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ABSTRACT

In this article our main topic of discussion is a very rare disorder known as “Bernard-Soulier syndrome”. It is a disorder associated with blood functioning. In today’s era such types of disorders have become very prevalent. In the modern system of medicine complete cure of such illness is not possible as they use blood transfusion procedure to treat this type of rare ailment and it will just provide symptomatic relief to the patient. Ayurveda offers numerous magical herbs that work quite efficiently in a healthy way by increasing the strength and stamina of the body. It teaches us about an ideal way of living so that we can prevent ourselves from such kinds of bleeding disorders. So let’s start discussing it in detail.

INTRODUCTION

Bernard soulier syndrome is a very rare inherited disorder associated with blood coagulation in which platelets acquire abnormally large shape, due to that there will be decrease in platelets count that leads to increase in bleeding time (difficulty in clotting), as platelets play a major role in the initiation of blood clotting mechanism.

DEFINITION OF BERNARD SOULIER SYNDROME

It is a very rare bleeding disorder of autosomal recessive origin that occurs due to deficiency of glycoprotein responsible to control bleeding from the injured blood vessels. The prevalence rate of this ailment is very less as there is a presence of 1 case over million persons.

AYURVEDIC CORRELATION OF BERNARD SOULIER SYNDROME

As per ayurveda we can correlate this ailment with “rakta pitta”. As rakta (blood) got vitiated due to elevated pitta dosha, as pitta has its main seat in rakta dhatu, so if pitta got aggravated due to any etiological cause it will ultimately affect the normal functioning of blood (rakta dhatu).

RAKTA PITTA IS OF THREE TYPES

1. Urdhva rakta pitta– bleeding from upper openings of the bodies, such as in nose bleed. In this there is an involvement of kapha dosha.
2.  Adhog raktapitta– oozing of blood from the lower body tract, as in hematuria. In this there is an involvement of vata dosha.
3. Triyak raktapitta– in this type there is a haemorrhage from different parts of the body such as in the limbs. In this there is an elevation of vata-kapha doshas.

ETIOLOGICAL FACTORS

These are the main causes (nidan as per ayurveda) that results in formation of an ailment.

• Hereditary- As it is an autosomal recessive disorder so genetics plays a major role in giving rise to this ailment.
• Deficiency of a glycoprotein called Ib-IX-V complex (GPIb). It plays a major role in binding two molecules of fibrinogen that helps in blood coagulation.

AS PER AYURVEDA THE MAIN CAUSE OF RAKTA PITTA ARE AS FOLLOW

• Consumption of katu (spicy), amala (sour), lavana (salty) rasa in abundant quantity.
• Excessive intake of Ushana(hot) and teekshna (penetrating) food substances
• Krodha (Excess anger)
• Atyasahana (Overeating)
• Viruddha ahara sewana (Intake of unhygienic diet)
• Vega dharna (Controlling of natural urges of urine and stool)
• Over use of alkalis (kshara)

CLINICAL FEATURES

It includes the signs and symptoms. It means the general complaints along patients present in front of the physician.

• Abnormal bleeding from very small injury.
• Easy bruising on different parts of the body.
• Excessive bleeding from gums.
• Heavy menstrual bleeding.
• Fatigue
• Body ache
• Generalized weakness
• Gum bleeding
• Gastrointestinal bleeding
• Burning sensation in the body
• Anorexia- loss of hunger
• Polydipsia- Increased thirst.

DIAGNOSIS

It means how we can identify the disease. There are various methods

• General physical examination of patients by using inspection, palpation, auscultation and percussion technique.
• Proper history taking.
• Laboratory findings, It includes:- Complete blood count test: Thrombocytopenia, Enlarge size of platelets

ACCORDING TO AYURVEDA

The two most preferred diagnostic procedures used by a physician are as follow

Asthavidh Pariksha (Set Of 8 Examination Procedure)

• Nadi pariksha– By palpating the pulse rate of the patient.
• Mutra pariksha– By examining the urine of the patient.
• Mal pariksha– By stool examination.
• Jivha pariksha– By examining the tongue whether coated or not.
• Shabad pariksha– By listening to the natural sounds within the body like intestinal sound (peristalsis) sounds of moving body fluids.
• Sparsh pariksha– By observing the patient skin texture whether it is cool, wet or dry.
• Drik pariksha– When a physician inspects patient symptoms through his/her eyes.
• Akriti pariksha– When a physician evaluates the general appearance of a patient whether he/she looks dull, lethargic or fatigued etc

Trividh Pariksha (Group Of 3 Diagnostic Procedures)

• Darsha- Diagnosis is made by visual examination of the symptoms.
• Sparshan- Physician uses tactile examination to evaluate whether skin is cold/hot
• Prashan– Physicians ask direct questions from the patient, to evaluate the root cause of the disease i.e. history taking.

MANAGEMENT

Supportive Therapy

• Blood transfusion is given to combat excessive bleeding.
• Iron and folate medications, as such patients suffer from complaints of anemia as well.
• Antifibrinolytic agents- they help in blood clotting by preventing breakdown of fibrin (a protein) that plays a major role in formation of blood clot.

So as these above mentioned treatment modality just provide symptomatic relief to the patient, ayurveda works very generously by going into the basic level of etiological factors.

