ABSTRACT
Blood coagulation is a normal phenomenon of our body which is required whenever we meet any accident or our vessels get ruptured. There are some factors that need to act in the local area to arrest the bleeding. If this coagulation does not take place, the person may collapse immediately. So, in this article, we are going to discuss a bleeding disorder named Bernard Soulier Syndrome is a life-threatening disorder. We will discuss the causative factors of the disease, its signs and symptoms, and treatment with the help of safe herbal medicines.
INTRODUCTION
Bernard Soulier Syndrome is an inherited bleeding disorder, an autosomal recessive disorder in which low levels of platelets or giant platelets are present in our body. Usually, this disease is present since birth, and individuals have the tendency to bleed and make it difficult for coagulation. This genetic disorder runs in families. This disease is also known by the name of Giant platelet Syndrome or Von Willebrand Factor receptor deficiency (occur due to the absence of a glycoprotein).
This is a rare disease but the incidence ratio is about 1 person will affect in millions and both the sexes are equally affected by this disease.
AYURVEDIC ASPECT OF BERNARD SOULIER SYNDROME
Again this disease is categorized under Raktapitta disorders. And these disorders occur due to the influence of Shada Garbhakara Vikaras i.e. six procreative factors that affect the healthy progeny. Hence they affect the tri doshas and Sapta dhatus in our body and develop such issues. Here in this disease major abnormality is seen in Vata dosha, Pitta dosha, Rasa dhatu and Rakta dhatu. So always the things which are beneficial to maintain the equilibrium in these things are favorable to manage the condition.
WHAT CAUSES BERNARD SOULIER SYNDROME?
The cause is the inherited genes from parents in which deficiency of glycoprotein is observed which makes the platelets unable to bind on the damaged vessels thus hindering the coagulation process. Here, the mutations occur in the genes lacking the glycoprotein for clotting. And these deficient factors run in generations.
SIGNS AND SYMPTOMS
There are various signs and symptoms of this disease, they are:
- The main characteristic feature of this disease is unable to coagulate. So, bleeding from the body is a major symptom of the disease.
- Bruising
- Epistaxis (Bleeding from the nose)
- Heavy menstrual bleeding
- Bleeding from the oral cavity especially gums
- Heavy bleeding in minute injuries
- Weakness
DIAGNOSIS
It is difficult to diagnose it from symptoms because in various coagulation disorders the symptoms are the same so we need to go through some lab investigations to confirm the diagnosis.
- Complete blood count
- Blood smears
- FVII –Von Willebrand complex is assessed
- Genetic testing is required.
TREATMENT ASPECT AS PER AYURVEDA
As per Ayurveda, genetic disorders can be managed with the help of safe herbs. In these disorders, we need some herbs which have rejuvenating and immune-modulating properties. So, there are abundant herbs, minerals already present naturally which can help in establishing an appropriate balance in maintaining equilibrium in our doshas and dhatus so they will stimulate the production of platelets and maintain their normal structure and functions. Let's discuss these medicines in detail with their action on our bodies.
- Ashwagandha
- Giloy capsules
- Immune booster
- Green essentials
- Suvarna basant malti ras
- Kumar Kalyan Ras
- Brahmi Chyawanprash
- Gandhak rasayan
1. Ashwagandha
This medicine acts as nectar in genetic disorders. These capsules are prepared from the standardized extract of herb Ashwagandha (Withania somnifera). It helps to maintain the hormonal balance in our body which is required for stimulation of our bone marrow to develop healthy platelets. It also affects at the levels of Dhatus so alters the structure of the cells.
Dosage: 2 caps twice daily, after meals with normal water.
2. Giloy capsules
Giloy capsules prepared from the standardized extract of the herb Guduchi (Tinospora cordifolia). Giloy works effectively in the case to boost the levels of platelets. It is also a type of rasayan that helps in rejuvenation. Giloy also because of its immune-modulating property can help to manage the shelf life of platelets.
Dosage: 1 cap twice daily, after meals with normal water.
3. Immune booster
These capsules help to boost the immune system of our body. It helps to modulate immune functions and can strengthen each and every organs function. These are enriched with antioxidants such as Grapeseed (Vitis vinifera), Go- Piyush (Bovine colostrums), Bhumi amla (Phyllanthus niruri) and Amalaki (Emblica officinalis).
Dosage: 2 caps twice daily, after meals with normal water.
4. Green essentials
This natural supplement is prepared from the standardized extract of Wheatgrass (Triticulum aestivum), Green Tea (Camellia sinensis), Amla (Emblica Officinalis), Grape seed (Vitis vinifera), and Spirulina (Spirulina platensis). These capsules help to nourish the cells due to its antioxidants, protein-rich and micronutrient enriched qualities.
Dosage: 2 caps twice daily, after meals with normal water.
5. Suvarna basant malti ras
This is a classical medicine which has multiple actions on our body. It contains the potent minerals, herbs and calcium compounds like Swarna (Gold), Mukta (Peals), Hingula (Purified cinnabar), Maricha (Piper nigrum), etc. It helps to strengthen the body, has rejuvenating action, and can work well on all kinds of structural as well as functional defects in our body.
Dosage: 1 tablet twice daily with water or milk, after meals.
6. Kumar Kalyan Ras
This is also a classical formulation which is prepared from Swarna bhasma (purified gold), Mukta bhasma (purified peals), etc. This medicine directly acts on our various systems to rejuvenate them and to stimulate their functions. It will also work on structural as well as functional defects in our bodies.
Dosage: 1 tablet twice daily with water or milk, after meals.
7. Brahmi Chyawanprash
A healthy rejuvenator which is well known for its immense actions on our body. This combination of herbs such as Brahmi, Bala, Rishbak, Kesar, Dashmoola, Vidarikanda, etc helps to boost immunity and the functions of the body. These herbs help to bring equilibrium in all the Tridoshas which are imbalanced and develop such genetic defects in our body.
Dosage: 1 teaspoonful twice daily, after meals with milk or normal water.
8. Gandhak rasayan
This is another classical formulation that is effective in this condition. It contains purified Gandhak (Sulphur) in it which helps to work well on DNA's and also effective in bruises and itching that occurs due to reduced platelets.
Dosage: 2 tablets twice daily, after meals with normal water.
The advised dosage is for adults, it varies as per age.
PRECAUTIONS
Those who are suffering from these disorders must protect themselves from injuries or accidents, if possible.
CONCLUSION
This is a rare inherited disorder that needs urgent care for leading a normal life. It is difficult to manage with the help of steroids and it's also not possible to transfuse the platelets throughout life. So, it's a better choice to manage the disease with the help of natural herbs that will boost the production of platelets and maintain their normal shape. And these remedies are totally safe for every age group.