CAPS.  They could not identify any bacteria or any of the many signs, only an indication of inflammation.

Yet because sepsis was the obvious diagnosis based on experience, no other investigation even was done when the evidence didn’t support it.  It was put down to just one of those things.

In Sept 2012 the same thing happened.  Between 2 and 16 Oct we visited many Drs, telling them all the same story.  He had stopped warfarin for 5 days, then had prostate surgery, and  had been unwell the entire time.

All that was heard was Warfarin and prostate surgery.  Clearly he had some form of infection, firstly a UTI, then a reaction to antibiotics, then a throat infection, another reaction to medication, a kidney infection……. Infection, infection, infection.  Never did he have a blood test. All the diagnoses were experience based presumptions, without evidence.  2 tests were done during the week, one throat swab and one swab of a rash.  Both these came back clear.  No one listened to the real symptoms and none of the Dr’s put all the symptoms together.  Just because there was symptoms 1 yesterday, didn’t mean it should be ignore when symptom 2 happened.  In the 2 weeks from 2 to 16 October, Derek only had 1 blood test.  Nobody queried his history of cessation of warfarin and the history of blood clots.  We told of a history of his INR going high above range, then a bleeding event in the previous 2 weeks.  All were ignored because those we spoke to had no experience with those, so they couldn’t comment.

This is where experience based medication falls down.  To use experience, the Dr must first have experience.  Because it was rare, they did not.

On 16 Oct Derek was finally admitted to hospital  He had had blood tests on the Friday which when finally looked at 4 days later, and were all way out of range.

He was rushed to hospital via ambulance, but still his own Dr, knowing his history, told the Ambulance officers that it was suspected sepsis.

On the morning of 17 Oct he had a CT.

This CT showed a small pocket of pneumonia and an accumulation of fluid around the lungs.

What was also mentioned on the CT report was what “has the appearance of a clot, and the left adrenal gland appears enlarged”.

These two findings were ignored as insignificant compared to the fluid around the lungs, and the presumption that he again had Sepsis.

For 6 days they ignored the history of APS, cessation of warfarin, and the findings on the CT and instead treated him (unsuccessfully) for sepsis.  No matter the treatment, his body continued shutting down.

It wasn’t until a junior Dr visited Derek.  Having left all presumptions at the door, she started from scratch.  She asked questions, studied his body, all the time taking note.  She then went and read the reports.  Including the report from 17 Oct.  It was only then, because a Dr threw all presumptions of the previous 2 weeks, out the window, that the true catastrophe revealed itself.

I am not saying that immediate treatment for sepsis wasn’t a good idea, but when they didn’t find any evidence of it, then they should have kept looking.

Yes, treat the easiest condition first, but consider other options, including, if all else proves negative, that zebra hiding in the dark corner.

“In my experience”

If the Dr says this to you and you have never met him/her before, point out that any experience they had before facing you, although useful, needs to be successfully married to evidence, and they need to have experience of using that evidence.

What you are looking for from them is their training and knowledge in interpreting investigations, with an open mind to the possibility that they have never experienced YOUR particular circumstances before.

Experience is great once they know what is wrong, but making a presumption based more on their experience than on fact, can leave many people remaining undiagnosed, misdiagnosed, untreated or feeling sicker than before.  When it happens time and time again, some patients just feel like giving up.

It can be hard to be taken seriously when you have to go from Dr to Dr because you KNOW there is something wrong, and all you get is Presumptive diagnoses, the treatment for which, is clearly not working.

It is hard to advocate for yourself, but if you are able to, when the Dr seems to be diagnosing you based on HIS/HER experience, not yours, ask them what they are basing their diagnosis on.  Get them to explain how they came to that decision.  Ask what evidence they have.

It can be hard, but if asked in the right way, and if necessary asking the same question in several different ways, it can make the Dr rethink their own ideas.  Sometimes however, the Experience and the Evidence are divorced. They will never live happily together with the Dr, and at this time, you need to find another Dr.

