The Story #13: The Diagnosis

With the exception of occasional visits to a chiropractor, I stopped seeing doctors in October 2005 when I could no longer afford medical insurance. Seeing doctors seemed useless anyway since they couldn’t figure out what was wrong with me, and the few medicines they prescribed weren’t helping me get well. I figured if I was ever going to get well or try to sue for malpractice, I needed to know what was wrong with me. The answer had to be in the binders full of test results I had amassed.

It’s one thing to keep focused on a project when you’re healthy. It’s quite another to try to sift through all this medical data and make sense of it when you can’t even hold a train of thought due to illness. The only way I could get a handle on what I was trying to accomplish was to start from the beginning and write down what I’d found out chronologically. That way, if I lost my concentration, I could go back and read where I left off. If I had a thought, I’d write it down in a notebook before I forgot and deal with it at the appropriate time. Thank God I took good notes after my doctor visits and procedures.

I researched the significance of each positive test finding by scouring the internet and medical books I had acquired. This was no easy task because you really have to dig past all the superficial information out there to find what’s useful. I found that if you ask five people the same question, you end up with five different answers. They could all be right — just approaching the subject from a different perspective. Some could have old information that’s changed. Science knowledge changes exponentially. Some could just be misguided. You use your best judgment.

The breakthrough came when I stumbled across a Korean radiologist’s study on abdominopelvic actinomycosis involving the gastrointestinal tract and cross referenced his CT scan findings with the findings on my CT scan reports. The wording matched precisely. Then I started examining other correlations between findings of patients with actinomycosis in his and other studies and my test result reports.

I found that actinomycosis is notoriously hard to diagnose. Acute or chronic signs of infection may be absent. The patient’s temperature may be within the normal reference range. White blood cell counts can be in the standard reference range or mildly elevated. According to Merck, symptoms are a local abscess with multiple draining sinuses, a TB-like pneumonitis and low-grade septicemia. There is usually mild, predominantly polymorphonuclear leukocytosis (white blood cells that appear to have multiple nuclei) and a normochromic anemia (red blood cell hemoglobin is within the standard reference range.) The erythrocyte sedimentation rate and C-reactive protein may be moderately raised as with any chronic illness, but these do not confer any diagnostic advantage. Plain chest radiograph findings in actinomycosis are nonspecific.

The clinical frequency of the disease in the U.S. diminished with the advent of antibiotics. Once a common disorder, actinomycosis is now said to occur in 1 of 300,000 cases. Some postulate that the occurrence is higher, but that the disease is either misdiagnosed or goes undiagnosed because the organism is sensitive to antibiotic administration, particularly penicillin G and VK.

Early undiagnosed occurrences of the disease may go into remission with the use of short courses of antibiotics prescribed for suspected bacterial infection only to recur in later years. There is a clinical pattern of remission and exacerbation of symptoms occurring in parallel sequence with initiation and cessation of antibiotic therapy.

Because the disease is rarely diagnosed, most physicians are unfamiliar with any aspect of the illness. It’s extremely difficult to find a practicing physician in the U.S. who knows anything about actinomycosis, even though www.healthatoz.com states that “cervicofacial actinomycosis can be diagnosed by a family doctor or dentist and the patient may be referred to an oral surgeon or infectious diseases specialist.” It also seems nearly impossible to get a practicing physician to consult research physicians, known for their study of actinomycosis. (Patients are prohibited from consulting research physicians directly because research physicians typically aren’t licensed to treat patients.)

Diagnosis of a case of actinomycosis in England in 2008 even made headline news because the disease was largely thought to have been eradicated from developed nations. Chest consultant Dr. Syed Tariq diagnosed Mrs. Christine Wicks of Long Sutton as suffering from actinomycosis — a diagnosis that eluded the numerous doctors Wicks saw over many years.

Because the clinical presentation is nonspecific, definitive diagnosis is generally based on a pathologist’s identification of actinomycotic granules, the characteristic “sulfur granules” (small yellow colored material in the pus) from a biopsy or tissue specimen. The surgical pathology report from my tonsillectomy in 2001 found actinomycotic granules in tonsilar crypts accompanied by an abundant accumulation of inflammatory cells.

