Visiting Our Cystic Fibrosis Center

What is an accredited CF Center, and why does it matter? It means that the center has been reviewed by the Cystic Fibrosis Foundation and has nationally-accepted standards. But what it really means is that your child has access to most advanced CF care available today.

Our center provides dedicated professionals who specialize in the treatment of Cystic Fibrosis and in the care of human beings. Your team may include but is not limited to your physician, nurse, respiratory therapist, dietician, and social worker–all in the same location. It's added convenience for patients and, because it allows increased communication between the staff members, detailed attention too.

Whether you're a new patient, an existing pediatric patient, or are seen in our adult program, you'll find one thing in common, exception care. The following information can help assure that your visits are as easy and smooth as possible.

These days, you'll find CF patients enjoying life: playing, getting married, working, having families, going off to college, celebrating the ups and downs of everyday lives; all thanks to the many advancements in care and treatment for children and adults living with CF. Many of them are patients at Cook Children's Medical Center. In fact we currently follow over 300 patients, approximately 200 in our pediatric program and around 100 in our adult program.

Going the distance
Carol Shepherd is in her 50s, and she was a patient of Dr. James C. Cunningham from 1987 until transferring to the adult program. Once told she would not be able to have children, she is the proud mom of twins–who are now in their 20s. Dr. Cunningham considers her a true role model for others, attributing her great attitude and her participation in research for helping her to stay healthy.

Over the past few decades, the outlook for cystic fibrosis (CF) patients has improved dramatically. Today, the median age range is late 30s to early 40s.

The Cook Children's CF Clinic sees patients from birth all the way through adulthood, typically transitioning our kids from our pediatric CF center to our adult CF center somewhere in their late teens.

Cook Children's pediatric center begins caring for a child from the moment they are diagnosed with CF until they are ready to transition to our adult center. Thanks to many advanced in technology, and newborn testing, we are able to diagnose and treat people with CF even before symptoms begin. Our family centered team approach allows us to observe patients, and provide state-of-the-art treatment when needed. We also assure that patients and families have access to education, training and support at each clinic visit to manage the disease and help maintain a healthy lifestyle.

Depending the type and severity of your child's CF, clinic visits will usually be scheduled every 2-3 months. Outpatient care may include some, all, or a combination of the following:

Regular physical exams
Pulmonary function tests (PFT)
Chest imaging, including x-rays and/or CT scans
Respiratory cultures
Annual lab screens
Review of diet
Nutritional therapy
Measure growth
Screen for and manage complications
Patient and family support

Inpatient care

When you have CF, medical issues may arise that require an inpatient stay. Our nationally recognized pulmonology team is at hand and ready to provide excellent care.

During your child's stay, your medical team may also include Child Life specialists, physical and occupational therapists, social workers, respiratory therapists, nutritionists and/or dietitians

Transition of care

Better treatments mean people with CF are living longer than ever before. That means more children are growing up and moving from a pediatric program to an adult program. From the time your child enters Cook Children's CF Clinic, we begin preparing them–and you–for that transition. We teach them about their disease and help them learn to take control at age and maturity appropriate stages of their growth. By the time they are teens we begin encourage them to manage their appointments as well. CF is a lifelong condition, at least until we find a cure. Until then, it important that they learn how to balance their CF and medical needs while developing healthy independence, relationships, jobs and life.

There are also many challenges faced by adults, including work, family, finances and, of course, health. Every aspect of your life is affected in some way by CF, and we provide ongoing care, including treatment, support and education for the various illnesses and risks that adults may deal with, such as:

Respiratory issues
Nutritional needs
Gastrointestinal disorders
Diabetes
Infection risk and control
Emotional challenges
Physical and occupation therapy
Medications
Research and clinical trials
Insurance and finances
Family planning
Daily living topics like home care, traveling, etc.

At Cook Children's CF Clinic, the pulmonologists head the care team, which includes the following members:

CF coordinators
Nurses to coordinate routine care and sick visits
Respiratory therapists to measure pulmonary function and teach airway clearance
Dietitian to monitor nutritional status
Social worker to assure the emotional health and well-being of your child and your family
Child Life specialist to develop appropriate education about illness and procedures
Research coordinator
Pharmacist

At your first visit and once a year after that, you'll meet with all of the care team mentioned above to develop a plan to optimize your child's health. This visit will be longer than a routine or sick visit since you will be meeting with the entire care team. It may include the following tests:

Lab work – CBC, electrolytes, kidney function, liver enzymes, blood sugar, IgE level
Throat or sputum culture
Chest X-ray, bone density
Oral glucose tolerance test starting at age 10
TB skin test

For those over age 4-5 (as developmentally appropriate) there is lung function testing (spirometry)and lung volumes; children not able to have the spirometry test done will still meet with Respiratory to review CPT (chest physical therapy) and nebulizer cleaning methods.

