An International Journal of Research in AYUSH and Allied

ayurvedic management of duchenne muscular dystrophy Suryanarayana Mudadla 1 *, Radhika Injamuri 2 * 1 Assistant Professor, P.G Dept. of Kaumarabhritya...

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AYUSHDHARA

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An International Journal of Research in AYUSH and Allied Systems Research Article AYURVEDIC MANAGEMENT OF DUCHENNE MUSCULAR DYSTROPHY Suryanarayana Mudadla1*, Radhika Injamuri2 *1Assistant Professor, P.G Dept. of Kaumarabhritya, SJGAMC&H Research Centre, Koppal, Karnataka, India. 2M.D. Scholar, Dept. of Kaumarabhritya, S.V.Ayurvedic College, Tirupati, Andhra Pradesh, India. Article info

ABSTRACT

Article History:

DMD is one among the most common muscular disorders. The incidence is 1:3600 live born infant boys worldwide. It is X-linked recessive disease caused by a deficiency of a normal muscle protein called dystrophin, which maintains the integrity of the muscle cell wall. Eventually irreversible progressive muscle weakness and degeneration of the skeletal or voluntary muscles, which control movement leading to lose the ability to stand, walk and loss of ambulation before 10years, with progression of the disease, most patients succumb to death in their early 20s. As there is no specific treatment in any system of medicine and the disease prognosis is unpreventable but if we start treatment in early stages of ambulant DMD boys, it may slow or stop the progressive degeneration of muscles. DMD cannot be directly co-related with any single disease in Ayurveda. All most all major neuromuscular disorders are identified with Vata dosha. In Ayurveda this pathogenesis can be clearly understood by the concept of Adhi bala pravritta vyadhi. Here the pathogenesis occurs due to the Beeja bhagavayava dusti which leads to Mamsa vata dushti. The present study was under taken to increase functional and physical capabilities, minimizing disability to delay further progression of disease and to maintain the ambulation for longer time and to improve quality in the activities of daily living. 30 children with DMD were taken up for the study of age between 05-16years and were given treatment for 2 weeks consisting of Abhyanga with Bala taila, shastika shali Pinda swedana followed by Vasti. The study has been subjected to statistical analysis, in which the results were found to be encouraging.

Received: 14-07-2015 Accepted: 05-08-2015

KEYWORDS:

DMD, Dystrophin, Beeja dushti, Abhyanga, Sastika Sali pinda sweda, Vasti.

*Corresponding Author Dr. Suryanarayana Mudadla Assistant Professor P.G Dept. of Kaumarabhritya, SJGAMC&H Research Centre, Koppal, Karnataka, India. Ph: 07411642191 Email: [email protected]

INTRODUCTION Among the more than 20 different genetic muscular disorders, the most common fatal one is DMD. It is ubiquitous and it occurs all around the world, causing considerable hardships to affected individuals and their families.DMD is x-linked recessive disease caused by a deficiency of normal muscle protein called dystrophin which maintains the integrity of the muscle cell wall. This leads to progressive muscle weakness and exhibits the symptoms like difficulty in getting up from floor, difficulty in walking, climbing stairs, frequent falls while walk and finally loss of ambulation1. The term dystrophy means “abnormal growth” or “faulty nourishment” or “poor nourishment” was popularised at the close of the 19th century, derived from Greek word “trophe” meaning nourishment. The incidence is 1:3600 live born infant AYUSHDHARA | May-June 2015 | Vol 2 | Issue 3

boys. The abnormal dystrophin gene on Xp21.1 locus and is one of the largest mammalian gene. In affected families which the mother is known to be a carrier of the gene, 50% of boys will be affected, and 50% of the girls will be carriers. Despite the X-linked recessive inheritance in DMD, about 30% of cases are new mutations and the mother is not a carrier. The new mutation rate in the dystrophin gene is high. Most cases are born now in families with no prior history of the disease. Patho physiology in brief, Dystrophin is a large, rod shaped cytoskeletal protein (molecular wt: 427000 Daltons) transcribed from a 14000 base mRNA, that is encoded by 79exons within the enormous 2.4million base DMD gene located at Xp21.2. Although dystrophin accounts for only 0.002% of total muscle protein, its absence reduction or abnormality and 188

