Preparing for a Cystic Fibrosis Hospital Stay

Arrival: You will get a phone call when your child's bed is ready. Try to get to the hospital within 3 hours if possible. The earlier in the day you arrive the easier it is to get your child's PICC line placed.

PICC Line: A PICC line is a soft, thin tube through which IV medications will be given to your child. Children may be given oral/nasal medication or general anesthesia to help them stay comfortable while the PICC line is placed. Anesthesia is limited to children under age 13 and requires no eating or drinking for 8 hours in advance.

Germ Protection: You will wear a gown and gloves in your child's room to protect your child and others from germs. Your child will wear a gown, gloves, and a mask if leaving the room for a walk.

Visitors: You are welcome to stay with your child during the day and overnight. Siblings under the age of 18 can't stay overnight, so try and make arrangements for them before you arrive. If the hospital is under "Flu Restrictions" siblings under the age of 18 can't visit at all. This may be frustrating, but it will keep your child safe.

Infection Control: Your child will shower and get new bed sheets daily to protect against infection. Nurses will wipe down all high-touch surfaces in the room twice a day. Dressing, tubing, and central line caps will also be changed according to policy.

What to Bring with You

• Your child's vest jacket for daily airway clearance
• Any medications that the hospital doesn't have (e.g. Orkambi, Kalydeco, Symdeko, ADHD Medicines, etc.)
• A pillow and blanket to make your child's room feel more like home
• Meals, snacks, and drinks to store in your room's mini fridge. Microwaves and toasters are also available
• Cozy slippers so you and your child don't have to walk barefoot
• Cell phone chargers
• Shampoo/soap for showers
• A blow up bed if you don't want to sleep on the couch
• Favorite games, books, and movies. Each room has a DVD player and a Wii System.

Communication Tips

 A hospital stay can be stressful, especially if it's your child's first time. Good communication can help you feel more comfortable and confident in your child's care. Here are a few tips:

• You may not always see your child's main pulmonologist at the hospital. Rest assured that the doctor who treats your child will work with your pulmonologist to agree on a treatment plan.
• Doctors and nurses will "round" in your child's room each morning to discuss the plan for the day. It's always okay to ask questions if you don't understand something or if the plan has changed.
• If you can't be at the hospital during the morning "round" ask for a phone update.
• Riley is a teaching hospital. You may see medical students and residents, along with the staff physician. Sometimes it may feel like you are answering the same questions several times. Try to be patient, as this is part of the process. The team is working together to deliver the best care possible for your child.
• Let the Respiratory Therapist know your preferred times for airway clearance. They may not always be able to keep your schedule, but they will try their best. Vest therapy is done four times a day. It may be done more often if your child needs it.
• Write down your child's medication and treatment schedule on the dry erase board in your room. Your child can check them off as they are done each day.
• Have your child draw a picture of his likes and dislikes on the dry erase board.
• All doctors and nurses will wash their hands when they enter your child's room. It's always okay to ask them to wash again.
• If you have concerns, contact Traci Dayhoff or the 8-West Charge Nurse. They are committed to giving your child a safe and positive experience.

Other Cystic Fibrosis Program News

Don't forget the Cystic Fibrosis Program's new number: 317.948.7208

Remember your child's mask: It will protect your child and others from germs in the PFT lab and waiting room.

Culture results are coming to the My IU Health portal: Stay tuned for more information on how to access them.

Clinical Update from the Cystic Fibrosis Center: The Cystic Fibrosis Center at Riley at IU Health is part of the Cystic Fibrosis Learning Network, a Cystic Fibrosis Foundation funded quality improvement collaborative. One of the distinguishing aspects of the Learning Network is its focus on patient and family input into Cystic Fibrosis care. As part of our effort to get more input ahead of patients’ visits, we are sending a pre-visit message through My IU Health. Please be on the lookout for these messages and let the Cystic Fibrosis team know of any issues you’d like to discuss at your visit or any team member you’d like to see. If you’re not registered on My IU Health, please ask about it or sign up at myiuhealth.org. You can do many things, such as prescription renewals or make appointments with select physicians through My IU Health. Many lab results are available on My IU Health, and in the near future we plan to make culture results available through the portal. 

New Division Chief: As many of you know, Dr. Stephanie Davis left Riley at IU Health in June to become the Chair of the Department of Pediatrics at the University of North Carolina. We have been very fortunate to replace her position very quickly. Dr. James Chmiel will be starting as Division Chief of Pediatric Pulmonology, Allergy, and Sleep Medicine in January 2019. Dr. Chmiel is coming from Rainbow Babies Hospital and Case Western Reserve University in Cleveland. He is an extremely well known and highly respected pediatric pulmonologist with a strong interest in Cystic Fibrosis. He has conducted extensive research in Cystic Fibrosis and has been in many leadership positions at the Cystic Fibrosis Foundation. In his role as Division Chief, he will be a strong advocate and supporter of the Cystic Fibrosis Center. He will be seeing a limited number of Cystic Fibrosis patients.

Research Studies

Savara:

•  In this study we want to examine the safety and efficacy of AeroVanc in the treatment of persistent Methicillin resistant Staphylococcus aureus (MRSA) lung infection in patient’s diagnoses with cystic fibrosis.
• Participants will be randomly assigned in a blinded fashion to receive either AeroVanc 30 mg twice daily (BID), or placebo BID (1:1 active to placebo) by inhalation for 24 weeks
• Age range: > 6 years olds

Saline Hypertonic in Preschoolers with Cystic Fibrosis and Lung Structure as Measured by Computed Tomography (CT). SHIP-CT Study:

• In this study we want to examine the efficacy of hypertonic saline in preschool children.
• Participants will be randomized 1:1 to receive 7% HS (treatment arm) vs. 0.9% IS (control arm) administered twice daily via jet nebulizer for 48 weeks.
• Age range: 3-6 years at screening visit

Teach:

• In this study we want to examine study of azithromycin in subjects with chronic PA airway infection using inhaled tobramycin
• Participants will be randomized in a 1:1 fashion to azithromycin (500 mg 3x per week) or matched placebo.
• Age range: > 12 years of age

Early Microbiome:

• The primary objective is to assess the effect of viral infections and antibiotics on the characteristics of the airway and gut microbiota in infants with CF.
• Participants will collect biological samples and complete surveys for information for the study. The participants may complete infant pulmonary function testing.
• Age range: infants enrolled if less than 16 weeks old

CHEC-SC:

• The primary objective is to assess the sweat chloride concentration in people who are currently taking CFTR modulators.
• Participants will complete a sweat chloride test in the lab. The participants need to be on treatment for at least three months. If the participant changes CFTR treatment they may be able to complete another sweat chloride after three months of being on the new treatment.
•  Age range: anyone on CFTR therapy greater than 1 year of age