A toddler who was taken to hospital for suspected teething problems was diagnosed with an ‘extremely rare’ life-threatening illness that affects only one in five million people.
Eight-month-old Henry Norton, who lives in Albury, NSW with parents Craig and Melissa and brother Lachlan, was diagnosed this month with atypical hemolytic uraemic syndrome (aHUS).
The genetic disease can cause abnormal blood clots to form in blood vessels in the kidneys, potentially restricting blood flow.
The disease, which affects one in five million people and has been detected in just 14 people in Australia, can lead to kidney failure if left untreated.
Henry’s mom thought her “typically happy baby” had teething over Easter weekend after he wouldn’t take fluids.
Henry’s mom thought her ‘typically happy baby’ had teething over Easter weekend after he wouldn’t take fluids (pictured, Henry)
Eight-month-old Henry Norton (pictured, with mom Melissa) was hospitalized with atypical hemolytic uremic syndrome (aHUS)
But when his condition didn’t improve and he started vomiting, she rushed him to the emergency department at Albury-Wodonga Health Hospital.
Hospital staff initially sent the family home to say he was simply dehydrated, but Ms Norton returned the next day, concerned about her son’s deteriorating condition.
“Henry was extremely pale, refused to eat and had thin and very dark brown urine and was very lethargic,” Ms Norton told Daily Mail Australia.
The boy’s kidneys were failing, so hospital staff placed a tube in his throat, allowing him to breathe, and recommended that he go to the Royal Children’s Hospital in Melbourne.
He also had to be put into an induced coma to stabilize his heartbeat.
Ms Norton said she and her husband were ‘literally walking on the floor’ at the hospital as staff fought to stabilize the boy so he could fly to the ICU at Melbourne hospital.
“I was really scared, I really thought the worst,” Ms. Norton said.
“To cuddle your baby and take him into a theater room and not know if he was going to survive this plane ride or not — that was ridiculously scary.”
It was 4am when she flew with Henry in the air ambulance that took the couple to Melbourne’s Rosella Ward hospital.
The genetic disease can cause abnormal blood clots to form in blood vessels in the kidneys, potentially restricting blood flow (pictured, Henry)
“Henry was extremely pale, refused to eat and had reduced and very dark brown urine and was also very lethargic,” said Ms Norton (pictured, Melissa and Henry pictured after being sent home by staff at Albury Hospital)
It was 4am when she flew with Henry in the air ambulance that took the couple to the Rosella Ward at the Royal Children’s Hospital Melbourne (pictured, as she loaded the air ambulance in Albury)
Ms Norton said she was on autopilot on the plane of the hospital’s PIPER team, after not sleeping for 30 hours.
She arrived in Melbourne with Henry and staff checked the boy’s vital signs and ran tests to rule out various causes – while her husband drove down to meet her.
“It was pretty overwhelming — you have 14 people in a room working on your son who’s sedated, it’s pretty crowded,” she said.
Henry woke up after two days when his breathing tubes were removed, which gave Ms Norton the “best feeling of being able to hold your baby and give him a bottle.”
His kidneys were still in trouble, so she was limited to how much milk she could give him.
She said the family felt really supported by the hospital.
“They were absolutely incredible with us. They saved our son’s life,” said Ms. Norton.
It was also the couple’s first time without their eldest son Lachlan, 23 months, who they’d left with her parents in Albury – so emotions were running high.
Henry was eventually diagnosed with aHUS – atypical hemolytic uraemic syndrome – a rare disease that causes clots to form in blood vessels.
It is a nasty condition that can lead to kidney failure.
The boy also got a blood infection of his thigh line that was placed in him instead of a normal IV that the staff couldn’t use because the boy was so swollen.
The infection caused his heart rate to rise to an alarming 207 beats per minute and his temperature rose to a maximum of 39.6.
The mother said they waited until he was well enough to take home and arrived back in Albury late last week.
From now on, Henry will have to go to Albury hospital twice a week for check-ups, in addition to four-hour fortnightly visits to Melbourne hospital to treat his condition.
Ms Norton said this routine will be ‘forever’ and that if the boy gets a cold or gastrointestinal it is likely to trigger a flare-up that will land him back in hospital.
The mother started with one GoFundMe page to help with the costs of her son’s care after she was forced to quit her job as a bartender.
Henry woke up after two days to have his breathing tubes removed, giving Ms Norton the ‘best feeling of being able to hold your baby and give him a bottle’ (pictured)
“It was quite overwhelming – you have 14 people in a room working on your son whose sedated, it’s quite full,” said Ms Norton (pictured, Henry sedated in ICU)
The funds raised will also support the family with hotel stays during their trips back and forth to Melbourne.
They eventually hope to gain access to housing at the Ronald McDonald House as soon as it becomes available.
Meanwhile, the doctors told the couple that the boy can live a normal life as long as he keeps up with the treatment and doesn’t miss a dose.
“Our goal is for him to live a normal life and for him to become more aware of this disease,” she said.
The crowdfunding page has so far collected $4,070 in support.
WHAT IS ATYPICAL HUS?
Atypical – this is a less common form of the disease
Hemolytic – tells us that the blood system is involved
Uremic – this tells us the kidneys are involved
Syndrome – more than one thing is going on at the same time
aHUS is an extremely rare disease caused by an error in the complement system. The complement system is part of your body’s immune response that attacks insects.
Your body has a built-in system of protective proteins that prevent complement from attacking your own cells.
In aHUS, complement begins to attack the body’s own cells, especially those lining the blood vessels. This leads to the formation of clots in the small vessels.
The most commonly affected organ is the kidney, but all organs can be affected. If left untreated, it can be a life-threatening disease and the majority of people would go on to develop end-stage kidney failure.
WHY DOES AHUS HAPPEN?
A genetic defect in the complement system or protective proteins causes complement to target the body’s own cells.
Some patients may also develop autoantibodies against the protective proteins. These autoantibodies then ensure that the protective protein no longer does its job.
An episode of aHUS can occur at any age, even if you were born with the genetic defect. This is because there is usually a ‘trigger’ that starts the process.
Common triggers include: gastrointestinal disease, respiratory disease, medications, and pregnancy (especially in the third trimester of pregnancy and the immediate postpartum period).
HOW DOES AHUS FEEL?
Most patients will initially feel unwell with increasing fatigue. Sometimes it can seem like you’ve picked up a bug or a virus that just can’t seem to get better.
If you are at risk of developing aHUS and are concerned about any symptoms, you should seek medical advice.
Source: National Renal Complement Therapeutics Center