Cystic Fibrosis: All Guides

Cystic Fibrosis (CF)

Cystic fibrosis (CF) is a genetic disease (passed down from parents to a child) that can affect many different organs in the body. More than 30,000 people are living with CF in the United States.

What is CF?

Cystic fibrosis affects a chloride channel in the body. These channels are found in a person’s lungs, pancreas, liver, sinuses, and other organs. People with CF make mucus that is extra thick and sticky. This sticky mucus can clog up a person’s lungs causing infections and trouble breathing over time. It also causes problems with digestion (processing food that is eaten).

What causes CF and who gets it?

CF is a genetic disease. This means that the disease is passed down from both parents to a child; similar to the way a person inherits the color of their eyes, hair, and skin. You need two copies of this gene to have CF, one from your mother and one from your father. If you have only one copy of the CF gene, you are a carrier; you do not have CF disease but can pass the gene to any children you have.

If two carriers of the CF gene have a child, there is a :

  • 25% (1 in 4) chance the child will have CF
  • 50% (1 in 2) chance the child will be a carrier, but will not have CF
  • 25% (1 in 4) chance the child will not be a carrier and will not have CF

What are the symptoms of CF?

CF causes the body to make thick and sticky mucus that is hard to clear from the lungs, pancreas, and other organs. This leads to lung infections and over time the lungs become damaged. In the pancreas and intestines, the thick mucus prevents the release of digestive enzymes, which may lead to poor nutrition and weight gain.

CF affects many organs and can have many different symptoms, ranging from mild to severe. Some people with CF may have only a few symptoms while others have a lot. Scientists who study CF don’t know exactly why this happens.

Symptoms and effects of CF can include:

  • Coughing
  • Lung infections, such as pneumonia or bronchitis
  • Wheezing or trouble breathing
  • Greasy, frequent bowel movements or difficult with stooling
  • Poor growth or weight gain
  • Salty tasting skin or sweat
  • Sinus infections
  • Liver disease
  • Diabetes
  • Urinary incontinence (leaking urine)
  • Puberty may start later than usual
  • Coughing during sex
  • Male infertility
  • Some women may have trouble becoming pregnant

How does someone find out they have CF?

More than 75% of people with CF are diagnosed before they are 2 years old. This is because most U.S. states screen every baby for CF when they are born. Most people reading this have been tested for CF already and probably don’t know it!

Different mutations (defects or abnormal cells) in the CF gene cause CF. There are over 1,700 different mutations that have been identified. The newborn screening process tests for the most common mutations.

If you are older and have symptoms that might be CF, you will need to have a “sweat test.” A sweat test is a painless procedure that collects a sample of your sweat and measures the amount of chloride (or salt) in it. If this test is positive, you have CF.

What is the treatment(s) for CF?

CF affects many organs, so the treatments for CF are different for each person. In general, most people with CF take medicine prescribed by their health care provider and have treatments that help break up the thick mucus in their lungs. These include inhaled medications and devices that help clear the mucus. People with CF must do these treatments several times every day to prevent long-term damage to their lungs.

About 85% of people with CF also need to take medicine that replaces the digestive enzymes that are blocked in the organ called the pancreas. These pancreatic enzyme medications must be taken every time someone with CF eats. If people with CF have other symptoms due to diabetes, liver disease, etc., they will have other medications to take every day as well.

What kind of limitations does someone with CF have?

People with CF can live very full, normal lives. There are no limitations to their exercise, diet, or activities. However, due to the different lung infections that they can get, they should not meet or talk with other patients with CF in-person. This is because they may catch new bacteria from each other than could lead to serious lung damage.

Is there a cure for CF?

Currently, there is no cure for CF. However, there has been a lot of progress in treating and managing CF, and people diagnosed with CF are living longer and healthier lives. Today, the average survival age is close to 40 as many people are living longer. In the 1950s, someone with CF usually didn’t live long enough to start kindergarten.

Because of the advances in therapy, over half of people with CF are over the age of 18 years. Many people with CF are living long enough to go to college, have a job, get married, and have and raise children.

I have a friend, sibling, or classmate with CF. How can I be supportive?

Most people with CF spend about two hours every day on medical treatments to stay healthy. Understanding that CF and these treatments are a part of their lives is important. It’s also important to realize that people with CF are just like you in most ways. You shouldn’t treat anyone differently because they have CF!

Supporting a friend can take many forms, such as going with them to appointments, spending time with them while they are having treatments, or simply giving them a safe place to talk about their feelings about CF or about anything else. Because everyone copes differently, the best way to know what someone needs is to ask!

Additional Resources:

The Cystic Fibrosis Foundation (CFF) is a large organization in the U.S. dedicated to giving all people with CF full, healthy lives. The CFF website has many resources for people with CF and their families and friends:

 

Cystic Fibrosis: Puberty and Menstrual Periods

Puberty

Puberty is the name for the time when your body begins to change and you go from being a child to an adult. Your hormone levels will change and you will develop breasts, grow taller, and start your menstrual periods. Puberty in girls usually starts between 8 and 13 years of age. Girls who have cystic fibrosis (CF) may start puberty later than girls who do not have CF. In fact, research shows that girls who have CF and live in the United States (US) begin breast development and get their first menstrual period about 6 months later on average.

Physical changes: Girls with CF go through all the same changes that happen to all girls during puberty. Breast development is often the first sign that puberty has begun. You will also start to grow hair around your pubic area and under your arms. Your skin may be more active during puberty, which can cause acne and body odor. Your bones will become stronger and double in density (thickness and strength).

Growth spurt: Most girls have a growth spurt the year before they have their first menstrual period. In addition to getting taller, your hips will get wider.

Vaginal discharge: Most girls notice vaginal discharge, or a thin yellow or white stain on their underpants, as they go through puberty. This is a normal fluid that helps clean and moisten your vagina. Some girls with CF have said that their vaginal discharge is thicker and can be affected by their CF medicines. These changes are normal. However, itching, burning, irritation around your vagina and/or a new discharge (often thick & white) could mean that you have a yeast infection, especially if you are on antibiotics for your CF. If you have one or more of these symptoms, talk to your CF provider as soon as possible.

Nutrition and having a healthy body weight is very important during puberty. Getting enough calories is important for breast development, growing taller, making strong bones, and getting regular menstrual periods. A lot of girls get their first period when they weigh at least 100 pounds. Nutrition is also very important for your CF health. It’s especially important to continue to eat a high-calorie, high-fat diet, take vitamins, and exercise to manage your CF and stay healthy during puberty. Feeling good about yourself and having a good body image is important for all girls.

Menstrual Periods

  • Most girls get their first period about 2 – 2 ½ years after they begin to develop breasts. The average age that girls have their first period is between 12-13 years old, but some girls start as early as age 9, and others as late as 15. Girls with CF may be slightly older when they get their first period. On average, American girls with CF report getting their period about 6 months later than girls without CF. However, if you are very ill or underweight, your first period may be delayed longer.
  • It can take a couple of years for periods to become regular (with or without CF). Most girls find that their menstrual cycles last anywhere from 21-35 days (counting from the first day of your period to the first day of your next period) and bleeding can last from 3 to 7 days. The amount of blood flow will likely be different each day. Typically, you will have heavier bleeding in the beginning of your period and a lighter flow towards the end. If you have CF, your periods and cycles can change when you are sick.
  • It’s a good idea to use a calendar or a phone app to keep track of when your periods start and stop. If your periods are irregular, you can share this information with both your CF provider and primary care provider (PCP).
  • If you are 15 or older and haven’t had your first period yet or it’s been 3 or more years since you started your breast development, tell your CF provider or your primary care provider who can check that you are on track.

Some girls with CF notice worsening of their CF symptoms during their period. This may be due to changes in the hormone levels that your body makes during puberty. You may cough more, have more sputum, or have other signs of an exacerbation of your CF. It is especially important to do all your CF treatments and airway clearance during this time of your cycle.  If you notice that your CF symptoms get worse with your periods, talk with your CF provider. Some girls have found that starting a type of birth control that regulates your menstrual cycle (and hormone levels) is helpful with controlling these symptoms.

Cystic Fibrosis: What Should I Know About Sex?

You may be thinking about what it means to be involved in a sexual relationship. It’s normal to think about sex and have sexual feelings. Having CF has no impact on your sexual orientation or gender identity.

Whether you have CF or not, you can have vaginal, anal, or oral sex. However, there are many things that are important to think about before you decide to have sex, including whether this is what you want, whether this is the right time in your life, and how to prevent sexually transmitted infections and unwanted pregnancies.

Click here to take a quiz to see if you are ready for sex.

You should never feel pressured to have sex. Sexual consent means there is a clear agreement by all participants for any type of sexual encounter for that one time. It’s important to have sexual consent every time, not just the first time. Sexual consent is not just about vaginal, anal, or oral sex, it also includes kissing, making out, cuddling, or touching.

Sex is an important part of life for many people. CF does not decrease your desire to have sex or the need to be intimate with your partner. You can absolutely still enjoy vaginal, anal, or oral sex if you have CF. However, whether or not you have CF, you may have certain concerns. For example: you may have trouble becoming aroused (getting “turned on”), lubricated (getting “wet”), or having an orgasm. If you are experiencing any of these problems, it’s always important to be able to talk to your partner and communicate about what feels good.

