World Sickle Cell Day | Symptoms and Emerging New Treatments

Approximately 70,000 to 100,000 Americans have sickle cell disease.  Though rare, it is the the most common form of inherited blood disorders. In Africa, the burden is much higher – Sub-Saharan Africa has an estimated 64% of the 300, 000 people born annually with sickle cell disease in the world,  Sickle Cell Day each June 19th provides an opportunity to examine progress and persistent challenges in managing the disease.

Sickle Cell, present in affected individuals at birth, causes the production of abnormal hemoglobin. Normal hemoglobin protein, which resides inside red blood cells, attaches to oxygen in the lungs and carries it to all parts of the body. Healthy red blood cells are flexible enough to move through the smallest blood vessels.

In sickle cell disease, the hemoglobin is abnormal, causing red blood cells to be rigid and shaped like a sickle – the shape from which the disease takes its name.  In order for a child to inherit sickle cell disease, both parents must have either sickle cell disease (two sickle cell genes) or sickle cell trait (one sickle cell gene). There are variations of sickle cell disease called sickle C or sickle thalassemia, which are serious conditions but sometimes less severe. If you have sickle cell disease, you will pass one sickle cell gene to your children.

Signs and symptoms of sickle cell disease usually begin in early childhood. Characteristic features of this disorder include a low number of red blood cells (anemia), repeated infections, and periodic episodes of pain. The severity of symptoms varies from person to person. Some people have mild symptoms, while others are frequently hospitalized for more serious complications.

Sickle cells can get stuck and block blood flow, causing pain and infections. Complications of sickle cell disease occur because the sickled cells block blood flow to specific organs. The worst complications include stroke, acute chest syndrome (a condition that lowers the level of oxygen in the blood), organ damage, other disabilities, and even premature death.

These signs and symptoms of sickle cell disease are caused by the “sickling” of red blood cells. When red blood cells sickle, they also break down prematurely, which can lead to anemia. Anemia can cause shortness of breath, fatigue, and delayed growth and development in children. The rapid breakdown of red blood cells may also cause yellowing of the eyes and skin, which are signs of jaundice.

Painful episodes can occur when sickled red blood cells- stiff and inflexible – get stuck in small blood vessels. These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially in the lungs, kidneys, spleen, and brain.

A particularly serious complication of sickle cell disease is high blood pressure in the blood vessels that supply the lungs (pulmonary hypertension). Pulmonary hypertension occurs in about one-third of adults with sickle cell disease and can lead to heart failure.

Mutations in the HBB gene cause sickle cell disease.

Hemoglobin consists of four protein subunits; typically, two alpha-globin subunits and two beta-globin subunits. The HBB gene provides instructions for making beta-globin. Variations of beta-globin result from different mutations in the HBB gene. One particular HBB gene mutation produces an abnormal version of beta-globin known as hemoglobin S (HbS). Other mutations in the HBB gene lead to additional abnormal versions of beta-globin, such as hemoglobin C (HbC) and hemoglobin E (HbE). HBB gene mutations can also result in an unusually low level of beta-globin; this abnormality is called beta thalassemia.

In people with sickle cell disease, at least one beta-globin subunit in hemoglobin is replaced with hemoglobin S. In sickle cell anemia, hemoglobin S replaces both beta-globin subunits in hemoglobin.

In other types of sickle cell disease, just one beta-globin subunit in hemoglobin is replaced with hemoglobin S.  The other beta-globin subunit is replaced with a different abnormal variant, such as hemoglobin C.  For example, people with sickle-hemoglobin C (HbSC) disease have hemoglobin molecules with hemoglobin S and hemoglobin C instead of beta-globin.  If mutations that produce hemoglobin S and beta thalassemia occur together, individuals have hemoglobin S-beta thalassemia (HbSBetaThal) disease.

Sickle Cell Trait

Sickle cell trait is an inherited blood disorder that affects approximately 8 percent of African-Americans. Unlike sickle cell disease, in which patients have two genes that cause the production of abnormal hemoglobin, individuals with sickle cell trait carry only one defective gene and typically live normal lives without health problems related to sickle cell. Rarely, extreme conditions such as severe dehydration and high-intensity physical activity can lead to serious health issues, including sudden death, in individuals with sickle cell trait.

