Chloe Temtchine – A very brave and creative young woman – PVOD cf IPF

As a ‘senior citizen’ man battling to stay alive with IPF I found this story moving and inspiring – it concerns a singer – Chloe Temtchine who is facing PVOD.

Annette Insdorf’s excellent article about Chloe is in The Huffington Post –

The article starts;

In February of 2013, singer was completing her album, No Pressure — produced by Grammy nominee, Greg Camp — and preparing the international promotional tour. Then one day she was rushed to the emergency room with congestive heart failure that left her unable to breathe, and barely alive. She was later diagnosed with PVOD, a rare and often fatal pulmonary disease.

The 30-year-old New Yorker was given a slim chance of recovery. But this past Thursday night at Chelsea Piers, Chloe Temtchine was back onstage, a portable oxygen tent by her side and a thin breathing tube in her nose. She amazed hundreds of people with her vibrant voice and soulful songs, performing three numbers that led to a standing ovation.

The occasion was the Pulmonary Hypertension Association’s first annual NYC “O2 breathe” Gala.

Chloe affectionately nicknamed her ever-present oxygen bottle “Steve Martin.”

PVOD is at least as deadly as IPF a median prognosis of around 2 years, 3 years in the case of IPF.

…… pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike (see “Causes and Symptoms” below). The PF family of lung diseases falls into an even larger group of diseases called the “interstitial lung diseases.” Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.

Both PVOD and IPF are designated as rare diseases.

You will get an idea of the complexity of this area of 200+ diseases from this site – http://www.pulmonaryfibrosis.org/life-with-pf/about-pf

I have clipped a few sections.

See particularly the diagram below;

WHAT IS PULMONARY FIBROSIS?

The word “pulmonary” means “lung” and the word “fibrosis” means scar tissue – similar to scars that you may have on your skin from an old injury or surgery. So, in its simplest sense, pulmonary fibrosis (PF) means scarring in the lungs. But, pulmonary fibrosis is more serious than just having a scar in your lung. In PF, the scar tissue builds up in the walls of the air sacs of the lungs, and eventually the scar tissue makes it hard for oxygen to get into your blood. Low oxygen levels (and the stiff scar tissue itself) can cause you to feel short of breath, particularly when walking and exercising.

Also, pulmonary fibrosis isn’t just one disease. It is a family of more than 200 different lung diseases that all look very much alike (see “Causes and Symptoms” below). The PF family of lung diseases falls into an even larger group of diseases called the “interstitial lung diseases.” Some interstitial lung diseases don’t include scar tissue. When an interstitial lung disease includes scar tissue in the lung, we call it pulmonary fibrosis.

Autoimmune diseases – From http://www.pulmonaryfibrosis.org/life-with-pf/about-pf

  30/03/2015

Autoimmune diseases

are also called connective tissue diseases, collagen vascular diseases, or rheumatologic diseases. “Auto” means “self” and “immune” refers to your immune system. With autoimmune diseases, your own immune system is attacking your lung. If you have an autoimmune disease, your immune system can cause inflammation and scarring in the lungs. Examples of autoimmune diseases that can cause PF include:

  • Rheumatoid arthritis

  • Scleroderma (now called “systemic sclerosis”)

  • Certain muscle diseases (polymyositis, dermatomyositis, and the anti-synthetase syndrome)

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Genetic/Inherited diseases

Approximately 10-15% of those with an “idiopathic” form of PF have another family member afflicted by the disease. This is called familial pulmonary fibrosis (FPF)

or familial interstitial pneumonia (FIP)

. A number of genes and genetic variants have been identified that are associated with the development pulmonary fibrosis, but frequently genetic tests are not performed when PF is diagnosed, as we are only just beginning to understand the significance of these genetic abnormalities. It is important to discuss the potential risks and benefits of genetic testing with a qualified genetic counselor and your medical provider. To speak with a Certified Genetic Counselor free of charge, contact Janet Talbert, MS, CGC at 800.423.8891, extension 1097.