AYUSHDHARA, 2015;2(3):188-193 changes in its associated membrane proteins appear to be the primary lesion in a no. of related myopathic or dystrophic disorders, resulting in variable phenotype of muscle wasting 2. Clinical presentation, Early gross motor skills such as rolling over, sitting, standing are usually achieved at the appropriate ages or may mildly delayed, Around 2-3 years of age, boys with DMD may appear to be “somewhat clumsy” due to hip girdle weakness. By the age of 3-5years they often have difficulty keeping with their peers on the play ground. In some children they can never run in their life. Toddlers might assume a lordotic posture when standing to compensate for gluteal weakness. An early “Gower’s sign” is often evident by age 3years and it is fully expressed by age of 5 or 6 years, delayed walking, frequent falls while walk, trouble in running, difficulty in climbing upstairs, toe walking, pseudo hypertrophy of calves, relentlessly progressive weakness of muscles, leading to wheel chair confinement at 12years. Death occurs usually at about 18-20years of age3. The cause of death are respiratory failure in sleep, intractable heart failure, pneumonia occasionally aspiration and airway obstruction.

factors bring Khavaigunya at Mamsa dhatu levels leading to vitiation of Vata which causes Bhutagni impairment6. Thus these two causes attribute to DMD may be analysed as Bheeja dusti and Atma karma are responsible for the X-linked recessive disease as well as gene mutations.

This disease cannot be directly co-related with any single disease in Ayurveda. All most all major neuro muscular disorders are identified with Vata dosha. This disorder can be considered under Vata vikara due to Bheeja dusti because of Adibalapravritta4. The cardinal symptoms is ‘Chestahani’ which indicates a decrease in the Chala guna and Pravarthaka chestana of Vayu. Finally causes paraplegia and Sarvanga vata.

b) PINDA SWEDAM10 – Shali dhanya (Rakta), Balamoola kwath, Milk, cloth and 2 vessels.

SAMPRAPTHI can be sort out as it is result of Mamsa-vata-kshaya due to Bheeja dosha which leads to Vata Vaishamya of Mamsa dhatu. This vitiated Vayu causes improper formation of Mamsa dhatu by its influence on the Dhatvagni of Mamsa7. So depletion of Mamsagni causes formation of Ama which leads to faulty nutrition and causes progressive relentless degeneration of muscle tissue8. Considering DMD as Vata vikara, Vasti treatment is adopted, as this best treatment for diseases of Vata. The treatment modality is applied mainly to combat Vata dosha along with drugs having Balya, Rasayana, Agni vardhaka & Vatahara properties. Any Shodana procedure should always be preceded by Snehana and Swedana. a) ABHYANGAM 9–Bala taila.

c) VASTI11– Madhu, saindavam, Shatapuspa, Tailam (Bala tail), Gomutram, Kashaya – prepared with Balamoola, Dasamoola, Yasti, Raasna quath churna d)

Instruments for Vasti – 50ml disposable syringe, Nel’s catheter 16 size.

The important causative factors can be brought under Aatma karmaja and beeja dosha5. These Table 1: Method of Preparation and Administration of Vasti Ingredients Madhu Saindavam Satapushpa Kashayam

4g 2g 1g

Tailam – Bala Taila Gomutram The above mentioned quantity of drugs is measured based on total dosage required for the particular age group. Method of preparation of Vasti Step 1: Madhu is first taken in the Khalwa and then Saindavam is added. They are mixed uniformly till the sticky sound disappears. Step 2: Tailam is added till the mixture becomes homogenous. Step 3: Shatapushpa is added, mixed carefully. Step 4: Kwatha prepared using above mentioned drugs in the ratio 1:8 and reduce to ¼ quantity, appropriate amount is added to Vasti preparation. AYUSHDHARA | May-June 2015 | Vol 2 | Issue 3

05 – 10yrs (150ml) 4 ml ½g 1g 66ml

11– 16 yrs (200ml) 6ml 2g 2g 80ml

75 ml 100ml 5 ml 10ml Step 5: Gomutram was added slowly & mixed uniformly. Step 6: Vasti dravya was made lukewarm by keeping it in hot water. Method of Administration ● The child was given very light food neither too Snigdha nor too Ruksha. It was checked that the child has passed the stool routinely in the morning. As Abhyangam with Bala taila and Pinda swedana has been done, after these Purvakarma the patient was made to lie in left lateral position with left lower extremity straight and right lower extremity flexed on knee and hip joint on the Vasti 189

Suryanarayana Mudadla, Radhika Injamuri. Ayurvedic Management of Duchenne Muscular Dystrophy table. Then the anal orifice was smeared with oil for lubrication. ● Required quantity of Vasti dravya was taken as per age and then was taken into syringe connected to Catheter (Nel’s catheter – 16 size). Then the Catheter was smeared with oil and after removing air bubbles the Catheter was introduced into rectum for about 4 inches and then Vasti dravya was gently pushed inside.

a. Patients between the age group 5 – 16 yrs. b. Children having mild to moderate physical disability

c. Children with DMD. Exclusion Criteria a.

children with advanced stage of DMD with cardio myopathies, respiratory complications

● During administration of Vasti, the child was made to take deep breath through his mouth.

b.

