Have you ever heard of Cystic fibrosis (CF)? Probably not. It’s one of the most dangerous illnesses of recent times, even more dangerous than COVID-19. You may not believe, but in Pakistan we have many children suffering from this condition and unfortunately, people lack awareness about it or how to manage it. Unfortunately, most children suffering from cystic fibrosis pass away without even being diagnosed despite the fact that early diagnosis means early treatment and better health, and consequently may save lives. CF severely impacts the growth, education, moods, self-esteem and social life of the affected children. In most of the cases, they cannot have normal life. Here, I would like to share my experience so that more people can be sensitized about this disease.
CF being a genetic disorder is caused by mutations in a single gene. CF affects the way your body makes mucus, a substance that helps your organs and systems work. In a normal person, mucus should be thin and slippery, but in a person with CF, it becomes thick and glue-like. This blocks tubes and ducts throughout your body. Over time, this thick mucus builds up inside your airways, making breathing difficult. It can also cause severe lung damage like cysts (fluid-filled sacs) and fibrosis (scar tissue). That is how CF got its name and people suffering from CF are called CFers. It also affects the small intestine. In people with CF, the sticky mucus blocks ducts in the pancreas and prevents enzymes from reaching the small intestine to digest food. Undigested food in the intestines can cause pain, cramping, gas, and either loose, greasy, floating stools or constipation and blockages. The mucus traps germs and leads to infections. Therefore, most of CFers are likely to suffer from recurrent pneumonia and diarrhoea.
Categories/ Levels of Cystic Fibrosis
There are many variants of CF which have been categorized into various shades of mild to severe in different children. Those suffering from mild CF will not even know that they have it and will continue living their normal life until they catch some disease, which is related to either lungs or small intestine and their condition becomes aggravated. Sometimes even doctors do not understand why a patient is not responding to medication.
Those suffering from medium level CF, are likely to face allergies from various things, as most asthmatic patients do. They can easily be confused with being asthmatic so the there is a chance of misdiagnosis that could lead to worsening of the patient’s condition. The worst category of the CF is where the patients will never be able to gain weight, get hospitalised frequently with chest infections and giardiasis/diarrhoea. I am so saddened to write that in Pakistan, this is the most common level of CF.
In the advanced stage, chronic progressive pulmonary disease and respiratory failure remain the major cause of morbidity and mortality. End-stage lung disease is characterized by cysts, abscesses, and fibrosis of lungs and airways. Patients frequently die from overwhelming lung infections.
In western world, they have started establishing CF clinics to cater to the needs of CFers. However, in Pakistan we do not have any such facility. In most cases, suspected cases are shown to child specialists and most of them do not have any expertise in CF handling, which a pulmonologist can have. Unfortunately, there is no CF clinic/facility to manage the needs/emergencies of CF children.
Since early diagnosis is important, mothers should read around and try to determine if their child may have CF. Some signs to look out for are poor growth, repeated lung or sinus infections or both. If you feel your child is showing these signs, consult a doctor who would then recommend the relevant tests. The standard test for CF is the ‘Sweat Test’, which measures the amount of salt in the sweat. Because newborns do not sweat, a test Immunoreactive Trypsinogen (IRT) may be used instead. This test involves drawing blood and looking for a specific protein. A positive IRT test may be confirmed at a later date with genetic testing or a sweat test. Other tools that help with diagnosis include chest X-rays and Lung Function Tests (LFTs), which may show lung problems common for CF patients. Tests of stool samples may show digestive problems.
Unfortunately, no cure has been discovered until now for CF anywhere in the world. However, there are various methods to manage the disorder. These methods include chest physiotherapy by using nebulizers or inhalers that slow down chest infections by removing the hard mucus from lungs and breathing vessels. Moreover, use of digestive enzymes prevent having frequent episodes of diarrhoea. Beyond that, there are some precautions and tips that can help a CFer in enjoying a relatively better life.
