Ayurvedic Treatment for Prader Willi Syndrome with Herbal Remedies

Abstract

Prader Willi syndrome is a genetic disorder. It occurs due to chromosome abnormality or error in one or more genes. In the present, it is the most common genetic cause of obesity. Patients of this syndrome are inclined to become obese at an early age because of functional impairment of CNS resulting in an extremely raised appetite. In 1956, the first case of this disorder was documented by Dr. Andrea Prader, Dr. Alexis Labhart and Dr. Heinrich Willi. Hence, it is also known as Prader–Labhart–Willi syndrome. Therapies and regulation of diet can help manage this condition and prevent complications.

Etiopathogenesis

• It is a genetic disorder whose exact mechanism is not known. It is random and can affect people of all backgrounds.
• It occurs due to loss of function or non-expression of genes on chromosome 15. This chromosome affects the hypothalamus which is the part of your brain responsible for appetite and growth of the body.
• How genes are affected in this case can be understood through the concept of genomic imprinting; one copy of chromosome 15 is inherited from each parent. Both copies are necessary for genes to get the instructions to execute body functions.
• The paternal copy of chromosome 15 is the only one that is in active form. The maternal copy is always inactive. Here are 3 types of genetic changes in chromosome 15 that are seen in Prader Willi syndrome:
• In 70% of the cases paternal chromosome 15 is missing or not working properly and the maternal copy is naturally inactive.
• In around 25% of Prader Willi syndrome patients, the child doesn’t receive the paternal gene but inherits two maternal copies of chromosome 15 instead, making both the copies inactive.
• In rare cases (less than 1%), a piece of chromosome 15 relocates itself to another chromosome. This renders the genes ineffective.

Risk Factors: Prader Willi syndrome can affect any race, ethnicity or gender, however, females are slightly more likely to be affected than males.

Clinical Features

Prader Willi syndrome presents with various features depending upon the age group of the patient:

Infancy

• Symptoms – Poor sucking reflex, weak cry, absence of crying, lethargy
• Signs – Weak muscle tone, almond-shaped eyes, narrow head, triangle-shaped mouth, delayed growth

Childhood

• Symptoms – Increased appetite, pain tolerance is high, disturbed sleep,
• Signs – Intellectually impaired, short height, habits like skin picking and squinting, small hands and feet, irritability, specific facial features of PWS become more defined

Adulthood

• Symptoms – Fatigue, obesity (due to raised appetite), feeling of not being full even after eating, stubbornness, delayed speech
• Signs – Scoliosis or kyphosis, puberty is delayed, testes are undescended, irregular or absence of menstruation, bow legs (curved)

Common Features

• Skin, eyes or hair are light in colour
• Vision impairment like near-sightedness
• Swelling of legs with varicose veins
• Presence of stretch marks due to obesity
• Underdeveloped enamel with small teeth
• Presence of thick saliva on the corners of the mouth
• Mouth palate is more arched than normal
• Underdeveloped labia in females and less folding of scrotal skin in males

Complications

• High grade obesity
• Anxiety
• Deep vein thrombosis (leg clots)
• High BP
• Stroke
• Cardiac arrest
• Pulmonary Embolism (clots to the lungs)
• Chest infections like pneumonia
• Diabetes mellitus type 2
• Osteopenia or osteoporosis
• Infertility

Diagnosis

• Physical examination to assess clinical features like almond eyes, triangular mouth etc.
• Genetic tests for confirmation is necessary
• X-ray to identify skeletal abnormality

Differential Diagnosis

• Angelman syndrome
• Congenital myasthenic syndrome
• Spinal muscular atrophy

Management Of Prader Willi Syndrome

In Prader Willi syndrome, Gene mutation cannot be reversed but symptoms can be managed and complications can be prevented to increase quality of life.

