Cystic Fibrosis Center

According to the Cystic Fibrosis Foundation, Cystic fibrosis is an inherited chronic disease that affects the lungs and digestive system of about 30,000 children and adults in the United States (70,000 worldwide). A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that:

• clogs the lungs and leads to life-threatening lung infections; and
• obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food.

In the 1950s, few children with cystic fibrosis lived to attend elementary school. Today, advances in research and medical treatments have further enhanced and extended life for children and adults with CF. Many people with the disease can now expect to live into their 30s, 40s and beyond.

People with CF can have a variety of symptoms, including:

• very salty-tasting skin;
• persistent coughing, at times with phlegm;
• frequent lung infections;
• wheezing or shortness of breath;
• poor growth/weight gain in spite of a good appetite; and
• frequent greasy, bulky stools or difficulty in bowel movements.

• About 1,000 new cases of cystic fibrosis are diagnosed each year.
• More than 70% of patients are diagnosed by age two.
• More than 45% of the CF patient population is age 18 or older.
• The predicted median age of survival for a person with CF is in the late 30s.

The two most common problems in CF, partly caused by abnormal mucus, involve the lungs (respiratory system) and the digestive system. The body makes mucus as a way to keep itself lubricated and clean (rather like motor oil in a car engine). In healthy people, the lungs and the digestive tract both make mucus. In people with CF, the body makes mucus, but the mucus is different; it is much thicker and stickier. Abnormal mucus causes several problems. The thick and sticky mucus in the lungs of people with CF is hard to cough up. When mucus stays in the lungs, it makes an ideal place for bacteria to grow. This is why most people with CF eventually have lung infections.

Most people with CF also have problems digesting food, and this is a big reason they may have problems with growing. The thick, sticky mucus blocks the ducts from the pancreas to the small intestine, so that the digestive enzymes from the pancreas cannot get to the small intestine to help digest food. This is called malabsorption or pancreatic insufficiency.

Download An Introduction to Cystic Fibrosis: For Patients and Their Families

Cystic fibrosis (CF) is a genetic disease. This means that CF is inherited. A child will be born with CF only if two CF genes are inherited - one from the mother and one from the father. A person who has only one CF gene is healthy and said to be a "carrier" of the disease. A carrier has an increased chance of having a child with CF. This type of inheritance is called "autosomal recessive." Autosomal means that the gene is on one of the first 22 pairs of chromosomes which do not determine gender, so that the disease equally affects males and females. "Recessive" means that two copies of the gene, one inherited from each parent, are necessary to have the condition. Once parents have had a child with CF, there is a one in four, or 25 percent, chance with each subsequent pregnancy for another child to be born with CF. This means that there is a three out of four, or 75 percent chance, for another child to not have CF.

The birth of a child with CF is often a total surprise to a family, since most of the time there is no previous family history of CF. Many autosomal recessive conditions occur this way. Since both parents are healthy, they had no prior knowledge that they carried the gene, nor that they passed the gene to the pregnancy at the same time.

Genes are founds on structures in the cells of our body called "chromosomes." There are normally 46 total, or 23 pairs of chromosomes in each cell of our body. The seventh pair of chromosomes contains a gene called the CFTR (cystic fibrosis transmembrane regulator) gene. Mutations or errors in this gene are what cause CF. This gene is quite large and complex. Over 1,000 different mutations in this gene have been found which cause CF.

The risk for having a mutation in the gene for CF depends on your ethnic background (for persons without a family history of CF):

Testing for the CF gene can be done from a small blood sample or from a "cheek swab," which is a brush rubbed against the inside of your cheek to obtain cells for testing. Laboratories generally test for the most common mutations, and most labs test for anywhere from 30 to 100 total mutations. The detection rate depends on the person's ethnic background. In general, the detection rate for the Caucasian population is around 90 percent, 97 percent or more for the Ashkenazi population, 57 percent for Hispanics, 75 percent for African-Americans, and 30 percent for Asians. The detection rate differs because CF is more common in certain geographical areas and certain populations of the world. The diagnosis is confirmed by another test call the sweat chloride test.

There are many people with CF whose mutations have not been identified. In other words, all of the genetic errors that cause the disease have not been discovered. Because not all mutations are detectable, a person can still be a CF carrier even if no mutations were found by carrier testing.

Testing for the CF gene is recommended for anyone who has a family member with the disease, or whose partner is a known carrier of CF or affected with CF.

Download Genetics of Cystic Fibrosis from the CF Foundation

The Cystic Fibrosis Center abides by strict infection control guidelines both inpatient and outpatient programs. These guidelines are set forth by the Cystic Fibrosis Foundation for the protection of all people living with CF while in the medical center. The following guidelines reflect current infection control procedures in clinic, including changes made recently based on the Updated Consensus guidelines from the CF Foundation Spring, 2014.

Information on Germs and Lung Health Information on Stopping the Spread of Germs

Outpatient Center

• Contact between CF patients needs to be very limited.  People with CF should stay at least six feet from each other.  Every attempt is made to move patients and families quickly through registration and into a visit room. 
• While in the waiting room, ALL patients will be asked to wear a mask. Masks are available at the registration desk in both the pediatric and adult clinics and should be worn any time you are outside your clinic or hospital room.
• For the pediatric program, we encourage parents to supervise children at all times and we also recommend that children bring their own toys from home rather than playing the with toys in the waiting room. 
• We discourage use of the computer in the waiting room for infection control reasons.
• All staff will gown and glove for your visits. This will include all front end nursing staff and the CF team.
• Staff wash their hands on the way into and out of each patient’s room. 
• The respiratory therapist will gown, glove and mask while assisting patients with PFTs as well as when collecting sputum samples. 
• Patients are also strongly encouraged to stay in the treatment room at all times unless using the bathroom.
• All treatment rooms, triage areas and equipment is wiped down with anti-bacterial wipes after each patient. These wipes are approved for all bacteria, including Pseudomonas and B. Cepacia.  
• MRSA Precautions: Current guidelines require that all patients be placed on contact precautions, Previously, this policy was only for patients colonized with MRSA.
• Pulmonary function testing and sputum cultures will take place in your clinic room rather than moving in and our of the pft lab. The purpose of this is to reduce exposure to bacteria that may remain airborne for up to 30 minutes after testing. If the pft lab is used for any testing, the RT will clean the room and allow 30 minutes between patients.
• Patients will be asked to wear gloves while performing pulmonary function testing.

