Category: Anemia

Hereditary Spherocytosis- Causes, Symptoms and its Ayurvedic Management

Hereditary Spherocytosis

Abstract

Genetic diseases are the ones that are caused by abnormalities in the genome. This usually results from the gene mutation that could be single gene mutation (monogenic) or multiple gene mutations (polygenic). Hereditary diseases can be due to abnormality of the chromosome. When these genetic disorders are inherited from one or both the parents then the conditions are considered as hereditary diseases. There are around six thousand known genetic disorders. Hereditary diseases run in the family. One of such hereditary diseases is hereditary spherocytosis. In the text below, we shall be discussing the same and we shall also hw this is treated in the contemporary medicinal system and we shall look forward to how the condition can be managed with the Indian traditional medicinal system of Ayurveda. Let’s get started!

Hereditary Spherocytosis

Introduction

Hereditary spherocytosis is an autosomal dominant disorder in which the principal abnormality relates to a deficiency of a red cell membrane protein named spectrin. Around twenty-five percent of patients have no family history. The erythrocyte envelope is abnormally permeable and sodium pumps are overworked. The erythrocytes lose their normal biconcave shape and become sphere shape and are more prone to osmotic lysis. These spherocytes are exclusively destroyed by the spleen. The severity is variable even within the family. Hemolysis mainly occurs extravascularly. In Ayurveda, the correlation of hereditary spherocytosis can be established with rakta vaha srotas dushti and beej dushti. We’ll see about it more further in the article. Let’s have a look at the causes, clinical features, investigation and management in the medical sciences.

Causes of Hereditary Spherocytosis

As the word hereditary implies, it is a disease that a person inherits from one’s biological parents. In Spherocytosis parents may pass on mutations in five different genes. Around seventy-five percent of people suffering from hereditary spherocytosis inherit the condition in an autosomal dominant way. It implies that it just takes one copy of the concerned gene to cause some sort of hereditary spherocytosis. Children born to a parent who has a mutated gene have a half possibility of inheriting the changed/mutated gene.

Others acquire/inherit the condition as they receive one copy of mutated genes from each parent. This is an autosomal recessive way. For this situation, parents are carriers yet typically don’t have any signs and symptoms of hereditary spherocytosis.

When carriers of hereditary spherocytosis have children. Then every child has an alternate possibility of developing hereditary spherocytosis. In particular, they have 25 percent possibility of abstaining from being a carrier and having hereditary spherocytosis, fifty percent chance of being a carrier but not developing the condition and a twenty-five percent chance of developing the hereditary spherocytosis.

Signs and Symptoms of Hereditary Spherocytosis

  • Lassitude
  • Fatigue
  • Breathlessness on exertion
  • Palpitations
  • Throbbing in the head or ears
  • Dizziness
  • Tinnitus
  • Dimness of vision
  • Headache
  • Paresthesia in toes and fingers
  • Angina
  • Insomnia
  • Pallor of mucous membrane, palms of hands, conjunctivae and skin
  • Oedema
  • Cardiac dilatation
  • Tachycardia
  • Systolic flow murmurs
  • Episodic jaundice may be seen
  • Spleen is often but not always enlarged
  • Liability of developing pigmented gallstones
  • Cholecystitis
  • Ulcers on the leg may appear sometimes

Investigations

  • Coombs test – negative
  • Acidified Glycerol Lysis Test – positive
  • Osmotic Fragility Test – positive
  • Complete blood count
  • Liver Function Tests
  • Peripheral blood smear – shows spherocytes on microscope
  • Eosin-5 maleimide Binding Test

Ultrasound is used to check the dimensions or size of spleen, gallbladder and liver.

Management

There is no cure for reversing the genomic derangements that lead to hereditary spherocytosis.

Folic acid supplements are given as symptomatic management.

Typical management available includes following

Splenectomy

Removing the spleen surgically stops the extravascular hemolysis but it makes the person immune compromised as the spleen has various immune functions.

Partial splenectomy

Instead of the entire organ, a part or a segment of the organ is surgically removed. It lowers the extravascular hemolysis that compensates for the level of homeostasis of the patient.