We recommend visits to our CF Clinic at least every 3 months and at the onset of illness. The following are ways to maximize your child's health between visits:

Follow the care plan your CF team has put together for your child
Administer your child's prescribed medications and vitamins
Do chest physical therapy and inhaled medications
Clean respiratory equipment as instructed
Eat a high-calorie, high-fat diet
Use effective hand washing either with soap or hand gel
Avoid contact with people who have a cold or contagious illness
Maintain a 6 feet distance from other CF patients
Get a flu shot each year
Keep your immunizations up to date
Do not be around smokers
Call us at the first sign of illness

At your first visit and once a year after that, you'll meet with all of the care team to develop a plan to optimize your child's health, or if you are a teen or adult, your own health. This visit will be longer than a routine or sick visit since you will be meeting with the entire care team.

On the day of your first visit:

Please arrive at least 30 minutes prior to your appointment to assure that you have time to fill out any additional paper work.
Check-In on the 1st floor of Dodson in Registration then with receptionist at the Pulmonary Clinic.
Make sure you bring your insurance information and a photo ID, such as a driver's license.
You will be given a patient binder to help you keep track of all appointments, medications, treatments, contact information, etc. You can also download our FREE Health Care Notebook here.

Your initial visit will include a complete medical history as well as any or all of the following:

Lab work: CBC, electrolytes, kidney function, liver enzymes, blood sugar; throat or sputum culture
Chest X-ray, IgE level and TB skin test

Your doctor may have you meet the research coordinator, if needed, to review studies you may enroll in to benefit those with CF.

Upon completion of your visit you will check-out and make your appointment for your next visit.

Routine follow-up appointments in the Cystic Fibrosis (CF) clinic may be scheduled every 3 months between annual visits. If your child is sick, has a cold that lingers, or develops a cough or other difficulties, we may advise treatment and/or schedule an "as needed" visit at the CF Center.

Other visits may be scheduled more frequently if your child is having other concerns such as weight gain, or has just recently been diagnosed with CF. In fact, if you are new to CF our center recommends more frequent visits at first, until you feel you have received the right amount of education, have met appropriate team members and feel comfortable with your child's care.

Every patient's visit will be based upon their individual needs, but the following are some things you may expect to happen when at a CF clinic appointment. Please be sure to bring a list of questions for you to ask at your clinic visit.

When you arrive

Please check-in on the first floor of the Dodson clinic building in Registration then with the receptionist at the Pulmonary Clinic. We recommend you arrive 30 minutes prior to your appointment. This ensures you have plenty of time to provide us with any updates in information, such as change of address, change of phone, or change of insurance.

What to bring

A valid ID
Your insurance card
Your patient binder
A list of any questions you may have for the medical team

What to expect

Medical assistants will take following measurements:

Weight
Height
Blood pressure
Temperature
Pulse oximetry

The nurse:

Review your medications
Update your health history
Take a throat culture (if needed)
Give you a flu shot (if needed)

Pulmonary functions will do the following:

A spirometry (if patient is old enough)
Review your respiratory treatments and medications
Review your order of therapy
Make recommendations for improving/maintaining respiratory status

Radiology will:

Perform a chest x-ray (if needed)

Your doctor will:

Perform a medical exam and review of systems
Provide a Pulmonary Exacerbation Score (if patient is old enough)
Write prescriptions for any new or continued treatment plan
Create a new or update an existing action plan

Your dietitian (if needed) will do the following:

Review your weight and BMI status
Review your eating practices and habits
Review your pancreatic enzymes and vitamins
Take a stool/GI history
Make recommendations for improving and maintaining nutritional status

Social worker (if needed) can offer

Assistance with insurance/financial concerns
Assistance with personal concerns that relate to CF

Child Life is available for

Distraction
Age appropriate education activities

Research (if needed) is available to

Review studies you may enroll in to benefit those with CF

CF Coordinator will help

Review age appropriate education/skills
Provide age appropriate education materials
Answer any questions

Check-out

Make your appointment for your next visit

While we recommend follow-up visits every three months to keep your child's health on track, there are also ways to maximize your child's health between visits:

Follow the care plan your CF team has put together for your child
Administer your child's prescribed medications and vitamins
Do chest physical therapy and inhaled medications
Clean respiratory equipment as instructed
Eat a high-calorie, high-fat diet
Use effective hand washing either with soap or hand gel
Avoid contact with people who have a cold or contagious illness
Maintain a 6 feet distance from other CF patients
Get a flu shot each year
Keep your immunizations up to date
Do not be around smokers
Call us at the first sign of illness

A typical yearly visit will average around 2-3 hours. At this visit, you will see your physician, your nurse, get weighed and measured, do pulmonary function tests, have a chest x-ray, do a sputum culture, have blood work done, get a T.B. skin test done, and consult with Nutrition, Social Work, and Child Life.