Risk Factors

Sickle cell disease is more common in certain ethnic groups, including:

  • People of African descent, including African-Americans (among whom 1 in 12 carries a sickle cell gene)
  • Hispanic-Americans from Central and South America
  • People of Middle Eastern, Asian, Indian, and Mediterranean descent

Because sickle cell disease symptoms can begin by four months of age, early diagnosis is critical. All newborns in the United States are now tested for the disease. Sickle cell disease can also be identified before birth by testing a sample of amniotic fluid or tissue from the placenta. People who carry the sickle cell gene can seek genetic counseling before pregnancy to discuss options.

Signs and Symptoms

Signs and symptoms of sickle cell disease can be mild or severe enough to require frequent hospitalizations. They may include:

  • Anemia (looking pale)
  • Dark urine
  • Yellow eyes
  • Painful swelling of hands and feet
  • Frequent pain episodes
  • Stunted growth
  • Stroke

Treatment

There are no standard treatments that cure sickle cell disease. However, there are regiments that help people manage and live with the disease. Treatment relieves pain, prevents infections, minimizes organ damage, and controls complications. At times, blood transfusions and other advanced options are needed.

Clinical trials provide access to experimental therapies for treating sickle cell disease. The American Society of Hematology (ASH) provides information on clinical trials for which you may be eligible. Researchers are looking at new drugs and also exploring the use of bone marrow transplants to treat sickle cell disease. Stem cell transplants, associated with significant risks, are appropriate only for some patients with severe forms of sickle cell disease and closely matched donors –  typically family member.

Beyond this, the completion of the Human Genome Project and the use of CRISPR/Cas9 for gene editing have begun to transform the diagnosis and management of disease. Sickle cell disease has been considered a perfect model for genomic research because: 1) it is a monogenic disease and 2.) it has no cure despite the significant incidence of morbidity and mortality. Recent use of gene editing to minimize disease severity, and a single report of a patient who received successful treatment with gene therapy, highlight the potential for translating genome-based knowledge into health benefits for sickle cell patients.

It is important for you to talk with your doctor if you believe you may have sickle cell disease. If you carry the sickle cell trait, make sure you tell your doctor before getting pregnant as well. Depending on your condition, your doctor may refer you to a hematologist, a doctor who specializes in blood conditions.

Finding the right physician or medical care team just got that much easier.  To find a healthcare professional, use HealthLynked. It is a first of its kind medical network built as a social ecosystem with a higher purpose – improving healthcare. Go to HealthLynked.com to learn more, sign up for free, connect with your doctor, find a new doctor, and securely store and share your health information. Download our HealthLynked app available on Apple and Android devices.

6.5 Ways to Refocus on Health this Father’s Day

At HealthLynked, we believe every day is a great day to focus on wellness and remind those around you health is true wealth.  Today, Father’s Day, is another great opportunity to heighten the awareness of preventable health disorders and disease and encourage the men in your life to be more active and health conscious.

For Father’s Day, we serve up these gentle reminders of 6 ways you can refocus on your health, courtesy of the CDC:

  1. Get Enough Sleep

Aim for seven to nine hours per night.  Your mind and muscles need the recovery.

  1. Stop Smoking

If you quit now, you’ll lower your risk for cancer, COPD, and other smoking-related illnesses.

  1. Exercise More

Enjoy at least 2 ½ hours of aerobic activity, plus muscle-strengthening exercises, each week.

  1. Eat Healthy

Your diet should include a variety of well proportioned proteins and fruits and vegetables daily.

  1. Reduce Stress

You’ll feel much more relaxed if you avoid drugs and alcohol, connect socially, and find support.

  1. Get Regular Checkups

You need to know your blood pressure and cholesterol numbers; if they’re elevated, your risk for heart disease and stroke goes up…. High blood pressure may even increase your risk for erectile dysfunction!  You also need screening for colorectal and prostate cancer.  Across every spectrum of disease, positive outcomes are more likely with early detection.

