6 Tips for Caring for a Family Member with Cystic Fibrosis
Cystic fibrosis (CF) is among the most common and severe genetic diseases in the United States, with about 1,000 new cases every year. The disease is caused by a defect in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and it affects the secretory glands, which are responsible for the production of sweat and mucus. Healthy people’s mucus 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 provokes difficulties in properly breathing.
Cystic fibrosis is a chronic disease for which there is currently no cure. However, improvements in diagnosis have made possible the definition of an early treatment plan. Newborn screening for CF has become widely conducted and the age of diagnosis has decreased to two years old. Therefore, in the majority of the cases, patients are diagnosed as children and their parents or other loved ones become their caregivers, a task with numerous challenges.
1. Caring for a Child: Identifying the Signs
Being an inherited disease, cystic fibrosis runs in the family, but in some cases, families may not realize this and the diagnosis may be a shock. Right from the start and during the entire process of caring for a patient with cystic fibrosis, knowing what to expect and what isn’t normal can be helpful. It prevents caregivers from being scared or surprised with the manifestations of the disease. 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.
2. Caring for a Child: 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 daily, including medicines and airway clearance. When the child experiences exacerbation of the disease or suffers from any other condition, the time raises 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. 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, mineral and vitamin supplements to help with digestion and calorie absorption.
3. Quotidian Life of a Family with CF
While the patient is still a baby, daily life has numerous demands, but parents tend to keep them home to watch for 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 for 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 the patient grows older, he or she will start school, which can be a factor of stress in several ways. For children with cystic fibrosis, the first day of elementary, middle and high school are moments of transition which hold additional responsibilities of managing the disease. Therefore, it might be helpful to discuss strategies with the medical care team and teachers. As an alternative, some parents opt for having their children home-schooled, which can be managed with the help of resources offered by the Cystic Fibrosis Foundation.
4. Lifestyle Alterations in a Family Affected by CF
Cystic fibrosis is a disease which affects not only the patient but the entire family, who will need to adapt to a new everyday reality. Continually seeking knowledge and news about the disease can help patients and families keep updated and ready for any 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 features of the disease and don’t neglect routine care like check-ups and immunizations.
People with cystic fibrosis are particularly likely to develop infections, and even just a cold can be problematic since viruses can have a major impact on health. Therefore, it is important to avoid exposing the whole family to illness. Similarly, smoking should be avoided. Smoking has severe consequences, even second-hand smoke and in the case of CF patients, who work daily to slow lung damage, it is even worse.
5. Emotional Challenges for Caregivers
As noted, caring for a patient with cystic fibrosis is not easy and it demands commitment. In addition to the physical features of the disease, patients are also more likely to develop emotional issues. Similarly, caregivers can also suffer emotional challenges. Even before the diagnosis, the process of seeking different physicians and looking for an answer can be frustrating. When the diagnosis is confirmed, parents may feel shock, denial and disbelief their child has CF, and grief as they mourn their hopes that their child would be fine and life would go back to normal.
Anger due to the situation and feeling what is happing is unfair is also common, as well as fear about their child’s health and life span. In addition, 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 particularly careful since brothers and sisters of children with CF may feel left out, neglected or jealous, due to the extra care given to their sibling and time spent with them.
6. Coping with CF in a Loved One: How Not to Be Alone
The most important thing for parents of children with cystic fibrosis is to remember 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 hear others’. Organizations and specialized CF centers can help find support. Engaging with the work of organizations and speaking out about the disease can also help families gain new hope and lessen their sense of aloneness.
Understanding that cystic fibrosis is a life-long disease with constant changes can be the first step to dealing with it and recognizing that long-term help is needed. In addition, parents should understand that the development of cystic fibrosis occurs when two faulty genes are inherited. This does not mean all siblings will suffer from it, but it is recommended that parents get tested to see if they carry CF and knowing what the probabilities are before having more children.
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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.
