Other symptoms may include

Diagram showing major structures of the human gastrointestinal tract.

The inflammatory bowel disease (IBD) of HPS is similar to Crohn's disease.  It is associated with HPS-1, 3, and 4 and causes significant symptoms in around 15 percent of people with HPS.  It is usually seen in the teens’ or early adulthood but can start earlier in childhood. There is no cure at present but treatment can help to control some of the symptoms in many cases. The cause of the inflammation is not understood.

The main symptoms include

Symptoms depend on which part of the gastrointestinal tract is affected.  The severity of symptoms typically fluctuates over time in a less than predictable relapsing remitting pattern with ‘flare-ups’ occurring in between intervals of remission or reduced symptoms. Flare-ups can occur without warning signs.

Inflammation of the lining of the intestine can result in ulceration, or ‘granulomas’.  The ulcers can become infected and form abscesses.  The IBD of HPS is more correctly referred to as a ‘glaucomatous Crohns-Like colitis’.  Unlike ulcerative colitis, ulcers can occur individually or in isolated patches.  Inflammation and ulcers can occur at any point along the intestinal tract from the gums to the anus but most commonly affect the small intestine and bowel (colons).  In some cases the ulcers may bleed and the blood may be seen in the stools or diarrhoea.

Care plans

The care \ treatment plans for people with HPS often closely resemble those for Crohn’s disease with one or two exceptions.

Although the ‘Crohn’s like’ colitis of HPS has its own distinct characteristics the following website might be useful: http://www.nacc.org.uk/content/home.asp


Like Crohn’s disease the symptoms may be controlled to some extent with drug therapies and, infact, the range of drugs used is typically the same.

Patients may receive drugs based on a pyramid or "Step up" approach.  Those with milder symptoms receive less powerful drugs from lower down in the pyramid with less side effects. As symptoms worsen, or if patients are sicker when diagnosed, they may receive stronger drugs. In some cases people may receive stronger drugs earlier depending on the severity of symptoms and extent of disease.  Although drugs can be effective in controlling the symptoms patients must take medications continuously to slow down disease progression and reduce relapses.  Doctors sometimes prescribe special liquid feeds during flare-ups to rest the intestine  If you stop or change your drugs for any reason you should discuss it with your doctor.

Diet & Nutrition

No specific diet has been shown to improve or worsen this IBD but specific food problems may vary from person to person.  Individuals may find that certain types of foods worsen diarrhoea and symptoms, especially during times of active disease.  Due to anaemia and mal-absorption some people might be prescribed an iron supplement, vitamin B12, calcium and vitamin D supplements to help keep your bones strong,


If medicines do not work, a type of surgery called ‘resection’ may be needed to repair or remove a damaged part of the intestine or to drain an abscess.  Unlike conditions such as ulcerative colitis removing a damaged portion of the intestine does not cure the condition. Sometimes it is possible to perform surgery with minimally techniques such  as Laproscopy.  Surgery might be necessary when there are complications such as:

What are fistulas

A fistula is an abnormal channel or passageway connecting one internal organ to another, or to the outside surface of the body. Many fistulas (or fistulae) involve the bowel or intestine. So, a fistula might connect two parts of the bowel to each other, or the bowel to the vagina, bladder, or outside skin. Fistulas may occur when inflammation spreads through the thickness of the intestine wall. When this happens in the lower parts of the bowel it is particularly likely to cause small leaks and abscesses (collections of pus). As the abscess develops it may ‘hollow out’ a chamber or hole.  This then becomes a passage or channel linking the bowel to another loop of bowel, another organ, or the outside skin.  When the abscess bursts, the puss will drain away, but the passage or channel may remain as a fistula.  Read more about Fistulas.

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Crohn’s-like inflammatory bowel disease of HPS

Diagram showing the major structures of the human gastrointestinal tract.  Crohns-like inflammatory bowel disease of Hermasnky-Pudalk Syndrome