Juanita Bradfield was not worried when she took her three-month-old son, Judah, for the routine post-birth check up in December 2021.
Since birth, Judah had great weight gain, he was meeting his milestones and he was in the healthy range on the growth chart.
He was seemingly the picture of perfect health.
But then Bradfield, a mother of four from Mount Croix, Gqeberha, mentioned casually to the paediatrician that Judah’s stools sometimes looked different from those of her other three children.
They were consistently loose and a pale white or grey.
Immediately the paediatrician admitted Judah to Life St George’s Hospital for testing.
“Juanita and Judah spent three nights in hospital and Judah underwent a barrage of tests and prodding and poking,” dad Glyn said.
“We were told on the afternoon of Saturday, December 11 2021, after all the scans and tests, that they suspect Judah has a severe and fatal liver disease called biliary atresia, but they would need to refer us to the Wits Donald Gordon Medical Centre in Johannesburg for more testing.
“Our first thought was ‘no ways, this can’t be true’.
“Utter shock and disbelief, but ultimately a resoluteness to do what we needed to correctly diagnose our little gift’s ailment and get him the best care possible.
“In December, his liver enzyme levels indicated his liver function was poorly and the biliary atresia diagnosis was apparent.
“Months later, however, a CAT scan would reveal that he actually had Alagille syndrome and not biliary atresia specifically.
“The end result is the same for him though — he needs a liver transplant,” Glyn said.
The Bradfields immediately got tested to see if they were suitable donors for their son.
“Initially Juanita was going to be his donor but after a CAT scan revealed her liver was anatomically unsuitable for Judah and I was deemed an unsuitable match, a call was made to close family and friends via our WhatsApp broadcast list for a donor.
“A bunch of people came forward to be tested.
“The first round of paperwork was sent out to people who put their hand up,” Glyn said.
One of the people who put up their hand was Ashleigh Darne, a family friend who turned out to be the perfect match.
Darne said: “It was never on my radar to even consider being his donor.
“Until the one day I felt the Lord whisper to me that I should consider asking for the initial application forms.
“To be honest, I brushed it away without much thought until I heard the Lord whisper to me again and again.
“I chatted with my husband, Danny.
“I realised this is something we were in the position to do.
“At the time I was not even 100% sure what blood type I was, but I suspected I was the same as Judah.
“Once I submitted those forms, my decision was already made. I had the support of my family.”
The transplant, which involves Darne donating a portion of her liver to Judah, is scheduled to take place at the Wits Donald Gordon Medical Centre on August 18.
“It is obviously a big thing for our family to digest, and I’m trying to prepare my two girls emotionally,” Darne said.
“I have had many hours lying awake.
“There are just so many working parts to this whole journey as it involves two families, grandparents who need to fly in from other provinces to assist, employers, households that need to be run and so on.
“With regards to the transplant, I have peace.
“The Bradfields are an incredible family and we love them very much.
“We are excited for Judah’s story that is only just beginning,” she said.
The Darnes offered to champion the Back a Buddy page for the Bradfields long before Ashleigh ever considered being a donor.
Glyn said: “The funds raised through Back a Buddy have helped alleviate this massive financial burden immensely.
“Those funds will enable us to cover our donor and her husband’s transport and accommodation to Johannesburg for the transplant, as well as ours.
“It was all of these peripheral costs that can add up so quickly and create massive burdens and the collective generosity of so many has helped us alleviate so much of that.”
The journey since Judah’s diagnosis has been described by Glyn as “nothing short of a Ratanga Junction-like rollercoaster — on steroids”.
“There have been extreme lows, some extreme highs and everything in between.
“Judah also suffered from severe reflux up until about nine months.
“He threw up just about everything that went into his mouth.
“This also made his sleep erratic.
“Sleep periods of longer than 40 minutes at any time of the day or night were non-existent.
“Countless nights Juanita would be up with him all through the night, trying to keep him as upright as possible to help his milk stay down, or to rock him to try prohibit him from scratching himself to pieces.
“One of the worst signs of liver disease is pruritus [itching skin].
“But the itch these babies feel is like an itch that can’t be scratched.
“It feels like it’s underneath the skin.
“Trips to Johannesburg for progress testing have made things very disruptive on family life with our other three kids as well.
“All these new challenges have certainly added some difficulty to life, yet we still have so much to be grateful for and we do our utmost to focus on our blessings not our difficulties,” Glyn said.
The team at Wits Donald Gordon Medical Centre working on Judah comprises transplant surgeons, paediatric hepatologists, paediatric intensivists, transplant co-ordinators, dietitians, physiotherapists, microbiologists and ward sisters.
“It takes many specialised people to make sure the child is afforded the best chance,” a representative from the team said.
“Ultimately, the disease results in chronic liver disease, which is fatal.
“The only options available are to mitigate the progress by re-establishing bile flow with the Kasai operation, or a liver transplant.
“Life expectancy varies greatly as each child has a unique course which is affected by multiple factors.
“The success of transplant is dependent on the support provided and close monitoring of the child from diagnosis to the point at which the liver begins to fail.”
Alagille syndrome explained
What is Alagille syndrome?
It is the name given to a particular type of liver condition called biliary hypoplasia in association with at least two other signs in other parts of the body.
Alagille syndrome affects about one in every 100,000 live births and occurs equally between boys and girls.
What are the associated features of Alagille syndrome and how do they affect the child?
Biliary hypoplasia: The drainage system in the liver is made up of bile ducts. In Alagille syndrome, these ducts are not fully developed and this leads to poor bile flow, causing some degree of liver dysfunction and sometimes jaundice.
Typical facial features: These include a prominent forehead, deep-set eyes and a small chin. These features do not make the child look abnormal, they are merely clues to the diagnosis.
Cardiovascular abnormalities: There may be a narrowing of the artery leading form the heart to the lungs; this is called pulmonary artery stenosis. Effects range from very mild with no symptoms to being more severe and requiring surgery.
Abnormal shape to some of the bones in the spine: There is no cure for Alagille syndrome but there are treatments to improve quality of life. — Children’s Liver Disease Foundation
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