Note: CF related MRSA is NOT a danger to people in the community.

**B. Cepacia:  Patients who culture Burkholdeia cepacia will continue to be scheduled to come to clinic on a different day than the primary CF clinic day.  Currently, these are the second and fifth Friday of every month in the pediatric program and one Thursday per month in the adult program. Similar infection control policies will be in place on B. Cepacia clinic days.

Hospital Infection Control Policy

All pediatric, adolescent and adult patients with CF will be placed on contact precautions during their stay at BMSCH and RWJUH.  This means the following:

• All patients with Cystic Fibrosis will always be in a private room.
• All hospital staff must gown and glove when entering the room and remove gown and glove and wash hands upon leaving the room.  This includes doctors, residents, nurses, assistants and housekeeping staff.
• All patients with CF should have their own stethoscope and blood pressure cuff in their room.  If you do not, please ask your nurse or medical technician. 
• All patients must wear a mask when leaving the room and will not be allowed to visit another patient with CF in the hospital.
• Patients who wish to leave their rooms must wear a mask and are strongly encouraged to go for walks off the unit.
• Doors to patient rooms should remain closed
• Respiratory therapists must wear masks in addition to gown and glove because they are inducing sputum.
• Whenever possible, CF patients will perform pfts in their hospital rooms rather than coming down to the pft lab.
• No two CF patients will be assigned to the same nurse on a given day
• Family visitors are not limited.  However, family members who are sick are discouraged from visiting. 
• Pediatric and adolescent patients are not allowed in the Child Life room or Teen lounge.  Representatives from the Child Life Department will visit patients frequently and bring various activities, games, etc to rooms.

Cystic Fibrosis can be a very demanding disease to manage and there is a lot to remember! Often in CF, children and adults get involved with doctors from various disciplines, like Gastroenterology or Endocrinology. Keeping track of medications, changes, appointments and events in your medical history can challenge even those of us with the best memory and organizational skills. You are also asked similar questions time and time again, resulting in the need to repeat yourself.

Many families have found that keeping a medical profile can be very helpful in keeping track of everything. It can also be very helpful to your medical providers to review it for any changes, new medications, etc. Medical profiles will also save you tremendous time when asked to recite all of your child's medications, medical history, etc.

We recommend you develop a medical profile and carry it with you to appointments, procedures, hospitalizations, etc. There are so many ways to do this and so many formats that people find helpful. Attached is one format that some of our families use. Feel free to print this or download it and use it.

Example of a Medical Profile

According to the CF Foundation, there are many things you can do to prevent illness and keep your lungs healthy.

Most people with cystic fibrosis can lead active lives with routine therapies and regular visits to a Cystic Fibrosis Foundation-accredited care center.

Knowing your CF is important to staying healthy. The staff at our center partner with you to develop individual treatment plans. These plans typically include high-calorie, high-fat diets, therapies to loosen the clogged mucus from the airways, and mucus-thinning drugs and antibiotics when needed.

By following a treatment plan developed your CF team, you can slow down the progression of the disease. A healthier body is better able to deal with bacteria and chronic lung infection.

Cystic fibrosis puts the airways at risk for lung infections. There are, however, effective ways to lessen the risk. One way is to limit contact with known germ sources.

Although germs are everywhere and cannot be avoided, one of the best ways to keep from catching or spreading germs is through effective hand-washing, whether with soap and water or alcohol-based hand gels.

Everyone with CF should avoid unnecessary contact with people who have a cold or any other contagious illness, and should cough and sneeze into a tissue.

Flu season, which begins in August and lasts throughout the winter, is the time of year that the influenza virus is easiest to catch. The flu is considered highly contagious, because it can spread by direct contact, coughing, sneezing, and when an infected person touches a surface that others then use, like door knobs and railings.

People with lung infections, including people with CF, can develop more serious cases of the flu, so it is important for you to get a flu shot to be immunized against the most prevalent strains.

Our care center offers the flu shot to all patients with CF beginning in August every year.

Everyone with CF and members of their households who are at least 6 months old need to be vaccinated for both viruses.

When summer comes, we want to work with you to make sure you have a healthy and happy summer. Here are some tips on staying healthy in the hot months!

• HYDRATE, HYDRATE, HYDRATE - Remember, people with CF can lose up to 5 times more salt in their sweat than someone without CF. Remember to drink even when you are not thirsty, especially during outdoor exercise. Drink before, during and after exercise. Sports drinks with added salt and carbohydrates are great at this time of year. Children are more susceptible to dehydration to make sure the little ones drink extra!
• Caffeine does NOT count as part of the total ounces of liquid you drink in a day. This includes all caffeinated sodas, coffees and tea.
• Infants dehydrate more quickly than young children and adults so keep your baby well hydrated and in cool clothing.
• EAT MORE SALT!! - Examples of foods to add during the summer are: pretzels, salted nurs and seeds.
• Know the signs of dehydration and hypoatremia (low sodium levels in your blood). These might include nausea, vomitting, headaches, muscle weakness and cramping. Call your CF team with any concerns.
• Medications - Keep your medications cool while out and about. Some medications require refrigeration so make sure you know which of your meds need to be in the fridge. Others, like enzymes, should be kept at room temperature. Keep a cooler handy if you are going out and plan to bring your enzymes or other medications..
• Wear your sunscreen!! SPF should be 30.

Exercise

In addition to respiratory therapies and oral medications, exercise has been found to be extremely beneficial to people living with CF at all ages. We cannot underestimate the importance of adding this to you/your child's routines. Patients report a much greater feeling of well being when involved in a regular exercise routine.

