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.

 

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.

 

 

 

To Get Bitter or To Get Better?

bitterorbetter

It’s Your Choice.

We are determined that for Derek, we will get Better.  To get Bitter would mean bad health, constant hospital visits, and probably the end of our marriage.

I have been reading back over the medical records of Derek’s Hospital stay, starting with his Diagnosis of Cancer in May 2012.

I have been reading everything with new eyes.  Not the eyes of someone investigating what went wrong.  Not the eyes of someone needing to prove failures in the system but with the eyes of someone who is reading for the first time, what actually happened to Derek.

It is bringing back interesting memories of sitting by his bed being told he is going to die, being told he was the 2nd worst off patient on the cardiac ward and they didn’t know why. As a side note, the worst patient was rushed to ICU an hour after Derek had been told that by the most Senior Dr on the ward.  Four years on it is finally safe to relive what happened without anger, fear, or distress.

Now it is what it is.  We have travelled the road of the mourning process you travel when you suffer a serious loss.  We have both come out the other side.  If you are able to, looking back to see how far you have come can be a good thing.  It’s something everyone should do when they have a major crisis in their lives.  But it must be “safe” to do it.  And for Derek and me now, it is safe.

Over the first 3.5 years I have read the medical notes trying to find specific things.  I missed a lot of what the nurses said in their daily reports.  I missed the real struggle Derek went through, how sick he was, and how bad he still was when he finally left hospital.

He will never be the man he was in 2011.  We have mourned that loss.  Now we are working on what is left. Learning what we can improve, and how we can improve it.  And just as importantly, learning to live with what can’t be improved by learning to work around it.

The biggest challenge was, and still is, trying to make the medical staff understand that we KNOW he won’t get better, but if we don’t know what is wrong, how we can work either with it, or around it.  There is a belief with some professionals that, if it can’t be fixed, you don’t need to know how bad it is, or sometimes even what it is.

The best example for Derek is that I began complaining while Derek was in hospital in 2012 that he had new issues with swallowing.  I told every medical professional we spoke to.   I became a cracked record, repeating the same thing over and over.  But it wasn’t until I got angry with the medical fraternity as a whole in 2015, that they finally did a swallow examination and discovered that he does indeed have dysphagia.  It is reported as mild, but also acknowledged that it will be worse when fatigued, or sick.  The problem is, one of his main symptoms is fatigue.  So we had to know.

It also turned out that the reason he would get throat infections and not know, was because he has no feeling on the left side of his throat.  Now that we do know, we have worked out what foods he can eat when he is feeling good, vs what foods he can’t eat when feeling tired.  It’s not about who’s at fault, or should they have done the investigations earlier.  It’s about keeping Derek safe because when he gets tired he chokes, and now we know why, we can work around it.

bitter or better

Derek and I came to that point where we had a choice.

We went for a second opinion in 2015.  We had ideas of what else we could do to try to improve his QOL.  We also wanted to try and keep him out of hospital.  He has come close a couple of times since 2014, but we have managed to keep him out.

How?  By talking to other sufferers of the condition, following the latest research, and learning to listen to his body.

By being proactive in his care, not leaving it to the medical world to fix or forget.

For 2 years we became like a stuck record on a lot of his symptoms.  The common question was BUT WHAT IS CAUSING IT?  Why did he always choke, why did he get so tired when taking the recommended replacement steroid doses, why did he get sick every time he suffered a fight or flight reaction (an adrenaline rush).  Why was he ending up in Hospital every time he got sick.

We know why he chokes now, we know that the text book answer for replacement steroids 3 times per day (an idea from 1973 which most Endo’s still follow) is wrong.  We know that when you get a fright you get an adrenaline rush.  But it also causes a call on cortisol to bring your body back from the fight or flight.  This is not something the Endo’s tell you when talking about “stress dosing for illness injury”.  We learned by trial and error.  We learned the difference between UP dosing, which is taking a little extra steroid for a one off situation and STRESS Dosing which is in the books and requires 2 or 3 times your daily dose for several days because of serious illness, injury, or stress like a family death.

So we have made changes to his dosing.  We have changed his eating, we have moved to 4 times a day with hydrocortisone, although we are considering 5.  The latest research out of England says maybe 6x/day and no more than 4 hrs apart would be ideal.  We have included DHEA, which is something most Endo’s don’t even consider for men, yet it is the most prevalent pre-hormone produced by the adrenals.

And we are getting on with life.  Medical appointments are now just annual routine follow ups where we tell the Dr’s what Derek is are currently doing, and how it is working.  We tell them what he has tried that hasn’t worked.

Otherwise he is at the GP’s because he is sick.  Sometimes we don’t know what is wrong, we just know that things are not right.

He still has problems.  As I write this he is sick and has been for 5 weeks, with what was for me a simple cold.  It lasted 5 days for me but for him it became a full on chest infection which he still hasn’t recovered from.

If Derek was working in the Office, he would have worked from home for most of that time, but because he was already working from home all week, he has been able to work for most of that time.  If he wasn’t able to work from home, he would have had to take sick leave for 2-3 weeks.