The Truth

Men simply do not visit caregivers as often as they should. According to a CDC report, women are 100% more likely to visit a physician for preventive services and routine checkups.  The same report remarks men are 33% less likely to see their doctor for any reason, even when symptomatic.

The American Heart Association outlines the following 10 common excuses men give for not seeing a doctor:

“I don’t have a doctor.”

Step one toward staying healthy is finding a doctor you trust, and you’ll never know if you trust one unless you try. Check your insurance company and our healthcare ecosystem for one in your area. Call their offices and ask questions.

“I don’t have insurance.”

Everybody should still have insurance under the Affordable Care Act. If you don’t, there are plenty of resources available from state, local and charitable organizations to pay for your care.  Seek them out….

“There’s probably nothing wrong.”

You may be right, but Some serious diseases don’t have symptoms. High blood pressure is one, and it can cause heart attack and stroke. (That’s why they call it “the silent killer.”)  High cholesterol is another often-symptomless condition. Ditto diabetes. Finding a health problem early can make an enormous difference in the quality and length of your life.

“I don’t want to hear what I might be told.”

Maybe you smoke, drink too much, or have put on weight. Even so, your doctor’s there to help you. You can deny your reality, but you can’t deny the consequences. So be smart: Listen to someone who’ll tell you truths you need to hear. Be coachable.

“I don’t have time.”

There are about 8,766 hours in a year, and you want to save … two? When those two hours could save your life if you really DO need a doctor? If you want to spend more time with your family, these two hours aren’t the ones to lose.

“I don’t want to spend the money.”

It makes more sense to spend a little and save a lot than to save a little and spend a lot. If you think spending time with a doctor is expensive, try spending time in a hospital.

“Doctors don’t DO anything.”

When you see a barber, you get a haircut. When you see the dentist, your teeth get cleaned. But when you get a checkup, the doctor just gives you tests. It may seem like you don’t get anything, but you do. You get news and knowledge that can bring better health, if you act on it.

“I’ve got probe-a-phobia.”

You don’t need a prostate cancer exam until you’re 50. Even then, remember that your chances of survival are much better if it’s caught early. So, it’s worth the exam, and it’s only one small portion of a physical. Don’t let one test stop you from getting all the benefits of an annual physical.

“I’d rather tough it out.”

If pro athletes can play hurt and sacrifice themselves for the team, you ought to be able to suck it up, right? Wrong! The Game of Life is about staying healthy for a long time – a lifetime.

“My significant other has been nagging me to get a checkup. I’m a Rebel!”

OK, so you don’t want to give in, but isn’t it POSSIBLE you could be wrong? Give in on this one. See the doctor.

The risks of avoiding preventive care are real and can be devastating. According to the Centers for Disease Control and Prevention (CDC), the 10 leading killers of American men (regardless of age or ethnicity and in order) are:

Heart disease

Cancer

Unintentional injuries

Chronic lower respiratory diseases (such as COPD)

Stroke

Diabetes

Suicide

Alzheimer’s disease

Influenza and pneumonia

Chronic liver disease

Unlike the majority of women, who tend to seek medical care when even when they do not have symptoms, men often believe if you “feel fine,” there’s no reason to go to the doctor. It is important to note many can feel normal with high blood pressure, high cholesterol, or abnormal blood sugar levels. Even when men don’t feel so great, they tend to wait for symptoms to go away on their own — like when they drive around aimlessly because asking for directions admits weakness!

Be aware, you do not need a “one-size-fits-all” physical.  Collaborate with your healthcare provider, and depending on your profile and lifestyle, decide which screenings, diagnostic tests, and immunizations are right for you. The timing and frequency should be based on your risk factors for developing a condition or disease, including family or personal history, age, ethnicity, and environmental exposure.

In honor of Father’s Day, dads and those who care for them, go to the HealthLynked.com to find a physician you really connect with….Spend a few moments building a healthier life by collaborating with physicians who care, and begin building a health record for yourself that will help you set records for living a full life.

From all of us here at HealthLynked, consider our Free profiles a great gift for becoming the best version of you!  We all wish you the very best today and every day!

