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

“On average, people with CF live into their mid to late 30s. But new treatments are increasing life expectancy.

CF affects several organ systems, including: Respiratory system, Digestive system and the Reproductive system.

Some people carry the CF gene without being affected by the disease. They often don’t know that they are carriers.Cystic fibrosis is inherited. A person with CF had both parents pass the changed gene to them. The birth of a child with CF is often a total surprise to a family. Most of the time there is no family history of CF. Caucasian people are more likely to have CF than people of African, Asian, or Hispanic ancestry.

There is currently no cure for CF. Scientists are investigating gene therapy. Some patients with advanced disease may be considered for surgeries like lung and pancreas transplant.

Goals of treatment are to ease symptoms, prevent and treat complications, and slow the progress of the disease.Treatment generally focuses on the following 2 areas.  Managing lung and digestive problems”.

MAYO CLINIC (https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700)

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.

“Cystic fibrosis (CF) is a genetic disease that causes sticky, thick mucus to build up in your organs, blocking and damaging them. Many people think of CF as a lung disease because it affects your lungs and airways, which can make it hard to breathe and cause frequent infections. But it’s called cystic fibrosis because it also causes cysts and scarring (fibrosis) in your pancreas. This damage, plus the thick mucus, can block ducts that release digestive enzymes, making it hard to get nutrients from your digestive tract. CF can also affect your liver, sinuses, intestines and sex organs.

The mucus that lines your organs and body cavities, such as your lungs and nose, is thin and watery. In people with CF, a change in a gene (genetic mutation) leads to low levels of certain proteins, or proteins that don’t work properly. Because of these faulty proteins, minerals that move water into your mucus (which thins it out) get trapped inside cells, leaving the mucus thick and sticky.

People with cystic fibrosis are born with it. It’s a lifelong illness that gets more severe over time. Most people with CF don’t live as long as people without it.”

The Cleveland Clinic (https://my.clevelandclinic.org/health/diseases/9358-cystic-fibrosis)

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?

“Cause: Hepatitis D virus and hepatitis E virus. Hepatitis D virus infection always occurs with hepatitis B infection, either with a chronic hepatitis B infection (superinfection) or as two simultaneous new infections (coinfection).

Illness and treatment: Hepatitis D and E typically have abrupt onset of fever, nausea, and abdominal pain followed by jaundice. Hepatitis D may progress to chronic hepatitis.

Sources: Humans are the reservoir for hepatitis D, which is usually transmitted by contact with blood or body fluids, particularly sharing drug paraphernalia. Humans and animals (swine) are the reservoir for hepatitis E which is transmitted most commonly through fecally contaminated food, water, and environment.

Additional risks: Pregnant women have higher risk for hepatitis E complications. Japan has reported more virulent hepatitis E strains.

Prevention: To avoid simultaneous hepatitis B infection, immunize all children and any adults with risks for exposure. Use safe sexual practices, avoid sharing drug paraphernalia, and screen blood and tissue products to prevent hepatitis D transmission. Use precautions while traveling to ensure safe food and water to avoid hepatitis E infection.

Recent Washington trends: Reports are rare. Cases of hepatitis D are typically associated with injection drug use. Cases of hepatitis E are typically travel associated.”

Washington State Dept of Health (https://doh.wa.gov/public-health-provider-resources/notifiable-conditions/hepatitis-d-and-e)

HEPATITIS D:

Hepatitis D (hepatitis delta) is a disease caused by the hepatitis D virus (HDV), a small circular enveloped RNA virus. This is one of five known hepatitis viruses: A, B, C, D, and E. HDV is considered to be a sub-viral satellite because it can propagate only in the presence of the hepatitis B virus (HBV).

How its transmitted:

Transmission of HDV can occur either be via simultaneous infection with HBV (coinfection) or superimposed on chronic hepatitis B or hepatitis B carrier state (superinfection).

Both superinfection and coinfection with HDV results in more severe complications compared to infection with HBV alone. These complications include a greater likelihood of experiencing liver failure in acute infections and a rapid progression to liver cirrhosis, with an increased chance of developing liver cancer in chronic infections. In combination with hepatitis B virus, hepatitis D has the highest fatality rate of all the hepatitis infections, at 20%.

The routes of transmission of hepatitis D are similar to those for hepatitis B. Infection is largely restricted to persons at high risk of hepatitis B infection, particularly injecting drug users and persons receiving clotting factor concentrates. Worldwide more than 15 million people are co-infected. HDV is rare in most developed countries, and is mostly associated with intravenous drug use.

