Clinical Care
Johns Hopkins provides a environment of patient and family-centered CF care.
Care at Hopkins
Johns Hopkins Hospital
We strive to provide patient and family-centered care to everyone admitted to the Johns Hopkins Hospital. Adults needing admission to the hospital can be placed on one of several units within the hospital. The units are staffed with expert nurses that are familiar with the care of individuals with cystic fibrosis. People with CF cared for in the Pediatric CF Clinic are admitted to the Bloomberg Children’s Center for inpatient care. View more information about Johns Hopkins Hospital.
Adult Hospital Admission
Adult CF Team
What to Expect:
Admission Process
You will go to the main admitting office to check in, give insurance information, sign paperwork and wait for your hospital room. The main admitting office is the area right off of the main hospital entrance on Orleans Street (1800 Orleans St). If there is a wait for your room, you can receive a pass to get food from the cafeteria. Occasionally, there may be multiple people with CF admitted on the same day. Therefore, it is important that you wear a mask in the Admission Office. Please wear a mask and keep a distance of 6 feet or more away from other patients. If you use supplemental oxygen, the admitting office usually has tanks (E size) behind the reception desk. You will likely be admitted to Nelson 4, which is on the 4 floor of the Nelson building in the historic hospital. This section of the hospital was completely renovated tin October, 2014. Once you arrive on the hospital ward, you will meet the nurse and doctors that will be taking care of you.
Airway Clearance (Chest Physical Therapy)
There is a team of physical therapists dedicated to performing airway clearance and exercise for all inpatients with CF. You should expect begin airway clearance on the day of admission, or the next morning. The therapist will see you in your room 3 times a day (usually around 8:30 am, 11:30 am and 3:30 pm) and take you to the gym for one of the sessions. Please plan the timing of your meals and any travel outside of your room with these times in mind. Sometimes a fourth visit is prescribed for an evening treatment will be performed by a respiratory therapist (RT).
Blood Sugar Monitoring
Some patients have their blood sugars measured while in the hospital. We will give you instructions for taking your blood sugar before and after meals as needed. There is also a log sheet to record your blood sugars. Please notify your nurse if you are carbohydrate counting and need to take your insulin.
Hospital policy requires any blood glucose that is treated with insulin to be done using hospital glucometers. If you bring in your own glucometer; you can use it to test your glucose after meals. If you have an insulin pump in place, you will be asked to complete some forms that pertain to the hospital policy on insulin pumps.
Meals
The At Your Request Room Service Dining program allows you to order what you want, when you want it. Please order your meal by dialing 2-FOOD (2-3663) anytime between 7am and 6:30pm. Please allow up to sixty minutes for delivery. Snacks are delivered straight to the floor’s pantry – please ask your nurse for your snack bag when you are ready for it. Some people like the hospital food, but others do not. We don’t want you to lose weight while in the hospital, so you may want to consider bringing somethings you like to eat, encourage family members to bring you outside food, or consider going to the hospital cafeteria.
Pancreatic Enzymes
You should continue on your usual pancreatic enzyme brand and dose during hospitalization. It is very important that your pancreatic enzymes are your usual brand. You will be allowed to keep your enzymes at your bedside to allow for more flexibility with meal and snack times.
Parking
Patients and their visitors may buy a book of coupons for discounted parking to use at any Johns Hopkins garage. Each coupon is good for one period in the garage (up to 24 hours) – you cannot exit and reenter without paying or using another pass. You can buy the discounted parking passes in main admitting (Zayed Tower) and the former admitting office (Nelson Building, 1st floor) during the week (Monday-Friday from 7:30am to 5pm) and the Outpatient Cashier’s Office (1st floor of the Outpatient Center) Monday-Friday from 6:30am to 3:30 pm. The cost is $30 for a book of 5 and $60 for a book of 10 parking coupons. Click here for more information or call 410-955-5333.