9 Potential Health Benefits of Vinegar Supported by Science

As an ancient folk remedy, Apple cider vinegar  has been used over centuries for various household chores, as a preservative and in cooking.  Today, it is the most popular vinegar in the natural health community, which promotes many of its benefits.  A few find some support in research, including weight loss, reduced cholesterol, lower blood sugar levels and improved symptoms of diabetes.

Below are 9 health benefits of apple cider vinegar supported by scientific study.

1. Boasts Potent Biological Effects

Apple cider vinegar is made in a two-step process related to how alcohol is made.  In French, the word “vinegar” actually means “sour wine.”  The first step exposes crushed apples (or apple cider) to yeast, which ferment the sugars and turn them into alcohol.  In the second step, bacteria are added to the alcohol solution, further fermenting the alcohol turning it into acetic acid — the main active compound in vinegar.

9 Potential Health Benefits of Vinegar Supported by Science

Organic, unfiltered apple cider vinegar (like Bragg’s) also contains “mother” – strands of proteins, enzymes and friendly bacteria that give the product a murky appearance.  Some people believe the “mother” is responsible for most of the health benefits, although there are few published studies to support this.

Apple cider vinegar only contains about three calories per tablespoon, which is very low.  There are not many vitamins or minerals in it, but it does contain a small amount of potassium. Quality apple cider vinegar also contains some amino acids and antioxidants.

2. Kills Many Types of Harmful Bacteria

Vinegar can help kill pathogens, including bacteria.  It has traditionally been used for cleaning and disinfecting, treating nail fungus, lice, warts and ear infections.

Hippocrates, the father of modern medicine, used vinegar for wound cleaning over two thousand years ago.  Vinegar has also been used as a food preservative, and studies show that it inhibits bacteria (like E. coli) from growing in food and spoiling it.  If you’re looking for a natural way to preserve your food, apple cider vinegar could be highly useful.

There have also been anecdotal reports of diluted apple cider vinegar helping with acne when applied to the skin, but there is not strong research to confirm this.  The main substance in vinegar, acetic acid, can kill harmful bacteria or prevent them from multiplying. It has a history of use as a disinfectant and natural preservative.

3. Lowers Blood Sugar Levels and Fights Diabetes

By far, the most successful application of vinegar to date is in patients with type 2 diabetes.  Type 2 diabetes is characterized by high blood sugar levels, either because of insulin resistance or an inability to produce insulin.  Also, high blood sugar can also be a problem in people who don’t have diabetes. It is believed to be a major cause of aging and various chronic diseases.

Pretty much everyone should benefit from keeping their blood sugar levels in the normal range. The most effective (and healthiest) way to do that is to avoid refined carbs and sugar, but apple cider vinegar can also have a powerful effect.

Vinegar has been shown to have numerous benefits for blood sugar and insulin levels:

  • Improves insulin sensitivity during a high-carb meal by 19–34% and significantly lowers blood sugar and insulin responses.
  • Reduces blood sugar by 34% after eating 50 grams of white bread.
  • 2 tablespoons of apple cider vinegar before bedtime can reduce fasting blood sugar in the morning by 4%.
  • Numerous other studies in humans show that vinegar can improve insulin function and lower blood sugar levels after meals.9 Potential Health Benefits of Vinegar Supported by Science

Apple cider vinegar may also increase insulin sensitivity. Insulin is the hormone responsible for transporting sugar from the blood to the tissues where it can be used as fuel. Sustaining elevated levels of insulin can cause insulin resistance, which reduces its effectiveness and leads to high blood sugar and diabetes.  A study in Diabetes Care showed that vinegar ingestion helped significantly improve insulin sensitivity by up to 34 percent in those with either type 2 diabetes or insulin resistance. (1)

To keep blood sugar levels stable, try diluting one to two tablespoons of apple cider vinegar in 8 ounces of water and consuming before meals. Additionally, be sure to moderate carbohydrate intake, increase your consumption of fiber and protein foods and get in plenty of regular physical activity to drop blood sugar levels even more.

For these reasons, vinegar can be useful for people with diabetes, pre-diabetes, or those who want to keep their blood sugar levels low for other reasons.  If you’re currently taking blood-sugar-lowering medications, check with your doctor before increasing your intake of any type of vinegar.

