August 23,2019

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Everything About Biliary Atresia – A Liver Disease That Affects Infants

One of the critical liver diseases that happen only in infants is Biliary Atresia. Even though the chances of its occurrence are rare, Bile Atresia has the potential to do some serious damage to the child’s body.

Bile Atresia causes the bile ducts to swell and become blocked. Some infants get Bile Atresia in the womb itself, while in general, the symptoms appear only after 2 to 3 weeks after birth. Before delving into the problem of Bile Atresia, one should know the importance of Bile ducts in the human body, and how the swelling of it can hamper the child’s overall health.

Bile is an essential fluid produced in the liver that helps in digesting fats and also carries waste products from the liver to the intestines for excretion. Bile ducts, on the other hand, are pathways that transport the bile to the small intestine.

When an infant has Biliary Atresia, the bile flow through the bile duct is blocked. It causes the bile to be trapped inside the liver, causing damage and ultimately scarring. Once this happens, the process of filtering toxins stops, leading to further deterioration of the liver.

CAUSES OF BILIARY ATRESIA

The exact causes of Bile Atresia in infants is still not known. However, doctors believe a certain number of factors can trigger Biliary Atresia in the babies.  Some of them are:

Variation in Genes: The changes in genes are called mutations, which increases the chances of Biliary Atresia in infants.

Immune System Problems: Several studies have suggested that an enhanced auto-immune response targeting the bile duct epithelia leads to its damage.

Exposure to Toxins: Researchers have found that exposure to select toxins and toxicants may contribute to the pathogenesis of Biliary Atresia.

Certain Viral Infections: Some viral infections can also cause inflammation in the bile duct, resulting in its blockage.

SYMPTOMS OF BILIARY ASTERIA 

It is vital to note that babies having Biliary Atresia seem normal at birth. The symptoms manifest only after 2 to 3 weeks. Some of the most common symptoms are:

1) Yellowishness of Skin and Eyes

The first signal of Biliary Atresia in babies is the yellowishness of eyes and skin. This condition is commonly known as jaundice. Infants can have jaundice at birth, but it usually goes away within 3 weeks. However, jaundice caused by BIliary Asteria lasts more than that.

2) Dark Urine

The build-up of bilirubin in the liver causes the darkness of urine. It is filtered by the kidneys and removed through the urine.

3) Gray or White Coloured Stool

This happens when there is no bile or bilirubin coloring in the intestine. The abdomen shall also swell due to an enlarged liver.

4) Sudden Weight Loss 

A rapid weight loss in infants is often due to an increase in jaundice. Aggravated jaundice can also cause frequent nosebleeds and itching.

Diagnosis Of Biliary Asteria 

Many other liver diseases have the same symptoms as Biliary Asteria. Hence, doctors shall do several tests to get the correct diagnosis. They may do some or all of the following tests:

X-rays: X-rays help detect the enlargement of the liver and spleen in the infants. The images are obtained with the help of small radiation that is shown on a screen or a computer.

Ultrasound: In ultrasound, high-frequency waves are created that will show clear images of the liver, gall bladder and bile ducts.

Liver Scan: Liver Scan helps to get a clear image of the liver and bile ducts, through special X-rays. Through this test, the blockage in bile flow is seen, if any.

Liver Biopsy: A sample of tissue is taken from the infant’s liver and is checked for any problem. This test reveals the likelihood of Liver Biopsy.  Liver Biopsy helps rule out other liver problems like Hepatitis.

Diagnostic Surgery: Unlike Liver Biopsy, a Diagnostic Surgery confirms a Biliary Artesia. In this surgery, the doctors can see the injured piece of bile duct going from liver to small intestine.

WHAT ARE THE TREATMENT OPTIONS FOR BILIARY ATRESIA? 

Surgery is necessary in the case of a Biliary Atresia, as it can prove fatal without it. There are mainly two types of surgery conducted to treat  Biliary Atresia. They are:

1) Kasai Procedure

In this surgery, an alternate pathway is created which connects the liver to the intestine, allowing the passage of the bile. This path goes around(bypasses) the blocked bile duct. This surgery keeps the child in good health for a few years, after which the bile can get accumulated again in the liver. A liver transplant is necessary after that.

2) Liver Transplant

In a liver transplant surgery, a patient’s diseased liver is replaced with a whole or partial liver from another person. After this surgical procedure, the liver begins working and the child’s health starts improving gradually.

The child should be regularly given medicines prescribed by doctors after liver transplantation. It is to ensure that the child’s immune system doesn’t attack or reject the new liver. These medications are commonly known as anti-rejection medicines and help in weakening the immune system’s response.

It is also crucial to note that in India, only 1 out of 3000 children get liver transplant surgery. It happens due to the lack of affordability and accessibility of this surgery.  The Pravin Agarwal Foundation (TPAF) is a philanthropic platform in India that supports the families of children requiring a liver transplant.  This foundation was set up in 2007 by Mr. Pravin Agarwal, Founder and Chairman, Sterlite Power Transmission Limited and Vice-Chairman, Sterlite Technologies.

Collaborated with leading crowd-funding organization in India like the National Liver Foundation, Jeet Liver Foundation, Milaap, who addresses the fund gaps, making the surgery successful. They have also teamed up with the best hospitals in India that makes liver transplant surgery more accessible. TPAF has achieved over 100 successful pediatric liver transplants in India and has a goal to support 100 more families before 2019.

To Summarize

Even though Biliary Atresia is a rare liver disease, parents need to be vigilant of its symptoms. Even normal jaundice in infants needs to be watched carefully, as the persistence of it can mean a Biliary Atresia in them. The points mentioned above about Biliary Atresia shall definitely help parents to safeguard their children’s overall health.

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You can also Join Our TPAF Facebook Group – The Pravin Agarwal Foundation (TPAF) Support group, created to form a network of families/caregivers of children who suffer from Liver Disease. We would request to join the platform to share experiences & concerns, provide emotional & social support and encourage one another in this journey.