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Typical Patient Journey for Cystic Fibrosis

What is Cystic Fibrosis (CF)?

Cystic Fibrosis is a genetic disorder that affects the respiratory, digestive and reproductive systems. A defective gene causes the body to produce abnormally thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections.  These thick secretions also obstruct the pancreas, preventing digestive enzymes from reaching the intestines that help break down and absorb food.  There are approximately 30,000 people in the United States with CF and more around the world.  As of 2003, the median age of survival for people with CF in the U.S. is in the early to mid 30s.  While there is currently no cure, the number of people with CF living active adult lives continues to grow.

Dartmouth-Hitchcock's Commitment to CF Care

Dartmouth-Hitchcock (D-H) provides care to people with CF at both its Lebanon and Manchester sites.  To accommodate and coordinate CF care at these sites, D-H has created the New Hampshire Cystic Fibrosis Center.  The New Hampshire Cystic Fibrosis Center's mission is to provide compassionate, responsive, collaborative care for people and their families affected by cystic fibrosis.  We promise to create an environment that promotes long term education and empowerment of the individual.  We will continuously improve our quality of care through an evidence based approach including research and consistent application of best practice.

CF Care at the New Hampshire Cystic Fibrosis Center

The New Hampshire CF Center cares for both pediatric and adult CF patients.  CF clinics are held at the D-H Lebanon and Manchester sites and facilitated by pediatric and adult CF care teams.  The CF care teams consist of a pulmonologist, a nurse continuing care manager, a social worker, a nutritionist, a respiratory therapist or physical therapist, and a secretary.  A psychologist is also available for patients with psychosocial issues or to assist teens with making the transition to the adult CF care team. Other specialties such as gastroenterology, endocrinology, radiology, and surgery are called upon by the care team as needed for consultation.  In addition to regularly scheduled clinics, patients are accommodated in other pulmonary clinics when they need to be seen earlier than the next scheduled CF clinic or for acute care.  

The CF Clinic exists to meet the needs of people with cystic fibrosis.  Patients are connected with the CF care team at D-H either at the time of diagnosis or shortly after.  Patients typically meet either the CF nurse continuing care manager or a CF physician to cover issues around the diagnosis and prognosis and also discuss the structure of CF care (e.g., the clinic appointment schedule, multi-disciplinary treatment by members of the team, and their own role in care).  The clinician also sets up the first clinic appointment for the patient and family. Newly diagnosed patients are usually seen monthly until they are stable, thereafter the interval between visits with the CF team depends on the patient's health status.  Stable patients are seen quarterly as recommended by Cystic Fibrosis Foundation Care Guidelines. At initial patient visits, the entire CF care team sees the patient.  

We encourage every patient to take good care of their body no matter what their condition.  Self management of this condition includes the following: being aware of infection control; following prescribed therapies as your doctor advises; eating as your doctor advises; exercising as your doctor advises; avoiding tobacco smoke; getting lots of rest;  scheduling and keeping regular appointments with the CF center.

A Typical Visit with the CF Care Team

Patients are greeted by a receptionist.  Once you are checked in, a nurse calls you from the waiting room, escorts you to an exam room and takes vital signs, measures weight, height and lung function, and charts a list of current treatments, medications, and allergies. 

Pulmonologist

The pulmonologist  performs a physical exam of the patient, checking specifically lungs, growth, and GI issues.  We review medications and therapies and request lab tests or consults with other specialists (GI, endocrine, radiology, surgery) should the need arise.  Patients typically have labs performed annually to check vitamin levels, glucose screening, and liver function.  Patients may also have annual chest x-rays.  These tests and x-rays are done at an initial visit to get a baseline for comparison at future visits.  The pulmonolgist also collects a sputum or throat culture from the patient and sends it to a lab to monitor for potential lung infection. 

Nutritionist

The nutritionist reviews daily caloric intake and timing and pancreatic enzyme dosage as appropriate (not all CF patients are pancreatic insufficient).  We also review vitamin dosages (CF patients require higher doses of fat soluble vitamins A, D, E, and K), and feeding supplements (high calorie shakes and oils).  If feeding issues are identified during the visit, the nutritionist may recommend consultation with a psychologist. 

Social Worker

The social worker reviews daily life activities with the patient and family.  We discuss financial, insurance, and support system issues.  The social worker reviews the home environment (exposure to environmental smoke and second hand smoke).  We assess the family's ability to cope with the disease with regard to siblings, grandparents, school, daycare arrangements, etc.  The social worker may make recommendations for further counseling or support and assist patients in making contacts within the community.

Physical Therapist or Respiratory Therapist

The physical therapist (PT) or respiratory therapist (RT) reviews airway clearance techniques with the patient and family.  Chest physical therapy is the most commonly prescribed airway clearance technique and it is recommended twice daily for 20 minutes each time.  Chest PT involves firmly striking on five positions (lobes of the lung) on each side of the chest while the patient is either bent over or held in a down ward position on a parent's lap.  Depending on the patient's age, other airway clearance therapies are prescribed, such as the Vest (a device that is similar to a life vest which connects to a compressor and shakes the lungs), Accapella, PEP, or Flutter Valve (these devices are similar to wind instruments and create a pressure similar to blowing up a balloon).  The PT/RT also discusses and sometimes asks the patient and/or family to describe how they use, clean, and disinfect their nebulizers.  The PT/RT encourages physical exercise, especially activity which produces a clearing cough, such as running.

Customization of CF Care

The number of visits and particular issues varies by age in CF care, but in general, the main tasks remain the same.  These tasks include weight checks, enzyme dosing, nutrition planning, family education, airway clearance, and psychosocial issues.  As with most chronic diseases, patient self-care is central to successful management of CF.  It is not aggressive care that produces the best results but persistent daily attention to lung function and nutrition and constant vigilance.  It has been estimated that good routine CF care takes as long as 2 hours a day.

The CFF recommends quarterly visits, meeting with each member of the CF care team at least annually.  While the general structure of the clinic visit has consistent components, these components must be customized to address the patient's life stages.
 
From birth to 1 year, children visit the CF clinic every 1 to 3 months, with the major goals of normalization of height, weight and body mass index (BMI).  From ages 2 to 4, the routine visits become quarterly.  At this point, the child is going through toddlerhood and behavioral and feeding issues dominate.  This is a complicated issue in CF care because it is difficult to measure caloric intake and correctly dose pancreatic enzymes in this age group. 
 
From age 5 to 10, the child continues quarterly visits. The big challenge is integration into school.  The CF team must work with the school nurses to make sure that necessary enzymes are dispensed at school, and help with care.  Meanwhile, the child must adjust to eating with their classmates and begin to cope with the realization that they are different.  This is the period where new therapies aimed at preserving lung function may be added that increase the treatment burden. 

From age 11 to 14, the transition is to adolescence.  Along with the difficult issues of independence, sexuality, and body image, the responsibility for care begins to shift from the parents to the child, with the child now doing their own airway clearance and managing food and enzymes.  As their understanding of cystic fibrosis deepens, the child may wonder how long they will live. 
 
From age 15 to 18, there is a transition to self-care and to the adult CF team.  The patient begins having the quarterly visits without the parents in the exam room and must take the primary role in their daily care.  In addition, if the patient is planning to go away to college or move away from the area, the search for a new CF team must take place.

As adults with CF the challenges of balancing a personal and professional life in the face of a chronic disease become the focus of many visits.  The adult CF care team works with patients to ensure a rich quality of life and maintain health status.

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