Chapter 11: Cystic Fibrosis

Question 1

Cystic Fibrosis (CF)

Answer 1

chronic, progressive, genetic multisystem disease

• a recessive disorder, may inherit from both parents
• A genetic disorder, in which the lungs and the digestive system get clogged with mucus.

Question 2

Pathophysiology

Answer 2

the major CF gene product is the protein cystic fibrosis transmembrane regulator (CFTR)

• when CFTR is not produced or altered in structure or function as a result of mutations in the CF gene, viscid secretions are produced that primarily affect the respiratory and gastrointestinal systems
• an absence or abnormality of the protein results in a blocked or altered chloride channel in the epithelial cell membranes; chloride ions are trapped within the cell and cause sodium ions and water to diffuse back into the cell, leading to dehydration and the mucus secretions

Question 3

Effects on Pregnancy

Answer 3

factors that may predict a poor outcome for a pregnant woman with CF include pre-pregnancy evidence of poor nutritional status, significant pulmonary disease with hypoxemia, and pulmonary hypertension

• the risk of maternal mortality with cor pulmonale and deteriorating pulmonary function in the first trimester is high, and may be advised to consider termination of the pregnancy
• risks to the fetus include preterm birth, growth restriction caused by uteroplacental insufficiency and CF

Question 4

Counseling Women with Cystic Fibrosis

Answer 4

pre-conception counseling is essential for women with CF
-because of extremely thick cervical mucus, fewer than one in five woman with CF are able to conceive
>women who achieve pregnancy, should be advised of the risks according to their health status

Question 5

Management: Nutrition

Answer 5

• important to recognize the fetus is at risk for uteroplacental insufficiency and IUGR
• maternal nutritional status and weight gain during pregnancy greatly affect fetal growth; ongoing collaboration with nutritionist is essential; close attention paid to patients nutritional status because maldigestion, malabsorption, and malnutrition are all complications of CF
• ensure caloric intake is met; especially if experiences hyperemesis gravidarum

Question 6

Management: Early recognition

Answer 6

early recognition and prompt treatment of pulmonary infections
-diagnostic tests include sputum and culture sensitivity, chest films, spirometry, pulse oximetry, and complete blood counts with a chemistry panel

Question 7

Management: Respiratory

Answer 7

baseline pulmonary function tests such as forced vital capacity (FVC), forced expiratory volume (FEV1), lung volumes, pulse oximetry, and arterial blood gases indicated
-lab values closely monitored; any deterioration in pulmonary function is addressed

Question 8

Management: Fetal growth

Answer 8

fetal growth should be monitored by fundal height and serial ultrasound evaluations for fetal growth and amniotic fluid volume
-doppler flow studies are also used to assess fetal well-being

Question 9

Management: During Labor and Birth

Answer 9

fetal oxygenation is of most importance

• continuous monitoring of maternal oxygenation saturation using pulse oximetry is used to gauge the need for and response to supplemental oxygen therapy
• respiratory depressants, anticholinergic drugs, and inhalation anesthesia should be avoided
• lumbar epidural analgesia is the preferred method for pain relief
• the fetal heart rate (FHR) should be continuously monitored during labor, and signs of fetal intolerance (i.e. decelerations and/or lack of FHR variability) must be addressed
• provide assistance to express couples feelings and emotional concerns, keep informed of changes as the labor progresses and encourage them to frequently ask questions