Archive | March 2021

QUOTE FOR WEDNESDAY:

“The link between obesity and cancer risk is clear. Research shows that excess body fat increases your risk for several cancers, including colorectal, post-menopausal breast, uterine, esophageal, kidney and pancreatic cancers.

What’s less clear is exactly how being obese increases that risk. Experts believe it’s largely due to the inflammation caused by visceral fat – the fat that surrounds your vital organs.

“The problem with excessive visceral fat is that it affects certain processes in your body. This includes how your body manages hormones, like insulin and estrogen,” says Karen Basen-Engquist, Ph.D., professor in Behavioral Science at MD Anderson.

“All of this can lead to an increased cancer risk by affecting how and when cells divide and die,” she says.

M.D. Anderson Cancer Center https://www.mdanderson.org/

Supporting studies on the link between Obesity and Cancer.

LOOKING AT ACTUAL STUDIES SUPPORTING OBESITY&CANCER LINK

Take for example through the American Cancer Society they stated in an article 2006 that a substantial evidence from clinical trials has established that obesity significantly increases the risk for heart disease and diabetes.  More recently, suspicions that obesity is linked to prostate cancer have been supported by a number of investigations, but the relationship has remained unclear.  Now through a pair of new studies provides scientists with some important insights that may have an impact on how physicians manage their patients with prostate cancer.

The pair of studies show 2 significant studies relating how obesity links with prostate cancer, which were:

The implication is that prostate cancer patients who are obese should probably be followed more closely than patients with similar cancer characteristics who are not obese. That could include regular digital rectal exams, more frequent prostate-specific antigen (PSA) testing, and perhaps setting a lower PSA cutoff point as an indication of recurrence, Kane explained.

The greater risk associated with obesity may be related to technical issues, Kane said. For example, it’s more difficult for surgeons to perform a radical prostatectomy in obese patients. However, surgical challenges offer only a partial explanation. In studies where surgeons verified that they had removed all cancer cells during radical prostatectomy, obese patients were still more likely to experience a recurrence of the disease.

The second study, a multi-center trial coordinated at the Duke University School of Medicine in Durham, NC, found that obese men under the age of 63 tend to have larger prostates, which makes finding tumors more difficult. As a result, there’s a real danger of delayed diagnosis, which decreases the chances of a cure and puts patients at greater risk for dying of the disease. The findings appear in the Journal of Urology.

“It’s harder to find cancer in larger prostate glands,” explained Stephen J. Freedland, MD, assistant professor of urology and member of the Duke Prostate Center at the Duke University School of Medicine. “Consequently, our data suggest that we may be underdiagnosing cancers in younger obese men. That also means that the tumors we do pick up are likely to be at a more advanced stage and perhaps more aggressive, and therefore more difficult to treat.”

Let’s look at this at a broader aspect, not just pertaining to prostate cancer.  This would be, “It’s not just patients with prostate cancer that studies like this should be directed toward,” Dr. Kane said. “Patients who are clearly at risk for developing the disease should also take notice of our findings. The central message is yet again that obesity has been identified as an important risk factor for a potentially deadly disease. For all of us, controlling our weight through diet and exercise is important, and we shouldn’t have blinders on and just think of prostate cancer. The number one risk of death for American men is heart disease, so anything we can do to reduce that risk that also reduces the risk for prostate cancer is useful.”  Including it reduces risk for other cancers as well that are impacted by disease, it makes sense.

Obesity links to an increased risk of ovarian cancer.

 Let’s look at this no: Atlanta 2009/01/05 -A new epidemiological study has found that among women who have never used menopausal hormone therapy, obese women are at an increased risk of developing ovarian cancer compared with women of normal weight. Published in the February 15, 2009 issue of CANCER, a peer-reviewed journal of the American Cancer Society, the research indicates that obesity may contribute to the development of ovarian cancer through a hormonal mechanism. Ovarian cancer is the most fatal of gynecologic malignancies, and has a 5-year survival rate of only 37 percent.

To investigate this issue, Dr. Michael F. Leitzmann of the National Cancer Institute and colleagues studied 94,525 U.S. women aged 50 to 71 years over a period of seven years. The researchers documented 303 ovarian cancer cases during this time and noted that among women who had never taken hormones after menopause, obesity was associated with an almost 80 percent higher risk of ovarian cancer. In contrast, no link between body weight and ovarian cancer was evident for women who had ever used menopausal hormone therapy.

