Archive | May 2022

Part III Cystic Fibrosis Awareness Month – Lung Replacement and how far medical advances have taken us!

Cystic Fibrosis has advanced with medical treatments and advocacy by patient groups such as the Cystic Fibrosis Foundation (CFF).

Prior to the 1950s, children with the most common and most serious forms of cystic fibrosis rarely lived past age 5. In the 1950s and 1960s pancreatic enzymes, airway clearance techniques, and antistaphylococcal antibiotics were introduced as treatments and CF life expectancy began to increase.

In the 1960s, average life expectancy grew to age 15 as antipseudomonal antibiotics were added to the treatment arsenal. Also, the first woman known to have CF had a successful pregnancy. Between the 1970s and 1990s, life expectancy gradually increased to age 31, thanks to still more treatments, including lung transplants and DNase.

Then, between 1993 and 2017, median life expectancy leapt to age 44. This means that among those born with CF in the U.S. between 2013 and 2017, half are predicted to live to age 44 or more.

According to the CFF’s Patient Registry, children with CF grow up to lead full, productive lives despite their disease. In 2017, the registry found that:

  • 51 percent of adults with CF work in full- or part-time jobs
  • 42 percent of adults with CF are married or living together
  • 31 percent of adults with CF earned a college degree

Thanks to advances in DNA testing, doctors are identifying more and more people with CF for the first time well into their 50s, 60s, and 70s.

The oldest person diagnosed with CF for the first time in the U.S. was 82, in Ireland was 76, and in the United Kingdom was 79.

Lung transplantation is a process in which you and the family and friends who make up your social support team will need to make thoughtful decisions throughout many phases. These decisions will affect you and your social support team medically, logistically, financially, socially, and emotionally. Starting the conversation early will give you more time to plan ahead by:

  • Getting the help you need to work on anything that could prevent you from being a candidate for transplant.
  • Making it easier for you and your family to make informed decisions.
  • Securing the necessary financial resources while you are still well enough to do so.

Know this:

Does a lung transplant cure cystic fibrosis? No. Cystic fibrosis is a genetic condition so even though the transplanted lungs will not have CF and will never develop it, the rest of the person’s body will continue to have cystic fibrosis.

People can live for 5, 10, or even 20 years after having one. About 87 percent of CF patients who receive lung transplants will live another year. Close to 50 percent of those who receive a lung transplant will survive for an extra 9 years says cysticfibrosis.com.

QUOTE FOR MONDAY:

“Screening for CF is part of newborn screening in every state in the U.S. A positive newborn screening is not a diagnosis of CF. But it does mean more testing is done.

The first test done to try to diagnose CF is a sweat chloride test. This measures the amount of salt in your child’s sweat.

Testing for the CF gene can be done from a small blood sample. Or it can be done from a cheek swab. For this, a brush is rubbed against the inside of your cheek. This is done to get cells for testing. Labs generally test for the 20 or so most common mutations.

Not all of the genetic errors that cause CF have been found.”

Stanford Children’s Health/Lucile Packard Children’s Hospital Stanford

 

Part II Cystic Fibrosis Awareness Month

 

 

Short review of what is cystic fibrosis?

Cystic fibrosis (CF) is a genetic disease. This means that CF is inherited.

Mutations in a gene called the CFTR (cystic fibrosis conductance transmembrane regulator) gene cause CF. The CFTR mutations causes changes in the body’s cell’s electrolyte transport system. Electrolytes are substances in blood that are critical to cell function. The main result of these transport system changes are seen in the body secretions, such as mucus and sweat.

The CFTR gene is quite large and complex. There are many different mutations in this gene that have been linked to CF.

A person will be born with CF only if 2 CF genes are inherited–one from the mother and one from the father.  An individual must inherit two non-functioning CF genes – one from each parent – to have CF. If both parents are carriers there is a 1 in 4 (25 percent) chance that both will pass on the non-functioning gene, which would result in a pregnancy affected with cystic fibrosis.

A person who has only one CF gene is called a CF carrier. They are healthy and don’t have the disease. But they are a carrier of the disease.

CF affects various organ systems in children and young adults, including the following:

  • Respiratory system
  • Digestive system
  • Reproductive system

How does CF affect the respiratory system?

The abnormal electrolyte transport system in CF causes the cells in the respiratory system, especially the lungs, to absorb too much sodium and water. This causes the normal thin secretions in the lungs to become very thick and hard to move. These thick secretions increase the risk for frequent respiratory infections.

Recurrent respiratory infections lead to progressive damage in the lungs, and eventually death of the cells in the lungs.

Because of the high rate of infection in the lower respiratory tract, people with CF may develop a chronic cough, blood in the sputum, and often even have a collapsed lung. The cough is usually worse in the morning or after activity.

