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Medicines for cystic fibrosis

Medicines that may be used to treat cystic fibrosis include:

medicines called CFTR modulators that help the lungs work better by reducing the effect the altered cystic fibrosis gene has on cells in the lungs

medicines to widen your airways and make breathing easier, or make it easier to cough up mucus

antibiotics for infections

steroid medicine to reduce inflammation in the airways

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Repiratory signs and symptoms of cystic fibrosis;

A persistent cough that produces thick mucus (sputum)

Wheezing

Exercise intolerance

Repeated lung infections

Inflamed nasal passages or a stuffy nose

Recurrent sinusitis

Digestive symptoms of cystic fibrosis;

Foul smelling, greasy stools

Poor weight gain and growth

Intestinal blockage, particulary in newborns (meconium ileus)

Chronic or severe constipation, which may include frequent straining while trying to pass stool, which can lead to rectal prolapse

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Balanced Diet-often high calorie to maintain weight

Respiratory medications to aid with lung function, this may include bronchodilators (relax the airways), steroids, antibiotics, mucolytics (loosen thick mucus in lungs to cough it up easier), CFTR modulators (help to correct the function of the CFTR protein, which is faulty in CF)

Physiotherapy to help loosen sticky secretions in the lungs

Enzymes to replace the naturally produced ones that cannot reach their destination. Pancreatic enzymes to help absorb nutrients from food

Vitamin Supplements - A,D,E and K

Nutritional supplements

Dietary Salt

Lung Transplant

Support of an MDT team and regular appointments

Ongoing emotional support

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CFTR modulators that help lungs work better by reducing the effect the altered gene has on the lungs.... Antibiotics to prevent chest infections .... Physiotherapy to help with breathing and posture ... Physical therapy to help with draining the lungs of the excess fluid ... Inhalers to help with breathing and mucus clearance ... Dietary and nutritional support to help with digestion ... Lung transplant eventually if other treatments do not work

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iol is usually recommendeddepending on lung function

cs will be recommended but most give birth naturally at 37 weeks due to respiration difficulties

early pain management intervention

higher risk of preterm labour

continue treatment during labour

epidural recommended

continuous ctg monitoring

different position due to breathing issues

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Antenatal considerations for CF

MDT involvement

Pre pregnancy counselling

Genetic counselling (PN too)

Respiratory monitoring plan by obstetricians/ respiratory consultant

More monitoring/ infection surveillance

Fetal surveillance- risk of IUGR

Diabetes screening (more frequent glucose monitoring due to risk of CF related diabetes)

Medication review

Plan for birth (vaginal unless indicated otherwise)

Anaesthetic review- consideration of support during labour

Birth in obstetric led unit

Psychological support

PN planning- feeding, monitoring (both mum and baby)

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Symptoms of asthma attack and available treatments for management

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Symptoms of asthma attack and available treatments for management

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Symptoms:

Severe shortness of breath

Wheezing

Coughing

Chest tightness

Pale or sweaty

Difficulty speaking

Blue lips or finger-nails

Drowsiness

Confusion

Fainting

Treatment:

Use of a quick-relief inhaler

Oral corticosteroids

Staying calm

Call emergency services if symptoms do not improve

Long-term treatment: avoid triggers such as dust or pollen, monitor with a peak flow meter to assess the on coming asthma attack, ensure that vaccinations are up to date eg flu to minimise the risk of getting infections that could increase respiratory infections that could trigger the asthma

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Delivery by 37 weeks dependant on lung function

Early pain management (epidural)

CF team involvement

Consider birthing position (upright may put less pressure on lungs)

Higher risk of pre term labour

Continue treatment as per CF team guidance

Elective LSCS if poor lung function

Interventions considered to shorten 2nd stage

Continuous CTG monitoring

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During your pregnancy you will have monthly multidisciplinary clinic reviews where a consultant, specialist nurse, physiotherapist, dietician and pharmacist will review you.

A cystic fibrosis social worker and psychologist are also available for support.

The cystic fibrosis team will be in close contact with your obstetrics team to plan your pregnancy and delivery. Regular airway clearance is important throughout pregnancy to reduce the risk of complications. Often in the early stages of pregnancy you will continue with your usual physiotherapy regime. Your usual airway clearance regime may need to be reviewed and optimised at this stage. This may involve a change of technique or device, a change in frequency of airway clearance or a change of position.The use of nebulisers such as DNase and hypertonic saline may be recommended or increased during pregnancy to help optimise airway clearance.

Kaftrio is not licensed for use in pregnancy and so the decision to continue it is individual to each patient. Many people choose to continue Kaftrio to support their health through pregnancy and we are not aware of harm caused to mother or baby.

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Most pregnant women with asthma experience changes in the condition during pregnancy, thought to be due to fluctuating hormone levels and tend to follow what has been termed the ‘rule of thirds’. That is, one third of women will maintain similar asthma or allergy control during pregnancy, one third of women will improve, and another third will worsen. Women with poorly controlled asthmas are more likely to worsen.

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Most pregnant women with asthma experience changes in the condition during pregnancy, thought to be due to fluctuating hormone levels and tend to follow what has been termed the ‘rule of thirds’. That is, one third of women will maintain similar asthma or allergy control during pregnancy, one third of women will improve, and another third will worsen. Women with poorly controlled asthmas are more likely to worsen.

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Asthma exacerbation may occur at any time during pregnancy though is most likely between 29-36 weeks gestation.

