A young girl & her father enters OPD. 14y and is deeply jaundiced. Teary eyed, her father requests ONLY one thing
“She’s advised liver transplant. We are poor. Can you please help us avoid it?”
I look at the girl the father
& brace myself for the worst case I’d ever seen…
It was just 2 months back, the little girl started feeling lethargic. Severe loss of appetite ensued.
She did extremely well in school, but the last 2 mo were difficult.
She had tremors of both hands and found it difficult to concentrate.
And it was not only that…
..her parents noticed that her skin was progressively darker than before.
But she was out in the sun most of the times.
6 wk ago as the fatigue progressed she started noticing high colored urine and then few days later, her eyes were jaundiced.
At the two hospitals she was admitted for over 6 wks before coming to our Unit, docs had initially diagnosed her with Wilsons Disease & later at second hospital, with Autoimmune Hepatitis.
Wilsons is a genetic disorder which results in problems with copper handling by the body.
Excess copper gets deposited in the brain and liver and leads to symptoms such as tremors, lack of concentration, severe neurological problems in certain children along with liver disease.
The liver disease is peculiar – it can be without symptoms, and later in life, present as cirrhosis
as a ‘flare’ of WD in which young patients suddenly develop jaundice and hepatitis
and, in some cases, leading to Wilsonian Crisis which is calamitous destruction of red blood cells, severe anemia and multiple organ failure which progress to acute or fulminant liver failure req transplant for survival.
Autoimmune hepatitis (AIH) – wer, immune cells in body goes haywire, starts attacking liver
in the girl, a particular antibody was positive, many features of AIH such as raised IgG levels were more suggestive of AIH…
But on various treatments, her jaundice sky rocketed and reached 26mg/dl and she started to develop fluid inside the abdomen – called ascites. This was terrible. It meant that her 14-y old liver was failing.
And there was only one thing left since the liver was failing and the treatments were not benefiting– a liver transplant.
On the advice from a relative, a doc, the girl’s father asked the Gastroenterologist regarding a liver biopsy – the only evaluation that was pending
but the answer was a cold ‘NO’.
“Bcoz,” the doc said,“anyway the failing liver had to come out and a new liver has to go in – so, there was no need to waste time on a biopsy, the child needs a transplant.”
Distraught, the father did not know what to do. He was teacher in a nearby school and he did not have 25 lakh rupees to spend on a lifesaving surgery. But it was his daughter and he had to find a way.
So, at the advice of another patient, who was also a distant relative of his, the father came to know about us and visited us with the girl for a third opinion.
One look at the girl, I knew…this would either end in a death or liver transplant.
But the only request he made was, that he wanted to avoid a transplant and I had to find ways to avoid death in the young girl, the absence of a transplant. A thorough review of all tests were done. Features of Wilsons…was there and so were features of autoimmune hepatitis. But this was impossible – how could 2 very different conditions work to injure the same organ?
Something was missing – there was a primary event that was creating some kind of confusion.
A red herring. A distraction.
So, we applied clinical scoring criteria for Wilsons disease & Autoimmune hepatitis.
Found that both were only “possible”, but not definitive
Without a liver biopsy, we could never be sure of autoimmune hepatitis.
Without a genetic study, we could never be sure of Wilsons…
Without both, this was a dead end for both the doctor and the young patient.
The father had little money left and the treatments had not yet started. Genetic testing was expensive, but was MANDATORY and so we decided to help him.
I paid for the genetic testing from my salary…..and samples were sent to Pune.
And then, we decided that it was time for the liver to speak and so a biopsy was done.
The pathologist was notified that we needed results within 48 hours bcoz a young girl’s life was at stake. She was getting sicker and her belly fluid was increasing.
After seeing the biopsy, the senior pathologist calls me up late eve. “Ask the patient to stop drinking alcohol. The liver biopsy is highly suggestive of severe alcoholic hepatitis.
So much of fat and inflammation and classical alcohol induced liver injury.”
I am dumbstruck
I tell her, ‘But Madam, this is a 14-year-old girl and there was absolutely no way she has alcohol use disorder.’
And it did not end there.
“There is something more”, she says. “You see, the severe alcohol liver injury was masking something and…..only after this is taken care of, can we actually see what the underlying liver looks like.”
This meant only one thing. I had to first, reverse the severe alcohol injury, and then subject the child again, to another biopsy – known as serial liver biopsy to identify…other hits to the liver.
But first, she had to survive, for me to do that. And I tell my pathologist “How is this possible? Alcohol on top of another unidentifiable liver disease!”
