ABSTRACT
Hereditary Spherocytosis is a congenital hemolytic disorder, which affects the red blood cells and causes hemolytic anemia. Blood Disorders affect the blood and blood-forming organs which include HIV, Sickle cell disease and many genetic disorders. Hemolytic anemia is a process where red blood cells are destroyed. Blood disorders can be cancerous or non-cancerous. Non-cancerous blood disorders can be due to an underlying condition which affects the blood and platelets. It is the destruction of the red blood cells before the normal lifespan of 100-120 days. When Erythrocytosis cannot match the destruction of red blood cells. Red blood cells carry oxygen to all parts of the body when the normal amount of RBC is reduced it causes Anaemia. Blood is a living tissue which is made up of liquids (made of plasma, water and salt) and solids (contains red blood cells, white blood cells and platelets). In this article, we are going to discuss causes, symptoms and ayurvedic aspects in detail.
INTRODUCTION
Hereditary Spherocytosis (HS) is a red blood cell condition which happens when there is a breakage of red blood cells faster than normal. In hereditary Spherocytosis, the red blood cells lose the disc-like structure and become round and in the shape of spherocytes which don't function like normal red blood cells. There is a genetic mutation code in the structure of phenotype which changes its shape to spherical morphology. The difference in the shape of normal red blood cells is more prone to rupture under osmotic pressure and during mechanical stress. The cells lead to dysfunction in proteins which further degrade the spleen and lead to a shortage of erythrocytes which further causes hemolytic anaemia. It was first described in 1871 and its most common cause is inherited hemolysis and various genetic mutations of the protein of the membrane. It is the Glucose 6 phosphate dehydrogenase deficiency and the haemoglobinopathies (sickle cell anaemia). Hemoglobinopathies are most common in black Africans and Thalassaemias is more common in the Mediterranean areas. Hemolysis is mainly extravascular.
CAUSES OF HEREDITARY SPHEROCYTOSIS
- It is due to the mutations of 5 genes and mutations in red blood cells resulting in rigid and misshapen cells.
- This dysfunctional membrane interferes with the ability of the cell to change its shape and destruct the spleen and this destruction undergoes hemolysis.
- Blood disease is a spontaneous mutation of the gene.
- Fever and stress destroy the more blood cells.
- The skin and eye white part become yellow as the level of bilirubin rises.
- Bilirubin destroys the red blood cells.
- Mutations in the ANK1 gene cause half of hereditary spherocytosis.
SYMPTOMS OF HEREDITARY SPHEROCYTOSIS
It can range from mild to severe.
1. Anaemia
It breaks down the healthy red blood cells faster and patients look paler which includes symptoms-
- Shortness of breath
- Increased heart rate
- Jaundice
- Fatigue
- Heart palpitations
2. Jaundice
Red blood cells break down and release bilirubin into the bloodstream causing yellowish discolouration of the skin causing jaundice.
3. GallStones
Excess bilirubin causes the formation of gallstones further causing blockages and includes symptoms such as -
- Nausea
- Vomiting
- Jaundice
- Decreased appetite
4. Symptoms in children include
- Jaundice is the most common symptom in newborns
- Difficulty in feeding
- Irritable mood
DIAGNOSIS OF HEREDITARY SPHEROCYTOSIS
- Complete blood count (CBC)
- Peripheral blood smear
- Reticulocyte count
- Direct Antiglobulin (Coombs Test)
- Bilirubin level
- Red cell osmotic fragility
- Plasma membrane electrophoresis
- Ultrasound
TREATMENT OF HEREDITARY SPHEROCYTOSIS
- Phototherapy
- Blood transfusion
- Splenectomy
Cholecystectomy (removal of gallbladder)
- Iron Chelation therapy
AYURVEDIC ASPECT
As it is a hereditary disease so according to ayurveda it is correlated with BEEJ DUSHTI. The smallest unit of Shukra and Shonita is considered Beeja (sperm and ovum). Bheejbhaga is a component which is responsible for the development of organs and various body tissues. It transferred the generation fom one part to another. The formation of the seven dhatus and the 7th dhatu takes place in the building of GARBHA. In hereditary Spherocytosis, it is the beej dushti which causes Kamala (jaundice) and Pandu (Anaemia). Kamala is the pitta region of the body which builds up toxins in the intestinal area, Kamala is the advanced state of Pandu roga. When Pandu is not treated properly it leads to Kamala.
