Hereditary Spherocytosis – Causes, Symptoms and Its Ayurvedic Treatment

ABSTRACT

Hereditary Spherocytosis is an inherited blood disorder characterised by Hemolytic anaemia.  It is a condition in which there is a genetic error that causes sphere-shaped RBCs like rigid basketballs. Normal RBCs are biconcave which makes it flexible but in Spherocytosis your RBCs are rounded and lack flexibility. Normal RBCs have a larger surface area and central pallor whereas spherocytes have a smaller surface area and no central pallor. RBCs can burst anytime and can cause anaemia or jaundice. Also when these cells pass through the spleen they are mistaken as damaged cells. As a result, the spleen destroys these cells resulting in anemia.So in this article, we will study about it in detail!

INTRODUCTION

Hereditary spherocytosis is an inherited hemolytic disorder clinically characterised by Anaemia, Jaundice and Splenomegaly. Genetic mutation in structural membrane protein phenotype leads to a spherical shape of RBCs. Abnormalities in proteins like Spectrin, Ankyrin and Band-3 protein lead to abnormalities in plasma membrane integrity, decreased surface area and formation of spherocytes. It is an autosomal recessive or autosomal dominant trait. The autosomal dominant inheritance pattern is seen in more cases. Symptoms of Hereditary Spherocytosis may range from mild to moderate. Shortness of breath, Tachycardia, Jaundice and Gallstones are some of the symptoms seen in this condition. The patient is also presented with splenomegaly because the spleen has to work more in order to filter and break down RBCs. It is manageable and should be treated in time in order to avoid complications like splenic rupture or hemolytic crisis, aplastic anaemia, and growth retardation.

CAUSES

Genetic mutations: Genetic mutations in cell membrane proteins (Spectrin, Ankyrin, Band-3 Protein and many other proteins) of RBCs.

SYMPTOMS

1. Anaemia
2. Jaundice
3. Splenomegaly
4. Shortness of breath
5. Tachycardia (Fast heartbeat)
6. Pale skin
7. Tiredness
8. Gallstones
9. Low blood pressure
10. A few symptoms seen in severe Hereditary Spherocytosis include delayed puberty and stunted growth.

DIAGNOSIS

1. Complete blood count (CBC)
2. Peripheral blood smear
3. Coombs test
4. Osmotic fragility
5. Direct antiglobulin test
6. Eosin-5 maleimide binding test

AYURVEDIC OVERVIEW

Hereditary Spherocytosis is a medical condition resulting from Bija Dushti (Abnormal sperm and ovum). It is a genetic disorder categorised under Sahaja Roga (Hereditary disease) or Kulaj roga. This bija dushti affects doshas (Vata, Pitta and Kapha) predominantly Pitta dosha resulting in Paittija Pandu, Kostha-Shakhaashrita Kamla and Plihodar. Hereditary Spherocytosis is a Pitta-predominant disease that affects the Rakta Dhatu (Blood). Pandu (Anaemia), Kamla (Jaundice), Plihodar (Splenomegaly), Shwashkrichta (Shortness of breath), Tachycardia (Fast heartbeat), Kalam (Tiredness) are some of the symptoms seen in Hereditary Spherocytosis.

TREATMENT

Hereditary spherocytosis is managed by taking Sanshodhan (Bio-cleansing therapy), Pitta Shamak (Pitta pacifying) and Raktavardhak (Erythropoiesis stimulating) herbs.

Samshodhan Chikitsa (Bio-cleansing therapy)

1. Virechana (Purgation)
2. Jalokaavcharan (Leech therapy)

Acharya Charak has mentioned the following formulations in order to manage Hemolytic anaemia, Jaundice and Splenomegaly:

1. Punarnava mandur
2. Navayasa churna
3. Mandur bhasma
4. Loha bhasma
5. Sawaran Makshik Bhasam
6. Mandur vatak
7. Shilajatu vatak
8. Gomutra Haritaki
9. Bijka arishta
10. Draksha Ghrita
11. Haridradi Ghrita

Following herbal formulations can also be given in Hereditary Spherocytosis:

1. Chausath Prahari Pippali
2. Shuddha Gairika
3. Arogyavardhini Vati
4. Panchatikta ghrita

HERBAL REMEDIES FOR HEREDITARY SPHEROCYTOSIS BY PLANET AYURVEDA

Planet Ayurveda provides the best combination of effective herbal remedies for the management of Hereditary Spherocytosis. For natural and herbal treatment always look for natural products which contain pure extracts and are formulated under GMP guidelines. Planet Ayurveda products are scientific and certified. They are free from any chemicals, additives, and preservatives. Planet Ayurveda products are formulated by MD Doctors and are 100% natural, pure, and free from side effects. Planet Ayurveda offers the following herbal remedies for the management of Hereditary spherocytosis.

