“One hundred years ago, at the time when Haematologica was first published, there was practically no treatment for the hemophilias or for the other inherited coagulation disorders. Whole blood was the only treatment approach available and this was of poor clinical efficacy, such that the life expectancy of hemophiliacs was 10-15 years, even in the most favorable circumstances.The success story of hemophilia care first began in the 1970s, when the availability of plasma-derived concentrates of coagulation factor VIII (FVIII) and factor IX (FIX) provided efficacious treatment of bleeding in patients with hemophilia A and B. This positive scenario was consolidated in terms of greater safety and availability in the 1990s, when the first recombinant coagulation factors were produced. This meant that, instead of only treating episodic bleeding events, prophylaxis regimens could be implemented as a preventive measure.  So, even until the 1960s, the life expectancy of patients with hemophilia was no more than 20-30 years.  Today success is way ahead.”

NIH – National Library of Medicine (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7049365/)

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 21^st 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

Check out a decade later 2023 treatments, tomorrow!

“Hemophilia is usually an inherited bleeding disorder in which the blood does not clot properly. This can lead to spontaneous bleeding as well as bleeding following injuries or surgery. Blood contains many proteins called clotting factors that can help to stop bleeding. People with hemophilia have low levels of either factor VIII (8) or factor IX (9). The severity of hemophilia that a person has is determined by the amount of factor in the blood. The lower the amount of the factor, the more likely it is that bleeding will occur which can lead to serious health problems.”

Centers for Disease Control and Prevention (https://www.cdc.gov/ncbddd/hemophilia/facts.html)

“April is National Donate Life Month, a time when organizations raise awareness about organ and tissue donation programs.

Nationally, more than 100,000 people are currently awaiting an organ transplant, including about 5,000 people in the Greater Philadelphia region, according to Rick Hasz, CEO of Gift of Life, a local organ donation program.

Hasz said most waitlisted patients in the area are waiting on a new kidney, and that some people wait for more than five years.

“With kidney disease, you have dialysis that can kind of get you through until that transplant, but the mortality rate on dialysis is very high. And so some people never get that second chance,” Hasz said.”

WHYY PBS (https://whyy.org/articles/april-national-donate-life-month-organ-transplants-philadelphia/)

More than 400,000 Americans in every corner of our country are alive today thanks to the tremendous generosity and courage of organ donors. During National Donate Life Month, striveforgoodhealth.com honors donors and their families who have turned pain into purpose by sharing the gift of life with loved ones in need or countless others whom they have never met.  Those who came to the most challenge in their life and lives who thought of others to save them with their organs THANK YOU A MILLION TIMES!!  It is encouraged to everyone to follow their lead and register as an organ, eye, tissue, or bone marrow donor, bringing hope and healing to so many others.

The White House Blog (https://www.whitehouse.gov) states, ” Last year, American doctors completed our Nation’s one-millionth organ transplant, a tremendous milestone in the history of a procedure pioneered and honed in America. We are now performing transplants at a record pace, with higher success rates and increased lifespans for recipients. Still, every 10 minutes, someone new joins the waiting list — fighting organ failure or blood cancer, their futures hanging in the balance. More than 100,000 people, including 1,900 children, are currently on the waiting list. A majority of them are people of color, for whom it can sometimes be more difficult to find a good donor match. Seventeen Americans die every day while waiting for a transplant.”

Remember you have the power to change that. Just one person can save up to 8 lives through organ donation after they die and improve another 75 lives through eye and tissue donation. Registering as a donor does not change the quality of care that you receive in your lifetime. It allows you to give countless others a second chance at life and your family to find peace amid grief while leaving an extraordinary legacy of compassion and dignity.

Each year, thousands of Americans choose to donate an organ while still living, a profoundly courageous act of connection and healing.  Can you be an organ donor at the end of your life whenever that may come about in your lifetime to a save another one’s life rather than your good organs just disintegrate by nature after death?

Bless all donor organs and again thank you for thinking of others!

“Without treatment, HIV infection advances in stages, getting worse over time. HIV gradually destroys the immune system and eventually causes acquired immunodeficiency syndrome (AIDS).

There is no cure for HIV, but treatment with HIV medicines (called antiretroviral therapy or ART) can slow or prevent HIV from advancing from one stage to the next. HIV medicines help people with HIV live longer, healthier lives. One of the main goals of ART is to reduce a person’s viral load to an undetectable level. An undetectable viral load means that the level of HIV in the blood is too low to be detected by a viral load test. People with HIV who maintain an undetectable viral load have effectively no risk of transmitting HIV to their HIV-negative partner through sex.”

NIH.gov (https://hivinfo.nih.gov/understanding-hiv/fact-sheets/stages-hiv-infection)

“

“Chlamydia, gonorrhea, trichomoniasis, and syphilis are the four most common sexually transmitted diseases (STDs). However, according to new global estimates, each day there are more than one million new cases of these sexually transmitted infections. The worrying thing is although we have never known more about how to prevent these infections, rates of infection remain very high worldwide. The good news is that these four infections are curable.

Some sexually transmitted diseases (STDs), such as human immunodeficiency virus cannot be cured and are deadly. You can find out ways to protect yourself from the following STDs by learning more about these diseases.”

Medicine Net (https://www.medicinenet.com/what_are_the_top_10_stds/article.htm)

Sexually transmitted diseases (STDs) have been known to mankind for centuries. Before the advent of modern medicine, people’s lack of awareness and understanding of STDs contributed to the widespread transmission of the infections while few or no treatments were available to treat the conditions.

In medieval times, syphilis and gonorrhoea were two of the most prevalent STDs in Europe.

