An Ayurvedic Management of Sickle Cell Anemia in Children: A case study

Introduction:- Sickle cell anemia is a term for a group of genetic diseases characterized by the production of hemoglobin (Hb) "S". This is the result of replacing the base pair thymine at the 6th position of the β -globin gene on chromosome 11 with adenine and replacing valine with glutamic acid. A single amino acid substitution is the reason for the profound changes in the stability and solubility of the hemoglobin "S" molecule. Due to its chronic nature and painful crisis, children’s quality of life is difficult. From now on, there is no final treatment other than a successful bone marrow transplant. Its symptoms and the nature of the disease can be compared with Pandu . If a drug improves the quality of life and maintains the health of the patient, then the drug and efforts will be beneficial to society. Material and Methods:- A 9-year-old male patient was diagnosed with sickle cell anemia. Symptoms include joint and limb pain, anorexia, fatigue, loss of appetite, and weight loss. of , Vadodara. Observations and Results:-The patient is taking modern medicine (1 mg folic acid once a day). The Childs parents also wanted to take Ayurvedic medicine, so they came here for 2 months of Ayurvedic medicine treatment, during which he did not have a pain crisis.Weight improved From 16 kg to 18 kg.The quality of life of a patient is also improved. Conclusion:-Therefore, the purpose of this case study is to study the pathophysiology and treatment of sickle cell anemia from an Ayurvedic perspective and to study the possible mechanism of action of the drug.


Introduction
Sickle cell anemia is an autosomal recessive genetic disease of red blood cells. It is also called a "molecular" disease because it is the result of amino acid mutations in the hemoglobin molecule (1). This is the result of replacing adenine with base pair 6 of the βglobin gene on chromosome 11 and replacing valine with glutamate. The substitution of amino acids will cause profound changes in the molecular stability and solubility of hemoglobin. Slight changes were also observed in the non-coding nucleotide sequence of the gene. Polymorphic variants are called haplotypes. Four haplotypes were found. The Indian-Arab haplotype is found in eastern Saudi Arabia and the Indian subcontinent. Bantu haplotype is common in Africa, Mediterranean countries, North and South American countries.The term SCD encompasses several different genotypes; the most common are homozygous sickle cell anemia (SS), heterozygous (AS), and sickle thalassemia (Sβ0), and the less common forms are SD, Punjab disease, SO Arab disease(3). They usually start when they are 5 to 6 months old. Various health problems may occur, such as pain episodes, anemia, bacterial infections, swelling of hands and feet, and strokes. Sickle cell crisis is divided into three categories: painful crisis, sequestration crisis, vascular occlusive crisis, and aplastic crisis (4).Although there is a lack of literature on sickle cell anemia in ancient Ayurvedic literature, this disease called Pandu shows some similarities in the clinical features of sickle cell anemia. Pandu Roga has been explained in all the classics of Ayurveda. Panduta being a prominent sign of the disease is termed as Pandu Roga. Panduta (paleness) is an important diagnostic feature of Pandu roga. The most common symptoms are pale, irritability, weakness, malaise, headache, etc. Pandu Roga is caused by intake of unwholesome food (hot, sour, salty, oily food articles), unwholesome activities like (day sleep, suppression of natural urges and physical activity when food is digesting).In Charak Samihita Dadimadi Ghrita is advised in treatment of Pandu(2)

History of present illness
Patient has pain in joints and limbs, anorexia, loss of appetite, fatigue, and weight loss. On examination pallor++ was found.

History of past illness
Nothing significant.

Birth history
No major medical intervention was required during prenatal, natal, and postnatal periods. immunization was done as per the national schedule.

Personal history
• Aharaja-vegetarian and quantity and appetite was poor. • Viharaja-The patient could not play outdoor games due fatique. Sleep was 6-8 hrs/night. • Examination-vitals were within normal range. No, abnormality was found in the cardiovascular system,respiratory system and per abdomen examination. Weight was 16 kg and height was 109cm. • Ashtavidha pariksha-Nadi(pulse) was normal.
Mala(stool)-Sama, constipated and consistency was semi solid and Mutra(urine) normal in frequency and no any associated complaints. Jihva(tongue) was Sama(coated) due to improper digestion and papillae found atrophied. Shabda(speech) was normal. Sparsha(touch) was Ruksha. Drik(eyes) are normal with slight pallor in conjunctiva but the sclera is normal(no icterus). Akriti (appearance)was lean and thin due to loss of appetite.