What to bring to your appointment:

Any referral form from your insurance company
Insurance cards
Outpatient Binder (you will receive at first visit)
Equipment you want to be checked by a Respiratory Therapist (VEST, Nebulizer, etc.)
Snack and drink for your child in case the appointment runs long

Who'll you meet with, and why:

A social worker – The social worker will work to answer any questions you may have as well as identifying needs and challenges you may face, such as insurance, resources, scholarships, etc.
A registered dietitian – The dietitian will assess your growth, body mass index, vitamin levels, and nutritional needs.
A Child Life specialist – Your Child Life specialist will provide developmentally appropriate education about illness and treatment; preparation and support before, during and after medical procedures; and parent/caregiver education on childhood growth and development and the effects of illness, injury and hospitalization.
CF Coordinator – CF coordinators will help review age appropriate education/skills, provide age appropriate education materials and answer any questions.
Respiratory therapist – Sometimes called an RT, the respiratory therapist will review your home respiratory routine and equipment. They will also check your PFTs (if old enough).

Tests you may have, and why:

PFT's – Pulmonary Function test. This is a test to see how well your lungs work. It tests the volume and flow rates your lungs are capable of producing. FEV1 (forced expiratory volume in 1 second) is commonly used to diagnose the progression of your lung disease. Yearly testing includes FVC (forced vital capacity) is the amount of air you can forcefully exhale. This test is done once without a bronchodilator (Albuterol or Xopenex) and one time with it. We do this to look at your lungs' response to medication. SVC (slow vital capacity) measures the amount of air you can slowly exhale. Lung Volumes measure the volume of gas in your lungs and can look for gas that is trapped in poorly communicating air space. We compare your current results to your historical results each time we do these tests.
A PPD skin test – this skin test determines if you've been exposed to someone with tuberculosis. Keep a close eye on your arm for 72 hours (3 days) from the time you get the test. You're watching for a very specific, small, hard lump under your skin – or "induration." If this occurs, call the clinic. If it is red or bruised, this is normal and is no need for concern.
Chest X-ray – We compare a chest X-ray this year to your past x-rays to compare them and determine changes in your lungs, if any.
Throat or sputum culture – We get a culture regularly on your sputum to try to maintain the most up to date records as possible of what bacteria or fungus might be colonized in your respiratory tract. With this information we get what are called "sensitivities," which tell us which antibiotics or antifungal medications will best treat you during an illness (exacerbation).
AFB sputum culture – an Acid Fast Bacillus test on your sputum is used to determine whether you have an active Mycobacterium Tuberculosis infection, or an infection due to another member of the mycobacterium family.
Pulmonary Panel, Lytes II, Comprehensive Metabolic Panel – Depending upon your laboratory, you will get one or more of these tests. They (collectively and apart) are generally looking at kidney function, liver function, hydration status, and early indicators of diabetes.
CBC – (Complete Blood Count) This test is another compilation tests which checks for anemia, infection, and general health.
Vitamin levels – A, E, and D – This is used to determine if you are taking in an adequate amount of fat soluble vitamins. These fat soluble vitamins are more likely to be low in patients who have CF due to malabsorption.
Serum IgE – IgE levels are drawn at least yearly after age 12 to determine whether or not there is the presence of an allergy.
Hemoglobin A1C if you have Cystic fibrosis related diabetes (CFRD) – This test evaluates the average amount of glucose in the blood over the last 2-3 months.
Urinalysis if you have CFRD – This is used as a tracking tool to see whether or not you are spilling glucose or protein in your urine.
Oral glucose tolerance test (starting at age 10) – Since CF related diabetes can cause you to lose lung function, we check for this every year.

Once your CF Care Team has decided a hospitalization is needed, your nurse will let you know when to check in at the Admissions desk. This is helpful in case you need to pack or plan for alternative care of siblings. Check in at the Admissions desk usually takes about 15-30 minutes. They wait for the room to be ready before registering your child. Paperwork is completed and a copy of your insurance card is needed. A representative then walks you and your child to the room. Shortly after you arrive in your room, your floor nurse will stop by to introduce her or himself to you. Information will be taken from you and the plan of care your physician has made will be gone over with you.

