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QUOTE FOR THURSDAY:

“Waldenstrom macroglobulinemia is a rare slow-growing cancer that affects your blood cells. It’s a type of non-Hodgkin’s lymphoma. (Healthcare providers may refer to this condition by the initials WM or the name lymphoplasmacytic lymphoma). WM happens when genetic mutations change certain blood cells, setting up a chain reaction of medical conditions that may be life-threatening. Providers can’t cure this condition, but they do have treatments that ease and sometimes eliminate its symptoms.”

ClevelandClinic.org (Waldenstrom Macroglobulinemia (Lymphoplasmacytic Lymphoma)

Part I Waldenstrom’s Macroglobulinemia (WM) also referred as Lymphoplasmacyctic Lymphoma (LPL)

Affects older persons; peak incidence between 6 -7th decades. Tumor cells secrete an M protein (commonly IgM) Mixture of B cells ranging from small lymphocytes to plasmacytic lymphocytes to plasma cells. Involves lymph nodes, bone marrow, and spleen at presentation.

Initially, you will need to learn some basics about Waldenstrom’s macroglobulinemia (WM), sometimes referred to as a lymphoplasmacytic lymphoma (LPL).  Newly diagnosed?  Well striveforgoodhealth.com understands that this is a very difficult time for you. Not only are you, your family and your friends experiencing the gamut of different emotions that come with a cancer diagnosis, but there is so much new information to learn.

Here’s one place to start in getting information on this diagnosis!

Let’s understand first what is effected in the body and answer the questions that probably you are asking yourself  (pt or family or friends).

What is effected in this disease? 

B Lymphocytes are effected; they are a type of white blood cell that makes antibodies. B lymphocytes are part of the immune system and develop from stem cells in the bone marrow. Same with T cells.  T- and B-cells are highly specialized defender cells – different groups of cells are tailored to different germs. When your body is infected with a particular germ, only the T- and B-cells that recognise it will respond. These selected cells then quickly multiply, creating an army of identical cells to fight the infection. Special types of T- and B-cells ‘remember’ the invader, making you immune to a second attack.

What’s so special about the B cells?

With the help of T-cells, B-cells make special Y-shaped proteins called antibodies. Antibodies stick to antigens on the surface of germs, stopping them in their tracks, creating clumps that alert your body to the presence of intruders. Your body then starts to make toxic substances to fight them. Patrolling defender cells called phagocytes engulf and destroy antibody-covered intruders.

With this disease what happens to the B Cells?

When diagnosed with Waldenstrom’s macroglobulinemia (WM) which is a lymphoma or cancer. It affects a type of white blood cell called a B-lymphocyte or B-cell, which normally matures into a plasma cell whose job is to manufacture immunoglobulins (antibodies) to help the body fight infection. In WM, there is a malignant change to the B-cell in the late stages of maturing and it continues to proliferate as a clone of identical cells, primarily in the bone marrow, but also in the lymph nodes and other tissues and organs.  It is known as lymphoplasmacytic lymphoma (LPL) and must be associated with the production of an antibody protein or immunoglobulin known as IgM in order for WM to be diagnosed.

What’s the difference of WM versus LPL?  Are they the same disease? 

Sometimes WM and LPL (lymphoplasmacytic lymphoma) are used interchangeably although WM is really an LPL. However, WM comprises about 90 – 95% of all LPL patients.

The cancer cells of LPL have the appearance of both B-lymphocytes and plasma cells, hence the term lymphoplasmacytic.  LPL cells can secrete immunoglobulin antibodies (IgM, IgA, IgG, IgE or IgD), but those who secrete IgM are called WM.  Currently, the clinical term in cases of LPL with circulating monoclonal IgM is WM.  LPL is the term that describes the appearance of the bone marrow or lymph node used by pathologists.
My doctor said WM was a rare disease. How rare is it?
What does that mean for me?
WM is a rare cancer seen only in approximately three to five per million people per year with about 1500 new cases diagnosed in the US each year. This disorder is age-dependent and is quite rare under the age of 40 (less than 1 percent
of patients).  Typically, patients present between the ages of 60 and 70. For reasons that are unclear, WM is almost twice as common in men as in women and is more common in Caucasians than other ethnic groups. There is a familial predisposition to WM, with most studies suggesting that approximately 20-25% of patients have a first degree relative with WM or other B cell disorders. WM is a rare disease and as such it does not command much support for research dollars because there are few financial incentives for pharmaceutical companies; as much as I hate to document this.
Is there a cure for WM?
 