CLASSIFICATION OF COMMON INTERSTITIAL LUNG DISEASES

Symptoms

The most common symptoms of PF are cough and shortness of breath. Symptoms may be mild or even absent early in the disease process. As the lungs develop more scar tissue, symptoms worsen. Shortness of breath initially occurs with exercise, but as the disease progresses patients may become breathless while taking part in everyday activities, such as showering, getting dressed, speaking on the phone, or even eating.

Due to a lack of oxygen in the blood, some people with idiopathic pulmonary fibrosis may also have “clubbing” of the fingertips. Clubbing is a thickening of the flesh under the fingernails, causing the nails to curve downward. It is not specific to IPF and occurs in other diseases of the lungs, heart, and liver, and can also be present at birth.

Other common symptoms of pulmonary fibrosis include:

  • Chronic dry, hacking cough

  • Fatigue and weakness

  • Discomfort in the chest

  • Loss of appetite

  • Unexplained weight loss

RP – The ones I have big time are the first 3 – but not the last two!

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Pulse Oximetry

A “pulse oximeter” is a device placed on the fingertip or earlobe that indicates the amount of oxygen in the blood. Normally, nearly all of your red blood cells are full of oxygen. When all the red blood cells are carrying oxygen, the blood is called “100% saturated.” A normal saturation level is between 95% – 100% when the test is performed at sea level. Low oxygen saturation values can help your medical professional identify the presence of lung disease. Early on in PF, oxygen levels may be normal while you are resting, but can decrease while exerting yourself. This drop in oxygen saturation (called “de-saturation”) can make you feel breathless. When oxygen levels drop below 89% during exertion, your medical professional may recommend using supplemental oxygen while active.

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GLOSSARY

  • Alveoli: Tiny air sacs in the lungs where carbon dioxide leaves the bloodstream and oxygen enters the bloodstream.

  • Bronchoscope: An instrument used for inspecting the airways of the lungs.

  • Comorbidity: A disease or other medical issue that occurs simultaneously with PF.

  • Diffuse parenchymal lung diseases (DPLD): Another name for interstitial lung diseases.

  • Diffusion capacity (DLCO): A measure of the ability of oxygen to diffuse into the bloodstream.

  • Dyspnea: Difficulty breathing or shortness of breath.

  • Exacerbation: An episode of rapid decline or the emergence of more severe symptoms.

  • Fibroproliferation:  Relating to the growth of fibroblasts (and fibrous tissue), one of the basic connective tissue cells.

  • Fibrosis: An increase in fibrous scar tissue.

  • Forced expiratory volume (FEV1): The amount of air you can blow out in one second, which is measured by spirometry.

  • Forced vital capacity (FVC): How much air you can blow out of your lungs, which is measured by spirometry.

  • Gastroesophageal reflux disease (GERD): A regurgitation of stomach acids into the esophagus and throat, causing heartburn, acid indigestion, and possibly injury to the lining of the esophagus. This is also called acid reflux disease.

  • Hospice care: Palliative care for patients at end-of-life.

  • Idiopathic: Of unknown cause.

  • Idiopathic interstitial pneumonias (IIP): A type of interstitial lung disease. Idiopathic pulmonary fibrosis is a type of IIP.

  • Interstitial lung diseases (ILD): A broad category of over 200 lung diseases that affect the lung interstitium.

  • Interstitium: The space between the alveoli (air sacs).

  • Palliative care: Non-curative therapy that treats symptoms and focuses on improving quality of life. It can be received at the same time as curative therapy.

  • Pathologist: A physician specializing in disease-associated changes in tissue and organs. Pathologists aid in making a medical diagnosis.

  • Pulmonary: Relating to the lungs.

  • Pulmonary hypertension: Abnormally high blood pressure in the pulmonary (lung) arteries.

  • Pulmonologist: A physician specializing in the lungs.

  • Radiologist: A physician specializing in using radiology tests(e.g., X-rays) to diagnose illness.

  • Rheumatologist: A physician specializing in rheumatic diseases, which may include arthritis, autoimmune diseases, and joint diseases.

  • Spirometry: A test that measures the amount of air inhaled and exhaled over time.

  • Usual interstitial pneumonia (UIP): A specific abnormal radiologic or pathologic pattern of interstitial lung disease.

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