Reduces inflammation that causes damage to lung tissue; slows lung deterioration

Trimethoprim sulfamethoxazole (Bactrim DS); tobramycin; clindamycin; piperacillin; other aerosol antibiotics

Depends on drug and patient


Prevent and treat lung infections

Dornase alfa recombinant

2.5 mg inhaled once daily using a recommended nebulizer


Breaks down the DNA from neutrophils, loosening secretions

Pancrelipase; Lipase

0.7 g of powder with meals


Aids in digestion of fats and proteins

Experimental Therapies: New peptide antibiotics (PA-1420, IB-357, IB-367, and SMAP-29) are being investigated as treatment for resistant bacteria. Gene and lung transplants are also being studied, and families should be informed of these options.


Educate to reinforce the importance of regular CPT and expectoration of the mucus. Encourage increased fluid intake to loosen the secretions, and provide frequent mouth care before meals. Teach the parents not to offer cough suppressants, which can lead to obstruction, lung collapse, and infection.

Support the child's or adolescent's body image concerns; compliment the patient on her or his strengths. Encourage the child to develop in as many areas as possible. Very often, other CF patients become a significant support group as the child matures. The child is always dramatically affected when another peer with CF dies. Plan group discussions with the patients and have a psychiatric nurse clinical specialist serve as facilitator of this grief work for both patients and staff. In addition, siblings often worry that they may contract the disease or they may exhibit feelings of jealousy of the attention given to the sibling with CF. A referral to a social worker or the Cystic Fibrosis Foundation may be needed.

Counsel couples on the risk that subsequent pregnancies may result in a child with CF, since there is a one in four chance with any pregnancy that a child could have CF if both parents are

264 Cystitis carriers. Discuss the role of amniocentesis and the difficult issues surrounding terminating a pregnancy if CF is confirmed prenatally.

• Physical response: Pulmonary assessment; color, odor, character of mucus; cardiac and GI assessment; pulse oximetry

• Nutritional data: Weight, use of enzymes, adherence to supplemental feedings

• Emotional response: Patient's feelings about dealing with a chronic illness, patient's body image, parents' coping ability, siblings' response

Teach the patient and family how to prevent future episodes of pneumonia through CPT, expectoration of sputum, and avoidance of peers with common colds and nasopharyngitis. Explain that medications need to be taken at the time of each meal, especially pancreatic enzymes and supplemental vitamins. Teach the parents protocols for home IV care, as needed. Teach parents when to contact the physician: when temperature is elevated over 100.5°F, sputum has color to it, or the child complains of increased lung congestion or abdominal pain. Also educate parents on the need to keep routine follow-up appointments for medication, laboratory, and general checkups. Teach the patient or parents proper insulin administration and the appropriate signs and symptoms of high and low glucose levels.

Cystitis is an inflammation of the bladder wall, which may be acute or chronic. It is generally accepted to be an ascending infection, with entry of the pathogen via the urethral opening. Non-infectious cystitis is referred to as interstitial cystitis (IC), but this is a poorly understood disorder with an uncertain cause. In this condition, in spite of symptoms of cystitis, the urine is sterile. The person develops a decreased bladder capacity, possibly because of healing of bladder ulcers (called Hunner's ulcer) that leave behind scar tissue. If IC is associated with chemical agents that lead to bleeding, it is termed hemorrhagic cystitis; otherwise, IC may also be termed painful bladder disease (PBD).

Although cystitis occurs in both men and women, the incidence in women is significantly higher. Sexually active women have 50 times more cystitis than men in general. Females are more susceptible to cystitis because of their short urethra, which is 1 to 2 inches long, as contrasted with the male urethra, which is 7 to 8 inches in length. The placement of the female urethra, which is closer to the anus than is the male urethra, increases the risk of infection from bacteria in the stool.

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