Puberty, sexuality, fertility and family planning can all be confusing, exciting and scary times in your life. Read more about whichever topic relates to you and talk to your CF team about any questions you have!
Both men and women with CF can experience a delay in the onset of puberty of about 2-4 years. In women, this can be due to low body weight, and with males, it has to do with hormones reaching peak levels. Sometimes this can affect self image or cause anxiety, as many CF adolescents will have to deal with a time where they are physically smaller than their peers. It is important to remember that the situation is only temporary! Once hormones reach regular peak levels, CF individuals will experience their growth spurt and will take on the physical appearance of a normal healthy adult. In the meantime, however, good nutrition and exercise can help with improving self-image.
About 98% of men with CF are infertile. This does NOT mean that they can’t have children. It means that they need the help of a doctor trained in assistive reproductive techniques to do this. Men with CF are infertile because they are partly or fully missing the tubes that normally carry sperm from the testes to the penis. These tubes are called the vas deferens. Males produce normal sperm but they can not reach their partner in the typical way. However, assistive reproductive techniques (ART) are able to retrieve sperm from the testes which is then used to fertilize the egg of the man’s partner. A test called a semen analysis can be used to see if you are infertile.
More Information Regarding Fertility In Men With CF
Although most men with CF are infertile, it is very important to know that men with CF are not impotent. In fact, men with CF have normal male characteristics and normal sexual function. They are infertile due to a blockage in their reproductive tract making it impossible for them to deliver sperm to their partner. Therefore, they are unable to father children naturally.
Up until approximately 20 years ago, there was no medical procedure or treatment available to treat the blocked vas deferens. The two options to have children at the time were adoption or artificial insemination by donor sperm.
In recent years, advances in assistive reproductive techniques (ART) have made it possible for men with CF to father their own biological children. The men go through a procedure to obtain sperm for in-vitro fertilization (IVF) . The most common technique is called PESA, percutaneous epididymal sperm aspiration. This procedure is usually performed under local anesthesia (numbing medication). The urologist/infertility specialist introduces a tiny needle into the head of the epididymis (a set of curvy tubes that sperm travel through as they leave the testes) and aspirates the fluid containing sperm into a syringe. Once the sperm are removed, they are quickly placed into a special solution that supports their survival. It is then further divided into several portions for cryopreservation (freezing). Each frozen portion serves as a source of sperm to be used for a cycle of fertilization in the future.
Fertilization of the egg occurs through a procedure called IntraCytoplastic Sperm Injection (ICSI). Using this technique, an individual sperm is directly injected into the egg of a woman. This markedly increases the chance of successful fertilization.
Once the couple has decided to use this method, the female partner will receive special hormones to increase ovulation (production of eggs). When the eggs are produced, they are removed from her body and placed into a test tube for sperm injection. In about three to five days after fertilization, two or three embryos will be transferred back into the woman’s uterus for implantation. Pregnancy will be assessed in ten to twelve days. The current estimated pregnancy rate is about 50-60% per cycle or even higher. Based on the current experience, the pregnancy rate is higher if the woman’s age is younger than 35 years.
Thanks to advances in assistive reproductive techniques, many men with CF and their partners are able to realize their dreams of having their own biological children.
Women with CF have a normal reproductive tract and sexual function.
A small number of women with CF sometimes have difficulty getting pregnant because of thick sticky mucus (similar to what happens in the lungs) accumulating in the opening of the uterus (called the cervix). This thick layer makes it harder for sperm to penetrate when women ovulate (release their egg).
The fluid in the uterus is also decreased in amount and this makes it more difficult for the sperm to travel up to the fallopian tubes where fertilization occurs.
In some woman, irregular menstrual periods can make it harder to get pregnant.
If you are considering planning a family it is important to tell your CF team in advance to set up a meeting with a genetic counsellor and to review important health information that you need to know when getting pregnant. Even though a small number of women with CF may have difficulty getting pregnant, it is strongly recommended that women who are, or plan to become sexually active should use birth control to prevent unwanted pregnancy and condoms are recommended to prevent sexually transmitted diseases.
Experience has taught us that a planned pregnancy is the key to having a successful pregnancy and a healthy baby. If you are a woman with CF and are thinking of getting pregnant, it is crucial that your lung function and nutritional status are optimized.
The couple starts this journey by having her partner tested for CF carrier status. If her partner is not a carrier of a CF gene, any children they have will be CF carriers but will not have CF. If the partner is a carrier for one CF mutation, then there is a 50% chance that their child will have CF.
It is generally accepted that the criteria for successful pregnancy include stable lung function and a healthy body mass index (healthy weight for the individual) between 20 to 25 kg/m2.