4. Helps You Lose Weight and Reduces Belly Fat

Surprisingly, studies also show that vinegar can help you lose weight.  Several human studies demonstrate vinegar can increase satiety, help you eat fewer calories and lead to actual pounds lost on the scale.  For example, when people take vinegar along with a high-carb meal, they get increased feelings of fullness and end up eating 200–275 fewer calories for the rest of the day.

9 Potential Health Benefits of Vinegar Supported by Science

A study in 175 people with obesity showed that daily apple cider vinegar consumption led to reduced belly fat and weight loss:

  • 15 mL (1 tablespoon): Lost 2.6 pounds, or 1.2 kilograms.
  • 30 mL (2 tablespoons): Lost 3.7 pounds, or 1.7 kilograms.

However, keep in mind that this study went on for 3 months, so the true effects on body weight seem to be rather modest.  Just adding or subtracting single foods or ingredients rarely has a noticeable effect on weight.  It’s an entire diet and improved lifestyle that matters;  you may need to combine several effective changes to see results.

Overall, it seems like apple cider vinegar is useful as a weight loss aid, mainly by promoting satiety and lowering blood sugar and insulin levels.  While it won’t work any miracles on its own, studies suggest vinegar can increase feelings of fullness and help people eat fewer calories, which leads to weight loss.

5. Lowers Cholesterol and Improves Heart Health

Cholesterol is a fat-like substance that can build up in the arteries, causing them to narrow and harden. High blood cholesterol puts a strain on your heart, forcing it to work harder to push blood throughout the body.

Heart disease is currently the world’s most common cause of premature death.  It is known that several biological factors are linked to either a decreased or increased risk of heart disease.  Several of these “risk factors” may be improved by vinegar consumption, but many of the supporting studies were done in animals.

These animal studies suggest that apple cider vinegar can lower cholesterol and triglyceride levels, along with several other heart disease risk factors.  There are also some studies showing that vinegar reduces blood pressure in rats, which is a major risk factor for heart disease and kidney problems.

The only human evidence so far is an observational study from Harvard showing women who ate salad dressings with vinegar had a reduced risk of heart disease.  However, this type of study can only show an association – it cannot prove that the vinegar caused anything.

As mentioned above, human studies also show that apple cider vinegar can lower blood sugar levels, improve insulin sensitivity and help fight diabetes. These factors should also lead to reduced risk of heart disease.

Besides including a tablespoon or two of apple cider vinegar in your diet each day, other ways to lower cholesterol fast include minimizing your intake of sugar and refined carbohydrates, including a good variety of healthy fats in your diet and eating a few servings of fish per week.

6. May Have Protective Effects Against Cancer

Cancer is a terrible disease characterized by the uncontrolled growth of cells.  There is a lot of hype online about the anti-cancer effects of apple cider vinegar.  In fact, numerous studies have shown that various types of vinegar can kill cancer cells and shrink tumors.  However, all of the studies on this were done in isolated cells in test tubes, or rats, which proves nothing about what happens in a living, breathing human.

That said, some observational studies in humans have shown that vinegar consumption is linked to decreased esophageal cancer in China, but increased bladder cancer in Serbia.  It is possible that consuming apple cider vinegar may help prevent cancer, but this needs to be researched more before any recommendations can be made.

7.  Improves Skin Health

Apple cider vinegar doesn’t just benefit your internal health; it has also been shown to treat acne and reduce scarring. Certain strains of bacteria often contribute to the development of acne. Vinegar is well-known for its antibacterial properties and has been shown to be effective against many strains of harmful bacteria.

Apple cider vinegar also contains specific components like acetic acid, lactic acid, succinic acid and citric acid, all of which have been shown to inhibit the growth of Propionibacterium acnes, the specific strain of bacteria responsible for causing acne.

These beneficial components may also reduce scarring. A study in the Journal of Cosmetic Dermatology showed that treating acne scars with lactic acid for three months led to improvements in the texture, pigmentation and appearance of treated skin as well as a lightening of scars.  Adding some probiotic foods into your diet, using healing masks and toners and keeping your skin well-moisturized are some other effective home remedies for acne as well.