Since most hospitalizations are for treatment of lung infections that don't respond to outpatient treatment. IV access will be made in the treatment room or child's room if a peripheral line is needed. PICC lines are put in by the PICC team. Ports can be accessed by your nurse in the room.

Infection control is a high priority at Cook Children's. The nurse will go over procedures you and your child should do to prevent acquiring an infection while hospitalized. Staff should always use gowns and gloves when entering the room. Staff encourages you to remind them if they should forget. Additional isolation procedures will be explained to you.

A nursing aide will stop by your room periodically to weigh, take your child's blood pressure and pulse ox. They are a good person to ask for extra blankets, sheets, or toiletries needed for your child.

The Child Life Department helps children and families cope with the medical center experience. Child Life staff provides support and information geared to the child's level of understanding and provides a variety of opportunities for play and expression. Child Life staff members are usually in the playrooms, or you may ask your nurse where to find them.

Respiratory Therapy will stop by your room and go over your treatment schedule with you. They will provide a disinfected therapy vest compressor for your child to use exclusively in the room. They will measure your child for a correct vest size. A disposable vest will then be given to your child for the duration of their stay. Patients may also bring in their own equipment if they prefer.

Physical Therapy will stop by if prescribed by your physician. They can do stretching exercises or bring balls to throw around your room if you cannot leave the room. Or, you will go downstairs to the PT gym where there is a treadmill, exercise bike and other therapy equipment.

Nutrition associates will also stop by to see your menu preferences and for you to order snacks. Please ask them for if your child has a preference for a certain food as they can usually attain it for your child. They will instruct you on how to order meals and snacks.

Your child will be a white board in their room where everyone will write their names for your reference. It is also a good idea to write down your IV infusion times, Respiratory treatments and Physical Therapy time. You can also ask Child Life for assistance with a daily schedule. Always check with your nurse before you leave the floor as they will also know if you have a scheduled treatment coming up.

What to bring

Being in the hospital doesn't mean you can be as comfortable as possible. In order to make your stay a bit easier, we recommend you bring the following items:

Pajamas
Slippers
Socks
Sneakers for PT/Exercise
Jacket (if needed) to play outside
Comfortable shorts/t-shirts or sweats for the day
Extra snacks and Enzymes
Homework (on-site school will help with lessons)
Toiletries
Camera–you never know what celebrity might pop-in to medical center, what new friends you might meet or what neat activity you may be doing.
Extra jacket for visitors (mom, dad, siblings, friends) as CF kids tend to want the room very cold if on steroids.
Cell phone/charger
Foam ball to throw around the room. Frisbee for outside.
Markers/paper/scissors/tape for making and hanging creations in your room
Teen magazines have great teen pop posters for hanging in room
Flutter (not available in the hospital)
Home Health Care numbers in case you get out early and continue IV's at home
Outpatient binder
Any game devices/computers (lease label with your child's name)

To make an appointment at the Cook Children's Cystic Fibrosis Center, please call 682-885-6299 Monday through Friday between 8:30 a.m.-5 p.m.

After hours and on weekends/holidays:

Pediatric patients should call 682-885-4000 and ask the operator to page the pulmonologist on call.
Adult patients should call 817-336-5864 or 817-335-5288.

Why make an extra stop on the way home when you can fill your child's prescriptions right here? Located right on our medical center campus, our full-service pharmacy offers a convenient option to meet your pharmacy needs. We offer:

Full pharmacy services, including consultations with our pharmacists
Same-day prescription filling for most medications
Compounding
Delivery of prescriptions to outpatient Dodson clinic appoinments
Prescription delivery on discharge from inpatient stay
Online refills
Kid-friendly flavors for liquid medications
Text messaging alerts and mail service
Medication assistance program for those who qualify

When you rely on our retail pharmacy, you'll get more than medications, you'll get experts that above and beyond the expected. And that's our prescription for assuring you have a pharmacy you can trust. You can learn more about our Retail Pharmacy services here.

We're here to help.

If you have been told your child might have CF, we know you have lots of questions. We can help. To schedule an appointment, refer a patient or speak to our staff, please call our offices at 682-885-6299. After hours and on weekends/holidays, call 682-885-4000 and ask the operator to page the pulmonologist on call.

Visiting Our Cystic Fibrosis Center

Who we treat

Pediatric center

Inpatient care

Transition of care

Adult center

CF Clinic program overview

New patients

On the day of your first visit:

Routine follow-up clinic visits

When you arrive

What to bring

What to expect

In between clinic visits

What to expect at a yearly visit

What to bring to your appointment:

Who'll you meet with, and why:

Tests you may have, and why:

A cystic fibrosis hospital stay

What to bring

How to make an appointment

After hours care

Retail pharmacy

We're here to help.