Results and discussion
Grading: • Pallor: 0-no pallor, 1-pallor of conjunctiva, 2-pallor o f c o n j u n c t i v a , n a i l s , t o n g u e , 3 -p a l l o r o f c o n j u n c t i v a , n a i l s , t o n g u e , s k i n 4 -p a l l o r o f conjunctiva,nails,tongue,palm and soles. • Pain in limb: 0-no pain, 1-pain in only one extremity, 2-pain in both extremities, 3-involvement of almost all bones. • Anorexia: 0-no anorexia, 1-taking a normal diet without any interest, 2-taking the food without interest and unable to complete it all the time, 3-no interest in taking food,resisting or crying while feeding. • Loss of appetite: 0-taking food in good quantity twice/thrice, 1-taking food in normal quantity twice a day, 2-taking food in moderate quantity twice a day, 3-taking food in less quantity twice a day, 4-taking food in less quantity once a day, 5-not at all taking food. • Fatigue: 0-normal active child, 1-playing and activities reduced, 2-feeling tiredness while playing, 3-easy fatigability while playing. 1=A lot of concern and interest=5 2=Some concern and interest=4 3=Uncertain=3 4=Little concern and interest=2 5=No concern and interest=1 20. How easy is it to get practical help from friends if you should need it? 5=Very easy 4=Easy 3=Possible 2=Difficult 1=Very difficult QOL Assessment-Quality of life assessment criteria is taken from WHO quality of life assessment. Quality of life improvement is very important in sickle cell anemia patient because in this disease quality of life of the patient is also hampered so reason for the assessment is to assess effect of the Dadimadi Avaleha in quality of life of patient. Assessment done before and after treatment and then compared the score for improvement.   Dadimadi Avaleha helped deal with all important aspects of Pandu i.e. Pallor, Anorexia, pain (limb), fever, periorbital edema, and fatigue. Dadimadi Avaleha contains the drug-like Chitraka, Dhanyaka, shunthi pippali which are Dipana i.e. improves the process of digestion, Pachana (like drug Pippali) detoxifies the body by removing Ama toxins. It improves digestion and reduces the accumulation of harmful cholesterol in the body, thereby improving metabolism and helping the body lose weight faster, which helps to establish proper nutrition for all Dhatu and is beneficial for Dhatuposhana. Dadim, Dhanyaka, and Shunti have antioxidant activity, help reduce sickle cell oxidative stress, and help prevent sickle cell anemia and SCA screaming(5). Dipana and Pachana preparations can improve the digestive process and eliminate damaged Dosha or Aama in the body. It also showed significant results of orbital edema, loss of appetite in Pitta and Vata Dushti, the elevation of Vata and Pitta Dosha, and the drug Rakta Prasadana Karma which can relieve Shootha. Dadimadi Avaleha, with the characteristics of Rasa Prasadana, can remove the ancient Dosha from the skin and help improve people's natural skin tone. hemopoetic action of the Chitraka which stimulates the hemopoesis and production of healthy RBCs in the children with sickle cell anemia(7). Coriandrum sativum L. has been recommended for relief of insomnia in Iranian traditional medicine. In sickle cell anemia there is painful condition which lead to disturbed sleep and it is relieved by the Dhanyaka(8). Dadimadi Avaleha helps activation of macrophages as shown by an increased macrophage migration index. Sickle cell anemia is characterized by painful vaso-occlusive crises.

Quality of life Assessment (WHO QOL parameters)
It is hypothesized that monocytes are activated in sickle c e l l d i s e a s e a n d c a n e n h a n c e v a s o -o c c l u s i o n by activating endothelium(8). Dadimadi Avaleha has s h o w n s i g n i f i c a n t i m p r o v e m e n t i n o b j e c t i v e criteria(Hb,PCV, MCV, MCH, MCHC AND RBC). The result of Hb% assessment revealed an increase in the mean during the intervention period.Quality of life of a patient is also improved. Dadimadi Avaleha is having Properties that helps to improve the nutrition and health of children. Activity, playfulness, and increase in concentration, improvement in irritability have been found.