He has to take extra cortisol just so we can go out at night.  This is something not in the books.  It has been learned from trial and error.  The trial being taking it, the error being not taking it and ending up sick for days afterwards.  We have learned that Derek needs to rest the day after we go out, to recover.

He is not the man he was, but looking back on how sick he was 4 years ago and the major insult his body received, we realise how far he has come.

In those 4 years we have both changed and we both understand a lot more about both the medical condition and the lack of knowledge by the medical profession, for what is after all, considered a rare condition.

We have also learned the truth about the bogus condition that attracts all the “natural health professionals” that promote snake oil for Adrenal Fatigue.

We have learned people that inform us that Derek can stop steroids if he takes their snake oil, that the only way to shut them down is to shut them up with scientific fact.

Like anyone with a Chronic Illness Derek has good days, and bad days.  He has days when he can’t get out of bed, he has days when he can do things around the house.  And that’s fine, because after 4 years, that is what we know is the life of the Chronically Ill.

It doesn’t matter the illness.  If you learn about it, you can learn to live with it, and go from being Bitter to being Better.

Please note when I say BETTER, I don’t mean WELL.  I mean learning what your limits are, and learning to manage them so you can be the best your Chronic illness allows you to be.

Now you know it, forget it!

That is the Mantra of the Lecturers to Medical Student’s after being told about rare diseases.

I was talking to a Medical Student (just completed her 5th year) while I was with someone at the Emergency Department of a hospital recently.

We began talking about Chronic Illnesses and the fact that the families of those with Chronic Illness know more than many Dr’s about their particular condition.

She asked me about Derek’s illnesses…. “do you mind me asking what he has?”

No, I did not mind her asking. I am all for education.

I told her Antiphospholipid Syndrome. “Oh” she says, “I have heard of that”.

To say I was very surprised would be accurate.

I then asked her how she had heard about it.

Apparently, they were told about it in a Gynecology Lecture, had to learn about it in relation to pregnancy, and had a question on it in their 5th yr Exam.

They were then promptly told to forget about it, as they would never meet anyone with it.

She was not aware that Men could get it, or that it was something that can and does occur outside pregnancy!

Given that I know at least 4 women with it, and Derek has it, it isn’t that rare.

She knew nothing about the Catastrophic variant  or even that such a beast existed.

This is not the first time I have heard this about rare conditions.

Every time we query a Junior Dr or fully trained Nurse they say the same thing about Addison’s Disease/Adrenal Insufficiency. “We are told it exists, what the symptoms are, then told “but don’t worry, it is so rare you will never meet anyone with it”.  They are then shocked to hear about Derek.

If he is in hospital, the Medical Students are told to go see him, they will never meet anyone like him again!

No, they won’t. Because if they have a patient with either of Derek’s conditions, the patient will probably die before they are diagnosed.

Then there are those patients who suspect they may have a rare chronic illness is who are frequently (almost always) told “No, you can’t have that, it is too rare!”

Another useful thing all Medical Students are taught is: “if you hear hooves, look for horses, not Zebras.”

Just because a condition is rare does not mean it doesn’t exist.  It also does not mean that a patient is imagining things.

So what does this mean?  It simply means you look for Zebra’s if the Horses aren’t in the room.

How can you rely on the Medical Fraternity, when they so often get it wrong.

That is not to say they should know everything. What they should be doing is listening to their patients. Don’t rule out things just because they are Rare, or not “normally” found in that sex.

Listen carefully to your patients. If what you think is wrong comes back negative, give your patient the benefit of the doubt. Do the basic test to see if it IS  a “Zebra in the Room”. If you want to be arrogant, do the test to prove your patient wrong.

What is the worst that can happen?  The Patient is right, and you have to apologize.

Heaven forbid a Dr should find out they are not GOD!

Waiting for a Life Line

This blog was going to be about a thing called DHEA. That will come later.

This is more important because I am really annoyed, and need to get it out there. There are lots of others, not only in New Zealand, but all over the world, that suffer this problem.

It’s when you fall through the cracks.

We had it described to us that Derek is one of those people.

It’s like an apple sorting system. The tree grows an apple, that apple is picked. The really bad apples get taken out and put in the pigswill. The rest are then put through different tests/checks. As the apples go through the system, the low grade ones are gradually discarded or put aside for fruiting, juicing, discarding, until all you have left is export grade apples.

Every now and again an export grade apple gets thrown out with the bad ones. It falls through the sorting system and ends up in the pigswill instead of on the shop shelf.

Liken Derek to that apple. He goes through the first check point, and falls off the belt with the pigswill apples, but hey, they get re-checked just in case, so that’s ok. Someone will discover he is actually a good apple, and put him back on the right track.

NO!

He has AGAIN fallen through the system. And I am getting quite mad about it. I should not have to spend my life advocating for him, and fighting the Medical Fraternity to get things done. It’s not that they don’t want to do things, they just keep stuffing up.

2.5 years ago, he experienced a Medical Misadventure and suffered multiple organ failure. They didn’t notice (even though it was pointed out to them) that he suffered a brain injury at the time.

