Cystic fibrosis, abbreviated as CF, is an inherited disorder that is passed on from parents to their children at the time of birth. It is associated with serious damage to the digestive system, lungs, and other organs of the body. To be more specific, cystic fibrosis affects the cells that produce sweat, mucus, and digestive juices. These secreted fluids are generally of a thin consistency and also slippery among people with CF. In contrast, the fluid takes a thick and sticky consistency in people with the medical condition due to their defective gene. These secretions block passageways and ducts, especially in the pancreas and lungs, instead of serving as a lubricant.
The Channel 46 caught up with Dr Anjali Kumar, MBBS, MD & Former CEO at Just Diabetes, who advises you on the symptoms of the medical condition, how its outcomes are more detrimental for women than men, and the available treatment options.
11 Symptoms of Cystic Fibrosis
The symptoms of cystic fibrosis are determined by the severity of the medical condition. Symptoms may improve or get aggravated with time. Among some patients, the symptoms may not manifest until their teenage years or adulthood. Such patients usually have a milder form of the condition. They are also more likely to have atypical symptoms like recurring pneumonia, pancreatitis or bouts of an inflamed pancreas, or even infertility (among males). People with CF also have an abnormally high level of salt in their sweat.
The other symptoms of CF mostly affect the digestive and respiratory systems.
5 Digestive Symptoms Of CF
The thick and slippery mucus blocks passageways and tubes carrying digestive enzymes from the pancreas to the small intestine. The lack of these enzymes make it difficult for the intestines to absorb the valuable nutrients from the food. This results in the following:
- Weight gain
- Poor growth
- Intestinal blockage, especially among newborns
- Foul-smelling, greasy stools
- Severe or chronic constipation
6 Respiratory Symptoms Of CF
The sticky mucus typical to cystic fibrosis can plug the tubes that specialise in carrying air in and out of the lungs. This can lead to symptoms like:
- Inflamed nasal passages or a stuffy nose
- Recurrent sinusitis
- Persistent cough that produces thick mucus, known as sputum
- Exercise intolerance
- Frequent lung infections
Read: 16 Lifestyle Mantras To Treat Stress-Induced Diabetes, By Nutritionist Dr Kumar
Cystic Fibrosis In Women
Both males and females can get affected by cystic fibrosis. About half of the patients diagnosed with this condition are females. However, they experience worse outcomes than males till the age of 20 years. Post that, women and men experience almost equal outcomes. Here’s how CF can affect women.
CF & Puberty
- Girls with CF may hit puberty comparatively late than other girls without the medical condition
- Vaginal discharge in girls with CF is thicker, which may be a side-effect of the prescribed medications. This is normal. However, symptoms like itching, irritation, and burning around the vagina or a new discharge may indicate a yeast infection. This may be the case among girls on antibiotics because of CF. in such cases, the doctor should be consulted immediately.
- Maintaining a healthy body weight through a regular fitness routine and a balanced diet is essential during puberty. Follow a high-calorie, high-fat diet that is high on vitamins to stay healthy during puberty.
CF & Menstruation
- Girls with CF may get their periods about 6 months later than their counterparts without the medical condition. The first period may get further delayed if the patient is underweight or is very ill.
- The menstrual cycles and the amount of blood flow during periods may frequently fluctuate among girls with CF.
- CF symptoms may get worse during periods. This may be because of changes in the levels of hormones that the female body makes during puberty. These symptoms may include more cough or sputum. Following the recommended treatments and clearance of airways becomes even more essential during periods. If the symptoms worsen, it is important to consult the doctor.
- If you are more than 15 years old and haven’t got your first period yet or it’s been more than 3 years since your breasts have started developing, see a doctor.
Read: 7 Effective Food Sources For Endometriosis Diet, By Nutritionist Dr Anjali Kumar
CF & Pregnancy
- CF usually does not cause infertility among women. However, the patient will have to be closely monitored during these 9 months to ensure the safety of both the mother and the baby.
- Apart from seeing the Pulmonologist who treats CF, it is recommended that you see a high-risk obstetrician while you plan to conceive. An obstetrician can determine whether it is safe for you to plan a baby, analyse your current health, and also guide you through your pregnancy.
- However, there might be complications that you may face due to CF. These complications include premature delivery, hypertension or high blood pressure, gestational diabetes, and nutritional deficiency that may affect the normal growth of the foetus in the womb.
4 Possible Cystic Fibrosis Treatment Methods
CF is not curable yet. However, there are various treatment methods that can help in keeping its symptoms under check. These treatment options are:
Medications aim to thin the mucus, open airways, help the body absorb nutrients from food, and prevent infections.
- Antibiotics: These may be prescribed to treat or prevent lung infections and enable the organ to function efficiently.
- Bronchodilators: These relax and open the airways.
- Anti-inflammatory Medications: They control swellings that block airways.
- Mucus Thinners: They help in thinning the thick and sticky mucus, clearing them off the airways.
- CFTR Modulators: They help in improving the efficiency of the lungs and also assist in gaining weight.
2. Airway Clearance Techniques
- Oscillating Devices: The patient needs to breathe into this special device that makes the airways oscillate or vibrate. This loosens the mucus, enabling the patient to cough up.
- Percussion Or Chest Therapy: The therapist taps on the patient’s chest or back to clear the lungs of mucus.
3. Pulmonary Rehabilitation
This long-term program may be recommended to improve lung function. These may include:
- Breathing techniques to enable loosen mucus and aid in breathing
- Physical exercises to eliminate the symptoms
- Nutritional counselling
- Educating and informing about the condition
4. Surgical & Other Procedures
The available surgical treatment options are as below:
- Oxygen Therapy: This therapy may be recommended to improve blood oxygen levels. The procedure helps the patient breathe pure oxygen to prevent pulmonary hypertension or high blood pressure in the lungs.
- Non-Invasive Ventilation: This involves using a mask for the mouth or nose to exert positive pressure in the lungs and airways when the patient breathes. Often used in combination with oxygen therapy, this can aid in improving air exchange in the lungs. This may also be beneficial for airway clearance.
- Nasal & Sinus Surgery: If the patient has nasal polyps that obstruct breathing, the doctor may remove these polyps through surgery to help the patient breathe. Chronic or recurrent sinusitis can be treated through a sinus surgery.
- Bowel Surgery: This helps in removing blockage in the bowel, if any.
- Feeding Tube: Cystic fibrosis impedes the absorption of nutrients from food and also interferes with digestion, as mentioned earlier. So the doctor may insert a temporary tube to ensure sufficient nutrition for the body. The tube can be inserted through the nose and guided till the stomach, or may be implanted surgically in the abdomen. This does not prevent eating by mouth.
- Lung Transplant: For life-threatening breathing problems, the option of lung transplant may be recommended by the doctor.
- Liver Transplant: Liver transplant may be a treatment option for severe cystic fibrosis-related liver diseases like cirrhosis. In certain cases, a liver transplant may be combined with pancreas or lung transplants.
For women, cystic fibrosis may pose some challenges during puberty, periods, and pregnancy. However, it does not affect the quality of life when the condition is treated effectively. Consult your pulmonologist at regular intervals, and also see an obstetrician, if you are planning to conceive. Introduce the lifestyle changes as recommended by your doctor, while getting treated for the condition, to keep its symptoms under control.
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