Cystic fibrosis (CF) is among the most common and severe genetic diseases in the United States, with about 1,000 new cases diagnosed every year. The disease is caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. It affects the secretory glands, which are responsible for the production of sweat and mucus. The mucus of healthy people is a slippery and watery substance that keeps the organs moist, but in CF patients, mucus is thick and sticky. The respiratory, pancreatic, gastrointestinal, and reproductive systems are affected by the genetic defect, and the accumulation of mucus makes it difficult to breathe properly.
Cystic fibrosis is a chronic disease for which there is currently no cure. However, improvements in diagnosis have made early treatment plans possible. Newborn screening for CF has become widely conducted, and the age of diagnosis has, in many cases, decreased to 2 years old. As a result, young children are diagnosed with the disease, and their parents or other loved ones become their caregivers — a task with many challenges.
Caring for a Child with Cystic Fibrosis: Identifying the Signs
Being an inherited disease, cystic fibrosis runs in the family, but in some cases families may not know it and the diagnosis may be a shock. From the start and during the entire process of caring for a CF patient, knowing what to expect and what is not normal can be helpful. It prevents caregivers from being afraid or surprised when the symptoms of the disease manifest. When medicated, children tend to suffer from mild symptoms, which can become more severe over time.
Parents should call a doctor if the CF patient gets worse for no apparent reason, which may include increased coughing, new or worse wheezing, more trouble breathing than usual, loss of weight or not gaining weight, or additional symptoms not discussed by the doctor. Common symptoms in older patients include persistent cough that produces thick (sputum) mucus, wheezing, breathlessness, exercise intolerance, repeated lung infections, inflamed nasal passages or a stuffy nose, foul-smelling and greasy stools, poor weight gain and growth, intestinal blockage, particularly in newborns (meconium ileus), and severe constipation.
Caring for a Child with Cystic Fibrosis: Medication and Therapies
Particularly with small children, caregivers are responsible for giving patients their medication and helping with therapies. Taking care of a child with cystic fibrosis when healthy is a task that can take between one and two hours a day, including medicines and airway clearance. When the child experiences disease exacerbations or suffers from any other condition, more time is needed for care and the patient may need IV medication.
Due to the demands of caring for children with a chronic disease, in some cases one of the parents stops working or they seek professional help full-time. Daily care and treatments vary among children, but it can include lung clearance therapies like chest physiotherapy (CPT) and vest therapy, bronchodilators, steroids, anti-inflammatory medicines, or antibiotics. In addition, patients need to take enzymes, and mineral and vitamin supplements to help with digestion and calorie absorption.
Daily Life of a Family with Cystic Fibrosis
While the patient is still a baby, daily life has numerous demands, but parents tend to keep them home to watch over their development and the development of the disease. In addition to medication, one of the most important aspects to keep a CF patient healthy is nutrition, and the meals of a family affected by cystic fibrosis can be slightly different. Patients need to drink plenty of water to keep hydrated and to consume foods higher in calories and proteins to avoid weight loss and malnutrition.
As patients grows older and they attend school, it can be stressful in several ways. For children with cystic fibrosis, the first day of elementary, middle, and high school are transitional times with additional responsibility for managing the disease. It might be helpful to discuss strategies with the medical care team and teachers. As an alternative, some parents opt for home-schooling their children, which can be managed with the help of resources offered by the Cystic Fibrosis Foundation.
Lifestyle Alterations in a Family Affected by Cystic Fibrosis
Cystic fibrosis is a disease that affects not only the patient but the entire family, which needs to adapt to a new reality. Continually seeking knowledge and news about the disease can help patients and families stay updated and prepared for any unexpected event. Even if one parent is more responsible for taking care of the child with cystic fibrosis, it is important that the entire family is aware of the disease and does not neglect routine care like check-ups and immunizations.
People with cystic fibrosis are particularly likely to develop infections, and even a cold can become problem, since viruses can have a major impact on health. Therefore, it is important to avoid exposing the family to illness. Smoking should also be avoided. Smoking, even secondhand smoke, has severe consequences, and in the case of CF patients, who work daily to slow lung damage, it is even worse.
Emotional Challenges for Caregivers of Cystic Fibrosis Patients
Caring for a patient with cystic fibrosis is not easy and demands commitment. In addition to the physical manifestations of the disease, patients are also more likely to develop emotional issues. Caregivers can also suffer emotional challenges. Even before the diagnosis, the process of seeking the right physicians and look for answers can be frustrating. When the diagnosis is confirmed, parents may feel shock, denial, and disbelief that their child has CF, and sadness for their hopes that their child would be fine and life would go back to normal.
Anger, frustration, and a feeling of unfairness about the situation is also common, as well as fear about the their child’s health and life span. Parents can also end up depressed, a deep and long-term feeling of sadness about either the diagnosis or the demands of life with it. Siblings are also affected by the disease and parents need to be particular careful since brothers and sisters of children with CF may feel left out, neglected, or jealous, due to the extra care given to their sick sibling.
Coping with Cystic Fibrosis in a Loved One: How Not to Be Alone
The most important thing for parents of children with cystic fibrosis to remember is that they are not alone. Other parents share the same experiences, challenges, and emotional issues. There are support groups not only for patients, but also for caregivers, in which people can share their experiences and listen to others. Organizations and specialized CF centers can help offer support. Engaging with the work of organizations and speaking out about the disease can also help families gain new hope and decrease their sense of feeling alone.
Understanding that cystic fibrosis is a lifelong disease with constant changes can be a first step in dealing with it and recognizing that long-term help is needed.
Note: Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.