Management of Sickling with the AYURVEDA

  INTRODUCTION :-

Sickling is an incurable and hereditary disease of the blood caused by genetic disorder in the hemoglobin pattern. It is found in some selected ethnic groups of black races all over the world. Unfortunately, in the Chhattisgarh a large number of people are suffering from sickling, why that it has become a major health problem in C.G. The sufferers may have to face the characteristic episodes of the crises of sickling. Such sufferers are very sensitive to pathogenic micro- organisms owing to the poor immunity. They remain prone to any infectious disorders and so they are common victims of the seasonal diseases and sometimes of the serious diseases too. It has been seen that sometimes both the sickling and thalessemia are found simultaneously to make the condition more worst. Sickle cell disease can cause death in children and disability in the adults.

GEOGRAPHICAL DISTRIBUTION:-

1. Africa, America, Mediterranion  countries, India, South-East and middle Asia.

2. Along with the distribution of Plasmodium Falciparum.

ETHNICAL DISTRIBUTION IN INDIA:-

     Tribes, Kurmi, Sahu, Yadav, Kumhar, Dhobi, Panika, Ganda, Satanami, Sindhi-------.

PROBLEMS:

1.      Unfortunately, sickling is an incurable and hereditary disease, so it is inherited from parents to generation and to next generations. In the modern medicine, genetic engineering may be the only ray of hope.

2.      There is defective opsonization of some patho-micro-organisms like pneumoccocci and salmonella in sickling, so the individual is very prone to such infections. On the other hand, owing to the poor immunity of the individual and oxidant nature of the allopathic medicines which are being prescribed to the sickling-patients for any ailment are rather less effective and more harmful to them.

3.      The sufferers of sickling almost always have some common ailments to be treated, but they take longer time to be cured rather than the other patients having the same problems.

4.      In some cases, the patient may get developed permanent physical disability which may ultimately cause him a burden to the family.

5.      More to it, unawareness of the nature of this disease and often late diagnosis make the condition more worst to care.

6.      Now, the problem is, what we have with us to give relief to the sufferers? Obviously, our doctors have not any medicine to cure it.    

 AIMES:

·        The main aim is making the people aware of the nature and cause of the sickling why that they should go through the right way and into the right direction.

• Enabling the ethnical people to identify the suspected cases and motivating them for confirm diagnosis.

• Prevention of inheritance of the disease into the next generation . 

• Enabling the patients to live normal life and improving their working capacities .

• Controlling the possible physical disabilities .
• Minimizing the pain of crises and preventing secondary diseases .    

HAEMOGLOBINOPATHY:-

    -  Sickling is a molecular disorder caused by molecular change in the genetic pattern.

    - Autosomal, 11th number chromosome is affected. More than 300 Hgb variants are known.

     - Haemoglobin molecule is composed of two pairs of polypeptide chains, each chain is attached with one haem group. On the basis of sequence of amino acids, the polypeptide chain may be of four types viz.a, b, g and d. In a normal haemoglobin molecule, are attached 2a chains and 2b or 2d chains. An alpha chain consists of 141 amino acids, while that beta and delta chains consists of 146 amino acids.

     - In case of sickling, b chain is abnormally formed in which valine is substituted for glutamic acid in the sixth position of amino acid sequence.                   

     -   Haemoglobinopathy results in dyserythopoisis and haemolysis.

                                               HAEMOGLOBINOPATHY

                                       /                             /

  

Haemoglobine variants(more than 300)                      Thalassemia syndrome

Caused by-                                                                          Caused by-

Gene mutation / Genetic material deletion.            Complex genetic defects.  

SICKLED R.B.Cs:-  In oxygen deficiency, haemoglobin S molecules get polymerized and form crystals to damage the membrane of corpuscles in the form of sickle cell.

TYPES OF SICKLING-

1. SICKLE CELL TRAIT:- Heterozygous. Mixed Hgb i.e. A/S, Ratio may be 40-50%. Usually asymptomatic otherwise any special condition. Spontaneous haematuria and hyposthenuria may occur. Haemolysis or other serious symptoms are not found in uncomplicated cases.  

2. SICKLE CELL DISEASE:- Homozygous. Hgb S/S ; Hgb- A absent. In children, there may be Hgb-S 90%, Hgb-F  2-10% and a trace of Hgb- A2.  In such children, the haemolytic process may begin in 6 to 8 weeks of age but signs and symptoms are not manifested before the age of 6 month. In other variants, Hgb S/C is also of serious nature.                                                            

FREE RADICALS AND ISCHAEMIC CELLULAR INJURY IN SICKLING:-

1.      During the production of ATP, oxigen is reduced to water with the removal of four electrons to form free radicals, they are capable of stimulating autocatalytic reactions with the further generation of toxic oxygen species-     superoxide, hydrogen peroxide and hydroxyl radicals.

2.      In ischaemia, diminished availability of ATP causes cell damage with loss of membrane permeability (reperfusion injury) which is secondary to the generation of free radicals. Free radicals are highly reactive and unstable atoms or molecules of oxygen having one electron in its outer orbital shell.