According to Dr. Leitzmann, these findings support the hypothesis that obesity may enhance ovarian cancer risk in part through its hormonal effects. Excess body mass in postmenopausal women leads to an increased production of estrogen, which in turn may stimulate the growth of ovarian cells and play a role in the development of ovarian cancer.

In another study done 2003, American Cancer Society researchers analyzed data based on 900,000 American adults with obesity and cancer that they monitored for a total of 16 years.  This is what they found in their landmark study:

1)The researchers found that the most obese women had a 62% increase in their risk of dying from cancer than women of normal weight; for obese men, the increase was 52%. The wide range of tumor types included colorectal, liver, gallbladder, pancreas, esophageal, kidney, prostate, breast, uterine, endometrial, and ovarian cancers. The researchers conclusion was that above-normal weight was associated with almost 20% of all cancer deaths in the United States. “There’s an incredibly powerful link between obesity and cancer,” says oncologist Joyce Slingerland of the University of Miami, Florida. “Everyone’s heard of obesity’s effect on heart disease and diabetes, and we’re now beginning to understand that the cancer risk is just as great,” she says.

Although researchers and epidemiologists had long suspected that diet and cancer were linked, efforts to explain why being fat makes cancer more deadly have only begun to deliver results in the past decade.

So it is up to the people in society of that country they live in to take on responsible actions to make their country a better for all, not just one.  I say that is becoming as healthy as possible that an individual can reach and without America has the answers in knowing how to make this happen 100% in our country.

A new study from American Cancer Society researchers finds it’s not just how much physical activity you get, but how much time you spend sitting that can affect your risk of death.

My final study supporting how obesity links with cancer:

Just think if this was you or someone you know in the world and this happens to you or them; GOD FORBID, but you can help yourself through prevention with losing the weight or just staying slim and tone if you already are.  This will just decrease your risk of getting the cancer.

The JCCC study, led by Dr. Guido Eibl, JCCC member and professor-in-residence in the department of surgery at David Geffen School of Medicine, revealed that mice made obese with high-calorie, high-fat diets developed abnormally high numbers of these lesions.

This is the first study to show a direct causal link in an animal model between obesity and risk of this deadly pancreatic cancer.  The JCCC study, led by Dr. Guido Eibl, JCCC member and professor-in-residence in the department of surgery at David Geffen School of Medicine, revealed that mice made obese with high-calorie, high-fat diets developed abnormally high numbers of these lesions. This is the first study to show a direct causal link in an animal model between obesity and risk of this deadly pancreatic cancer.

The mice eating the normal diet gained an average of approximately 7.2 g over 14 months. Mice on the high-fat, high-calorie diet more than doubled this with an average weight gain of 15.9 g.

Pathological tests showed that mice fed the normal diet had mostly normal pancreases with very few scattered PanIN lesions=intraepithelial neoplasias=precursors to pancreatic lesions.  They are used as markers but can only be seen microscopically so a biopsy is needed.  The mice fed high fat & cholesterol in their diet had significantly more PanIN lesions with less overall healthy pancreases.

The study showed that the mice fed a diet high in fats and calories gained significantly more weight, had abnormalities of their metabolism and increased insulin levels, and had marked pancreatic tissue inflammation with the development of PanIN lesions.

These observations suggest that such a diet like this which leads to weight gain, metabolism disturbances, pancreas inflammation and pancreas lesions that are precursors to cancer.

Obviously research is showing obesity has a link with cancer.  We now know this information let’s make a move America and people elsewhere to become a healthier nation including world.  For diversity is the US and other countries filled with all cultures, races and genders need to learn this knowledge to help make their country whether they were either born there and stayed or those born elsewhere moving to a new country, like America and we the people making up the country need to be responsible not just for yourselves but for your children and future generations in spreading good habits, good dieting, and good exercise to prevent high disease in the country for yourself and your children and the future generations by being a good role model in thinking=live healthy not unhealthy.  In time this would play an impact on your countries economy and health care system in how its run.

References:

1 –American Institute for Cancer Research, Schernhammer ES et al. Circulating levels of insulin-like growth factors, their binding proteins, and breast cancer risk. Cancer Epidemiol Biomarkers Prev. 2005 Mar;14(3):699-704 ES et al. their binding proteins, and breast cancer risk—Cancer Epidemiol Biomarkers Prev. 2006 Mar;14(3):699-704/ AICR ScienceNow /Volume 16/Spring 2006

2 – Studies Help Clarify Link Between Obesity and Prostate Cancer Article date: March 2, 2006 Impact of Obesity on Prostate Cancer Recurrence After Radical Prostatectomy: Data from CaPSURE.” Published in the Nov., 2005 Urology (Vol. 66, No. 5: 1060-1065). First author: William W. Bassett, University of California, San Francisco.“Obesity, Serum Prostate Specific Antigen and Prostate Size: Implications for Prostate Cancer Detection.”Published in the Feb. 2006 Journal of Urology (Vol. 175, No. 2: 500-504). First author: Stephen J. Freedland, MD, Duke University School of Medicine.