People with CF also have upper respiratory tract symptoms. Some have nasal polyps that need surgical removal. Nasal polyps are small protrusions of tissue from the lining of the nose that can block and irritate the nasal cavity. People with CF also have higher rates of sinus infections.

How does CF affect the gastrointestinal (GI) system?

CF mainly affects the pancreas. The pancreas secretes substances that aid digestion and help control blood sugar levels.

The secretions from the pancreas also become thick and can clog the ducts of the pancreas. This may cause a decrease in the secretion of enzymes from the pancreas that normally help digest food. A person with CF has trouble absorbing proteins, fats, and vitamins A, D, E, and K.

The problems with the pancreas can become so severe that some of the cells in the pancreas die. Over time, this may lead to glucose intolerance and Cystic Fibrosis-Related Diabetes (CFRD), a unique type of insulin-dependent diabetes.

The symptoms of CF that may be due to involvement with the GI tract include:

  • Bulky, greasy stools
  • Rectal prolapse (a condition in which the lower end of the bowel comes out of the anus)
  • Delayed puberty
  • Fat in the stools
  • Stomach pain
  • Bloody diarrhea

The liver may also be affected. A small number of people may develop liver disease. Symptoms of liver disease include:

  • Enlarged liver
  • Swollen belly
  • Yellow color to the skin (jaundice)
  • Vomiting of blood

How does CF affect the reproductive system?

Most males with CF have blockage of the sperm canal. This is called congenital bilateral absence of the vas deferens (CBAVD). This results from the thick secretions clogging the vas deferens and keeping them from developing properly. It causes infertility because sperm can’t travel out of the body. There are some newer techniques that allow men with cystic fibrosis to have children. These should be discussed with your healthcare provider. Women also have an increase in thick cervical mucus that may lead to a decrease in fertility, although many women with CF are able to have children.

Who is at risk for cystic fibrosis?

Cystic fibrosis (CF) is inherited, and a person with CF had both parents pass the altered gene to them. The birth of a child with CF is often a total surprise to a family, since most of the time there is no family history of CF.

Symptoms can include with above symptoms the following:

All U.S. states require that newborns be tested for cystic fibrosis (CF). This means that parents can know if their baby has the disease and can take precautions and watch for early signs of problems.

The following are the most common symptoms of CF. However, people may experience symptoms differently, and the severity of symptoms can vary, too. Symptoms may include:

  • Thick mucus that clogs certain organs, such as the lungs, pancreas, and intestines. This may cause malnutrition, poor growth, frequent respiratory infections, breathing problems, and chronic lung disease.

Many other medical problems can point to cystic fibrosis, as well. These include:

Sinusitis, Nasal polyps, Clubbing of fingers and toes. This means thickened fingertips and toes because of less oxygen in the blood, Collapse of the lung often due to intense coughing, Coughing up blood, Enlargement of the right side of the heart due to increased pressure in the lungs (Cor pulmonale), Abdominal pain, Excess gas in the intestines, Rectal prolapse. In this condition, the lower end of the bowel comes out of the anus, Liver disease, Diabetes, Pancreatitis, or inflammation of the pancreas that causes severe pain in the belly, Gallstones, Congenital bilateral absence of the vas deferens (CBAVD) in males. This causes blockages of the sperm canal.

The symptoms of CF differ for each person.

Infants born with CF usually show symptoms by age 2. Some children, though, may not show symptoms until later in life. The following signs are suspicious of CF, and infants having these signs may be further tested for CF:

Diarrhea that does not go away, Foul-smelling stools, Greasy stools, Frequent wheezing, Frequent pneumonia or other lung infections, Persistent cough, Skin that tastes like salt, Poor growth despite having a good appetite.

The symptoms of CF may resemble other conditions or medical problems. See a healthcare provider for a diagnosis.

QUOTE FOR THE WEEKEND:

“Cystic fibrosis (CF) is a genetic disorder that causes problems with breathing and digestion. CF affects about 35,000 people in the United States. People with CF have mucus that is too thick and sticky, which

  • blocks airways and leads to lung damage;
  • traps germs and makes infections more likely; and
  • prevents proteins needed for digestion from reaching the intestines, which decreases the body’s ability to absorb nutrients from food.
  • Sometimes it blocks the pancreas”

Center of Disease Prevention and Control CDC

Part I Cystic Fibrosis Awareness Month

Cystic fibrosis (CF) is a genetic disorder that causes problems with the lungs=breathing and digestion sytem.  It can obstruct the pancreas. CF affects about 35,000 people in the United States. Cystic fibrosis (CF) can be life-threatening, and people with the condition tend to have a shorter-than-normal life span.   This diagnosis can have mucus that is too thick and sticky, which

  • blocks airways and leads to lung damage;
  • traps germs and makes infections more likely; and
  • prevents proteins needed for digestion from reaching the intestines, which decreases the body’s ability to absorb nutrients from food.