Phone/TV/WiFi
The phone in your room works for local calls (dial 9 first, then the number) and calls inside the hospital (dial the last 5 digits of the phone number). To make long distance phone calls, you will need a calling card. Cell phones can be used on Nelson 4, but not in certain other areas of the hospital.
The charge for TV service, which provides local channels and limited cable channels, is $10.00 per day (maximum cost is $100). You can bill the service to your home phone for an additional charge ($3 or $4), or you can use a credit card at no additional charge. The TV service should be activated immediately upon your providing payment information. Free wireless Internet service (JHGuestnet) is available throughout the hospital and in all patient rooms.
Visitors
Visiting hours are unlimited on most hospital floors at Johns Hopkins. During the winter virus season, limitations on visitors may be in effect.
Who Will You Meet?
The team directing your care in the hospital will be the Medicine Pulmonary Service. The Adult CF Team works in conjunction with this team of physicians. You will meet many new faces during your inpatient stay. Many of them will ask you questions about your health which you may find repetitive at times. Please be patient as everyone is trying to take care of you in a safe manner.
Physicians
A medical resident will examine you daily, arrange for any required tests, and be available to handle any emergencies. You will have a primary medicine resident who you met on admission; there are three other residents on the team to help care for you when your primary resident is not in the hospital. A pulmonary fellow and attending faculty member are also a part of this team. The pulmonary fellow is a doctor receiving specialized training in pulmonary medicine, and the attending doctor is a colleague of Drs. Boyle, Dezube, Jennings Lechtzin, Merlo and West who is responsible for all pulmonary patients in the hospital that month. Occasionally, the attending pulmonologist is one of our CF doctors, but often it is not. The attending pulmonologist is the name you will see on your hospital bills.
CF Team
You can expect to see one of the CF attendings the first day or two after admission and one of the CF nurses most of the other days. If your attending pulmonologist if one of our CF doctors, then you will see that person daily. If the attending pulmonologist is one of our colleagues, a CF doctor will check in with you at least once a week. Please know that while one of our colleagues is primarily responsible for you and seeing you on a daily basis, a CF doctor and a CF nurse will be looking at your chart in the computer daily to review results and medication orders.
Bedside Nurses
If you need to go off the unit, please check with your bedside nurse to ensure that medications or airway clearance (chest PT) is not due. The nurse case manager will help coordinate your needs with the pulmonary team during your stay and home care coordinator will help with any needs that you will have when you go home.
Physical Therapist
There is a team of physical therapists dedicated to performing airway clearance and exercise on all hospitalized patients with CF.
Nutritionist
You will see a nutritionist during your stay that will monitor your weight, nutrition, and vitamin needs while in the hospital. He or she will also help to make sure you are receiving the correct diet and any needed snack preferences.
Social Worker
Megan Lagergren is the outpatient CF social worker. She can be reached at 410-502-7039. The social worker can help address personal issues with you and help to deal with insurance related issues.
IV Access
If you require intravenous (IV) antibiotics, you will need an IV that will last for several weeks. Examples include an implanted Port-a-cath® or peripherally inserted central catheter (PICC) or Hohn® or Hickman® catheter. A PICC is usually placed at your bedside by a specially trained nurse. A PICC is similar to a standard IV but is longer and ends in a bigger vein in your chest. When you are done with your antibiotic therapy the PICC can easily be removed by your home care nurse or in CF clinic. If the IV team is unable to place a PICC, you will receive a central catheter (Hohn® or Hickman®) which is inserted near the upper chest or neck. This line is placed by an interventional radiologist, and will need to be removed by in the Interventional Radiology Department. If you need this type of a line, you will not be able to eat on the morning of the procedure.
When will You get to go home?
