Week 10: Cystic Fibrosis

what are the impairments of cystic fibrosis?

• airway obstruction

• muscle weakness

• fatigue

what are the activity limitations of cystic fibrosis?

• less tolerance for running

• harder to climb stairs

• more rest during play

what are the participation restrictions of cystic fibrosis?

• difficulty keeping up in PE

• reduced access at school

• less time with friends

what is cystic fibrosis?

• an inherited condition with autosomal recessive inheritance

• changes in the CFTR protein affect salt and water movement

• thick, sticky mucus can build up in the lungs and digestive system

  • symptoms and functional abilities vary between children

how does CF affect breathing and activity?

thick mucus is difficult to clear leading to airway obstruction and reduce airflow, leading to infection and inflammation and airway damage over time. the functional impact is increased work of breathing, coughing, and fatigue can limit play and exercise

why is nutrition important with patients with CF?

• poor nutrient absorption

• poor growth or weight loss

• feeding and treatment routines are important

what should the PT know about CF diagnosis?

• newborn screening can ID infants who need further testing

• sweat chloride testing helps confirm a CF diagnosis

• genetic testing can help ID CFTR variants

• PT team review the dx and medical hx with the CF team

which members are part of the CF care team?

• medical provider/nurse

• respiratory therapist

• dietitian

• child and family

what is medical management like for CF?

• inhaled therapies to manage mucus and infection

• CFTR modulators to improve CFTR protein function

• nutrition support to support growth and energy

which tests are used on patient who have CF?

• spirometry/FEV1 to look at airflow and obstruction

• exercise tolerance test to look at response to physical activity

• daily function to assess impact on play and school

what is important to assess about posture and breathing for patients with CF?

observe trunk alignment and accessory muscle use, consider how posture affects breathing and movement

what are some possible test that can be used for exercise testing?

• walking test (6 minute walk test)

• cycle test

• treadmill test

need to monitor symptoms, physiologic response, and recovery with each option

what are some common options for airway clearance?

• positioning/chest PT to mobilize secretions

• PEP/oscillating PEP

• chest wall oscillation

• huffing/coughing to move and clear mucus

what is huffing?

a huff uses an open mouth and an open throat to move air out- the CF team teaches the child how to coordinate huffing and coughing to clear mucus from the airway