Cystic fibrosis is a hereditary disease that affects the lungs and digestive system. The body produces thick and sticky mucus that can clog the lungs and obstruct the pancreas. 

What is this disease?

Cystic fibrosis (CF) is a progressive, genetic disease that causes persistent lung infections and limits the ability to breathe over time.

In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause the CFTR protein to become dysfunctional. When the protein is not working correctly, it’s unable to help move chloride — a component of salt — to the cell surface. Without the chloride to attract water to the cell surface, the mucus in various organs becomes thick and sticky.

In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, minimizing contact with germs is a top concern for people with CF.

In the pancreas, the buildup of mucus prevents the release of digestive enzymes that help the body absorb food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children.

Symptoms of CF

People with CF can have a variety of symptoms, including:

  • Very salty-tasting skin
  • Persistent coughing, at times with phlegm
  • Frequent lung infections including pneumonia or bronchitis
  • Wheezing or shortness of breath
  • Poor growth or weight gain in spite of a good appetite
  • Frequent greasy, bulky stools or difficulty with bowel movements
  • Male infertility

Cystic fibrosis is a genetic disease. People with CF have inherited two copies of the defective CF gene — one copy from each parent. Both parents must have at least one copy of the defective gene.

People with only one copy of the defective CF gene are called carriers, but they do not have the disease. Each time two CF carriers have a child, the chances are:

  • 25 percent (1 in 4) the child will have CF
  • 50 percent (1 in 2) the child will be a carrier but will not have CF
  • 25 percent (1 in 4) the child will not be a carrier and will not have CF

The defective CF gene contains a slight abnormality called a mutation. There are more than 1,700 known mutations of the disease. Most genetic tests only screen for the most common CF mutations. Therefore, the test results may indicate a person who is a carrier of the CF gene is not a carrier.

Diagnosing cystic fibrosis is a multistep process, and should include a newborn screening, a sweat test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center. Although most people are diagnosed with CF by the age of 2, some are diagnosed as adults. A CF specialist can order a sweat test and recommend additional testing to confirm a CF diagnosis.

Read the CF Foundation’s clinical care guidelines for diagnosing CF.

According to the Cystic Fibrosis Foundation Patient Registry, in the United States:

  • More than 30,000 people are living with cystic fibrosis (more than 70,000 worldwide).
  • Approximately 1,000 new cases of CF are diagnosed each year.
  • More than 75 percent of people with CF are diagnosed by age 2.
  • More than half of the CF population is age 18 or older.

 Stay tune for Monday’s topic Part II on CF in How it affects different parts of the system and more!

 

QUOTE FOR FRIDAY:

“Harvest is typically a six to seven week season. Harvest is typically in full swing around the middle of May and through June.  Michigan moved up in the ranks as the 2nd largest producer of asparagus in the nation, producing just less than 21 million pounds.  It’s nutritious, flavorful and one of Michigan’s first signs of spring. Michigan asparagus is the state’s first green vegetable harvested each year. A typical Michigan asparagus harvest begins in mid-April, but in wake of a long, cold winter and cooler spring, it can be pushed back.

Pure Michigan (https://www.michigan.org/article/trip-idea/celebrate-national-asparagus-month-pure-michigan)

 

 

May is National Asparagus Month!

Why?  Here are some reasons asparagus is a health topic for May!

1- Asparagus is a low-calorie vegetable that is an excellent source of essential vitamins and minerals, especially folate and vitamins A, C and K.

2- Asparagus is a good source of antioxidants!

3- Antioxidants are compounds that help protect your cells from the harmful effects of free radicals and oxidative stress.

Oxidative stress contributes to aging, chronic inflammation and many diseases, including cancer.

Asparagus, like other green vegetables, is high in antioxidants. These include vitamin E, vitamin C and glutathione, as well as various flavonoids and polyphenols.

Asparagus is particularly high in the flavonoids quercetin, isorhamnetin and kaempferol.  These substances have been found to have blood pressure-lowering, anti-inflammatory, antiviral and anticancer effects in a number of human, test-tube and animal studies.

Purple asparagus contains powerful pigments called anthocyanins, which give the vegetable its vibrant color and have antioxidant effects in the body.  This increasing anthocyanin intake has been shown to reduce blood pressure and the risk of heart attacks and heart disease. So eating asparagus along with other fruits and vegetables can provide your body with a range of antioxidants to promote good health.

4- Dietary fiber is essential for good digestive health.

Taking a half a cup of asparagus contains 1.8 grams of fiber, which is 7% of your daily needs.

Studies suggest that a diet high in fiber-rich fruits and vegetables may help reduce the risk of high blood pressure, heart disease and diabetes!

