Information & Education

Topics
Airway Clearance Techniques
Bravery Bead Program
Chest X-rays (CXR) Interpretations
Pulmonary Function Testing
Requesting a Nurse Case Manager
Talking With Your Child's School About Cystic Fibrosis
Ten Tips For Family Caregivers

Transition
  What is "Transition"?
  The Steps of Transition
  Transition Tips For Parents
  Transition Tips For Teens
  Instructions for Transition Checklists
  Self-Care Checklist - What is it
  Self-Care Checklist - Download
  Transition Checklist - ages 12-15
  Transition Checklist - ages 15-16
  Transition Checklist - ages 16-18

Nutrition
  Salt Replacement
  Pancreatic Insufficiency and Enzymes


Bravery Bead Program
Your CF Center offers Connecticut Children's Medical Center's BRAVERY BEAD PROGRAM to our pediatric patients and families living with Cystic Fibrosis. The Bravery Bead Program offers children with Cystic Fibrosis opportunities to collect a different bead for each procedure or event experienced while visiting Connecticut Children's. The beads may be strung on a colorful rope that can be worn as a necklace or bracelet or displayed as a lanyard. The collection of beads represents the child's unique and personal journey throughout treatment and serves as a reminder of what the child has experienced and overcome in living each day with the challenges presented by CF.

Participation in the program is open to pediatric patients of all ages, including parents of infants and pre-schoolers who wish to begin a representation of the journey on their child's behalf. A word of caution to parents, however, to keep young children away from the beads in order to prevent swallowing!

Joanne Stevens, LCSW, Pediatric Social Worker, will introduce The Bravery Bead Program to each of you during your child's Clinic visits. Participation in the Program is optional and free of cost. The Program is sponsored by Connecticut Children's Friends. Families who wish to purchase the "Bravery Heart" to add to the necklace/lanyard/bracelet may do so at the Connecticut Children's Friends Gift Shop located on the 2nd floor. All proceeds from sales of the hearts will go to the Connecticut Children's Friends. Please contact Joanne at 860-545-9675 or at jsteven@connecticutchildrens.org with your questions, or ask to see her at your next Clinic visit!

Let the journey begin as a reminder of your child's (and your family's) resiliency in taking charge of CF!

Chest X-Rays (CXR) Interpretations
Once a year, a routine baseline chest x-ray (CXR) is performed on our CF community.  Just like the person's medical history, physical exam, and pulmonary function tests (PFTs), CXRs help to provide information that your CF care providers use in watching over you, and directing your care.  And similarly to medical history, physical exam, or PFTs, the changes in CXRs over time are most significant. 

When we look at a CXR, we evaluate several different aspects of the x-ray that relate to changes that may occur as CF progresses.  X-rays scoring systems have been developed by CF researchers to help compare x-rays between centers and over time.  At CCCFC we use the Brasfield score.  It grades 5 aspects (see below); a perfect score, absolutely normal, is assigned a score of 25.  Points are taken off as changes develop in the features assessed.  These include:

Hyperinflation - people with CF often develop mucus plugging that can cause air trapping.  When this occurs, x-rays look "hyperinflated". 

Line markings - this refers to inflammation and scarring (fibrosis) that can occur.

Nodular and cystic changes - the airways may dilate (bronchiectasis), mucus plugs increase and the lung tissue may change and become more cystic.

Large lesions - most of the time, changes are scattered.  Occasionally specific areas may become focally impacted with mucus or more consolidated as the lung tissue compresses down on itself, like a sponge being 'squished'.

Overall - this part of the score is the general 'overall' impression of the evaluator. 

Generally, scores of 23 - 25 are essentially normal.  Mild changes would be associated with scores of 18 - 22, moderate disease with 13 to 17, and a severe score would be less than 13.

