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What

Whipple’s disease is a rare systemic disease that affects the small intestine, the joints, the central nervous system (the brain and spinal cord) and the cardiovascular system. A certain strain of bacteria infiltrates these areas of the body and causes a variety of complications. However, the major complications in Whipple’s Disease involve the intestine. This bacterial infection damages the lining of the small intestine and interferes with the body’s ability to absorb certain nutrients from this area of the digestive tract.

Who

Whipple’s Disease characteristically occurs in middle-aged Caucasian men, but it can also occur in children. The earliest reported diagnosis of the disease is at 3 months of age. Whipple’s Disease is very rare with fewer than 500 total reported cases. The disease is rarely described in females. The ratio of males to females is 8:1.

Signs and Symptoms

Whipple’s Disease usually has the following symptoms:

• Diarrhea
  
• Intestinal bleeding
  
• Abdominal pain/cramps
  
• Loss of appetite
  
• Weight Loss
  
• Fatigue
  
• Weakness
  
• Joint pain
  
• Arthritis
  
• Fever
• dementia
  
• Heart problems (heart failure, heart murmurs)
  
• Neurological Problems

Possible Causes

Whipple's disease is caused by a bacterial strain called Tropheryma whippelii. The clinical manifestations of the disease are believed to be caused by infiltration of the various body tissues by T whippelii. Malabsorption is the major complication in Whipple's Disease. The T whippelii bacterium infiltrate into the lining of the small intestines, thicken the lining and destroy the tiny hairlike structures (villi) in the walls of the intestines that absorb the nutrients from the foods we eat. When villi are destroyed, intestinal lesions are created that can lead to the diarrhea, blood loss, and all the other intestinal symptoms listed above as well as malabsorption of the foods we eat leading to malnutrition, weight loss, fatigue and weakness.

Diagnosis

The diagnosis of Whipple’s disease is based on the clinical presentation and the results of a biopsy of the small intestine or other affected areas of the body. (A biopsy is a special test where a small amount of tissue from the body is removed and examined under the microscope). Other lab tests may also be abnormal in children with Whipple’s Disease, especially if there is a problem absorbing nutrients. The abnormal laboratory tests can include: low blood counts, low cholesterol, low calcium, low iron, increased bleeding times, low protein, low magnesium, and low vitamin B12 levels. More recently a technique called PCR (polymerase chain reaction) has been used to detect the DNA of T whippelii, thus increasing the likelihood of Whipple’s disease as the diagnosis.

Treatment

When recognized and treated, Whipple's disease can be cured. Antibiotic medications are used to treat the disease because they destroy the T whippelii bacteria. These medications are usually prescribed for a 12 to 18 month period. Supplements such as extra iron, folate, vitamin D, calcium, and magnesium may also be given to help compensate for the vitamins and minerals the body was not absorbing on its own prior to treatment.

Prognosis

Without treatment, Whipple’s Disease may be fatal. With treatment, especially when caught early, the chance of a good outcome is greatly increased. Relapse is very common, even after successful treatment, so patients need to be observed for some time and seek more treatment if symptoms reoccur.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

National Digestive Diseases Information Clearinghouse
This website provides a general description of Whipple’s Disease

Medline Plus Medical Encyclopedia
This site has general information on the disease.

Ecureme.com/Whipple’s Disease
Great site with more information on Whipple’s Disease

Google Search for Whipple's Disease

References and Sources

1. Roberts, I. (2003), Whipple Disease www.emedicine.com/med/topic2409.htm 2. Bobustuc, G. (2002) Whipple Disease www.emedicine.com/NEURO/topic397.htm 3. Andreoli, Cecil Essentials of Medicine 5th ed., Saunders, p.722