When to Call the Cystic Fibrosis Center

Your/your child's health is our primary concern. We are available to assist you in maintaining your health and prescribing treatment when necessary. There is always someone on call to help at any time of day or night. Here are some things to look for that may require a call to the CF center:

• Increased respiratory symptoms such as: increased cough and/or sputum production, chest tightness, shortness of breath, increased congestion, wheezing, nighttime awakening due to cough
• Increased fatigue, fever, lethargy, weight loss, change in appetite
• Symptoms of dehydration, including excessive thirst, lack of tears, increased sweating, dry mouth, dry diaper, decreased urination, lethargy
• Any signs of hemoptysis (spitting of blood). Be prepared to describe color, amount and when it started,( ie. streaking, size of a coin, tablespoon, shot glass)
• Change in bowel habits, i.e. constipation, diarrhea, loose or greasy stools, blood in stool, nausea, vomiting
• Abdominal pain, moderate to severe, abdominal distention, bloating, gas, vomiting
• Unexplained fatigue, increased urination or thirst

In this section, you will learn about the most common tests ordered for CF patients.

At diagnosis, we perform several tests to help us make the diagnosis and determine what treatment you will require.

After diagnosis, we ask all CF patients to have annual studies. Annual studies are a critical part of your ongoing care and are strongly recommended by the Cystic Fibrosis Foundation. They are an important tool we use to help you monitor your (your child's) health, nutrition status and the progress of your lung disease. Our goal is to help you (your child) remain as healthy as possible.

Routine testing is also done at every clinic visit. This includes pulmonary function testing and sputum cultures.

Bronchoscopy is also described in this section.

If you have any questions about the tests we perform, please feel free to discuss with the team.

Newborn Screening

Newborns screened for cystic fibrosis can benefit from early diagnosis and treatment, which can:

• Improve growth;
• Help keep lungs healthy;
• Reduce hospital stays; and
• Add years to life

While newborn screening is not a definitive diagnostic test for cystic fibrosis, it may lead to tests that can rule out or confirm a CF diagnosis. All states in the U.S. screen newborns for cystic fibrosis.

In New Jersey, all newborns are screened with 1-2 days after birth. Results of the newborn screen will be sent directly to your pediatrician along with recommendations for repeat or referral if screen was positive. A positive newborn screen is NOT a diagnosis of Cystic Fibrosis. Usually the screen is repeated and if the second screen is also positive, you and your child should be referred to an accredited CF Center for further testing.

Genetic Testing

When a patient is referred to our center for testing, we may order a few genetic tests to look for the presence of two CF causing mutations.

Only about one of every 3,000 Caucasian newborns has CF. We know there are more than 1,800 known mutations of the gene that causes CF. However, not all mutations cause CF. The ones that do cause CF are called "disease-causing" mutations. Current tests look for the most common disease-causing mutations.

More than 10 million Americans are carriers of one mutation of the CF gene. Among Caucasian Americans, about one in 29 people carry one mutation of the CF gene. In other races or ethnicities, one in 46 Hispanic Americans, one in 65 African Americans and one in 90 Asian Americans carry a mutation of the CF gene.

People inherit (or get) genes from their parents. To have CF, a child must inherit one copy of a mutation of the CF gene from each parent. In other words, the child must have two copies of the gene with mutations to have CF. Both males and females can have the disease.

People who have only one copy of the CF gene do not have. They are called "carriers" of the CF gene.

The chances of being a carrier of one CF mutation or having CF (with two CF disease-causing mutations) depend on your race and ethnicity. CF gene mutations are most common in Caucasian Americans (white people whose ancestors or family are from Europe).

In the U.S., the number of people who carry a CF gene is about:

• 1 in 29 Caucasian Americans;
• 1 in 46 Hispanic Americans;
• 1 in 65 African Americans; and
• 1 in 90 Asian Americans.

In the U.S., the number of people who have CF is about:

• 1 in 2,500–3,500 Caucasian Americans;
• 1 in 4,000–10,000 Hispanic Americans;
• 1 in 15,000–20,000 African Americans; and
• 1 in 100,000 Asian Americans.

According to the CF Foundation, the pancreas is a gland in the body connected to the small intestine. The small intestine is where most of the digestion and absorption of food occurs. One of the functions of the pancreas is to make enzymes that digest food. Digestive enzymes from the pancreas enter the small intestine through a small duct, or passageway. In cystic Fibrosis, the ducts int he pancreas become clogged with thick, sticky mucous. The mucous blocks the enzymes from reaching food in the small intestine. This can lead to poor digestion and absorption of food, as well as problems with weight gain. Fortunately, pancreatic enzyme replacements or "enzymes" are available to help people with CF digest and absorb their food.

Signs of Maldigestion and Malabsorption:

• Poor weight gain despite a good (sometimes ravenous) appetite;
• Frequent, loose and/or large bowel movements;
• Foul-smelling bowel movements;
• Mucus or oil in the bowel movement;
• Excessive gas and/or stomach pain;
• Distention or bloating.

Just under 90% of patients with CF are pancreatic insufficient (PI) and require life-long supplementation with pancreatic enzyme replacement therapy (PERT). Fecal elastase is an enzyme- linked immunosorbent assay (ELISA) that can be performed on a single stool sample to define pancreatic functional status. Your physician will order a Pancreatic Fecal Elastase to be performed at most local laboratories. The result of this test will tell us if your/your child's pancreas is functioning well enough or if there is a need for enzymes.

Persons with CF who have not yet started taking enzymes may have any or all of the symptoms listed above. Improvement is generally noted once enzymes are started. Sometimes, people with CF who already take enzymes experience these symptoms. This may suggest that the dose or type of enzymes may need to be adjusted. Do not increase or decrease the dose of enzymes without talking to your CF dietitian or care provider. Always give the prescribed dose of enzymes.