No, although quality of life and survival for WM patients are continuing to improve because of better treatments.
How long do I have left to live?
Although WM is incurable, in most cases it can be effectively treated to provide a good quality of life for many years.
In most patients, WM is a fairly indolent, chronic disease.

The median survival has varied in studies, from 5 years to 10

years. Median survival suggests that half of all patients survive 5 to 10 years. Another way to answer this question is to look at the 5
-year survival rate. The 5-year survival rate tells you what percentage of people live at least 5 years after the
cancer is found. Percent is how many out of 100. The 5
-year survival rate for people with Waldenstrom’s
macroglobulinemia is about 75%.
However, it is important to note that survival rates vary based on a number of individual factors including the patient’s age and whether the patient has other medical problems.
It is also important to remember that statistics on the survival rates for people with WM are an estimate.
As newer agents and treatments that
are more effective and less toxic become available, the life expectancy will continue to increase. The main causes of
death because of WM include disease progression, transformation to high-grade lymphoma, or complications of
therapy. However, because of the advanced age of patients with WM, many will die of unrelated causes.

 

 

 

QUOTE FOR WEDNESDAY:

“Dietary and lifestyle changes are the first step in treating GERD. Certain foods make the reflux worse. Suggestions to help alleviate symptoms include:

  • Lose weight if you are overweight — of all of the lifestyle changes you can make, this one is the most effective.
  • Avoid foods that increase the level of acid in your stomach, including caffeinated beverages.
  • Avoid foods that decrease the pressure in the lower esophagus, such as fatty foods, alcohol and peppermint.”

John Hopkins Medicine (Gastroesophageal Reflux Disease (GERD) Treatment | Johns Hopkins Medicine)

Part II How to stay healthy with acid reflux-GERD.

 

Understanding what GERD is, its causes, its symptoms, the effects it has on your body and the way your body responds to specific treatments is necessary to help you devise a plan with your activities of daily living, including diet, to help manage the condition. If you haven’t seen my Part 1 just click on it onto my webpage than read yesterday’s article part 1 than read this. All you can do is apply your own GERD diet experimentation to the suggestions of others and try available treatments until you find what works for you. Also, there are foods that hve a reputation for being ‘safe’ (foods to avoid acid reflux) in most cases, as well as others that are known for being main contributors to acid reflux.

I recommend you go tohttp://www.gerd-diet.com which helps explain about foods and how they work with GERD. They do state the following:

Armoring Yourself with Patience and Willingness to Experiment (meaning this takes time in finding out what exacerbates your GERD and what helps it heal—Like an open wound, it takes time.) Sorry,There Is No One-Size-To-Fit-All With-A-GERD Diet (for relief or resolution)

Your Own Trigger Foods.
Given the complex nature of GERD, this one has to be added. I know it is repetitive, but everyone has certain foods that irritate their condition and causes severe symptoms. Make a list of these foods and avoid them in the future. This means make a journal for the next 2 weeks or more and see what caused your GERD symptoms to remember them with not eating fast which can cause GERD also (habit change).

GERD Diet FoodsA List of Food Groups and What Is Safe and Best to Avoid The following lists are comprised of foods that are typically known to be ‘safe’ or to ‘trigger’ symptoms.

Again, just because something is on the “avoid” list does not mean that you cannot eat it. Similarly, something appearing on the “safe” list does not guarantee that it will not cause a problem for you. This is simply provided as a guideline to give you a place to start and to make it easier for you design your own personal GERD diet.

