Sarcodosis- Tak…

Sarcodosis- Taking Your Breath Away

                                                                      Velva Boles, MD

Alan is a proud African American man -the bread winner in his family. He grew up knowing the true meaning of “being the first born son” and a “good big brother”; he shouldered responsibility well. Alan never turned away from a challenge and he had been rewarded with a successful career, a loving wife and family. But recently, he has had trouble breathing -just can’t seem to catch his breath. He has to pause often and consciously take slow deep breaths that don’t quite quench his hunger for oxygen. He yawns a lot and has an annoying dry cough. Alan is experiencing feelings of constrictive breathing that come and go lasting a few days each week. During these times, he is more tired than usual and sleep does not totally rejuvenate him; he drags – no energy, his eyes burns because they are so dry and his vision is sometimes blurry. A rash appeared on his legs two weeks ago; it doesn’t itch or bother him, but his wife is concerned about it. Alan is not one to complain and his scheduled annual physical is a few months away, so he’s content to wait until then to talk to a doctor.  

Alan sat nervously in his doctor’s office. He had gotten the blood work, chest x-ray and urinalysis done two days before his clinic visit and now he waited to get the results. His doctor walked in without the usual smile and joking manner. Alan felt the chill of cold sweat on his forehead. The doctor looked at Alan and cleared his throat then said, “Alan it appears that you have something going on in your lungs and you have enlarged lymph nodes in your chest.”

“Are you saying that I have cancer?” Alan asked.

“Let’s not jump to conclusions”, the doctor answered. There are other things that it could be – like sarcoidosis. It could be sarcoidosis. The chest x-ray pattern strongly suggests sarcoidosis.”

” What’s that?” Alan asked in abject fear. “I have been short winded off and on for about a year; now it comes more regularly and lasts longer. I think that it is getting worse. What do you suggest that I do? Alan asked tentatively. “I don’t have comprehensive insurance”.

The doctor cleared his throat and said, “The good news is that 70% of patients who have sarcoidosis don’t require therapy.  Often, it goes away on its own. But when therapy is needed steroids can usually keep the symptoms under control.  “Let’s start steroids, Predisolone; it can slow or reverse the course of the disease and it is not very expensive.”

“Can I give this to my wife and family? Is this contagious? Do I need to take off work? How did I get this?  I got to call my wife. Will you talk to her and tell her what I got?”

 

“Alan, sarcodosis is not contagious; not like the flu or a cold”, answered the doctor. “Please call your wife, I will be glad to talk to her. I am going to have my nurse take you to the small conference room where you can call your wife. I will be there in a few minutes. The nurse will also show you a video and give you some literature to read.”

 

 

Working hypotheses for the cause(s) of sarcodosis begin with a belief that sufferers have an alteration in immune response after exposure to an environmental, occupational, or infectious agent. Some people appear to have a genetic predisposition for developing the disease, which may be triggered by exposure to bacteria, viruses, dust or chemicals which cause inflammation that does not go away instead immune system cells cluster to form lumps, called “granulomas”, throughout various organs in the body.  Several infectious agents appear to be significantly associated with sarcoidosis, but none of the known associations is specific enough to suggest a direct causative role. Propionibacterium acnes has been implicated. It is a bacterium found in bronchoalveolar lavage (lung washings) in 70% of patients with symptomatic disease. Sarcoidosis usually occurs in people between the ages of 20 and 40. Black Americans have a higher incidence and more severe assault from sarcoidosis than other Americans and are more likely to have lung and skin involvement. In the United States, several studies suggest that the presentation in people of African origin is more severe and disseminated than for Caucasians, who are more likely to have asymptomatic disease.

Sarcoidosis is a systemic disease that can affect any organ. Signs and symptoms of sarcoidosis tend to vary, depending on which organs are affected and how long the disease has been present. Granulomas appear most often in the lungs and within lymph nodes. Sarcoidosis granulomas lead to organ damage in about one-third of the people diagnosed with the disease. Rarely, sarcoidosis can be fatal. Death usually is the result of problems with the lungs, heart, or brain. About 50% of people who have sarcodosis show symptoms within 3 years of diagnosis and about 10% develop serious disabilities.

 

Symptoms and signs involving the skin include rashes and small bumpy plaques, maculo-papular eruptions, and subcutaneous nodules. Disfiguring skin sores may occur on the nose, cheeks and ears and areas of skin may get darker or lighter in color. Growths, just under the skin, develop around scars and tattoos.  A rash of reddish-purple bumps may appear on the shins and ankles and be warm and tender to the touch. The scalp may present with diffuse patches of hair loss (baldness).

Diseased lung is by far the most common diagnostic site for sarcoidosis which causes permanent progressive fibrosis of the lung tissue. Pulmonary manifestations of sarcoidosis are two-fold:  the amount of air drawn into the lungs (vital capacity) is limited and lung changes due to scarring and enlarged lymph nodes in the chest impede  the amount of air that can be easily pushed out (exhaled). Within the lung, the inflammatory process involves the alveoli, small bronchi, and small blood vessels. Lymph nodes in the chest cavity are enlarged in 75 to 90 percent of all sarcoidosis patients; their presence is obstructive to lung movement. Pulmonary Hypertension is the greatest threat with sarcoid lung. It causes abnormally high blood pressure in the arteries of the lungs which makes the right side of the heart work harder to force the blood through these vessels; over time, the right side of the heart becomes enlarged and less efficient in pumping blood and ultimately results in heart failure.

