Whipple disease: Causes, Symptoms, Treatment and Cost

Whipple disease is a rare infectious bacterial illness caused by the bacterium Tropheryma whipplei. It affects multiple organs in the body, including the digestive system. Whipple disease is often misdiagnosed or underdiagnosed due to its rarity and mild presentation in some cases.

There are three main types of Whipple disease:-

• Classic Whipple Disease: This type involves inflammation and destruction of multiple organs including the small intestine, heart, lungs, joints and central nervous system. Symptoms can range from gastrointestinal issues to neurological problems such as dementia or depression.
• Oculomucocutaneous Syndrome: This type affects primarily mucous membranes in the eyes (conjunctivitis), skin (dermatitis) and joints (arthritis). The most common symptom is ocular discharge, although it may also cause pain in multiple joints or sensitivity to light exposure.
• Neurological Form: This type primarily affects neurological symptoms such as memory loss, confusion or difficulty speaking or understanding speech due to encephalopathy in the brain caused by Tropheryma whipplei bacteria taking up residence there.

• Whipple disease is caused by a bacterium called Tropheryma whipplei.
• Risk factors include a weakened immune system from certain medications or conditions, and exposure to contaminated food or water.

• GI symptoms: Abdominal pain, diarrhoea, weight loss and malabsorption of nutrients.
• Neurological Symptoms: Cognitive decline, seizures, psychiatric disturbances and weakness in the arms or legs.
• Respiratory System Symptoms: Coughing, shortness of breath or difficulty breathing.
• Cardiovascular System Symptoms: Fever, fatigue and rapid heart rate.

• Whipple disease can be prevented by regularly practising good hygiene, practising safe sex, and eating a healthy diet.
• Wash hands often with soap and water, avoid contact with anyone who has an infection, avoid sharing food items, utensils and other personal items.
• Eating a balanced diet that includes fruits, vegetables, whole grains can boost immune system strength and reduce your risk of developing Whipple disease.
• Hepatitis A and B vaccinations should also be taken to reduce the chances of contracting the bacteria that cause Whipple disease.

Diagnosis and tests for Whipple disease include:

• Blood Tests:Blood tests such as complete blood count (CBC), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) can be done to check for infection, inflammation, or other abnormalities in the body.
• Faecal Test:A faecal test may be performed if the patient has gastrointestinal symptoms such as diarrhoea, pain, or malabsorption. The test looks for T. whipplei bacteria in a stool sample and is a reliable way to diagnose Whipple's disease.
• Microscopic Examination:To search for T. whipplei bacteria, samples of body fluids or tissue may be examined under a microscope to look for clumps of bacteria known as “volutin granules” characteristic of Whipple's disease.
• Antibody Testing:Diagnosis of whipple disease can be made by testing a patient’s serum for antibodies to Tropheryma whipplei. This usually involves the use of ELISA (enzyme-linked immunosorbent assay) or an antigen-capture assay, which measures IgM, IgA and other antibodies against the bacteria in a patient's blood sample. A positive result shows that the body has developed an immune response to fight off the infection, making it likely that Whipple disease is present.
• MRI/CT scan Imaging:MRI and CT scans provide exceptional anatomical views of internal organs and structures, allowing for the detection of organ abnormalities, tissue damage, and other signs associated with Whipple disease.

• Nervous system complications:Cognitive decline, Seizures, Weakness, Memory loss and Tremors.
• Gastrointestinal complications:Abdominal pain, Diarrhoea, Loss of appetite and Malnutrition.
• Respiratory complications:Difficulty breathing and Chronic cough.
• Cardiac complications:Cardiac arrhythmia and Congestive heart failure.
• Rheumatic complications:Joint pain and Arthritis.

Infections caused by opportunistic microorganisms such as fungi or bacteria in the lungs or brain can also occur.

• Drink freshly extracted juice of ginger, turmeric and garlic on an empty stomach.
• Consume cooked vegetable soups 2-3 times a day.
• Increase intake of food items rich in Vitamin B like wheat germ, bananas and potatoes.
• Take multi-vitamins and calcium which can be prescribed by an ayurvedic practitioner.
• Add honey to warm water and consume it twice a day for better immunity building results.

• In Whipple disease, eating a healthy, well-balanced diet is important in helping to manage the symptoms, strengthen the immune system, and improve overall health.
• Patients should focus on increasing their intake of fruits and vegetables, lean protein sources, whole grains, and healthy fats while avoiding processed foods and high-fat animal products.
• Additionally, it is also beneficial to incorporate dietary supplements such as probiotics and omega-3 fatty acids since these have been shown to reduce inflammation and provide other health benefits.

• Raw meats and seafood:Uncooked meat, poultry, and seafood should be avoided as they can potentially be sources of bacterial contamination like salmonella or E coli.
• Raw eggs:Foods such as mayonnaise and eggnog which contain uncooked eggs are also high risk for contamination and should not be eaten.
• Unpasteurized dairy products:Dairy products like raw milk can also contain T whipplei, so only pasteurised dairy products should be consumed.
• Unwashed fruits and vegetables:It is important to thoroughly wash all fresh produce before it is consumed as this can help reduce potential bacterial contamination from the soil or water where it was grown/cultivated.

