Minutes from one of our Support Group meetings.

Third Raleigh Duke Support Meeting February 3, 2016

Mary Tate, our group leader, assisted by Rose Marie Smigla from the Duke Hospital Pulmonary staff, opened the meeting promptly at 10:00AM at the St. Thomas More Catholic Church ( 940 Carmichael street, chapel Hill, NC ,27514) by reminding the attendees about the standing guidelines and agreements about the conduct of our meetings:

1- Whatever discussed in our group will be considered confidential and only shared by permission. 2- Support meetings are held every other month unless the attendees vote to change that. 3- Everyone is invited to attend and bring interested friends. 4- Copies of previous meeting minutes are available by sending a request to Mary Tate – mtategraham@embarqmail.com. 5- We ask every interested person to provide an email address so we can communicate news about future meetings.

The general topic for this meeting is a presentation by Doctor Lake Morrison, assistant Professor and Program Director, Dule Pulmonary Directorate, His topic is entitled “Cough, Cough” an issue all patients of Interstatial , Ideopathic Pulmonar Fibrosis deal with. (This was an important and detailed discussion. Please bear with me if I misspell or erroneously state an item… Jerry),

This topic deals with various exposures that aggravate the IPF condition to such as drugs, tobacco, dust and inorganic sources such as asbestos , and mold, and bacterial sources (birds, etc.). The term “Idiopathic “ generally refers to the fact that we may not clearly trace the condition of pulmonary scarring to a single common cause; but observations are that scarring begats scarring ( continues to spread and grow worse over time). The tissue inside and between air sacks becomes thick , stiff, and scarred. As the lung tissue becomes scarred, oxygen cannot pass through to the red cell, which

carries oxygen. This creates hypoxia, creating a reduction in all body tissues including the heart and the brain. Once the lung tissue has become scarred, the damage cannot be reversed. Thus, paying attention to the symptoms for the disease is important… the consequence of which is that various factors contribute to the point of being Interstation Pulmonary Fibrosis with attendant and troubling coughing. Our discussion involves trying to identify what causes coughing.

Dr Morrison sketched an outline of a typical body with graphical representation of the location of individual receptors that are tied to vega nerves that are tied to the medulla in the brain that connect to nerves that deal with how we breath.

Inside the brain-lung system:

a) Pneumonia-flu, colds, spicy foods, etc.

b) Esophageal-gastro reflex in the lungs.

c) Sinus and post nasal drip … leaks into the airways.

d) Brain stimulated coughing

Receptors outside the lungs

a) Inhaling smoke, scents perfumes, exhaust

b) Exercise – exercise induced cough ( related to a drop in Oxygen level)

c) Talking and laughing

d) Changes in temperature and humidity

Some statistics related to this discussion … frequency, within our US general population

Asthma 62 new cases per day

COPD , 118 new cases per day

IPF , 226-520 new cases per day

General impact information

We are still learning researching and about detecting and treating this disease

– For some reason, IPF patients don’t seem to cough much during sleep.

– The condition impacts your daily exercise day.

– Coughing frequently happens when our oxygen level drops … when this happens it detracts from your life span

Fundamental Question : Why do patients cough ?

It’s the scarring in the lungs that causes that. The distortion in the airways triggers

receptors that lead to irritating coughing.

– Doctors sometimes prescribe low dose Prednesone (5-10 mg range) and anti-fibrotic

drugs in this range.

– Reflex is a big thing ; specifically acid that enters the esophagus. The goal is to suppress that acid. Dr Morrison drew a diagram to show that as the lung shrinks and the stomach gets smaller, the stomach creates a hiatal hernia that pushes up into the chest. Three things a typical patient can do to “manage” this condition:

1- Diet – use less chocolate

2- Diet – lose weight

3- Eat smaller meals

4- Elevate the bed while sleeping …3-4 inch wedge, and use several pillows

– Sleep Apthena – the use of sleep mask equipment helps the system to work while we sleep when the airways get smaller, in cases where the chest is still moving but the patient intermittently stops breathing. Low levels of Oxygen contribute to scars which supports to herniation.