Per CF Foundation Guidelines, sputum cultures are obtained at every clinic and hospitalization to see what bacteria you may have and what antibiotics can be effective in fighting it. The CFF consensus statement on infection control now recommends quarterly sputum cultures and cultures during acute exacerbations. Children and adults who cannot produce a sputum sample will receive a throat swab. However, a sputum sample is preferred once a child is old enough to produce sputum.

The Robert Wood Johnson University Hospital microbiology laboratory meets the Cystic Fibrosis Foundation’s (CFF) standards of care for processing CF sputum cultures. The CFF established standards for processing CF sputum require that CF sputum must be plated within 3 hours on specific media to obtain accurate results. Inaccurate sputum culture results can impact treatment decisions and health outcomes

There will be times when we might also choose to run a "fungal culture" or AFB (Acid Fast Bacilli) test for other bacteria that may be causing illness. These tests can only be run on sputum samples, rather than a "throat swab". Below is a list of terms used to describe sensitivity testing done for the bacteria grown in your culture.

There may be times when the doctor believes there is a need for a bronchoscopy. This is often suggested when patients are not responding to therapy as well as expected, are getting sick more frequently, or the doctor suspects there is unidentified bacteria in the lungs which needs treatment. During a bronchoscopy, the doctor can obtain sputum cultures from deeper sections of the lungs for testing. The doctor also has an opportunity to clear mucous plugs from the lungs during the procedure.

Is a minimally invasive procedure done under general anesthesia with a flexible bronchoscope. After obtaining consent from a parent or guardian, the patient is placed under general anesthesia by our pediatric anesthesia team. Bronchoscopy is performed through an airway provided by our anesthesia team, both lungs are examined and limited washings for cultures are performed and samples are combined and sent for testing.

Culture results from the bronchoscopy are sometimes immediately available but some cultures will grow for up to six weeks before they are finalized.

When you or your child is diagnosed with Cystic Fibrosis, you, like many others, may know very little about this illness. Diagnosis may come as a shock and can be a very difficult thing to face. Diagnosis also leads to many questions and may result in feelings of being overwhelmed, fear, anxiety and sadness. As your CF care team, we are committed to helping you and your family adjust to the diagnosis, develop skills and knowledge to manage the illness, and find a balance between taking care of CF and living a normal life. It is critical for parents to help their children develop normally and have the same expectations of their child as parents do for children who do not have Cystic Fibrosis. This will give your child the best quality of life and help them to prepare for adulthood.

Your CF team will give you books developed by the Family Education program of the Cystic Fibrosis Foundation. These books will help to address most areas of concern and provide a great deal of knowledge about treatments and healthy living with CF. It is also important to note that patients and families become part of the care team. We want you to be a partner in your/your child's care.

In this section, you will hear from other families about their experiences living with CF. You will also be able to get more insight into the various areas that are impacted by Cystic Fibrosis, including education and employment.

This is a common diagnosis story sent in by a family at our CF Center.

It was a Friday evening at approximately 4:45pm. The telephone rang and I headed to my phone in the kitchen. I answered the phone and the caller on the other end of the line identified herself as the receptionist from my 9-day old newborn’s pediatrician’s office. The receptionist said, “The results from your newborn’s screening just arrived and it is presumptive positive for Cystic Fibrosis, can you please hold?” As I stood holding the phone receiver in my hand, I immediately felt numb and confused. Being a new mom and young, I had no idea of what Cystic Fibrosis is (I was barely able to say it in my head), but knew it did not sound good. After what felt like an eternity had passed, the receptionist came back on the line and said, “The doctor would like to see you on Monday to discuss the results and schedule a test to confirm the results.” An appointment was scheduled for Monday and the phone call ended. I stood there for a moment trying to process what was just told to me. I tried to call the office back to see if the doctor was available so I could ask “What is Cystic Fibrosis?” However, being that it was two minutes until five o’clock on a Friday evening, no one would answer the phone. I quickly glimpsed at my newborn, who was peacefully asleep on the couch. I immediately logged onto my computer, went to a search engine and typed Cystic Fibrosis. I could feel my heart pounding as I waited for the webpage to load. I clicked on the first site that popped up and started reading. My eyes were flying over the words and seeing words like, Life-Threatening, median age, skin salty to taste. The last one caught my eye, “skin salty to taste”. I got up and ran through my apartment to my baby. I licked her forehead only to find that it had a light salt taste; I licked my arm as a comparison. I knew right then that the words on my computer screen were true and did not need a medical test to confirm my thoughts. I began to cry which turned into weeping as I went back to my computer and kept reading. Upon my husband returning home, I was crying so uncontrollably that I was barely able to speak. All I could say between sobs was, “She has Cystic Fibrosis.”

The following week involved driving to the local Cystic Fibrosis Center and having our newborn go through the Sweat Test Screening. We waited at the hospital for the test to be run. As we ate lunch in the hospital cafeteria, I kept looking at my new baby girl thinking she looks okay and that there is nothing wrong with her. Some of the information on the website mentioned problems that some infants have with regaining their birth weight. I knew in the back of my head that since we left the hospital 15 days ago she had no problems regaining her weight. So, I had a small hope that I was worrying too much as I always do and there was nothing wrong after all. We finished lunch and had another short wait before being lead into the exam room. My husband and I sat in the room in silence while our newborn slept in the car carrier. The doctor entered the room with the nurse practitioner and delivered the news that our daughter in fact has Cystic Fibrosis. As the doctor talked and gave general information about Cystic Fibrosis I half-listened. I had so many thoughts running through my head like, “What does this mean? Is she going to die? What do I now?” The doctor handed us prescription after prescription saying, “This is for this, this is for that”. I tried to take notes, but it was all so confusing. I left that appointment feeling scared, confused and sad; but most of all I was determined to learn as much as I could.

You may or may not find similarities in my recollection of how we learned of our daughter’s disease. However, one thing is certain for all of us, we all have a story of how we began on the CF journey. A journey that is constantly changing and unique for each person.