International Travel with a Chronic Illness

leaving-on-an-aircraft

Our Next Adventure Part 1

Having managed tripping up and down the North Island of New Zealand over the last 4 years, including flying, just to see how it would go, Derek and I want to venture a little further.

The Practice

To begin our preparation for an international flight, the first thing we did was try flying to Auckland.

It required a trip to the Airport, then flying to Auckland, and driving to Hamilton.

Derek took extra medication to fly.  He took extra hydrocortisone for the drive to Hamilton from Auckland.  He then lay down for a long rest when we got to Hamilton.  He also had to rest the next day, but that is normal when we travel any kind of distance.

On the whole, the trip was good.  We listened to others’ advice, had learned what Derek could manage, and when he needed to up dose.

Flying Further

This time we decided to go to Australia.  Originally our first trip overseas was going to be to Sydney or Melbourne for a weekend for a Show. After being offered a trip to Fiji earlier this year, and the realisation that a 2 day trip would be way too hard, we thought a longer trip would be better.  Because a friend with Addison’s was heading from Hong Kong to Brisbane for medical treatment, and we had friends in Brisbane, that was going to be our destination.

We are not the first to travel with chronic illness, and we won’t be the last.  When you look around the cue of people going to the flight, you don’t know who has a chronic illness, who has spent days and weeks preparing, and who has just grabbed a ticket and headed to the airport.

I asked my cousin, who’s husband has several serious medical issues, what they do to take a trip.

Trev just sorts all his medication and I just carry it…never had any problems, I think I have only been asked once and they were good about it. I never carry hospital documents but for you guys being a first since Derek has been sick, just get your GP to write a letter out lining the diagnosis and a list of the medications on the letter. Never really had problems with insurance just be up front about it all, you may not get full cover but shop around. We don`t stress about overseas travel, there is always a hospital where you go if things don`t go to plan. Just relax and enjoy the trip. We are probably not a good example…as we are pretty relaxed about it and just roll with it…lol…isn`t that naughty, but thats just us…great place we have just come back from there.

The trip they had returned from was a trip to celebrate 25 years since “Trev” had an organ transplant.  (By the way, I am totally in favour of organ transplants.  It saves lives, including that of my cousin’s husband so please think about donating organs if the unfortunate need should arise).

Booking the ticket.

We have made the decision to travel over to Australia.

Now What?

Do we need to get permission from the Dr?

We don’t believe so because we went to see her about going to Fiji with a work trip for Derek, and she said no, because of the risk of food poisoning, the possible need for INR while there, and the short time frame.

But she said that if we wanted to travel somewhere else, like Australia or England, then yes, she would make sure we could do it.

We bought the tickets. tickets

When I booked it, I also requested a wheelchair at both ends.  That meant an alert on the ticket booking, and I had to call a number, and speak to someone.

The flight is a Partner flight, which means we are booking through one airline, but the flight is provided through another, so there was a delay while the confirmed that the wheelchair was available.

We knew from experience going places with Derek he could not stand in line long.  He gets very fatigued just waiting at the supermarket, and if there are more than 2 in a line, he has to sit while we wait.

Then there is the timing of the flight.  We could fly out at 11am.  That would mean Derek could wake up as normal, and we could take our time.  But that also meant 9-12 hours flying as we would have to fly from Wellington to Auckland, wait for 2 or more hours, then fly to Brisbane.  We would arrive at night, and it would be a very long day.

That would take more out of him.

Alternatively we could fly out from Wellington at 7am.  And then fly for 4 hours, directly to Brisbane, and land at 8am Brisbane time.

This meant a lot less travel time, but a very early morning.  Derek normally wakes at 6am, takes his first round of HC, then goes back to sleep for an hour while he waits for his meds to kick in.

This trip would mean waking him at 4am to take his first round of HC, Drive to the airport, have breakfast, then check through Customs. (hopefully we haven’t got any fines that hold us back).

