Sickle-cell disease is a genetic disorder that causes normally flexible red blood cells to contort into a crescent-moon shape that makes them clump, blocking blood vessels and causing most patients bouts of intense pain. Normally, red blood cells, which carry oxygen throughout the body, are round and smooth. In sickle cell anemia, the red blood cells are sickle-shaped, which causes them to get caught up in smaller blood vessels, sometimes blocking blood flow. The complications of sickle cell anemia are numerous and include pain, infections, gallstones, leg ulcers, priapism in males (sustained and painful erections), pulmonary hypertension and stroke.
Sickle cell anemia prevalence
About 1 in 650 African-Americans and up to 1 in 1,000 Latinos in the United
States has some form of sickle cell disease including sickle cell
anemia. Sickle cell anemia
affects about 72,000 Americans, according to the National Heart, Lung, and Blood
Institute (NHLBI). Those most at risk of the disease are blacks and people of
Mediterranean and Middle Eastern descent.
Natural therapy for sickle cell disease
research study
Discuss with your doctor before starting any supplement
program.
Oral Glutamine supplementation decreases resting energy expenditure in children
and adolescents with sickle cell anemia.
J Pediatr Hematol Oncol. 2004
To determine the effects of orally administered glutamine on the
resting energy expenditure (REE) and nutritional status of children and
adolescents with sickle cell anemia. Twenty-seven children and
adolescents (13 boys, 14 girls), 5.2 to 17.9 years old,
received orally administered glutamine (600 mg/kg per day) for 24 weeks.
Measures of REE and other nutritional parameters were compared at baseline and
24 weeks. After 24 weeks, the patients' median REE (kcal/d) decreased
by 6%. Patients with less than 90% ideal
body weight had even greater declines in REE after 24 weeks. Improvements in nutrition parameters and in two amino acids in
the plasma were observed. After 24 weeks of orally administered
glutamine, children and adolescents with sickle cell anemia had a decrease in REE and improvement in nutritional parameters. Those who were underweight had a
greater decrease in REE than those of normal body weight. Lowering REE may be an
effective way to improve the growth of these children and adolescents.
Anthocyanins from Justicia secunda (anthocyanins are present abundantly in most berries) were found to possess anti-sickling activity. Our findings suggest that anthocyanin extracts play a role in both stabilising the red blood cell membrane and inhibiting polymerisation of haemoglobin S. This provides a possible molecular basis for earlier reports on the anti-sickling properties of anthocyanins from some Congolese plants and their use in the management of sickle cell disease by Congolese traditional healers. Blood Transfus. 2010 Oct. In vitro effects of anthocyanin extracts from Justicia secunda Vahl on the solubility of haemoglobin S and membrane stability of sickle erythrocytes. Department of Chemistry, Faculty of Sciences B.P. 190, University of Kinshasa, Kinshasa XI, D.R. Congo.
Blood
Transfusions
Some patients with severe sickle cell disease may need a lifetime of
blood transfusions to reduce the chances of suffering a stroke. A study, published in
December 2005 New
England Journal of Medicine, found that the stroke risk reappeared after blood
exchanges were stopped.
Regular transfusions of red blood cells typically cut the stroke risk by 90
percent.
Priapism and sickle cell disease
Priapism, an unwanted painful erection of the penis, is a common
complication of sickle cell disease. What is known about the prevalence of
priapism, efficacy of management approaches, and outcome is drawn primarily from
retrospective and single-center reports. Priapism occurs in two patterns:
prolonged and stuttering (ie, recurrent brief episodes that resolve
spontaneously). If priapism persists for 4 hours or more without detumescence,
the patient is at risk for irreversible ischemic penile injury, which may
terminate in fibrosis and impotence.
Lactate dehydrogenase as
biomarker
Lactate dehydrogenase levels are a biomarker for a subphenotype of sickle
cell disease that includes pulmonary hypertension, leg ulcerations, priapism,
and higher mortality risk.The lactate dehydrogenase level can be a convenient
risk marker for clinicians to use in patients with sickle cell disease.
Sickle cell attack tTrigger
Asthma is a risk factor associated with painful events in children with
sickle cell disease. A sickle cell attack can occur a day or two after an asthma
attack.
Bone marrow transplant treatment
Bone marrow transplants, already used to treat some children with sickle cell
disease, also may cure some adults with this deadly genetic defect that causes
red blood cells to contort. Nine of 10 adult patients given an experimental bone
marrow transplant treatment were cured of sickle cell disease, researchers at
the U.S. government's National Institutes of Health reported. If the early
results hold, the treatment "could be ideal for patients with severe sickle cell
disease," Dr. Miguel Abboud of American University of Beirut Medical Center in
Lebanon said in an editorial accompanying the study. Such transplants already
are used to cure children with the disease who have a compatible donor who
provides bone marrow. Bone marrow gives rise to blood cells. New England Journal
of Medicine, December 2009.
Sickle cell blood test
A commonly ordered blood test may help doctors predict which sickle cell
patients might develop serious complications, such as pulmonary hypertension and
leg ulcers, and who's at risk for early death. When the
enzyme lactate
dehydrogenase (LDH) is present in high levels in the blood of someone with
sickle cell anemia, the risk of pulmonary hypertension (high blood pressure in
the lungs) is quadrupled compared to someone with low levels of LDH.
LDH is an enzyme found in many cells throughout the
body. Red blood cells, the heart, the kidneys, the liver and muscles are
especially rich in LDH. Usually, the upper limit of LDH in healthy adults is 200
international units per liter (IU/L). However, when organs become diseased, LDH
is released in increasing amounts. Low LDH levels in sickle cell patients
conferr the lowest risk of developing leg ulcers, priapism and pulmonary
hypertension.
Questions
Q. I was doing some reading on L Arginine and found your site. I remember
reading a number of years ago about nitric oxide being tested on sickle cell
patients. After reading the effects of arginine; I wonder if supplementation
would be advantageous for this population of folks. If too short acting for day
to day use; could higher dosing help ease pain during a crisis? There should be
a study.
A. I have no idea if arginine would help patients with sickle cell
anemia.
Q. I have tried using Passion Rx and Stamina Rx and
Hersolution with no success. So I have stop buying any female libido enhancement
pills. While Passion Rx I think really will work, these pills make my head feels heavy and then I have some dizziness. Once I stop
taking the pills I feel okay again. What can I possibly take that is all natural
that can help me. I suffer with very low libido or should I say none at all. I
am a 36 year old female and I am not sure if this maybe the reason why the pills
make me sick or not but I have sickle cell anemia. Is there anything that can
work for me?
A. I am not sure how Passion Rx and Stamina Rx and Hersolution
formulas affect people with sickle cell disease or other blood cell conditions.
if you do try sexual herbs again, we suggest using a half a capsule to
avoid side effects and to use them two days on, one day off. Using lower dosages
reduces side effects and may actually work better. Please have approval by your
doctor.