Whether you are the parent (or any relative) of a child newly diagnosed or you yourself have been diagnosed, either situation brings on more questions/concerns than answers. You will go through many mixed emotions and may feel overwhelmed with this diagnosis.

The best advice that I, as a parent of a child with CF, would give to others on this journey would be to take one day at a time. You are NOT going to understand everything overnight. While you are adjusting to the diagnosis, try to get into a “routine” of giving/taking meds as soon as possible. The sooner you establish a “routine”, the better for the health of your child/self.

As a non-medical person, I would suggest the following:

1. Read, Read, Read – Educate yourself about this disease to best of your ability.
2. Ask Questions – Do not be afraid to ask your CF team any question (hopefully this guide will answer some of your questions).
3. Get Support - Surround yourself with support from others that are going through the same experiences and/or seek assistance from family and friends.

In the end, if you still feel that the diagnosis is too much for you to handle on your own I would recommend seeking professional help.

It is important to note that CF is a chronic disease and you/your child will live with this for your entire life. You will be asked to learn about various treatments and medications that you/your child requires daily. These treatments are intended to slow the progression of the disease and keep you/your child healthy. These treatments typicallly include airway clearance, nebulizer treatments, pancreatic enzymes, CF specific vitamins and oral antibiotics. We strongly encourage you to think about taking some of these steps to help with the adjustment:

• Make CF treatments part of your daily routine
• Share responsibilities with your partner/family member
• Find people to talk to, develop a support system
• Expect letdowns, do not expect this to be a smooth road
• Take time for yourself and do things you enjoy

Once you have made the initial adjustment to CF, you will start looking forward and planning ahead. As you and your family move through life, here are some age specific resources that might be helpful.

The Cystic Fibrosis Center is committed to patient and family centered care. Our pediatric and adult teams work closely with one another and with our families to make sure that the care in clinic and in the hospital is always improving to meet your needs. In order to assure that we remained focused on empowering patients and families and providing sensitive and wholistic care, we have implemented several programs:

Family Advisory Council

This council is central to our efforts to remain patient and family centered and is comprised of family members from both the pediatric and adult programs as well as staff from both programs. The council meets several times per year and is involved in planning new outreach programs for families, communication strategies, family education day, etc. This group is also instrumental in providing feedback about the quality of care in clinic and in the hospital. Membership on this council is always open and we welcome new ideas. Members can participate in person or via conference call. In the last year, the council has focused on several priorities:

• Family Resource Website - This guide, originally envisioned as a book to give to patients and families, has expanded to the development of an entire website dedicated to our CF center and families. This is a comprehensive guide to our CF center and the hospital, both pediatric and adult. The most important aspect of this project is that families have been involved in the development from the beginning and are an integral part of the project.

• Center Newsletter/EBlast - This Eblast is managed entirely by the Family Advisory Council with contributions from the staff at both programs. The first edition of the newsletter was released in May 2012. The purpose is to keep families involved in the center and up to date on anything new in the CF community.

• Family to Family Support Network - This is an informal network at this time which provides support to families of CF patients at challenging times in their lives. Matches are made by the center staff and only with permission from both parties. The staff at the center has already paired family members or patients within the center who express a need for additional support.

• EPal program - This program was launched in early spring, 2012. The intent of this program is to give our patients and siblings someone “like them” to talk to, with the approval and supervision of their parents. The center staff assist in making the connection but has no involvement in the relationship beyond that point.

The CF center has also developed a patient satisfaction survey. Information from the survey is used to improve care.

The social worker regularly uses an email network with willing families to keep you updated quickly on changes, new information, etc. This email network has made communication much easier and has helped families feel more connected to their center and new information that comes from the foundation and other sources.

In 2013, the CF team started our own CF walk in New Brunswick with the help of the NJ Chapter of the CF Foundation. This was the first walk for the New Brunswick location and raised over $30,000 in its first year. In its second year, the walk moved to Edison and raised $52,000. We hope this walk will grow with every passing year and will include as many families from this center as possible.

The CF Center holds Family Education Day once per year. All the members of the CF team from both the pediatric and adult program attend the meeting. This day is usually full of updates for families on activities at our center as well as research and other programs sponsored by the CF Foundation. We always welcome a keynote speaker and the afternoon is full of small group discussions, where family members get the chance to meet one another and talk about life with CF. Many families find this a rewarding part of the day.

We strongly encourage you to plan with your child to have a long and full life. Education is part of that. Children with CF can participate in all the activities that children without CF can participate in. They should be encouraged to work hard in school and plan for their future. However, there may be times when having CF interferes with their daily routine or their ability to meet all expectations. Children and adults with CF also have special needs that we need to prioritize in order to keep them healthy in school and give them the best chance of success.

To that end, we usually encourage all families to work with their schools to establish an appropriate 504 plan or IEP if needed. This is often something that you initiate when your child begins Kindergarten but can be initiated at any time over the years they are in school.

When students get to college and post graduate education levels, schools have fewer guidelines that they must follow and the rules change but you are still eligible for certain accommodations and services. Registering with the Office For Disabilities at the university or college your are enrolled in should be a first step. Accommodations for residency are very common and your CF center social worker will assist you with this.

You will find links below to helpful information about school and CF, as well as stories from our families about common issues they have dealth with in school. For those in college, there is also information on going away to school and what to think about. The social worker at our center can assist you with any of your school related needs.

People with CF are now living full lives well into adulthood. Our patients have careers and families and work hard every day to stay well. Young adults transition to the adult CF program by age 23 or before. The adult program works hard to provide the same level of care that patients have grown up with in their pediatric centers. However, adulthood brings different challenges and adventures and the adult team pays close attention to those issues. The major decisions and events that adults with CF face are career, marriage, families, future planning and planning for their healthcare.

Pretty soon, over half of the population living with CF will be over 18. The CF Foundation and adult CF programs around the country are working hard to address the special needs of adults. Below are some links to more information:

Information on Managing Cystic Fibrosis

It is important to remember, whether you have a newly diagnosed infant or you are a young adult graduating from high school, you can have a full life.

