EMS Particular Patient Populations - Cystic Fibrosis

EMS providers should be aware of the following key points when responding to patients with cystic fibrosis (CF):

1. Understanding Cystic Fibrosis:

   • Cystic Fibrosis is a genetic disorder that primarily affects the lungs and digestive system. It results in the production of thick, sticky mucus that can clog the airways and lead to respiratory infections and digestive problems.
   • CF is a lifelong condition that requires ongoing medical management and treatment to maintain respiratory function, prevent complications, and improve quality of life.

2. Respiratory Symptoms:

   • Patients with CF may present with respiratory symptoms such as coughing, wheezing, shortness of breath, and increased sputum production.
   • Be vigilant for signs of respiratory distress or exacerbation of CF-related lung disease, including increased work of breathing, decreased oxygen saturation, and changes in mental status.

3. Infection Control:

   • CF patients are at increased risk of respiratory infections, including bacterial, viral, and fungal pathogens.
   • Practice appropriate infection control measures, including hand hygiene and personal protective equipment (PPE), to prevent transmission of pathogens to and from the patient.

4. Nutritional Status:

   • Malnutrition and poor weight gain are common issues in patients with CF due to pancreatic insufficiency and impaired nutrient absorption.
   • Be alert for signs of malnutrition or dehydration, such as weight loss, poor appetite, or electrolyte imbalances, and provide supportive care as needed.

5. Medications & Treatments:

   • CF patients may be prescribed a variety of medications and treatments to manage their condition, including bronchodilators, mucolytics, antibiotics, and airway clearance techniques.
   • Be familiar with common CF medications and treatments and ensure that the patient has access to their medications during the emergency response.

6. Psychosocial Considerations:

   • Living with CF can have a significant impact on a patient's quality of life and emotional well-being. Be empathetic and supportive, and address any psychosocial concerns or stressors that the patient may be experiencing.
   • Communicate with the patient and their caregivers in a sensitive and respectful manner, and involve them in decisions about their care and treatment.

7. Transport Considerations:

   • Consider the patient's respiratory status and need for supplemental oxygen when determining the mode of transport and level of care required.
   • Ensure that the transport vehicle is equipped with appropriate oxygen delivery devices and monitoring equipment to support the patient's respiratory needs during transport.

8. Collaboration with Healthcare Providers:

   • Communicate effectively with the patient's primary care provider or CF care team to obtain relevant medical history and treatment information.
   • Provide a detailed report to the receiving healthcare facility to facilitate continuity of care and ensure that the patient's ongoing medical needs are addressed.

By being knowledgeable about the unique challenges and needs of patients with cystic fibrosis, EMS providers can deliver timely and effective care that supports optimal outcomes and enhances the patient's overall well-being.

Further Reading:

Alexander, M. & Belle, R. (2017) Advanced EMT: A Clinical Reasoning Approach (2nd Ed). Hoboken, New Jersey: Pearson Education

Bledsoe, B. E., Cherry, R. A. & Porter, R. S (2023) Paramedic Care: Principles and Practice (6th Ed) Boston, Massachusetts: Pearson

Mistovich, J. J. & Karren, K. J. (2014) Prehospital Emergency Care (11th Ed). Hoboken, New Jersey: Pearson Education

Peate, I. & Sawyer, S (2024) Fundamentals of Applied Pathophysiology for Paramedics. Hoboken, New Jersey:  Wiley Blackwell