Pathogenesis
Ancient Reference - Charak Chikitsa Sthan 16/34
In this shloka, It is explained that when the person is suffering from Pandu (Anaemia) and consumes Pitta aggravating food in excess, the blood gets aggravated and burns the muscles which further leads to Kamala (jaundice). It is of two types-
- Koshthashakaha shrita Kamala
- Shakhashrita Kamala
Nidana Of Shakhashrita Kamala
- Excessive intake of Ruksha, Sheeta and Madhur Ahar
- Ati Vyayam
- Vega Nirgah
Samprapti Ghataka
- Dosha- Pitta
- Dushya- Rakta and Mamsa
- Adhisthana- Kostha and Shakha
- Srotas- Rasvaha, Raktavaha, Annavaha and Pureeshvaha
- Sroto Dushti- Ati Pravritti, Sanga and Vimargagamana
Chikitsa
The management of this disease is divided into three parts-
- Nidana Parivarjan
- Shodhana (purification therapy) and Shamana Therapy (Palliative therapy)
- Pathya Apathya
1. Shodhana Therapy
- For the management of Kamala, Acharya Charak mentioned Virechana (purgation therapy).
- Purgation therapy eradicates the aggravated Pitta doshas in the body.
- Before Virechana one must have Snehana as a purvakarma.
2. Shamana Therapy
- After Virechana therapy, Shamana Chikitsa is administered.
- Actions of Shaman drugs are
- Pitta Rechana
- Pittahara
- Deepana (appetizer)
- Sroto Shodhan (channel purifier)
- Raktashodhana (blood purifier)
HERBS USEFUL IN HEREDITARY SPHEROCYTOSIS
1. SARIVA
Sariva (Hemidesmus indicus) is helpful in pitta-related disorders used as detoxification of the body and helpful in improving the texture of the skin. It provides cooling properties which neutralise the abnormal acid in the gut. Improves strength and vitality of the body.
2. SHATAVARI
Shatavari (Asparagus racemosus) has antioxidant and antispasmodic properties. It enhances the level of white blood cells and stimulates the macrophages. Relieves stress and has a cooling effect thus lowering fever and inflammation. Balances Vata and Pitta doshas in the body.
3. MANJISTHA
Manjistha (Rubia cordifolia) is helpful in pacifying vitiated Kapha and Pitta doshas. Has blood purifying properties and removes toxins from the body. Has astringent properties which reduce motility and is a good appetiser. Maintains healthy blood circulation and supports healthy skin and tissues.
4. ASHWAGANDHA
Ashwagandha (Withania somnifera) is helpful in balancing tridoshas in the body which improves body mass and nourishment. Has antioxidant properties which boost the immune system and is considered a nervine tonic which pacifies the nervous system. Supports the good health of the heart and maintains a proper supply of the blood in the body.
Herbal Supplements For Hereditary Spherocytosis By Planet Ayurveda
Planet Ayurveda is a GMP Certified ayurvedic clinic which formulates various remedies to prevent multiple health issues and relieve the disease of the diseased person. All these formulations are free from any kind of additives, colour and gums. These remedies are 100% pure and formulated under the supervision of M.D Ayurveda practitioners as they did not cause any side effects. For the purpose of Hereditary Spherocytosis planet ayurveda provided herbal remedies which we will discuss further in this article in detail.