With increased life expectancy, we are seeing more people with CF developing CF related diabetes. For a woman with CF and CF related diabetes, it is also important to make sure that her diabetes control is optimized for 6 months prior to trying to become pregnant. Signs of having good diabetes control include daily blood sugars that fall within normal target and a HBA1c (glycated haemoglobin) of 6 to 6.5%.
Although it sounds like a lot to do prior to getting pregnant we are here to help you! Talk to your CF team i.e. doctors, nurse practitioner, dietitian if you are planning to become pregnant.
As a woman reaches middle age, she will start having symptoms related to so-called “change of life”. Actually, it simply reflects that she is transitioning into her perimenopause period and subsequently reaching menopause.
Menopause is defined as one year without menstrual flow. However, all women go through the journey of perimenopause along with some of the unique signs and symptoms due to hormonal changes before finally reaching menopause.
Perimenopause (‘peri’ meaning around) is defined as the period immediately prior to menopause and one year after menopause. Women may feel their first sign of perimenopause anywhere between 35 and 50 years of age. Women may also experience symptoms of perimenopause for 6 to 8 years.
Due to hormonal changes during the period of perimenopause, women may experience different physical symptoms and psychological stresses that can affect their quality of life. Therefore, it is helpful to gain knowledge about the changes that occur during this period and learn to deal with them with healthy and positive approaches.
Irregular cycles, heavier or prolonged menstrual flow, hot flashes, night sweats and sleep disturbances, joint stiffness and muscular discomfort and lower sex drive.
Women with CF may experience symptoms of perimenopause and reach menopause 3 years earlier than women without CF as demonstrated by a study conducted in our CF clinic. It was also noted that sleep disturbances appeared to be the most dominant symptom around the time of menopause. If you have symptoms, questions, or concerns please do not hesitate to speak with your CF care team.
Delayed puberty, altered body image, and worries of early death, along with inadequate knowledge about their reproductive potential, can sometimes lead to anxiety and frustration in adolescents and young adults with CF. Both males and females with CF can experience a delay in puberty for about two to four years due to a delay in sexual hormones reaching their peak levels. When these hormones eventually reach normal peak levels, they too will have the growth spurt and take on the physical appearance of a normal adult. It is important to realize that the situation is only temporary. CF adolescents may have to deal with a period of time when they are physically smaller than their peers. Poor body image is often influenced by physical features such as small stature, thinness, finger clubbing, and stained teeth. However, good nutrition and exercise may prove to benefit body image. For those individuals with more advanced lung diseases, they may develop a barrel chest or a slight hump on their back just enough to make them feel unattractive. Some medical apparatuses placed inside the body such as gastrostomy tube/button or port-a-cath (permanent IV access device) to maintain health can further affect one’s self image. The most common struggle continues to be the issue regarding when and how to tell someone whom they are interested in about their CF and the possibilities of reduced fertility. Based on many people’s experience, the best way to tell a prospective partner is to have an open and honest discussion about their CF diagnosis, explaining the kind of medical regimen that they must perform regularly to stay healthy.
Health related problems such as urinary incontinence due to constant strong cough, yeast infection resulting from frequent antibiotics, and frequent flatus (gas), caused by indigestion, can lead to embarrassment at the most inappropriate times. However, many of these symptoms can be controlled. Exercise to strengthen muscles in the pelvis and avoidance of caffeine may help lessen the degree of incontinence. Anti-fungal medication can be used to treat yeast infections. Proper use of pancreatic enzymes may improve digestion and absorption, which will decrease the amount of gas.
People with CF have normal sexual function. Mild hemoptysis (coughing up blood) has been reported during sexual intercourse but it is usually associated with chest infection. Taking prescribed antibiotic(s) to treat the infection and inhalation therapy containing bronchodilators (medications that relax and open airways) prior to having sex may prevent it from occurring. Some people with CF take supplemental vitamin K to help strengthen the blood clotting mechanism. Once end-stage lung disease develops, supplemental oxygen may be used. Also, avoiding sexual positions that cause restriction to chest expansion may be helpful. Remember, within the context of a meaningful relationship, an expression of love in any way or form can be a satisfying experience for both partners.
For more information, please explore this brochure from Cystic Fibrosis Canada.
Sex is a delicate subject that is often difficult to speak about with healthcare professionals. Sex is an essential part of life, regardless of age, sex, health, or physical activity and is considered by many a wonderful way to express love for one another. CF does not diminish sexual desire or the need to be intimate with your partner.
Although most men with CF are infertile, they are not immune to sexually transmitted infections or STIs. Women must use contraceptives if they do not wish to become pregnant and they must have protected sex since they are also at risk for contracting STIs.