 

 

FOOD GROUPS

 

SAFE

 

AVOID

FRUITS Apples (fresh and dried), apple juice, bananas, pears, peaches, melons, strawberries, grapes Oranges and orange juice, lemons and lemonade, grapefruit and grapefruit juice, tomato and tomato juice, cranberries and cranberry juice
VEGETABLES Baked potato, broccoli, cabbage, carrots, green beans, peas, asparagus, lettuce, sweet potatoes Raw onions, peppers, radishes, french fries, mashed potatoes
MEAT Extra lean ground beef, steak (London Broil), skinless chicken breast, egg whites or substitute, fish (with no added fat), white turkey meat Fatty ground beef, marbled sirloin, chicken nuggets, buffalo wings, fried meat
DAIRY Feta or goat cheese, fat free cream cheese, fat free sour cream, low fat soy cheese Whole milk, chocolate milk, ice cream, high fat cream cheese or sour cream
GRAINS Cereal (bran or oatmeal), corn bread, graham crackers, pretzels, rice (brown or white), rice cakes, millet, quinoa High fat grain products (cheese bread or products made with whole milk)
BEVERAGES Water, herbal teas, non-citrus drinks, skimmed milk Caffeinated beverages, whole milk, alcohol, carbonated beverages
FATS/OILS/CONDIMENTS Low fat salad dressing, herbs ( basil, thyme, sage, oregano), hummus, mild sauces Mustard, chili sauces, creamy salad dressing, black pepper, vinegar, curries, pickles, mint

 

At this point, there is no alternative medicine therapies that have been accepted as proof for treatment(s) or 100% resolution for GERD.

However, like every other aspect of the disease, it really is an individual decision. Some people have reported natural remedies that have given amazing results with their GERD diet (healthier approach) while others find that prescription medications have little effect (meds can cause side effects temporary or permanently, depending on what it is).

GERD can be due obesity, lifestyle (unhealthy habits) and diet.

**It is recommended to see an MD first before making any decision on your own in treatment with a combination of or just one of the following: meds, diet or even health habit changes, especially for those people with disease/illness (s) present (Ex. Diabetes, cardiac disease, etc…).** 

 

 

QUOTE FOR TUESDAY:

“Gastroesophageal reflux disease, or GERD, happens when acid from your stomach backs up into your esophagus, the tube connecting your stomach to your mouth.

This digestive disorder affects the ring of muscle between your esophagus and your stomach. This ring is called the lower esophageal sphincter (LES). If you have GERD, you may get heartburn or acid indigestion. Doctors think that some people may have it because of a condition called hiatal hernia. In most cases, you can ease your GERD symptoms through diet and lifestyle changes. But some people may need medication or surgery.”

Web M.D. (WebMD.com/GERD: Symptoms, Causes, Treatments, Remedies for Relief)

What is GERD or ACID REFLUX? Learn the symptoms, complications, risk factors, & treatments.

What is acid reflux?

A gastrointestinal (GI) disease that is caused by a valve located between the esophagus and stomach, which normally is a strong muscular ring of tissue. This valve normally opens and closes completely preventing food backing up (called reflux) in the esophagus allowing the contents to reach the throat to the mouth. With acid reflux, what happens is this valve gets over expanded frequently to the point the valve gets overstretched and no longer fits over the opening between the esophagus and the stomach with a tight fit. Due to loss of the elasticity it now is allowing leakage from the stomach with both food and fluids going up the esophagus to the throat to the mouth due to the action of reflux, during the digestion process. What happens during digestion the stomach contents with digestive fluids from the stomach (which are acidic) are refluxed, that are NOT normally in the esophagus. In conclusion, reflux sends stomach contents that’s returning (an acidic environment) upward in the esophagus which is now in an environment not use to being exposed to the digestive fluids=acidity, which are needed to perform the digestion process of our foods/fluids that are in the stomach only. This leakage of the valve is the cause of this problem occurring=GERD or GASTROINTESTINAL REFLUX.

When you don’t have GERD food and fluids, the nutrients we swallow goes down the esophagus to the valve where it opens letting the contents into the stomach.  Than digestion takes place in about 1-2 hours after eating with no feeling of heartburn or foods or fluids we ate don’t come back up in our throat to the mouth. Normally the valve is tight enough in preventing reflux=no leakage going back up to the mouth with food and acid during digestion (the primary purpose of its function).  This means during digestion the food gets into the stomach which is broken down into smaller particles by the digestive acid fluids allowing the nutrients to pass into the bloodstream with the waste products staying in the stomach to reach the rectum.  So when stomach digestion is complete it passes all the waste products onto the smaller intestines to the larger intestines to the rectum to be evacuated, without leakage or reflux.  With GERD this contents does go backward sending it to the mouth again.