– Another cause for cough is Nasal Dripping often treated using Flonase spray. Other tools the Doctor uses to reduce cough (based on individual patient conditions) are various narcotics such as : Tessalon Pearl pills, McClariphan, Dextrophic Clariphan, Menthol, Gabba Penton. Dr Morrison stated that while these medicines are useful in reducing coughing, they don’t “fix the lung problem”.

– The morning cough ( to clear morning secretions that pool) is a very common problem for IPF patients. A teaspoon of Codine can be helpful, but the negative side is it can lead to an addiction. NAC is an anti-oxidant that has been used and was thought to be useful for IPF until a 2004 study confirmed that NAC is not a recommended solution.

Some patients have a dry morning cough; some have a white substance cough. Coughing is an interesting reflex, but for the most part the cough won’t eliminate the stimuli. Sometimes a small dose of honey bourbon with lemon can be useful in the management of the automatic brain response ( i.e. I’ve experienced that reaction before … I’ve got to get ready to deal with that) “There’s nothing intrinsically about a cough that tells us where it’s from”

– IPF is still quite rare. It starts at the far reaches of the lungs ( typically crackles heard at the bottom of the lungs) . There are a variety of areas that are under review;

– tobacco infections with some notion of genetic susceptibility

– Genetic codes and questions about stem cell and bone marrow research, the idea that we might be able to use stem cells to repair genetic conditions( essentially an “unpainted canvas”).

Before closing his presentation, Dr Morrison commented that there are items you can provide to assist in diagnosing your personal situation.

1- What has happened (to you) in the last three months? Be prepared to talk about this.

2- What medicines are you taking?

3- What is happening concerning your quality of life?

4- Are there any new symptoms you are experiencing?

5- Provide a chronology ( a diary) of what has happened to you over time.

These minutes were prepared to the best of my ability by Jerry Moon.

Mary Tate provided a special cake during the refreshments period to honor the retirement of Elaine Pelletier, a pulmonary triage nurse, from Dr Morrison’s Duke Pulmonary Clinic. Elaine has been the “lifelink” for many. She has announced that she will remain active with Pulmonary Fibrosis issues and our support group. She has already posted an active blogsite on this issue.

Everyone should access this blog for current, pertinent information. See https://pulmonaryfibrosistogether.com/home/

Why Pulmonary Rehab?

Pulmonary Fibrosis is a disease caused by scarring in the lungs. The tissue inside and between the air sacs becomes thick, stiff and scarred. As the lung tissue becomes scarred, oxygen cannot pass through to the red blood cell, which carries oxygen. Creating hypoxia, a reduction in oxygen to all your body tissue. Once the lung tissue becomes scarred, the damage cannot be reversed. So what can we do to maintain our quality of life?

Let’s start by looking at pulmonary rehabilitation. Pulmonary rehabilitation is a comprehensive, multidisciplinary program that uses a combination of teaching, counseling, and behavior modification techniques to improve self-management, reduce symptoms, optimize functional capacity, and increase participation in patients with all kind of lung disease. Typical PR programs offer an intense component (variable time period, often 6–10 weeks) followed by a maintenance component for those patients who wish to continue to participate. Usually, rehabilitation staff performs a comprehensive assessment of patients’ physical strengths and deficiencies along with their emotional and other needs prior to enrollment. An exercise and overall intervention (e.g., psychosocial, nutritional) program is tailored to the individual, and the patient returns to the center 2–3 times per week to complete it in a setting with close monitoring by staff—usually physical or respiratory therapists.

Components of pulmonary rehab target many of these limiting factors. For example, pursed-lip breathing aims to decrease respiratory rate (prolong expiration) and dyspnea while increasing tidal volume (amount of oxygen the lungs will hold) and oxygen saturation. The exercise component of pulmonary rehab has several potential beneficial effects including improving cardiac conditioning, increasing fat-free body mass, making the quadriceps more fatigue resistant; and enhancing the efficiency of skeletal muscle function at the cellular and molecular levels.

From a clinical outcomes standpoint, pulmonary rehab has been shown to not only improve exercise capacity—as measured by distance walked during a 6-min walk test or by oxygen uptake during a maximal cardiopulmonary exercise test. Moreover, emerging data suggest that pulmonary rehab improves psychological adjustment and cognitive impairment in patients with pulmonary fibrosis.