Splenic ablation

It is an interventional radiology and non-operative method that uses radiological techniques to cauterise or coil vasculature within the spleen.

Experimental management

Bone marrow transplant – A bone marrow transplant replacing the bone marrow with the bone marrow of a person who is free from spherocytosis.

Ayurvedic Aspect and Management of Hereditary Spherocytosis

Ayurveda is an ancient Indian medicinal system that has been serving people for ages. And the concept of Ayurveda still continues. Ayurveda is such a positive science that deals with acute to chronic conditions. Its objectives make it unique as it focuses on the alleviation of the ailments of the diseased person and keeping up the health status of the healthy person. Ayurveda is based on the main three doshas though there are various things that make Ayurveda as vast as the sea. Ayurveda focuses on lifestyle and food management in literal terms. According to Ayurveda, the food a person eats and deeds a person does decide the health status of that person.

Hereditary Spherocytosis is an inherited disorder that one inherits from one or both parents. This occurs due to abnormal changes in the genome which usually result from the mutation of gene/s. This can be correlated in Ayurveda with beej dushti which results in the abnormal functioning of the body part anatomically and physiologically and gives rise to a new pathology. Being a raktaj dosha, the pittaja component is thus involved in the pathology of the condition.
Genomics can be correlated with prakriti of a person that cannot be changed but the symptoms that are caused by the altered genes can be well managed. If prakriti of a person is pittaja then he is more prone to develop pittaja disorders. Abnormal genes likewise result in the symptoms of a disease according to the mutated gene. The management thus depends on alleviation of the symptoms that is caused by the genomics.

The vitiated component of the body in hereditary spherocytosis is erythrocytes, the red blood cells. So the main focus of the management is on preventing anaemia with the production of red blood cells and alleviating other associated symptoms of the conditions.

Some medicines that are effective in hereditary spherocytosis include Gandhak Rasayan, Mahamanjisthadi kwath, Khadirasrisht, Khadiradi vati, Lohasav, Punarnava Mandur, Dhatri loha, Ashwagandhaghan vati and many others.

Herbal Remedies of Planet Ayurveda for Hereditary Spherocytosis

Planet Ayurveda is a ISO 9001:2015, USA-FDA, GMP certified ayurvedic company. Planet Ayurveda looks forward to spreading the wise words and knowledge of Ayurveda around the globe. Planet Ayurveda produces the product on the lines of various ayurvedic texts and ayurvedic pharmacopoeia. The products are available in different forms and can be consumed according to ‘the patient’s comfort. Furthermore, the products are chemical and preservatives free. Planet Ayurveda does not use any sweet enhancers and degraded herbs for the formulations. All the products are effective and pure. The products are halal certified and gluten free. Planet Ayurveda extends its products that are effective in the management of hereditary spherocytosis.

Given below are some of the products that help in managing Hereditary Spherocytosis

  1. PUNARNAVA MANDUR
  2. HEMO PLAN SYRUP
  3. GANDHAK RASAYAN
  4. GREEN ESSENTIALS
  5. YASTHIMADHU GHANVATI
  6. DRAKSHASAV
  7. ASHWAGANDHAGHAN VATI

Herbal Remedies for Hereditary Spherocytosis

Buy Herbal Remedies For Hereditary Spherocytosis

Product Description

1. PUNARNAVA MANDUR

Punarnava Mandur is classical ayurvedic medicine used to manage a number of haematological diseases. Punarnava Mandur contains punarnava (Boerhavia diffusa), shunthi (Zingiber officinale), pippali (Piper longum), maricha (Piper nigrum), mandoor bhasma (Ferric oxide calx), haridra (Curcuma longa) etc. Punarnava Mandur helps in production of healthy blood cells and prevents the unwanted changes in the already persisting blood cells. Punarnava is therefore a medicine of choice in anemic conditions. Punarnava Mandur cleanses the blood thus removing the impurities from the impure blood. Furthermore, Punarnava Mandur helps in preventing the bleeding. In this way, Punarnava Mandur is a perfect remedy to attain the optimum blood cell levels that are damaged by Hereditary Spherocytosis.