Frequently Asked Questions

Do people with CF have any symptoms when they have sex?

Coughing

For people with CF, coughing during or after sex is very common. In a recent survey, about 1 in 3 young women with CF reported coughing during or after sex more than half the time. A small number of people with CF also reported coughing up blood during or after sex. If you have any of these symptoms, you are not alone—other people with CF have experienced the same thing!

Shortness of breath (SOB)

Some people with CF, especially those who have some severe lung disease, can have trouble breathing during or after sex. During sexual activity your heart rate increases, just like it does when you exercise. If you have become short of breath when you work out, you may have the same trouble during sex. Generally, if you feel fine after climbing two flights of stairs, you are physically fit enough to have sex and do not worry about being short of breath. If you need oxygen when you exercise or any other time during the day, you should wear your oxygen during sex.

Practical tips to help you enjoy sex when you have CF:

  • Take your time, take breaks, and don’t be afraid to tell your partner if you are having symptoms.
  • Use your short-acting bronchodilator (your albuterol or Ventolin®) 20 to 30 minutes before you have sex. This may help open up your airways and prevent symptoms.
  • Do your airway clearance before you have sex to clear mucus and help you breathe easier.
  • Avoid anything that makes you cough or triggers issues with breathing such as using or being around strong perfumes or cigarettes.
  • Try sexual positions that require less energy and do not put pressure on your chest.
  • Use pillows under your back and talk with your partner about them playing a more active role during sex.

Why does sex hurt sometimes?

There are different reasons why sex might be uncomfortable or hurt sometimes. Don’t try to tough it out. Pain is the body’s signal that something isn’t right! Here are a few reasons why sex may hurt:

  • Vaginal dryness: Sometimes sex can be painful because the inside of your vagina is dry and can cause irritation and/or pain. You may have noticed that your vagina makes its own lubricant (fluid), and your body may make more of it during times when you’re feeling aroused or “turned on.” Taking things slowly and not rushing to have a sexual encounter before you’re ready can help give your vagina a chance to make more of that fluid which will likely make the experience more comfortable for you.
  • Yeast infections: If you notice that you feel pain or burning when you’re having sex, you could have a yeast infection. Yeast infections are very common in women with CF and can be treated with medications.
  • Allergy to latex or spermicides: Some people are allergic or sensitive to latex (which is what most condoms or dental dams are made of) and/or spermicides (the gel that kills sperm). If you are allergic or sensitive, symptoms may include pain, burning, frequent urination, rash in or around the vaginal area and/or a vaginal discharge that is different for you. Talk to your provider about your symptoms.
  • Sexually Transmitted Infections: If you are sexually active and notice a vaginal odor or discharge that isn’t normal for you, pain when you urinate (pee), or itching in your vaginal area, talk to your CF team and make an appointment and get checked.
  • Vulvodynia: Vulvodynia is a treatable condition that causes discomfort around the opening of the vagina and can make sexual intercourse painful that has no known cause. Only a health care provider can tell if you have this condition.

What should I know about lube?

“Lube,” short for lubricant, can help make sex comfortable and water-based lubricant can help prevent condoms from breaking. Most pharmacies sell lube right next to the condoms – look for something simple. Do not use flavored lube, or lube that claims to be warming or numbing as they can be irritating. Most latex condoms come with a small amount of lube, however, you may choose to use more. Some lube contains glycerin, a type of sugar, which can increase the chance of getting a vaginal yeast infection. If you get a lot of yeast infections as someone with CF, try choose lube that does not contain glycerin.

NEVER use oil, lotion, or petroleum jelly (Vaseline) for lubrication because these products can cause the condom to break! Remember, if you’re having sex, always use a condom for vaginal sex or a dental dam for oral sex to help prevent STIs.

What if I’m allergic to latex?

Some people can be allergic or sensitive to latex, which is what most condoms and dental dams are made of. Symptoms can include soreness, itchiness and/or rash in or around the vaginal area. Try using a polyurethane condom (including the female condom) or a silicone dental dam and see if that helps.

What if I’m allergic to spermicides?

Some people are allergic to spermicides, while others have some irritation in or around their vagina, vulva, or penis when they use them. Nonoxynol-9 is the only active chemical in spermicides made in the United States. Women/men who are truly allergic to nonoxynol-9 should not use any birth control method that contains spermicide with this ingredient. Talk to your health care provider to see if there is another spermicide you can use.

How do I talk with my partner about my symptoms during sex?

Being able to talk with your partner about being intimate or having sex is important in any relationship. Tell your partner what feels good and what doesn’t. Try to be open and honest if you are having symptoms. If you always cough during sexual encounters, tell your partner beforehand and talk about how common that is in CF. Tell your partner you may need breaks if you are short of breath and may need to slow things down. Be sure to tell your partner if you are having any pain or soreness. If you have a G tube, a port, or a PICC line in place for a round of antibiotics, it’s a good idea to talk about that with your partner.

You may be embarrassed to talk about these things and it’s ok to feel that way, but these conversations are important. Just remember that your partner should be supportive and want to help you enjoy a sexual experience. Many people with CF struggle with how to talk with their sexual partners and significant others about CF. Talk to your CF team if you are having trouble finding a good way to start this conversation.

Some people find it helpful to talk with their parents or family members about the decision to have sex.  Family can be a great support for getting sexual health care and talking about relationships. Other people chose to make decisions by talking with other trusted adults, including someone on their CF team or primary care doctor.

What about confidentiality? I don’t want my parents to know that I’m having sex.

What you tell your health care provider or CF team about your sexual behavior is confidential. It’s okay to ask your parents or family members to step out of the room for parts of your visit. By law, your medical providers cannot talk about this information to anyone else unless he/she seriously believes there is danger to you or to others, or that you are not able to make safe decisions on your own. This means your parents, teachers, partners, or friends can’t find out any information from any of your medical providers or CF team about anything, including if you are having sex. Although your medical providers do their very best to keep your information private, there is a chance your health insurance company or pharmacy may send information to your home. You may find it helpful to talk to your parents about your health and your worries.

Talk with your CF team if you are experiencing any symptoms during sex. Although it may be embarrassing to start a conversation with your health care providers about this, it gets easier and they can help you think about options for treatment and help you manage your symptoms.

Here are some specific talking points and tips that may help you discuss sexual health with your medical team:

  • Tell your CF team if you are having symptoms or if you are worried about having any symptoms during sex.
    • Example 1: “During sex, I sometimes cough/have shortness of breath/cough up blood.”
    • Example 2: “Sex is sometimes painful for me. I worry about having [describe your symptoms] during sex.”
  • Tell your CF team if you are having trouble talking with your sexual partner(s). Ask for suggestions on how to bring certain issues up with your partner.
    • Example: “It’s hard for me to talk to my partner(s) about symptoms I’m having or I might have during sex.  Do you have any guidance about how to have that conversation?”
  • Ask your CF team if you should be referred to see a specialist.
    • Example: “What can I do to help with my symptoms during sex? Should I see a specialist to help with these symptoms?”

There is a lot of information to consider when you have CF and start having sex. You can print this guide or have your provider refer to this webpage during your visit.

Cystic Fibrosis: Vaginal Yeast Infections

Vaginal yeast infections are very common. About 75% of all women (with or without CF) get at least one yeast infection during their lives. About half of 15-24 year old women with CF have had a yeast infection. Yeast infections can happen at any age and some women experience many yeast infections over their lifetime.

What causes yeast infections in women with CF?

Yeast infections are typically caused by an overgrowth of a type of fungus called Candida (also called yeast). Yeast is normally found in many parts of your body, including your mouth, digestive tract and vagina. A vaginal yeast infection occurs when there is an overgrowth of yeast. There are many reasons why the level of yeast becomes too high.

In women with CF, one of the most common reasons for vaginal yeast infections is the use of antibiotics. Antibiotics kill normal bacteria in your vagina and allow the yeast to grow. You are most likely to get a yeast infection after puberty, around the time of your menstrual period, or when you start or change antibiotics.

Other causes for a vaginal yeast infection include: use of steroid medication, diabetes, pregnancy, being overweight, HIV infection, use of contraceptives, or wearing tight underwear made of material that traps moisture and heat (such as silk or rayon).

What are the signs and symptoms of a vaginal yeast infection in women with CF?

Women with CF experience the same symptoms as any woman with a vaginal yeast infection. The most common symptoms are itching, burning and/or irritation in and around the vagina and a change in your vaginal discharge. When you have a yeast infection, your vaginal discharge may become thick and white, but it usually does not have an odor. Sex may be painful and you may feel sore when you pee. If you are having any of these symptoms, you should talk with your CF team. It’s important to be evaluated for a yeast infection and/or other possible causes of your symptoms as well.

How is a vaginal yeast infection treated when you have CF?