The signs and symptoms (s/s) of GERD or acid reflux:

1-Heartburn is the classic GERD symptom. It’s best described as a burning sensation in the chest and/or discomfort in the upper belly or abdomen accompanied by a feeling of fullness.
2- Regurgitation is the involuntary return of partially digested food from the stomach into the mouth. This uncomfortable symptom is commonly caused by GERD, since the esophageal sphincter (valve) is damaged to such a severe degree that the stomach juices (acidotic) can freely reflux to the level of the throat or mouth.
3-Pain present behind the sternum (chest pain) to the upper mid abdomen (where the stomach is). If severe call your M.D. or doctor to have evaluated (especially if in the chest).
4-Chronic cough to hoarseness                                                                       5-Recurrent pneumonias                                                                         6-Bloating 7-Nausea 8-Vomiting (yellow/green)
9-Lump in the throat 10-Difficulty swallowing                                                   11-Chronic sore throat 12-Laryngitis 13-Post nasal drip
14-Ear Aches 15-Tooth decay or gingivitis (inflammation of the gums) or bad breath this is due to the acid fluids with the foods and fluids regurgitated back to the mouth from the stomach.

Complications that are caused by GERD when left untreated:

1-Narrowing of the esophagus called esophageal stricture. This is due to damage to cells in the lower esophageal from acid exposure that leads to scarring of the tissue. The scar tissue narrows the food pathway causing difficulty to swallow called dysphagia.
2-Esophagitis – inflammation of the esophagus. This constant backwash of acid can irritate the lining of your esophagus. Over time, the inflammation can cause complications such as bleeding or breathing problems leading into the next problem. 3-Esophageal Ulcers – Due to frequent exposure to acidic fluids and foods to the esophagus the mucosa gets irritated so bad it will even erode the mucosa causing skin ulceration. The esophagus environment is not use to the stomach’s = acidic. Take the outer skin of the body, if exposed long enough to acidic chemicals the skin will burn. Same principle for the esophagus constantly exposed to the environment of the stomach’s content of acidic fluids every time digestion takes place.
4-Precancerous changes to the esophagus (Barrett’s esophagus). In Barrett’s esophagus, the color and composition of the tissue lining the lower esophagus change. These changes are associated with risk of esophageal cancer. The risk of cancer is low. Cancer is rare but can happen (adenocarcinoma of the esophagus).

Risk factors:

Conditions that increase the risk of GERD would
include: Obesity, Pregnancy, Smoking, Dry Mouth, Diabetes, Asthma, Connective Tissue Disorders like scleroderma, delayed stomach empting, Zollinger-Ellison syndrome (ZES) (This is a rare disorder characterized by one or more tumors in the pancreas, duodenum, or both. The tumors cause the stomach to make too much acid, leading to peptic ulcers in the duodenum. The tumors are sometimes cancerous and spread to other areas of the body.).

Treatment:

The key to treatment is prevention but if already diagnosed GERD than it would be maintenance. There is no one answer but start with being checked by your physician if you have any symptoms indicative of this diagnosis. Start with a getting a very good diagnostic tool ordered by your doctor called an Upper GI series (endoscopy) and when it’s done it will tell the M.D. a lot in what’s going on.

Then there is medications as a remedy, that can be useful, they are classified as proton pump inhibitors to H2 Inhibitors with more.

Another great key to the treatment is your LIFESTYLE=Diet (not eating acid foods, not eating fast), activity/exercise, your height compared to your weight (BMI or simply what you weigh) and lastly if you practice healthy vs. unhealthy habits.

If you would like to learn more about this come back tomorrow to my web page when I go further on the topic GERD (part 2) regarding the diet for the disease.

QUOTE FOR MONDAY:

“Idiopathic pulmonary fibrosis (IPF) is considered a rare, sporadic disease. According to the National Institutes of Health (NIH), about 100,000 people in the United States have IPF. Approximately 30,000 to 40,000 new cases are diagnosed each year. Worldwide, IPF affects 13 to 20 out of every 100,000 people.

IPF can be difficult to diagnose, mainly because there are few if any symptoms in its earliest stages. Additionally, symptoms of IPF — such as a dry, hacking cough, shortness of breath, and chest discomfort — mimic other conditions.

