Living Life with Idiopathic Pulmonary Fibrosis

After being given the diagnosis, I had to take time to ponder the way my life was going to go. I mean, being given a possible early death sentence wasn't exactly the easiest thing in the world to take. I was a senior in high school; I had dreams of going to college, working at a job I loved, helping others through that job, creating my own family. I would like to say that I took it all in stride, but to be honest, I didn't. I was angry at first, and then I was depressed for a while. It took quite a bit before I could finally accept the fact that I had this disease. Now, what I chose to do while going through this was the question.

With this disease, my healthy means more than ever, if I want to live as long as I possibly can. I was prescribed the three general medicines for IPF: prednisone, azathioprine, and n-acetylcysteine. In addition, I had medicines to combat the side effects of these medicines. For me, this disease means that I need to keep up with my medicines as well as keep my body as healthy as possible, in whatever way I can.

My short term goals really haven't changed. I will continue to focus on the rest of my senior year, look forward to graduation, and just live life every single day in a way that I will have no regrets.

As for my long term goals, I still look forward to working hard at college, obtaining my dream job, creating a family. If I am part of the 60%, then these goals probably aren't realistic, but hoping is better than moping a fate that probably won't change no matter how I react towards it.

It does make me stop and think. Not only about the end of my life, whenever that may be, but how I live life now. It isn't easy, yes, but I have wonderful friends and family who have supported and will support me every step of the way and that's a great foundation to be grounded in. I can still look forward to the life I will live and I will still fight--I've always been quite stubborn after all--though it is a losing battle. Having this disease and the looming fear that I could die soon makes me realize that life is a gift. Whenever you feel pain, it's a gift, because it tells you that you are breathing, that you are alive. For now, I will treasure the past but not dwell on what has happened because after all, how I live in my present will determine how I will face my future.

Treatment and Medicine

Doctors may prescribe medicines, oxygen therapy, pulmonary rehabilitation, and/or a lung transplant to treat idiopathic pulmonary fibrosis.

Though there are currently no medicines proved to slow the progression of IPF and there is not quite enough information to support the use of the medicines that currently are used, there are three medicines available.

1. Prednisone: an anti-inflammatory medicine that is usually taken by mouth everyday. It may also be given through a needle or a tube inserted into a vein in the arm for several days. However, prednisone can cause serious side effects and so may only be prescribed for 3-6 months at first.
2. Azathioprine: suppresses the immune system that is also taken by mouth. Because of the serious side effects it can cause as well, it may be prescribed with prednisone for 3-6 months as well.
3. N-acetylcysteine: an antioxidant that prevents lung damage, also usually taken by mouth. A common treatment is by giving all three however, this was recently proven harmful by the NHLBI.

Other treatments include:

• flu and pneumonia vaccines
• cough medicines or oral codeine
• Vitamin D, calcium, or a bone-building machine to help prevent bone loss if taking prednisone or another corticosteroid
• anti-reflux therapy may help gastroesophageal reflux disease

Pulmonary rehabilitation is also available such as:

• exercise training
• nutritional counseling
• education on lung disease or condition and how to manage it
• breathing strategies
• psychological counseling and/or group support (a website including a list of support groups: http://www.pulmonaryfibrosis.org/life-with-pf/support-groups)

Finally, a lung transplant may be recommended if the condition is quickly worsening and/or severe. There are, however, major complications such as rejection and infection.

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References:

• What Is Idiopathic Pulmonary Fibrosis? (2011, September 20). Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/ipf

Forms of Diagnosis

Idiopathic pulmonary fibrosis forms the same kind of scarring as other lung disease, which makes it quite hard to distinguish and thus, diagnose. The important thing is to seek help as soon as possible, and preferably from a pulmonologist.


They will diagnose based off of medical history, a physical exam, and/or test results.


Questions that may be inquired, in order to ascertain your medical history, are:

1. age
2. history of smoking
3. things in the environment that could irritate your lungs
4. hobbies
5. history of either legal or illegal drug use
6. other medical conditions
7. family's medical history
8. how long symptoms have persisted

Though there is no single test that can diagnose IPF, there are several recommended tests.

