Len Woodward was a founding Trustee of aHUSUK and a founding affiliate of the aHUS Alliance. He is also a Director Trustee of aHUS Alliance Global Action. In this series of blogs Len Woodward describes how and why he reluctantly became an aHUS advocate, and where this journey continues to lead.
Click HERE to read Part 1: A Change in Gear
Where the heck did that come from to hit us?
No one in our family had experience of kidney failure, or so we thought, on 19th January 1997 when our daughter went into respiratory and heart failure in front of our eyes, in an old Victorian Hospital that had begun life as a workhouse and used also as a prison for soldiers during World War 1(now long pulled down to make way for housing development).
It was a Sunday, the hospital could not afford to open its dialysis unit on a Sunday and so the fluid that builds up when your kidneys stop working cannot be expelled without an artificial kidney machine. So the fluid builds up in the lungs like someone drowning and the heart struggles against the odds to continue to function.
“Well someone got their figures wrong” said the Consultant in the Intensive Care Unit after the renal ward team acted professionally to undo the effects of the treatment which was killing her.
Too much fluid in (kidney patients are often treated for dehydration) not enough fluid out. The Intensive Care Consultant said, while showing us an X ray of our daughter’s lungs, that the errors in sums had caused one lung to fill up completely and the other was half full, our daughter was now on dialysis to get the fluid out of her. It was the middle of the night, but now Monday,so a dialysis machine could be brought into use to resolve something which earlier the previous day it could have prevented. That realisation would mean our patient engagement and acceptance of clinical decisions would now change for ever .
We were in the “car crash” scenario, which we now know would be familiar to many of the those rare people who have experienced a catastrophic episode of atypical Haemolytic Uraemic Syndrome, or aHUS .
aHUS is very rare although no one knows how many people in the world have survived an encounter with it. Estimates vary between 15000 and 42000. Each year in the UK there will only be around 25 incidents. Though we were told by nursing staff back then that what my daughter had was often seen; about 1 case a month in that one hospital alone. Clinical staff sometimes bluff when they do not know.
Haemolytic and Uraemic are just describing the symptoms of the disease. aHUS patients will experience severe anaemia caused by their red blood cells being destroyed because the uncontrolled clotting in the body’s capillaries leave smaller and smaller gaps for the blood cells to squeeze through. The gaps gets so small that the cells explode like a balloon would if squeezed too much. This is known as haemolysis.
This uncontrolled process seems to happen most frequently, but not exclusively, in the capillaries in the kidney . When it does, it causes the filtration system in the kidneys to become blocked. Once blocked the kidneys are not doing what they should be doing , there is, among many other effects, a buildup of uraemia in the blood a sign that the kidneys are failing to do their work.
aHUS is not the only disease in which this happens. There is typical version ,HUS , which is ten times more common. It is triggered by e.coli poisoning. The excessive haemolysis is the result of the virus binding to red bloods cells so that the body’s immune system targets every cell to get rid of the virus in a form of friendly fire. Whilst HUS is the result of poor hygiene in food preparation or animal contact, aHUS can be triggered by other factors and aHUS patient’s immune response is uncontrolled because of minute inherited defects in the aHUS patient’s own immune control system. The controls are needed to stop an excessive and unnecessary immune response to whatever has triggered it.
No one can be sure what triggered the illness in our daughter. At the time she was living in Glasgow when the news was full of a story about an E. coli outbreak, the largest outbreak ever experienced in the UK. In a nearby town poor hygiene practices by a local butchers had resulted in E.coli contaminated meat being served at a party causing party goers to become ill. The youngest and oldest of them dying from kidney failure .HUS was not necessarily something that would have been mentioned in the newspapers.
There were many people in the Glasgow area who were experiencing stomach upsets at the time, including our daughter. But unlike others who recovered quite quickly she did not. In the following six weeks or so her condition deteriorated until eventually a local doctor discovered from a simple blood test that she was in kidney failure.
It did not take long for the Renal Consultants, in the hospital that she was hastily referred to, to see from the tell tale signs in the blood that the cause of the kidney failure was HUS, although by then no evidence of E.coli could be found in any cultures that were taken.
Just what had hit us out of the blue. How could someone who had that summer toured the USA including universities in New York and Bloomington in perfect health now be laid so low.
Although we did not understand much at the time and had no idea then why the doctors were looking for other possible causes pregnancy, AIDS, drug use there was a clue in our family history which we did not know was significant.
Little did we know about how our lives would now change course and where the journey would take us.
Click here for Part 3