However, with IPF, breathing becomes so difficult that even being at rest is straining on the body. Other common symptoms include extreme fatigue and clubbing, where the fingertips and nails are enlarged and rounded. People with IPF may also experience unintended weight loss or aching muscles and joints.”

Healthline (www.healthline.com/IPF: Statistics, Facts, and You)

Part II Pulmonary Fibrosis Awareness – The symptoms, complications, how its diagnosed and the treatments for it.

Symptoms

Signs and symptoms of pulmonary fibrosis may include:

  • Shortness of breath (dyspnea)
  • A dry cough
  • Fatigue
  • Unexplained weight loss
  • Aching muscles and joints
  • Widening and rounding of the tips of the fingers or toes (clubbing)

The course of pulmonary fibrosis — and the severity of symptoms — can vary considerably from person to person. Some people become ill very quickly with severe disease. Others have moderate symptoms that worsen more slowly, over months or years.

Some people may experience a rapid worsening of their symptoms (acute exacerbation), such as severe shortness of breath, that may last for several days to weeks. People who have acute exacerbations may be placed on a mechanical ventilator. Doctors may also prescribe antibiotics, corticosteroid medications or other medications to treat an acute exacerbation.

Complications

Complications of pulmonary fibrosis may include:

  • High blood pressure in your lungs (pulmonary hypertension). Unlike systemic high blood pressure, this condition affects only the arteries in your lungs. It begins when the smallest arteries and capillaries are compressed by scar tissue, causing increased resistance to blood flow in your lungs.This in turn raises pressure within the pulmonary arteries and the lower right heart chamber (right ventricle). Some forms of pulmonary hypertension are serious illnesses that become progressively worse and are sometimes fatal.
  • Right-sided heart failure (cor pulmonale). This serious condition occurs when your heart’s lower right chamber (ventricle) has to pump harder than usual to move blood through partially blocked pulmonary arteries.
  • Respiratory failure. This is often the last stage of chronic lung disease. It occurs when blood oxygen levels fall dangerously low.
  • Lung cancer. Long-standing pulmonary fibrosis also increases your risk of developing lung cancer.
  • Lung complications. As pulmonary fibrosis progresses, it may lead to complications such as blood clots in the lungs, a collapsed lung or lung infections.

Diagnosis

To diagnose your condition, your doctor may review your medical and family history, discuss your signs and symptoms, review any exposure you’ve had to dusts, gases and chemicals, and conduct a physical exam. During the physical exam, your doctor will use a stethoscope to listen carefully to your lungs while you breathe. He or she may also suggest one or more of the following tests.

Imaging tests

  • Chest X-ray. A chest X-ray shows images of your chest. This may show the scar tissue typical of pulmonary fibrosis, and it may be useful for monitoring the course of the illness and treatment. However, sometimes the chest X-ray may be normal, and further tests may be required to explain your shortness of breath.
  • Computerized tomography (CT) scan. CT scanners use a computer to combine X-ray images taken from many different angles to produce cross-sectional images of internal structures in the body. A high-resolution CT scan can be particularly helpful in determining the extent of lung damage caused by pulmonary fibrosis. Also, some kinds of fibrosis have characteristic patterns.
  • Echocardiogram. An echocardiogram uses sound waves to visualize the heart. It can produce still images of your heart’s structures, as well as videos that show how your heart is functioning. This test can evaluate the amount of pressure occurring in the right side of your heart.

Lung function tests

  • Pulmonary function testing. Several types of pulmonary function tests may be conducted. In a test called spirometry, you exhale quickly and forcefully through a tube connected to a machine. The machine measures how much air your lungs can hold and how quickly you can move air in and out of your lungs. Other tests may be conducted to measure your lung volumes and diffusing capacity.
  • Pulse oximetry. This simple test uses a small device placed on one of your fingers to measure the oxygen saturation in your blood. Oximetry can serve as a way to monitor the course of the disease.
  • Exercise stress test. An exercise test on a treadmill or stationary bike may be used to monitor your lung function when you’re active.
  • Arterial blood gas test. In this test, your doctor tests a sample of your blood, usually taken from an artery in your wrist. The oxygen and carbon dioxide levels in the sample are then measured.