3.      Free radicals oxidized the LDL cholesterol in absence of antioxidants.

4.      LDL is responsible for the formation of many hormones.

5.      Antioxidants neutralize free radicals. 

ANTIOXIDANT   DEFENCE   SYSTEM:- Three enzymes are -

1. Superoxide dismutase enzyme          -converts superoxide to hydrogen   

                                                                          Peroxide and oxygen.

2. Catalase enzyme                                   -breaks hydrogen peroxide to 

                                                                          water and Oxygen.

3. Glutathione peroxidase enzyme        -converts hydroxyl radicals and

                                                                                 Hydrogen peroxide to water.

ANTIOXIDANTS:-

1. Vitamin C, E, A, beta carotene, coenzyme Q-10, alfa lipoeic acid, pigmented bioflavinoides.
2. Antioxidants work in presence of Cu, Zn, manganese and selenium.
3. Antioxidants need folic acid, vitamin –B1, B2, B6 and B12 for enzymatic reactions.
4. Ultra violate rays produce more free radicals in our body.
5. Vitamin C prevents the oxidation of LDL in the plasma and sub endothelial cells of the vessels.

Oxidative stress can cause-

(a) degeneration of DNA of the nucleus and

(b) cell mutation.   

PRECIPITATE FACTORS IN SICKLING:-

1. Oxygen deficiency, flying at high altitude, excessive exercise, dehydration, shock .
2. Petroleum fumes, acidosis,  severe  cold, smoking, alcohol.
3. Pregnancy.
4. Minor infections.
5. Oxidant drugs e.g. Antipyretic, Antimalarial, Aspirin, Sulphonamides, Naphthaquinolones, Nitrofurantoin, Vitamin K.   

 HOW TO IDENTIFY:-

1. Frequent cold or pneumonia .
2. Lethargy, weakness .
3. Episodes of malaise, joints pain, abdominal pain (some times acute).
4. Spleenomegaly.
5. No relief even with the appropriate medicines .

MOST AFFECTED ORGANS:- Bones, liver, spleen, lungs, brain and eyes.

EFFECT ON GROWTH:-

                                                   1- Normal in beginning,   

                                                   2- later on under weight.    

                                                   3- Delayed puberty, specially in male.       

                                                   4-  Bony disfigurement after pregnancy .

SUSCEPTIBILITY:-

In first year of life the patient is more susceptible to pulmonary infection, septicemia, pneumococcal meningitis, osteitis and salmonella osteomyelitis. In early five years of life, death rate may reach to 25% in 10% sufferers of sickle cell anemia patients due to this susceptibility, if care is not taken properly.

CRISES OF SICKLING:-                   

1.     Vaso-occlusive crisis .

1.      Lack of blood supply in the affected part of the organ . Tissue ischemia and infarction.

2.      Symmetrical hand-foot syndrome in children, leg ulcer in adolescents .

3.      Spleenomegaly with functional hypoplasia . Later fibrosis or autolysis.

4.      Acute abdomen, painful swelling in large joints in adults .

5.      Strokes, hemiplegia or death .

6.      Pneumonia like symptoms due to pulmonary infarction .

2.          2.     Sequestration crisis:-

                 - A major cause of child death in homozygous.

                    - Abundant blood supply to liver and spleen .

                    - Massive enlargement of spleen, signs of circulatory collapse develop   soon.

3.          3.   Aplastic crisis (bone marrow hypoplasia):-

                      - Failure of erythropoisis .

                         - Circulating reticulocytes are <0.1%.

                         - Erythrocytic precursors are less in bone marrow .

                         - Lasts for 10-14 days, the condition resolved spontaneously .

1. Hyper haemolytic crisis :-

Unusual, found in homozygous having G6PD deficiency and any   infection or use    of Oxidant drugs.

      ii.             Jaundice may occur. Some times choelithiasis too, even in three years of age.

    iii.             Nephrotic syndrome, due to fibrosis in glomerular and tubular regions .

     iv.             Hemiplegia due to infection in CNS . 

PATHOLOGICAL INVESTIGATIONS:-

Confirmative test is ‘electrophoresis of haemoglobin’.

1-Screening test for SC Disease              - in new born or before the age of 9 months.

2-Screening test for Hgb S & C traits     - at the age of 12-15 yrs.

3-Pre-natal screening                               - as early as 16-20 weeks of gestation.

PREVENTION:-

1-      Before the marriage -

Hgb. electrophoresis examination of bridegroom and bride.

2-      During pregnancy -

Nutritious and balanced diet, protection from infections and radiation, avoiding oxidant drugs, proper utilization of anti-oxidant substances and drugs as mentioned in Ayurvedic treatises (masanumasik garbhini parichrya), diagnosis of high risk enceinte in due time, management of safe and atraumatic delivery.

3-      On birth -

screening test in new born.