CF will present you or your adult child with challenges and hurdles but there will be a lot of support along the way. Be open to the possibilities and you will achieve much more than you might think.

Health insurance and health care costs can be very intimidating.  Health insurance has become very complex in today's world and it can sometimes feel like your health insurance gets in the way of good health care! As a parent of someone living with CF or an adult who is living with CF, it is very important that you understand your benefit plan and stay on top of the changes that often happen in a plan on an annual basis. The social worker at your CF center would be happy to help you understand and navigate this universe if things get complicated. Our office also has a department for prior authorizations that helps to get things approved when needed, whether that is an expensive test or a medication that might requires approval.  Please feel free to call our office when you have questions. 

The recent health care legislation and addition of Obamacare has helped people with CF in many ways but has also complicated things. If you have questions about the healthcare legislation or Obamacare, visit HealthCare.gov.

Below is also a helpful breakdown of the changes that came with the new healthcare legislation. If you believe you might qualify for NJ Family Care or medicaid, this is also a good site to go to for information. You can also visit www.njfamilycare.org.

In some cases, you may find yourself without health insurance and not knowing what to do. Please be assured that we are here to help guide you through this and can help you get many of your medications. Please call the center social worker if you need help.

The CF Foundation has also established a patient resource center that might be very helpful for those who find themselves in difficult situations with health insurance.

Below are two more helpful documents developed by the CF Foundation to help navigate the world of health insurance.

Know Your Health Insurance Coverage Working With Your CF Center and Insurance Company

Good nutrition plays a vital role in the health of a person with Cystic Fibrosis. This cannot be underestimated or understated. Nutrition care is an important focus of attention in CF care and your dietician will meet with you regularly throughout the year to make sure you/your child are reaching and maintaining your nutrition goals. These include height and weight but also vitamin levels. Years of research through the CF patient registry has shown the following:

1. Maintaining normal growth and weight that is average or above average is associated with better lung function, less frequent infections and hospitalizations in people with CF.
2. Monitoring nutrition and growth is very important in CF because, as a group, infants, children, adolescents and adults with CF have heights and weights that are below average for their age and sex.
3. Poor growth and lower than average weight are both correlated with decreased lung function in people with CF.

Impact of CF on Nutrition

• Chronic lung inflammation increases calorie and protein needs so children and adults with CF need more calories than a child without CF
• Appetite and food intake are often affected by illness so treating infections early can help keep appetite and weight stable. Decreased appetite and weight loss can also be a sign of illness
• CF can cause a number of gastrointestinal issues which could results in loss of nutrients due to poor absorption, vomiting, and or discomfort associated with eating
• Endocrine problems associated with CF cause abnormal metabolism (processing) of nutrients
• Social and psychological issues can affect nutritional status in any age group

Meeting Nutritional Needs and Goals

Infants, children and adolescents with CF are expected to gain weight and grow like their peers without CF.

1. Your child’s weight and length are measured and monitored closely by your CF team using GROWTH CHARTS
2. Length and height should progress at a predictable rate for age, sex and genetic potential.
3. Weight should be maintained at an average weight for length or height according to National

Standards for all healthy children

• From birth to age 2 years weight and length are plotted on the growth chart.

  A weight for length at or above the 50th percentile is an “ideal body weight” for an infant or young child with CF

• From age 2 to 20 years the body mass index (BMI) is plotted on the growth chart

  BMI is the relationship between weight and height (BMI= kg body weight/m2 height)

  A BMI at or above the 50th percentile is an “ideal body weight” for a child with CF.

• Adults with CF over the age of 20 are expected to maintain an average BMI

  Adults with CF over the age of 20 are expected to maintain an average BMI

  A Male’s ideal BMI is 23kg/ m2

Critical Growth Periods are times of life when energy and nutrient needs are at their highest and can be challenging times for maintaining optimal nutrition for people with CF

• Calories - People with CF have calorie requirements that are higher than people without CF. They need to consume up to twice as many calories daily

• Protein - People with CF need 1.5 to 2 times more protein in their diets

• Fat - Due to poor fat absorption, people with CF need 10% more fat than the average person

• Carbohydrates - Carbs are a great source of energy and calories and do not need to be avoided, even in the setting of diabetes

• Salt and chloride (Table salt) - All people with CF need additional salt in their diet to replace higher than normal salt losses.

• Vitamin and mineral supplementation - People with CF and pancreatic insufficiency have difficulty absorbing fat soluble vitamins A, D, E and K. CF specific vitamins were designed to offer higher levels of vitamins and minerals that CF patients need

• Enzyme replacement therapy - 90-95% of all CF patients have pancreatic insufficiency and will need enzyme therapy to boost absorption of fat, calories, vitamins and minerals consumed.

More Information on Nutrients

Meeting Calorie and Nutrient Needs

High calorie, high protein, high salt diet

• Calorie boosting starts at birth and will continue as needed throughout life
• Making meals and snacks a priority takes additional time and attention
• Oral Supplements and shakes can be an easy, healthy and convenient way to add calories to the day when you are too busy to think about making calorie dense snacks
• Parents and children benefit greatly from taking a behavioral approach to feeding young children. See social and psychological issues for more information.
• Tube Feeding is sometimes necessary in more serious cases of malnutrition or when people simply cannot meet their calorie needs orally. There are two types of tube feeding, nasogastric or gastrostomy. If this option is necessary, the team will discuss this with you at length and will involve our pediatric or adult GI specialists.

Tube Feeding

Tube feeding can be a great way to get the calories and nutrients that you or your child with cystic fibrosis need to gain and maintain a healthy weight. Explore this as an option with your CF care team. Parenteral Nutrition is nutrition given through a large vein. This is only used in more serious cases of malnutrition when a G tube is not possible. There is a greater risk of infection with parenteral nutrition but it is closely monitored.

