Tag Archives: pathology

My mother’s pathology – sarcoidosis

A lifetime of medication, and side-effects. At some point my mother began to store package inserts of the drugs she was prescribed, annotated with her reactions to them.

At 30 years old my mother had a radical mastectomy, meaning her left breast was removed along with the chest wall muscle underneath, and the lymph nodes under her left arm. Years later as a medical student I learned that she had not needed this extreme surgery. But back then the distinctions between types and stages of breast cancer were still being worked out, and my mother and her surgeon opted not to take any chances.

The mutilating operation changed the course of her life. She also underwent some form of ovarian ablation (her ovaries were rendered non-functional so that they produced no hormones which might stimulate the cancer to grow.) This brought on early menopause, further adding to the negative effects on her physical health and psyche.

Ten years after the breast surgery she complained of chest pain and a cough. Her chest X-ray showed a “white-out”, a blizzard obliterating both of her lungs. As I remember it, the possibilities were lymphangitis carcinomatosa (indicating cancer finely spread throughout the lungs), bird fancier’s lung (an allergic reaction to her parrot), tuberculosis (a chronic bacterial infection) or sarcoidosis (an inflammatory disease with no known cause). She had a bronchoscopy (for which she refused anaesthesia or sedation) and the biopsy sample was compatible with TB or sarcoidosis, since they have a very similar appearance under the microscope. Because tuberculosis is rife in South Africa, she was started on a course of anti-tuberculous drugs, which made her very unwell. The polymerase chain reaction (PCR) was a brand new form of testing back then, but training as a virologist I was able to arrange a PCR to detect Mycobacteria tuberculosis in the biopsy tissue, which proved negative. Her treatment was changed to the only available option for sarcoidosis, which was high dose prednisone. She fought to have the dose reduced to a level where the side-effects were tolerable. After more than a decade of treatment the disease seemed to “burn out”, leaving her with lung fibrosis (scarring) and bronchiectasis (dilated airways).

There are many similarities with my mother’s story in this case of pulmonary sarcoidosis from our collection. However this patient died soon after diagnosis.

By now, in her 50s, her skeleton had become dangerously osteoporotic (brittle), this because of the long-term prednisone treatment and premature menopause. Attention turned to her bone density and a number of drugs were tried to improve it, but all of them induced problematic side effects.

In her late 60’s chest symptoms occurred again, and a right-sided pleural effusion (fluid between the lung and the chest wall) was found. This needed repeated drainage and finally the pleural space was simply eliminated by fusing the lung to the chest wall, using a chemical irritant. By now she also had signs of pulmonary hypertension and cor pulmonale (right heart failure), and required a pace-maker for atrial fibrillation (heart flutter).

At age 73 the final event was sudden. Overnight she became desperately ill, and in the emergency unit her oxygen saturation was low. Laboratory tests showed that her acute pneumonia was caused by parainfluenza-3 virus, a virus usually seen in very young children. She required high flow oxygen in intensive care and was given massive doses of cortisone to try to prevent further inflammation in her lungs. She sank into delirium with a steroid psychosis. Already weakened by pulmonary cachexia (wasting), she stopped eating and then drinking.

Cause of death on on her death certificate was “end-stage lung disease”. I don’t recall if sarcoidosis was specifically mentioned.

There is little lung with which to breathe in this case of long-standing pulmonary sarcoidosis. There is fibrosis (scarring) of the pleural surfaces as well as a lot of fibrosis around the bronchi (airways) within the lung. Fibrosis of blood vessels within the lungs causes pulmonary hypertension.

At most, 1 in 20 people diagnosed with sarcoidosis will die of the disease, usually of respiratory failure. Although the inflammatory response in sarcoidosis has been well studied, the actual cause of the disease remains unknown. Aside from the lungs, it commonly involves lymph nodes, skin and liver, and less often other organs such as the heart and eyes. 

The full story of any disease is really only known through personal experience of it.