1. Punarnava Mandur
2. Hemo Plan Syrup
3. Gandhak Rasayan
4. Green Essentials
5. Yastimadhu Ghan Vati
6. Drakshasav
7. Ashwagandha Ghan Vati

PRODUCT DESCRIPTION

1. Punarnava Mandur

Punarnava Mandur is a classical Ayurvedic formulation that contains Punarnava (Boerhaavia diffusa), Shunti (Zingiber officinale), Pippali (Piper longum), Maricha (Piper nigrum), Vidanga (Embelia ribes), Mandoor Bhasma (Ferric oxide calx) and many other herbs. Punarnava is Vata and Kapha shamak. It is Panduhara (Subsidies anaemia), Hrudrogajit (useful in cardiac disorders), Mutrala (Diuretic) and is used to manage anaemia in Hereditary Spherocytosis. Punarnava mandur contains Mandur Bhasma popularly known as Iron oxide. It balances Pitta and manages iron deficiency and the underlying disease associated with it. Punarnava mandur is used to manage a wide range of disorders like anemia, splenomegaly, inflammatory conditions and skin diseases.

Dosage: 2 tablets twice daily with lukewarm water.

2. Hemo Plan Syrup

Hemo Plan Syrup is a herbal formulation that contains Manjistha (Rubia cordifolia), Shatavari (Asparagus racemosus), Gorakhmundi (Sphaeranthus indicus), Ashwagandha (Withania somnifera) and various other herbs. Manjistha pacifies Kapha and Pitta and purifies the blood. Shatavari balances Vata and Pitta and is cold in potency. It helps in reducing fatigue associated with anaemia. Ashwagandha balances Kapha and Vata. It is balya (Improves strength) and a rich source of antioxidants. It helps in strengthening heart muscles and helps in the normal growth of the body.

Dosage: 10 ml twice a day.

3. Gandhak Rasayan

Gandhak Rasayan is a classical herbal formulation that contains Shuddh Gandhak (Sulphur). It helps in balancing all three doshas (Vata, Pitta and Kapha). It is Pustikara (provides nourishment), Drudhadeha (improves strength), and Raktadoshahar (treats blood disorders). It is rich in antioxidants and helps in managing an enlarged spleen.

 Dosage: 2 tablets twice daily.

4. Green Essentials

Green Essentials is a wonderful herbal formulation that contains Grape seed (Vitis vinifera), Spirulina (Spirulina platensis), Green tea (Camellia sinensis), Amla (Emblica officinalis) and various other herbs. Grape seeds and Amla present in green essentials are rich in Vitamin C and antioxidants. It helps in the formation of healthy RBCs and removes toxins from the blood. Spirulina is anti-inflammatory and helps in reducing the enlargement of the spleen.

Dosage: 1 capsule twice daily with plain water after meals.

5. Yastimadhu Ghan Vati

Yashtimadhu Ghan Vati is a herbal formulation that contains Yashtimadhu (Glycyrrhiza glabra). Yashtimadhu balances Vata and Pitta dosha. It is cold in potency and used to relieve anaemia. It is a known Rasayan and helps in the healthy functioning of blood cells.

Dosage: 1 tablet twice a day.

6. Drakshasav

Drakshasav is an Ayurvedic formulation that contains Munakka (Vitis vinifera), Ber root bark (Ziziphus mauritiana), Supari (Areca catechu), Javitri (Myristica fragrans), Clove (Syzygium aromaticum), Sonth(Zingiber officinale) and various other herbs. Munakka balances Vata and Pitta and is a great blood purifier. It is Sharamahar that relieves tiredness and lethargy due to anaemia. Draksha asav mainly acts on Vata and Pitta dosha. It has good results in managing anaemia, jaundice, shortness of breath, and fatigue associated with Hereditary Spherocytosis.

Dosage: 10 ml twice a day with an equal quantity of lukewarm water.

7. Ashwagandha Ghan Vati

Ashwagandha Ghan Vati is a herbal formulation that contains Ashwagandha (Withania somnifera). Ashwagandha balances Vata and Kapha. It is hot in potency and helps in reducing tiredness, and low blood pressure and is good for heart muscles.

Dosage: 2 tablets twice daily with lukewarm water.

Contact Planet Ayurveda Support Team to provide you the costing/ordering and delivery information at – costing.planetayurveda@gmail.com or Call at 0172-521-4040 (India), +91-172-521-4040 (Outside India) or Whatsapp at (+91) 842-749-4030.

CONCLUSION

Hereditary Spherocytosis is an inherited hemolytic disorder characterised by Anaemia, Jaundice and Splenomegaly due to a genetic mutation leading to a spherical shape of RBCs. Symptoms of Hereditary spherocytosis may range from mild to moderate. It is associated with complications like splenic rupture or hemolytic crisis, aplastic anaemia, and growth retardation. Ayurveda helps in managing Hereditary Spherocytosis through  Samshodhan and use of Pitta Shamak and Raktavardhak herbs which are good for spleen and hepatoprotective in nature.