Yeast infections can be treated with a pill you swallow or with a vaginal cream or suppository (a pill that you insert in your vagina where it dissolves and releases medicine). Your health care provider can explain what the choices are and help you decide which one is best for you. Here are a few things to remember:

  • You should NOT use tampons if you are being treated with a vaginal cream because the tampon will absorb the medication and make it less effective.
  • Some vaginal creams or suppositories are oil-based and may weaken latex condoms and diaphragms, so they are more likely to break. You should talk to your health care provider about whether to use a polyurethane condom or not have sex during your treatment.
  • Some anti-yeast medications are available over the counter in pharmacies (you don’t need a prescription). Do not use anti-yeast medications without seeing your CF team, unless you’ve been diagnosed with a yeast infection more than once and you’re really sure of the symptoms and signs. Anti-yeast medications will not treat other vaginal infections, such as bacterial vaginosis, which requires a different prescription medicine.
  • If you take Orkambi or Kalydeco and have a vaginal yeast infection, make sure you talk with your CF team before starting any treatment. Many anti-yeast medications may not work when taken with these medications. You may also need to decrease your dose of Kalydeco while you are being treated for your yeast infection.
Vaginal yeast infections can be treated, but even with treatment, they can return or not improve completely. If you have CF, you may need to take your anti-yeast medication for a longer amount of time. If you often get yeast infections when you take antibiotics, you may be able to start anti-yeast medication at the same time that you begin your antibiotics.

How can I prevent getting a vaginal yeast infection? 

You can lower your risk of getting a yeast infection by:

  • Never using vaginal douche products
  • Wearing cotton underwear and loose fitting pants
  • Changing out of a wet bathing suit or workout clothes as soon as you can

Some women with CF find that using probiotics or eating one cup of yogurt (which contains acidophilus bacteria) a day is helpful in preventing yeast infections.

It’s important to realize that doing these things alone will NOT necessarily cure or prevent vaginal yeast infections. If you have to take antibiotics on a regular basis and you are getting a lot of yeast infections, talk to your CF team about using an anti-yeast medication every time you have to take antibiotics.

It may be hard to start a conversation with your CF team about sexual and reproductive health. Here are some specific talking points and tips that may help you discuss vaginal yeast infections with your provider(s):

  • Tell your team what your symptoms are. CF providers are all familiar with yeast infections because they happen so often in women with CF. Do not be embarrassed to say that you are having symptoms and state that you are worried that it might be a yeast infection.
    • Example: “I have vaginal itching/my vaginal discharge is thicker and white (or whatever your symptoms may be). I am worried that I have a yeast infection.”
  • Remind your CF team if you take Orkambi or Kalydeco. These medications can have interactions with certain anti-yeast medications. You may need to avoid certain medicines or change your medication doses.
    • Example: “I take Orkambi/Kalydeco. I know this medicine may have an interaction with certain types of medication used to treat a yeast infection. Are there medicines I should not use? Should my dose of Orkambi/Kalydeco change?”
  • If you get vaginal yeast infections often, ask your CF team if you should take anti-yeast medication to try to prevent infections from happening.
    • Example: “I get vaginal yeast infections a lot when I am on antibiotics. Should I be using an anti-yeast medication to try to prevent this from happening?”

There is a lot of information to think about when you have CF and get a vaginal yeast infection. You can print out this guide or have your provider review this webpage during your visit.

More information on CF and yeast infections can be found on the Cystic Fibrosis Foundation (CFF) website: www.cff.org.

 

Cystic Fibrosis: Talking with Your CF Team about Sexual and Reproductive Health

Many people with CF have known their CF team or doctor since they were a baby. This may make it easier for you to talk about your sexual and reproductive health or you may still feel embarrassed. Either way your CF providers care about you and your health and will try to answer any questions you have as well as discuss options for treatment if you need it. Read on to learn about helpful ways to talk about sexual health with your CF team.

Why should I be concerned about my sexual health?

CF can have unique effects on a woman’s sexual health such as:

  • delayed puberty
  • worsening of CF symptoms during your menstrual cycle
  • urinary incontinence
  • vaginal yeast infections
  • coughing during sex
  • special considerations around what type of contraception to use
  • some CF medications may interact with your birth control method and medicines used to treat vaginal yeast infections and STI’s.
  • fertility
  • pregnancy

Each member of your CF team is an expert in CF. They can help you understand issues related to your sexual and reproductive health and give you resources and/or referrals for specialized care if needed. They also know you and your medical history and medications better than any other health care provider.

Are my conversations about sexual and reproductive health confidential?

It’s important to remember that your CF team is your medical provider, not your parent’s or family’s. What you tell your CF team about your sexual behavior is confidential. Your provider(s) will likely offer you time to talk without your parents present but, if they forget, it’s okay to ask. It’s also okay to ask your family members or partner to give you time alone with your doctor to discuss these topics. By law, your medical providers cannot talk about this information to anyone else unless they believe there is danger to you or to others, or that you are not able to make safe decisions on your own. This means your parents, teachers, partners, or friends can’t find out any information from your CF team about anything, including if you are having sex, unless you give your permission. Although your CF team does their very best to keep your information completely private, there is a chance your health insurance company or pharmacy may send information to your home. Talk with your CF team if you are worried about this.

Another option might be to get your sexual health care at a clinic that offers free or low-cost care without billing your insurance company (such as a Planned Parenthood or a local family planning clinic). If you choose to seek care with another women’s health provider, make sure to tell them you have CF and may have some disease-specific sexual and reproductive health issues that they should know about.

How should I start a conversation about sexual and reproductive health with my CF team?

Know that your CF providers are there to help.

Your CF team talks with patients about things that may seem embarrassing or personal all the time. Your questions are not going to surprise them and they have probably heard the same concerns from other patients before. It is part of their job to take care of the “whole” you affected by CF! Knowing about your sexual and reproductive health can help them provide better care for you.

Example: You could start by saying, “I feel embarrassed about what I’m going to tell you, but I need your advice.”

If you feel embarrassed to ask questions out loud, you could bring a list of questions to your provider. You could also call your team and ask your questions over the phone or email your questions/concerns to your CF team before your scheduled clinic appointment.

Be direct.

If you have a problem that needs immediate attention, it’s best not to wait too long to bring it up. You don’t want to walk away from the conversation feeling like you missed an opportunity to get your questions answered. Also, by asking a direct question, you’re more likely to get a clear and direct answer, which is usually much easier to understand. Don’t leave without getting answers to all your questions. Also, it might be a good idea to review the treatment plan with your provider before you leave the office, to make sure you understand what you need to do.

Example: “You would like me to _______________, right?”

Think about talking to another member of the CF team or another health care provider.

You have many different people on your CF team that you can talk to, including your doctor, nurse, social worker, dietitian and others. You may be more comfortable talking to a particular member of the team about sexual and reproductive health issues. If you talk to one of your providers and you don’t feel like they answered your entire question, you may find it helpful to talk to someone else on the team or your primary care doctor. You can also ask your provider to address the issue again or ask if there’s a specialist you can speak to.

Cystic Fibrosis: Talking with Your Partner about CF and Sexual and Reproductive Health

Talking about CF

Being able to communicate with your partner is an important part of any relationship. When you have CF, your treatments, airway clearance, and exercise all take up a lot of time. This makes it especially important to talk with your partner about the details that are involved in your care in order for you to stay healthy. Also, being able to tell your partner how you are feeling is a way to build trust and enhance your relationship.

Explaining CF to someone who doesn’t know a lot about it may be frustrating and tiring at times. One thing you can do to help others understand the details of your CF and your medical needs is to use resources on this website or at http://www.cff.org. You can also bring your partner to your CF clinic appointment(s) to see what your visits are all about and introduce them to your CF team.

Talking about Sexual and Reproductive Health

CF can have a variety of effects on the sexual and reproductive health of women, such as delayed puberty, more coughing or difficulty breathing during the menstrual cycle, urinary incontinence, yeast infections, coughing during sex, and special considerations around what type of contraception to use, fertility, and pregnancy. If you are experiencing any of these issues or are worried about anything else, you should feel comfortable sharing these concerns with your partner.

It’s normal to feel embarrassed to talk about these things, however, these conversations are important to have, and with time, it will get easier. Here are some examples:

  1. Try telling your partner if your CF symptoms get worse during certain times of your menstrual cycle.
    • “Sometimes during certain times of the month, I cough more and may feel tired.”
  2. Talk about what feels good and what doesn’t if you are having sex.
    • “This position really makes it hard for me to breathe and makes me cough more. Can we try something else instead?”
  3. Try to be open and honest if you are having symptoms so your partner can be supportive.
    • “I am going to try to tell you when I’m feeling well enough to have sex or when I’m feeling like I a need a break, so sex is more fun for the both of us.”

Having a conversation about preventing sexually transmitted infections and/or pregnancy (if you are having sex with a male partner) is important for everyone, regardless of whether or not you have CF. Talking to your partner about condoms or dental dams may seem awkward at first. Try your best to be open with your partner, as it will be easier to discuss things such as how to prevent sexually transmitted infections. Your partner cares about you and should not have a problem agreeing to use protection every time you have sex. Making a plan to use protection and both partners following through when you have sex is an important part of being in a sexual relationship.