With asthma exacerbation there is increased risk of adverse perinatal outcome. Maternal asthma can cause hypoxia due to decreased placental blood flow. When maternal oxygen falls rapidly during an exacerbation, fetal oxygen saturation can be compromised.

Severity of asthma symptoms during pregnancy may also be influenced by the baby's gender. Carrying a female baby is associated with asthma exacerbations and a near double incidence of hospitalisation for asthma as well as a higher incidence of intrauterine growth restriction.

Some studies have found an increase in complications such as placental abruption and premature rupture of membranes, which might contribute to the higher rate of caesarean births among women with asthma.

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Prophylactic antibiotics to prevent infection

Early mobilisation and chest physiotherapy for lung health

Emotional support as they are more at risk of postnatal depression

Side room may be given to help prevent infection

Constant coughing with CF increases the chance of urinary incontinence making pelvic floor exercises more important

Ensure adequate nutritional supplements especially for those BF

Medication may have been changed in pregnancy so weighing up the pros and cons of going onto better medication vs BF

Ensuring early heel prick to screen for CF

Regular visits from CF team for ongoing management

Considering long term contraception to promote appropriate birth spacing

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Considerations for labour and postnatal, including asthma plans

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Considerations for labour and postnatal, including asthma plans

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Asthma plans;

Medications available;

preventer inhalers

reliever inhalers

MART and AIR inhalers

Montelukast

Theophylline

Avoiding ibuprofen for pain relief

A review with a GP or nurse early in pregnancy is crucial to discuss asthma management strategies and how pregnancy may affect asthma.

Share your asthma action plan with your midwife and ensure it's included in your birth plan.

Continue to exercise and maintain a healthy diet but consult with your doctor if exercise triggers asthma symptoms.

Continue using your preventer inhaler (steroids) as prescribed to maintain good asthma control. Asthma attacks can reduce oxygen levels in the blood, potentially impacting fetal growth.

Poorly controlled asthma can increase the risk of complications during pregnancy, such as pre-eclampsia, premature birth, and low birth weight.

Children of parents with asthma may have a slightly increased risk of developing asthma.

Limit contact with known asthma triggers, such as pet fur and grass pollen.

Seek guidance from your GP or pharmacist on safe hay fever treatments during pregnancy, as hay fever can worsen asthma.

Regular tests, including spirometry and peak flow monitoring, may be necessary to check lung function.

If you have indigestion, discuss it with your GP or nurse, as it can worsen asthma.

Consider getting the flu jab in pregnancy, according to the NHS.

If you have moderate to severe asthma or poorly managed asthma, your doctor may recommend additional fetal monitoring, such as ultrasounds or non-stress tests.

Smoking during pregnancy increases the risk of breathing problems for the baby, including asthma, and complications for the mother.

Labour care;

During labour care for women with asthma, the primary focus is on maintaining good respiratory control and providing appropriate pain relief. This includes continuing prescribed asthma medications, closely monitoring the mother and baby, and considering pain management options that minimize the risk of asthma exacerbation, such as epidurals.

During pregnancy, women with asthma should prioritize maintaining well-controlled asthma to minimize risks to themselves and their baby. This includes having a review with their GP or nurse, avoiding smoking, sharing their asthma action plan with their midwife, and managing hay fever and indigestion.

Anaesthesia

If anaesthesia is required, regional anaesthesia is preferred over general anaesthesia for virtually all CS as this reduces airway irritation and potential postoperative complications.

For women with severe asthma, an anaesthetic consult early in pregnancy is recommended to establish a labour plan including anaesthetic agents to use. A few inhalation agents are known to irritate the airways of patients with severe asthma and induce bronchospasm due to the pungent smell on induction, primarily with desflurane and isoflurane. Other agents like sevoflurane can be used in asthmatic patients to help relax the airways on induction as they do not have such pungent smells.

If general anaesthesia is required, a laryngeal mask airway is safer than endotracheal intubation.

Post-partum

Hypoglycaemia is a general issue with all preterm babies, but also may occur when high doses of beta-agonists have been given within the last 48 hours prior to delivery. In addition to asthma treatment, beta-agonists are also used for suppression of pre-term labour. If high doses of short acting beta-agonists have been given during labour and birth, blood glucose levels should be monitored in the baby (especially if preterm) for the first 24 hours.

Women with asthma are at increased risk of postpartum haemorrhage due to uterine atony.

Postnatal

Continue using effective asthma management, including medication, while also addressing breastfeeding and potential exacerbations. Mothers should continue their prescribed asthma medications as normal during breastfeeding, ensuring compliance with manufacturer's recommendations. Following delivery, monitoring for potential postpartum asthma exacerbations is crucial, and appropriate treatment should be initiated as needed

Asthma guidelines are clear that the benefits of steroid treatments outweigh the risks.

• A short course of steroid tablets (usually prednisolone) is safe in pregnancy. Oral steroids are an important, life-saving treatment and can help you get back in control of your asthma after an asthma attack.

Azithromycin should be avoided when BF Regional rather than general anaesthesia is preferred due to the decreased risk of chest infection and post-op atelectasis Recommend having active management for 3rd stage

Have an asthma review soon after your baby is born so you and your GP or nurse can check how your asthma is now and update your asthma action plan.

Not continuing corticosteroids inhalers might be higher in the first six months postpartum, leading to worsened asthma related incidents.