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Pulmonary Function Testing
Pulmonary Function Tests (PFTs) are a way of testing lung health.  It helps your CF Team get a better picture of how you or your child is doing.  It is important to remember, however, that it is only one piece of the picture; other pieces include your medical history (the description of any symptoms and any illnesses you may have had recently), your physical examination, and other tests (such as cultures, x-rays, or labs).  Depending upon how you or your child is doing, the history, physical examination, PFTs, or other tests may carry different levels of importance.  This section will help you understand how your CF team looks at you or your child's PFTs.

First, there are the testing techniques.  For any PFT to be useful, the person with CF must be able to perform the test properly.  The testing is usually done with coaching by RTs (Respiratory Therapists), and by looking at the tests, they can tell if it was done right.  Not only does the test need to be done correctly, but also the person doing the test should be able to do the test the same way again, and again. 

The most common PFT is called 'spirometry', and we usually start asking children with CF to attempt the test around 5 years of age.  Some might be able to do it younger; many are still learning how to do it properly for two or three more years.  All breathing tests are done through a tube.  The RT will ask the person with CF to hold the tube in their mouth, with their lips around the tube.  The RT coach you or your child in how to breathe.  The key to doing spirometry testing is to take as big a breath in as you can, and then to breathe it out 'explosively', as hard and fast as possible, AND keeping exhaling until you can't breathe out any more; until there's no more air left inside anymore; then you take as big a breath in as you can, and you're done your first test.  As mentioned above, we need to be sure that the testing is consistent, so you or your child will be asked to do the test at least 3 times to be sure there is little variation.  The computer compares the value of these tests done by the person with CF, with similarly aged persons of the same size (one reason you are weighed and measured) and gender without CF, and gives the 'percent predicted value'.  Thus if you are normal, you would get 100%, if you were 10% better you would get 110%, and if you were 10% worse, you would be 90%. 

Spirometry testing has several components to it.  FVC or Forced Vital Capacity is the size of the biggest breath someone can blow out.  FEV1 is the Forced Expired Volume after 1 second of blowing out.  FEF25-75 is the Forced Expiratory Flow between the 25th to 75th percent of your breathing out.  FVC and FEV1 (as well as their ratio, FEV1/FVC) mostly reflect a person's larger airways.  Forced expiratory flows (FEFs) are usually thought to be more related to smaller airways.  FEFs often are more 'sensitive', changing up or down more easily, but are less specific, meaning when they change it may not necessarily reflect anything significant.  There has to be a bigger drop in FEFs before we say they are abnormal.   

One of the most important things about PFTs is that we can look at how a PFT changes over time, between two clinic visits, or over any period of time, revealing in some ways how a person's CF is changing.  PFTs can only be compared, however, if the person doing the PFT is able to do them correctly.  If the person's technique varies, the results vary, and we are not able to compare.  Assuming we can compare tests, what constitutes something important?  It is common for a person, with or without CF to have some minor changes, up or down, in their PFTs.  If a person's FEV1 goes up or down 5%, is that significant?  The American Thoracic Society has established certain changes as being "clinically" significant, meaning a person might feel a change in their breathing (e.g. some degree of one or more of the following: less tolerance of exercise, more tired, shortness of breath, cough, wheeze, chest tightness, or other symptoms).  We also use these numbers to determine whether a person has a significant response to a breathing treatment or other medications.

Although the above is true, because of the progressive nature of pulmonary decline in CF, CF care providers often use a lower threshold to decide if something is significant.  For example, in the research studies that looked at inhaled TOBI average FEV1 improved 10%, with Pulmozyme average FEV1 increased 7%, with chronic azithromycin it was 7%, and hypertonic saline showed 5% improvement.  Additionally, at the Central CT CF Center, we are very concerned with a 10% decrease, but also worry if PFTs drop 5%.  We want to stop any decline early.

Although spirometry (FVC, FEV1, and FEFs) are the most common PFT done, there are other testing techniques.  These include lung volumes that look at total lung capacity (TLC) and residual volume (RV).  These are more complex tests that actually measure air within the lung that a person cannot exhaled no matter how hard they try.  They can show whether a person has restriction due to scarring, or gas trapping.  Newer PFT techniques are being developed so that we may assess children younger than 5 or 6, but who are somewhat cooperative with instructions.  Finally, you may be aware or have read about 'infant pulmonary function tests'.  Infant PFTs require very specialized equipment, and are generally done as part of research studies, rather than for routine care.