Congenital muscular dystrophies

c.

Myelopathies

● After the procedure the child was made to lie down on supine posture after gently tapping his buttocks. Then the child was made to lie down and mother/ attendant were advised to notice the time of retention.

d.

Congenital myopathies

e.

Metabolic muscle diseases

f.

Becker’muscular dystrophy

● The same procedure is done for 11 days. The patients were advised to take usual food. The review of follow up is done & asked to come for next sitting with gap of 2 months Clinical Plan Patients were selected from OPD /IPD of S.V. Ayurvedic hospital in department of Kaumarabhritya, based on the inclusion criteria and clinical symptoms. Inclusion Criteria

Diagnosis: already diagnosed cases were taken and also repeated serum creatine kinase muscle enzyme,which is sensitive indication of active muscular dystrophy. Study design All the patients were given Vasti for 11 days. All were subjected to Abhyangam with Bala taila and Pinda swedana with Rakta shali as per classical method prior to Vasti therapy for 14 days. During this period oral medication is also given which is continued between the sittings also

Table 2: Treatment plan Group 05– 10 Yrs

11– 16 Yrs

Treatment Abhyangam with Bala taila (20 – 25minutes) Pinda swedam (10 – 25 minutes) Vasti Abhyangam with Bala taila (20 – 30 minutes) Pinda swedam (20 – 35 minutes) Vasti

Dose 30 – 40 ml 150ml 40 – 50 ml 200ml

Duration 14 days 14 days 11 days 14 days 14 days 11 days

Table 3: Assessment criteria Criteria Symptoms Falling down while walk Difficulty in walking

Difficulty in climbing stairs Muscle power in the upper limbs Toe walking

3- severe

2- Moderate

1- Mild

0- No complaint

>5 times /day

1-5 times/day

1-5 times/week

ceased

Difficulty in walking even small distance With assistance

Difficulty in walking long distance with stops With support

Difficulty in walking long distances without stops Without support

Able to walk without difficulty

G1

G2

G3

continuous

often

2) Barthel Index:12 Grooming 0- Needs help with personal care 1- Independent face/hair/teeth/shaving/brushing

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Without support & difficulty G4

occasional absent Feeding 0- Unable 1- Needs help cutting/spreading butter, etc 2- Independent Toilet use 0- Dependent 1- Needs some help, but can do something alone 2- Independent (on and off, dressing, wiping) 190

AYUSHDHARA, 2015;2(3):188-193 Bathing 0- Dependent 1- Independent Dressing 0- Dependent 1- Needs help (but can do about half unaided) 2- Independent (including buttons, zips, laces) Stairs 0- Unable 1- Needs help (verbal/physical/carrying aid) 2- Independent Transfer 0- Unable- no sitting balance Results

1- Major help (one or two people, can sit) 2- Minor help (verbal or physical) 3- Independent Mobility 0- Immobile 1- Wheel chair independent 2- Walks with help of one person 3- Independent (but may use any aid) The efficacy of therapy was studied on various parameters and the results were derived after subjecting to statistical methodology by using Student paired t-test.

Table 4: The effects of the therapy on various parameters were as follows S. No Characteristics 1 2 3 4 5

Falling down while walk Difficulty in walking Difficulty in climbing stairs Muscle power in the upper limbs Toe walking