If you are at a point where you are thinking about pregnancy or having a child, it is important to have an honest conversation with your partner about how CF affects that decision. This conversation is also an opportunity to answer questions your partner may have and a way for you to express your feelings and concerns about starting a family. Your partner will likely be grateful for the information as it will help him/her better support you, which they may or may not know how to do without your guidance. It is also important to think about how you will balance caring for yourself and caring for a child. At different times you will need extra help from your partner and support network in order to stay healthy and provide child care. Many people with CF struggle with how to talk with their partners about these issues. Your CF team can help.

Cystic Fibrosis: Contraception

Women with CF are able to get pregnant. An unplanned pregnancy can have a big effect on your health. Some CF medications are “teratogenic,” meaning that they can cause birth defects, so it is very important to prevent pregnancy if you are taking these medications. Whether or not you have CF, if you have sex, you definitely need to think about how to prevent getting pregnant and how to protect yourself from sexually transmitted infections (STIs). There are many different types of contraceptives or birth control you and/or your partner can choose from. Hormonal contraceptives can also be used for other reasons such as irregular periods, menstrual cramps, or acne. Some women report that using a hormonal form of contraception may help with CF symptoms that are worse during their menstrual cycle.

Women with CF have special things to consider when choosing contraception.

What do I need to know about contraception and CF?

There are many different types of contraceptives. They vary in how effective they are at preventing pregnancy, how much they cost, how easy they are to use, and whether they also protect against STIs.  The table below gives some key facts about contraceptives and CF. All of these methods have different success and failure rates.

  • All women, with or without CF, should think about the pros and cons of each contraceptive method, before deciding on the right one for them.
  • It’s important to choose a contraceptive method that is effective, but also fits with what you want and need. It’s a good idea to talk to your partner, parent(s), guardian, or a trusted adult, and your CF provider if you are thinking about having a sexual relationship. Talking about contraception and getting all of your questions answered will help you figure out the best contraceptive choice for you.
MethodSuccess Rate with Typical UseSummaryKey facts for CF
Hormonal implants

 

 

99%
  • Long-term method of birth control (protects against pregnancy for 3 years after insertion–it can be removed by a health care provider when you want to or you can wait for 3 years when it’s time for a change of implant)
  • May cause light or no menstrual periods or irregular menses
  • Requires minor surgery to insert the tiny rod(s) underneath the skin and to remove the device
  • Safe and effective for use by women with CF
  • Lumacaftor/ivacaftor (Orkambi), may make this method less effective. If you are taking Orkambi, non-hormonal contraceptive methods are recommended.
Intra-uterine Device (IUD)99%
  • Long-term method of birth control that protects against pregnancy as long as it is in place in your uterus (anywhere from 3 to 10 years depending on the type of IUD)
  • Needs to be inserted by a healthcare provider
  • Hormonal IUDs lessen menstrual flow and can be used to treat heavy periods
  • Copper IUDs can have side effects such as menstrual cramping, longer and/or heavier menstrual periods, and spotting between menstrual periods
  • Can fall out or can rarely puncture the uterus
  • Can be removed at any time and you can get pregnant right after removal
  • Lumacaftor/ivacaftor (Orkambi), may make hormonal IUDs less effective. If you are taking Orkambi, non-hormonal contraceptive methods are recommended.
  • Copper IUDs are not affected by Orkambi and are a very effective form of contraception.
Tubal Ligation (Female Sterilization)99%
  • Permanent (although it is possible to undo sterilization with major surgery, it’s not always successful)
  • Requires minor surgery
  • Only should be used by women who are absolutely sure that they do not want any or any more children
  • This method of birth control is permanent.
  • Essure, a form of tubal ligation in which coils are placed in the fallopian tubes, may not be effective if you are taking steroids such as prednisone.
Depo-Provera ® Hormonal Injection94%
  • Each injection provides 3 months of protection against pregnancy
  • Need to see your health care provider every 3 months for an injection
  • Many women stop getting their menstrual period while getting injections. (This is not a medical problem and menstrual periods usually return 6-18 months after you stop taking injections)
  • Generally NOT recommended in CF as it may cause loss of bone density and increase the risk of low bone mass, such as osteopenia or osteoporosis, in women with CF.
  • Lumacaftor/ivacaftor (Orkambi) may make hormonal contraceptives less effective. If you are taking Orkambi, non-hormonal contraceptive methods are recommended.
Birth Control Pills91%

 

Perfect Use

99%

  • Makes menstrual periods more regular and lighter
  • Decreases menstrual cramps and acne
  • Need to remember to take every day at the same time
  • Birth control pills have to be taken at the same time every day and may add on to your CF care routine.
  • If you are pancreatic insufficient, not taking your enzymes as directed can make birth control pills less effective. If you have major issues with malabsorption, birth control pills may also not be as effective.
  • Lumacaftor/ivacaftor (Orkambi), may make hormonal contraceptives less effective. If you are taking Orkambi, non-hormonal contraceptive methods are recommended.
  • Some antibiotics (i.e., rifampin) and other drugs used to treat HIV, certain anti-seizure and antifungal medicine, and some herbal medicine will make hormonal contraceptive methods less effective. A second contraceptive method, such as condoms, should be used to prevent pregnancy while you are taking these medications.
  • Hormonal birth control with estrogen may increase your risk of blood clots and may not be a good option if you have CF-related liver disease or have an implanted device such as a port.
Hormone Patch (Ortho-Evra) (Xulane)91%

 

Perfect Use

99%

  • Makes menstrual periods more regular and lighter
  • Decreases menstrual cramps and acne
  • Lumacaftor/ivacaftor (Orkambi) may make hormonal contraceptives less effective. If you are taking Orkambi, non-hormonal contraceptive methods are recommended.
  • Some antibiotics (i.e., rifampin) and other drugs used to treat HIV, certain anti-seizure and antifungal medicine, and some herbal medicine will make hormonal contraceptive methods less effective. A second contraceptive method, such as condoms, should be used to prevent pregnancy while you are taking these medications.
  • Hormonal birth control with estrogen may increase your risk of blood clots and may not be a good option if you have CF-related liver disease or have an implanted device such as a port.
Vaginal Hormonal Ring (Nuva-Ring)91%

 

Perfect Use

99%

  • Makes menstrual periods more regular and lighter
  • Decreases menstrual cramps and acne
  • Lumacaftor/ivacaftor (Orkambi), makes hormonal contraceptives less effective. If you are taking Orkambi, other contraceptive methods are recommended.
  • Some antibiotics (i.e., rifampin) and other drugs used to treat HIV, certain anti-seizure and antifungal medicine, and some herbal medicine will make hormonal contraceptive methods less effective. A second contraceptive method, such as condoms, should be used to prevent pregnancy while you are taking these medications.
  • Hormonal birth control with estrogen may increase your risk of blood clots and may not be a good option if you have CF-related liver disease or have an implanted device such as a port.
Emergency Contraception (EC) 89%
  • Backup method of birth control for preventing pregnancy after unprotected sex.
  • Even though it’s commonly called the “morning-after pill,” there is both a pill and an intrauterine device that can be used within 5 days (120 hours) of unprotected intercourse.
  • The sooner you take it, the more likely it will work.
  • Side effects are usually mild and may include nausea and irregular periods.
  • This is a good back-up method to always have available if you have any issues with your regular contraception.
  • In the United States any woman can get EC at most pharmacies without a prescription. If you want to use an intrauterine device as EC, you will need to be seen at a clinic to have the device inserted.
  • Safe and effective for all women with CF.
  • If you are pancreatic insufficient not taking your enzymes as directed can make the pill form of EC less effective.
  • You can use the EC website to find a health care provider or pharmacy if you have questions about emergency contraception or talk with your PCP, gynecologist, or CF team.
* fertility awareness based methods (FAB), and the lactational amenorrhea method (LAM) are not known to be affected by CF.

Barrier methods can also be used to protect against pregnancy. Types of barrier methods include the male condom, female condom, diaphragm, cervical cap, spermicide, and contraceptive sponge. With typical use, the effectiveness of these methods ranges from 72-88%.  CF is not known to affect barrier methods of contraception.

Does contraception protect against sexually transmitted infections (STIs)? 

It’s very important to remember that most contraceptives do not protect against STIs. Apart from practicing abstinence or not having sex, only certain barrier methods, such as condoms used every time you have sex, can lower your risk of getting STIs! Types of barrier methods that protect against STIs include the male condom, female condom, and dental dams (for mouth to genital or anal contact).

What about CF medicines and contraception?

  • If you are taking lumacaftor/ivacaftor (Orkambi), it is recommended that you do NOT use a hormonal method of contraception. You can use a copper intra-uterine device (Paragard IUD), barrier methods, or abstinence.  The method you choose should be very effective because the effects of Orkambi during pregnancy have not been studied.
  • Common CF medications, such as Cayston, Tobi, Pulmozyme, and Hypertonic Saline, are not known to interact with any forms of contraception.
  • If you are participating in a study for a new medication for CF, you may have additional rules around the type of contraception you can take.
When you have CF, there is a lot to consider when choosing the best contraceptive method for you. If you are or planning to become sexually active, make an appointment with your CF team and/or a women’s health doctor (such as a gynecologist or an adolescent medicine specialist) to talk about your birth control options.

Here are some specific talking points and tips that may help you discuss contraception with your CF team:

  1. Tell your team that you are having sex or are thinking of having sex and that you are interested in starting birth control.

