Alternative Medicines for Progressive Familial Intrahepatic Cholestasis (PFIC)

Abstract

Progressive familial intrahepatic cholestasis is a condition of genetic disease in which bile is secreted in defective mode. It leads to progressive liver disease and at the end liver failure takes place. Clinical features start in childhood and cause liver disease. It is specially targeted at people with experiences like portal hypertension, hepatosplenomegaly, jaundice or severe itching etc. This disease mostly causes cirrhosis and urgent need of liver transplantation. There are three types of progressive familial intrahepatic cholestasis:- PFIC1, PFIC2 AND PFIC3. In this article we will discuss about progressive familial intrahepatic cholestasis in brief.

Introduction

Progressive familial intrahepatic cholestasis is a rare autosomal recessive inheritance which results in abnormal secretion of digestive juices called bile. It is a life threatening disease.

It is divided into three types

• PFIC1
• PFIC2
• PFIC3
  
  

Each type has a different genetic cause. It is mostly diagnosed in the early phase in childhood with features like

• Dark urine,
• Yellow stool
• Pruritus
• Anorexia
• Tiredness

Causes

• Gene mutation  ATP8B1- PFIC1
• Gene mutation ABCB11-PFIC2
• Gene mutation ABCB4-PFIC3

Pathogenesis

PFIC1:-It occurs by various alterations in ATP8B1, which code for P- type ATPase protein which is responsible for translocation of phospholipid across membranes. It is also called Byler's disease.

Symptoms

• Watery diarrhea
• Deafness 
• Pancreatitis 
• Short stature
  
  

PFIC2:-It occurs by various alterations in ABCB11, which code for bile salt export pump. The people with PFIC2 have greater risk for hepatocellular carcinoma.

• Clinical features are more severe than experienced by people with PFIC1.
  
  

PFIC3:- It occurs by various alterations in ABCB4, which code for translocation of phosphatidylcholine. It leads to absence of phosphatidylcholine in bile and causes biliary epithelium damage.

Symptoms

In this stage clinical features are mostly concerned with liver disease. Most symptoms are not revealed until later in early childhood.

Sign

• Splenomegaly
• Portal hypertension
• Hepatomegaly
• Jaundice 

Symptoms

Symptoms presented in early childhood

• Intense pruritus
• Failure to thrive
• Cholestasis
• Fat malabsorption
• Nausea 
• Vomiting 
• Osteopenia 
• Weakness in body 
• Poor body weight gain
• Bleeding (vitamin k deficiency)

Diagnosis

• Genetic Testing
• Bile analysis
• Electron microscopy
• Liver biopsy
• Liver function test 

Treatment

• Drug therapy is the first line of treatment.
• Liver transplantation
  

Ayurvedic overview

In ayurveda we can correlate this disease with yakrit vikara in which pitta dosha is dominant. Impairment of pitta dosha leads to yakrit vikar. Agni is represented by pitta in the body and produces good and bad effects depending on its normal or abnormal state, i.e. digestion or indigestion, vision or loss of vision, normal or abnormal temperature and other dual functions. Vitiation of pitta dosha leads to various diseases.

Herbal Remedies for PFIC by Planet ayurveda

Planet ayurveda provides ayurvedic products by following all the principles of ancient ayurveda. All the remedies are made from pure and natural herbs which are effective for human diseases. Planet Ayurveda gives a wide range of formulations of capsules, powders, tablets, juices, teas and cosmetic products. All the products are GMP certified. All the medicines have no preservatives, chemicals, binders and are safe to consume. Planet ayurveda provide medicines for progressive familial intrahepatic cholestasis

1. YAKRIT PLIHANTAK CHURNA
2. SANJIVANI VATI
3. KALMEGH GHAN VATI
4. BHOOMI AMLA SWARAS
5. LIV SUPPORT 
   
   
   
   
   