AS PER AYURVEDA

This disease occurs due to elevation of pitta dosha so our main focus will be on diminishing the pitta dosha level. For this we need to use below mentioned treatment modalities

A. Sanshodhan Chikitsa (Detoxification And Purification Therapy)

In sanshodhan chikitsa we will purify the inner channels of our body (srotas) by using different procedures. Acharya charaka included five procedures in it, they are as follows- vaman (emesis), virechana (purgation), anuvasana basti (enema with medicated oil), niruha basti (enema with medicated decoction)  and nasya karma (adding drops of medicated oil/ churna in the nostrils).

Before using sanshodhan chikitsa we need to use purva karma (foremost procedures) – they are the set of procedures that need to be done before main panchakarma procedures. They are very important as they help in preparing the body for the main purification procedures. It includes: deepan karma, pachana karma, snehana karma and swedana karma.

• Deepan karma: In this we use deepaniya dravya to increase the digestive fire of the body.
• Pachana karma: in this we use pachana dravya to digest the food substances.
• Snehana karma (oleation therapy) – Smoothing the body dosha’s by using herbal ghrit/oil.
• Svedan karma (sudation therapy) – Semi-hot medicated kwath or other herbal solution is sprayed on the patient’s body by a specially made swedan yantra (instrument).
• Vaman (emesis) – In this procedure we do elimination of toxins by using emetic herbs to take out vitiated kapha dosha from the body.
• Virechana (purgation) – In this we use purgative herbs to take out vitiated pitta dosha from the anal route by purgation technique.
• Basti karma (enema) – It is of two types:
• Anuvasana basti (enema with oil) – In this medicated oil is inserted into the body by anal route with the help of rectal route by using basti yantra (enema intrument)
• Niruh basti (enema with medicated decoction) – In this medicated solutions are inserted into the rectal route by using basti yantra (enema instrument)

Both these types of basti (enema) are used to balance the vitiated vata dosha within the body.

B. Sanshaman Chikitsa (Alleviating Treatment)

In this treatment modality we use the herbal medicines by oral route to balance vitiated doshas within the body itself

HERBAL REMEDIES OF PLANET AYURVEDA

Planet ayurveda is a leading herbal manufacturing unit serving people worldwide with their holistic approach of healing by using principle of ayurveda. They offers various herbal formulations that will work very efficaciously in Bernard soulier syndrome, it includes

1. Giloy Capsules

This capsule contains pure and standardized extract of giloy (Tinospora cordifolia) and it is known as “amrita” in ayurveda (because of its numerous health benefits). It helps in balancing tridosha (vata, pitta and kapha) as it possesses great anti-inflammatory and antipyretics properties that will help in reducing bruising as it is seen commonly in patients of Bernard-Soulier syndrome.

Dosage: 1-2 capsules two times in a day with plain water.

2. Spirulina Capsules

This capsule contains a pure form of spirulina (Spirulina platensis). It is a rich source of various healthy micro and macro nutrients. It also contains a rich amount of antioxidants so helps in excreting harmful free radicals from the body. It also has anti cancerous properties so helps in maintaining overall strength and stamina of patients of Benard- Soulier syndrome.

Dosage: 1-2 capsules two times in a day with plain water.

3. Pitta Balance

This potent formulation is an efficacious blend of praval pishti, akik pishti, jawar mohra pishti, giloy satva etc. Minerals and herbs present in it have sheet virya (cold potency) thus works very well in reducing elevated pitta dosha within the body and elevated pitta dosha is considered as the main culprit of Bernard-Soulier syndrome.

Dosage: 1-2 capsules two times a day with plain water after meals.

4. Praval Panchamrit

This herbomineral formulation contains mukta bhasma (pearl bhasma), shankh bhasma (conch shell bhasma), shukti bhasma (pearl oyster bhasma), pravala bhasma (coral bhasma) and arka (Calotropis procera). This potent combo of ark and other minerals is very effective in an inflammatory condition as it is great ‘kaphavatashamak’ in action. It also gives strength to cardiac muscles and improves the cardiac function and rhythm.

Dosage: 1-2 pills twice or thrice daily with plain water or honey.

5. Kamdudha Ras

This herbomineral formulation is a very efficacious blend of amrita satva (extract of giloy), abhrak bhasma (silica bhasma), praval pishti, shankh bhasma etc. This combination is made to balance the vitiated pitta dosha, as it is the main culprit behind Bernard soulier syndrome so this formulation works really well in this ailment.

Dosage: 1-2 tablets twice daily with plain water after meal.

6. Plato Plan Capsules

This herbal formulation is a very generous combination of herbs such as errand karkati (Carica papaya), giloy (Tinospora cordifolia), ashwagandha (Withania somnifera), amalaki (Emblica officinalis), jevanti (Leptadenia reticulata) etc. All these herbs are natural blood purifiers and assist in increasing the haemoglobin content as the patient of Bernard soulier syndrome generally suffers from anemia as well.

Dosage: 1-2 capsules twice daily with plain water after meal.

Contact my assistant to provide you the costing / ordering and delivery information at – costing.planetayurveda@gmail.com or call at +91-172-5214030 Or Check Website – www.PlanetAyurveda.com

CONCLUSION

Now we can conclude that with ayurveda we can surely attain good results in rare genetic disorders such as in Bernard-Soulier syndrome (BSS). If you are opting to go for ayurvedic treatment, consider planet ayurveda as your prime choice. They are serving mankind with their ayurveda experts. You can directly visit them at their clinic in Mohali, Punjab or can also send your queries on their official email id: herbalremedies123@yahoo.com