The discharge process begins upon admission. Patients receiving intravenous antibiotics can typically leave the hospital and complete their antibiotics at home. Discharge is considered once a patient shows an improvement in symptoms, for example less coughing or less need for supplemental oxygen, antibiotic levels are appropriate and home care has been arranged. You can expect to meet with a home care coordinator who will assist in arranging home IV antibiotics if necessary. You can expect a phone call from your CF nurses the next business day after discharge.
Children's Center Admission
What to Expect
Patients and parents should expect to meet many people during the hospitalization. While CF physicians you know may be supervising your child’s care, they will not be responsible for the day-to-day management of your child’s hospitalization. Physicians will round each morning to discuss the previous day’s events and to plan care for the coming day. This is the time when medication changes are typically made and tests are ordered. Physicians will most likely see your child again during the day, but morning rounds are typically when major decisions are made.
Admission to the hospital can be stressful for both parents and patients. Younger children may act out or even regress, a normal reaction for children. Finding ways to help cope with the hospitalization is the job of the Child Life staff. Social workers and other professionals are also available to help you and your child through this experience. Parents and guardians may visit 24 hours a day, and one parent can sleep in the room with a hospitalized child. Siblings and grandparents may also visit from 9 a.m to 9 p.m. More information about your child’s hospital stay can be found at the Johns Hopkins Children’s Center website.
Who will You Meet?
There are a number of health care professionals, principally a pulmonary team, who will care for your child during the hospitalization.
Attending Physician
This is a pediatric pulmonologist who is a faculty member of the Johns Hopkins School of Medicine. There are 9 physicians who care for hospitalized children with CF at the Johns Hopkins Children’s Center. In addition to Drs. Peter Mogayzel, Shruti Paranjape and Lori Vanscoy who care for patients in CF clinic, Drs. J. Michael Collaco, Mandeep Jassal, M. Cecilia Melendres, Jessica Rice, S. Christy Sadreameli and Laura Sterni, care for hospitalized children. These physicians will supervise the care of your child throughout his or her stay.
Pediatric Pulmonary Fellow
The fellow is a physician who has completed a residency in pediatrics and is training to become a pediatric pulmonologist. He or she will manage the day-to-day care of your child in conjunction with the attending physician.
Senior Assistant Resident
The senior resident is in his or her third year of pediatric training. There are 2 residents assigned to the pediatric pulmonary team at any time, one working during the day and the other at night. The senior resident supervises the intern on the team.
Intern
The intern has completed medical school and is in his or her first year of pediatric residency. The intern is likely the physician you will see the most. He or she is responsible for writing orders and taking care of any medication changes that are required.
Case Manager
Theresa Maggitti, R.N., and Anne Goldsbourgh, R.N. are the case managers for children with CF admitted to the Children’s Center. The case manager is responsible for coordinating your child’s transition from hospital to home. She facilitates nursing services, home antibiotics, durable medical equipment and other supplies required after discharge, as well as follow-up for your child in the CF clinic.
Behavioral Psychologist
The behavioral psychologist can provide help in dealing with the stress of hospitalization and dealing with a chronic illness.
Child Life Specialist
The child life specialist is available to help children cope with the stress of hospitalization as well as provide schooling, if needed.
Physical Therapist
The physical therapist (PT) provides airway clearance for patients, typically 3 times a day. You should plan to bring in your child’s Vest® so that it can be used for airway clearance during the hospitalization.
Respiratory Therapist
The respiratory therapist (RT) administers the inhaled medications. He or she also performs airway clearance.
Hopkins CF Pediatric Team
What to Bring
Patients should bring to the hospital things that will lessen stress, like special toys and pillows. The following list of thing to bring to the hospital was compiled from recommendations of the Johns Hopkins Family Advisory Board. It is not all-inclusive but is meant to help you think about what to bring for your child’s inpatient stay.
- Insurance cards, medication lists, important legal documents (e.g., custody, etc.)
