CF 101: Lung Disease in Cystic Fibrosis

The first thing to note is that CF is different for everyone. Not only do the thousands of potential gene combinations play a role in how CF manifests and progresses – there are almost 2,000 known mutations – but so does family history, other health conditions, access to services (for example, health insurance and financial support to obtain these resources), etc.

While they may share many common symptoms and characteristics, no two CF patients are the same. Please do not make any changes to your treatment regimen without consulting your CF team or physician(s). All of the information presented on this website is from my personal perspective and should not be taken as medical advice, nor as any official statement on Cystic Fibrosis, by the CF Foundation, my doctors or hospital(s).

Lung Disease

My mother used to joke that she could never lose me in a crowd, that all she had to do is listen for my cough and she’d find me.

The hallmark manifestation of CF is the colonization of bacterial respiratory infections. Common symptoms include a chronic cough, wheezing, shortness of breath, chest pain, increase in amount/color/consistency of mucous, sinus infection, etc.

This was taken during my 3-week hospitalization during October-November 2012, at the rooftop "Healing Garden" at Yale New Haven Hospital.

This was taken during my 3-week hospitalization during October-November 2012, at the rooftop “Healing Garden” at Yale New Haven Hospital.

The thick mucous in CF lungs provide a moist, warm place for bacteria to take hold and colonize; it’s not uncommon for CF patients to culture several strains of bacteria in their lungs by the time they are adults. While there can be periods of time when there is not an “active” infection, the bacterial colonies are still in the lungs, building defenses and even encouraging other bacteria to grow. Some of these bugs are so smart that they actually can alter their own DNA to create thicker, stronger cell walls to protect against antibiotics (this is called resistance – essentially, the bug resists the medication and it no longer works).

How does this happen to us? Either our own repeat use of a particular antibiotic over time has caused the bug to build new defenses, or we acquire a bacteria strain that is already resistant to the antibiotic(s).

NOTE: There will be times when the bacteria seem “quiet” and aren’t causing a lot of problems (symptoms). My husband and I refer to this as the bugs going into remission, but this is not a standard term for anything having to do with CF. It’s kind of the opposite of the term “flare up”. It is our term, and how we explain what the bacteria does in between acute flare ups.

When the bacteria start to multiply and causing symptoms of respiratory infection, such as increased coughing, wheezing, thicker and larger amounts of mucous and shortness of breath, the patient is said to be experiencing a “flare up” or “acute pneumonia”.

One particular bug – Pseudomonas Aeriginosa – is a common CF enemy. But because it is typically found everywhere in the environment – in the water, soil, on everyday surfaces – it’s very difficult to prevent all possible sources. Other bugs commonly colonized in CF patient lungs include Staph Aureus (which is everywhere, too), and more recently, the “superbug” known as MRSA (Methicillan-Resistant Staph Aureus).

Treatment for pulmonary flareups are pretty standard in the United States:

It’s now common among U.S. CF clinics to aggressively treat infections and help prolong the time in between needing antibiotics. However, even once acute infections subside, the patient often continues to have coughing and other lung issues on a regular basis. Various treatments help maintain lung function, clear the airways and help boost the body’s ability to fight off future flare ups, with the goal of slowing down the inevitable progression of scarring of the lung tissue, which is what ultimately leads to the decrease of lung function.

1. Treating the infection with Antibiotics. The only way to slow down and temporarily eradicate acute Pneumonia infections is to do a lot of work clearing the mucous from our lungs and taking antibiotics to kill off as many bacterial colonies as possible. Because we often carry more than one bacterial strain, and any or all of the bacterial strains carry various levels of resistance, it often takes a cocktail of several different classes of medications and various methods of administration to treat the current “flare-up”.

There are three different methods of antibiotic administration – oral, inhaled and IV (intravenous). The number, classes, and types of antibiotics depend on the patient’s specific bugs and other medical considerations (allergies, drug interactions, home vs. hospital, insurance coverage, medical equipment, etc.). YES, access to medical treatment for potentially lethal lung infections still depends on a patient’s ability to pay.

Therein lies the conundrum:

We takes courses of antibiotics to treat flareups of the bacterial infections – basically trying to knock down the number of colonies to a point where acute symptoms – cough, mucous production, shortness of breath, etc. – improve, bringing the patient as closely back to their typical baseline health as possible. Well, until the next time.

