is a genetic condition that affects the hemoglobin in
red blood cells. Hemoglobin is a pigment which gives the blood its red color.
It picks up oxygen from the lungs and carries it to various parts of the body,
and also carries carbon dioxide in the reverse direction.
‘L-glutamine has recently received FDA approval for the treatment of sickle cell disease.’
with sickle cell disease have abnormal hemoglobin
. If the oxygen levels
are low, the disc-shaped red blood cells assume a sickle shaped. The shape does
not allow them to pass through blood vessels easily and they can therefore
obstruct the flow of blood to various organs. Patients suffer from frequent
severe painful episodes, which often require painkiller injections. The cells
also have a tendency to break, as a result of which anemia ensues. Currently,
hematopoietic stem cell transplant is the definitive treatment for sickle cell
disease, though it may be beyond the reach of several patients.
medication called hydroxyurea is used for the treatment of sickle
. It increases the levels of a type of hemoglobin called
fetal hemoglobin, and therefore reduces the complications of sickle cell
Studies found that the
red blood cells affected by sickle cell disease are under high oxidative stress
and try to protect themselves by producing a chemical called nicotinamide
adenine dinucleotide (NAD) molecules. The
newly approved drug, l-glutamine, is an amino acid required to produce NAD
molecules, and can therefore protect the red blood cells.
can be taken as an oral powder by sickle cell disease patients as young as 5
years of age.
The approval comes following the results of a trial, which
compared the use of L-glutamine to a placebo taken for a duration of 48 weeks.
The participants of the trial had previously suffered at least two attacks of
painful crises in the previous year. The patients who took the medication had:
- Fewer visits to
the hospital for painkiller injections for painful episodes
- Reduced duration
- Fewer incidences
of acute chest syndrome. Acute chest syndrome is a condition where
obstruction to blood flow to the lungs can damage the lungs
The side effects of
l-glutamine noted in the study included constipation, nausea, headache,
abdominal pain, cough, pain in the extremities, back pain and chest pain.
The new drug will
hopefully be a boon to several patients affected by sickle cell disease.
- FDA approves new treatment for sickle cell disease - (https://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm566084.htm)
- What is Sickle Cell Disease - (https://www.nhlbi.nih.gov/health/health-topics/topics/sca)
- Niihara Y, Macan H, Eckman JR, Koh H, Cooper ML, et al. (2014) L-Glutamine Therapy Reduces Hospitalization for Sickle Cell Anemia and Sickle β°-Thalassemia Patients at Six Months - A Phase II Randomized Trial. Clin Pharmacol Biopharm 3:116. doi:10.4172/2167-065X.1000116