Example: “I am thinking about/have started having sex with my partner.  I want to protect myself from getting pregnant and from STIs.”

  1. You can also be direct and just say that you need a form of contraception.

Example: “I need a form of birth control/contraception.”

  1. Remind your CF team if you take Orkambi or are often prescribed any inhaled or oral antibiotics (especially rifampin). These medications can have interactions with certain types of birth control.

Example: “I take Orkambi. /You often prescribe me rifampin for my exacerbations./etc.  I know this medication may have an interaction with certain types of birth control. What do you recommend?”

There is a lot of information to remember and consider when choosing a type of birth control. You can print out this guide or have your provider pull up the webpage during your visit. More information on CF and contraception can be found on the Cystic Fibrosis Foundation (CFF) website: www.cff.org.

 

Cystic Fibrosis: Urinary Incontinence

Urinary incontinence (UI) means that you leak urine when you don’t mean to. When you have CF, your pelvic floor muscles (the muscles in your groin that control your bladder) may become weaker from frequent coughing.  If this happens, you may not always be able to control when you urinate or pee.

Who gets urinary incontinence (UI)?

Anyone can have urinary incontinence or UI. However, it is a common issue for people with CF.

About 1 in 6 teen and young adult women with CF have experienced UI at some point in their lives. UI can happen at any age, but usually becomes more common as you get older or after you have a baby. Men with CF can also have UI, although it is not as common.

Just because you have CF does not mean you will have UI!  However, it is something to be aware of in case it happens to you.

What triggers UI?

The most common things that trigger UI are coughing, laughing, exercising, sneezing, and movements such as lifting, bending, sitting down, turning, or standing up. Some women with CF say that UI also happens when they do their airway clearance or use their vest.

It is very important that you continue to do your airway clearance EVEN if it causes UI! Airway clearance helps clear your mucus and keeps your lungs healthy.  If you are having UI during your treatments, talk with your CF team to see what treatment is available for your UI symptoms!

What are the symptoms of UI?

The symptoms of UI can range from mild to more severe. UI can happen only occasionally or can occur several times a day. Some women leak just a few drops of urine and some have larger leaks that soak through their clothes. UI may also happen during or after sexual activity. If you have a CF exacerbation and are coughing more, you may have more UI. Some women who have UI also have fecal incontinence, or leak stool when they do not mean to.

What can make UI worse in people with CF?

A few other conditions may worsen UI in people with CF. If you have UI, your CF team should check to make sure the following conditions are not causing your symptoms.

  • Urinary tract infections (UTIs): UTIs happen when bacteria get inside the bladder. Common symptoms include: pain or burning when you urinate (pee), feeling the need to pass urine more frequently, and urinating very little even though you feel like your bladder is full. Sometimes you may have a UTI and not have any symptoms. UTIs are usually diagnosed by a urine test that checks for bacteria. You will be asked to pee in a cup and your urine sample will be tested for signs of an infection. The sample will then be sent for further testing. If bacteria are found, the lab will inform your provider of the name of bacteria that is causing the infection as well as what antibiotic(s) will treat the infection.
  • CF-related diabetes (CFRD): Untreated or poorly controlled CFRD can sometimes make UI worse because of increased urination. Talk with your CF team if you have UI about whether you should be screened for CFRD or if your treatment regimen should be changed.
  • Constipation: Constipation often makes UI worse. If you have pain when you have a bowel movement, strain, or don’t go very often, you may be constipated. Talk with your CF team about constipation. There are many medications and treatments that are available that can help with constipation and may improve symptoms of UI.

What is the treatment for UI?

There are different treatment options for UI when you have CF. Talk with your CF team to see what treatment is right for you. Even though UI is common in people with CF, it is not something that you have to live with.

  • Exercises that strengthen your pelvic floor muscles

If your UI is caused by weak muscles that control your bladder, you can learn to do exercises to make these muscles stronger.

One type of exercise is called Kegels (pronounced: KEY-GULS).  Kegels are easy to do and can be done anywhere at any time without anyone even knowing! Here are a few easy steps to learn how to do them:

  1. First, while you are passing urine (peeing), find the muscles that start and stop the flow of urine.
  2. Next, squeeze these muscles for 3 seconds and then relax for 3 seconds. Try not to tighten your stomach or thighs when you do this.
  3. Repeat 10 to 15 times and try to do this 3 times a day.

IMPORTANT: You should only do Kegels (while you are peeing) the first time. DON’T make it a habit to do these exercises every time you pee as this can cause incomplete emptying of your bladder which puts you at risk for getting an infection. You can do Kegels anywhere, anytime–lying down or sitting–no one will know.

Another important type of exercise strengthens your core muscles (a group of muscles in your middle body and stomach). These muscles help with your balance and overall strength. They also work together with your pelvic floor muscles. If you strengthen these, you can help out your pelvic floor!

  • Referral to a urologist or a uro-gynecologist

If you have UI and CF, your CF team may want you to see a doctor who specializes in urinary tract problems, such as a urologist or uro-gynecologist. They may do tests to find out more about your urinary issues and help guide treatment. In some cases, very severe UI can be treated with special medications or with an operation.

  • Work with a physical therapist who has special training in treating UI. Some CF clinics have physical therapists on the team who are specialists in CF and physical therapy.
Practical tips:

Some women who have UI wear panty-shields, light pads, or special underwear to protect their clothes from urine leaks. You can buy them in a store or order them online. It may also be helpful to be prepared if you have a large leak with an extra change of clothes or underwear.

Talk with your CF team if you are experiencing urinary incontinence. They can help you manage your symptoms and explain treatment options.

Here are some talking points and tips that may help you to bring up UI with your CF team:

  1. If your UI is bothering you, tell your team what your symptoms are and what seems to trigger your UI. CF providers are familiar with UI because it happens so often in people with CF. It’s okay to feel embarrassed, but that shouldn’t stop you from talking to your providers.
    • Example: “Sometimes I leak urine when I don’t mean to. This happens rarely/once a week/every day/several times a day. I usually leak a few drops of urine/I have to change my underwear/clothes when this happens. The main things that trigger my leaks are coughing/sneezing/laughing/doing my airway clearance/exercise/having sex.”
  2. Talk with your CF team about other things that may make UI worse.
    • Example: “Should I be screened for a urinary tract infection? Do I need to be screened for CF-related diabetes?  I read that these things may make UI worse if not treated.”
  3. Ask what your treatment options are and if your team should refer you to a specialist.
    • Example: “What are the treatment options for my UI?  Should I see a physical therapist to learn about certain exercises like Kegels?  Do I need a referral?

There is a lot of information to consider when you have CF and urinary incontinence. You can print this guide and review the recommendations with your provider(s) or you can ask if they can access the webpage during your visit.

More information can be found on the Cystic Fibrosis Foundation (CFF) website: www.cff.org

Cystic Fibrosis: Sexually Transmitted Infections (STIs)

Deciding to have a sexual relationship is a big deal for everyone for many reasons, including the possible risks of getting a sexually transmitted infection and/or becoming pregnant.

What are sexually transmitted infections (STIs)?

Sexually transmitted infections (STIs) (sometimes called sexually transmitted diseases or STDs) are passed from person to person through contact with infected body fluids such as blood, vaginal fluids, or semen. They can also be spread through contact with infected skin or mucous membranes, such as sores in the mouth. You may be exposed to infected body fluids and skin during sexual contact. Sexual contact includes any sexual act with another person involving contact with the vulva, clitoris, vagina, mouth, anus, penis, or testicles. STIs can be spread with or without intercourse (penetration). Anyone who has sexual contact with another person is at risk of getting a STI. This includes different-sex couples and same-sex couples.

People with CF have the same risk of getting STIs as people without CF.

STIs include:

Frequently Asked Questions about STIs

What are the symptoms of an STI?

Symptoms vary depending on the type of sexually transmitted infection. Many STIs have no symptoms, or are “asymptomatic.” Symptoms can include a different type of discharge than usual from the penis or vagina, genital sores, pain or itching of the vagina or penis, lower stomach pain, pain or burning when you pee, pain during sex, or bleeding other than your menstrual period.

If you think you have had sexual contact with someone who might have an STI or if you have any new symptoms, talk to your health care provider or CF team right away. It might not be anything serious, but it’s best to get checked out to be safe. Be sure to get checked for STIs at least once a year and also if you change partners or have symptoms.

How can I prevent getting an STI?

The best way to prevent getting an STI is to not have sex. Some STIs can’t be cured, so you should always practice safe sex or find ways to be close in a romantic relationship without having sex. You can lessen your risks of STIs by using a barrier method EVERY time you have sex. 

Barrier methods prevent direct oral, anal and genital contact, and the passing of body fluids (blood, semen, and vaginal fluids) from one person to another.  Barrier methods include male condoms, female condoms, and/or dental dams (for mouth to genital/anal contact). Barrier methods provide protection against most STIs, but not all. For example, HPV (human papillomavirus) can be spread from skin to skin contact around the genital area, not covered by condoms. It is important to remember that other types of contraceptives (such as IUDs or birth control pills) do NOT protect against STIs but are effective in lowering the risk of pregnancy.