• Lifestyle changes:Stress management, getting regular exercise, and avoiding smoking are all important measures to take when living with Whipple Disease as they can all reduce inflammation of the intestines and other organs that could be negatively impacted by the disease.
• Supplementation:Taking a multivitamin and mineral supplement may help to improve nutritional status in those affected by Whipple Disease due to malabsorption issues arising from this condition.
• Long-term antibiotic therapy:Long-term antibiotic therapy is often required for the treatment of Whipple disease. This is to help control and manage the bacterial infection, as well as reduce the symptoms associated with it.
• Intestinal Resection Surgery:Removal of a portion of the small intestine (duodenum) where bacteria accumulate.
• Appendectomy:Removal of the appendix that is often associated with Whipple disease.
• Enterostomy:Creation of an opening in the stomach to allow food and liquids to bypass the affected part of the intestines while they heal.
• Re-sectioning:Surgical cutting away dead tissue, infection, and blockages caused by Whipple disease in parts of the digestive system or organs connected to it, such as the pancreas or liver.
• Bowel Anastomosis Surgery:Reconnection of separated segments or parts of the small intestine after removal or re-sectioning during surgery for Whipple disease treatment; restoring normal flow for digestion and absorption in the faeces (stool).

A doctor who specialises in infectious diseases should be consulted for a diagnosis and treatment of Whipple disease. A doctor specialising in gastroenterology may also be helpful as this condition mainly affects the gastrointestinal tract.

• Antibiotics:These are the most commonly prescribed medication for Whipple disease, and they are typically used to fight bacteria that may be causing the symptoms of the disorder. Examples include clarithromycin, doxycycline, and TMP-SMX.
• Corticosteroids:Steroids can help reduce inflammation in the body caused by Whipple disease and improve overall energy levels. Prednisone is a common corticosteroid used for this condition.
• Immunosuppressants:These can help control inflammation and restore some organ function that might have been affected by Whipple Disease. Methotrexate is one example of an immunosuppressant.
• Probiotics:Probiotics may help reduce diarrhoea related to Whipple Disease and restore gut health. Examples of probiotic supplements include Lactobacillus acidophilus and Bifidobacterium breve.
• Enzymes:Enzyme therapy can be used to replace missing enzymes in the body which would otherwise be unable to digest food particles properly due to cells destroyed during Whipple Disease progression.

It typically takes several months for patients with Whipple disease to recover. The period of recovery can vary depending on the extent and severity of the infection, as well as the treatment prescribed by a healthcare professional. Following treatment, patients usually return to normal health in a few weeks or up to a few months.

The treatment for Whipple disease is aimed at restoring the body's normal functions, and can provide significant improvement in many patients.

However, the results of this treatment are often not necessarily permanent, and symptoms may recur due to potential relapses. The duration of symptom recurrence varies from patient to patient, with some experiencing long-term remission, while some may experience recurrent episodes of infection.

• Prevention of complications:Careful monitoring is recommended postoperatively, including assessment for signs of infection and wound healing.
• Pain management:Patients typically receive pain medications following surgery, typically opioid based narcotics. These medications should be taken as prescribed and gradually tapered off until the patient’s discomfort is manageable without them.
• Diet restrictions:Depending on the type of procedure, dietary restrictions may need to be kept in place for a certain amount of time after the procedure to allow for proper healing. A low-fat diet is usually recommended postoperatively as well as an increase in fibre intake to encourage healthy bowel function and prevent constipation which can occur due to taking pain medications or other postoperative factors.
• Follow up care:Following surgery regular follow up appointments are necessary with your physician or provider to assess healing progress, discuss any concerns or issues that arise during recovery, adjust medications if needed, monitor laboratory work, etc.

The cost of treating Whipple disease in India varies and depends on the severity of the condition and which type of treatment is chosen. Generally, hospitalisation and medication costs range from 50,000 to 5 lakh rupees. The cost also depends on the doctor’s fee and laboratory tests. The cost can be reduced up to 20% if health insurance coverage is available for Whipple disease treatment.

• Common side-effects of Whipple disease treatments include nausea, vomiting, abdominal pain, diarrhoea, and fever.
• Other more serious side-effects include anaemia, low blood counts, allergic reactions to medications, pancreatitis and other gastrointestinal problems.
• In rare instances there can be complications from medications such as liver damage or excessive bleeding.
• Additionally, the vaccinations that may be necessary for treating this condition can pose side-effects such as fever and redness at the injection site.

If you are suffering from any complications relating to whipple disease then you should consult a doctor nearby as they can cause complications like 'cognitive decline, malnutrition, cardiac arrhythmia' in which treatment courses can range from a few months to years depending on the severity of the situation.