A large study shows that pulmonary rehab will improve functional capacity (as measured by 6-min walk), dyspnea for a given level of exertion, anxiety, mood, fatigue, and better quality of life in patients with pulmonary fibrosis. The chart below shows the results of pulmonary rehab and how it can improve quality of life (QOL).

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Exercise is a well-documented safe and effective intervention for prevention and rehabilitation of chronic lung diseases patients.

Often without pulmonary rehab patients start to loose their ability to exercise, which increases the level of dyspnea, depression, anxiety, and fatigue. These patients often see a decline in their quality of life and become more dependent on their caregivers. They have more trouble bouncing back from a flare.

Facts that pulmonary fibrosis patient need to keep in mind.

  • Exercising may require more oxygen at first. Keep moving. Allow the respiratory therapist or nurse to increase the amount of oxygen you need during exercise. Often this is a sign of deconditioning, and can be corrected. No one is telling you that you will come off oxygen. Remember everyone is oxygen dependent since taking their first breath.
  • Exercise in a temperature controlled environment. Too hot or too cold uses more oxygen, increasing oxygen needs is not healthy for a chronic lung disease individual.
  • Pulmonary rehab is not just walking. It is weight training, muscle building and education on nutrition and pursed lip breathing. All of these elements help you use the oxygen in the blood stream more efficient.
  • Pulmonary rehab is not completed; it is a life long program. After you have completed the course, normally about 8-12 weeks, it is advised to start a maintenance program. The more you do, the more you will be able to do!
  • Weight loss or maintaining a healthy weight is critical. Pulmonary rehab can help with this goal.
  • Depression. Pulmonary fibrosis is a debilitating chronic fatal disease. Patients often suffer with depression without even realizing the change in their mood. Exercising enhances the action of endorphins, chemicals that circulate throughout the body. Endorphins improve natural immunity and reduce the perception of pain. They may also serve to improve mood. Another theory is that exercise stimulates the neurotransmitter norepinephrine, which may directly improve mood.
  • An exercise regiment can improve cardiopulmonary, musculoskeletal, and psychosocial.

If you are not in pulmonary rehab, call your physician, ask for a referral. Then let’s get moving…….. YOU CAN DO IT.

 

PulmonaryFibrosisTogether.com support group will be meeting on Friday Feb 3, 2017.  Add your email address to this blog if you would like to get reminders and notices regarding this blog and support meetings.

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Pulmonary Fibrosis: diagnosis, causes and treatment.

What is pulmonary fibrosis?

Pulmonary fibrosis is a disease caused by scarring in the lungs. The tissue inside and between the air sacs becomes thick, stiff and scarred. The scarring is referred to as fibrosis. As the lung tissue becomes scarred, oxygen cannot pass through to the red blood cell, which carries oxygen. Creating hypoxia, a reduction in oxygen to all your body tissue. Once the lung tissue becomes scarred, the damage cannot be reversed.

In some cases, the cause of pulmonary fibrosis can be found. Most cases of pulmonary fibrosis have no known cause.

Possibles Causes

In most cases, there is no know cause of pulmonary fibrosis. So your doctor my refer to it as idiopathic. In other cases, there maybe so possible causes:

  • Cigarette smoking
  • Certain viral infections
  • Exposure to environmental pollutants, including silica and hard metal dusts, bacteria and animal proteins, gases and fumes
  • The use of certain medicines
  • Some chemotherapy treatments
  • Genetics. Some families have at least two members who have pulmonary fibrosis. At this time no markers have been found.
  • Gastroesophageal reflux disease (GERD). GERD is a condition in which acid from your stomach backs up into your throat. Some people who have GERD may breathe in tiny drops of acid from their stomachs, which may injure the lungs.

Sign and symptoms

  • Shortness of breath, particularly during exercise
  • Dry, hacking cough
  • Fast and/or shallow breathing
  • Gradual unintended weight loss
  • Fatigue
  • Aching joints and muscles
  • Clubbing (widening and rounding) of the tips of the fingers or toes

Diagnosis

There are many types of lung disease that can cause pulmonary fibrosis. It is important that your doctor refers you to a specialist to try and make the right diagnosis. Your doctor will do a physical exam. Your doctor may ask about your history of smoking, things in the air at your job that could irritate your lungs, your medication history, other medical conditions, your family’s medical history, and your hobbies.