Asparagus is particularly high in insoluble fiber, which adds bulk to stool and supports regular bowel movements.

It also contains a small amount of soluble fiber, which dissolves in water and forms a gel-like substance in the digestive tract.  Soluble fiber feeds the friendly bacteria in the gut.  Examples of friendly bacteria like Bifidobacteria and Lactobacillus.  Increasing the number of these beneficial bacteria plays a role in strengthening the immune system and producing essential nutrients like vitamins B12 and K2.  Eating asparagus as part of a fiber-rich diet is an excellent way to help meet your fiber needs and keep your digestive system healthy.

Endling line asparagus helps your digestive system by promoting regularity, digestive health and may aid in reducing your risk of heart disease, high blood pressure and diabetes.

5- It helps to support a healthy pregnancy!  How?  Asparagus is an excellent source of folate, also known as vitamin B9.  Just half a cup of asparagus provides adults with 34% of their daily folate needs and pregnant women with 22% of their daily needs.  Getting enough folate from sources like asparagus, green leafy vegetables and fruit can protect against neural tube defects, including spina bifida (both happening during fetal developement).  Folate is so vital during pre-pregnancy and early pregnancy that folate supplements are recommended to ensure women meet their requirements. Folate is an essential nutrient that helps form red blood cells and produce DNA for healthy growth and development. It’s especially important during the early stages of pregnancy to ensure the healthy development of the baby.

6- It helps lower the blood pressure!  High blood pressure affects more than 1.3 billion people worldwide and is a major risk factor for heart disease and stroke.  Research suggests that increasing potassium intake while reducing salt intake is an effective way to lower high blood pressure.  Potassium lowers blood pressure in two ways: by relaxing the walls of blood vessels and excreting excess salt through urine.

Asparagus is a good source of potassium, providing 6% of your daily requirement in a half-cup serving.

What’s more, research in rats with high blood pressure suggests that asparagus may have other blood pressure-lowering properties. In one study, rats were fed either a diet with 5% asparagus or a standard diet without asparagus.

After 10 weeks, the rats on the asparagus diet had 17% lower blood pressure than the rats on the standard diet.

Ending line, eating more potassium-rich vegetables, such as asparagus, is a great way to help keep your blood pressure in a healthy range.

7- It can help if your dieting to lose weight.  How?  First asparagus is about 94% water. Research suggests that consuming low-calorie, water-rich foods is associated with weight loss.  It can definitely help in dieting!

8- It’s inexpensive!

 

 

QUOTE FOR THURSDAY:

“First discovered in an Aboriginal Australian woman in 1961, the RH null (Rhesus null) is one of the rarest and most precious blood types in the world. Like a needle in a haystack, less than 50 people in the world are known to have it!

People who have the ‘golden blood’ type lack these Rh antigens. Their DNA lacks the genes responsible for building those RBC protein complexes. These people don’t just lack one, two or three of these 61 Rh antigens, they actually lack all of them. Yes, you read that right: all of them. As you might have guessed, people with Rhnull blood type have abnormal RBCs. They have deformed shapes, leaky membranes and shorter lifespans, which sometimes result in mild anaemia for the individual. Still, the absence of all Rh antigens makes Rhnull the ‘golden blood’, which is highly admired for its rarity and medical purposes.

To find out why this blood type was coined as the ‘golden blood’, we need to open the world of blood types and its systems.”

The University of Melbourne

QUOTE FOR WEDNESDAY:

“Hepatitis D and E typically have abrupt onset of fever, nausea, and abdominal pain followed by jaundice. Hepatitis D may progress to chronic hepatitis.  Hepatitis D  is known as “delta hepatitis,” is a liver infection caused by the Hepatitis D virus (HDV). Hepatitis D is uncommon in the United States. Hepatitis D only occurs among people who are infected with the Hepatitis B virus (BHV) because HDV is an incomplete virus that requires the helper function of HBV to replicate. HDV. Hepatitis E is a liver infection caused by the Hepatitis E virus (HEV). Hepatitis E is a self-limited disease that does not result in chronic infection. While rare in the United States, Hepatitis E is common in many parts of the world. It is transmitted from ingestion of fecal matter, even in microscopic amounts, and is usually associated with contaminated water supply in countries with poor sanitation. There is currently no FDA-approved vaccine for Hepatitis E. ”

Washington State Dept of Health

QUOTE FOR TUESDAY:

“Hepatitis C is an inflammation of the liver caused by the hepatitis C virus.  The hepatitis C virus is a bloodborne virus and most infection occur through exposure to blood from unsafe injection practices, unsafe health care, unscreened blood transfusions, injection drug use and sexual practices that lead to exposure to blood.  Globally, an estimated 58 million people have chronic hepatitis C virus infection, with about 1.5 million new infections occurring per year.”.

World Health Organization WHO