Pulmonary Fibrosis (PF) is a debilitating disease where there is progressive scarring of the lung tissue and interferes with the person’s ability to breathe. It was only recognised as a disorder in its own terms in 2001 and before that was grouped under other similar lung disorders and was referred to as other disease titles.
It can in some cases be linked to a specific cause such as environmental exposures, chemotherapy, radiation therapy, residual infection or due to an autoimmune disease such as rheumatoid arthritis. However in cases where the cause is unknown it is referred to as idiopathic pulmonary fibrosis (IPF).
While the cause of PF remains a mystery it is suspected that PF involves changes in the lung’s normal healing process. Patients may have an exaggerated or uncontrolled healing response that, over time, produces excessive fibrous scar tissue – or fibrosis – in the lungs. This scarring, in turn, causes the lung’s alveoli to thicken and stiffen-rendering them less able to function and provide the body with the oxygen it needs.
Exactly what sets this abnormal tissue-repair process in motion is unclear. The body’s own immune response may play a major role. Researchers are investigating a number of potential risk factors that may make a person more likely to develop Pulmonary Fibrosis.
These risks may include:
Occupational exposure to dusty environments (e.g. wood or metal dust)
Genetic predisposition (10-15 percent of cases)
Viral or bacterial lung infections
Acid reflux disease
Pulmonary Fibrosis hinders a person’s ability to take in oxygen. It causes shortness of breath and is usually associated with a persistent dry cough. The disease progresses over time, leading to an increase in lung scarring and a worsening of symptoms. Unfortunately, Pulmonary Fibrosis is ultimately disabling and fatal.
Symptoms of Pulmonary Fibrosis usually have a gradual onset and may include:
Shortness of breath, especially after exertion
Gradual, unintended weight loss or weight gain
Fatigue and weakness
Clubbing (enlargement of the ends of the fingers or toes) due to a build-up of tissue
PF affects each person differently and progresses at varying rates. Generally, the patient’s respiratory symptoms become worse over time and activities (such as walking or climbing stairs) become more difficult.
The patient may require supplemental oxygen
Advanced PF makes it difficult for a person to fight infection
Pulmonary Fibrosis puts a strain on the heart and on the blood vessels in the lungs, and may lead to high blood pressure in the lungs
PF has also been associated with heart attacks, respiratory failure, strokes, blood clots in the lungs (pulmonary embolism), lung infections, and lung cancer.
An important part of the treatment is the use of supplemental oxygen to provide your body with the required level of oxygen it needs but cannot get due to the scarring in the lungs.
Supplemental oxygen can:
Decrease your shortness of breath
Improve your ability to carry out daily tasks
Improve your overall level of fitness
Improve your quality of life
Increase life span by decreasing the extra work your heart is doing because of low oxygen saturation levels
PF patients should also discuss the possibility of a lung transplant with their GP as soon as possible as unfortunately 30% PF patients succumb to their disease while waiting on the transplant list. This is due to the unpredictable progressive nature of the disease in combination with long transplant waiting lists.
There are new treatments being currently tested, which look promising at reducing symptom severity and aiding in decreasing the progression of the disease and ultimately aiding in improving life expectancy.
References: http://www.coalitionforpf.org and http://www.breathingmatters.co.uk
COPD includes two different lung diseases: emphysema and chronic bronchitis. People with these conditions have damage to the airways and the air sacs inside their lungs. This damage makes it harder for them to breathe and to get enough oxygen into their bodies with each breath. COPD produces many outward symptoms, which slowly get worse over time and all of which originate from the lung and airway damage. The main and most commonly known symptom is the difficulty to breathe and the requirement for Home Oxygen Therapy, where patients can use oxygen at home to help ease their symptoms.
1. Mucus-Producing Cough
The cough is the most obvious symptom of COPD. The damage to the lungs causes them to produce more thick mucus than normal which can block the airway and make it difficult to breathe. Coughing it up is the body’s natural and most efficient way to clear it from the body. One way to ease a phlegmy cough is to drink extra fluids, which helps loosen up the mucus so the body can remove it more easily.
With COPD the air that you inhale with each breath has to travel down narrowed airways. The air struggles to get through and causes the walls of the airways to vibrate. This vibration combined with moving air produces a whistling sound which we call wheezing. Bronchodilators and steroid medicines can be given to widen the airways and relieve the wheezing and ease breathing.
