Cystic fibrosis or Mucoviscidosis is a type of genetic disorder that mostly affects the lungs; however the liver, pancreas, intestine and kidneys or in other words, the digestive and excretory system too is likely to be affected. Cystic fibrosis or Mucoviscidosis associates its expressions through breathing difficulties as well as coughing up sputum, in case of long term issues. More than 70,000 people are suffering from Cystic fibrosis or Mucoviscidosis worldwide and every year, 100 new cases of Cystic fibrosis or Mucoviscidosis are being diagnosed. Amongst these, more than 75% people are diagnosed by the age of 2 years and the rest are diagnosed with Cystic fibrosis or Mucoviscidosis within 18 years.
The self-care for Cystic fibrosis or Mucoviscidosis is only possible when one has the knowledge of what the disease is, the symptoms and prevention of the symptoms and from there arises the self-care.
What is Cystic Fibrosis or Mucoviscidosis All About?
The basic cause of Cystic fibrosis or Mucoviscidosis is the mutations in both the copies of gene for the protein CFTR or cystic fibrosis trans-membrane conductance regulator. The disease happens when the CFTR is the regulator that produces digestive fluids, sweat as well as mucus becomes dysfunctional due to the mutation. All the thin secretions in the body become thick. Usually, those who have only one mutated gene and the other one is normal, he or she is considered to be only a carrier of this disease and will not be affected by the disease itself.
This inherited genetic condition is a common phenomenon in the white people, especially people from the Northern European ancestry. However, African-Americans, Hispanics and some Native Americans are also seen to suffer from this condition.
What Are The Symptoms of Cystic Fibrosis or Mucoviscidosis?
This genetic disorder that a person is born with has different severity levels. However, the symptoms of Cystic fibrosis or Mucoviscidosis can clearly denote whether the disease is present or not. The infants or babies with Cystic fibrosis or Mucoviscidosis can be, however, screened even before the signs and symptoms start to appear. But, mostly the symptoms help the doctors to physically determine the possibility of this disease. Moreover, only when one knows what the symptoms are, the diagnosis is possible and so is the recurrent occurrence of symptoms and worsening of the condition. The symptoms of Cystic fibrosis or Mucoviscidosis are:
- Persistent production of thick mucus or sputum during coughing that persists too
- Breathlessness and wheezing
- Repeated lung infection
- Reduced ability to exercise
- Stuffy nose and inflamed nasal passages, polyps inside nostrils
- Greasy stool with foul smell
- Severe constipation and rectal prolapse
- Intestinal blockage
- Poor weight gain and growth
Life Expectancy of Cystic Fibrosis or Mucoviscidosis
Cystic fibrosis or Mucoviscidosis is more common in children, especially in infants as they are generally born with this genetic disorder. However, sometimes even the adults also suffer from this disease. In the previous decades, the quality of life for the patients suffering from Cystic fibrosis or Mucoviscidosis was not quite good, but with recent advancements of treatment procedures, people suffering from Cystic fibrosis or Mucoviscidosis can now live up to the age of 20s to 30s and sometimes even up to 40s. However, there is no cure for Cystic fibrosis or Mucoviscidosis.
Self-Care of Cystic Fibrosis or Mucoviscidosis:
With considerable command over the proper diet and exercise regime, Cystic fibrosis or Mucoviscidosis can well be managed right at home, with a positive increase in the life span. Though it requires the guidance of your general physician as well as cystic fibrosis clinicians, but the self management measures are easy to be followed all by yourself.
As children are the most vulnerable victims of Cystic fibrosis or Mucoviscidosis, even before they understand what is happening to them, they start suffering from its adversities. However, the self management program helps the children to cope with their problems, limitations as well as take the necessary measures all by themselves, through which they can develop their knowledge, skills and behaviours. Although most of the time, children suffering from Cystic fibrosis or Mucoviscidosis have to undergo health service programmes and supports, as they are unaware of their condition and it cannot be assumed out of them that they would understand the responsibility of their participation in the self management programmes, studies have shown that self care of the children suffering from Cystic fibrosis or Mucoviscidosis has improved the quality of their lives and increase their life span.
Self Care for Infants and Toddlers Suffering From Cystic Fibrosis or Mucoviscidosis:
As your child is diagnosed with Cystic fibrosis or Mucoviscidosis, it will be difficult for you to accept it. However, if as a parent you start to take part in the self care programme for your child, they too will soon participate in the same. You must try to adjust with this fact and try to enjoy every moment with your baby. As he grows up, teach him all his personal information. You must spend ample time with him and as his exercise regime and medicinal regime starts, make it as interesting and part of life as possible. This will make sure that as your child grows up, he will:
- Accept his daily physiotherapy
- Help in setting up the treatment area by bringing towels, medicines and pillows
- Keep an inhaler with himself
- Learn to hold a mask on face when using a nebuliser.