• Nutrition – If the child has poor sucking reflex, special bottle nipples can be provided to help infants get proper feed.
• Growth hormone treatment – It will help in growth and development of the child, improve muscle tone and decrease body fat.
• Weight management – Diet management is extremely necessary t o prevent obesity complications. A dietician can provide a calorie deficit nutritional diet.
• Sex hormone therapy – Hormone replacement therapy is opted to regulate low levels of sex hormones like testosterone in males and progesterone and oestrogen in females.
• Behaviour management – Disciplined life can help regulate behaviour such as irritability and stubbornness.
• Psychiatric help – Seek the help of a professional to address obsessive habits like skin picking.
• Medicines – Psychotropic medicines can be used in adults.
• Surgery – Complications like scoliosis and inguinal hernia demand surgical intervention.

Ayurveda On Prader Willi Syndrome

Being a genetic disorder, Prader Willi syndrome is attributed towards previous life actions in Ayurveda (Karma). Genetic disorders are called Sahaj Roga in Ayurveda. Prader Willi syndrome consists of chromosomal abnormality which is called Beeja Bhaga Avayava dosha in Ayurveda terminology. This disorder entails the aggravation of all the three doshas; Vata, Pitta, Kapha. Charak Samhita, Sharira Sthana, Chapter 3rd – Khuddika Garbhavakranti Sharira mentions the effect of chromosomal abnormality in the offspring:

यच्चोक्तं- यदि च मनुष्यो मनुष्यप्रभवः, कस्मान्न जडादिभ्यो जाताः
पितृसदृशरूपा भवन्तीति;
तत्रोच्यते-यस्य यस्यह्यङ्गावयवस्य बीजे बीज भाग उपतप्तो भवति,
तस्य तस्याङ्गावयवस्य विकृतिरुपजायते, नोपजायते
चानुपतापात्;तस्मादुभयोपपत्तिरप्यत्र|
सर्वस्य चात्मजानीन्द्रियाणि, तेषां भावाभावहेतुर्दैवं; तस्मान्नैकान्ततो
जडादिभ्यो जाताः पितृसदृशरूपा भवन्ति||

Reference – Charak Samhita, Sharira Sthana 3/17

The above shloka states answers the question that if a human is a product of another human then why is the offspring of a dull human not always dull? The concept of seed, i.e., sperm or ovum (Beej) plays a vital part. Seed or chromosome (Beejbhaga) are responsible for the development of a specific organ. So if they are affected somehow, the respective organ will also get affected. If the seed or chromosome are healthy, then the organ developed will be healthy. There is a presence of both possibilities. The sense organs of all living beings are derived from the soul and their existence is determined by the result (Karma) of the past actions. So it is definitely possible that the progeny of dull parents will not necessarily resemble that aspect, it also depends on the chromosomes of the child.

Ayurveda Etiopathogenesis (Samprapti)

• Due to causative factors like improper diet and regimen during gestational period, previous life actions, Kapha dosha of the body gets aggravated and obstructs all the Srotas (channels of formation and transportation of body tissues) including the Manovaha Srotas.
• This leads to vitiation of Vata dosha which travels throughout the body affecting all the Srotas.
• Throughout the life of a Prader Willi syndrome patient, Kapha and Vata will increase and decrease.
• According to that symptoms may change and vary frequently or at different age groups.
• This reflects the mobile quality of Vata Dosha and can be inferred that this syndrome is heavily controlled by Vata. Without proper care and management, Pitta dosha also aggravates and pollutes the Srotas of the body.

Ayurveda Management

• As a preventive step to avoid progeny with congenital disorders, mother and father should go through purification procedures before conception:
• Couples can undergo purification therapy (Shodhana) that includes preparatory therapies like oleation (Snehana), sudation (Swedana), then major procedures like therapeutic emesis (Vamana ), purgation (Virechana) or decoction based/oil based enema (Asthapan/Anuvasana Basti). Enema through the vaginal route (Uttar Basti) can also be done for females.
• It helps keep the hormones at a normal level.
• It would help in the removal of endotoxins.
• Symptoms and complications like obesity, deep vein thrombosis, high BP, Diabetes mellitus can be managed to increase quality of life.
• Intellectual limitation can be managed with memory enhancing and rejuvenative herbs (Medhya Dravya).