1. YAKRIT PLIHANTAK CHURNA

It is polyherbal churna made from various herbs like Bhumiamla (Phyllanthus niruri), Bhringraj ( Eclipta alba), Kalmegha (Andrographis paniculata) etc. It is pungent and bitter in taste and light, dry and hot in effect. It pacifies vata and kapha but aggravates pitta. It has a salutary effect on the liver. It is also carminative, digestive, diuretic and laxative. It helps in blood formation and is a rejuvenator and tonic of immense value. It protects the liver cells from various harmful radicals and reverses liver damage. It is a good adjunct in the treatment of jaundice and also in the enlargement of liver and spleen. This improves appetite and digestion too.

DOSE:- 1 tsp twice daily

2. SANJIVANI VATI

It is a herbal tablet made of Vidanga (embelia ribes), Ginger root (zingiber officinale), Pippali (piper longum), Haritaki (terminalia chebula) etc. It is pungent and bitter in taste and light, unctuous and hot in effect. While pacifying vata and kapha but aggravate pitta. It is carminative, digestive, sialagogue, blood purifier and anti-colic. It is also a cardiac stimulant. It brings heat into the body, increases perspiration and stimulates the circulatory system. It is used for upset stomach, gas trouble and nausea. It also helps in the digestion of proteins due to the overwhelming presence of an enzyme called protease. It soothes sore throat and relieves congestion and sinusitis.

DOSE:- 2 tablets twice daily after meal with plain water

3. KALMEGH GHAN VATI

It is a herbal tablet made from Kalmegh (Andrographis paniculata). It acts like a rasayan in our body. It effectively balances pitta dosha. It maintains healthy liver enzymes and cells. It is widely used in liver disorders due to its hepato-protective nature. It acts as an antioxidant and anti-inflammatory. It helps in boosting immunity and is used in sinusitis, common cold, allergies etc. it also lowers the blood sugar in diabetic patients. It maintains the blood pressure by dilating the blood vessels. It eliminates the toxins from the body and improves the digestive fire. It protects the liver cells by free radicals.

DOSE:- 2 tablets twice daily after meal with plain water

4. BHOOMI AMLA SWARAS

It is a herbal juice made from bhumi amla (Phyllanthus niruri)). It balances the pitta dosha due to its cold potency.  It contains alkaloids, glycosides, flavonoids, amarin, gernanin, etc. It acts as hepato-protective and helps to manage liver diseases. It contains antioxidants which clear all the channels of the body and expel out the toxins from the body and prevent ulcer by decreasing the amount of gastric acid. It is indicated in various diseases like cough, sore throat, indigestion, vomiting, hyperacidity, peptic ulcer, anemia, etc. It also allays the stubborn and recurring infections of the urinary tract.

DOSE:- 2 tsp twice daily

5. LIV SUPPORT

It is a polyherbal capsule made from  Kalmegh (Andrographis paniculata),  Bhringraj ( Eclipta alba), Kasani (Cichorium intybus) etc. It is the pungent and bitter in taste and light, dry and hot in effect. It pacifies vata and kapha but aggravates pitta. It is digestive, diuretic, laxative and carminative. It helps in blood formation and is a rejuvenator. It is used in skin and ophthalmic diseases, anemia, hyperacidity, migraine and non specific glandular swellings. It is also used in jaundice and hepatomegaly, splenomegaly. It improves digestion and appetite too. It maintains a healthy liver in people of all ages.

DOSE:- 1 capsule twice daily after meal with plain water

Conclusion

PFIC is a genetic disease in which bile is secreted an inappropriate amount. In ayurveda we can correlate this disease with yakrit vikara caused by pitta dosha. Yakrit vikara is managed in ayurveda by various herbs like Kalmegh (Andrographis paniculata),  Bhringraj ( Eclipta alba), Kasani (Cichorium intybus), etc. Along with this you can use herbal medicines for the liver like bhoomi amla swaras, kalmegh ghan vati, yakrit plihantak churna etc.