Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
Vascular access devices, or PICCS and ports, allow repeated and long-term access to the bloodstream for frequent or regular administration of drugs, like intravenous (IV) antibiotics.
Vascular access devices may help people with cystic fibrosis who need IV antibiotic treatments for more than just a few days, such as during an exacerbation. After a few days, regular IVs can become infected, irritate the small veins or fall out of the vein. Venous access devices, such as peripherally inserted central catheters (PICCs) are designed to be used for days, weeks or months. Implantable devices, such as ports, are designed to be used for years. They are made of material that doesn't become infected easily and will remain in place if treated with proper care. Plus, the complication rate is low.
Ports are made of flexible, strong plastic and come in many sizes. They can be split into channels (called lumens) to deliver multiple drugs, but for routine CF care, a single lumen is usually enough. For people who need them, however, double ports are available.
Although there are many different types and brands of access devices, the two main categories are PICC lines and ports. The choice of which one to use is based on how long the catheter will be left in place.
PICCs are used for short-term delivery of IV medications, usually over weeks. Ports are used for longer-term delivery of IV medications, usually over months or often years.
Although PICCs and ports have many similarities, if you need IV antibiotics to treat an exacerbation, you will likely get a PICC line placed. They are much more practical than regular IV catheters, which are often inserted in the crook of the arm or the back of the hand. A PICC can be left in place for several weeks or even months, which may be necessary for people who are on long-term antibiotics.
A PICC line is a long, very thin, flexible tube (a catheter) that is usually placed into one of the large veins in the arm (with a short portion of tube and an injection cap showing). It is secured to your skin with an adhesive device, an internal securing device or stitches. This tube is threaded into a large vein above the right side of the heart.
PICCs are often placed just above or just below the elbow. They are occasionally placed through a vein on the side of the neck or near the collar bone. You may hear the term "midline" catheter. This is a type of PICC, but it is a bit shorter and usually goes through a vein in the upper arms.
Your care team will use the PICC line to give IV medications or fluids. PICCs are intended for temporary use for people who need therapy only once or twice a year. They typically receive one round of antibiotic treatments before the PICC is removed.
Because the tube is so small and flexible, the line can last several weeks, which means fewer needle pokes and less pain. The PICC line can be flushed and capped when not in use. When it is time to give medicine, the medicine is connected to the PICC line and disconnected again when the medicine is finished.
An implanted port is a device that allows repeated and long-term access to your bloodstream. It can be used for antibiotics, IV nutrition and other IV fluids. When someone gets a port placed, they will not need IV catheters like peripheral IVs or PICC lines (unless more than one access is required).
The port is made of two parts: a medical-grade rubber catheter, which is placed in a large vein in the chest (similar to the location of the PICC catheter), and the "port" which is attached to this catheter.
Both of these parts are "implanted," that is, placed completely beneath the skin. No part of the device lies outside the skin. There is usually a small bump, about the size of a nickel. You will not need to wear a bandage after the incision is healed unless the port is being used.
Because the device is completely under the skin, it must be accessed to be used. When medications or other fluids are needed intravenously, the device is reached using a small, right-angled needle (only this type of needle should be used with the port). This fine-gauge needle is inserted through the skin into the rubber round part of the port. You might feel a small prick. If you prefer, a numbing cream can be used. The needle is connected to the IV tubing and capped.
A dressing (type of bandage) is then applied to protect the area (keep out air, dirt and germs) where the IV is inserted. The IV tubing protrudes from the dressing, allowing you to connect it to your medication. When you no longer need to use the port, the dressing and needle are removed, leaving the intact skin. A nurse, either in the hospital or in the clinic, usually accesses the port. If you like, you or a family member can learn this.
When access to the port is not needed, the port needs to be flushed once a month. This can be done by a nurse, or by you or a family member if you feel comfortable doing it.
Your care team may suggest a port if you need IV antibiotics frequently or if placing a PICC has been difficult because it is hard to locate a vein or the PICC has been painful. One advantage of having a port is that it only has to be placed once to be available for a long time when you need it (and little maintenance is required between infusions).
The advantages of having a port over having a PICC or peripheral IV is that it is a long-term device. A port lasts for many years and may be used repeatedly. When IV access is not needed it stays in place and there is less maintenance. The port does not interfere with your daily activities. It is hidden beneath the skin, with only a small bump to indicate where it is.
Choose the device that is best for you. If you need help deciding, ask the team of specialists who place implantable devices to give you information about the procedures and share their experiences. There is minor surgery involved in having a port placed, and there is a device hidden beneath your skin that stays in place, even when you do not receive any medication through it. There may be some self-consciousness about how visible the small bump will be. Ask questions of your care team, and speak to a member of the team that will actually be placing the port.
The care team responsible for your device placement will provide specific pre-procedure instructions. You will receive information about the time you should arrive for placement (if you are at home before placement).
Typical instructions include:
These lines can be placed by many medical professionals who have received special training to perform the procedure. This includes physicians, physician assistants, nurse practitioners, registered nurses or radiology technicians.
