Cystic fibrosis
Our pulmonologists work with the Cystic Fibrosis team to provide care for children, teens and adults.
Most people with cystic fibrosis can lead active lives—depending upon the severity of their disease—with routine therapies and regular visits to a Cystic Fibrosis Accredited Care Center such as ours. Our Cystic Fibrosis (CF) Clinic team members partner with people with CF to develop individual treatment plans. These plans typically include high-calorie, high-fat diets, and therapies to loosen the clogged mucus from their airways, and mucus-thinning drugs and antibiotics when needed.
Cook Children’s Cystic Fibrosis center is nationally accredited by the Cystic Fibrosis Foundation.
Treatment
By following a treatment plan developed with their CF care center team, many people with CF can slow down the progression of their disease. A healthier body is better able to deal with bacteria and chronic lung infection. We also provide age-specific education for patients and their families.
Cook Children's Cystic Fibrosis Clinic
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
- Physical Therapist who will help develop an exercise plan to maintain your child’s optimal lung function
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 will include the following tests:
- Lab work- CBC, electrolytes, kidney function, liver enzymes, blood sugar; throat or sputum culture
- Chest X-ray, IgE level and 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 3 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
How to make an appointment
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 care
After hours and on weekends/holidays, call 682-885-4000 and ask the operator to page the pulmonologist on call.
Newborn screening
Common CF tests
Most people are diagnosed with CF at birth through newborn screening, or before the age of 2. A doctor who sees the symptoms of CF will order a sweat test or a genetic test to confirm the diagnosis.
A sweat test is the most common test used to diagnose cystic fibrosis and is considered the diagnostic "gold standard." A small electrode is placed on the skin (usually on the arm) to stimulate the sweat glands. Sweat is then collected and the amount of chloride, a component of salt in the sweat, is measured. A high level of chloride means that the person has cystic fibrosis.
For children who are less than six months old:
- Chloride levels at or above 60 mmol/L (a measure of concentration) means the child has CF.
- Chloride levels between 30 and 59 mmol/L are considered borderline and need to be examined on a case-by-case basis.
- Chloride levels below 30 mmol/L are considered negative for CF.
For people over the age of six months:
- Chloride levels at or above 60 mmol/L means the person has CF.
- Chloride levels between 40 and 59 mmol/L are borderline.
- Chloride levels below 40 mmol/L are considered negative for CF.
Chest X-ray
X-rays use electromagnetic waves that create images of the body. They are usually done while the patient is standing, but can be performed sitting or lying down. The patient will need to hold his or her breath for a short time while the image is being created. Usually both side and front view images are taken. X-rays are painless.
Complete blood count
This is the most common blood test, and measures the three major types of cells in blood: red blood cells, white blood cells, and platelets. The test can check for infections, and can tell if the patient has any side effects from medications.
A small sample is collected from a finger, a heel (in infants), or from a vein. A machine then processes the sample, counting cells, measuring hemoglobin (the part of red blood cells that carries oxygen), and sorting the white blood cells into five subtypes. Results are often available the next day.
Kidney function tests
Kidney function tests measure the health of the kidneys, which filter your blood of wastes. The tests check the amount of waste products passed in urine or left in the blood, and also check the blood's mineral levels. The tests can tell if the kidneys are not functioning as they should, or if medications used to treat CF are affecting the kidneys.
For a blood test, a sample is drawn from a vein—usually from the inside of the elbow or the back of the hand—with a thin needle. For a urine test, the patient urinates in a small jar to collect the sample. In both cases, the samples are sent to a lab for analysis.
Liver function tests
Liver function tests can tell if the liver has been injured by disease or other factors. The liver stores enzymes for digestion, and injury to the liver can release some of these enzymes into the blood. Blood tests measure the level of enzymes in the blood, and can indicate liver damage, or blockage in the ducts around the liver.
Blood is drawn from a vein using a thin needle. The sample is then sent to a laboratory for analysis. Results are often available the next day.
Lung (pulmonary) function tests (PFTs)
This series of breathing tests measures how well the lungs take in and exhale air. They are usually done in a doctor's office or another medical facility.
Because these tests are hard to do with young children, we wait until a patient is five years old to do PFTs.
Oxygen saturation test
Red blood cells carry oxygen to the organs of the body. When these red blood cells pass through the lungs, they emerge saturated with oxygen. In a patient with lung disease, or another lung condition, not all of these red blood cells leave the lungs carrying oxygen. An oxygen saturation test measures the percentage of red blood cells that carry oxygen. It can tell if a person's lungs aren't working as well as they should.