Dosage: Two tablets twice a day after meals.

2. HEMO PLAN SYRUP

Hemo Plan Syrup is a product of Planet Ayurveda which has been prepared for increasing the number of blood cells that are not up to an optimum level due to any of the reasons. Hemo Plan syrup is composed of the herbs such as manjistha (Rubia cordifolia), shatavari (Asparagus racemosus), gorakhmundi (Sphaeranthus indicus), draksha (Vitis vinifera) and various others. All these herbs work on the blood cells and thus manage all the diseases related to blood cells. The herb purifies the blood and promotes the healthy production of blood cells. It removes toxins from the blood. In anaemic conditions, Hemo Plan Syrup promotes the production of red blood cells. In case of Hereditary Spherocytosis, the syrup optimises the blood cell levels with the healthy and normal shape. Hemo Plan Syrup, thus, an important remedy by Planet Ayurveda for the management of Hereditary Spherocytosis.

Dosage: 10 ml twice a day after meals.

3. GANDHAK RASAYAN

Gandhak Rasayan is a classical ayurvedic proprietary medicine used to manage various blood related and skin related disorders. Gandhak Rasayan is composed of pure sulphur. Gandhak Rasayan is known to be of great benefit in the blood disorders as it helps in the production of healthy blood cells. Gandhak is known for its rejuvenating properties. Gandhak heals damaged blood cells. Furthermore, Gandhak Rasayan alleviates the other symptoms associated with hereditary spherocytosis. Hence with the healing properties of Gandhak Rasayan, the above mentioned condition is well managed.

Dosage: Two tablets twice a day after meals.

4. GREEN ESSENTIALS

Green Essentials is a product of Planet Ayurveda which is composed of wheatgrass (Triticum aestivum). Wheatgrass is known for its various beneficial properties for health. It promotes overall wellness of the body and it works wonderfully in the blood disorders. It promotes the proper and healthy development of the new blood cells. Furthermore, it purifies the blood and removes toxins out of it. It contains a certain vitamin K, C, E etc. Its constituents help in bleeding disorders as these are helpful in clotting the blood. With these properties Green Essentials are of great benefit in the person suffering from hereditary spherocytosis.

Dosage: One capsule twice a day after meals.

5. YASTHIMADHU GHAN VATI

Yasthimadhu GhanVati is a classical ayurvedic medicine composed of the herb yashtimadhu (Glycyrrhiza glabra). Yashtimadhu is considered as medhya rasayan as mentioned in the Charak Samhita. Rasayan is a herb or formulation which benefits the overall body, improves immunity, boosts stamina, benefits skin and promotes longevity. Yashthimadhu in Hereditary Spherocytosis helps in coping up with the symptoms of the same. Yasthimadhu stops the destruction of red blood cells and prevents bleeding and jaundice. Yashimadhu alleviates the other manifestations on the other body parts expressed by Hereditary Spherocytosis.

Dosage: One tablet twice a day after meals.

6. DRAKSHASAV

Drakshasav is a classical ayurvedic proprietary medicine that is used to boost energy life. Drakshasav contains a lot of herbs such as draksha (Vitis vinifera), ashwagandha (Withania somnifera), ajmoda (Carum roxburghianum), shunthi (Zingiber officinale), patha (Cissampelos pareira) and others. All these herbs provide strength to the body and fulfil the energy requirements that have been lost due to the severe breakdown of red blood cells and have resulted in anaemia. Therefore, Drakshasav helps in increasing the blood cells and supports the functioning of healthy hematopoiesis. Thus, it helps in Hereditary Spherocytosis extensively.

Dosage: 10 ml twice a day after meals.