Other tests for pulmonary fibrosis may include:

  • Chest X-ray
  • Breathing tests to determine how much lung damage is present
  • Blood tests to check for oxygen levels in your bloodstream, autoimmune disease, and for possible infections
  • High resolution CT scan of chest (HRCT)
  • Lung biopsy if diagnosis not able to be made by HRCT. The biopsy is done as a surgical procedure and removes small pieces of lung tissue to find a diagnosis.
  • Exercise testing to find out how well your lungs move oxygen and carbon dioxide in and out of your bloodstream. Sometimes referred to as a 6 minute walk.

Once all of these test have been resulted, your doctor will develop a diagnosis.

Treatment

There is no cure for pulmonary fibrosis. Once the scarring has formed there is no reversal. Current treatments are aimed at slowing the course of the disease, relieving symptoms, including oxygen and helping you stay active and healthy. Treatment cannot reverse lung damage that has already occurred.

Treatments include:

Medicine. Depending on the type of pulmonary fibrosis you have, there may be medications to treat you. Only your medical provider can determine if any medications may be beneficial for you.

Many people diagnosed with pulmonary fibrosis will be prescribed a corticosteroid (prednisone) to reduce inflammation. Corticosteroids can have some serious side effects, especially if used long-term, and need to be carefully monitored. Corticosteroids are not indicated for all types of pulmonary fibrosis. Other medicines sometimes prescribed to reduce inflammation are azathioprine and cyclophosphamide, which suppress the immune system.

The FDA has approved two drugs for one type of pulmonary fibrosis, idiopathic pulmonary fibrosis IPF. They are nintedanib (Ofev®) and pirfenidone (Esbriet®, Pirfenex®, Pirespa®). Both have been shown to slow the progressive decline in lung function in patients with IPF.  These drugs are approved for patients with mild, moderate, and severe IPF.

Oxygen therapy may be prescribed if the amount of oxygen in your blood gets low. Supplemental oxygen therapy may help reduce your shortness of breath and make it easier for you to stay active.

Pulmonary rehabilitation is a program that teaches you about your lung disease, how to exercise and how to manage your disease, and provides support and counseling.

Lung transplant evaluation should be recommended early after diagnosis by your physician. To be considered eligible for a lung transplant, you will need to be in good physical condition besides your lung function, have no other life-threatening illnesses, and be willing to accept the risks of surgery and be compliant with subsequent medical treatment.

 

Finding an oxygen company.

Welcome  back! On our last blog we discussed when you need to start wearing oxygen. Now let’s talk about oxygen companies, how they are alike and how they are different.

Not all oxygen companies are alike. More importantly not all lung disease are the same. In very simple terms, pulmonary fibrosis is a scaring of the lungs. Oxygen can not cross the scared tissue to the get into the blood cells. For this reason, pulmonary fibrosis suffers often require high doses of oxygen. With this in mind, you need to be smart about the company you get your oxygen from.

Here are some important questions you need to ask before you choose an oxygen supplier.

1- What do you have in the form of Portable Oxygen Concentrators (POC)? In early stages of PF, you may be able to use low dose oxygen; 2-4 liters per minute (LPM). A POC is easier to get around outside your home. Most POC offer pulsating doses of oxygen. This means the oxygen is supplied to you in spurts or shooting doses. These concentrators are about the size of a ladies purse, and weight about 4-5 pounds. Most come with an AC and DC plugs, making them great for travel. They are also FDA approved for air travel. (More about this in another blog).

Cons: A POC sounds like the perfect solution; however, not all PF suffers can use the pulsating dose. So often PF suffers need a continuous flow of oxygen. Before you accept any POC device; request a trail period. Then get moving. Day one start with a brisk 6 minute walk. Wear a pulse oxiometer to be sure your saturation do not drop below 88%. No fair stopping to caught your breath if you see your saturation drop. This simple means you cannot use a pulsating dose. Send it back. You are only hurting yourself if you are not getting the correct oxygen dosing. (remember you have been addicted to oxygen from the first breath).

