*3.4.3. Lung function and infections*

be tested before conception. Genetic counsellors should be involved in order to discuss these

Genetic tests have a sensitivity <100% with detection rates from 70% to 95% of CFTR mutations. If the partner has not been tested, given a CF carrier frequency of 1:25, the risk of an affected

When the partner is a known carrier or if he has not been tested, clinicians should suggest to perform prenatal genetic diagnosis to the couple, with the analysis of chorionic villus sample (CVS) within the first trimester of gestation. This procedure includes technical risks that should

Psychological counselling is also an important part of the counselling activities: CF team should provide information about sexual health and reproduction to all of their patients, particularly teenage girls. Psychologists and clinicians play an important role also in psycho‐ logical counselling for CF women who want to become pregnant, even if women with advanced disease with a very strong wish to have a child may proceed whatever the advices.

Most drugs have not been tested on pregnant women. Relevant issues about their use in pregnancy are timing of exposure (periconception, first, second, third trimester, or perinatally), systemic availability of the drug, and its ability to cross the placenta. Side effects can consist in teratogenesis, growth retardation, death, renal insufficiency, neurological disorders,

Many pregnancies are unplanned and drugs could have been taken at the time of conception and continued in the first weeks of pregnancy, and many women with serious illnesses required treatment to be continued. Even if nowadays there is experience to guide prescribing in pregnancy, the principle remains to avoid drug use where possible, except when the risk of

The Swedish FASS information catalog provides information on the risks of drugs to the fetus during pregnancy and to the infant during lactation. Each drug is classified to one category of

**1.** Drugs that have been used widely during pregnancy and are assumed safe for the fetus

**2.** Drugs not known to cause harm to the human fetus but with insufficient experience to

**6.** Drugs that could theoretically cause harm to the fetus by their pharmacological actions

infant is 1:50; while if the partner is a CF carrier, the risk is 1:2.

the drug is outweighed by the risk of the condition being treated.

consider them safe. This category can be subdivided into

**7.** Drugs known or believed to cause harm to the fetus

**4.** drugs with insufficient animal data

**3.** drugs that have been demonstrated to cause no harm in animal studies

**5.** drugs that have been demonstrated to harm the fetus only in animal studies

delicate issues with the couple.

122 Cystic Fibrosis in the Light of New Research

be discussed with the couple.

*3.4.2. Medications during pregnancy*

stillbirth, etc.

safety:

CF women can become pregnant in all pulmonary disease stage, but the outcome for the mother and the newborn is closely related to lung function (FEV1 predicted) and clinical stability. Lung function should be optimized and chronic infections may be suppressed before pregnancy: oral flucloxacillin could be administered in case of *Staphylococcus aureus* coloniza‐ tion; for *Pseudomonas aeruginosa* nebulized colistin or aminoglycoside treatment should be employed.

When a pregnancy is unplanned, pregnancy is frequently connected to a worsening in lung function: if necessary, one or more courses of IV antibiotic treatment could be administered in the usual format also during pregnancy (β-lactam + aminoglycoside).

During the first trimester, most patients will feel breathless and the frequency of hospitaliza‐ tions could rise up: at each visit, physical examination, sputum cultures, weight and oxygen saturation measurement, and pulmonary function test should be performed.

### *3.4.4. Physiotherapy*

If the pregnancy is planned, preconceptional period could represent a good opportunity to optimize the daily physiotherapy program. Inhalation therapies and techniques should also be reviewed and optimized; the timing of these treatments in relation to airway clearance therapy (ACT) is a relevant feature during pregnancy, especially in CF patients who produce big volumes of sputum. ACT adherence and technique have to be adapted in pregnant women. Advice on physical exercise and pelvic floor strength should be given as soon as possible.

The breathing pattern is affected by physiological and mechanical changes during pregnancy, and in the last trimester these features can lead to increase in closing volume and determine atelectasis. Physiotherapists should meet the pregnant patients weekly to optimize the physiotherapy regimen, monitor lung function and sputum production in terms of colour and quantity. Also maintaining exercise capacity should be useful during pregnancy and in last trimester.

### *3.4.5. Nutrition*

Dietetic counselling is crucial in preconceptional period, because maternal nutritional state is one of the most important factors influencing outcomes for mother and infant. A low prepregnancy BMI is strictly associated with reduced birth weight. Preconceptional assessment is similar to non-CF population and should be performed by a CF specialist dietitian, who can advise not only on increasing energy density of the diet but also oral supplements and invasive nutritional support (enteral tube feeding) can be purposed to the patient if nutritional status is unsatisfactory. Vitamin supplementation with folic acid, vitamin A, and vitamin D are recommended.

An overall weight gain of 12.5 Kg is considered normal and in CF it is recommended a weight gain of at least 11 Kg. Also gastro-oesophageal reflux, heartburn, nausea, recurrent vomiting, and constipation may occur more frequently in women with CF and required clinicians monitoring.