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Benefits of Requesting a Nurse Case Manager for Private Health Insurance and Connecticut Husky-Medicaid

• The Nurse Case Manager serves as your advocate within the health plan, ensuring that the patient receives every possible benefit covered by the plan.
• The Nurse Case Manager serves as your point of contact for the patient and/or parent allowing him/her to deal with just one person within the health plan.
• The Nurse Case Manager will help the patient and/or parent to understand the benefits and limitations of the plan's coverage.
• The Nurse Case Manager will assist the CF Care Team with letters of medical necessity and appeals to the plan's medical review board.
• The Nurse Case Manager will help you complete forms required for coverage under the health plan.

Your Nurse Case Manager can get results for you!

Call your health insurance company and ask for the Case Management Department. Explain that you/your child requires an assigned Nurse Case Manager due to the chronicity of a CF diagnosis and the need for daily medications and care, both preventative and therapeutic. Be prepared to provide the patient's Member ID #, date of birth, full name, diagnosis and current care required.

The plan will assign a Nurse Case Manager within a few days. Request a courtesy call from your new Case Manager so you can introduce yourself and your or your child's medical needs.

Transition
What is "Transition"?
When we describe transition, we define it as a process, a series of steps involved in ending care with your pediatric healthcare providers and beginning treatment with a team of providers who specialize in the care of adults with CF. The key aspect of our Center's philosophy of transition is that we do NOT view it as simply a "transfer" of your medical records to an adult physician. No one expects you to see your Pediatric CF Team one day and then the Adult CF Team the next without preparation. We believe we have a responsibility to help you get ready to take charge of your health as one part of your overall transition to adulthood. The most important thing about transition, then, is that it is gradual, not something that happens overnight.

You will be taking charge of many areas of your life such as deciding where you want to live, go to college or what kind of work you want to do. This is the exciting part of transition: making more of your own decisions as you take on more responsibility. But as a person with an illness that requires daily attention, there are some additional tasks involved in planning for your future. Therefore, it's important to know that you, your family and your healthcare team all share the responsibility for making your transition as smooth as possible, that we will work together to get you ready one step at a time. This way, completing your move to the Adult CF Program will be a goal you look forward to reaching just like any of the important goals in your life.

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The Steps of Transition
The following is a description of the actual steps involved in moving from the Pediatric to the Adult CF Program. It is our policy that all patients 18 years old and older are seen in the Adult Program; also if hospitalization is needed, patients in this age group are admitted to Hartford Hospital.  However, the other time frames described below are approximate as no two patients are alike in their readiness to move from one step to the next. For instance, one patient may be fully independent with taking their medications at age 13, but another may not. (We have created "Transition Checklists" to use as self-assessment tools to help you review your knowledge and skills. Then you, your family and your healthcare team will all know what needs to be accomplished to get you ready for the transition. Please review the Checklists and instructions for completing them for more details.)

Another important aspect of the transition process is that we are sensitive to each family's need to attend appointments together, separately or in some combination of the two. Again, patients are not all alike and families will have different approaches to their involvement in a patient's care. We do, however, strongly encourage patients to begin learning how to take full responsibility for their health as early as possible, and part of that learning includes spending time alone with healthcare providers to practice self-advocacy.  You should feel free to discuss any questions you may have about this process with any of the CF Team members.

1. At about age 12, the Pediatric CF Team will begin discussing with you and your parents in increasing detail what you need to know about your CF and daily self-care. The Social Worker will introduce the first Transition Checklist in order to assess what you know and what you need to learn more about. (These will be completed once per year in order to review your progress.)
    
2. Around 15 years old you may meet one or more of the Adult CF Program team members during a Pediatric Clinic visit. You and your parents will complete the next Transition Checklist which is geared toward young adults. (It is also possible to tour the Adult CF Clinic prior to your first appointment there. Inquire with your CF Social Worker about how to arrange this.)
    