2- Can you continue to supply me oxygen if I move or travel out of the state? I know you are not planning to move; however, one of the treatments for PF is a lung transplant. You may not have a transplant hospital in your area. Or you make need to move closer to a different transplanting hospital. Let’s do a what if: What if you want to go to a wedding 2000 miles from home. A good oxygen company will place a stationary concentrator at the location you will be moving/traveling too. It will be a loaner at no charge. If you are  moving can they still supple you with oxygen tanks for portability?  (once you reach this stage of the disease process you will need tanks only).

Cons: If you are on Medicare or disability you may not be able to change oxygen companies during your disease process. This is due to the federal laws regarding how Medicare pays. In short, after you have been on oxygen for 2 years with Medicare or disability you can not change oxygen companies for the next 3 years. This is the standard of Medicare as of 2017. We are all in hopes this gets better in the future, but it has been true for the last 5 years plus.

3-What is the maximum liter flow you can supply in stationary oxygen? Don’t be surprised if they stumble with this question. See most companies are not willing to put two 10 liters per minute concentrators in your home. As your disease progresses you are going to need up to 15 LPM That requires two concentrators connected together. Some companies will not do this, look for one that will.

Cons: If you are not able to find one that will, ask your doctor what is available in your area. At Duke, it took a lot of work on the part of the triage nurses to get this high liter flow. But it can be done.

4-Does this company accept your insurance? Oxygen companies are just like doctor’s offices when it come to being out of network. The same is true with your copay and out of pocket expenses. My advice to you is check your health insurance policy really well. Your lung disease is going to be life long and treatment can be expensive.

 

 

 

When do I need to wear oxygen?

At our last support group we were blessed to have Lincare Oxygen Company come by and discuss oxygen with us. The support group discussed portable oxygen concentrator vs oxygen tanks. We also talked about pulsating dose vs continuous flow oxygen. All this information is great, assuming you know when you need to wear the oxygen your physician ordered.

Let’s step back and  look at when you need to be thinking about using your oxygen.  What sort of sign and symptom will you see; and how will you feel? What does the 6 minute walk test really saying? Let’s look at Mr X and the issues he experienced when he first was told to wear oxygen.

Case Study: Mr X is Short of Breath

Mr X just completed a 6 minute walk. This is the results of his test. On room air  his saturation are 94%.  He started his walk on room air, however at 1 minutes 53 seconds, his saturation dropped to 86% and Mr X was placed on 2 liter per minute.

He continued his walk on 2 liter per minute and at 2 minutes 40 seconds his saturation dropped to 87% and Mr X was placed on 4 liter per minute. Mr X was able to complete the 6 minute walk on 4 LPM of continuous flow oxygen with his saturation greater than 88%.

Several days later, Mr X’s wife calls saying he is short of breath. He reports wearing his oxygen when he is exercising. But does not think he needs it while he is just walking around the home.  Sometimes Mr X gets short of breath walking to the mailbox, or talking  a shower. He is looking for an inhaler that will reduce the shortness of breath.

Mr X heard he was to wear his oxygen when exercising. Which is true, however, in only 1 minute and 53 seconds of walking without oxygen his saturation dropped below the save range of 88%. In this case, we need to ask; what can Mr X do in  1 min and 53 seconds?   Answer is not much. Mr X needs to be wearing his oxygen almost all the time. Walking to the mailbox, takes longer than 2 minutes for him, which is why he needs his oxygen to go to the mailbox.

So how much oxygen should Mr X be wearing? Based on his 6 minute walk, if he is going to be walking for about 3 minutes, he needs to be on 4 liters per minute. Mr X shortness of breath can be taken care of by using the correct liter flow of his oxygen. The right amount of oxygen is important for Mr X’s health. From the minute we were born, every person is addicted to oxygen. Some just need more than others.

Please tell me and others your oxygen story. After all we are all here to learn from each other. Feel free to ask any questions. I will do my best to respond in a timely manner.

 

Our next Pulmonary Fibrosis Support Group is Feb 3rd, 2017.