Granulomas cause morbidity in other major organs: Myocardial sarcoidosis results in sudden cardiac death where granulomas within the heart cause dysfunctional (fatal) heart rhythms.   In men, sarcoidosis can affect the testes and cause infertility. Sarcoidosis can affect how the body handles calcium giving rise to kidney failure. Neurologic (brain and spinal cord) involvement is observed as bilateral facial nerve palsy, optic nerve dysfunction causing blurred vision or blindness, swallowing dysfunction, and hearing abnormalities. Appearance can be adversely affected by “mump-like” bilateral parotid salivary glandular obstruction and enlargement.

Sarcoidosis and cancer can mimic one another, making the distinction difficult. Diagnosis of sarcoidosis is often a matter of exclusion. Common symptoms are vague, such as fatigue unchanged by sleep, lack of energy, weight loss, aches and pains, arthritis, dry eyes, swelling of the knees, blurry vision, shortness of breath, a dry hacking cough and  skin lesions. Differential diagnoses include metastatic cancer, lymphoma, septic emboli (blood clots), rheumatoid nodules, mycobacterial infections and fungal disorders. Cases presenting with pulmonary symptoms require evaluations via chest Xray, CT (computer topographic) scan of chest, bronchoscopy with lung biopsy, lymph nodes biopsy and tuberculosis bacilli stain of tissue to be able to make a definitive diagnosis of sarcoidosis.

Perhaps Alan will be one of the lucky ones; more than half of the people who have symptomatic sarcoidosis have spontaneous remission (disease becomes in-active, but it can return) within three years of diagnosis. There’s no cure for sarcoidosis, but treatments help manage the symptoms. Prednisone and other corticosteroids are the first-line treatment for sarcoidosis; however, this treatment has side-effects. Oral corticosteroids can cause weight gain, mood swings, insomnia and weakened bones. There are other medication used to treat sarcodosis life-threatening symptoms and they have correspondingly severe side effects: Anti-rejection drugs (used in organ transplant maintenance) reduce inflammation by suppressing the immune system; thereby reducing granulomas formation in lungs, heart and brain, but these drugs carry HIGH risks for bone marrow suppression. Anti-malarial drugs are helpful in controlling sarcodosis-associated skin disease, nervous system pathology and elevated blood-calcium levels (that damage the kidneys), but have the potential to harm the eyes (cause cataracts and blindness). Anti-cancer drugs (TNF-alpha inhibitors) are commonly used to treat the inflammation associated with sarcoid arthritis; potential side effects include congestive heart failure, blood disorders and lymphoma.

For more information about sarcodosis go to the American Lung Association Website at: http://www.lung.org/lung-disease/sarcoidosis/understanding-sarcoidosis.html and sarcodosis help group websites: http://www.jsof.org/ and http://www.sarcoidlife.org/.

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Ignored Hypertension Kills

Silent Killer

She was all dressed up and her hair was perfect; her cheeks were rouged and she
wore her favorite lipstick.  She was picture perfect.  The mortician had done
a fine job.  It really wasn’t that difficult because she was only 44 years old and still had a youthful appearance.  She was the victim of the “Silent Killer”, hypertension.

Alice was too busy to eat right; between her career, school and taking care of her
elderly parents she often forgot to take medicine twice a day as her doctor
advised following her mandatory annual physical examination.  She did not feel bad and her head hurt only occasionally.  When she got really stressed, her vision blurred and it seemed that she could hear her heartbeat throbbing in her ears, but that did not happen very often.

Fifty million Americans have hypertension and an estimated fifteen million do not know that they have it. Hypertension, or high blood pressure, is a chronic medical condition in which the blood pressure throughout the body’s blood vessels is levated.  The cause of  high blood pressure vary; some cases (95%) result from conditions that affect the kidneys, arteries, heart, or endocrine system, other cases (5%) have unknown causes. Untreated persistent hypertension results in strokes, heart attacks, heart failure,arterial aneurysms, and chronic kidney failure.  Measured blood pressure values represent the systolic and diastolic blood pressures within blood
vessels. Systolic blood pressure is the blood pressure within vessels generated
during a heartbeat. Diastolic blood pressure is the pressure present within
blood vessel and capillaries between heartbeats (heart is at rest). The American
Heart Association defines hypertension as a systolic blood pressure greater
than 130 and diastolic blood pressure greater than 80.  Blood pressure readings are measured in millimeters of mercury (mmHg) and are recorded as two numbers; for example, 130 over 80, written as 130/80 mmHg. Hypertension is diagnosed when blood pressure is persistently elevated when measured at three separate times  within one month.