Because actinomycetes are normal micro-organisms found in the mouth, especially in periodontal pockets, in dental plaque and calculus, and in the tonsilar crypts, the presence of actinomycotic granules by itself may not indicate infection. It’s said that up to 40% of surgically removed enlarged tonsils will show one or more colonies of Actinomyces, sometimes large enough to appear as yellowish grains of rice. The offending pathogens assume a parasitic role when they result in infection with an inflammatory tissue response.

Research found that the inflammatory response occurs due to the synergistic presence of associated bacteria, often co-existing gram-positive or gram-negative cocci and rods. Actinomycotic infections are polymicrobial with other pathogens (typically staphylococci, streptococci, enterobacteriae and Candida) frequently cultured from lesions. It’s possible that these microorganisms enhance the pathogenicity of Actinomyces by creating an anaerobic milieu in which Actinomyces thrive.

This polymicrobial aspect of the disease has been little studied. Yet treatment that addresses simultaneous eradication of all infecting pathogens may hold the key to faster recovery.
Actinomycosis is a chronic, slowly progressive, pus-forming disease in which the organisms invade tissues, produce a granular discharge, extend to contiguous structures across anatomic boundaries and form lesions with burrowing sinus tracts and fistulas (narrow, elongated channels in the body that allows the escape of fluid). Actinomycosis begins as an inflammatory soft tissue mass, which can enlarge into an abscesslike swelling. The condition forms a hard lump of tissue and tightens the muscles around it. The surrounding tissue swells up and can leak pus through narrow passages (sinuses) onto the surface of the skin.

Unlike other bacterial infections, actinomycosis moves easily from one layer of tissue to the next. It can move to other parts of the body without traveling through the lymphatic system. The infection is most commonly caused by Actinomyces israelii, gram-positive, anaerobic and rod colonizing pathogens normally present in healthy individuals, especially in the mouth, tonsilar crypts, the head and neck region, the bronchi, the gastrointestinal tract (particularly the cecum), the colon, and the pelvis.

Actinomycotic infection takes several forms and has been reported from virtually every site of the head and neck.

The cervicofacial form usually begins as small, flat, hard, sometimes painful swellings in the mouth, on the skin of the neck or below the jaw. The infection can spread to the cheek, tongue, throat, the tubes that connect the throat to the nose, the larynx, the trachea, salivary glands, the ears, the scalp, facial bones, brain or the tissues surrounding the brain (meninges). If the infection spreads to the meninges (the membranes that cover the brain and spinal cord), meningitis can develop. The main symptom of cervicofacial actinomycosis is the presence of hard lumps in the soft tissue in the head and neck region (thus the acronym “lumpy jaw”.)

Traumatic disruption of mucosa enables organisms to penetrate into deeper tissues following surgical procedures, such as tonsillectomy, oral surgery, root canal therapy or infection. Periapical actinomycosis is reported usually in association with trauma to a tooth or dental procedure. Actinomycosis of the teeth usually presents with painful local swelling.

Ischemia (tissue damage due to lack of oxygen caused by constriction of blood vessels) usually accompanies the trauma. The anaerobic bacterium is difficult to treat because it thrives in deep tissues where oxygen levels are low. An aerobic environment stops the infection. Exposure to an aerobic environment may compromise biopsy specimens, so prompt transport of specimens (preferably in an anaerobic transport device) to the microbiology lab is necessary for isolation of actinomycetes.

The thoracic form causes chest pain, possible fever and a cough that brings up sputum. Pulmonary disease results from aspiration of oral secretions and resembles TB.

The abdominal form is caused by swallowing saliva contaminated with the bacteria or a break in the mucosa of a diverticulum or the appendix or during trauma. One or more abdominal masses that cause signs of partial intestinal obstruction occur most often in the ileocecal region. Usually the cecum, the appendix and peritoneum are infected. Pain, fever, vomiting, diarrhea or constipation are characteristic of infection affecting the intestines and lining of the abdominal cavity.

The pelvic form is typically caused by complications or perforations from certain types of intrauterine device (IUD) contraceptives.