- Comfy clothes for child and parents (sweats, zip up hoodies)
- Socks, slippers, slipper socks
- Pajamas/robe
- Exercise/gym clothes and shoes for physical therapy
- Toiletries
- Airway clearance devices (Acapella®, Vest®, inCourage™ system)
- Books, crosswords, puzzle books, etc.
- iPods, mp3 players, and chargers
- Cell phone and charger
- Laptop computer with Skype™ access, and charger
- Favorite pillow and blanket, pillow and blanket for parent(s)
- Arts and crafts supplies
- Favorite stuffed animal
- Card and board games
- School work
- DVDs
- Blackout material to darken room
- Earplugs and eye mask
- Empty suitcase/duffle bag
- Cooler that can be filled with ice for perishables (request ice from RN on floor)
- Extra snacks, microwave meals
- Handheld game systems
IV Access
Most children are admitted to the hospital to receive intravenous antibiotics or fluids, which can be administered in several ways. Typically, a peripheral intravenous (IV) catheter is placed in the child’s hand or arm upon admission. However, IVs often malfunction after a few hours or days, so children requiring longer courses of antibiotics typically have a peripherally inserted central catheter (PICC) placed by specialized nurses. This type of IV can stay in place for several weeks and can be used at home to administer intravenous antibiotics. For most children, a PICC can be placed at the bedside.
Placing the PICC does not hurt more than a peripheral IV, but it does require children to keep their arm still for 10 or 15 minutes. Some children are given a medicine to help them relax. But in some cases, because of the child’s anatomy or because he or she cannot stay still long enough, the PICC is placed in the operating room under sedation provided by a pediatric anesthesiologist.
Children who require intravenous antibiotics on a regular basis will typically have an IV placed permanently under the skin by a surgeon. This type of IV, called a Port-A-Cath®, can be accessed at any time so that patients no longer require peripheral IV or PICC placement. Typically, for patients to receive intravenous antibiotics at home, they must have a centrally placed catheter such as a PICC or a Port-A-Cath® in place prior to discharge.
When WIll You Get to Go Home?
There are several criteria used to determine if patients are ready for discharge from the hospital. Children typically must have improvement in symptoms, for example less coughing or less need for supplemental oxygen, and improved lung function testing. Patients receiving intravenous antibiotics can typically leave the hospital and complete their antibiotics at home. Discharge home depends on having a functional PICC or Port-A-Cath® in place, antibiotics that are at an appropriate dose, and availability of a homecare company to provide medications at home. Typically, patients can be discharged home after 5 to 7 days of hospitalization. They will then complete a 2 to 3 week course of antibiotics at home.
Infection Control
It is now well established that the bacteria can be transmitted between people with CF. Therefore, the CF Foundation has established Infection Control Guidelines to prevent the transmission of potentially harmful bacteria between people with CF. The Johns Hopkins Hospital has created policies based on the CF guidelines that apply to adult and pediatric patients in both outpatient and inpatient settings.
There are a few basic practices that underlie the Johns Hopkins CF Center Infection Control Guidelines. First, frequent hand washing with soap and water or alcohol based cleaners, such as Purell®, can prevent the spread of germs. Second, the best way to minimize this spread of bacteria between individuals with CF is to prevent prolonged direct contact. Finally, proper cleaning of equipment and environmental surfaces will minimize exposure of people with CF to potentially dangerous bacteria.
To prevent the spread of bacteria between people with CF care providers use specific procedures, called “Contact Precautions.” These precautions require that health care providers wear gowns and gloves when in close contact with patients. We ask that all individuals with CF wear a mask when they enter the Johns Hopkins Hospital.
Infection control
In The Clinic
Because outpatient CF clinics bring together a large number of individuals with CF at the same time, implementing the practice of separating those with CF can be rather difficult. Optimally, patients should keep a distance of 6 feet from other individuals with CF . We ask that all individuals with CF wear a mask when they enter the Johns Hopkins Hospital. There are alcohol-based cleaner (Purell®) dispensers available throughout the clinic areas that should be used when entering or exiting a new clinic area.
