Introduction

Constipation is a problem among some people with osteogenesis imperfecta. In the medical literature there are two studies that indicate that children and adults who have OI Type III and pelvic deformity are more likely to have constipation and complaints of abdominal pain (Lee, 1995 and Violas, 2002). Constipation, in medical terms, is a decrease in frequency of stools or bowel movements with hardening of the stool. As a consequence, individuals with constipation may have feelings of being bloated and having gas but find it difficult to pass stools. Because the stool becomes quite hard, passing it may be uncomfortable and lead to further unwillingness to try, especially in infants and young children. Fecal impaction, or clogging of the bowel with hardened stool, can be a serious complication. Treatment of constipation in people with OI is often challenging.

Causes of Constipation

Constipation can be caused by inadequate dietary fiber, decreased activity, and dehydration. Colon obstruction and illness, leading to immobility, are additional causes. Some medications, especially pain medications, can be constipating because they decrease bowel wall motion. Adults with OI and parents of children with OI are encouraged to check with their doctor and/or pharmacist about side effects from their medicines and dietary supplements or for possible drug interactions.

OI may contribute to constipation especially in individuals with short stature and pelvic deformity associated with OI Type III. It is not clear whether the major contributing factor is simply the diminished mobility of people with OI Type III. Some doctors believe that in people whose hips and pelvis are either narrow or deformed, the bowel and colon may be "crowded," preventing them from functioning normally. A pelvic deformity known as acetabular protrusion is not uncommon among people with OI Type III and may contribute to a predisposition toward constipation as a consequence of a decreased volume in the pelvis.

A frequent concern is that children with OI who have increased perspiration may be slightly dehydrated, also contributing to constipation.

Managing Constipation

Managing constipation usually involves a combination of approaches. It is essential to determine the extent of the problem and develop a plan to correct it.

* Keep a record of bowel movements, diet and fluid intake. Even when a child appears to be "regular," keeping records about diet, illnesses, exercise and bowel movements will help caregivers figure out if something in particular triggers the problem.

* Be consistent about diet, exercise and activity and fluids.

Children with OI often respond to diet changes, a change in activity level, and mild home remedies. The primary care doctor should be consulted regarding the appropriateness of "home remedies." A nutritionist or registered dietician may offer useful suggestions for modifying the diet. If the problem persists, a gastroenterologist may be needed.

Treatment choices include the following:

* Diet and fluids

* Exercise and activity

* "Home remedies"

* Medications

Diet and Fluids

Dietary changes can include:

* Add fiber to the diet including whole grain breads, whole grain cereals, bran cereals or muffins, and popcorn. Ground beans can be added to other foods.

* Increase amount of fruits, fruit juice and vegetables.

* Add beans and nuts to the diet. For example serve chili with beans.

* Reduce amount of processed foods and refined sugars.

* Avoid "junk" foods that are high in fat and low in fiber.

* Include foods such as yogurt with active cultures that contain the bacteria lactobacillus acidophilus.

* Limit soft drinks and drinks containing caffeine such as colas or tea.

* Drink water throughout the day.

Exercise and Activity

Adding exercise or movement can help to prevent and relieve constipation. Exercise helps move digested food through the intestines. Infants, children and adults who sit, recline, or use a wheelchair require regular position changes. Individuals should consult with their primary care doctor and physical therapist about beneficial exercises. Water play that encourages an infant to kick, swimming, and walking are examples of beneficial exercise.

Home remedies

Home remedies can include:

* Karo syrup

* Prune juice mixed with a good quality apple juice or applesauce to make it more palatable.

* Allowing enough time on the toilet. Children's Hospital of Philadelphia suggests 10-15 minutes at least twice a day.

* Enemas should only be used with a physician's recommendation.

Medications

For some children, regular medication or even a prescription laxative may be necessary. Use of
medication, whether prescription or over-the-counter, must be discussed with your physician. Children on a medication need to be carefully monitored. Taking a medication too often can reduce its effectiveness.

Medications can include:

* Suppositories

* Mineral oil

* Stool softeners

* Laxatives such as Senokote®

* Prescription laxatives such as Miralax®

Confer with the doctor about all treatment options, including "home remedies." Persistent, painful constipation should not be ignored, and a referral to a gastroenterologist may be required.

References

Lee JH, Gamble JG, Moore RE, Rinsky LA. Gastrointestinal Problems in Patients Who Have Type III Osteogenesis Imperfecta. The Journal of Bone and Joint Surgery, 1995, Sep; 77: 1352-6.

Violas P, Fassier F, Hamdy R, Duhaime M, Glorieux FH. Acetabular Protrusion in Osteogenesis Imperfecta. Journal of Pediatric Orthopedics, 2002, Sep-Oct; 22: 622-5.

Web site: The Children's Hospital of Philadelphia www.chop.edu.

This fact sheet was prepared by the Osteogenesis Imperfecta Foundation with assistance from Dr. Horacio Plotkin, pediatrician, Children's Hospital, Omaha, NE.

This information is brought to you by the
NIH Osteoporosis and Related Bone Diseases~National Resource Center (ORBD~NRC)
and the Osteogenesis Imperfecta Foundation

National Institutes of Health
Osteoporosis and Related Bone Diseases
National Resource Center
1232 22nd St., NW
Washington, DC 20037-1292
Tel: 800/624-BONE or 202/223-0344
Fax: 202/293-2356, TYY: 202/466-4315
http://www.osteo.org
E-mail: orbdnrc@nof.org

The National Resource Center is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases with contributions from the National Institute of Child Health and Human Development, National Institute of Dental and Craniofacial Research, National Institute of Environmental Health Sciences, NIH Office of Research on Women's Health, Office of Women's Health, PHS, and the National Institute on Aging. The Resource Center is operated by the National Osteoporosis Foundation, in collaboration with the Paget Foundation and the Osteogenesis Imperfecta Foundation.