3. Blue Lips and Nails
This condition can also result in your lips and nails turning a bluish colour. This illustrates the lack of oxygen being delivered to those parts of the body and an indication that there isn’t enough oxygen in your blood generally. Normally, the blood is red but when it’s deprived of oxygen, blood turns blue which gives the lips and fingernails their blue hue. A bluish discolouration of the skin is also called cyanosis. It’s a very serious symptom as it indicates extremely low oxygen levels and if noticed an immediate call for emergency medical care should be made.
4. Lower Body Swelling
To compensatge for the damage to your lungs, your heart has to pump harder to get enough oxygen to your body. Over time the heart muscle can become damaged and enlarged from the extra work and lower body swelling is an indicator of this. The heart doesn’t beat as forcefully as it should and low blood pressure could lead to a blood clot if not treated.
5. Barrel Chest
Long term sufferers may develop a bulging in their chest which takes on a barrel-like appearance, called a barrel chest. This can form because your lungs are so filled with air that they eventually stretch out your ribcage. A barrel chest can worsen existing breathing problems from COPD, making it even harder for you to catch your breath.
6. Weight Loss
With your lungs not working as well your body has to exert more energy than normal to try to compensate and maintain minimal oxygen levels. This causes you to burn up to 10 times more calories than usual and can result in hunger and fatigue if the calorie intake is’nt increased. Shortness of breath and coughing can also make you less interested in eating and with this combination over time, you’ll start to lose weight. Any weight loss in COPD is a serious sign and when your body is too thin, it can’t protect you as well against infections.
There are many stories of patients being misdiagnosed and given treatment for asthma when in fact years later the patient is then diagnosed with COPD.
Pat Crowe was a healthy, active 39 year old but was told he had developed asthma and was given an inhaler. Over the next few years his health deteriorated, he grew dependent upon and overused his inhaler, suffered from side effects and eventually the inhaler wasn’t enough. Being so breathless meant his confidence plummeted, making him almost housebound.
‘I panicked about going outside and crossing busy roads because I was having trouble breathing and couldn’t move quickly.’
During bad spells and prolonged attacks he was also prescribed strong steroid pills, which caused side-effects, including a swollen face and hunger.
‘My wife became my carer, and my family were very scared watching me struggle to breathe,’ he says. ‘I didn’t even want to get out of bed and go down stairs because I was worried about getting back up. My life changed completely.’
It was only then that Pat’s consultant performed a spirometer test which confirmed that he didn’t have asthma after all but suffered from COPD, which explained why he’d found the condition impossible to control.
COPD is incurable and affects 900,000 people in the UK, usually above the age of 35, and an estimated 30,000 people die from it each year. Experts believe there are also more than two million undiagnosed cases.
Specialist advice is crucial when it comes to achieving an accurate diagnosis says Dr Kurukulaaratchy: ‘GPs need better awareness of these conditions. They both rank highly in hospital admissions, but if management is right early on, you can improve quality of life and possibly even life expectancy.’
Nick Hopkinson, honorary chest physician at the Royal Brompton Hospital, London, adds: ‘Asthma patients usually have normal lung function, but when they experience asthma symptoms or an attack their airways go into spasm, becoming narrow and inflamed. Then, after taking medication – usually an inhaler – their lungs return to normal. However, COPD patients have relatively fixed lung disease, so even on a good day they still have symptoms. Most of the damage isn’t reversible and progressively worsens.’
One major consequence of misdiagnosis is that patients could be given the wrong medication.
COPD patients usually start with bronchodilator inhalers to relieve narrowing of the airways and then go through a variety of drugs, starting with shorter-acting medications and moving to longer-acting ones, as the disease worsens, says Dr Kurukulaaratchy.
‘Research has shown steroids can lead to an increased risk of pneumonia (inflammation of lung tissue) in COPD patients, so this is a risk if they are wrongly labelled as having asthma.’
If the condition doesn’t improve, they can end up using a lot of steroids, which have side-effects such as weight gain, mood changes and osteoporosis.
‘If patients are told they have COPD but it’s asthma and they don’t have inhaled steroids, they won’t be able to control the disease – leaving them prone to worsening symptoms and even death from an asthma attack,’ says Dr Hopkinson. ‘We see 1,100 asthma deaths a year in the UK, often because they weren’t on preventer treatments.’
Also oxygen therapy treatment is a major component of COPD treatment which is prescribed by your doctor, so if you are misdiagnosed then you may not be prescribed oxygen as early as you should have been which can affect your health and long-term diagnosis as COPD damage to the lungs is irreversible.
If you have any doubts about your diagnosis then speak to your doctor or get a second opinion, just to be sure as your health is the most important thing.