If you were asked to name the common symptoms of digestive diseases, abdominal pain and diarrhea might be the sort of symptoms you would list, but what about losing your eyesight? If that seems unlikely, you would be right, but Whipple’s disease does exist, and damage to your eyesight is a distinct possibility if you leave this rare disease untreated.
Though this infection is commonly found in the small intestine, getting an untreated infection of the bacterium Tropheryma Whipplei could result in an infection in your central nervous system that could damage your joints, eyes, heart, brain, or even end your life.
What is Whipple’s Disease?
Unlike other gastrointestinal problems that are usually constrained to the tissues of your digestive tract, Whipple’s disease is defined by the National Institutes of Health (NIH) as a multi-system bacterial infection that interferes with your ability to metabolize fats.
First clearly identified in the early 1900s, Whipple’s disease is a rare bacterial infection that, while easily treatable with modern antibiotics, can be debilitating or fatal if left untreated. Though generally diagnosed as a gastrointestinal disease, the T. Whipplei bacteria responsible for this infection can spread throughout the body. By attacking your central nervous system, many of your organs could be at risk from what begins as an intestinal infection.
The most common symptoms of the disease in its early stages are gastrointestinal in nature, with anemia, diarrhea, abdominal pain, malabsorption of nutrients in your food, weight loss, weakness, and fatigue being present in many cases.
Unlike many other intestinal diseases or conditions that tend to remain localized to your digestive tract or abdomen, the pathogenesis of T. whipplei infections can spread beyond your small intestine where infections generally begin, and more varied symptoms can begin to manifest. These can affect a wide range of tissues and organs throughout the body, and can even have neurologic manifestations. A few common symptoms include the following:
- joint pain (arthralgia)
- loss of hearing
- facial numbness
- changes in personality or memory problems
- difficulty walking or loss of mobility
- various eye problems, such as uveitis
Whipple disease can even be responsible for creating other diagnosable conditions that can arise from various causes. Endocarditis is a diagnosis of inflammation of the lining and muscles of the heart can be caused by various bacteria or even fungal infections. Though treatment for this condition usually involves a course of antibiotics, it is possible that surgery could become necessary. Lymphadenopathy, or a change is the size, consistency, or number of lymph nodes, is also an inflammatory disease, and may result in you needing to have biopsies done on lymphatic tissue to rule out malignancy in your lymphatic system.
How does Whipple’s Disease Affect Your Intestines?
T. whipplei infections, also known as intestinal lipodystrophy, typically begin in the mucosa of the small intestine. If the offending bacteria becomes lodged there and an infection forms, lesions can form that begin to thicken the tissue that lines the intestine. This thickening can eventually destroy the viili, which are the finger-like projections that are responsible for absorbing nutrients out of your food.
These lesions in the lamina propria can eventually disrupt the natural process of digestion. If enough damage to the mucosa occurs, you can begin to suffer from nutrient malabsorption. This can eventually lead to severe malnutrition.
As your body struggles to digest food, some nutrients such as fats or albumin can become difficult to process. This can eventually lead to other health problems throughout your body as the fats your body needs to keep various chemical processes in check begin to fail.
How do you Catch Whipple’s Disease?
Like all infectious diseases, contracting Whipple’s disease requires you to come into contact with the bacteria itself. What is unclear is exactly how this occurs when it comes to t. Whipplei. There is no clinical evidence of human to human transmission, and there is some question of how common the bacteria is in human digestive tracts.
There is some evidence that this bacteria, which is commonly found in soil and water samples, could be encountered more often by people who work in trades that could bring them into more regular contact with this soil-dwelling bacteria.
What has been verified in salivary samples is that many healthy people have this bacteria living in their gut flora without any symptoms. This raises the question of what causes some people to experience symptomatic infection while others remain healthy.
Is Whipple’s Disease Hereditary?
Unlike some diseases with clear hereditary causes, T. whipplei infections are caused by bacteria, so theoretically anyone who comes into contact with Tropheryma Whipplei bacteria could be at risk for infection.
That said, there is an identifiable increase in the likelihood you will get the disease based on your genetic makeup. Genetic studies have found that individuals with the HLA-B27 antigen have a predisposition to develop symptoms when other people may not.