Age Related Guidance on Nutrition

Cystic Fibrosis Related Diabetes (CFRD)

Cystic Fibrosis Related Diabetes is a unique form of diabetes that occurs frequently in people with CF. It shares features of both Type 1 diabetes and Type 2 diabetes but it is very different from these forms of diabetes in many ways

• Causes
• Prevalence
• Symptoms
• Screening and Diagnosis
• Treatment

Pubertal Delay

CF Bone Disease

• Osteopenia and Osteoporosis

Feeding Problems in Young Children

Problematic feeding behaviors occur more often in children with CF and other chronic diseases. The use of problematic feeding behaviors is associated with lower nutrient intake and lower weight in children with CF. Children use ineffective feeding behaviors to seek caretaker attention no matter how negative the attention may seem. Caretakers give increased amounts of attention to the child in response to these behaviors and so they are repeated.

Prevention of Behavioral Feeding Problems

• Start purees at 4-6 months of age
• Transition to soft table food starting at 8-10 months
• Transition to cup by one year of age

Maintain a separation of responsibility when feeding your infant or child

• The caretaker should be responsible for when and what food and beverages are offered and for providing a non-pressured, enjoyable family meal environment. The caretaker should also be aware that appetite may decrease because of any of the gastrointestinal issues common to CF as well as during illness.
• The child should be responsible for how much is eaten, which foods are eaten, and even if he or she is going to eat at all.
• Support your child with alternative feeding methods. It is not uncommon for caretakers to engage in ineffective feeding behaviors that result in less food intake rather than more and can cause a large amount of family stress.

Treatment for behavioral feeding problems

Recognize common ineffective feeding behaviors of young children and commonly employed but ineffective behavioral responses caretakers engage in.

• Child: Chatting instead of engaging in eating
• Parent: Prompting child to eat, take one more bite, eat his veggies etc
• Child: Repeatedly leaving the table or disrupting the meal
• Parent: Talking to the child and insisting they stay at the table and have to eat eat, eat one more bite, eat their veggies etc.
• Child: Drinking instead of eating
• Child: Not taking bites
• Parent: Urging, bribing, negotiating with the child to eat, eat one more bite etc.
• Child: Not trying, refusing novel or “non-preferred food”
• Parent: Urging, bribing, negotiating with child or forcing the child to eat the food, short order cooking, or providing only preferred food.

In each scenario the caretaker is giving his or her attention for a negative behavior.

Behavioral strategies that include ignoring negative behaviors and giving attention for positive eating behaviors have proven effectiveness in improving food intake and weight status in children with CF.

Positive reinforcement of specific behaviors by giving your attention when your child is eating and ignoring when not is a powerful tool in helping your child eat normally. Let your child know specifically what he or she is being praised for.

• "Good job taking that bite"
• "Good job eating your veggies"
• "I like the way you finished your meat so quickly"
• "I think it's great the way you take your enzymes when I put it by your plate"
• "Wow, I am so proud of you for trying those carrots"

Using these reinforcers with other good eaters at the table while ignoring the child who is not eating, refusing to try, demonstrates to your child how to get your attention in a positive way and that negative ways are not effective in getting your attention.

Your child may “push back” during this change in your behavior with even more attention seeking behaviors including screaming or throwing food. Continue to ignore these behaviors, hang in there, be consistent and the child will figure it out.

Some may need assistance from a skilled mental health professional

CF patients under 1 year of age are normally seen 1x per month until 12 months of age, then every two months. Children, adolescents and adults are seen every two to three months or more frequently depending on your health status.

The scheduling line is open Monday through Friday, 8:30 am to 4:30 pm.

*If you need to cancel an appointment, simply call the scheduling line to cancel and request a reschedule. If you have difficulty rescheduling, please contact our office directly.

• Pediatric Pulmonary Scheduling: 732-235-7899
• Adult Pulmonary Scheduling: 732-235-7839, prompt 1

Many patients find that keeping a record of all medications and treatments is helpful in managing their health and remembering all there is to do. This plan can also be a helpful tool in clinic when changes are made to your/your child's healthcare regimen. You can work with your care team to review it at each clinic visit and make necessary changes.

Download the Care Plan Form

Living with a chronic disease brings many challenges throughout the lifespan for the whole family. Your CF team is here to help with those challenges, whether they are medical, emotional, educational, financial or employment related. Our licensed clinical social worker is available at any time to talk with you, either in clinic or by phone or appointment. If we don't have the resources you need here, we can help connect you. Below is a list of most commonly used resources but this is not intended to be an exhaustive list. Please contact your CF social worker for more information.

The Cystic Fibrosis Center at Robert Wood Johnson Medical School is a designated center for the TDN (Therapeutics Development Network). 

The Cystic Fibrosis Therapeutics Development Network (TDN) is the largest cystic fibrosis clinical trials network in the world.

The TDN brings together experts from across the United States to evaluate the safety and effectiveness of new CF therapies through clinical studies. We work continually to improve research methods and pursue new areas of scientific inquiry.

Through efficient study design, optimized clinical trial execution and high-quality data, the TDN helps speed the delivery of new and better therapies to people with CF.

This center, along with many CF centers around the country, is involved in multiple clinical trials to develop new drugs to treat this disease. For more information on CF related research, please go to: Therapeutics Development Network (TDN) | Cystic Fibrosis Foundation Therapeutics.

If you have questions about your eligibility to participate in a research study, please talk to your CF team.

To search clinical trials, you can also go to Clinical Trial Finder.

Looking for a great way to get your family more involved with the local community? Searching for a serious challenge that will push you mentally and physically? Or just want to contribute to a worthy cause?

You’ve come to the right place. Each year, thousands of volunteers, families, coworkers and friends come together at exciting fundraising events to help add tomorrows to people living with cystic fibrosis — and you can, too.

The Cystic Fibrosis Foundation offers a number of unique opportunities that fit a range of interests, budgets and lifestyles. Find the event that’s right for you and get involved today!

For information on the various fundraising campaigns that the CF Foundation is involved in, go to: Foundation Events | CF Foundation.