• a chest x-ray: a painless test that takes pictures of the structures in the chest. It can show shadows that may suggest scar tissue. However, many people diagnosed with IPF show normal chest x-rays.
• high-resolution computed tomography: HRCT scan; an x-ray that provides sharper and more detailed pictures. It can show scar tissue and how much lung damage there is. This helps spot IPF at an early stage or rule it out completely.
• lung function tests: also called a spirometry, which is a breathing test. This measures how much air can be blown out after taking a deep breath as well as how fast the air releases.
• pulse oximetry: a small sensor is attached to a finger or an ear and a sensor uses light to estimate how much oxygen is in the blood.
• arterial blood gas test: a blood sample is taken, usually from the wrist, and then sent to a laboratory where oxygen and carbon dioxide levels are measured. This blood sample can also be tested to see if an infection is causing the symptoms.
• skin test for tuberculosis: done to rule out TB.
• exercise testing: shows how well lungs move oxygen in and out of the bloodstream while active. This test is measured using an exercise machine or by walking for a few minutes. After, they determine blood pressure, heart rate, and how much oxygen there is in the bloodstream.
• lung biopsy: there are several procedures to get lung tissue samples: video-assisted thorascopy, bronchoscopy, bronchoalveolar lavage, and thoractomy.

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References:

• What Is Idiopathic Pulmonary Fibrosis? (2011, September 20). Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/ipf

Pathophysiology of IPF

A disease that usually occurs in middle-aged to older adults, patients diagnosed with IPF usually only live for about 2-5 years following the diagnosis. Estimated mortality rates are 64.3 deaths per million in men and 58.4 deaths per million in women. About 60% of patients die from IPF. The most common cause of death is respiratory failure. Other causes include pulmonary hypertension, heart failure, pulmonary embolism, pneumonia, and lung cancer.


Idiopathic pulmonary fibrosis occurs when the tissue in between the lungs thickens and stiffens until it forms an almost crisscross pattern of hard tissue around and between the lungs, making it difficult for the lungs to move oxygen into the bloodstream.


The main signs and symptoms of IPF include shortness of breath, not just during exercise, but even at rest, and a dry, hacking cough that over time, turns into repeated bouts of coughing that is uncontrollable. Other symptoms that may be developed over time can be rapid, shallow breathing, gradual and unintended weight loss, fatigue or just a general feeling of being unwell, aching muscles and joints, and clubbing, which is the widening and rounding of the tips of the fingers and/or toes.


Idiopathic is put in front of the name of a disease if the cause of said disease is unknown. However, there are many different possible causes for pulmonary fibrosis.


Currently, however, it is believed that it is an epithelial-fibroblastic disease in which unknown internal or environmental stimuli disrupt the homeostasis of alveolar epithelial cells, causing these cells to spread as well as repair abnormally. It is also found in excess alveolar epithelial cell apoptosis and/or fibroblast resistance to apoptosis.


Outside of acquired pulmonary fibrosis, it can also be genetic, which is said to be associated with mutant telomerase. Telomerase is a specialized polymerase that adds telomere repeats to the ends of chromosomes which helps prevent shortening that can occur during DNA replication. It has been said that pulmonary fibrosis in patients with short telomeres is provoked by a loss of alveolar ephithelial cells.


Another possible cause is if a patient has low levels of caveolin-1, which is considered a protective regulator of pulmonary fibrosis.

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References:

• Godfrey, A. (2015, August 12). Idiopathic Pulmonary Fibrosis. Retrieved from http://emedicine.medscape.com/article/301226-overview#a6
• What Is Idiopathic Pulmonary Fibrosis? (2011, September 20). Retrieved from http://www.nhlbi.nih.gov/health/health-topics/topics/ipf

My Diagnosis

It started with just shortness of breath, which was no big thing especially with my asthma. However, the frequency of the time when it became harder to breathe increased. In addition, after a brief period of illness, I acquired a dry cough that hurt every time it was released. Symptom after symptom followed after. My breathing became rapid, shallow. I also began losing weight and my muscles and joints ached for no reason. As if to tie the ribbon on a package of disaster, fatigue weighed me down in every aspect of my life.


Finally, I agreed to go to the doctor. They only asked questions at first, trying to glean any information that they could from my answers. However, when those were unhelpful, I underwent a simple chest x-ray. Which, oddly enough, turned up 100% normal. Because I already had too much problems for it to merely be "nothing", I asked for further testing. This came in the form of a high-resolution computed tomography scan. When the pictures came back and specialists had fully examined it, my diagnosis became clear: idiopathic pulmonary fibrosis.