CONCEPT OF AYURVEDA

It begins with the vitiated VAAT in the foetus and becomes  TRIDOSHAJ VYAADHI. Sickling is the consequence of haemoglobinopathy, in which relatively short life span of R.B.Cs and vasoocclusive crisis cause inadequate oxygen supply to the tissues. Consequently, there are abundant formation of free radicals to cause oxidative stress and acidosis to impair and weaken the normal physiological functions and ensuing pathological deterioration in the tissues. Oxidative stress causes a pathological phenomenon called KAFAVRITA-VAATA .In this phenomenon the pran-vayu is unable to reach the cells owing to the obstruction created by vitiated kafa .Again, acidosis caused by wrong habits of diet and conducts makes the kafa more vitiated to impair the function of prana-vayu. So, we have to defend at three points viz.

            1. The act of vitiation of the kafa should be inhibited ,

            2. Oxidative stress should be minimized and 

            3. Acidosis must be controlled .

         Normally, one gram of haemoglobin can carry 1.34 ml of oxygen and 100 ml of blood plasma can carry 0.3 ml of oxygen. The depletion of oxygen can be supplemented by enriching the blood plasma with extra oxygen supply by Yogic breathing, practicing Ayurvedic life style and by changing the food habits. Our life style influences the internal environment of our body systems and is one of the factors responsible for alterations in the genetic pattern. Ayurveda suggests the way of scientific life style which is close to the nature of the human being. So, with these measures, we can presume the correction in the genetic pattern ……..consecutively, in the subsequent generations. We know that oxidant nature and unwanted effects of the modern medicines have limited the use of these medicines for the symptomatic treatment of crises of sickling. Fortunately, the Ayurvedic medicines are relatively more effective, safe and valuable for the symptomatic treatment of the crises as well as for curative treatment of the secondary ailments in case of sickling. The anti oxidant nature of the Ayurvedic medicines has made them more acceptable. Non the less, in this case counselling is more valuable than the medicines.      

MANAGEMENT:-

·        Ayurvedic life style.

·        Daily practice of pranayam and havan.

• Avoidance of heavy exercise, dehydration and factors causing acidosis.
• Abstinence from all the narcotic drugs and drugs of oxidant nature.
• Use of sufficient water, succulent fruits, products of aquatic plants, nutritious and easily digestible food.
• Use of Ayurvedic medicines for the treatment of common ailments and even of syndrome too.
• Campaigning for identification of the sickling patients and dividing them into two groups viz. A- unmarried and B-married. Again, on the basis of electrophoretic examination of hemoglobin they can be subdivided into sickle cell disease and sickle cell trait groups for the sake of easy management.
• Counselling for correcting the life style of the affected individuals in the light of Ayurvedic teachings in the presumption of building the suitable conditions (DOSH-DHATU SAMYAVASTHA i.e. balancing state of tissues and body humors )for initiation of genetic correction in the succeeding generations.
• Promotion of meticulous matching of the blood-horoscopes of bride and bridegroom before the settlement of marriage .
• Health awareness programs through specially designed lectures on sickling for schools and colleges.
• Management of sickling patients in Ayurvedic dispensaries .                                           

TREATMENT:-  No specific treatment. जीवनीयगण की औषधियाँ लेने तथा संयमित जीवन शैली से लाभ होता है.

   FORMULATION:

1.      Of havan-samagri – amrita , arjun , go-ghritam , jaggery , and tandulam .

2.  Of special medicine for episodes of crises – containing  amrita , aravinda , ashwagandha , ashwathha , chaulai, doda shak ( Leptadenia reticulata ), dashamula , dhatri, durva , ela , haridra , haritaki , Kamal, Kevuka , laksha , Madhu, Makoya, Mung dal, punarnava ,Panchatrin mul,  rohitaka , rudanti , sharapunkha , shatavari , Shigru, shringataka , spirulina , trifala , trikatu , tulasi , twaka and yashada-bhasma .

EXPECTED RESULTS: Sickling could be controlled with this mission at six levels –

1.      Pre marriage hemoglobin investigation and appropriate matching would prevent the inheritance of sickle cell disease in the next generations.

2. Number of sickling patients could be declined and secondary diseases would be controlled .
3. Child death due to sickling would be declined by genetically approach .
4. Anemia would be controlled and symptoms could be minimized in sickle cell trait patients by advising them to inhibit the precipitative factors of anemia in SCT cases . And it would be     possible just because of health education and Ayurvedic counselling.
5. Disability would be prevented and the patients would be enabled to enjoy their normal and happy life.
6. Harmless and effective Ayurvedic medicines should be made available to the sufferers to cure other ailments.

-DR. Kaushalendra

Govt. K.D. Disstt. Hospital, Kanker, C.G. 394334

Reference :-

1-  Text book of pediatrics, Eleventh Asian Edition, W.B. Saunders company       publication. Hemoglobinopathies.

2-  Muir’s Text book of pathology, thirteenth Edition, ELBS Publication. Hemoglobinopathies. Cell injury and death.

3-  Charak Samhita by R.K. Sharma and Vaidya Bhagawandas, Volume 1-2, Chaukhamba Sanskrit Series , Office- Varanasi.