Chest physiotherapy plays a major role in every CFer’s life. We all have a natural cycle in which our lungs create mucus and dissolve it naturally, but a CFer produces thick mucus, which is hard to dissolve and clogs the air pipes giving the patient bouts of coughing. Therefore, it is necessary to remove the hardening mucus otherwise the layers of mucus not only facilitate bacteria but also result in shortness of breath and uneasiness. It is mandatory for all serious CF patients to keep their lungs clean by doing physiotherapy before going to bed and after waking up in the morning. Mothers will have to help the younger CF patients. However, as they grow up they must learn to handle their physiotherapy themselves. Four basic steps of chest physiotherapy for a CFer are:
• Take a Ventolin inhaler or a nebulizer to open the airways
• Take hypertonic saline nebulization, which helps in the dilution of mucus
• Take a deep breath and hold for 5 seconds then exhale powerfully, do this cycle 5 times and cough out phlegm in a disposable container. Repeat about 10 times
• Take an antibiotic nebulization or inhaler to relax lungs and kill bacteria
As I learnt from doctors, pancreas helps to break our food in tiny parts and filter all the nutrients from the food. Unfortunately, in CFers the thick mucus in their pancreas blocks pancreatic enzymes from entering the small intestine. The lack of pancreatic enzymes means that digestive tract has to pass partially undigested food therefore, do not forget to give the prescribed enzymes to your CF child so he/she is able to get enough nutrition from the food. It will prevent from severe abdominal pain and it will help in the digestion of food properly.
Although, CF is a severe condition that needs daily care, there are many ways to treat it, and there has been great improvement in those treatments over the years. People who have CF now can expect to live a much longer life than those who had it in the past.
All the precautions which we take for COVID-19, like frequently washing hands, sanitising, keeping distance from ill people, etc., CFers need to take these measures for their lifetime. For people with CF, being close to others with disease puts them at greater risk of getting and spreading dangerous germs and bacteria. This is called cross‐infection. Not only these dangerous germs are difficult to treat, but they can also lead to worsening symptoms and faster decline in lung functioning.
Some CFers may be allergic to carpets, stuffed toys, polyester, curtains, perfumes, deodorants or makeup, etc. CFers should avoid the areas where there is fresh paint, barbecue smoke, or pesticide sprays. CFers should wear cotton clothes and socks. Materials like wool, polyester, nylon, silk, etc., not only give them skin rash but can also result in bouts of coughing. A CFer’s blanket should be made of cotton and even if it is not made of cotton, it must be covered with a cotton cover so that the patient can be protected from allergic reactions. Due to the allergy, taking a flu shot every year is compulsory for them. Dust or dirt are no less than a threat to a CFer because they might cause the patient to have shortness of breath and cough.
CFers should avoid eating outside and instead eat homemade, hygienic food, although one can eat food from hygienic trusted restaurants. A CFer should always be at least six feet from another CFer as both are susceptible to different infections and there can be a bacterial exchange among them. If there is a patient of flu or viral infection in the house, the CF patient should be kept away from them. A normal person will recover from the virus after a week, but the CF patient may end up in the hospital. A CFer should take good care of his fitness. Football, swimming, gym workout, etc., are very helpful in the fight against this disease.
Parenting/ Social Pressures
CF itself is not only testing for the child suffering from it but also a difficult journey for the parents. Watching your child suffer, and struggling for breath is heart-breaking. Since it is a hereditary disease, it has often been seen that after the diagnosis of this disease in a child, parents start taking precautions against having more children lest the next child also suffers from this disease. For parents, finding this disease in their genes is nothing less than a nightmare. Although, medical science has progressed so much that in the fourth month of pregnancy, a specific test can be used to find out whether the child is also suffering from this disease or not. CF brings mental, emotional and financial difficulties for parents. CF is very expensive to treat as there is no cure. Frequent hospital admissions, expensive drugs, repeated use of oxygen makes the hospital bill astronomical.
In addition, parents are concerned about their child’s education. However, be assured that if your CFer is taken good care of, he can participate in all the activities that children without CF can participate in. They should be encouraged to work hard in school and plan for their future. However, there may be times when having CF interferes with their daily routine or their ability to meet all expectations. Children and adults with CF also have special needs that should be prioritized for better physical and mental health to give them the best chance at success.
There are many facilities available to CF children in other countries. Their school hours are short and they are provided with all the facilities that they need to keep up with the world, while fighting their illness. In Pakistan, however, because of the relative lack of awareness about the disease, much needs to be done. There is an urgent need for the establishment of CF centres and to educate people about this deadly disease. In addition, all educational institutions should develop a separate program for children with CF and similar diseases.
Social Media Support Groups
Since this disease is not a common disease, sometimes the patient and their caregivers start thinking of themselves as alone. That cannot be further from the truth; there are many out there who are going through what you are and thanks to various social media platforms, you can now connect with them. These support groups offer a space to unload yourself emotionally with the feeling that the others there understand where you are coming from. As everyone is in the same boat, it becomes a little easier to understand each other’s mental and physical pain. Living with the disease becomes a little easier in the light of each other’s experiences. Moreover, many groups provide support in the form of medication and equipment needed for the patients. HH
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