Sickle Cell anaemia is characterised by malformed sickle-shaped RBC into blood circulation.
Pathophysiology of Sickle Cell Anaemia
In Normal adult humans the RBC are bi-concave dics shaped and predominantly has Heamoglobin-A.
Heamoglobin-A is comprised of 2 apha-globulin chains and 2 beta-globulin chains. Certain mutations in gene present on chromosome 11 that is responsible for production of beta-globulin chain of heamoglobin results into formation of so-called heamoglobin S.
Heamoglobin S is fragile and less elastic. It deforms the regular bi-concaved disc shape of RBC into sickle-shape which are not only obstructive in nature but also has low oxygen carrying capacity are less elastic resulting into excessive destruction of RBC and anaemia.
Due to reduced electricity of RBC their flexibility reduces which impairs their ability to flex and pass through small capillaries also they get deformed as they aren’t able to maintain their patent form due to loss of elasticity resulting in deformed RBC in sickled shape and also due to the shape they get caught up with each other and causing blockages in blood vesseles resulting into Infarction in corresponding tissue.
These Sickled RBC’s have 1/5th to 1/10th life span (10-20days)compared to life span of normal RBC(90-120days) in healthy individuals also they are being destroyed by our immune system all these factors caused excessive hemolysis and overload on bone marrow which is constantly trying to produce more RBC to compensate loss.
SIGNS AND SYMPTOMS
Signs and symptoms of sickle cell disease usually starts appearing from early childhood like dactylitis frequently presenting as earliest sign in many cases around age of 6mnths with dactilitis usually being the very early symptoms.
SCD patient frequently presents with various mild to severe and lifethreatening symptoms. The severity of disease may vary in each individual.
- Swelling of extremities
- Delayed growth and puberty
- Frequent pain in chest, abdomen, head muscles and joints
- Deterioration of vision to complete loss vision
- Frequent infections
Symptoms are due to lack of sufficient oxygenation of various tissues due to reduced oxygen carrying capacity of sickled RBC and also reduced number of RBC(due to destruction of sickled RBC by immune system and reduced life span of sickled RBC’s), blockages of blood vessels supplying various organs causing Infarction, stressed out immune system fighting with sickled RBC’s and its complications.
Sickle Cell Anaemia reduces life expectancy.
Symptoms may vary from chronic symptoms like retinopathy, CKD, hematopoietic ulcers to severe acute complaints like “Sickle Cell Crisis” triggered by dehydration, infections, acidosis and other obscure reasons.
Sickle Cell Crisis:-
In SCD, RBC’s due to their low elasticity gets disfigured to assume sickle shape. Due to low elasticity RBC couldn’t easily squeeze out from small capillary lumen and adding to it a sickle shape, entraps them in capillaries and arteries causing obstruction in vessels, reducing blood flow resulting in ischemia or even infarction due to complete obstruction of blood flow. It is the cause of frequent episodes of pain and stroke in SCD patients. Obstruction of arteries supplying vital organs is medical emergency.
Splenic Sequestration Crisis:-
RBCs disfigured due to low elasticity in SCD gets trapped in spleen reducing circulating heamoglobin resulting hypovolemic shock and enlarged spleen. It is medical emergency and patient can die within couple of hours if not treated.
Splenic functions are reduced considerably in SCD patients due to frequent splenic infarctions reducing its capability to remove defective cells and certain organisms from circulation, this reduces immunity of person significantly thus increasing the risk of frequent infections.
Parvovirus B19, which causes reduced production of RBC by infecting and destroying RBC’s precursor cells, is a major concern in SCD patients as the RBC lifespan is 10-20days in SCD patient as compared to 90-120days in normal individuals. So normal individual can easily cope up with reduced production of RBC due to infection as it lasts only few days but in SCD patients, already there is stress on bone marrow to produce more reticulocytes due to increased turnover as result of reduced lifespan of RBC and now adding to it parvovirus B19 infection that reduces production even further to which system cannot cope up in SCD patients and they present with Cyanosis, Tachycardia and severe Fatigue.
Hemolytic Crisis :-
Due to reduced oxygen carrying capacity of RBC there is imbalance between oxygen that body receives and oxygen radicals produced this causes further stress on already stressed out RBC in SCD patients now patients with coexisting G6PD deficiency will contribute to further hemolysis and cause sudden reduction in Heamoglobin levels.
Acute Chest Syndrome
It one of the most common complication accounting for 25% of deaths of all the SCD patients, it is characterised by co-existence of any 2 of the following symptoms:
- Chest pain
- Pulmonary infiltrate
- Focal abnormality
- Respiratory symptoms
SCD patients are at risk of:-
- Reduced/ Abnormal immunity due to failure of functioning of spleen in SCD patients
- Avascular Necrosis of bones
- Infarction of penis, priapism
- Acute papillary necrosis of kidney
- Hematopoietic ulcers,Leg ulcers
- retinopathy, proliferative retinopathy, vitreous haemorrhage, retinal detachments
- Spontaneous abortion, pre-eclampsia, intrauterine growth retardation
- Sickle cell nephropathy causing CKD
- Pulmonary hypertension
- Cardiomyopathy, arrhythmias, LVDD left ventricular diastolic dysfunction, Dyspnoea on slightest exertion
- Pain crisis of Sickle Cell Anaemia
Homeopathic treatment for Sickle Cell Disease is completely dependent on how well and detailed the Homeopathic-case-taking has been done to derive exact constitutional details and individualise each patient as per totality as its a deep genetic disease a deep acting constitutional medicinal force is required to bring about gradual changes in constitution.
In case of SCD, indicated homeopathic similimum medicine should be selected strictly as per homeopathic principles of case-taking, individualisation and should be supported with proper repertorisation. Other than regular constitutional medicines patients may require intercurrent homeopathic theraputic treatment during phases of acute exacerbations.
Below acute/theraputic and constitutional medicine list commonly preferred in my practice( for reference purpose only). Not to self medicate in SCD case. Always treat under guidance of qualified and registered homeopathic practitioner.
- Cinchonna Officinalis
- Ruta Graveolens
- Ferrum Phosphoricum
- Crotalus Horridus
- Natrum Muriaticum
- Arsenic Album
- Arnica Montana
- Aspidosperma Quebracho
- Borrheavia Diffusa