3 –Director, Medical & Scientific Communications
American Cancer Society
david.sampson@cancer.org
  Article: “Body mass index and risk of ovarian cancer.” Michael F. Leitzmann, Corinna Koebnick, Kim N. Danforth, Louise A. Brinton, Steven C. Moore, Albert R. Hollenbeck, Arthur Schatzkin, and James V. Lacey, Jr. CANCER; Published Online: January 05, 2009 (DOI: 10.1002/cncr.24086); Print Issue Date: February 15, 2009.

4 –Medical News Today-“Direct  Link to Obesity and Pancreatic Cancer” Author Belinda Weber

5 – Proceedings of the National Academy of Sciences of the United States of America –Article “Link between obesity and cancer” by Sarah C.P. Williams-Science Writer

6 – National Cancer Institute at the National Institute of health – Under their national cancer fact sheet regarding their article “Obesity and Cancer Risk”.

QUOTE FOR TUESDAY:

“Men tend to develop disease in the main arteries that feed the heart (CAD), while women are more likely to develop disease in the smaller arteries (microvascular disease). When plaques exist in the main arteries, they behave differentlyMen are more likely to have plaque ruptures, while women more frequently have plaque erosions.”

The Society of Thoracic Surgeons (ctsurgerypatients.org)

How women differ from men in heart disease and why!

          Women and Heart DIsease

Many many women and their doctors don’t know that heart disease is the number one killer of women. Furthermore, the heart disease that is seen in women is often not quite the same as heart disease in men.

Let’s remember from Part I that Heart disease is an umbrella term that includes heart failure, coronary artery disease (CAD), arrhythmias, angina, and other heart-related infections, irregularities, and birth defects

These facts lead to two common (and sometimes tragic) misapprehensions held by many women and their doctors: That women don’t really get much heart disease, and when they do, it behaves pretty much like the heart disease that men get.

The truth is that not only is heart disease very common in women, but also, when women get heart disease it often acts quite differently than it does in men. Failing to understand these two fundamental truths leads to a lot of preventable deaths and disability in women with heart disease.

If you are a woman, you need to know the basics about heart disease – especially heart disease as it behaves in women.

When women have angina, they are more likely than men to experience “atypical” symptoms. Instead of chest pain, they are more likely to experience a hot or burning sensation, or even tenderness to touch, which may be located in the back, shoulders, arms or jaw – and often women have no chest discomfort at all. An alert doctor will think of angina whenever a patient describes any sort of fleeting, exertion-related discomfort located anywhere above the waist, and they really shouldn’t be thrown off by such “atypical” descriptions of symptoms. However, unless doctors are thinking specifically of the possibility of CAD, they are all too likely to write such symptoms off to mere musculoskeletal pain or gastrointestinal disturbances.

Women are more likely than men to have heart attack symptoms unrelated to chest pain, such as:

      • Neck, jaw, shoulder, upper back or abdominal discomfort.
      • Shortness of breath.
      • Right arm pain.
      • Nausea or vomiting.
      • Sweating.
      • Lightheadedness or dizziness.
      • Unusual fatigue.

Heart attacks (or myocardial infarctions)  also tend to behave differently in women.

Frequently, instead of the crushing chest pain that is considered typical for a heart attack, women may experience nausea, vomiting, indigestion, shortness of breath or extreme fatigue – but no chest pain. Unfortunately, these symptoms are also easy to attribute to something other than the heart. Furthermore, women (especially women with diabetes) are more likely than men to have “silent” heart attacks – that is, heart attacks without any acute symptoms at all, and which are diagnosed only at a later time, when subsequent cardiac symptoms occur.

The Diagnosis Of CAD in Women Can Be More Difficult.

Diagnostic tests that work quite well in men can be misleading in women. The most common problem is seen with stress testing – in women, the electrocardiogram (ECG) during exercise can often show changes suggesting CAD, whether CAD is present or not, making the study difficult to interpret. Many cardiologists routinely add an echocardiogram or a thallium study when doing a stress test in a woman, which greatly improves diagnostic accuracy.

