Why I’m Not Grateful I Have Churg Strauss Syndrome: Part 2 Asthma

Posted by Lew on November 28, 2009

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Hi Everyone.

Before I start out on part two in this new series, I want you to take a look at the video below…this was taken almost two weeks ago on one of my worse days as far as my asthma condition was concerned….

Okay, now I want to explain right from the start that over the years I have been diagnosed with several different Asthma conditions…

• Sarcoidosis
• Bronchial Asthma
• COPD

Okay, that’s a whole heck of a lot of Asthma to be diagnosed with, but I want to point out the history of each of these different strains of Asthma, along with my diagnosis history of each for everyone…

Okay let’s begin with Sarcoidosis

Sarcoidosis

Sarcoidosis (sarc = flesh, -oid = like, -osis = a process), also called sarcoid or Besnier-Boeck disease, is a multisystem granulomatous inflammatory disease^[1] characterized by non-caseating granulomas(small inflammatory nodules). The cause of the disease is still unknown. Virtually any organ can be affected; however, granulomas most often appear in the lungs or the lymph nodes. Symptoms usually appear gradually but can occasionally appear suddenly. The clinical course generally varies and ranges from asymptomatic disease to a debilitating chronic condition that may lead to death.

Epidemiology

Sarcoidosis most commonly affects young adults of both sexes, although studies have reported more cases in females. Incidence is highest for individuals younger than 40 and peaks in the age-group from 20 to 29 years; a second peak is observed for women over 50.^[2][3]

Sarcoidosis occurs throughout the world in all races with an average incidence of 16.5/100,000 in men and 19/100,000 in women. The disease is most prevalent in Northern European countries, and the highest annual incidence of 60/100,000 is found in Sweden and Iceland. In the United States, sarcoidosis is more common in people of African descent than Caucasians, with annual incidence reported as 35.5 and 10.9/100,000, respectively.^[4] Sarcoidosis is less commonly reported in South America, Spain, India, Canada, and Philippines.

The differing incidence across the world may be at least partially attributable to the lack of screening programs in certain regions of the world and the overshadowing presence of other granulomatous diseases, such as tuberculosis, that may interfere with the diagnosis of sarcoidosis where they are prevalent.^[2]

There may also be differences in the severity of the disease between people of different ethnicity. Several studies suggest that the presentation in people of African origin may be more severe and disseminated than for Caucasians, who are more likely to have asymptomatic disease.^[5]

Manifestation appears to be slightly different according to race and gender. Erythema nodosum is far more common in men than in women and in Caucasians than in other races. In Japanese patients, ophthalmologic and cardiac involvement are more common than in other races^[3].

Sarcoidosis is one of the few pulmonary diseases with a higher prevalence in non-smokers.

Okay, I want to just point out the last sentence above ” few pulmonary diseases with a higher prevalence in non-smokers ” in my case I had been a smoker from my early days in the service, so at the time of my diagnosis ( way before I would be diagnosed with CSS ) that statement was a non-factor.

My Sarcoidosis Diagnosis

This was early in the eighties, after my daughter was born, I was employed at a local ( and now long gone Dairy Mart ) and after a particularly terrible evening of running a fever and coming home and going to bed, came down with a terrible chest cold.

After almost a week of being ill, and running a fever, my mother wound up having to take me to a local hospital that her then pulmonary doctor had privileges ( isn’t it amazing the way we use the term ” Privileges ” in this case, as this doctor actually could not work out of a different local hospital at that time ), so after taking a chest x-ray, which showed inflamed or irritated ” Nodes ” my mother’s doctor decided that a lung biopsy was necessary to rule out ” Lung Cancer “…now, I could not have been older than say 31 years old ( maybe 32 ) and after the lung biopsy came back negative for cancer the pulmonologist said it was  Sarcoidosis or Sarcoid.

My Bronchial Asthma Diagnosis

My diagnosis of Bronchial Asthma, came about five years later, and not surprisingly came about due to another problem with a bad cold, that led to a slight case of Pneumonia in my lungs ( not at this point in time, I was already using a ” Puffer “, although not religiously ) I should also point out that typically, this classification of Bronchial Asthma seems to fall under the Asthma general heading…

Asthma

Asthma is characterized by a predisposition to chronic inflammation of the lungs in which the airways (bronchi) are reversibly narrowed. Asthma affects 7% of the population of the United States,^[1][2], 6.5% of British people and a total of 300 million worldwide.^[3] During asthma attacks (exacerbations of asthma), the smooth muscle cells in the bronchi constrict, the airways become inflamed and swollen, and breathing becomes difficult.