The problem is, over time, the bugs they colonize and ones they acquire often become resistant to most classic antibiotics.  As more and more bugs learn to fight the antibiotics – resulting in resistance – researchers, doctors and pharmacists have to come up with new methods and combinations of medications to try to slow down the progression of infections.

Eventually, there comes a time when oral and inhaled antibiotics just aren’t working on an infection, and we require IV antibiotics, which is a whole other ball of wax. This requires having some sort of permanent or semi-permanent IV – like a Mid-Line, a PICC line or a Port – placed in the body to receive the meds, because A. the meds are so toxic that regular veins cannot handle them for long, and B. it allows patients the ability to do IV antibiotic treatment at home.

My PICC line allows me to receive strong antibiotics and nutritional support during my last hospitalization.

My PICC line allows me to receive strong antibiotics and nutritional support during my last hospitalization.

It is usually the level and frequency of infections and the resulting lung damage that kills CF patients. Lung transplant does offer a second chance, but it requires a certain level of health and extreme dedication on behalf of the patient. While the new lungs will not have CF, the rest of the body will.

2. Treating the Inflammation. People with CF often experience a lot of inflammation in the lungs, whether or not they have an active bacterial infection “flare up”. Inflammation is the immune system’s response to things that shouldn’t be there. A variety of things are theorized (and confirmed) to contribute to inflammation in the lungs of CF patients –

  • Pathogens such as Bacterial infections
  • Excess mucous – Like the animated Mucous People in the Mucinex television commercials, these bugs like to set up shop and make themselves comfortable.
  • Presence of the CFTR gene itself – currently being researched

There are various medications to help treat inflammation, including oral and inhaled steroids, asthma-type medications like bronchodilators and various anti-inflammatories to open the airways.

3. Thinning the mucous that harbors the infections and scars the airways. Several different methods are used to accomplish this:

    • Medication – Many patients use both over-the-counter medications like Mucinex, and prescription mucous-thinners delivered by pill and nebulizer.
    • Airway clearance. Perhaps one of the most important treatments fora CF patient with chronic infection, Airway Clearance used to be a time-consuming and exhausting practice that required someone (usually a parent, caretaker or Respiratory Therapist) to bang on various lobes of the lungs while the patient changed positions to help loosen the mucous and allow the patient to spit it out. Thanks to research and technology, there is now a choice of devices that allow the patient to self-administer airway clearance.
      • Many patients use some form of a Vest, which is attached to a compressor that vibrates the Vest, helping loosen secretions.
      • Acapella or Flutter devices are hand-held, light, and made of plastic, and contain a mechanism inside that helps create air pressure changes and vibrations that amazingly cause the airways to shake and release mucous.
    • Physical activity. It’s important for  CF patients to remain as active as possible, helping the lungs work as efficiently as possible, help airways clear secretions, maintain good cardiovascular health, and helping the body stay as healthy as possible.

4. Nutritional Support. CF patients, due to malabsorbtion and chronic infection, often need extra calories and nutrients (including extra vitamins) to keep them healthy. Many of us are deficient in fat-soluble vitamins A, D, E and K, because many of us have problems digesting fat.

Growing up, I was really thin and malnourished, and had to drink high-calorie shakes in addition to three full meals and countless snacks per day. When I am sick, I can very easily lose large amounts of weight because my body is working overtime to get the air it needs to run and support my immune system to fight off infection. In Many CF patients are very thin, so I am lucky at this point in my life to have a few extra pounds. Though it’s not sure which comes first, studies have shown a correlation between lung function and BMI.

5. Oxygen Support. Many patients also rely on Oxygen – from needing it only when sick, to wearing O2 during sleep and exercise, to needing it constantly. Whether it’s delivered via a cannula in a hospital or “home” setting (the patient wears oxygen attached through a tube the attaches the inside of the nostrils), or the patient is in the hospital and attached to a Ventilator, Oxygen supplementation becomes necessary when a patient’s lungs cannot hold and deliver enough oxygen to sustain their life.

This past fall, when I spent several weeks inpatient on IV antibiotics to treat aggressive bacterial infections, I needed Oxygen for 18 out of 20 days. It was only the second time I needed Oxygen outside of a surgery situation; the first time, which was eight years ago, I only needed it for about 24 hours. It was really scary to need the Oxygen 24/7 – just laying in bed, my lungs couldn’t support enough O2 for me to just lay there. It was really scary to be that sick, and made me ever so thankful for the health I have enjoyed in between flare ups.

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