Herbal Medications for Prader Willi Syndrome By Planet Ayurveda

Planet Ayurveda is purely an Ayurvedic company that provides a first-class range of herbal medications, supplements and various other products used in day-to-day life. Planet Ayurveda is safe and GMP certified. It is applauded for its effective and potent herbal remedies. Few such remedies that can manage Prader Willi Syndrome are listed below:

1. Gandhak Rasayan
2. Telo Extension
3. Arvindasav
4. Kumar Kalyan Ras
5. Ashwagandharisht

Products Description

1. Gandhak Rasayan

Gandhak Rasayan contains purified sulphur as the sole ingredient of the product. It helps increase body strength and stamina. If the patient indulges in obsessive habits like skin picking, this medication speeds up the healing process of the skin. It is rejuvenating in nature and helps flush out toxins from the body. It will also be helpful in managing complications such as obesity and diabetes.

Dosage: 1 tablet twice daily, with water after meals.

2. Telo Extension

This herbal formulation is manufactured by Planet Ayurveda. It contains potent extracts of Pine Bark (Pinus longifolia), Ashwagandha (Withania somnifera), Rose Hip (Rosa indica), Giloy (Tinospora cordifolia) etc. It reduces oxidative stress with its antioxidant property which prevents further damage to the nervous system. As a result, cognitive function in the patient may improve. It will help induce calmness in the body thus decreasing irritability and temper tantrums in affected children. It will also help in balancing blood sugar levels caused due to overeating.

Dosage: 1 capsule twice daily after meals with water.

3. Arvindasav

Planet Ayurveda’s Arvindasav is a very effective remedy. It is regarded as a tonic for children. It contains ingredients like Kamal Phool (Nelumbium speciosum), Khus (Vetiveria zizanioides), Manjishtha (Rubia cordifolia) etc. It helps in proper growth and development of children. It manages psychological problems associated with Prader Willi syndrome. It counters weakness and exhibits anti-diabetic activity. It may help improve the complexion of skin.

Dosage: 2 tsp twice daily with equal water after meals.

4. Kumar Kalyan Ras

As the name suggests, this classical formulation is especially used for the benefit of children. It has immunostimulatory properties along with adaptogenic benefits, thereby, improving overall health and promoting longevity. It contains minerals like Mukta Bhasma (Calyx of Pearl), Swarna Bhasma (Calyx of Gold), Abhrak bhasma (Calyx of Mica) etc. It will also help strengthen the bones and spine with its satisfactory calcium content preventing conditions like scoliosis. This preparation is Vata pacifying in nature which helps to regulate distorted appetite and disturbed sleep. It aids in increasing height of the child alongside strengthening the brain and heart.

Dosage: 1 tablet twice a day with 1 tsp cow ghee after meals.

5. Ashwagandharisht

It is a herbal liquid preparation containing ingredients such as Ashwagandha (Withania somnifera), Vidarikand (Pueraria tuberosa), Nishoth (Operculina turpethum) etc. It acts as an energy booster and stress reliever. It provides both physical and mental health benefits. It also helps regulate sex hormones. It helps to capacitate for neuromuscular insufficiencies. Its neuroprotective action will help reduce obsessive habits in patients. It will provide strength to the muscles that will eventually help to prevent complications like hernia later in life.

Dosage: 2 tsp twice daily with equal water after meals.

Conclusion

We have discussed above that Prader Willi Syndrome patients exhibit distorted appetite with complex physical, behavioural and intellectual issues. It is a genetic disorder which is irreversible but effective measures such as administration of Ayurvedic formulations can manage this disorder well. Ayurvedic herbal remedies can also help prevent serious complications of the disease. Children suffering from Prader Willi syndrome deserve a fair chance to prove themselves in life and Planet Ayurveda is here to give it to them.