Cystic Fibrosis is an inherited genetic disorder occurring in approximately 1 in 2,500 live births. The disease is characterized by a disruption in sodium and chloride transport across cells due to a defective protein present in the genes. As a result, the body produces abnormally thick, sticky mucus, causing problems virtually everywhere in the body, but particularly in the respiratory and digestive systems.
The biggest challenge to the digestive system in persons with CF involves the failure of the pancreas to secrete the enzymes necessary to break down food before it reaches the intestines. A number of supplemental synthetic enzymes have been developed by pharmaceutical companies to aid in this process. However, complications can still occur.
Thick mucus present in the intestines prevents the body from absorbing vitamins, nutrients, fats and proteins thus rendering a person with CF malnourished. Blocked intestinal and pancreatic ducts contribute to episodes of bowel obstruction, which occur when stool content blocks the bowel and prevents the digestion process from proceeding normally.
One of the first symptoms of bowel obstruction is pain, localized in the lower portion of the abdomen, to the patient’s right. Early on, this pain is easily confused with appendicitis since it may present with similar symptoms including cold sweats, nausea and vomiting. This area is the most likely location for a bowel obstruction to occur, since that is where the small intestine and large intestine come together.
Anti-pain medications should be used with great caution at this point, since they may actually work to slow down the body’s functions. It is best to use low-dose over the counter medications rather than asking the doctor to prescribe something stronger like a narcotic (e.g. vicodin, dilaudid).
As the obstruction becomes larger, the area becomes increasingly painful; an accompanying stomach ache and/or nausea is evidence to the fact that abnormal stool is collecting in an area where it should not be. The liquid that should be present in the intestine as a result of the digestion process cannot proceed because of the blockage and therefore cannot become fecalized. It is at this point that water content is lost and constipation occurs, requiring intervention of some sort.
If the obstruction is only partial and diagnosed early, treatment by means of dietary changes may be used. Foods high in natural water content, such as melons or carrots, may help alleviate the loss of water in the intestines. Additionally, fiber supplements and plenty of water may help move the obstruction along naturally. However, since the digestive system of the CF patient generally does not function as smoothly as that of the general population, even partial bowel obstructions may require more aggressive treatment.
Enemas, stool softeners, and ingestible liquids like GoLytly® may be utilized to break up the obstruction. As unpleasant as these can be, they are highly effective in many patients and can work quickly to relieve the pain and discomfort. Complete bowel obstructions accompanied by very intense pain and weakness, may indicate that the supply of blood to the intestine is being cut off. When this occurs, surgery is most likely the course of treatment.
A bowel obstruction resolved by means of enemas or GoLytly® enables the patient to feel better almost immediately. Recovering from surgery will of course, take longer. In either case, care must still be taken to ensure that the digestive system is not overly taxed and that the likelihood of recurring blockage is reduced. A CF patient should discuss with his or her specialist whether an increase in the number of enzymes is appropriate, as well as which foods should be avoided for a time. Initially, the doctor may recommend an all liquid diet to help replenish a dehydrated system. Gradually the patient may be permitted to progress to soft foods and then a normal diet.
One of the most important things to remember about nutrition and bowel obstruction is the different between roughage and fiber. Fiber is found in things like oatmeal, bread, and the fleshy parts of fruit (e.g. the white part of an apple.) Roughage on the other hand are vegetables, greens, and the skins of fruit. As a general rule of thumb, if you cannot chew the food into something mushy before you swallow it, it’s probably roughage. The obvious exception to this rule is meat.
Adequate nutrition for persons with cystic fibrosis is an ongoing battle, made even more complicated by things like bowel obstruction and malabsorption. However, the right types of food in the right amounts may do much to boost the overall health and energy of the cystic fibrosis patient.