Our center participated in the Great Strides Walk every year to support the NJ Chapter of the CF Foundation.  We encourage all of our families to join us at this great event. Your center coordinator can give you more details about walk locations and dates in New Jersey.

CF is a Multi-system disease that affects every area of your body and your life. Despite new drugs to “modify” the effects of this disease, management of CF is complex and time consuming (averaging 2-4 hours per day). As a result, CF continues to be one of the most difficult chronic conditions to manage. Studies measuring psychological distress in individuals with CF , and their parent caregivers, have found high rates of both depression and anxiety. Psychological symptoms in both individuals with CF and caregivers have been associated with decreased lung function, lower BMI, worse adherence, worse quality of life and more frequent hospitalizations. (Quittner AL, et al., 2015)

The TIDES study was an international study, sponsored by the CF Foundation and the European CF Society, which looked at the prevalence of anxiety and depression in CF.

• Screened over 6000 patients ages 12 and older and 4000 parents.
• Indicated higher rates for both depression and anxiety
• Higher rates of anxiety

Important Finding: When parents report elevated depressive or anxiety symptoms, their adolescent with CF is more than twice as likely to experience depression and anxiety (Quittner AL, et al., 2015).

A panel of experts worked together over 18 months to:

• Review the research
• Develop recommendations for clinical care

The Panel developed 15 recommendation statements for screening and treatment of depression and anxiety in individuals with CF and parent caregivers (Quittner AL, et al., 2015).

Consensus Statement

Periodic screening of depression and anxiety symptoms and appropriate interventions can significantly improve quality of life and health of individuals with CF and their parent caregivers

• All CF Centers have been asked to provide ongoing support and education to all patients and families to promote stress management, health coping skills, etc.
• The Cystic Fibrosis International Guidelines Committee recommends the following for screening and treating depression and anxiety as part of comprehensive CF care:
  1. Learning new coping skills
  2. Getting screened
  3. Getting help
• All CF centers will initiate a screening program for anxiety and depression to include annual screenings during clinic visits conducted by the clinical social worker to all patients 12 years and offered to parent caregivers of patients 0-17 years old. (Parent/family screening is optional)
• PHQ-9 to screen for depression
• GAD-7 to screen for anxiety (Quittner AL, et al., 2015).
• Results will be reviewed with you in clinic and will fall into one of three categories:
  1. Normal Screening- proceed to get annual screening
  2. Mild Range- receive supportive intervention and will be rescreened at next visit
  3. Elevated range- receive clinical assessment and psychological and/or psycho-pharmacological interventions
• Recommendations include a stepped care model for anyone who is experiencing higher than average levels of depression or anxiety
• Recommendations also include detail on the types of therapeutic interventions that have been found to be the most effective
• Our center received a grant for three years to help fund a screening and follow up program. This gives us the ability to:
  1. Hire our social worker to work full time
  2. Hire a psychologist to help with counseling needs
• Screenings will be kept confidential and will be repeated annually when “normal” or more frequently if elevated
• We will work closely with you to get additional support

Promote Emotional Wellbeing

• Foster resilience: through purpose, setting expectations, letting go and savoring the good
• Self compassion: studies show that people who have compassion for themselves are happier, more optimistic and more grateful vs. self criticism: leads to higher rates of depression and stress, with less effective coping
• Self acceptance: reduces your battle with yourself, frees you to be more peaceful and happy
• Learn mindfulness: or relaxation practices that soothe and center you
• Healthy lifestyle: Exercise, Sleep, and Eat!
• "Neurons that fire together, wire together" : Change your thoughts and you will change how you feel
• Be flexible: expect the unexpected, redefine "success" to find your happiest life
• Acknowledge fear: and develop inner strength

Cystic Fibrosis Center
Medical Center Director - Thomas Scanlin, MD

Child Health Institute of New Jersey
89 French Street
New Brunswick, NJ 08901
Appointments: 732-235-7899

Adult CF Center
125 Paterson Street. 5th Floor
New Brunswick, NJ 08901
Appointments: 732-235-7840

The following team members cover both pediatric and adult programs but are here on a limited basis.  Please feel free to reach out to them directly or request to see them during your next clinic visit. 

Weekdays 8:30 AM to 4:00 PM:

• To Reach the Adult Program - Dial 732-235-7840
• To Reach the Pediatric Program - Dial 732-235-7899

Sick Calls

For Sick Calls, please use the MAIN NUMBER and follow prompts for the nurses line during office hours, and the service during off hours. Leaving messages on a staff members personal line WILL NOT guarantee a call back.  If a nurse is unable to answer the line at that moment, please leave your name, phone number, the patient's name, date of birth and what the problem is.  A nurse will call you back as promptly as possible.  If you do not receive a call back in a timely fashion, please do not hesitate to call back and try to speak to somebody. If you need to reach a physician after hours, please follow the prompts to call the answering service.

For Prescription Renewals

Please call the above number and follow the prompts for prescription renewals. Call our staff at least 1 business day before your prescription is about to run out (longer for mail orders).  We will need to know the pharmacy telephone number, the names of the medications needed and your/your child's date of birth.  Please specify whether the prescription is for 30 or 90 days.

For Scheduling Appointments

Please call the designated program and make an appointment with the pulmonary scheduler.  Inform the scheduler that your child has CF so that you/your child gets an appropriate appointment time and day. If you need an appointment urgently or cannot get an appointment through the appointment line, please call our office and we will assist you.

Weekdays 4:30 PM to 8:30 AM and Weekends and Holidays

Please call the above numbers and the answering service will page the physician on call.  Please call only with an emergency or illness.  Non-urgent messages will be answered the next day and Prescriptions will not be REFILLED during non-business hours.

* If you have trouble getting through the phone system, please let us know so that we can rectify the problem.

* We will make every effort to call you back/ call in your prescriptions as soon as possible.  We get many calls each day and need to return the most urgent calls first.  Depending on the activities of the Center on any day, non-urgent calls may need to be returned later in the day