Some STDs can have severe, life-changing consequences; syphilis, for example, can eventually cause progressive destruction of the brain and spinal cord, leading to mental dysfunction and hallucinations, speech problems and general paresis.

It’s kind of puzzling that sexually transmitted diseases are so prevalent—particularly when you consider that you have to get pretty up close and personal to contract one. An STD is characterized by any disease that is spread by one partner to another via sexual contact, and that can be orally, vaginally, anally, or via hand to genital contact. Regardless, they are spread when one partner passes the disease-causing organism on to the other. Obviously, preventing STD transmission is first and foremost by practicing safe sex (PREVENTION) and not enough do it in America for some crazy reason hurting themselves and other people. However, if you think you might have contracted one of the most common STDs, recognizing the disease is imperative for swift treatment and preventing further spreading.

Top venereal diseases in the USA:

1-Gonorrhea

The Centers for Disease Control estimate that 700,000 new cases of Gonorrhea, or the “clap”, crop up every year. This long-term STD that is spread bacterially, affecting a female’s cervix, a male’s urethra, or the throat in both sexes, which means that it’s transmitted by vaginal, oral, and anal sex. The symptoms of gonorrhea are pretty subtle; the most noticeable being burning when urinating or a yellowish penile discharge in men.

2-Hepatitis

Sexually transmitted hepatitis is hepatitis B (or HBV), which afflicts more than 1.25 million individuals in the U.S. even though there is a vaccine. If left untreated, a Hep B infection will scar and damage the liver, causing cirrhosis and liver cancer. Unfortunately, over half of those affected show no symptoms, but those who do suffer muscle pain and fatigue, yellowing of the eyes (or jaundice), nausea, and a distended stomach.

3-Syphilis

Syphilis is a particularly sneaky STD that caused by a type bacterial infection of the genital tract, known as Treponema Pallidum. Syphilis is transmitted when direct contact is made between the small, painless sores on the mouth, rectum, vagina, or around the genitals in areas not protected by latex condoms. It can also be transmitted via infected mother to her baby during pregnancy. When there are no sores, the disease is still present. Syphilis symptoms are rare , however, the most telling are sores or lesions on and around the genitals, as well as hair loss, sore throat, fever; headache; and a white patchy skin rash.

4- Chlamydia

Like Gonorrhea, Chlamydia affects a man’s penile urethra and a woman’s cervix. However, oftentimes those who’ve contracted Chlamydia don’t show symptoms for months or even years, which explains why it’s the most common and rampant STD. If you do show symptoms, you’ll feel pain during intercourse and have a discolored, thick discharge from the vagina or penis. Transmitted via sexual penetration with an affected partner, using latex condoms can prevent transmission of this curable STD.

 5. Crabs

If you feel a creepy-crawly, itchy sensation in your genitals, you may have crabs (or public lice). They show themselves as visible eggs or lice in the coarse hair of the genital region (even if you shave it off), and they can spread to the armpits and eyebrows if left untreated. Typically transmitted via sexual contact, crabs can also be passed via contact with infested linens or clothing.

6. Human Papilloma Virus

Human Papilloma Virus (or HPV) is currently the most wide spread STD. It affects roughly three-quarters of the sexually active population and a staggering one-quarter of sexually active women, which is why there is a North American vaccine to protect young women from certain types of HPV that are linked to genital warts and cervical cancer. HPV is transmitted through genital contact—via vaginal and anal sex, and also oral sex and genital-to-genital contact. Most times HPV doesn’t show any symptoms until it’s far advanced, but genital warts as well as RRP, a condition where warts grow in the throat and eventually cause breathing difficulties are common.

7. Bacterial Vaginosis

Bacterial Vaginosis, or BV, is not always considered an STD even though it typically afflicts those of child-bearing age with multiple or new sex partners. BV occurs when healthy bacteria in the vagina overgrow and become imbalanced, causing burning and itching around the vagina and a thick, grey discharge with a strong fishy odor. Antibiotics will quickly clear up bouts of BV, but it can reoccur, leaving the victim prone to pelvic inflammatory disease, other STDs, and premature births (if pregnant).

 8. Herpes

Painful sores or lesions on your mouth or genitals may indicate herpes, a viral STD that comes in two forms HSV1 (herpes of the mouth) and HSV2 (herpes of the genitals). Herpes is transmitted skin-to-skin—for instance, from genital to genital, mouth to genital, or mouth to mouth contact with an infected individual, even when they don’t have visible sores. Even though herpes symptoms be treated with antibiotics, the virus never goes away and reoccurs typically 2 to 4 times per year.

9. Trichomoniasis

Trichomoniasis, or “trich”, often masks itself as a yeast infection or bacterial vaginosis (BV) in women with similar symptoms—including a thick, grey discharge, offensive vaginal odor, pain or burning intercourse, and itchiness. A parasitic trichomonas vaginalis infection affects the urethra and the vagina in women. It can be transmitted back and forth between sex partners (man to woman and woman to woman) via vaginal intercourse and contact. However, most men typically don’t have any symptoms.

10. HIV

HIV is transmitted via the exchange of body fluids—such as semen, vaginal secretions, blood, or breast milk. Within a month or 2 of contracting HIV, about 40 to 90-percent of those afflicted suffer from flu-like symptoms including fever, fatigue, achy muscles, swollen lymph glands, sore throat, headache, skin rash, dry cough, nausea, rapid weight loss, night sweats, frequent yeast infections (for women), cold sores, and eventually, pneumonia. Luckily, many individuals who are diagnosed early can live a long, productive life with HIV thanks to a combination of highly active anti-retroviral drug therapy, which prevents to progression to AIDS.