On many occasions I have shared that my preferred stance is prepared, but sometimes a surprise can appear.
Back at the beginning of February 2024 I got a monumental surprise, one I certainly wasn’t looking for.
After routine bloods, I received a phone call from my favourite number 4980, Southampton General Hospital.
I know the number well, but it is always a surprise whom maybe ordering my attention.
On this day it was the Endocrine Specialist Nurses, enquiring if Billy was available for a Synacthen stimulation test on the 29th February.
The blood test had shown up deranged Cortisol levels.
After a very cryptic conversation, the Specialist Nurse explained that all would become clear at the appointment.
Before, she ended the call I asked her 2 questions to gauge how worried I should be, due to the nurse being very reluctant to share what they were looking for.
- Is this a lifelong condition?
- Can this condition be managed or cured by an operation or medication?
So, the answers where yes.
Not great but manageable if this becomes Billy’s reality.
If you know me personally, you know what I did next.
I underwent the ultimate cardinal sin; I went to Google to find answers.
Folks this isn’t recommended, but can be done with caution.
If you are going to delve into this endless world of information, remember there is a lot of unreliable sources that tell more horror stories than truth.
My normal go to sites is the NHS or charities like the Children’s Liver Disease Foundation (CLDF) before going anywhere else, this gives a good foundation of understanding to build on.
Also, its raw facts rather than personal experiences.
Within this quick search, I found out we were looking at Addison’s disease.
Addison’s is a rare autoimmune disorder that affects the Adrenal glands.
The Adrenal glands have one main job, hormone production, it makes Cortisol and Aldosterone.
When this system is damaged it can under produce these hormones that lead to unpleasant symptoms like fatigue, muscle weakness, low moods, weight loss, increased thirst and so much more.
Fatigue is a beast we know well and fight quite regularly.
This can be a battle, especially when Billy’s expectation are out of control.
It feels at times he’s trying to make up for lost time, but also stock pile them just in case isolation or hospital happens.
We love this about Billy and we have tried to go with him, but it ended in burn out.
So, these days we try and help Billy manage his diary, to keep this beast under control.
After seeing that Billy had some of the traits of Addison’s, I went in to have a look at the causes.
On first glance, I was a little confused why this would be connected to Billy.
Nothing jumped out.
Yes, Billy had cancer and his immune system was compromised, but it hadn’t spread or attacked his Adrenal glands.
After a couple more searches I saw it
Prednisone is a type of oral corticosteroid that can be used to replace cortisol.
However, taking prednisone for a long time can disrupt hormone production in the adrenal glands.
I hadn’t made the link between Corticosteroid and the Prednisone.
Billy had been put on Prednisone after transplant alongside his anti-rejection drugs.
Prednisone is an Immunosuppressant drug used to prevent the body from rejecting the new organ.
Billy started to be weaned off Prednisone in May 2022 after an Endocrine doctor had suggested this was the cause of Billy’s short stature.
But, by this time Billy had already been on Prednisone since August 2018, so 3 years and 9 months.
Also, within this time period, Billys dose had significantly increased due to his cancer treatment.
This was done to protect his liver due to his anti-rejection medication being stopped as it was the cause of the cancer.
So, in Billy’s case they were working at the motion that the Prednisone had made his Adrenal glands lazy.
So, what was I feeling?
My initial emotion was sadness, not for me but for Billy.
It feels Billy never gets a break and it would be one more thing he would have to learn to live with.
He copes really well with his ever-changing life, but I do worry he may grow to resent his many conditions and the commitments they bring.
For me, this probably sounds a little hard-nosed, but what will be will be.
I have aways questioned his stamina and fatigue and thought they may be right.
If so, they may be able to perk him up and give him a new lease of life.
Who knows.
After milling these findings over in my brain, I must try and put them to bed until we need to go in for active investigation.
I have found worrying about the ‘what if’s’ can ruin the time we do have together.
And we can’t be having that!
But also, I must remind myself that we are not there yet, it’s only a query not a reality.
Billy is a child that hates surprises.
So, for us, a couple of days before the investigation I start prepping Billy.
He likes to be told what will happen to him and we do this through social storying.
I tell him a story about ‘Billy the Brave’ and then he likes to ask questions.
This is repeated over a couple of days.
This helps alleviates his worries and allows us to understand the areas that he’s struggling with.
If Billy is worried about an area, he will repeat this section like a broken record.
I have found if this is left to the day, he may have a melt down when time is not on our side to work through such worries.
Also, if needed, we role play with Kevin the doll or play with face masks if he’s being sedated.
Kevin is a little naughty too, which makes Billy laugh and helps him retain this information.
We also talk about what makes the medics lives difficult and easy.
I have found preparation is the key for long term success with Billy.
On the morning of the 29th Billy attended school before going to see the Community Nurses at their offices next the hospital at 1pm.
The Nurses accessed his Port-a-cath for the bloods and solution to be administered.
Arrived at hospital at 2pm and bloods were taken from his Port-a-cath as a base line.
Synacthen solution was given, then wait and take bloods at 30 and 60 minutes intervals.
Then remove Port-a-cath and come home.
Results take about a week.
On this day, Billy was on good form.
He was cheeky, fun, talkative and happy to be the patient.
We did have a little complication with the Port-a-cath not working, but we made fixing it into a game.
With a little problem shooting between us all, nurses, Billy and myself, we managed to fix it with a large drink.
Billy is so good at remembering what worked before and will offer this advice with a laugh.
I think the medics think we are bonkers, as one of Billy favourite Port-a-cath fixes is suggesting dangling him upside down by his legs.
He looks for their reactions before busting into laughter and saying ‘Joking’.
What makes it funnier is, Billy has natural timing with pranks.
After the wait, the call finally came.
Billy’s test results were in normal range.
So just like that, the motion was forgotten and life continued.
Remember you may not feel strong but you are
Love
The Rose-Tinted Mum
The Rose Tinted mum 