To which she says, “you are missing something in history. Keep her alive…so that we can diagnose her,”
Transplant was not an option and we were not ready to embrace death. Yet.
So, we go back and ask the parents about her diet, supplements and alcohol-based drugs and she has not been on anything remotely connected to alcohol UNTIL…the mother says, “what about her anti-seizure syrups?” to which I get up and ask “anti-epileptics?”
I was furious – “Why did you not tell me about anti-seizure medicines?”
To which, the father replies, “But Sir, these are not medicines – these are supportive…natural supplements prescribed by our Ayurvedic practitioner,
becoz she had childhood seizures and we were quite concerned about modern medicines causing long term harm”
And I said
Show me those syrups and lo and behold – a bunch of Ayurvedic Kashayams/Arishtams…
and some herbal mixtures came into the spotlight.
“How much did she take and for how long?”, I ask
“Sir, its been going on since 3 years, we have lost count of the bottles we have bought,” he says
“But we have never stopped these, as she has never had seizure on these herbals”.Arishtams are Ayurvedic alcoholic decoctions “supposed” to have medicinal value. But there is absolutely no evidence of benefits.
At times when real alcohol is difficult to procure, people flock Ayurveda shops to beget this ‘alcohol’ medicine for cravings.
Arishtams contain anywhere from 10% (ideally) to 30% (in poor preparations) alcohol and this girl was on it for 3 years.
So, we collected the herbal decoctions and sent it out for analysis.
Meanwhile, the genetic testing for Wilsons disease came back negative…
Tests for alcohol comes back at total 26%
And without wasting more time, we start her on steroids for her severe alcohol-injury (this is the recommended treatment)
Initially steroids at the other hospital had worked, but the addition of Azathioprine had caused more trouble…
After 4 weeks on steroids, her jaundice drastically reduced, coming down to 4.2 mg/dl and the abdominal fluid resolved and she started gaining appetite. Alcoholic hepatitis responded to steroids. Finally.
But it did not end there. It was time to look at the liver again…And I tell the father, that there was something more. A second biopsy was needed. And without even thinking twice, he says “Sir she has come this far, and we know how your team has been working hard. My daughter is now under your care. Please take care of her…and give her back to me when you know she is safe.”
A second biopsy was done. Strong girl. What a fighter. At the brink of liver failure, transplant candidate.
TWO liver biopsies. And it gave me courage.
If this girl can go through this, I should go through with her. The second biopsy reports came in. And it opened up a Pandora’s Box. It showed “hepatoportal sclerosis” – a rare pattern of liver injury which comes under the group of diseases called – non-cirrhotic portal hypertension – in which in the absence of cirrhosis, patients develop… increased liver pressures, fluid in abdomen. The alcohol liver injury was masking it.
But what was causing it? Genetic studies were normal. So, we stepped back, to look at the whole picture again. Pigmentation, tremors, poor concentration, liver…
showing features of non-cirrhotic portal hypertension. There was only one cause and we knew where it came from.
Arsenic poisoning is associated with a host of systemic diseases. The most commonly affected systems are skin, liver and brain… In the liver,
Arsenic poisoning is associated with non-cirrhotic portal hypertension and has been studied in people consuming ground water contaminated by Arsenic.
This girl had Arsenic poisoning – hair and nail samples showed high levels.
But hair and nail samples…of the parents and her other siblings did not show Arsenic.
Meaning it was not a common source. Only something that the girl was exposed to. And so, we took the untested Ayurvedic herbal formulations and send it for analysis.
And to much surprise, the tests..show high Arsenic in Ayurvedic drugs
And now we did it.
We finally had a diagnosis.
Severe alcoholic hepatitis due to Ayurvedic Arishtams, along with hepatoportal sclerosis due to chronic Arsenic poisoning from Ayurvedic mixtures.
Even though the girl barely escaped… transplantation, her Arsenic related liver disease was to stay. She went home after spending 6 months of follow up and treatments with us. Before leaving, her father thanked me and said, “Sir, I asked you to help me avoid a liver transplant. And your team did that for me I can go home becoz you kept your promise. I can now spend time with my daughter, and this time gained, is like a bonus for me. I do not care what the future holds.”
The future is bleak.
Chronic arsenic poisoning brings out its ugly head in the long term.
The biggest issue is development of skin and liver and lung cancers called Angiosarcomas
It has been 4 years now
The girl is well and is acing her studies
She can never get married. But I have not told the father this yet.
And I am waiting for the day when I have to see her again and diagnose her with another heart-wrenching event due to the Arsenic that is slowly eating her from inside and becoz herbal medicines were considered natural and safe.