In women with typical CAD, coronary angiography is every bit as useful as in men; it identifies the exact location of any plaques (i.e., blockages) within the coronary arteries, and guides therapeutic decisions. However, in women with atypical coronary artery disorders (to be discussed in the next section), coronary angiograms often appear misleadingly normal. Thus, in women angiography is often not the gold standard for diagnosis, as it is for most men.

CAD In Women Can Take Atypical Forms.

At least four atypical coronary artery disorders can occur in women, usually in younger (i.e., pre-menopausal) women. Each of these conditions can produce symptoms of angina with apparently “normal” coronary arteries (that is, coronary arteries that often appear normal on angiogram). The problem, obviously, is that if the physician trusts the results of the angiogram, he/she is likely to miss the real diagnosis.

DALLAS, February 19, 2013 — A new study show women’s heart disease awareness is increasing.  A study with the number of women aware that heart disease is the leading cause of death nearly is doubling in the last 15 years, but that this knowledge still lags in minorities and younger women, according to the American Heart Association (AHA).

Among the study’s major findings, researchers comparing women’s views about heart disease in 1997 and today found:

  • In 2012, 56 percent of women identified heart disease as the leading cause of death compared with 30 percent in 1997.
  • In 1997, women were more likely to cite cancer than heart disease as the leading killer (35 percent versus 30 percent); but in 2012, only 24 percent cited cancer.
  • In 2012, 36 percent of black women and 34 percent of Hispanic women identified heart disease as the top killer — awareness levels that white women had in 1997 (33 percent).
  • Women 25-34 years old had the lowest awareness rate of any age group at 44 percent.

Among the women surveyed in 2012, researchers found:

  • Racial and ethnic minorities reported higher levels of trust in their healthcare providers compared with whites, and were also more likely to act on the information provided—dispelling the myth that mistrust of providers contributes to disparities.
  • Compared with older women, younger women were more likely to report not discussing heart disease risk with their doctors (6 percent among those 25-34 versus 33 percent for those 65 and older).

Risk Factors for Heart Disease in Women – Those we can’t change = Nonmodifiable Factors:

Age and Family History, Gender, Ethnicity.

The risk of having heart disease increases with age and this is due to stiffening of heart muscles which makes the heart less efficient in pumping blood around the body. You can determine your heart age by using this tool, developed by the British Heart Foundation: https://www.bhf.org.uk/heart-health/risk-factors/check-your-heart-age.

Another risk factor you cannot change is if you have a history of heart disease among family members. This can double your risk, so if your mother, father, sister or brother has suffered from heart disease before the age of 60 you are at a greater risk of developing heart disease.

Modifiable Risk Factors – Those we can change are:

1-Smoking is the single largest preventable cause of death in Australia, and approximately 40% of women who smoke die due to heart disease, stroke or blood vessel disease. Smokers are 2-4 times more at risk of developing heart disease compared to non-smokers. In 2011/2012, over 1.3 million women in Australia smoked, and 89% of them did this on a daily basis. While these numbers are for women aged 15 and over, the largest group were in the 25-34 age group.

Passive smoking (exposure to the cigarette smoke of others) also causes an increase in the risk of developing heart disease, which increases further in people having high blood pressure or high cholesterol. Women who smoke and also take the contraceptive pill have a 10 times higher risk of having a heart attack.

2-Alcohol. Do you know that drinking too much alcohol increases the risk of heart disease? Excessive drinking causes more weight gain (due to increased calories!), increase in blood pressure and blood lipids. Over a long period of time it can weaken the heart muscle and cause abnormal heart rhythms. Try and not drink alcohol every day, limit it to two standard drinks at a time and aim for at least two alcohol free days a week and make sure you don’t increase the amount you drink on the other days. Periodically take a break from any alcohol for a week or more and you will notice many benefits including a better nights sleep.

3.High Blood Pressure or Hypertension. Your blood pressure is a measurement of how ‘hard’ your heart is working to push blood around your body, through the blood vessels. It can be a ‘silent’ killer and if you do not know your blood pressure then it is worth having it checked by your GP. Changing your lifestyle will reduce your blood pressure. A recent study suggests that keeping your blood pressure under 140/90 can increase your life expectancy by 5 years at the age of 50 years. You can assess your high blood pressure through your MD monthly or less expensive buy a b/p machine and check your b/p everyday especially if your on antihypertensive meds to make sure your b/p isn’t under 100/60 to prevent hypotension.