The generalized form of actinomycosis results when bacteria are carried in the blood to the skin, vertebrae, brain, liver, kidney, ureters and, in women, the uterus and ovaries. Diverse symptoms, such as back pain, lower abdominal pain and headache, may occur.

Slowly developing abscesses branch out with a fibrotic scarlike texture that is easily mistaken for carcinoma or tuberculosis. (My mom was said to have died of carcinoma, and my aunt was afflicted with tuberculosis.) Fistula formation across normal tissue planes leads to invasion of viscera, joints, bone and/or the central nervous system. Progression of the disease leads to multifocal involvement — a diagnostic challenge that could easily result in a failed diagnosis. In fact, the pathogen, originally classified as a fungus, is now classified as a branching bacteria.

Signs of the disease can be found on cross-sectional imaging. CT of the pelvis depicts diffuse bowel wall thickening, enhancement of the pelvic fat, obliteration of tissue planes and intensely enhancing pseudotumoral phlemonous foci with or without abscess formation. Ascites and lymphadenopathy are not typical features of this disease. MRI with its multiplanar capability illustrates the infiltrative behavior of the disease.

A CT scan of my pelvis and abdomen taken on March 11, 2005 found a stool-filled colon with decompression of the descending colon, questionable bowel wall thickening and enhancement of the sigmoid colon. Lack of fat planes within the pelvis made evaluation difficult. There was no ascites nor retroperitoneal or pelvic lymphadenopathy seen. The cecum was redundant and extended into the deep right pelvis. Dr. Elise Berman, the radiologist, recommended further evaluation through colonoscopy to exclude an underlying obstructing left colonic mass, which she and the prescribing gastroenterologist, Dr. Carol Schuffler, suspected might be colon cancer.

On May 12, 2005 Gastroenterologist Dr. Ronald Barkin performed a colonoscopy at Inova Alexandria Hospital. The operative report noted notable diffuse inflammatory change with ulceration in the descending colon, the sigmoid colon and rectum. The pathology report confirmed the presence of prominent infiltrate of lymphocytes, neutrophils and eosinophils within the lamina propria and surface erosion.

Treatment for actinomycosis requires long term antibiotic therapy and may require adjunct surgery. The course of treatment is two to four weeks of intravenous penicillin, followed by six to 12 or more months of oral antibiotics. Anecdotal reports suggest hyperbaric oxygen (oxygen under high pressure) therapy in combination with the antibiotic therapy has been successful.

The cure rate for certain forms of the disease with early intervention and appropriate antibiotic therapy is over 90%. More than 50% of those with actinomycosis of the brain and spinal cord have persisting neurologic damage, and more than 25% die. Untreated, the disease causes various complications including degeneration of the spine and osteomyelitis. A three-view radiograph of my lumbar spine obtained from Virginia Hospital Center October 2008 as part of my application for Social Security Disability Insurance showed “severe osteoarthritic changes” and degenerative disc disease.

Concisely, my self-diagnosis is based upon:

1. A pathology report indicating the presence of actinomycotic granules in inflamed tonsilar crypts from a biopsy taken during my tonsillectomy in 2001.

2. A CT scan of my abdomen taken March 2005 showing questionable bowel wall thickening, enhancement of the sigmoid colon, a stool-filled colon with decompression of the descending colon as well as a redundant cecum that was extending into the deep right pelvis.

3. A colonoscopy performed May 2005 which found abnormal mucosa located mid-sigmoid with diffuse inflammatory change with ulceration in the descending colon, the sigmoid colon and the rectum.

4. The presence of hard lumps in the soft tissue of my head and neck, particularly in the submandibular region. (Signs of “lumpy jaw.”)

5. Missing left and right lower molars, extracted due to infections coming from the root of the tooth in the last few years. Multiple oral and periapical surgeries and root canals.

6. Pap smears positive for bacterial infection. (I had a copper 7 IUD removed due to perforation and infection in 1979 in the emergency room of what was then Prince George’s General Hospital now run by Dominion Healthcare.)

7. Various x-rays of my spine taken from the 1990s through October 2008 demonstrating severe osteoarthritic changes and degenerative disc disease — the bone destruction repercussions from not treating the infection.

~ by doctorblue on November 12, 2008.