No. of Observations 27 28 26

Mean Score BT AT 2.5 0.4 2.3 1.4333 2.1 1.3

SD

P

Remarks

0.9595 0.7761 0.6644

<0.001 <0.001 <0.01

Highly Significant Highly Significant Highly Significant

30

2.4667

0.8667

0.7701

<0.001

Highly Significant

27

2.4667

1.1

0.7112

<0.001

Highly Significant

Improvement in the ADL Improvement in the ADL was assessed by Barthel index which is useful for measuring the functional status of the individual. In this score total possible score ranges from 0 to 20. If used to measure improvement after treatment, changes of more than two points in the total score reflects a probable genuine change and is also likely to be reliable. After treatment 04 points improved in two cases, 03 points improvement in 14 cases, 02 points improved in 10 cases, one point improved in two cases while remaining 02 cases were unchanged. The overall change in the total score is 2.6 which denote genuine change after the treatment. Table 5: Percentage of overall improvement in each ailment after treatment in 30 patients with respect to assessment criteria Ailment Falling down while walk Difficulty in walking Difficulty in climbing stairs Muscle power in the upper limbs Toe walking

Percentage improvement 84% 37.68% 38.09% 64.86% 54.05%

3 2 1 0

BT Falling Difficulty in Difficulty in down while walking climbing walk stairs

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At Muscle power in the upper limbs

At BT

Toe walking

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Suryanarayana Mudadla, Radhika Injamuri. Ayurvedic Management of Duchenne Muscular Dystrophy DISCUSSION By this treatment encouraging progress observed in the following major complaints Frequent falls while walk completely ceased in 18patients, good progress in 6cases, while remaining 3 children got moderate results. Overall 84% of progress is observed may be due to increased muscle power in the both lower limbs and softening of the pseudo hypertrophy of calves. After treatment there was good result in 15 patients with improvement in quality of walking, in 7patients there is moderate improvement and in 6patients no improvement. Overall percentage of progress is 37.68% Difficulty in climbing stairs in 09 there was moderate progress and no improvement in 07 patients. Overall percentage of progress is 38.09%. It was statistically highly significant. Muscle power in the upper limbs: There was an overall progress of 64.86%. Among 27 cases there is excellent improvement in 03 cases, 24 cases got good improvement, while remaining 03 cases got moderate improvement. After treatment most of the patients were able to raise both hands by overcoming the gravity due to improved muscle power. In Toe walking there was excellent result in one case with complete correction of toe walking. In 14 cases there was good improvement, moderate results in 08 cases and remaining 04 patients had no improvement. This result is due to reduction of spasticity in the Achilles tendons and by lengthening of already contracted connective tissue. And there may be reduced hyper tonus in calves, increased muscle power. Overall progress was 54.05%. The overall change in the total score of Barthel index is 2.6 Effect of therapies on other ailments Contractures Most often involve the Ankles, Knees, Hips and elbows, because the muscle fibers shorten and fibrosis occurs in connective tissue. Among 30 one child had contractures at knees. He got good result with improvement in the extension of knees (increased range of motion). Pseudo hypertrophy of calves As per the observations all young ambulant boys had the complaint of pseudo hypertrophy of calves with hard consistency. After treatment there was marked change in consistency i.e. calves become soft and size is also reduced in all the cases of DMD. Frequent falls and toe walking has come down due to decreased tonicity in the calves. Gower’s sign

a clinical assessment of pelvic muscle weakness in which considerable result was not observed and Gower’s sign is remained persisted. All these results were obtained by Abhyanga, Pindasweda and Vasti. Their mode of action presumed to be as follows. Abhyanga stimulates circulatory system, enhancing cell activity, increases blood flow, vasodilatation result in nourishment of the muscles, strengthening the muscles, releasing facial constrictions, assisting in reducing connective tissue thickening and provide flexibility by decreasing fibrous adhesions from muscle tissue injury. Due to Abhyanga progress is seen in reduction of toe walking, relieving contractures, nourishment of atrophied muscles, increasing muscle power and assisting muscle tone.13 Heating the skin has been demonstrated to produce a decrease in gamma activity. With a decrease in gamma activity, the stretch on the muscle spindle would be less, thus reducing afferent firing from the spindle. This indirect method ultimately results in decreased alpha motor neuron firing and thus less muscle spasm. Elevating muscle temperature can also alter strength and endurance. Heating can result in decreased joint stiffness and increased tissue extensibility, thus facilitating ease of motion and gains in range of movements14. Vasti is having two actions, expelling the Dosa & nourishing the body as it is indicated in Gambhiragata vata also15. First, potency of the Vasti drugs gets absorbed to have its systemic action. Its second major action is related with the facilitation of excretion of morbid substances responsible for the disease process into the colon, from where they are evacuated. CONCLUSION The present study was under taken to increase functional and physical capabilities, minimizing disability to delay further progression of disease and to maintain the ambulation for longer time and to improve quality in the activities of daily living. 30 children with DMD were taken up for the study of age between 05-16years and were given treatment for 2 weeks consisting of Abhyanga with Bala taila, Shastika shali Pinda swedana followed by Balamooladi Vasti which was continued even during the interval period of 2 months. Based on the observations and results it is concluded that  The given treatment is efficacious.  Frequent fall down while walk was stopped in many patients, while it was reduced in some other patients. Overall 84% of progress is observed.