Self-Care for Preschoolers and Schoolers Suffering From Cystic Fibrosis or Mucoviscidosis:
By the time your child reaches this age, he will understand what his limitations are and what his daily routine is. Make it a habit for your child to take the medicines and exercise regularly. However, as a parent, you must not limit his life in too many boundaries. Let him spend time and play with other non Cystic fibrosis or Mucoviscidosis children. With proper self care regimes, they will be able to:
- Describe Cystic fibrosis or Mucoviscidosis in a few sentences, so that when they meet non Cystic fibrosis or Mucoviscidosis people, they have the ability to manage their survival on their own.
- Remember the contact information of their friends, so that in case of emergency, as a parent you can get in touch with them.
- Learn the benefits of balanced diet and meal
- Understand the need for hygiene, dressing and other self care.
Self-Care for Adolescent or High Schoolers Suffering From Cystic Fibrosis or Mucoviscidosis:
During this time, the adolescent will learn at a self care programme about what is normal for their body and what is not. Identifying their symptoms and be able to deal with them on their own at least in the initial stages is what is taught in these sessions. The management programme will teach the adolescent suffering from Cystic fibrosis or Mucoviscidosis to:
- Understand their lungs, how they work and what are the 'bugs' that infect them
- Learn why airway clearing is necessary for them
- Know how their body uses food and why it is important for them to take food high in energy.
Self Care For Young Adults Suffering From Cystic Fibrosis or Mucoviscidosis:
This programme helps the young adults suffering from Cystic fibrosis or Mucoviscidosis to:
- Know their dosage of medicines, why they take them and when to take
- Organise them for refilling
- Make healthy lifestyle choices
- Think of their life goal
Diet Management For Cystic Fibrosis or Mucoviscidosis:
When self care is being concerned, diet plays a crucial role in Cystic fibrosis or Mucoviscidosis. This is because; keeping a steady and healthy body weight will help you to fight back possible infection. It is also necessary to be prepared to fight the infection when it attacks you and stay strong enough to reserve the energy for such circumstances. However, keeping control over what the Cystic fibrosis or Mucoviscidosis patient eats is very important by the time he grows up to the age of an adult. This is because the mucus that is built up in the body will block the duct of his pancreas that is instrumental in secreting digestive enzymes. Hence, whatever you eat, will not be digested completely and all the nutrients will not be absorbed by the body. Therefore, the diet should be rich in calories.
The diet of a Cystic fibrosis or Mucoviscidosis patient must include:
- High calorie nutritional supplement
- Highly fibrous food to avoid intestinal blockage
- Fat soluble vitamin dosage
- High level of salt, especially during hot weather
- Lots of drinks to keep the mucus levels thin.
As you will need more energy, you must eat 110% to 200% more calories than a person without Cystic fibrosis or Mucoviscidosis. The BMI should be above 50% of what your age would require on an average. Below 25% for your age will mean you are at risk.
Diets for Babies and Children Suffering From Cystic Fibrosis or Mucoviscidosis:
- Babies should be on general breastfeeding at the initial stage
- As they move to solids, they should be given extra salt
- Salt should not be mixed raw in their food or drink, the GP should recommend the possible ways
- Take pancreatic enzymes for additional support to digest the food the baby eats
- To compensate the lost vitamin A, D and E through their stool, vitamin supplements should be given.
Diet for Adults Suffering From Cystic Fibrosis or Mucoviscidosis:
- Eat several times a day
- Take more fatty fish and nuts and eat plenty of eggs, meat and fish
- In case you are not gaining weight due to illness, you must be tube fed at the hospitals
- Continue taking multivitamin pills.
Exercise as a Regular Self Care Regime For Cystic fibrosis or Mucoviscidosis:
Regular exercising is probably the best way through which you can stay healthy even with cystic fibrosis. This will help your mucus to be released through the airways. Even your heart and lungs will be strengthened through exercise. Participation in sports will help them to stay healthy as well as boost up their energy and confidence levels. Even simple walking, swimming and biking will be helpful. If your child has Cystic fibrosis or Mucoviscidosis, make sure that you let his school know that sports and exercise are encouraged in this disease and he must be allowed to participate.
If all these measures the child suffering from Cystic fibrosis or Mucoviscidosis practices right from his infancy till his adulthood, he can continue to stay healthy. These practices will gradually become a habit and part of his life, and he will well manage his disease all by himself. Without any help, he will feel confident and this will make his lifespan enjoyable.