8.  Reduces Blood Pressure

High blood pressure is a major risk factor for heart disease. It forces the heart to work harder, causing the heart muscle to weaken and deteriorate over time.

Apple cider vinegar benefits your blood pressure levels, helping to keep your heart healthy and strong. An animal study in Japan showed that giving rats acetic acid, the main component in vinegar, resulted in reduced levels of blood pressure. Another animal study had similar findings, demonstrating that vinegar effectively blocked the actions of a specific enzyme that raises blood pressure.

Other natural ways to lower blood pressure include increasing your intake of magnesium and potassium, upping your fiber intake and swapping the salt and processed foods for whole foods.

9. Relieves Symptoms of Acid Reflux

Gastroesophageal reflux disease, also known as GERD or acid reflux, is a condition characterized by acid backflow from the stomach up into the esophagus, causing symptoms like heartburn, belching and nausea.  Acid reflux is often a result of having low levels of stomach acid. If this is the case for you, drinking apple cider vinegar may help provide relief from acid reflux symptoms by introducing more acid into the digestive tract to prevent acid backflow.

For best results, dilute one to two tablespoons of apple cider vinegar in an 8-ounce glass of water and drink just before eating. Additionally, remember to follow an acid reflux diet rich in vegetables, healthy fats and fermented foods to slash symptoms even more.

Dosage and How to Use

The best way to incorporate apple cider vinegar into your diet is to use it in your cooking — for salad dressings, homemade mayonnaise and that sort of thing.

Some people also like to dilute it in water and drink it as a beverage. Common dosages range from 1–2 teaspoons (5–10 ml) to 1–2 tablespoons (15–30 ml) per day, mixed in a large glass of water.

It’s best to start with small doses and avoid taking large amounts – too much vinegar may have harmful side effects.  It is recommended to use organic, unfiltered apple cider vinegar with the “mother.”

The Bottom Line

There are a number of likely over-inflated claims about apple cider vinegar.  Some say it can increase energy levels and deliver all sorts of beneficial effects on health.  Unfortunately, many of these claims are not supported by science.  Of course, absence of proof does not invalidate that something is happening, and anecdotes often end up becoming supported by science down the line.

Apple cider vinegar also has various other non-health related uses like hair conditioning, deoderant, dental care, pet use and as a cleaning agent (to name a few).  These can be highly useful for people who like to keep things as natural and chemical-free as possible.

From the little evidence available, it appears apple cider vinegar may be useful and is definitely a viable candidate for some experimentation if you’re interested and your physician agrees.  At the very least, apple cider vinegar seems to be safe as long as you don’t go overboard and take excessive amounts.

Precautions

While apple cider vinegar appears to be very healthy, it is not a “miracle” or a “cure-all” like so many seem to believe.  It does clearly have some important health benefits, especially in lowering blood sugar and aiding in weight control.  Still, it should not be viewed as a quick fix when it comes to your health.  Instead, it should be paired with a nutritious diet and healthy lifestyle for best possible results.

Although apple cider vinegar is healthy and safe for most people, consuming large amounts may lead to some negative effects on health. Apple cider vinegar side effects include erosion of tooth enamel, burning of the throat or skin and decreased levels of potassium.

Be sure to always dilute apple cider vinegar in water instead of drinking it straight to prevent negative side effects. You should also start with a low dose and work your way up to assess your tolerance, and always consult with a physician.

If you’re taking blood sugar medications, talk to your doctor before using apple cider vinegar. Because apple cider vinegar may help reduce blood sugar levels, you may need to modify your dosage of diabetes medications to prevent hypoglycemia symptoms.

To find a healthcare professional, use HealthLynked. It is a first of its kind medical network built as a social ecosystem with a higher purpose – improving healthcare. Go to HealthLynked.com to learn more, sign up for free, connect with your doctor, find a new doctor, and securely store and share your health information. Download our HealthLynked app available on Apple and Android devices.

Sources:

  1. Johnston CS, et al. Vinegar improves insulin sensitivity to a high-carbohydrate meal in subjects with insulin resistance or type 2 diabetes.  Diabetes Care. 2004 Jan;27(1):281-2.
  2. MedLine