4.Diabetes. Do you have diabetes and if so, is it under control?

Diabetes doubles your risk of having heart disease. People who have uncontrolled diabetes are at risk of having heart disease at an earlier age. For pre-menopausal women, having diabetes cancels the protective effects of hormone present in women and significantly increases the risk of heart disease. Taking steps to find out what your blood sugar is and keeping it well-controlled is essential.

5.Obesity- Do you know your body fat content?  If you think that you are overweight then you put yourself at risk of having heart disease. Being overweight will increase your blood pressure and contribute to developing diabetes. In addition to that, women who carry weight around their middle (belly fat) as opposed to their hips are twice as likely to develop heart disease.

By taking the steps to reduce your weight, you can reduce your risk of heart disease. A great tool developed by National Heart Foundation of Australia calculates if you might be at risk: http://www.heartfoundation.org.au/healthy-eating/Pages/bmi-calculator.aspx

6- INACTIVE-Are you physically active every day? Recent research indicates that “sitting is the new smoking” and being physically inactive can double your risk of having heart disease. It is important to get some exercise every day, such as a 30 minute walk where you raise your heart rate. It also raises your serotonin levels (feel-good hormone) and can reduce depression

7- STRESS-We could almost ask – do you know anyone who is not stressed?! However, while everyday life is stressful, those people who are almost constantly stressed are at risk of adopting unhealthy behaviours in order to reduce their stress levels. Examples include increasing their alcohol intake or smoking in order to relax; or tending to eat more junk food because they are often short of time. All of these factors increase their risk of heart disease.

Women, stress and the risk of heart disease

Along with poor diet, lack of exercise and smoking, unmanaged stress may increase the risk for heart disease. Now medical experts are discovering that mental stress affects women in different, and in some cases, more devastating ways, especially if they already have coronary conditions. One study that

Heart disease is the leading cause of death for men and women in the United States. Every year, 1 in 4 deaths are caused by heart disease. The good news? Heart disease can often be prevented when people make healthy choices and manage their health conditions. Communities, health professionals, and families can work together to create opportunities for people to make healthier choices. Make a difference in your community: Spread the word about strategies for preventing heart disease and encourage people to live heart healthy lives

 

QUOTE FOR MONDAY:


“Reye syndrome is a rare illness that can affect the blood, liver, and brain of someone who has recently had a viral infection. It always follows another illness. Although it mostly affects children and teens, anyone can get it. It can develop quickly and without warning. It is most common during flu season.”.

U.S.. Library of Medicine / Medline

Reye’s Syndrome

Reye’s syndrome is a rare but serious disease that causes swelling in the liver and brain. It can affect people of any age, but it is most often seen in children and teenagers recovering from a virus such as the flu or chickenpox.Studies have found that the main risk factor for Reye’s syndrome is taking aspirin or other related drugs, called salicylates.

Because of this, doctors recommend that children and teenagers recovering from viral infections should avoid taking aspirin.

What Causes It?

Doctors don’t fully understand what causes Reye’s syndrome. They do know that some people are prone to get it when they take aspirin for a virus.

Others have a greater chance of getting it if they:

  • Have a disorder that affects how their bodies break down fatty acids
  • Have been exposed to certain toxins, including paint thinners and products to kill insects and weeds

When Reye’s syndrome strikes, cells throughout your body become swollen and build up fats. In turn, your blood sugar levels drop. Ammonia and acid levels in the blood rise. These changes can hit many organs, such as the brain and liver, where severe swelling can occur.

Symptoms

The signs of Reye’s typically appear 3 to 5 days after the start of a viral infection.

In children younger than age 2, early symptoms may include diarrhea and rapid breathing. In older children and teenagers, early symptoms may include ongoing vomiting and unusual sleepinessAs the syndrome goes on, symptoms can become more severe, and may include:

  • Personality changes (more irritable or aggressive)
  • Confusion or hallucinations
  • Weakness or inability to move arms or legs
  • Seizure or convulsions
  • Extreme tiredness
  • Loss of consciousness

Reye’s can be life-threatening. You should call 911 if you see these severe symptoms. Early diagnosis and treatment is crucial.

The syndrome can be mistaken for other conditions, including meningitis (a swelling of membranes covering the brain and spinal cord), a diabetes reaction, or poisoning.

Diagnosis

Doctors don’t have a specific test for Reye’s. They usually do urine and blood tests. They also screen for disorders involving fatty acids.

