Updated: Apr 7
Thus, the novelty and uniqueness of the present invention rest on providing a prophylactic or preventive method of sickle cell anemia control based on the demonstration of anti-sickling activity of potassium thiocyanate in certain dose dependent amounts
Intravenous use of a thiocyanate solution, as a sterile physiologically suitable, pyrogen-free sodium thiocyanate solution in amounts from 20 mM to about 50 mM would also appear to inhibit sickling of the red blood cells.
Further, in the practice of the invention, red blood cells can be withdrawn from the patient and treated with the thiocyanate solution prior to replacement into the patient. In this procedure of replacement therapy, a portion of the patient's blood is drawn and placed into a vessel containing acid-citrate-dextrose (ACD). The blood is then centrifuged and the plasma is returned to the patient. The red blood cells are incubated with an aqueous solution of the thiocyanate, preferably, potassium thiocyanate (20 mM-50 mM) at about 37 degrees Centigrade for at least an hour. The cells are then washed to remove excess thiocyanate and then re-introduced into the patient.
Studies1,2 have shown that thiocyanates and their dietary precursors, i.e. beta-cyanogenetic glucosides (nitrilosides) are non-toxic and safe for humans, and when these studies are factored into the orbit of the present invention, the evidence suggests that the anti-sickling effect of the thiocyanate observed in vitro corresponds directly to the use of these compounds in vivo, both upon deoxygenated and oxygenated red blood sickle cells (Hbss).
The routes of administration of the thiocyanate, or thiocyanate-vitamin B6 admixture will mainly include the oral and intravenous modes; however, other modes can also be used. For the prophylactic approach, it has been found that the oral route is the most convenient. For therapeutic control of sickle cell crisis, the oral or the intravenous route could be used. The purpose of the administration is to bring the thiocyanate, or thiocyanate and vitamin B6 in contact with the blood stream. Here, the thiocyanate permeates the erythrocyte membrane, binds to hemoglobin and prevents sickling, and vitamin B6 stimulates hemoglobin biosynthesis.
It has been established that vitamin B6 is essential for hemoglobin biosynthesis, and it is known that vitamin B6 is the coenzyme of delta-aminolevulinic acid (ALA) synthetase, the first enzyme in hemoglobin formation in humans, see Hindmarsh, Clin. Chem. 32 (7): 1255-1263, (1986). Therefore, when it is also desirable to prevent depression of hemoglobin biosynthesis arising from Vitamin b6 deficiency, best results are achieved when vitamin B6 is used in combination with potassium thiocyanate. In this way, the thiocyanate-vitamin B6 mode of administration would ensure optimal hemoglobin synthesis and effective prevention of anemia in sickle cell homozygotes.
Thiocyanate and thiocyanate admixed with vitamin B6 can be prepared for administration in various forms, such as tablets, solutions, granules, syrups, suppository and other forms known to the drug making technology. In this respect, thiocyanate and pyridoxine hydrochloride (vitamin B6) are both water soluble. Employing the methods of this invention, it was discovered that thiocyanate and vitamin B6 are not antagonistic to each other.
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The article below is a feature our family put together to bring more awareness to the struggles of the sickle cell community and how the broader collective community can be more supportive of our efforts towards healing.