What do we do to minimize the spread of bacteria in clinic?
- We make every attempt to move patients into rooms as quickly as possible.
- Purell® dispensers have been placed throughout the clinic area.
- Healthcare providers wash their hands when entering and leaving examination rooms.
- Health care providers wear gowns and gloves when examining patients.
What should you do to minimize the spread to bacteria?
- Wash your hands when leaving and entering a new clinic area.
- Try to stay at least 6 feet away from other patients in clinic.
- Children and adults with CF should wear a mask in clinic.
- Make sure your children avoid playing with the toys in the waiting room. Bring toys and books.
- Do not use the computers in the exam rooms.
In the Hospital
What do we do to minimize the spread of bacteria in the hospital?
Health care providers wash their hands when entering and leaving examination rooms.
- Patients with CF are cared for by different nurses.
- Patients with CF are not allowed to be in the room of another patient with CF.
- Patients with CF are not allowed to be in communal areas, such as playrooms or the gym, at the same time.
- Patients with CF are cared for using Contact Precautions.
- Health care providers wear gowns and gloves when examining patients.
- Patients are hospitalized in a single room.
- Patients colonized with Burkholderia cepacia are hospitalized on a different floor from other patients with CF.
What should I do in the hospital to minimize the spread of bacteria?
- Wash your hands when leaving and entering your or your child’s room.
- Make sure you or your child stays at least 6 feet away from other patients.
- Children and adults with CF should wear a mask when leaving their room.
Home IV Antibiotic Therapy
Individuals with cystic fibrosis (CF) who experience severe pulmonary exacerbations or who have failed to respond to oral antibiotics, may be candidates for home intravenous (IV) antibiotic therapy. Antibiotics are usually given at home via a peripherally inserted central catheter (PICC) or an in-dwelling catheter such as a Port-a-cath®. Many people are admitted to the hospital to begin their antibiotic therapy, which is completed at home.
A home nurse will make an initial visit to demonstrate how to administer the antibiotics, and then visit periodically during the course of therapy. The patient or patient’s family will be responsible for administering the antibiotics. Antibiotics are typically given 3 times per day. Depending on the antibiotic regimen, your physician may order blood tests to monitor antibiotic levels or potential toxicity during therapy.
Most exacerbations are treated with 2 weeks of antibiotic therapy. Then, patients typically return to clinic for evaluation including pulmonary function testing. If the response to therapy is not adequate, then a longer course of antibiotics may be prescribed.
Port-a-cath®
Bronchoscopy
Bronchoscopy
Bronchoscopy is a procedure that allows physicians to obtain samples of mucus from the lungs. Bronchoscopy is typically performed in patients whose airways are plugged with mucus or to obtain samples of mucus for culture. This procedure is especially beneficial for patients who have an unexplained decline in lung function who fail to respond to antibiotic therapy, or who are unable to cough up sputum.
Bronchoscopy is performed by adult or pediatric pulmonary faculty member often with the aid of a pulmonary fellow. At Johns Hopkins, pediatric bronchoscopy is performed in an operating room in the Bloomberg Children’s Center and sedation is performed by a pediatric anesthesiologist. While the child is asleep, the bronchoscope, a thin tube with a light and camera, is inserted into the lungs. This procedure allows the physician to determine the amount of inflammation and mucus present in the lungs and obtain mucus samples for culture. The bronchoscopy procedure takes about 15 minutes, but because sedation is required, people typically spend about 45 minutes in the operating or procedure room. Patients are then taken to the recovery room, where they remain until they have fully awakened.
Following bronchoscopy, patients typically have low-grade fevers that can be treated with acetaminophen or ibuprofen. People also cough more for 24 to 48 hours after a bronchoscopy. People with asthma may also wheeze following a bronchoscopy. Following the procedure, some people require extra oxygen, which may lead to a longer stay in the hospital.