Asthma causes 4,000 deaths a year in the United States. Medicines such as inhaled short-acting beta-2 agonists may be used to treat acute attacks. Attacks can also be prevented by avoiding triggering factors such as allergens or rapid temperature changes and through drug treatment such as inhaled corticosteroids and then long-acting beta-2 agonists if necessary.^[4][5] Leukotriene antagonists are less effective than corticosteroids, but have no side effects. Monoclonal antibodies, such as mepolizumab and omalizumab, are sometimes effective. Prognosis is good with treatment.

In contrast to chronic obstructive pulmonary disease and chronic bronchitis, the inflammation of asthma is reversible. In contrast to emphysema, asthma affects the bronchi, not the alveoli.

The National Heart, Lung and Blood Institute defines asthma as a common chronic disorder of the airways characterized by variable and recurring symptoms, airflow obstruction, bronchial hyperresponsiveness (bronchospasm), and an underlying inflammation.^[6]

Public attention in the developed world has recently focused on the predisposition because of its rapidly increasing prevalence, affecting up to one quarter of urban children.^[7]

Asthma is classified according to the frequency of symptoms, FEV₁ and peak expiratory flow rate.[

Signs and symptoms

Because of the spectrum of severity within the asthma, some people with asthma only rarely experience symptoms, usually in response to triggers, where as other more severe cases may have marked airflow obstruction at all times.

Asthma exists in two states: the steady-state of chronic asthma, and the acute state of an acute asthma exacerbation. The symptoms are different depending on what state the patient is in.

Common symptoms of asthma in a steady-state include: nighttime coughing, shortness of breath with exertion but no dyspnea at rest, a chronic 'throat-clearing' type cough, and complaints of a tight feeling in the chest. Severity often correlates to an increase in symptoms. Symptoms can worsen gradually and rather insidiously, up to the point of an acute exacerbation of asthma. It is a common misconception that all people with asthma wheeze—some never wheeze, and their disease may be confused with another chronic obstructive pulmonary disease such as emphysema or chronic bronchitis.

An acute exacerbation of asthma is commonly referred to as an asthma attack. The cardinal symptoms of an attack are shortness of breath (dyspnea), wheezing, and chest tightness.^[9] Although the former is often regarded as the primary symptom of asthma,^[10] some patients present primarily with coughing, and in the late stages of an attack, air motion may be so impaired that no wheezing may be heard.^[11] When present the cough may sometimes produce clear sputum. The onset may be sudden, with a sense of constriction in the chest, as breathing becomes difficult and wheezing occurs (primarily upon expiration, but sometimes in both respiratory phases). It is important to note inspiratory stridor without expiratory wheeze however, as an upper airway obstruction may manifest with symptoms similar to an acute exacerbation of asthma, with stridor instead of wheezing, and will remain unresponsive to bronchodilators.

In my case, the onset of an asthma attack usually happens for me late in the evening, usually after I go to bed and will start with a low wheezing sound in my chest, as I am lying down on my side ( although over the past two years that has not been the case, as I can be lying down on my back in bed and will still have the wheezing noise in my chest ) and of course will lead me to get up and head to the bathroom to try to bring up whatever congestion I might have in my chest.

Sometimes this will lead to my having to use a ” Puffer ” or ” Bronchiodialtor ” such as the one I use now called ” ProAir HFA, which of course is an ” Albuterol ” inhaled steroid.

My Diagnosis Of COPD

Before I go into this last diagnosis of COPD, I need to clarify a few things here..

• First, as I said in the above video, I have been and still work in a ” Fragrance Factory ” everyday. In other words, my lungs are exposed to whatever chemicals  and dust particles are in the air when I enter this place of business everyday.
• Just refer to the above sentence!

Before I go into this final area, I want to just highlight what COPD is…

Chronic Obstructive Pulmonary Disease

or COPD for short

Chronic obstructive pulmonary disease (COPD) refers to chronic bronchitis and emphysema, a pair of two commonly co-existing diseases of the lungs in which the airways become narrowed.^[1] This leads to a limitation of the flow of air to and from the lungs causing shortness of breath. In contrast to asthma, the limitation of airflow is poorly reversible and usually gets progressively worse over time.

COPD is caused by noxious particles or gas, most commonly from tobacco smoking, which triggers an abnormal inflammatory response in the lung.^[2][3] The inflammatory response in the larger airways is known as chronic bronchitis, which is diagnosed clinically when people regularly cough up sputum. In the alveoli, the inflammatory response causes destruction of the tissues of the lung, a process known as emphysema. The natural course of COPD is characterized by occasional sudden worsenings of symptoms called acute exacerbations, most of which are caused by infections or air pollution.