An early Gower’s sign is often evident by age 3 years and is fully expressed by age 5 or 6 years. This is AYUSHDHARA | May-June 2015 | Vol 2 | Issue 3

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AYUSHDHARA, 2015;2(3):188-193  Considerable improvement in difficulty (37.68%) in walking and climbing stairs (38.09%) was observed.  The muscle power in the upper limbs improved (64.86%) significantly which was expressed by raising hands above the shoulder without difficulty.  Toe (54.05%) walking also shown considerable reduction.  Contractures also released to some extent. There is overall progress observed functionally, physically, psychologically, and nutritionally in these children with minimum or no side effects, which is much economical. REFERENCES 1. Nelson text book of paediatrics by Kliegman, Stanton, St.Geme, Schor, Behrman, W B Saunders company edition 19th, vol. 2, 2119. 2. Mokri B, Engel AG (1975) : Duchenne dystrophy : electron microscopic findings pointing to a basic or early abnormality in the plasma membrane of the muscle fibre., Neurology (Minneapolis) 25: 1111 3. Kevin p.campbell (1995) three muscular dystrophies: review loss of cytoplskeletonextracellular matrix linkage, cell, vol.80, 675-679. 4. Sushrutha samhitha : Ayurveda tatva sandeepika hindi commentary by kaviraj ambica dutt sastri, Chaukamba Sanskrit series office, Varanasi, edition 2009. su.su.24/4,page no.113. 5. Charak samhitha: Text with English translation and critical exposition based on Chakrapani Dutta”s ayurveda Dipika by Dr.R.K. Sharma and Vaidya bhagavan dash, Chaukamba Sanskrit series office, Varanasi, edition 2009,Ch.Sa 3/17,p.no 384.

6. Jain Mukesh D. (2006) Mamsagni Rasayana in the treatment of Duchenne Muscular Dystrophy: 28 Patients. Ayurvedic Patrika Nasik (MS) India Vol. 1:3 March. 24-29. 7. 7.Ashtanga sangraha: by Dr. Shivapras Sharma, chowkamba Sanskrit series office, Varanasi. 2014, page.no 142 8. Nair P Ramchandran et al (1980) : Pseudohypertrphic muscular dystrophy- An Ayurvedic Approach. Journal of Res. In Ayurveda & Siddha 1:3 (429-437) 9. Charak samhitha: Text with English translation and critical exposition based on Chakrapani Dutta”s ayurveda Dipika by Dr.R.K. Sharma and Vaidya bhagavan dash, Chaukamba Sanskrit series office, Varanasi, edition 2009,Ch.si 1/7.page.no 2 10. Sushrutha samhitha: ayurveda tatva sandeepika hindi commentary by kaviraj ambica dutt sastri, Chaukamba Sanskrit series office, Varanasi, edition 2009. su.chi.32/4,page no.300 11. Ashtanga Hridayam: Text with English translation by Prof. K R Srikantha Murthy, Chaukambha Krishnadas Academy, Varanasi, Edition: 6th 2009.A.H.SU 11/5.page no 155. 12. Disability evaluation - Stephen L.Demeter, Gunnar B.J Anderson – 2nd edition. page.no. 356. 13. Principles and Practice of Therapeutic massage by Akhoury Gourang Sinha, Jaypee brothers medical publishers, Edition: 2nd, page.no.243. 14. MartinFH. fundamentals of anatomy and physiology chapter 5.4th ed, New jarsy. prentice hall inc. simon & Schuster;1998.p.148-149& 162. 15. Agnivesa, charaka samhitha, with chakrapanai data: In, acarya Yadavji Trikamji, ed, Ayurveda dipika, commentary, reprint ed. Newdelhi: chaukamba surabharati parkashan; 2008.p.731732.

Cite this article as: Suryanarayana Mudadla, Radhika Injamuri. Ayurvedic Management of Duchenne Muscular Dystrophy. AYUSHDHARA, 2015;2(3):188-193. Source of support: Nil, Conflict of interest: None Declared

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