TYPES OF CONTRACEPTION
Male Condom When used correctly, it is a highly effective means of birth control and provides protection against STIs almost 100% of the time. Perfect use, i.e. correct use every single time, can still result in a 2% per-year pregnancy rate. Typical use is not necessarily using it correctly or at every sexual contact, meaning the pregnancy rate can go up to 15% or higher. Accidental breakage can also happen even with perfect use. It is an excellent method of protection from STIs and transmission of organisms that cause AIDS, genital herpes, genital warts, syphilis, and gonorrhoea.
Female Condom Similar to the traditional male condom, but inserted into the vagina, it also offers highly effective birth control and STI protection; the female condom is suitable for people who are allergic to latex.
Birth Control Pill If used properly, is very effective in preventing pregnancies, but does not offer any protection against STIs; its effectiveness may be decreased when certain antibiotics are taken. Recent pharmacokinetic data has shown that plasma levels of oral contraceptive steroids (birth control pill) remained unchanged whilst using antibiotics commonly prescribed in CF with the exception or rifampicin which does alter plasma hormone levels, leading to contraceptive failure. Some complementary medicines such as St. John’s Wort can also affect liver enzymes and reduce the effectiveness of oral contraception.
Depo-ProveraTM Delivered by an intramuscular injection every three months. It is NOT recommended for women with CF because it increases the risk of osteoporosis (having to do with bone mineral density), a condition to which individuals with CF are already predisposed
“The Patch” (EvraTM)
Is an effective once-a-week birth control patch that works on the same principles as the pill. Like the pill, it does not offer protection against STIs, and its effectiveness may be decreased when certain antibiotics are taken.
Intrauterine Device (IUD)
A highly effective device that is inserted into the uterus by your gynaecologist or family doctor, and can remain there for several years. It does not protect against STIs.
A device that prevents sperm from entering the uterus; it should be used in combination with spermicidal cream or jelly and offers no protection against STIs.
A contraceptive substance that eradicates sperms. It is inserted into the vagina prior to intercourse to prevent pregnancy. It is NOT an effective method when used alone. Usually, Spermicides are combined with contraceptive barrier methods (i.e. diaphragm, condom, cervical caps and sponges. When combines use can result in lower pregnancy rate. *Important to note that Spermicide is NOT recommended by CDC. Spermicides can cause irritation, increased risk of contracting HIV, urinary tract infections, yeast infections, and bacterial vaginosis in women, and itching and burning sensation in the genital area of either partner.
Vaginal Ring (NuvaRing)
Delivers estrogen to the source and is inserted into the vagina for 3 weeks and removed for the 4th week. It has the same efficacy as the pill.
A sponge placed inside the vagina that contains spermicide. You add water and place it inside up to 24 hours before intercourse and you take it out no sooner than 6 hours after intercourse. Toxic Shock Syndrome can occur if it is left inside longer than 30 hours total. Similar to spermicide, it has been reported to irritate the genital area. It has 64 to 82% effective if used correctly and at every intercourse.
INEFFECTIVE METHODS OF CONTRACEPTION
The following are not-so-effective methods but have been used by people due to religious regions or fear of side-effects from the above methods.
Rhythm Method / Natural Family Planning
This method has been known for thousands of years. It works by focusing on a woman’s most fertile period during her menstrual cycle and then restricting sexual intercourse during that time. As mentioned earlier in the section about the menstrual cycle, the egg is ready for fertilization after ovulation. This matured egg will survive for 24 to 48 hours at the most. So, if a woman who is using the rhythm method to avoid pregnancy, she should not have sex during this period of time. Unfortunately, it time that takes to ovulation within a menstrual cycle varies slightly from woman to woman. In CF, many girls have irregular periods. Therefore, it would be quite difficult to do the calculation and pinpoint the exact day of fertility. An unplanned pregnancy can bring in significant physical and psychological stress to a woman and her family. Therefore, this method is not recommended.
Withdraw (Coitus Interruptus)
This method involves the male partner pulling his penis out of the vagina before ejaculation. It is not an effective method because it is not easy to withdraw early enough and sperm containing in the first squirt may have already reached into the woman before his complete withdrawal. Also, it is believed that the “dew drop” of fluid man produces when he becomes excited contains sperm. So sperm may have already deposited before actual ejaculation. With all the modern reliable methods and knowing the possible physical and psychological stresses that can result from unwanted and unplanned pregnancy especially in women with CF, this method is not recommended. *Please note that both the rhythm method and the withdrawal method are NOT effective methods of contraception and should be avoided. Because of the risk of side effects and potential interactions with your CF medications, it is important to talk to your doctor to find the best contraceptive for you. You may also seek advice from a family doctor; however, it is important that the CF team is aware of all treatment.