The diagnosis of COPD requires lung function tests. Important management strategies are smoking cessation, vaccinations, rehabilitation, and drug therapy (often using inhalers). Some patients go on to requiring long-term oxygen therapy or lung transplantation.^[2]

Worldwide, COPD ranked sixth as the cause of death in 1990. It is projected to be the third leading cause of death worldwide by 2020 due to an increase in smoking rates and demographic changes in many countries.^[2] COPD is the 4th leading cause of death in the U.S., and the economic burden of COPD in the U.S. in 2007 was $42.6 billion in health care costs and lost productivity.^[4][5]

COPD is also known as chronic obstructive lung disease (COLD), chronic obstructive airway disease (COAD), chronic airflow limitation (CAL) and chronic obstructive respiratory disease (CORD).

Signs and symptoms

One of the most common symptoms of COPD is shortness of breath (dyspnea). People with COPD commonly describe this as: “My breathing requires effort,” “I feel out of breath,” or “I can’t get enough air in”.^[6] People with COPD typically first notice dyspnea during vigorous exercise when the demands on the lungs are greatest. Over the years, dyspnea tends to get gradually worse so that it can occur during milder, everyday activities such as housework. In the advanced stages of COPD, dyspnea can become so bad that it occurs during rest and is constantly present.

Other symptoms of COPD are a persistent cough, sputum or mucus production, wheezing, chest tightness, and tiredness.^[7][8]

People with advanced (very severe) COPD sometimes develop respiratory failure. When this happens, cyanosis, a bluish discoloration of the lips caused by a lack of oxygen in the blood, can occur. An excess of carbon dioxide in the blood can cause headaches, drowsiness or twitching (asterixis). A complication of advanced COPD is cor pulmonale, a strain on the heart due to the extra work required by the heart to pump blood through the affected lungs.^[9] Symptoms of cor pulmonale are peripheral edema, seen as swelling of the ankles, and dyspnea.

There are a few signs of COPD that a healthcare worker may detect although they can be seen in other diseases. Some people have COPD and have none of these signs. Common signs are:

• tachypnea, a rapid breathing rate
• wheezing sounds or crackles in the lungs heard through a stethoscope
• breathing out taking a longer time than breathing in
• enlargement of the chest, particularly the front-to-back distance (hyperinflation)
• active use of muscles in the neck to help with breathing
• breathing through pursed lips
• increased anteroposterior to lateral ratio of the chest (i.e. barrel chest).

I think that you can well imagine that after more than ten years of working on the production floor in this type of environment, I would have issues where the statements ” My breathing requires effort,” and  “I feel out of breath ” would certainly ring true.

This would be the case back in 2005, the week of my birthday and I had a weeks vacation planned…including taking a test to check out my lung capacity, as I was having serious issues again with my breathing.

I was also having issues with stress due to my father’s continued illness which was due to another culprit, Emphysema.

After the test results came in, I got the diagnosis of COPD, and I remember going to see my father and mother 20 minutes later at their home to tell them the ” Great News !”

I was also given my first prescription of Advair at the lesser dosage of 250/50 mcgs

Okay, let’s forward to the present 2009…last year after being diagnosed with CSS, and of course doing some research at the time ( but not a lot at that point ) I found that all of the above were tied in with CSS, and of course a lot of unanswered questions..

Were all of the above actual pre-cursors to my eventual CSS diagnosis?

Were some of my past jobs partly to blame for this eventual outcome and diagnosis ( to be honest, I have not worked in the best of environments..a ceramic plating plant where I was exposed to all kinds of chemicals in that process including a form of Cyanide, and working in the family ” Radiator ” business for a three year stint, also being exposed to certain chemicals, even while using proper masks and ventilation ) and just whichy job could be termed the ” Ultimate Culprit ?”

But what I find most aggravating about this, is the simple fact that I cannot even get my pulmonologist to commit to CSS being the real culprit in any of my Asthma issues, and just why is this Lew your asking…because there still is not enough information known about CSS in general ( I believe anyway ) where they ( doctors ) are able to differentiate from an asthma attack brought on by CSS vs an attack brought on by allergies or other environmental issues.

One thing I can leave you with is the fact that one worker in the factory I work in now, just a few years ago came down with asthma…is working in a fragrance factory such as ours a factor…I tend to think so.

In closing this second part to this series, I guess the real question could be ” Am I Just Doing This To Myself? ” in other words I know I have these conditions, and yet I’m still employed at the same place that might very well be causing me more harm than good  as far as all of them are concerned…

Truthfully, I could very well be my own worst enemy, in this case anyway.

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