Other tests may include:

  • Spinal taps (a needle is inserted into a space below the end of the spinal cord to collect fluid)
  • Liver biopsies (a needle is pushed through the abdomen into the liver to get a sample of tissue)
  • Skin biopsies (a doctor scrapes a small skin sample to test)
  • CT or MRI scans (which can also rule out other problems)

Is There a Treatment?

There’s no single treatment that will stop Reye’s syndrome, but doctors can do some things to make sure it is managed. They can also try to prevent more severe symptoms and see that brain swelling is held down. These steps include:

  • Intravenous (IV) fluids that includes glucose and an electrolyte solution may be given through an intravenous (IV) line.
  • Diuretics to help your body get rid of salt and water (and stop swelling)
  • Medications to prevent bleeding
  • Vitamin K, plasma, and platelets (tiny blood cells that help form clots) in instances of liver bleeding
  • Cooling blankets. This intervention helps maintain internal body temperature at a safe level.

 

QUOTE FOR THE WEEKEND:

“Bleeding disorders are a group of conditions that result when the blood cannot clot properly.  In normal clotting, platelets, a type of blood cell, stick together and form a plug at the site of an injured blood vessel. Bleeding can result from either too few or abnormal platelets, abnormal or low amounts of clotting proteins, or abnormal blood vessels.”

American Society of Hematology

The breakthrough treatments for Hemophilia over the past 1900s to 2013.

Treatment Breakthroughs

In the early 1900s, there was no way to store blood. People with hemophilia who needed a transfusion typically received fresh whole blood from a family member. Life expectancy was 13 years old.

In 1901, the US Surgeon General’s Catalogue listed lime, inhaled oxygen and the use of thyroid gland or bone marrow, or hydrogen peroxide or gelatin, as treatments for hemophilia. By the 1930s, it was discovered that diluting certain snake venoms caused blood to clot. These treatment  were used in patients with hemophilia.

By 1926, the US Surgeon General’s Catalogue contained an entire section on the use of blood transfusions to replace missing clotting factors. Physicians discovered that patients responded readily to infusions of plasma when given promptly after they sustained spontaneous joint and muscle bleeding.

In 1937 Harvard physicians Arthur Patek and FHL Taylor published a paper describing anti-hemophilia globulin found in plasma. It could decrease clotting time in patients with hemophilia.

By the late 1950s and early 1960s fresh frozen plasma was transfused in patients in the hospital. However, each bag of the plasma contained so little of the necessary clotting factor that huge volumes of it had to be administered. Many children experienced severe joint bleeds that were crippling. Intracranial hemorrhage could be fatal. By 1960, the life expectancy for a person with severe hemophilia was less than 20 years old.

A paper written by Robert Macfarlane, a British hematologist, in the journal Nature in 1964 described the clotting process in detail. The interaction of the different factors in blood clotting was termed the “coagulation cascade,” now called the clotting cascade.

In 1965, Dr. Judith Graham Pool, a researcher at Stanford University, published a paper on cryoprecipitate. In a major breakthrough, she discovered that the precipitate left from thawing plasma was rich in factor VIII. Because cryoprecipitate contained a substantial amount of factor in a smaller volume, it could be infused to control serious bleeding. Blood banks could produce and store the component, making emergency surgery and elective procedures for patients with hemophilia patients much more manageable.

By the 1970s, freeze-dried powdered concentrates containing factor VIII and IX became available. Factor concentrates revolutionized hemophilia care because they could be stored at home, allowing patients to “self-infuse” factor products, alleviating trips to the hospital for treatment.

By the mid-1980s, it was confirmed that HIV/AIDS could be transmitted through the use of blood and blood products, such as those used to treat hemophilia. Approximately half of the people with hemophilia in the US eventually became infected with HIV through contaminated blood products; thousands died. The overwhelming impact of HIV on the bleeding disorders community was felt into the next few decades.

The hepatitis C virus (HCV) infection was also transmitted through contaminated factor products, pooled from the blood of hundreds of thousands of donors. Before testing for HCV began in 1992, an estimated 44% of all people with hemophilia had contracted it. With the advent of more sophisticated screening methods and purification techniques, the risk of contracting HCV through factor products is virtually nil.

Treatment for hemophilia and other bleeding disorders advanced in the 1990s. Factor products became safer as tighter screening methods were implemented and advanced methods of viral inactivation were used. In addition, synthetic (not derived from plasma) factor products were manufactured using recombinant technologies. In 1992, the first recombinant factor VIII product was approved by the Food and Drug Administration (FDA). In 1997, the first recombinant factor IX product was granted FDA approval. Additional synthetic drugs, such as desmopressin acetate (DDAVP), were also introduced to treat mild-to-moderate hemophilia A and von Willebrand disease.