Tissue sample (biopsy)

If other tests haven’t diagnosed the condition, doctors may need to remove a small amount of lung tissue (biopsy). The biopsy is then examined in a laboratory to diagnose pulmonary fibrosis or rule out other conditions. The tissue sample may be obtained in one of these ways:

  • Bronchoscopy. In this procedure, your doctor removes very small tissue samples — generally no larger than the head of a pin — using a small, flexible tube (bronchoscope) that’s passed through your mouth or nose into your lungs. The tissue samples are sometimes too small for an accurate diagnosis. The biopsy may also be used to rule out other conditions.The risks of bronchoscopy are generally minor and might include a temporary sore throat or discomfort in your nose from the passage of the bronchoscope. However, serious complications can include bleeding or a deflated lung.During bronchoscopy, your doctor may conduct an additional procedure called bronchoalveolar lavage. In this procedure, your doctor injects salt water through a bronchoscope into a section of your lung, and then immediately suctions it out. The solution that’s withdrawn contains cells from your air sacs.Although bronchoalveolar lavage samples a larger area of the lung than other procedures do, it may not provide enough information to diagnose pulmonary fibrosis. It might also be used to rule out other conditions.
  • Surgical biopsy. Although a surgical biopsy is more invasive and has potential complications, it may be the only way to obtain a large enough tissue sample to make an accurate diagnosis. This procedure may be done as a minimally invasive surgery, called video-assisted thoracoscopic surgery (VATS), or as an open surgery (thoracotomy).During VATS, your surgeon inserts surgical instruments and a small camera through two or three small incisions between your ribs. The camera allows your surgeon to view your lungs on a video monitor while removing tissue samples from your lungs. This procedure is performed after you’ve been given a general anesthetic, so you’ll be asleep during the procedure.During open surgery (thoracotomy), a surgeon removes a lung sample through an incision in the chest between your ribs. The procedure takes place after you’ve been given a general anesthetic.

Blood tests

Doctors may also order blood tests to evaluate your liver and kidney function, and to test for and rule out other conditions.

Treatments

The lung scarring that occurs in pulmonary fibrosis can’t be reversed, and no current treatment has proved effective in stopping progression of the disease. Some treatments may improve symptoms temporarily or slow the disease’s progression. Others may help improve quality of life. Doctors will evaluate the severity of your condition to determine the most appropriate treatment for your condition.

Medications

Your doctor may recommend newer medications, including pirfenidone (Esbriet) and nintedanib (Ofev). These medications may help slow the progression of idiopathic pulmonary fibrosis. Both medications have been approved by the Food and Drug Administration (FDA). Additional medications and new formulations of these medications are being developed but have not yet been FDA approved.

Nintedanib can cause side effects such as diarrhea and nausea. Side effects of pirfenidone include rash, nausea and diarrhea.

Researchers continue to study medications to treat pulmonary fibrosis.

Doctors may recommend anti-acid medications to treat gastroesophageal reflux disease (GERD), a digestive condition that commonly occurs in people with idiopathic pulmonary fibrosis.

Oxygen therapy

Using oxygen can’t stop lung damage, but it can:

  • Make breathing and exercise easier
  • Prevent or lessen complications from low blood oxygen levels
  • Reduce blood pressure in the right side of your heart
  • Improve your sleep and sense of well-being

You may receive oxygen when you sleep or exercise, although some people may use it all the time. Some people carry a canister of oxygen, making them more mobile.

Pulmonary rehabilitation

Pulmonary rehabilitation can help you manage your symptoms and improve your daily functioning. Pulmonary rehabilitation programs focus on:

  • Physical exercise to improve your endurance
  • Breathing techniques that may improve lung efficiency
  • Nutritional counseling
  • Counseling and support
  • Education about your condition

Lung transplant

Lung transplantation may be an option for people with pulmonary fibrosis. Having a lung transplant can improve your quality of life and allow you to live a longer life. However, a lung transplant can involve complications such as rejection and infection. Your doctor may discuss with you if a lung transplant may be appropriate for your condition.

QUOTE FOR THE WEEKEND:

“Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it harder for the lungs to work properly. Pulmonary fibrosis worsens over time. Some people can stay stable for a long time, but the condition gets worse faster in others. As it gets worse, people become more and more short of breath.”