The risk of developing high blood pressure increases with age. Hypertension is a condition most diagnosed in men until the age of 45; from age 45 to 54, men and women are equally at risk for high blood pressure. After the age of 54, the incidence of hypertension flips such that women are more likely to have high blood pressure than men. The medical community still debates the reason for this, but it appears that estrogen, produced in abundance in young women, has a protective role in blood pressure control by maintaining blood vessel flexibility and working with other hormones to reduce the risk of high blood pressure in younger women.

People with relatives who have hypertension are more likely to develop high blood pressure; this observation is referred to as a “positive family history”.  African
American and Native American ethnicities have greater incidence and prevalence
of high blood pressure compared with white Americans. African Americans develop
hypertension earlier in life and their average blood pressures are much higher than other ethnic groups. People who regularly eat foods that are high in salt and those who are overweight (more than 25 pounds above the recommended weight or a body mass index greater than 30) are more likely to have high blood pressure. Studies
have also shown that people with heightened anxiety, intense anger, and
suppressed expression of anger are more at risk of developing hypertension.

People with high blood pressure usually experience no symptoms unless their blood pressure is extremely high or if they have had untreated high blood pressure for a long time and there is end-organ damage. In these cases, the heart, brain, kidneys, and the small blood vessels within the eyes are damaged before the time of diagnosis. These symptoms are reported by patients with poorly managed hypertension: chest pain, confusion, ear buzzing noise, irregular heartbeat, nosebleed, tiredness, and blurred vision. When a patient’s blood pressure is high enough to warrant the
use of medications for better control, positive lifestyle changes improve the
patient’s prognosis (predictive outcome).Very few patients have hypertension
that is not well-controlled with the combination treatment of appropriate medication and healthy lifestyle practices. Biofeedback and relaxation exercises are alternative holistic treatments aimed at reducing psychological stress that have been shown to provide improved blood pressure management.

Initial assessment of all patients should include a complete medical history and physical examination. Physicians are trained to look for warning signs of pre-hypertension which is defined as having a systolic blood pressure between 120 and 139 and a diastolic blood pressure between 80 and 89 measured during three or more clinic visits. Having pre-hypertension indicates that a person is more likely to develop high blood pressure and experience complications if untreated.  Once the
diagnosis of hypertension has been made, physicians work to identify the underlying cause based on symptoms described by the patient, obvious risk factors  such as obesity, family history of hypertension and associated diseases and at-risk lifestyle. Blood and urine laboratory tests are performed to identify possible causes of high blood pressure and any associated health problems such as diabetes and high cholesterol levels which are additive factors for the development of heart disease.

Establishing a lifestyle that prevents hypertension is the recommended line of defensive against this condition;people need to eat nutritious food, exercise regularly, and maintain a healthy bodyweight, all of which have been shown to significantly reduce the incidence of hypertension. However, when a diagnosis of hypertension is realized, treatment must start with lifestyle changes: (1) Lose weight; excess weight adds to strain on the heart. In some cases, weight loss may be the only treatment needed;  (2) Exercise regularly for 30 minutes/ day ; (3) Eat a diet rich in fruits, vegetables, and low-fat dairy products while reducing total and saturated fat intake;  and (4) Avoid smoking.

Alice can’t hear the sadness in her husband’s voice as he speaks to the
assembled mourners. “She was kind, dedicated to others, loving and determined.
No one expected that she would just leave us like this.” …..Not even Alice.

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Getting Healthy

—    UNDERSTANDING ALTERNATIVE MEDICINE CHOICES
   Alternative medicine practices are as diverse in their foundations as in their methodologies. Practices may incorporate or base themselves on traditional medicine, folk knowledge, spiritual beliefs, or newly conceived approaches to healing. Home remedies are natural cures made at home from natural ingredients such as
fruits, vegetables and herbs. They are simple with no side effects, uses no processed chemicals, and usually
inexpensive.  In earlier times mothers were entrusted with concocting serious remedies to keep husbands work-ready and improve the survival of their children. Natural remedies can be used to effectively treat a range of ailments through correct and regular usage.
       The human body has its own life-force which sustains it. When we look for herbal remedies in natural substances, we want something which is easily assimilated and safe.  Herbal  remedies work with the body to repair it, without interfering with its natural processes. One of the best known home remedy is the use of chicken soup to treat respiratory infections such as a cold. A medically proven successful home remedy is Willow bark tea which was used to cure headaches and fevers (willow bark contains salicylic acid, which is chemically similar to aspirin). —Many people utilize mainstream medicine for diagnosis and basic information, while turning to alternatives for therapy or health-enhancing measures. —Chinese medicine and Oriental medicine includes acupuncture, herbology and massage therapy. —Acupuncture and  Chinese medicine schools provide holistic training and techniques that utilize knowledge and principles of Yin Yang, the Five Phases and the body as an interconnected system. Acupuncture dates back to prehistoric times, with written records from the second century. Treatment is based on the existence of qi or meridians which are  concepts central to acupuncture theory. There is general agreement that acupuncture is safe when administered by well-trained practitioners. —Alternative approaches are often used in conjunction with conventional medicine.
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