So next we book an appointment with the Dr.  We need:

  • A letter for treatment protocol (if he suffers an Adrenal Crisis)
  • A letter confirming he is able to fly (because of Antiphospholipid Syndrome and risk of DVT)
  • A list of medication (it needs to be declared at every port entry and exit).
  • Any meds he may run low on before he leaves, or soon after he gets back.
  • We also need to organise an INR when we land in Australia. This is the bit we are unsure of, but the essential bit to make sure it hasn’t dropped too low while flying, which could mean a blood clot.

Then there is Travel Insurance.

Normally you just buy it when you buy your tickets.  All you need for Australia is cover so if you get waylaid, you can get a change of ticket.  But if you have extra conditions, you need to tell them about them.  Then the cost goes way up.  It went up by $150 for Derek.

But we don’t know if we have declared everything as they didn’t have the ability to declare the catastrophic event he had.  And I hadn’t declared his prostate cancer.  Oh well, looks like a phone call.

I got two quotes.  So we needed to call both companies to see how things would change.

After a long phone call to each company, Derek’s insurance was going to cost $6 extra for the prostate cancer which is in remission.

Medication

Emergency Kit

The Take Every Where Kit

We would need a list to make sure we took everything.   It currently sat in multiple areas of the house.  We also needed to make sure it all had a proper prescription labels.

When my friend Wendy travelled from Hong Kong to Australia I told her to declare, declare, declare.  If she declared everything she would be fine.

I was going to work on the same principle.  But that is not the case for Derek as he takes DHEA (Dehydroepiandrosterone).  It is classed as an anabolic steroid, and therefore restricted.  Derek needs a licence to carry it into Australia, even as a prescription medication.

And one of his other medications needs authority to carry it out of New Zealand and then back into the country.

So, we have a Dr’s note x 2, we have an emergency letter, we have an application to take his medication into Australia.

We can only carry a month’s supply in and out of New Zealand but that’s fine because we are only away 6 days.

We have to wait to hear from the Dr regarding whether he needs 1 more medication for the trip, but otherwise, we have things ticked off that we need.

Getting all the advice is key to a successful trip.  So I went seeking advice.

Travel Advice from the Experts

The Addison’s Disease Self Help Group UK has some great advice:

  • Good general advice for long distance air travel includes:
  • Remember that air travel is dehydrating so you will need to drink more fluids than usual in the air. Drink alcohol, cola drinks, coffee and tea sparingly as these dehydrate the body further. If possible, carry a large bottle of water in your hand luggage. If you forget to bring your own water, be assertive about requesting extra refreshments from the cabin crew.
  • Walk around the plane as much as possible. Try to get up out of your seat every two hours to stretch your legs and keep the blood flowing.
  • Many chemist shops now stock knee-length support socks, which can help prevent the formation of blood clots that might lead to a deep-vein thrombosis (‘stroke’).
  • Adjust your watch to the time of your destination as the flight begins, and adjust your in-flight activities to that new time zone as well. Sleep through the in-flight meals, if necessary, to get attuned to the new time zone.
  • Try to book flights that allow you to arrive at your destination in the late afternoon or early evening local time, so that you get a night’s sleep at the end of your travelling. Flights which arrive in the early morning local time will leave you tired after travelling but having to stay up all the day before you get a proper night’s sleep.

So we missed the last one.

But we are going to stay at the house of an Addisonian, so they will know that Derek will be going to have a sleep when he arrives.

So everything we can think of is ticked off.  Now to wait.

Now to wait for the actual trip.

We are excited about the trip.  We have thought of all the possible issues, and taken care of everything we can.  Derek isn’t being wrapped in bubble wrap, but we are reducing the risk as much as possible without saying, “too hard, not going.”

Next Chapter – The trip.

I am hoping this will be a very dull chapter with just the excitement of having the Sunday lunch with other addisonian’s, and enjoying visiting somewhere I have never been.  I won’t apologise if my after trip post is boring, as that would be the best trip ever.