If you are having or plan to have sex, you should:

  1. Understand the possible risks and benefits that are involved before becoming sexually active.
  2. Talk with your partner about ways you can protect yourselves from STIs. Make a plan and agree to use the method you have chosen.
  3. Always tell your medical providers (including your CF team) if you have had any sexual contact or are sexually active so that you are given important and necessary information to stay healthy.
  4. Use a barrier method of protection (condoms/dental dams) 100% of the time. You need to make sure that you use a new latex condom or dental dam correctly every time you have oral, anal, or vaginal sex.
  5. Change condoms in between different types of sexual activity such as from anal to vaginal sex. If you are allergic to latex, use polyurethane male or female condoms or silicone dental dams. Do not use lamb-skin condoms or plastic wrap for sexual intercourse.
  6. Use a water-based lubricant with condoms (such as KY Jelly®). The lubricant will keep the condom from breaking. Never use lubricants that contain oil or fat, such as petroleum jelly or cooking oil. These products weaken latex and can cause the condom to break.
  7. Do NOT have sexual contact with anyone who has signs of an STI (sores, blisters, rashes, or discharge from the mouth/genital area).
  8. Get tested for HIV and STIs and encourage your partner to be tested before you have sexual contact. You should do this with each new sexual partner.
  9. Limit the number of people you have sex with. The more partners you have, the greater your risk of being exposed to an STI.

Can vaccinations protect me from getting STIs?

Yes. Some STIs can be prevented through vaccination such as human papillomavirus (HPV) and hepatitis BIt is especially important that people with CF get vaccinated.  If you have a lung transplant someday and are immunosuppressed, STIs, such as HPV, can cause cancers of the skin and genital area.

What should I do if I think I have an STI?

If you have any symptoms of an STI (sores, rashes, or discharge from the genital area), any unexplained problems, or you think you may have been exposed to an STI (even if you don’t have symptoms), see your primary care provider or CF team right away to get tested. Some STIs may not show up on the test right away, so if you think you have been exposed and get a negative result back, it’s important to ask your health care provider if you should get tested again a few weeks or months later. Ask your CF team or primary care provider when you should come back for testing.

  • You can’t test or diagnose yourself with an STI. Only a health care provider can do that. Most STIs can be treated, and the earlier you get treatment, the better. More serious problems can develop if you wait. Whenever possible, treatment is given in a single dose, but sometimes you’ll need to take medication longer.
  • If you are diagnosed with an STI, call your CF team and let them know what you are being treated for and the name and dose of the medicine you are taking.

What should I do if my partner or a past partner tells me that he/she has an STI?

Ask your partner the name of the STI and what medication they took. Tell your health care provider or CF team right away so that you can get tested and treated.

Are all STIs curable?

No. Some STIs, such as herpes or HIV, stay with a person forever. These infections can be controlled with medications, but cannot be cured. Other STIs, such as chlamydia and gonorrhea, can be cured, but it’s important to be treated right away to avoid complications and fertility problems later.

How are you tested for STIs?

STI screening means your blood or body fluid is tested for STIs. Screening tests may include blood tests, urine tests, or vaginal swabs. If you have an infected body part or sore, your health care provider may use a swab (Q-Tip) to take a sample for testing. Blood tests require a quick needle stick. Urine tests and swabs are usually painless.

The test is sent to a laboratory to check whether you have a STI and your medical team is notified of the results.

Where can I get tested for STIs?

You can be tested for HIV and STIs at your primary care provider’s office, your CF clinic, Planned Parenthood and other family planning clinics, or at special clinics for HIV and STI testing.

Do I need a Pap test or a pelvic exam?

Most health care providers agree that you should have your first pelvic exam when you have symptoms such as discharge, pain, or irregular periods, when you want an IUD, or when you turn 21. You should get your first Pap test earlier if your immune system doesn’t work well, such as if you have HIV or if you are taking meds for a transplant. Pap tests help detect pre-cancerous/cancerous lesions of the cervix (inside of the vagina) that are most often caused by human papilloma virus (HPV). If you have already had a lung transplant, you should have a pelvic exam and Pap test when you start to have sex regardless of your age or if you have symptoms. Your CF team can help you figure out if you need a pelvic exam or Pap test.

What about confidentiality? What if I don’t want my parents to know that I’m having sex?

What you tell your primary care provider or CF team about your sexual behavior is confidential.

It’s okay for you and your CF team to ask that your parents or family members step out of the room for parts of your visit with your medical providers or CF team. Ask your CF team and PCP about their confidentiality policies and if the health care provider’s notes from your medical visit can be viewed by your parents through the online portal. Your medical providers should not talk about confidential information to anyone else unless he/she seriously believes there is danger to you or to others, that you are being abused, or that you are not able to make safe decisions. Although your medical providers do their very best to keep your information private, there is a chance your health insurance company or pharmacy may send information to your home. Many teens find it helpful to talk to their parents about their health and their worries.

When you are eighteen or older, all of your health care and decisions are confidential. If you are younger than 18, confidentiality laws vary from state to state; however, in all states STI testing and results are confidential.

Find someone on your CF team you can talk with if you have questions about STIs or if you need to be screened. They can help get you the care you may need. 

Here are some specific talking points and tips that may help you discuss STIs:

  • Tell your CF team if you are sexually active and ask about STI screening. Screening is recommended for everyone who is sexually active, so don’t hesitate bringing it up!
    • Examples: “I am having sex and I know that I should be screened for STIs. Can I get STI testing in this clinic today? Or can you recommend when and where I can get confidential testing?”
  • Ask about a referral to a women’s health specialist or other doctor if you are interested.
    • Example: “Is it okay to ask for a female health provider?(such as an adolescent medicine specialist or a gynecologist) Is there a local clinic you would recommend?”
  • If you are diagnosed with an STI, don’t be afraid to ask if the health care provider has more time to talk or if there is someone else you can talk to. It can be hard to hear that you have a STI. It’s also important to let your CF team know right away if you are prescribed a medication to treat your STI and who prescribed the med(s).
    • Examples: “It is hard to hear that I have an STI. Do you have more time to talk about this? Or is there someone else I could talk to about how to handle this information?”
    • “I was diagnosed with an STI and I was prescribed [this medication]. Does this drug have any interactions with my regular CF medications?”

There is a lot of information to consider when you have CF and are having sex. You can print this guide or have your provider access the webpage during your visit.

Cystic Fibrosis: Pregnancy and Fertility Basics

Can I get pregnant if I have CF?

YES.  Most women with CF can get pregnant and give birth to healthy babies, but it’s always best to PLAN AHEAD with the help of your partner, family and CF team! Girls with CF have the same female anatomy (ovaries, fallopian tubes, uterus, cervix, vulva and vagina) as girls without CF and usually have normal menstrual cycles.  However, the mucus in and around your cervix may be thicker than in a woman without CF, which may make it slightly harder for sperm to reach an egg. It is still very important to use contraception (if you are having sex with men) unless you are ready to become pregnant.

If you are thinking about becoming pregnant, it is very important to talk with your CF team. Your CF team can help you have a healthy planned pregnancy. They will help you think about your health and any risks that pregnancy could pose for you. Some factors that your CF team will consider are your lung function, nutrition, and if you have diabetes or liver disease. If you have had a lung transplant, there may be more risks to consider before planning a pregnancy.

Is pregnancy different when you have CF?

Pregnancy can have a big impact on your health, especially if you have CF. If you are healthy and have stable lung function, pregnancy is generally safe.  However, if you are unwell or have poor lung function, pregnancy will put an additional strain on your body. Careful planning for pregnancy at a time when you are as healthy as possible can lead to the best outcomes for both you and your baby. There are many different options for becoming a parent aside from becoming pregnant and having a baby.

Can I have a healthy pregnancy?

Pregnancy does not affect the life span of women with CF. However, during pregnancy, many women experience more CF exacerbations.  You may also experience constipation and issues with nutrition and gaining enough weight for your baby’s growth.  Just like women without CF, you can also develop a form of diabetes called gestational diabetes during pregnancy that usually improves after the baby is born. Because of these health concerns, it is very important to work closely with your CF team and your obstetrician throughout your entire pregnancy.

Can I take my regular CF medications if I plan on becoming pregnant?

Some CF medications are dangerous for a developing fetus and can cause birth defects. That’s why it’s very important to tell your CF team when you are thinking about becoming pregnant. Your CF team will help you figure out any changes to your medications and therapies that may be required before you become pregnant. Your provider(s) will also likely have you take prenatal vitamins and folic acid before you try to become pregnant.

What if I’m having trouble getting pregnant? 

Most women with CF are able to become pregnant.  As stated above, some research shows that thicker cervical mucus and poor nutrition may make it harder to get pregnant. If you have stopped your birth control and have been trying to become pregnant without success, you can talk to your CF team about getting a referral to see a fertility specialist. Typically, fertility testing isn’t done until a couple has been trying to get pregnant for about a year.

What should I do if I think I am pregnant?

If you think you may be pregnant, the most important first step is to call your CF team. They can confirm pregnancy through a pregnancy test and help you figure out the best options based on your needs and your health. It’s important to know that you have many options.