By 1995, prophylaxis, a preventive treatment regimen performed 2-3 times weekly in children with hemophilia, became more common. Since the advent of prophylaxis, most children in the developed world live with  less pain, without the orthopedic damage associated with chronic bleeding. As a result, most children born with hemophilia in the US today can look forward to long, healthy and active lives.

However, some children develop inhibitors, or antibodies, to infused factor product. The development of a bypassing agent in 1997 offered these patients an alternative product to help stop bleeds and joint damage.

The early years of the 21st century have brought new recombinant products made without human or animal plasma derivatives, lowering the possibility risk for  allergic reactions to the products or inhibitors? New longer-lasting products promise to decrease regular  infusion rates from 2-3 times per week to once-weekly or even less.

In 2013, three separate gene therapy trials were begun at institutions across the country. They are testing the use of viruses as vector, or vehicles, to deliver factor IX genes into patients’ livers, correcting their hemophilia. Because the factor VIII gene is larger and more complicated to use, gene therapy clinical trials have not yet begun for patients with hemophilia A.

TIMELINE

1828 – Term “haemorrhaphilia” first used. Later shortened to “haemophilia.”

1926 – Erik von Willebrand identifies a bleeding disorder, later called von Willebrand disease (VWD)

1940s – whole blood transfusions given at hospital

1948 – National Hemophilia Foundation (NHF) opens as The Hemophilia Foundation, Inc.

1952 – Researchers describe what is now called factor IX clotting protein

1954 – NHF establishes a Medical Advisory Council, later called Medical and Scientific Advisory Council (MASAC)

1955 – First infusions of factor VIII in plasma form

1957 – Researchers in Sweden identify von Willebrand factor as the cause of VWD

1958 – First use of prophylaxis for hemophilia A

1964 – Dr. Judith Graham Pool discovers cryoprecipitate

1968 – First FVIII concentrate available

1970s – Primary prophylaxis therapy experiments begin

1970s – Freeze-dried plasma-derived factor concentrates available

1977 – Desmopressin identified to treat mild hemophilia and von Willebrand disease

1980s – Factor VIII, FIX and von Willebrand factor genes cloned

1982 – CDC reports first AIDS cases among people with hemophilia

1985 – First inactivated factor concentrates available

1992 – FDA approves first recombinant FVIII products

1995 – Prophylaxis becomes standard of treatment in US

1997 – FDA approves first recombinant FIX products

1998 – First human gene therapy trials begin

2000s – FDA approves first recombinant factor products made without human or animal plasma derivatives

2009 – FDA approves RiaSTAP to treat factor I deficiency

2011 – FDA approves Corifact to treat factor XIII deficiency

2013 – Gene therapy trials underway at three sites in the US

QUOTE FOR FRIDAY:

“Hemophilia is a rare disorder in which your blood doesn’t clot normally because it lacks sufficient blood-clotting proteins (clotting factors). If you have hemophilia, you may bleed for a longer time after an injury than you would if your blood clotted normally.

Small cuts usually aren’t much of a problem. If you have a severe deficiency of the clotting factor protein, the greater health concern is deep bleeding inside your body, especially in your knees, ankles and elbows. That internal bleeding can damage your organs and tissues, and may be life-threatening.”.

MAYO CLINIC

March Awareness on Hemophilia.

hemophilia3hemophilia2

hemophilia1hemodialysis explained

What is this condition?

Hemophilia is a bleeding disorder characterized by low levels of clotting factor proteins. Correct diagnosis of Hemophilia is essential to providing effective treatment. Blood Center of Wisconsin offers one of the largest diagnostic menus to accurately and confidently diagnose Hemophilia.

The X and Y chromosomes are called sex chromosomes. The gene for hemophilia is carried on the X chromosome. Hemophilia is inherited in an X-linked recessive manner.  Females inherit two X chromosomes, one from their mother and one from their father (XX). Males inherit an X chromosome from their mother and a Y chromosome from their father (XY). That means if a son inherits an X chromosome carrying hemophilia from his mother, he will have hemophilia. It also means that fathers cannot pass hemophilia on to their sons.

But because daughters have two X chromosomes, even if they inherit the hemophilia gene from their mother, most likely they will inherit a healthy X chromosome from their father and not have hemophilia. A daughter who inherits an X chromosome that contains the gene for hemophilia is called a carrier. She can pass the gene on to her children. Hemophilia can occur in daughters, but is rare.