How its diagnosed:

HDV infection is diagnosed by high levels of anti-HDV immunoglobulin G (IgG) and immunoglobulin M (IgM), and confirmed by detection of HDV RNA in serum.

However, HDV diagnostics are not widely available and there is no standardization for HDV RNA assays, which are used for monitoring response to antiviral therapy.

How it’s treated:

The vaccine for hepatitis B protects against hepatitis D virus because of the latter’s dependence on the presence of hepatitis B virus for it to replicate.

Low quality evidence suggests that interferon alpha can be effective in reducing the severity of the infection and the effect of the disease during the time the drug is given, but the benefit generally stops when the drug is discontinued, indicating that it does not cure the disease. Interferon is effective only in ~20% of cases.

The drug myrcludex B, which inhibits virus entry into hepatocytes, is in clinical trials as of October 2015

Hepatitis E:

Hepatitis E infection is found worldwide and is common in low- and middle-income countries with limited access to essential water, sanitation, hygiene and health services.

How it’s transmitted:

In these areas, the disease occurs both as outbreaks and as sporadic cases. The outbreaks usually follow periods of fecal contamination of drinking water supplies and may affect several hundred to several thousand persons. Some of these outbreaks have occurred in areas of conflict and humanitarian emergencies such as war zones and camps for refugees or internally displaced populations, where sanitation and safe water supply pose special challenges.

Sporadic cases are also believed to be related to contamination of water, albeit at a smaller scale. The cases in these areas are caused mostly by infection with genotype 1 virus, and much less frequently by genotype 2 virus.

In areas with better sanitation and water supply, hepatitis E infection is infrequent, with only occasional sporadic cases. Most of these cases are caused by genotype 3 virus and are triggered by infection with virus originating in animals, usually through ingestion of undercooked animal meat (including animal liver, particularly pork). These cases are not related to contamination of water or other foods.

How its diagnosed:

Definitive diagnosis of hepatitis E infection is usually based on the detection of specific anti-HEV immunoglobulin M (IgM) antibodies to the virus in a person’s blood; this is usually adequate in areas where the disease is common.

How it’s treated:

There is no specific antiviral therapy for acute hepatitis E. Physicians should offer supportive therapy. Patients are typically advised to rest, get adequate nutrition and fluids, avoid alcohol, and check with their physician before taking any medications that can damage the liver, especially acetaminophen.  If any form of treatment, NIH NIDDK recommends this:  “Treatment for acute hepatitis E includes resting, drinking plenty of liquids, and eating healthy foods to help relieve symptoms. Talk with your doctor before taking any prescription or over-the-counter medicines, vitamins or other dietary supplements NIH external link, or complementary or alternative NIH external link medicines—any of these could damage your liver. You should avoid alcohol until your doctor tells you that you have completely recovered from hepatitis E.”

“Hepatitis means inflammation of the liver. When the liver is inflamed or damaged, its function can be affected. Heavy alcohol use, toxins, some medications, and certain medical conditions can all cause hepatitis. However, hepatitis is often caused by a virus. In the United States, the most common hepatitis viruses are hepatitis A virus, hepatitis B virus, and hepatitis C virus.

Hepatitis C is a liver infection caused by the hepatitis C virus. Hepatitis C can range from a mild illness lasting a few weeks to a serious, long-term illness. Hepatitis C is often described as “acute,” meaning a new infection, or “chronic,” meaning long-term infection.

Hepatitis C can range from a mild illness lasting a few weeks to a serious, long-term illness. Hepatitis C is often described as “acute,” meaning a new infection, or “chronic,” meaning long-term infection.

• Acute hepatitis C occurs within the first 6 months after someone is exposed to the hepatitis C virus. Hepatitis C can be a short-term illness, but for most people, acute infection leads to chronic infection.
• Chronic hepatitis C can be a lifelong infection if left untreated. Chronic hepatitis C can cause serious health problems, including liver damage, cirrhosis (scarring of the liver), liver cancer, and even death.

There are vaccines to prevent hepatitis A and hepatitis B; however, there is no vaccine for hepatitis C.  Cirrhosis due to chronic hepatitis C is the leading indication for liver transplantation in Europe, United States and Japan.”