7. ASHWAGANDHAGHAN VATI

Ashwagandhaghan vati is a classical ayurvedic proprietary medicine that is used in severe weakness and various other health disorders. Ashwagandhaghan vati is filled with the wonderful properties of ashwagandha (Withania somnifera). Ashwagandha acts as rasayan and rejuvenates the body. Ashwagandha purifies the blood and removes toxins out of the bloodstream. Furthermore, Ashwagandhaghan vati helps in the healthy and normal shaped production of red blood cells. With rasayanic properties of Ashwagandha, Ashwagandhaghan vati does an impressive job in the management of Hereditary Spherocytosis.

Dosage: Two tablets twice a day after meals.

Contact Planet Ayurveda Support Team to provide you the costing / ordering and delivery information at – costing.planetayurveda@gmail.com or Call at 0172-521-4030 (India), +91-172-521-4030 (Outside India) or Whatsapp at (+91) 9915-593-604.

Conclusion

Hereditary diseases are what a person inherits from the parent. There are many inherited conditions that hamper the normal life of a healthy being. Hereditary Spherocytosis is such a condition in which the production of the sphere shaped red blood cells occurs instead of healthy disc shaped. This sphere shape of red blood cells results in easy breakdown of the cells which results in severe anaemia and jaundice as well. This results in a number of symptoms that we have discussed above. The management of the condition in the modern medicine system is purely surgical and the bone marrow transplant is highly dubious and expensive. Whereas Ayurvedic treatment is purely non-invasive and based on the herbs. As we discussed above the condition can be well managed with Ayurvedic herbs as they fight the symptoms of hereditary spherocytosis such as anaemia and jaundice and also promotes the production of healthy red blood cells. The products above of Planet Ayurveda are pure and much effective in the management of Hereditary Spherocytosis.

Herbal Remedies for Paroxysmal Nocturnal Hemoglobinuria

Abstract

The destruction of red blood cells is known as hemolysis and this can be caused by different reasons. Hemolysis is responsible for releasing hemoglobin into the bloodstream and normal blood cells have 120 days of lifespan. When they break down they are released into the circulation by the spleen. There is a rare medical condition that results in increased destruction of the blood cells known as Paroxysmal Nocturnal Hemoglobinuria. Through this article, we are going to discuss this rare blood condition in detail and its help through the Ayurvedic condition.

Introduction

Paroxysmal Nocturnal Hemoglobinuria is a rare acquired condition of the blood and can result in death. It is mainly characterized by clotting of blood (thrombosis), increased destruction of blood cells (hemolytic anemia), and impairment of bone marrow function. It was first discovered by a German physician known as Paul Strubing in 1852 to 1915.

  • Epidemiology: – It is sporadic, and one in every one million people is affected with PNH.
  • 25% of females are affected with PNH and most of them are discovered during the time of their pregnancy

Sign and Symptoms

symptoms of Paroxysmal Nocturnal Hemoglobinuria

There are wide ranges of treatment seen in Paroxysmal Nocturnal Hemoglobinuria and sometimes is not noticed for months or years

This includes signs like

  • Weakness and fatigue
  • Unusual bleeding or bruising all over the body.
  • Difficulty in breathing
  • Flu-like symptoms or reoccurrence of infections.
  • Unable to control bleeding even from small cuts or wounds
  • Headache that is very severe
  • Fever because of infection
  • Blood clots

Other issues like back pain and abdominal pain are also noted. Some of the classic signs are that the urine appears bright red in 50 percent of patients. The hemoglobinuria is especially very noticeable in the morning. The attack of hemoglobinuria can be caused by consumption of alcohol, infections, stress, exercise, and some certain type of medications.

Blood clots can be formed in veins and it is one of the leading causes of death in a patient with Paroxysmal Nocturnal Hemoglobinuria. The common sites for the blood clots are the sagittal vein and hepatic vein.

Causes

The main cause for PNH occurs when there is a mutation of a gene known as PIG-A present in the bone marrow stem cells. Stem cells are responsible for giving rise to the mature blood elements which include red blood cells, these cells are helpful in carrying oxygen to the tissues and also have white blood that helps in fighting the infection.

In patients with Paroxysmal Nocturnal Hemoglobinuria, the stem cell moves the PIG-A mutated gene to all the cells making the stem cells abnormal.