3. Then, once you turn 17 you will begin alternating your Clinic visits between the Pediatric and Adult clinics until you are 18. Again, the Social Worker will give you and your parents the last Transition Checklists to complete.
    
4. By age 18 you will begin attending the Adult CF Clinic only. During the previous year you will have already met with the Adult Team and determined what areas of your CF care you still need assistance with.
    
5. We recognize that just because you turn 18 DOES NOT necessarily mean you or your family feel you are ready to take on all the responsibility for your healthcare. Therefore, the Adult Team will continue to work with you and your parents on any areas you believe need more practice, information or skill.
    
6. As stated previously, it is our policy that if hospitalization is needed, those patients 18 and older will be admitted to Hartford Hospital. However, you and your parents will be given an Admission Brochure before you turn 18, and as with the Adult Clinic you may even arrange a tour of the unit well in advance of a hospital stay.

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Instructions for Transition Checklists
The purpose of the Transition Checklist is to encourage you and your family to think about what specific steps are involved in you taking more charge of your health care and in your overall "transition" to adulthood. First, by completing the Checklist, we will see what you and your parents think you already know and do by yourself. Next, we will figure out what you need to practice and which topics you need more information about. Then we can list specific things you, your family and your health care team can all do to help you feel confident in your ability to take on more responsibility. We will regularly review how well you are learning and practicing what you need to know. The "Tips for Transition" sheets include some helpful hints on the kinds of things both you and your parents can practice or think about along the way.

1. During a Clinic visit, the Social Worker will provide you and your parents each with Transition Checklists. Both you and your parents should complete the Checklists separately during the visit if time allows. (If not, you may complete them at home and mail/ bring them to Clinic.) That way we can see if you and your parents have similar or different ideas about what areas we need to focus on.
    
2. Follow the instructions at the top of the Checklist.
    
3. On the last page, the more detailed you can be in your answers the better.
4. Review them with the Social Worker.
    
5. Together, we can decide what needs work and what you already do well enough on your own.
    
6. Then, we will all have some "homework" to do. For example, you may want to practice making your own Clinic appointments, so the Social Worker can provide you with instructions as to who to call and the phone number, then your parents can follow up by asking you if you made the call.
    
7. The Checklist can be completed yearly or more often if it would be useful to do so.
    
8. Finally, keep completed Checklists in your Education Notebook or create a separate one just for transition if that's easier. Keeping them will allow you to see just how far you've come in your knowledge and skills!

It is important to remember that we can be as creative as we want to be in how the Checklists are used, how to go about "practicing" new skills or how to test your knowledge about CF. We can involve other members of the team, include other family members, just focus on what you will be doing or any other ways you can think of that you will learn best. The point is that as you get better at doing things on your own, you will be better prepared to move on in all aspects of your life and plan for a great future!

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Self-Care Checklist
What is the "Self-Care Checklist" and how to use it
Although "self-care checklist" is somewhat self-explanatory, it's worth a brief description and consideration of why it might be helpful to complete and review this tool with the CF social worker, your spouse or a friend. As you are already well aware, adults with CF have additional/ different medical and practical needs to think about. So this Checklist is a tool to use to review various areas of your life and figure out if you want more information on a particular topic and/ or for planning ahead. It may be way too soon, for instance, to consider the possibility of lung transplantation, but at least knowing where to find out more about it might be helpful.

Although this checklist is far from exhaustive, it does cover most aspects of adult CF care that need attention going forward. The instructions at the top of the form explain how to complete it using a simple self-rating scale. It may be best to complete it when you have a chance to really give it some thought, especially the last page where more detailed responses are involved. Once completed, you may want to review it yourself, with someone close to you or with the CF social worker to identify areas you may want to research or plan for. You may also want it just for reference, even if you do not fill it out. At the very least, looking it over will get you thinking about various topics and/ or prompt conversations with your CF team, spouse, family or friends.
Click here to download the Self-Care Checklist