Different hospitals have different approaches and protocols for the placement of vascular access devices. They are always placed under germ-free (sterile) conditions. The skin in the area where the device will be placed will be cleaned and disinfected.
In some institutions PICCs can be placed at the bedside with local anesthesia to numb the skin. In others, providers may perform the procedure in the radiology suite. Usually your veins are located using an ultrasonography machine or a fluoroscopy machine (X-ray projected onto a TV screen). After it is placed, you'll probably have an X-ray taken to make sure the catheter is in the right place. Then the PICC is covered with a small dressing to prevent infection.
The procedure to put in a port is more invasive than the one used for a PICC but usually done as an outpatient procedure. Ports are usually placed in the radiology department or in an operating room.
Typical locations for port placement include your upper chest area just below your collarbone or inside the upper part of your arm. The team placing the port will mildly sedate you, numb the area, make an incision about 1 inch long and then place the port in a skin pocket. Once healed, there will be a bump and a small scar. Once a port is placed, you will need to care for the implantation site while the incision heals. Your care team will give you instructions about how to care for the incision and pain medication if it is necessary.
To start infusions, your team will swab an antiseptic over the port, then pierce the middle part just underneath the skin (called the "septum") with a special type of needle called a Huber needle. Once the needle is in place, you can receive medications or have blood drawn for up to a week before the needle needs to be replaced.
Peter Frey discusses the role he plays in helping use and care for his wife, Mary's, port in the following video.
You can perform most normal activities with a PICC (except swim) as long as you keep a dry dressing on the site. You can shower by covering the site with a plastic covering. You will also be advised to avoid any type of activity that might irritate or dislodge the catheter. This includes activities that require strenuous use of your arm, such as weightlifting or tennis. Your care team will show you how to keep the site dry and what activities to avoid.
Keeping the PICC line clean is a must. It is very important to prevent infection. An infection might require removal of the line. A nurse will show you how to keep your supplies sterile, so that no bacteria can enter the line and cause an infection. The dressing and cap must be changed weekly. You should wash your hands well before handling the line.
The PICC line must be flushed regularly so fluids will flow easily. If the line becomes clogged, it may have to be removed. A nurse will teach you how to flush the line and give medicines.
To properly care for your PICC line, remember:
Make sure you have information on hand about your PICC line, including:
The PICC must be flushed by injecting a small amount of germ-free saline into it. Your care team can show you how to flush the PICC.
Some people find flushing a port difficult. If so, a nurse in the hospital or even a nurse who comes to your home can help you with flushing your port. Many people want to learn to do this themselves to be as independent as possible, but must be trained to perform flushing properly to reduce the chance of infection at the site of the catheter.
Managing the upkeep of PICCs and ports, and monitoring for complications, is generally simple but should be done regularly and with the help of your care team.
When the port is new, you may experience some discomfort. This lasts usually about a week. During those first few days, look for any increase in redness, swelling, drainage or warmth around the incision on the skin. If you see unusual changes on the skin notify your CF doctor or nurse. This small incision is normally tender, as any small cut would be, and as it heals this feeling disappears. If you have stitches, they will be removed in the first week or so.
Your doctor may give you some special instructions for caring for your port when it is new. These usually include avoiding lifting your arm on the side where the port is placed and resting for the first 24 hours after the procedure. You may eat your regular diet.
There are usually no restrictions on getting the port wet, as long as the incision is healed and the port is not in use. Check with your CF care team for specific instructions.
Many patients continue to use a vest with a port in place. You can avoid irritation, which can be caused by movement of the vest, by rolling a small washcloth into a log, and wrapping it in a circle around the port while you use the vest.
The manufacturer of your vest may also provide a durable foam piece to prevent irritation while you use your vest. Ask your CF care team about how you can get a durable foam piece, or contact the manufacturer of your vest directly.
Both PICCs and ports need to be flushed with germ-free saline after an infusion of medication to prevent the catheter from getting plugged by a blood clot. Your care team will tell you how often to do this, but it is at least daily for PICCs to about monthly for ports that have not been used. If you are currently receiving IV therapy, you or your nurses will flush the line after each dose of medicine.
Your device should never be painful or uncomfortable to use. If it is, notify your care team immediately. This could be the beginning of an infection or clot. Your port will be sore for a few days after it has been inserted, but it should be free of discomfort after that.
Contact your care team if you experience any swelling, redness or puffiness around your device. This could be an early sign of an infection or a clot.
Although rare, implantable devices can become infected. Contact your care team if you have fever, chills or other signs or symptoms of infection. Contact your CF health care team if you see these other problem signs:
Insurance plans typically cover placement of a PICC or port, as well as the equipment you will need at home. However, you should check with your insurance provider to ensure that placement and equipment are covered and identify what your out-of-pocket expenses may be.
You can also contact the Cystic Fibrosis Foundation Compass, a free, personalized service that can help you with insurance, financial, legal and other issues. Dedicated Compass case managers can assist in coordinating benefits or providing information about benefits offered under your plans. Contact Compass at:
Monday - Friday, 9 a.m. - 7 p.m. ETcompass@cff.org
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