Cystic Fibrosis: Deciding Whether to Become a Parent

Whether or not you have CF, there is a lot to consider when you are deciding to have children. As someone with CF, you will need to think about your health, passing CF down to your children (if you decide to become pregnant), and what it would be like to be a parent. You may also want to consider other options to start a family such as surrogacy, adoption or foster parenting.

Should I have children?

Deciding to start a family is a big decision. It is a personal choice and there is no right or wrong answer. Some women with CF want to become a parent and some do not. Both of these choices are okay! If you are thinking about having children, the best first step is to talk with your partner, family, and/or friends. You should also always talk with your CF team because they know you and your health and can support you with your choice.

How do I know if I will have the support I need?

Think about the people in your support system and how they help you now and how their role or availability may change if you have one or more children. You should also consider how having children will affect your daily life. Will you be able to balance taking care of yourself and a child? When will you do treatments and take care of your CF? Who are the people who will help you with child care if you get sick?

Am I healthy enough for pregnancy?

Many women with CF have asked this question! Studies have shown that pregnancy does not affect survival for women with CF. However, there are many changes that happen inside your body if you become pregnant. You may feel very tired, extra hungry, and/or nauseous (feeling like you want to throw up). Your breasts will likely get bigger and feel tender, and you may have stomach pains similar to menstrual cramps. If you have CF, these changes may be tough at times for your body to handle.

In addition to your CF medications, you’ll need to take a daily prenatal vitamin, which includes folic acid. Folic acid (also known as “folate”) prevents serious spinal cord birth defects.

When you talk with your CF team about pregnancy, they will consider your lung function, your nutritional status, whether you have CF-related diabetes or liver disease, or whether you’ve had a lung transplant. For more information, read our health guide on Pregnancy and CF.

When should I become pregnant?

You should talk to your CF team if you decide you want to become pregnant. The best time to begin trying is when you are in your best health. This means that your lung function, weight, and overall health are the best that they can possibly be. This will make pregnancy and childbirth easier and safer for you and your baby.

Will my baby have CF?

CF is a genetic disease. This means that the disease is passed down from both parents to a child. You need two copies of a CF gene mutation (one from each parent) to have CF.

A person with one CF gene mutation is a carrier for CF. CF carriers do not have CF symptoms, but could pass on the CF gene on to their kids. If you have CF and are thinking about getting pregnant and having children, it’s important to think about finding out if your partner is a CF carrier and the chances of passing on CF to your children. If you become pregnant through in-vitro fertilization (or IVF), your fertilized eggs can be tested for CF before implantation.

Below is an image that shows the chances of passing CF on to your children with a partner who is a carrier for CF:

If you have CF and your partner is a carrier for CF, there is a 50% chance that your child will have CF and a 50% chance your child will be a carrier for CF.

Below is an image that shows the chances of passing CF on to your children with a partner who is NOT a carrier for CF:

 

If your partner is not a carrier for CF, there is a 100% chance your child will be a carrier of CF and a 0% chance that your child will have CF.

How can I find out if my partner is a CF carrier?

A genetic test can be used to tell if a person is a carrier for CF. This is done using a blood sample or a scraping of the cells from the inside of a person’s mouth. This test only looks for the most common CF gene mutations. This means if it comes back negative, there is still a small chance a person is a CF carrier (just carrying a less common CF gene mutation.)

About 1 out of every 31 Americans is a carrier of a CF gene mutation. Since carriers do not have symptoms, you may think about having genetic testing to find out if your partner is a CF carrier.

Should my partner get genetic testing for CF?

It is a personal decision to get genetic testing to find out if your partner is a CF carrier. In addition to your family and friends, there are many people you can talk to about whether this is the right choice for you. Genetic counselors are a great resource to ask questions and to help you understand the testing options. You should also always talk with your CF team because they know you and often know genetic counselors that are familiar with CF.

The Cystic Fibrosis Foundation (CFF) website has more information on genetic testing: https://www.cff.org/What-is-CF/Testing/Carrier-Testing-for-CF/

You can also visit the National Society of Genetic Counselors website or call 610-872-7608.

What are the options for becoming a parent besides pregnancy?

There are many ways to become a parent, outside of pregnancy, including surrogacy, adoption, and foster care.

What is surrogacy?

Surrogacy involves taking an egg from your ovary or a donor and sperm from a male partner or a sperm donor, and fertilizing the egg with the sperm in a lab. When the egg fertilizes it becomes an embryo. The embryo is then placed in the uterus of another woman, who is called the “surrogate” or the “gestational carrier.” A “surrogate” has a genetic connection to the baby and has donated her egg to the embryo. A “gestational carrier” is a woman who carries a baby for an individual or couple but does not have a genetic connection to the baby. There are a lot of steps in between that involve much coordination by a fertility team. Insurance coverage for this procedure can unfortunately be somewhat complicated. Different insurance companies cover different costs and coverage can vary drastically between states and countries. The procedure itself, as well as paying a surrogate, can get very expensive. You’ll need to do your research to find out if surrogacy is legal in your state and also think about what it would cost.

State by State Surrogacy Law Practice: This is a private website founded by a lawyer who has expertise with surrogacy. There is a color–coded map of the United States on the website that clearly explains the surrogacy laws that apply to each state. You can find out how the law works in each state just by clicking on the state image. Keep in mind that the practice of surrogacy is ever changing. An experienced lawyer must still determine the prevailing practice in a particular state court at the time surrogacy is initiated.

What is adoption?

There are many ways to adopt a child. You can choose to adopt a U.S.-born child, a child with special needs, or a child from another country. You can also choose to adopt through an agency or independently. The cost of an adoption varies depending on the process you choose.

Adoption–Parents Magazine: This website is sponsored by “Parents” magazine and does a nice job explaining the different types of adoption offered in the United States. You’ll also find stories from families who talk about their adoption experience.

US Department of Health and Human Services–Administration for Children and Families: Adoption laws vary significantly from state to state yet they must comply with general rules set by the United States Federal Government. This resource answers some of the questions you might have about adopting a child from child welfare services; however it is not meant to replace a conversation with a lawyer that is knowledgeable about adoption laws, the cost, etc. where you live.

Intercountry Adoption–Bureau of Consular Affairs–U.S. Department of State: This website offers information for United States citizens who are considering adopting children from abroad. Adoption between the U.S. and other countries is different than domestic adoptions and is governed by both the laws of the country in which a child lives and the country in which the adoptive parents live. You can learn about the adoption practices in most foreign countries by clicking “Country Information,” then searching for a country in the drop down menu.

What is foster care?

Foster programs support children who can’t remain in their parents’ homes because they’ve been abandoned, mistreated, or they are unsafe in their family home. Foster care takes place in a variety of settings, including the home of a relative or friend, a foster family home, a staffed group home, or, for special-needs kids, an institution that offers professional therapy and treatment. With federal guidance, states and local communities find people to become foster parents. If you’d like to become a foster parent, you have to complete required training and a background check and have a caseworker conduct a home visit to determine whether you’re ready to foster. In some cases, you can adopt your foster child.

Foster Care–Parents Magazine: This website is sponsored by “Parents” magazine and explains the process of foster care in the United States.

Helpful tips for Parenthood and CF:

Establishing a Routine

New parents find that a new child is a big disruption to their regular routine. As someone with CF, you should re-establish a routine that works with your and your family’s schedule. Fit in treatments during nap time or have your partner or support person do some feedings so you can take care of yourself.

Involving Children in Treatments

As your child gets older, you can include them in your CF care. Have your child help you set up a treatment or do your airway clearance or treatments during playtime. This way you can spend as much time with them as possible and introduce the idea of CF to them early.

Emotional Considerations

Being a parent is hard work.  You may find it tough to balance your care and the care of your child, and you may often need to depend on others to help. Do not be afraid to ask for help for your emotional and mental health needs if you choose to become a parent. Encourage your children to ask questions about CF to help them understand what your CF means for them and your family. Your CF care team can be a great resource to help you if you decide to become a parent.

Deciding Not to Have Children

After taking everything into consideration, you may decide that becoming a parent is not the best option for you. That’s OK. Many people, with and without CF, decide not to have children and go on to lead full and happy lives.

Here are some specific talking points and tips that may help you talk with your CF team about deciding to have children:

  • Tell your CF team what you want. If you want to have children, tell them that! If you do not want to have children, tell them that! If you aren’t sure, tell them that as well. Talking with your team now can help you both PLAN for what having children might mean for your health and life.
    • Example: “I want to talk with you about having children. At this point in my life, I want to have children/do not want to have children/am not sure if I want to have children. Since I know deciding to have children is a big decision, I want to make sure I can talk about it with you.”
  • Ask your CF team questions.  If you are curious whether you are healthy enough to become a parent, you should ask! Some questions to consider asking are:
    • “Am I healthy enough to become pregnant or become a parent?”
    • How can I get my health to the best it can possibly be before I become pregnant or become a parent?”
    • “Will my baby have CF if I become pregnant?”
    • “How does my partner get tested for CF gene mutations/find out if they are a carrier?”
    • “Should my partner get carrier testing?”
    • “Do you know a genetic counselor that is familiar with CF who I can talk to?”
    • “What are other resources that could help me explore options for becoming a parent that you could refer me to?”
  • Talk to your CF team about creating a plan to prioritize your health when you also have to parent a small child.
    • “I’m worried about prioritizing my health when I have a child to care for. How can I make a plan so I am able to stay healthy while raising a child?”

