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.

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.

N-Acetylcysteine antioxidant
Annals Hematology. 2012. N-acetylcysteine reduces oxidative stress in sickle cell patients. Department of Internal Medicine, Slotervaart Hospital, Amsterdam, The Netherlands. Oxidative stress is of importance in the pathophysiology of sickle cell disease (SCD). In this open label randomized pilot study the effects of oral N-acetylcysteine (NAC) on phosphatidylserine (PS) expression as marker of cellular oxidative damage (primary end point), and markers of hemolysis, coagulation and endothelial activation and NAC tolerability (secondary end points) were studied. Eleven consecutive patients (ten homozygous [HbSS] sickle cell patients, one HbSβ(0)-thalassemia patient) were randomly assigned to treatment with either 1,200 or 2,400 mg NAC daily during 6 weeks. The data indicate an increment in whole blood glutathione levels and a decrease in erythrocyte outer membrane phosphatidylserine exposure, plasma levels of advanced glycation end-products (AGEs) and cell-free hemoglobin after 6 weeks of NAC treatment in both dose groups. One patient did not tolerate the 2,400 mg dose and continued with the 1,200 mg dose. During the study period, none of the patients experienced painful crises or other significant SCD or NAC related complications. These data indicate that N-acetylcysteine treatment of sickle cell patients may reduce SCD related oxidative stress.

Fish oils or omega-3 fatty acids
Background: Blood cell aggregation and adherence to vascular endothelium and inflammation play a central role in vaso-occlusive crisis in sickle cell disease. The antiaggregatory, antiadhesive, antiinflammatory, and vasodilatory omega-3 (n−3) fatty acids (DHA and EPA) are significantly reduced in patients with the disease. One hundred forty patients recruited from a single center in Sudan were randomly assigned and received, daily, 1 (age 2–4 y), 2 (age 5–10 y), 3 (age 11–16 y), or 4 (age ≥17 y) omega-3 capsules containing 277 mg DHA and 39 mg EPA or placebo for 1 y. Omega-3 treatment reduced the median rate of clinical vaso-occlusive events, severe anemia, blood transfusion, white blood cell count, and the inability to attend school at least once during the study period because of illness related to the disease: The findings of this trial suggest that omega-3 fatty acids can be an effective, safe, and affordable therapy for sickle cell anemia. Effect of omega-3 (n−3) fatty acid supplementation in patients with sickle cell anemia: randomized, double-blind, placebo-controlled trial. 2013 American Society for Nutrition.

Prostaglandins Leukot Essent Fatty Acids. 2013. Docosahexaenoic and eicosapentaenoic acid supplementation does not exacerbate oxidative stress or intravascular haemolysis in homozygous sickle cell patients.

Blood Cells Mol Dis. 2015. Omega 3 (n-3) fatty acids down-regulate nuclear factor-kappa B (NF-κB) gene and blood cell adhesion molecule expression in patients with homozygous sickle cell disease. Chronic inflammation and reduced blood levels of omega-3 fatty acids (n-3) are known characteristics of sickle cell disease (SCD). The anti-inflammatory properties of n-3 fatty acids are well recognized. Omega-3 treated, hydroxyurea (HU) treated, and n-3 untreated homozygous SCD patients (HbSS) and healthy (HbAA) controls matched for age (5-16 years), gender and socioeconomic status were studied. According to age (5-10) or (11-16) years, two or three capsules containing 278 mg docosahexaenoic (DHA) and 39 mg eicosapentaenoic (EPA) or high oleic acid placebo (41%) were assigned to n-3 treated and n-3 untreated groups, respectively. Hydroxyurea treated group was on dosage more than 20 mg/kg/day. The n-3 treated group had higher levels of DHA and EPA and lower white blood cell count and monocyte integrin compared with the n-3 untreated. No difference was detected between the two groups regarding C-reactive protein, granulocytes integrin and selectin, plasma tumour necrosis factor-α and interleukin-10. The n-3 treated group had lowered nuclear factor-kappa B (NF-κB) gene expression compared to n-3 untreated and HU treated groups. This study provides evidence that supplementation with n-3 fatty acids may ameliorate inflammation and blood cell adhesion in patients with SCD.

Glutamine amino acid
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 to 17 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. 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, University of Kinshasa, Kinshasa XI, D.R. Congo.

Vitamin D supplementation
J Pediatr Hematol Oncol. 2015. Safety and Efficacy of High-dose Daily Vitamin D3 Supplementation in Children and Young Adults With Sickle Cell Disease. Suboptimal vitamin D (vit D) status (<32 ng/mL) is ubiquitous among African American children with type SS sickle cell disease (SCD-SS). Five to 20-year-old African American children with and without SCD-SS were randomized to vit D3 supplementation (4000 or 7000 IU/d) and evaluated at 6 and 12 weeks for changes in vit D and SCD status. At baseline 95% of subjects with SCD-SS and 87% of healthy controls had suboptimal vit D status . After 12 weeks supplementation, both D3 doses were safe and well tolerated. For subjects with SCD-SS, by 12 weeks there was a significant increase in fetal hemoglobin, decrease in high-sensitivity C-reactive protein, and reduction in the percentage of subjects with a high platelet count.

Herbal treatment
Clin Hemorheol Microcirc. 2015. Pfaffia Paniculata extract improves red blood cell deformability in sickle cell patients. Suma is another name for this herb.

Physical activity, exercise
Br J Sports Med. 2015. Does physical activity increase or decrease the risk of sickle cell disease complications? Sickle cell disease (SCD) is the most common inherited disease in the world. Red blood cell sickling, blood cell-endothelium adhesion, blood rheology abnormalities, intravascular haemolysis, and increased oxidative stress and inflammation contribute to the pathophysiology of SCD. Because acute intense exercise may alter these pathophysiological mechanisms, physical activity is usually contra-indicated in patients with SCD. However, recent studies in sickle-cell trait carriers and in a SCD mice model show that regular physical activity could decrease oxidative stress and inflammation, limit blood rheology alterations and increase nitric oxide metabolism. Therefore, supervised habitual physical activity may benefit patients with SCD.

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 Trigger
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.