PRODUCTS LIST
- PUNARNAVA MANDUR
- HEMO PLAN SYRUP
- GANDHAK RASAYAN
- GREEN ESSENTIALS
- YASHTIMADHU GHANVATI
- DRAKSHASAV
- ASHWAGANDHAGHAN VATI
PRODUCTS DESCRIPTION
1. Punarnava Mandur
Punarnava Mandur is a herbal tablet formulation which is made by planet ayurveda using various ingredients such as Punarnava (Boerhaavia diffusa), Shunti (Zingiber officinale), Amalaki (Emblica officinalis) Chitrak Mool (Plumbago zeylanica) and many more. It is helpful in balancing Kapha and Pitta doshas which have diuretic properties which reduce swelling activity.
Dosage: 2 tablets twice daily after meals
2. Hemo Plan Syrup
Hemo Plan Syrup are herbal syrup formulations which are made by planet ayurveda using Manjistha (Rubia cordifolia), Gorakhmundi (Sphaeranthus indicus), Draksha (Vitis vinifera) and many more. It is helpful in increasing the level of hemoglobin in the blood and increases the absorption of iron. Known for its balya (strength) promoting herb.
Dosage: 10 ml twice daily
3. Gandhak Rasayan
Gandhak Rasayan is a herbal tablet formulation made by planet ayurveda using purified Sulphur (Shuddh Gandhak). It is helpful in treating chronic fever and improves digestion. Balances tridoshas in the body, useful in all types of skin diseases. Treats all types of acne and pimples and purifies the blood and clears away dirt.
Dosage: 2 tablets twice daily after meals
4. Green Essentials
Green Essentials are polyherbal capsules made by planet ayurveda using various ingredients such as Grape seed (Vitis vinifera), Wheatgrass (Triticulum aestivum), Amla (Emblica officinalis) and more. It is helpful in boosting the immune system and has antioxidant properties. Has anti-inflammatory properties and boosts metabolic rate naturally.
Dosage: 1 capsule twice daily after meals
5. Yasthimadhu Ghan Vati
Yasthimadhu Ghan Vati are herbal tablets formulated by planet ayurveda using ingredients standardized herb Yasthimadhu (Glycyrrhiza glabra). It has Anti-inflammatory and antioxidant properties. Balances Vata and Pitta doshas, helpful in easy healing of the bone and supports the good health of the liver, has antimicrobial activity.
Dosage: 1 tablet twice daily after meal.
6. Drakshasav
It is a classical ayurvedic formulation made by planet ayurveda using various ingredients such as Munakka (Vitis vinifera), Supari (Areca catechu), Sonth (Zingiber officinale), Dalchini (Cinnamomum zeylanicum). It is helpful in cardiac disorders and acts on Vata and Pitta doshas. Useful in curing digestive problems such as in constipation, bloating and in flatulence.
Dosage: 10 ml twice daily after meals.
7. Ashwagandhaghan Vati
Ashwagandhaghan Vati is herbal tablet formulation which is prepared by planet Ayurveda using ingredients such as Ashwagandha (Withania somnifera). It is helpful in relieving stress and anxiety and boosts fertility and testosterone level in the body. It increases the production of insulin and reduces pain and inflammation.
Dosage: 2 tablets twice daily after meals.
Conclusion
Hereditary Spherocytosis is a congenital hemolytic disorder, which affects the red blood cells and causes hemolytic anemia. It is the destruction of the red blood cells before the normal lifespan of 100-120 days. In hereditary Spherocytosis, the red blood cells lose the disc-like structure and become round and in the shape of spherocytes which don't function like normal red blood cells. As it is a hereditary disease so according to ayurveda it is correlated with BEEJ DUSHTI. The smallest unit of Shukra and Shonita is considered Beeja (sperm and ovum).When Pandu is not treated properly it leads to Kamala. Further we studied herbal supplements from planet ayurveda in detail which is helpful in this disease without causing any side effects.
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