After 23 days in hospital (the first 5 fighting for his life) he was discharged from Wellington Hospital (CCDHB) and handed back to Hutt Valley Hospital (HVDHB). He then spent the next 12 weeks at home.

He was referred to a Rheumatologist for his Antiphospholipid Syndrome (APS), and consequent Catastrophic APS. He was referred to Endocrinology for his now trashed adrenal glands.

He SHOULD have also been referred to Occupational/Physiotherapy, for rehabilitation given that he suffered lung damage, transient heart damage and was laid up for 15 weeks trying to get some form of health back.

He wasn’t. He was examined by the Rheumy who said “keep taking warfarin, you look great, see you in a year”. He was seen by his Endo who said “decrease your HC to what the book says, oh, maybe you could also take fludro as well, see you in a year”.

We went back to both of these a year later. Same thing.

In the mean time, I am becoming more and more concerned about other things that are happening to Derek. Each visit to a medical “professional” I mention that he has difficulty swallowing. Each visit we are told, yes, we need to look at that. We will talk to/refer you to XYZ…

After changing General Practitioners in August 2014, we finally got a referral. On 18 Dec 2014 Derek went for a gastroscopy (read about that elsewhere, it’s a story all by itself). At the end of that visit we discussed with the Endoscopy Registrar the fact that the problem is at the top of his throat, between his mouth and his shoulder line. The Gastroscopy looks lower down.

The Registrar agreed that the Gastroscopy was not going to find the issue, and he needed to be referred to another department. Hang on. Haven’t I heard this somewhere before, MULTIPLE times? The question we were then asked, “did we want the her to do the referral, or our GP?”.

We definitely wanted the Registrar to do it, so we knew it would go to the right department in a good timeframe.

On 2nd Februaray we received a letter about an X-ray appointment. Yay, finally somebody that actually did as they promised, and sent the referral. Now they could do the correct X-ray to see why he couldn’t swallow properly.

WRONG!!!!!!!!!!!!!!!!

This was a repeat X-ray 6 months post his last bout of Pneumonia. When we checked in for that x-ray, I asked about the other one. NO, not in the system, never referred, not happening. Best course of action is to go back to the Endoscopy Dept and ask them what happened.

While sitting in the waiting room for Derek to have his X-ray he received a txt message which I read. Hello. It’s a reminder for an appointment for Monday at Rheumatology. That’s great, but what is the apt for, and why are we receiving a reminder. We have never received a letter telling us about the apt. Oh, did I mention that today is Friday?

After the Xray, we trudge up to Level 6, and ask about the reminder, but no original letter telling us about the apt. “Oh, we send them out about a week out from the apt in case it has to be changed”. That’s fine but again, Today is FRIDAY ,and the appointment is for MONDAY. The snail mail has been delivered today, and there is no letter telling us of the apt.

After this discussion, we trudge down to the ground floor again, and along the corridor to Endoscopy. We are told that the notes say that our GP is to do the referral. “Actually, No. We clearly agreed that the Registrar would do it, so we knew it would happen”. The Receptionist asks us to wait while she goes out the back and tries to sort it.

The Manager of the Dept comes out, she has the referral in her hand, which has been sent to the right dept? We told her that it hadn’t been sent anywhere. There was no record of it on the system.

She asks us to wait while she goes to X-ray herself to sort it. She then comes back and promises she will sort it. We try to explain our frustration at the inability of the system to get it right with Derek EVER.

We leave the hospital with a promise from the Manger of that department, that she will get back to us today regarding the referral.

We have now had a phone call back to say that it has been put on to another department, she is waiting for it to be “triaged” which means some obscure little officious clerk, at some desk somewhere, will put it in the system as “non urgent”, which means they have 4 months to act on it.

While I was sitting in the Endoscopy department waiting for the Manager, I was listening to the booking clerk making a booking. There is no denying that when they get a referral there is a timeframe for which they must make a booking. They DELIBERATELY book you on the last possible date to fit the timeframe. I you are classed as semi urgent and must be seen in 3-4 weeks, they put you on the last day of week 4, even if there are spaces available on the first day of week 3! I actually heard her doing this.

We are due to have another phone call from HVDHB to confirm his booking on Monday. We have been promised (and I have the phone call recorded), that once the decision is made as to urgency, the Manager of Endoscopy will get back to the relevant department and tell them that they need to hurry up and make it urgent.

I then jokingly asked if they had a system in place to make sure that the booking didn’t clash with any other booking Derek might have (he gets lots). I was gobsmacked (absolutely shocked) to find that NO, there is no system in place for that! They have to manually check!   And we all know how many of the booking clerks would waste their time doing that!

So where does that leave us? In the hurry up and wait chair again.

We currently have a Formal Complaint being investigated by the Health and Disability Commissioner about his treatment at CCDHB. Once we have seen the report on that, I will be putting in a complaint about the HVDHB and their treatment of Derek.

Sometimes all the stars align and you sail through life with ease. Other times, all the mud pits align, and you end up sitting in pigswill for years until someone throws you a lifeline.

We are still waiting for our lifeline!