Centers for Disease Control and Prevention – CDC (https://www.cdc.gov/hepatitis/hcv/cfaq.htm)

Hepatitis C is an infectious disease affecting primarily the liver, caused by the hepatitis C virus (HCV). The infection is often asymptomatic, but chronic infection can lead to scarring of the liver and ultimately to cirrhosis, which is generally apparent after many years. In some cases, those with cirrhosis will go on to develop liver failure, liver cancer, or life-threatening esophageal and gastric varices.

HCV is spread primarily by blood-to-blood contact associated with intravenous drug use, poorly sterilized medical equipment, and transfusions. An estimated 150–200 million people worldwide are infected with hepatitis C^. The existence of hepatitis C – originally identifiable only as a type of non-A non-B hepatitis – was suggested in the 1970s and proven in 1989^.  Hepatitis C infects only humans and chimpanzees. It is one of five known hepatitis viruses: A, B, C, D, and E.

The virus persists in the liver in about 85% of those infected. This infection of the liver is caused by the hepatitis virus. About 3.2 million people in the U.S. have the disease. But it causes few symptoms, so most of them don’t know.

There are many forms of the hepatitis C virus. The most common in the U.S. is type 1. None is more serious than any other, but they respond differently to treatment.

Jaundice (a condition that causes yellow eyes and skin, as well as dark urine), Stomach pain, Loss of appetite, Nausea, and Fatigue.

HOW IT’S TRANSMITTED

The virus spreads through the blood or body fluids of an infected person.

You can catch it from:

-Sharing drugs and needles

-Having sex, especially if you have an STD, an HIV infection, several partners, or have rough sex.

-Being stuck by infected needles

-Birth — a mother can pass it to a child

Hepatitis C isn’t spread through food, water, or by casual contact.

HOW IT’S DIAGNOSED

You can get a blood test to see if you have the hepatitis C virus.

Are There Any Long-Term Effects?

Yes. About 75% to 85% of people who have it develop a long-term infection called chronic hepatitis C. It can lead to conditions like liver cancer and cirrhosis, or scarring of the liver. This is one of the top reasons people get liver transplants.

WHAT’S THE TREATMENT:

Hepatitis C treatments have changed a lot in recent years. The latest is a once-daily pill called Harvoni that cures the disease in most people in 8-12 weeks. It combines two drugs: sofosbuvir (Sovaldi) and ledipasvir. In clinical trials, the most common side effects were fatigue and headache. However, the medicine is expensive.  Interferon and ribavirin used to be the main treatments for hepatitis C. They can have side effects like fatigue, flu-like symptoms, anemia, skin rash, mild anxiety, depression, nausea, and diarrhea.

THOSE AT HIGHEST RISK:

Certain things may increase your risk of becoming infected with the hepatitis C virus. Just because you are at risk for getting hepatitis C does not mean that you have the virus.

Many people do not know how they became infected with hepatitis C.

Recommended Related to Hepatitis

Tips to Deal With Hepatitis C Fatigue

When you have hepatitis C, being tired — really tired — can be a fact of life. But there are ways you can boost your energy. Here, three people who have experience with fatigue from the disease share their tips.

You can get hepatitis from:                                                           

–Sharing needles and other equipment (such as cotton, spoons, and water) used to inject drugs.              

-Having your ears or another body part pierced, getting a tattoo, or having acupuncture with needles that have not been sterilized properly. The risk of getting hepatitis C in these ways is very low.                          

-Working in a health care environment where you are exposed to fresh blood or where you may be pricked with a used needle. Following standard precautions for health care workers makes this risk very low.

“With five different types of viral hepatitis, it can be difficult to understand the differences between them. Some forms of hepatitis get more attention than others, but it is still important to know how they are transmitted, what they do, and the steps that you can take to protect yourself and your liver!

While hepatitis A and B both impact the liver, the two viruses differ greatly from one another. Hepatitis B is a blood-borne pathogen; its primary mode of transmission is through direct blood-to-blood contact with an infected person. In contrast, hepatitis A can be spread by fecal-oral transmission or by consuming food or water that has been contaminated. It is important to note that a person cannot contract hepatitis B through casual interactions such as holding hands, sharing a meal with, or eating foods prepared by someone who is infected. There is no need to keep plates and utensils separate. However, hepatitis A can be spread through food that is prepared by an infected person. Hepatitis A is primarily caused by poor sanitation and personal hygiene. Poor sanitation and hygiene can be the result of a lack of essential infrastructure like waste management or clean water systems. It can also result from a lack of education.”

Hepatitis B Foundation (https://www.hepb.org/blog/whats-difference-hepatitis-vs-hepatitis-b/)