Aside from distinct genetic markers, there are concentrations of the incidence of T. Whipplei disease in certain areas or populations around the world. In the United States, the disease is more prominent in white men between the ages of 30 and 60. Throughout the world, however, rates of the disease tend to be higher in children, especially in areas where sanitation is lacking.
How is a T. Whipplei Infection Diagnosed
T. Whipplei infections can involve many tissues throughout the body, but from a healthcare perspective, this disease is typically addressed through the field of gastroenterology. Nearly all cases of T. whipplei infections manifest in lesions in your small intestines, so the best way to confirm a diagnosis is through an upper endoscopy.
In this procedure, a thin, flexible tube called an endoscope is threaded down your esophagus and through the stomach. A small camera is attached to the tube to allow your gastroenterologist to see what is going on inside your body. During endoscopic examinations, it is sometimes also possible to take a biopsy of tissue from lesions to confirm the presence of T. Whipplei bacteria.
Conclusively diagnosing a Tropheryma Whipplei infection is important, as the symptoms accompanying an infection can mimic other conditions and diseases of very diverse origin. Everything from HIV infections, cerebrovascular disease, infectious diarrhea, mesenteric lymphadenitis, and lymphoma can all present with similar symptoms, but require very different treatments.
A conclusive T. whipplei diagnosis may require different diagnostic techniques depending on how advanced a given case is. In cases where it is suspected a T. Whipplei infection has moved from your intestines into the rest of your body, other tests may need to be done to help understand the extent of your infection. One critical test is sampling cerebrospinal fluid to determine whether or not the infection has spread to your nervous system.
How Do You Treat Whipple’s Disease?
As a bacterial infection, the primary treatment for Whipple’s disease is antibiotic treatment. There are several classes of drugs that can be used, though the first line of defense is typically penicillin or related drugs from that family. Ceftriaxone is another drug that can be used in initial stages of treatment. Symptoms of the infection typically disappear over the one to four weeks after treatment begins.
In addition to clearing up the infection itself, replacing lost fluids and nutrients is crucial to helping you recover as quickly as possible. In many cases this means electrolyte replacement, vitamin, and nutrient supplements. Your body may be missing vital nutrients such as calcium, magnesium, iron, and folate which help your body heal and fight off infections. If your diagnosis has been delayed, your body may already be weakened and need help resolving any ongoing infection.
Recurrences of Whipple’s disease are common, as the bacteria that causes the disease is common and re-infection is possible. For this reason, long-term treatment with trimethoprim-sulfamethoxazole is common for up to one year after your initial diagnosis.
Follow-up appointments are an essential part of treatment to help prevent a relapse. During this phase of treatment, more testing will be done to verify that the offending bacteria has, in fact, been eradicated. It is often recommended that a biopsy be performed a year after your initial infection to help verify the efficacy of treatment. This can involve looking for traces of the infection through polymerase chain reaction (pcr) testing in stool or saliva samples, and can even involve electron microscopy to look for presence of macrophages and bacilli, which can persist long after your initial treatment is over.
Getting Care for Whipple’s Disease
Unlike other infectious diseases, Whipple’s disease is not known to pass from human to human. This means there is little fear of catching it from someone else. This is no reason to take an infection lightly, though. Following the guidelines of healthcare professionals is essential in recovering. With the very real possibility of damage to the heart, brain, and other organs, this is an infection that should be taken very seriously.
COVID-19 has disrupted many things in the last year, but it is important not to let it disrupt your access to healthcare. Leaving other conditions untreated can have drastic consequences, and this can be especially tragic in cases of T. whipplei infections where early diagnosis can bring about effective treatment in many cases.
At Cary Gastroenterology, we are constantly working to ensure the highest standard of patient care possible, even during the ongoing pandemic. In addition to our several physical locations, we now have a fully featured telemedicine option to help answer questions and get you on the road to the diagnosis and treatment you need from the comfort and safety of your own home.
If you are having concerning symptoms that could indicate a threat to your gastrointestinal health, don’t wait. Reach out to us through our telemedicine services online, or request an appointment today.