There is a lot of information to consider when you have CF and are deciding whether to become a parent. You can print this guide or have your provider refer to the webpage during your visit.

The Cystic Fibrosis Foundation (CFF) website has reliable resources that you can look at if you are deciding to have children: https://www.cff.org/Life-With-CF/Transitions/Family-Planning-and-Parenting-With-CF/

Cystic Fibrosis: Pregnancy and CF

Most women with cystic fibrosis (CF) can get pregnant. Some things may be different for a woman with CF during a pregnancy than for women without CF. Since pregnancy can have a big impact on your health, it’s important to work with your medical team and PLAN for a pregnancy when you are as healthy as possible. This will help ensure the best outcomes for you and your baby.

What impacts a pregnancy when you have CF?

There are a few things your CF team will consider when thinking about how a pregnancy could impact your health.

Your lung function

  • In a perfect world, your forced expiratory volume (FEV1) should be 50% or higher before pregnancy. The risk of complications is higher with a lower FEV1.

Your nutrition

  • Your nutritional status must be good enough to support you and a growing baby. It is recommended that your body mass index (BMI), or the ratio of your weight and height, is at least 22 kg/m2 before pregnancy.
  • The formula to measure your BMI is: Weight (in pounds) x 703 divided by Height (in inches) squared.
  • If you have a lower BMI, your baby may not grow as expected and may be born early. Talk with your CF team to find out your BMI and your nutritional status.

If you have CF-related diabetes

  • Having diabetes during pregnancy may lead to more health problems. It’s important that your blood sugar is well-controlled and stable before you become pregnant. If you take insulin, your doses may change during pregnancy.

If you have CF liver disease

  • Liver disease can cause additional problems during pregnancy. It is very important to talk with your CF team if you have liver disease or cirrhosis.

If you’ve had a lung transplant

  • It is possible to become pregnant after a lung transplant. However, pregnancy after lung transplant has more health risks, including possible rejection, worsening lung function, poorly controlled blood pressure, and development of diabetes. Your baby may not grow well and may be born earlier than expected. Some transplant medications are dangerous to take when you are pregnant. Getting pregnant is not recommended within the first 2 to 3 years after transplant. If you are thinking about pregnancy and have had or might be a candidate for a lung transplant, talk with your CF team.

Are there common complications that women with CF experience when pregnant?

Yes, women with CF can experience a few common complications during pregnancy. It is important to talk with your CF care team and your obstetrician frequently during pregnancy to make sure that you and your baby have the best outcomes possible. Your CF team and your obstetrician should also work together to understand how your pregnancy is affecting your CF and vice versa. You may benefit from seeing an obstetrician familiar with CF, or one trained in high-risk obstetrics or maternal-fetal medicine (MFM).

  • Pulmonary exacerbations Women with CF have been shown to experience more CF exacerbations during pregnancy. Your CF team will review the medications that you have been prescribed to treat your exacerbations in the past. Since many antibiotics are not safe to take during pregnancy, the antibiotics you are prescribed may change. It’s important to work with your CF team to continue your daily therapies.
  • Preterm or premature delivery Some women with CF may deliver their baby early. A preterm or premature delivery is a birth that takes place more than 3 weeks before a baby is due. Babies born early are often small and have a low birth weight, have heart problems, gastrointestinal issues, breathing problems, and feeding difficulties. Depending on how early a baby is born, they may have to spend time in the neonatal intensive care unit (NICU).
  • Gestational diabetes This is a type of diabetes that a mom develops during pregnancy and usually improves after she gives birth. Women with CF (who do not have CF- related diabetes) are at a higher risk of developing gestational diabetes than women without CF.
  • Nutritional deficiency A woman should gain about 25 to 35 pounds during pregnancy. Since many women with CF struggle with maintaining their BMI, it may be more difficult to gain enough weight during pregnancy. Women with CF can consider nutritional supplements during pregnancy in order to gain the right amount of weight. Prenatal vitamins and folic acid should be started before conception and taken throughout her pregnancy.
  • Constipation Almost all women experience some constipation during pregnancy. Women with CF have a higher risk of having constipation while they are pregnant. This can be treated using stool softeners and/or laxatives. Talk with your obstetrician about which medications you can use when you are pregnant.
  • Hypertension Women with CF have a higher risk of hypertension (high blood pressure) during pregnancy. If you become pregnant, your CF team and your obstetrician will monitor your blood pressure closely. Hypertension during pregnancy can be treated with different types of medications.

What CF medications are dangerous to take during pregnancy?

Common medications used by women with CF that should be avoided during pregnancy include:

Antimicrobials
Acyclovir
Chloramphenicol
Ciprofloxacin
Clarithromycin (avoid in first trimester of pregnancy)
Colistin (intravenous and inhaled/nebulized forms)
Doxycycline
Fluconazole
Gancyclovir
Gentamicin (intravenous form)
Imipenem
Itraconazole
Meropenem
Metronidazole
Posaconazole
Rifampin
Tobramycin (intravenous form)
Trimethoprim-sulfamethoxazole (Bactrim)
Vancomycin
Voriconazole
Transplant medications
Azathioprine
Mycophenolate
Tacrolimus
Other medications
Bisphosphonates
Ranitidine (Zantac)
Ursodiol (avoid in first trimester of pregnancy)
Vitamin A supplements
If you are taking any of these medications and are pregnant or thinking about becoming pregnant, talk with your CF team right away!

The effects of ivacaftor (Kalydeco) and ivacaftor/lumacaftor (Orkambi) when used during pregnancy are not known.  Tell your CF team if you are taking these medications and are pregnant or thinking about becoming pregnant.

What if I’m having trouble getting pregnant?

Fertility issues can happen for many reasons. If you and your partner have been trying to get pregnant for a while, your CF team can refer you to a fertility specialist. Fertility testing is usually done after a couple has been trying to get pregnant for about a year.

Addressing fertility is the same for women with CF as for women without CF. There are different types of assisted reproduction technologies that are available for women to pursue, such as fertility medications, in-vitro fertilization (IVF), and surrogacy.

  • Fertility medications stimulate ovaries to release eggs. Sometimes, these medications can cause ovarian hyper-stimulation syndrome, which can cause you to have difficulty breathing. Your fertility specialist and CF team should monitor you closely when you are taking fertility medications.
  • Intrauterine insemination (IUI) is when sperm is placed directly inside of a woman’s uterus. It is done to increase the number of sperm reaching the egg and increase the chance of pregnancy.
  • In-vitro fertilization (IVF) is when sperm and eggs are retrieved from a man and woman and fertilized outside of a woman’s body. Fertilized eggs (embryos) are then implanted into the uterus with the hope of pregnancy. IVF can be a good option for women with CF whose partners have been found to be carriers of CF as fertilized eggs that do not have CF gene mutations can be selected for implantation. With IVF, more than one embryo can be implanted resulting in multiple pregnancies (twins or triplets). Women with CF interested in IVF should talk with their IVF team about transferring just one embryo to reduce the chance of a multiple (high risk) pregnancy and the added stress of caring for more than one baby.
  • Surrogacy is when another woman carries a pregnancy for a couple. A surrogate pregnancy can be done using IVF, in which case the baby is genetically related to the mom and the dad, but not the surrogate. Donor eggs or sperm can also be used. Laws around surrogacy vary in different states, so it’s important to check the laws in your home state if you are interested.

There are other options, such as adoption, to consider if you want to become a parent and may not be able to do so through pregnancy. Read more about these options in the guide “CF: Deciding Whether to Become a Parent.

Can I breastfeed?

Women with CF can safely breastfeed their babies. However, there are some considerations for women with CF when deciding whether or how long to breastfeed. Breastfeeding uses up many calories and if you are struggling with weight gain, it may be in your best interest not to breastfeed. You will need to work with your CF team to make sure you are maintaining a healthy weight. Your CF team will also let you know which of your medications may be unsafe if you are planning on breastfeeding.

Below are examples of specific talking points and tips that may help you talk with your CF team about pregnancy:

Tell your CF team as soon as you think you may be pregnant since pregnancy can have a big impact on your health.

Examples:

  • “I think I might be pregnant. Can I get a pregnancy test done while I’m in clinic today?”
  • “How could pregnancy affect my health? What CF medications are safe for me to use during pregnancy?”

If you think you may want to become pregnant in the future, talk with your CF team about your health, and how you can plan a pregnancy when you are at your healthiest.

Examples:

  • “I think that in the future I may want to become pregnant and have a child. Am I healthy enough for pregnancy?”
  • “How can I improve my health before becoming pregnant?”

If you and your partner have been trying to get pregnant for a while and haven’t had success, speak with your care team about possibly being referred to a fertility specialist.

Examples:

  • “My partner and I have been trying to get pregnant for [___ months/years]. Should we see a fertility specialist to get testing done?”
  • “Can you refer me to a specialist familiar with CF?”

There is a lot of information to consider when you have CF and are deciding whether a pregnancy is right for you. You can print out this guide or have your provider refer to the webpage during your visit.