MAYO CLINIC (Pulmonary fibrosis – Symptoms and causes – Mayo Clinic)

Part I Pulmonary Fibrosis Awareness Month-Risk Factors and Causes!

 

Pulmonary fibrosis is a lung disease that occurs when lung tissue becomes damaged and scarred. This thickened, stiff tissue makes it more difficult for your lungs to work properly. As pulmonary fibrosis worsens, you become progressively more short of breath.

The scarring associated with pulmonary fibrosis can be caused by a multitude of factors. But in most cases, doctors can’t pinpoint what’s causing the problem. When a cause can’t be found, the condition is termed idiopathic pulmonary fibrosis.

The lung damage caused by pulmonary fibrosis can’t be repaired, but medications and therapies can sometimes help ease symptoms and improve quality of life. For some people, a lung transplant might be appropriate.

Risk factors

Factors that make you more susceptible to pulmonary fibrosis include:

  • Age. Although pulmonary fibrosis has been diagnosed in children and infants, the disorder is much more likely to affect middle-aged and older adults.
  • Sex. Idiopathic pulmonary fibrosis is more likely to affect men than women.
  • Smoking. Far more smokers and former smokers develop pulmonary fibrosis than do people who have never smoked. Pulmonary fibrosis can occur in patients with emphysema.
  • Certain occupations. You have an increased risk of developing pulmonary fibrosis if you work in mining, farming or construction or if you’re exposed to pollutants known to damage your lungs.
  • Cancer treatments. Having radiation treatments to your chest or using certain chemotherapy drugs can increase your risk of pulmonary fibrosis.
  • Genetic factors. Some types of pulmonary fibrosis run in families, and genetic factors may be a component.

Causes

Pulmonary fibrosis scars and thickens the tissue around and between the air sacs (alveoli) in your lungs. This makes it more difficult for oxygen to pass into your bloodstream. The damage can be caused by many different factors — including long-term exposure to certain toxins, certain medical conditions, radiation therapy and some medications.

Occupational and environmental factors

Long-term exposure to a number of toxins and pollutants can damage your lungs. These include:

  • Silica dust
  • Asbestos fibers
  • Hard metal dusts
  • Coal dust
  • Grain dust
  • Bird and animal droppings

Radiation treatments

Some people who receive radiation therapy for lung or breast cancer show signs of lung damage months or sometimes years after the initial treatment. The severity of the damage may depend on:

  • How much of the lung was exposed to radiation
  • The total amount of radiation administered
  • Whether chemotherapy also was used
  • The presence of underlying lung disease

Medications

Many drugs can damage your lungs, especially medications such as:

  • Chemotherapy drugs. Drugs designed to kill cancer cells, such as methotrexate (Trexall, Otrexup, others) and cyclophosphamide, can also damage lung tissue.
  • Heart medications. Some drugs used to treat irregular heartbeats, such as amiodarone (Cordarone, Nexterone, Pacerone), may harm lung tissue.
  • Some antibiotics. Antibiotics such as nitrofurantoin (Macrobid, Macrodantin, others) or ethambutol can cause lung damage.
  • Anti-inflammatory drugs. Certain anti-inflammatory drugs such as rituximab (Rituxan) or sulfasalazine (Azulfidine) can cause lung damage.

Medical conditions

Lung damage can also result from a number of conditions, including:

  • Dermatomyositis
  • Polymyositis
  • Mixed connective tissue disease
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Sarcoidosis
  • Scleroderma
  • Pneumonia

Many substances and conditions can lead to pulmonary fibrosis. Even so, in most cases, the cause is never found. Pulmonary fibrosis with no known cause is called idiopathic pulmonary fibrosis.

Researchers have several theories about what might trigger idiopathic pulmonary fibrosis, including viruses and exposure to tobacco smoke. Also, some forms of idiopathic pulmonary fibrosis run in families, and heredity may play a role in idiopathic pulmonary fibrosis.

Many people with idiopathic pulmonary fibrosis may also have gastroesophageal reflux disease (GERD) — a condition that occurs when acid from your stomach flows back into your esophagus. Ongoing research is evaluating if GERD may be a risk factor for idiopathic pulmonary fibrosis, or if GERD may lead to a more rapid progression of the condition. However, more research is needed to determine the association between idiopathic pulmonary fibrosis and GERD.