Sickle cell disease is common in India. The prevalence is higher in the tribal belt of Central, Western and Southern India. Sickle cell disease is rampant in Vidarbha
Acute painful episodes or vaso-occlusive crises are the hallmarks of sickle cell disease (SCD). They are also the most common reasons for hospitalizations in them. Opioids have been used to manage pain, but there are concerns about addictions, adverse effects and tolerance with their long-term use. The association of nephropathy with SCD precludes the use of NSAIDs in these patients. Various non-pharmacological approaches have also been employed for pain relief such as cognitive behavioural therapy, biofeedback, relaxation techniques etc.
Other therapeutic options that target the pathophysiological processes such as hydroxyurea, pharmaceutical-grade L-glutamine, RBC transfusions and hematopoietic cell transplantations are being used to prevent pain. Only hydroxyurea and pharmaceutical-grade L-glutamine are US Food and Drug Administration approved to prevent pain episodes in SCD.
A new multicenter, randomized, placebo-controlled, double-blind, phase 3 trial in patients with SCD who had at least two pain events in the prior year found that compared with placebo, pharmaceutical grade L-glutamine, administered twice daily by mouth in dose of 0.3 g/kg/dose, alone or with hydroxyurea, reduced acute pain events, acute hospitalizations and length of hospital stay.
The observed beneficial effects of glutamine (vs placebo) in the trial were modest: 3 vs 4 pain episodes in a year, 2 vs 3 hospitalizations for pain, respectively independent of hydroxyurea use.
Based on the results of this trial, L-glutamine has been suggested for individuals >5 years of age who have persistent pain despite hydroxyurea, those who are intolerant to hydroxyurea. Although, no significant adverse effects were reported, taking glutamine requires mixing powder with food or beverages twice a day.
However, when given L-glutamine as a therapeutic option, the patient should be explained about the benefits that are only modest as the patient may then choose not to take L-glutamine if he/she finds that the cost of treatment is too high or the burden of taking the drug is prohibitive.
A decision to start L-glutamine should only be taken together with the patient.
1. Niihara Y, Miller ST, Kanter J, et al; Investigators of the Phase 3 Trial of l-Glutamine in Sickle Cell Disease. A phase 3 trial of l-glutamine in sickle cell disease. N Engl J Med. 2018;379(3):226-235.
Dr KK Aggarwal
Padma Shri Awardee
President Elect Confederation of Medical Associations in Asia and Oceania (CMAAO)
Group Editor-in-Chief IJCP Publications
President Heart Care Foundation of India
Immediate Past National President IMA