For a female carrier, there are four possible outcomes for each pregnancy:

  1. A girl who is not a carrier
  2. A girl who is a carrier
  3. A boy without hemophilia
  4. A boy with hemophilia

Hemophilia is an X-linked inherited bleeding disorder caused by mutation of the F8 gene that encodes for coagulation factor VIII or the F9 gene that encodes for coagulation factor IX. The degree of plasma factor deficiency correlates with both the clinical severity of disease and genetic findings. Severe hemophilia is characterized by plasma factor VIII or factor IX levels of under 1 IU/dl. Moderate and mild hemophilia are characterized by factor VIII or factor IX levels of 1-5 IU/dL or 6 – 40 IU/dL, respectively. Genetic analysis is useful for identification of the underlying genetic defect in males with severe, moderate or mild hemophilia and for determination of carrier status in the female individuals within their families. Additionally, data is emerging regarding the correlation between a patients mutation status and the risk of that patient developing an inhibitor.

People with hemophilia A often, bleed longer than other people. Bleeds can occur internally, into joints and muscles, or externally, from minor cuts, dental procedures or trauma. How frequently a person bleeds and the severity of those bleeds depends on how much FVIII is in the plasma, the straw-colored fluid portion of blood.

Normal plasma levels of FVIII range from 50% to 150%. Levels below 50%, or half of what is needed to form a clot, determine a person’s symptoms.

  • Mild hemophilia A-  6% up to 49% of FVIII in the blood. People with mild hemophilia Agenerally experience bleeding only after serious injury, trauma or surgery. In many cases, mild hemophilia is not diagnosed until an injury, surgery or tooth extraction results in prolonged bleeding. The first episode may not occur until adulthood. Women with mild hemophilia often experience menorrhagia, heavy menstrual periods, and can hemorrhage after childbirth.
  • Moderate hemophilia A. 1% up to 5% of FVIII in the blood. People with moderate hemophilia A  tend to have bleeding episodes after injuries. Bleeds that occur without obvious cause are called spontaneous bleeding episodes.
  • Severe hemophilia A.  <1% of FVIII in the blood. People with severe hemophilia A experience bleeding following an injury and may have frequent spontaneous bleeding episodes, often into their joints and musclesHemophilia A and B are diagnosed by measuring factor clotting activity. Individuals who have hemophilia A have low factor VIII clotting activity. Individuals who have hemophilia B have low factor IX clotting activity.Genetic testing is usually used to identify women who are carriers of a FVIII or FIX gene mutation, and to diagnose hemophilia in a fetus during a pregnancy (prenatal diagnosis). It is sometimes used to diagnose individuals who have mild symptoms of hemophilia A or B.There is currently no cure for hemophilia. Treatment depends on the severity of hemophilia.People who have moderate to severe hemophilia A or B may need to have an infusion of clotting factor taken from donated human blood or from genetically engineered products called recombinant clotting factors to stop the bleeding. If the potential for bleeding is serious, a doctor may give infusions of clotting factor to avoid bleeding (preventive infusions) before the bleeding begins. Repeated infusions may be necessary if the internal bleeding is serious. When a person who has hemophilia has a small cut or scrape, using pressure and a bandage will take care of the wound. An ice pack can be used when there are small areas of bleeding under the skin.
  • When bleeding has damaged joints, physical therapy is used to help them function better. Physical therapy helps to keep the joints moving and prevents the joints from becoming frozen or badly deformed. Sometimes the bleeding into joints damages them or destroys them. In this situation, the individual may be given an artificial joint.
  • Treatment may involve slow injection of a medicine called desmopressin (DDAVP) by the doctor into one of the veins. DDAVP helps to release more clotting factor to stop the bleeding. Sometimes, DDAVP is given as a medication that can be breathed in through the nose (nasal spray).
  • Treatment of the condition:
  • Genetic testing is also available for the factor VIII gene and the factor IX gene. Genetic testing of the FVIII gene finds a disease-causing mutation in up to 98 percent of individuals who have hemophilia A. Genetic testing of the FIX gene finds disease-causing mutations in more than 99 percent of individuals who have hemophilia B.
  • For Diagnosing the condition:
  • Researchers have been working to develop a gene replacement treatment (gene therapy) for Hemophilia A. Research of gene therapy for hemophilia A is now taking place. The results are encouraging. Researchers continue to evaluate the long-term safety of gene therapies. The hope is that there will be a genetic cure for hemophilia in the future.