In some cases, a doctor may use a simple detector that fits on a finger to estimate oxygen saturation. To get a more accurate reading, a doctor will draw blood from an artery in the wrist—where the pulse is normally taken—and then test the blood for its oxygen level. The results of this test are usually available within a half-hour.
Sputum cultures
A sputum culture test analyzes a patient's phlegm or mucus for bacteria that cause lung infections. It can tell if a patient has an infection in his or her lungs.
The patient coughs deeply and spits any phlegm or mucus into a sterile cup. If a patient can't cough up mucus, a throat swab may be performed. The sample is then analyzed in the laboratory. The lab can also test to see which antibiotics will work best against the infection.
Cystic Fibrosis coordinator services
- Coordinates what tests need to be done in each visit (i.e. cultures, vitamin levels, dexascan, etc.).
- Coordinates who patients need to see in each visit (i.e. dietician, social worker).
- Assists with admissions and discharges of patients from the hospital as well as see patients daily while in the hospital.
- Coordinates new patient education.
- Helps with appeals, prior authorization, forms, etc.
- Monitors CF Centers quality improvement measures.
Contact your CF coordinators at 682-885-6299 and follow instructions (#6). Please call your nurse for urgent/sick patient assistance as our phone and phone messages are not picked up if we are not in the office.
Dietitian services in the Cystic Fibrosis Clinic
- Weight gain & growth
- Energy goals (calories, protein, fat, etc.)
- Weight gainers & oral supplements
- Calorie boosters & recipes
- Enzyme dosing & stool changes
- Vitamin & mineral supplements
- Herbal supplements
- Eating habits & patterns
- Picky eaters & mealtime behavior
- Tube feedings
- Bone health
- Cystic fibrosis related diabetes
- Sports nutrition
- Nutrition during pregnancy
- Appetite stimulants
Contact your dietician services team at 682-885-7496.
Respiratory services in the Cystic Fibrosis Clinic
- Performs pulmonary function tests and provides copy of PFT for patient binder.
- Assists the pulmonologist with bronchoscopies.
- Provides educational materials and teaching of the following:
- Aerosol therapy or ACT
- Chest physical therapy (CPT)
- Nebulizer sterilization methods
- Huff coughing methods
- Inhaler use
- Correct nebulizer choice for each inhaled medication
- Provides durable medical equipment & parts such as nebulizers, air compressors, vests, oxygen, spacers, flutters, etc.
Contact your respiratory services team at 682-885-4189
Social worker services for the Cystic Fibrosis Clinic
- Provides information and referrals to appropriate community services.
- Provides patients with information on available scholarships.
- Provides information related to Medicaid, CHIP, SSI, CSHCN (Children with Special Healthcare Needs) Medicare, and insurance.
- Assists with patient assistance programs for medication costs.
- Inter-agency collaboration (where we can get supplies/equipment if the family has no money).
- Emotional support, counseling, and problem solving (be available for the families if they need support or problem solving).
- Sees patients and families in the clinic and in the hospital.
- Provides Make A Wish information for families.
Contact your social worker services team at 682-885-3991.
Child Life services in the Cystic Fibrosis Clinic
- Developmentally appropriate education about illness, treatment and recovery using tools such as dolls, medical equipment, books, and photographs.
- Preparation and support before, during and after medical procedures and other stressful times; assistance with coping techniques (toys, books, guided imagery and relaxation techniques).
- Therapeutic play opportunities (medical play) to understand the child’s perspective of his/her diagnosis or hospital stay.
- Developmental assessments, through observation or formal tests, to determine a need for additional services.
- Parent/caregiver education on childhood growth and development and the effects of illness, injury and hospitalization.
- Visits to a child’s school to explain illness, injury, treatment and recovery to classmates, easing a child’s transition from the hospital to the classroom.
Contact your Child Life services team at 682-885-4892.
Cystic Fibrosis clinical research services
For all our CF patients:
- Provides information to patients and families regarding:
- What's involved when you participate in a research study
- Ongoing and upcoming CF research studies at Cook Children’s as well as other CF centers
- Referrals to other CF centers for studies that are not being conducted at Cook Children’s
For our CF patients interested in participating in research:
- Identifies which patients qualify for each research study
- Notifies patients when they qualify for a research study
- Provides information to patients and families about the research study they are eligible for, allowing them to make an informed decision about participation
For patients that participate in a research study:
- Coordinates all the activities and procedures that will take place throughout the research study
- Guides study participants through every aspect of the study and each study visit.
Contact your CF clinical research coordinator at 682-885-1244