Pathophysiology

All the cells in the body have protein which is attached to the membranes and helps in communication between the two cells. These proteins are physically connected to the membrane of cells in many ways, commonly with glycolipids known as Glycosyl phosphatidylinositols. In patients with PNH the glycolipid protein is defective.

Herbal Medicine Provided By Planet Ayurveda for Paroxysmal Nocturnal Hemoglobinuria

Planet Ayurveda provides herbal medicine that is 100 percent pure, natural, and vegetarian. All the herbal medicine made in Planet Ayurveda is clinically tested and made under the supervision of an MD Ayurvedic Doctor. Each herbal medicine is clinically tested making them safe to use and free of side-effects

Paroxysmal nocturnal hemoglobinuria

Treatment of Paroxysmal Nocturnal HemoglobinuriaHere is the list of Herbal Medicine

  • Hemo Plan Syrup
  • Plato Plan Syrup
  • Giloy Capsules
  • Spirulina Capsules
  • Immune booster
  • Manjishtha Capsules

Product Description

1. Hemo Plan Syrup

It is a herbal syrup formulated with herbs like

  • Shatavari (Asparagus racemosus)
  • Saariva (Hemidesmus indicus)
  • Draksha (Vitis vinifera)
  • Saariva (Hemidesmus indicus)
  • Ashwagandha (Withania somnifera), and many more.

The syrup is a gift from nature that helps in removing toxins from the blood and also a natural source of iron. Hemo plan also helps in removing ailments like weakness, pain, bruising, etc which is very commonly seen in patients with Paroxysmal Nocturnal Hemoglobinuria

Dosage:- One teaspoonful twice daily with water after meals.

2. Plato Plan Syrup

This herbal syrup helps in improving the count of platelets as it formed from herbs like Giloy (Tinospora cordifolia), Eranda Karkati (Caricapapaya), Draksha (Vitis vinifera), etc. Plato plan helps in giving strength to the body and is packed with antioxidants that helps in preventing the platelets from getting damaged. It is also very useful in flushing out the toxins from the blood.

Dosage:- One teaspoonful twice daily with water after meals.

3. Giloy Capsules

It is one of the best herbal medicines packed with anti-inflammatory and antipyretic properties as it is made from an excellent herb like Guduchi (Tinospora cordifolia). It is one of the wonderful herbs in removing any type of fever and lowering down the inflammation from the body. Giloy is also very good for removing ailments related to skin, infections and best for people who have weak immunity.

Dosage:- One capsule twice daily with water after meals

4. Spirulina Capsules

These capsules are made from algae known as Spirulina (Spirulina platensis)  which is rich in nutrients like vitamins, proteins, minerals, chlorophyll, and omega-3 fatty acids. This herbal medicine helps in maintaining the good health of the Cardiovascular system

Dosage:- One capsule twice daily after meals with lukewarm water.

5. Immune booster

As the name describes it helps in making the immunity stronger and powerful as it is formed from herbs like

  • Grapeseed (Vitis vinifera)
  • Amalaki (Emblica Officinalis)
  • Cow colostrum (Bovine colostrums)
  • Bhumi Amla (Phyllanthus niruri)

It is very useful for people who have recurrent infections and have an immuno-compromised state. The immune booster helps in fighting infections, aging, asthma, liver inflammation, and many more

Dosage:- One capsule after meals twice daily with water.

6. Manjishtha Capsules

This is made from the extract of the single herb known as Manjishtha (Rubia cordifolia) which is well known for relaxing the body and reducing stress as well as anxiety. These capsules are rich in antioxidants and are useful in purifying the blood and also treating all kinds of fever present in the body

Dosage:- One capsule twice daily after meals with water.

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

Conclusion

Paroxysmal Nocturnal Hemoglobinuria is a very rare disease and can result in a life-threatening situation. So, it is important for the patient to be treated carefully by a good Doctor. Allopaths have no treatment for the disease but Ayurveda provides their herbal medicine which is very useful in treating the disease. All the medicine mentioned above is best for treating Paroxysmal Nocturnal Hemoglobinuria as they remove the root cause of the disease.