Butterfly Rash

Lupus is an inflammatory disease that is caused when the auto, meaning “self”, immune system attacks its own tissues.

Lupus is a chronic, an autoimmune disease that can damage any part of the body (skin, joints, and/or organs inside the body). Chronic means that the signs and symptoms tend to last longer than six weeks and often for many years.

In lupus, something goes wrong with your immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu). Normally our immune system produces proteins called antibodies that protect the body from these invaders. Your autoimmune immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues and creates auto-antibodies that attack and destroy healthy tissue. These auto-antibodies cause inflammation, pain, and damage in various parts of the body.

Lupus is also a disease of remissions (flare ups) whereby the symptoms worsen and you feel ill and remissions (the symptoms improve and you feel better) but the disease remains. So your goal is to keep the disease as best as possible in remissions.

FACTS TO KNOW IF YOU HAVE LUPUS:

• Lupus is not contagious, not even through sexual contact. You cannot “catch” lupus from someone or “give” lupus to someone.
• Lupus is not like or related to cancer. Cancer is a condition of malignant, abnormal tissues that grow rapidly and spread into surrounding tissues. Lupus is an autoimmune disease, as described above. However, some treatments for lupus may include immunosuppressant drugs that are also used in chemotherapy.
• Lupus is not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome) except that they both affect the immune system (but differently) in having remissions and exacerbations, that’s it. In HIV or AIDS the immune system is underactive; whereas in lupus, the immune system is overactive.
• Lupus can range from mild to life-threatening and should always be treated by a doctor. With good medical care, most people with lupus can lead a full life.
• Another factor in helping you reach remission is being compliant with the taking the meds ordered by your doctor.
• Research estimates that at least 1.5 million Americans have a form of lupus. The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in the U.S. living with lupus.
• More than 16,000 new cases of lupus are reported annually across the country.
• It is believed that 5 million people throughout the world have a form of lupus.
• Lupus strikes mostly women of childbearing age (15-44). However, men, children, and teenagers develop lupus, too. Most people with lupus develop the disease between the ages of 15-44.
• Women of color are two to three times more likely to develop lupus than Caucasians.
• People of all races and ethnic groups can develop lupus.
• Lupus effects 80% women.
• Discoid lupus erythematosus—causes a skin rash that doesn’t go away
• Subacute cutaneous lupus erythematosus—causes skin sores on parts of the body exposed to sun
• Drug-induced lupus—can be caused by medications
• Neonatal lupus—a rare type of lupus that affects newborns.      
• Approximately 1.5 million Americans have a form of lupus.
• Systemic lupus erythematosus cases: 70% of total lupus cases.
• Of individuals diagnosed with lupus, 90% are women.
• Eighty percent of people develop lupus between 15 and 45 years of age.
• Lupus is two to three times more prevalent among people of color.
• Chance of a parent or sibling having or developing lupus: 20%

There are 4 major kinds of lupus. Here are the types:

1-Systemic lupus erythematosus (SLE) is the most common and most serious type of lupus.

2-Cutaneous lupus erythematosus, which affects only the skin.

3-Drug-induced lupus, a short-term type of lupus caused by certain medicines.

4-Neonatal lupus, a rare type of lupus that affects newborn babies.

The most common type and serious type of lupus is systemic lupus erythematosus (SLE)

• Systemic Lupus Erythematosus  or also known as (SLE):
• The cause(s) of SLE are unknown, however, heredity, viruses, ultraviolet light, and drugs all may play some role.

Signs and Symptoms of Lupus:

1. *butterfly rash on the face – very common symptom with a flare up.

Including other possible symptoms:

2. appetite loss 3. hair loss 4. fever 5. fatigue 6. photosensitivity 7. Raynaud’s phenomenon 8. pleuritis and 9. pericarditis.

• Up to 10% of people with lupus isolated to the skin will develop the systemic form of lupus (SLE).
• The criteria listed at the top in the picture help doctors diagnose SLE!

Stayed tune for part II tomorrow on how its diagnosed and the type of treatments!

There have been many advances in CF treatment.

Antibiotics and Anti-inflammatories are used in treat for some with cystic fibrosis.

Antibiotics are frequently needed to treat bacteria that grow in the mucus. These can be given in one of three ways:

• Orally or by mouth – this is the easiest and cheapest route.
• By inhalation – this is more expensive but very effective.
• Intravenously (IV) – this is usually reserved for those who are sicker.

Anti-inflammatory medications have also been found to be helpful in CF. Two medications are currently in use, azithromycin (an antibiotic that’s used as an anti-inflammatory agent in CF), and ibuprofen.

Managing cystic fibrosis is a must!

Regular check-ups with your doctor are needed as patients with CF will require constant monitoring and health management to control symptoms and prevent complications.

Your doctor may request that you receive some other tests depending on your condition. Common tests include blood tests, bone mineral density tests, glucose monitoring, pancreas functionality test, and respiratory cultures. Ultrasounds, chest CTs or X-rays, colonoscopy and a lung biopsy may be needed in some situations.

In addition to doctor visits, lifestyle changes can help manage symptoms. Practicing good hygiene and receiving all recommended vaccines can prevent patients with CF from getting an infection which in turn could lead to more severe complications. To maintain a healthy weight many patients with CF follow healthy, high-calorie, high-sodium diets. Physical activity is also encouraged to help improve and maintain lung function.

Most importantly all patients with CF need to continue treatments, including medicines, supplements, and daily airway clearance techniques as directed by their physicians. Your physician should also be alerted immediately to any complications so that they can provide supplemental medication to help the immune system fight off infection as quickly as possible.”

American Heart Association (https://www.lung.org/lung-health-diseases/lung-disease-lookup/cystic-fibrosis/treating-and-managing)

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.”