According to the Cystic Fibrosis Foundation Patient Registry, about 30,000 people in the United States live with cystic fibrosis. Doctors diagnose about 1,000 new cases of this hereditary disease each year.
In addition, the Children’s Hospital of Pittsburgh reported that 10 million Americans carry the gene for cystic fibrosis but do not have the disease themselves.
While there is not a cure for this disease, advances in treatments for cystic fibrosis have extended the life expectancy of people living with cystic fibrosis. Supportive care can also improve the quality of life for people with cystic fibrosis, empowering them to live full lives.
In honor of Cystic Fibrosis Month, here’s a look at what cystic fibrosis is, how it affects people, and what types of care and treatment can help to manage the symptoms.
What is cystic fibrosis?
Cystic fibrosis is a disease that is transmitted genetically and affects the glands in the body that create mucus.
Normally, mucus is a watery fluid, but in people with cystic fibrosis, this substance becomes thin and sticky. This means that instead of flowing through the lungs and other organs, the mucous lodges in the airways and ducts, thus blocking these passageways. The location of the blockage determines the type of symptoms the person with cystic fibrosis experiences and which organs will be affected.
Some of the organs affected by cystic fibrosis include:
- Lungs, resulting in shallow breathing and risk of more severe types of respiratory distress or infections
- Pancreas, affecting the process in which nutrients are absorbed by the body and potential malnutrition
- Sweat glands, resulting in disruption to the balance of minerals in the blood, which affects heart functioning leading to palpitations, dehydration, and fatigue
What triggers cystic fibrosis flare-ups?
Thanks to advances in the diagnosis and treatment of cystic fibrosis, people are living longer lives with cystic fibrosis. However, people will continue to have symptom flare-ups, which means their caregivers need to have the flexibility to ensure their loved one is monitored and following their treatment protocols.
Flare-ups can be triggered by disruptions to the respiratory system, such as chest or lung infections or inhaling higher amounts of pollutants. Symptoms of flare-ups include:
- Frequent coughing that may produce phlegm
- Repeated lung infections
- Shortness of breath
- Obstructed airways
Caring for cystic fibrosis
If you notice any of those symptoms, the best form of treatment that can be completed at home is Manual Chest Physical Therapy. This helps to break up mucus buildup in the patient’s lungs.
ACT is often prescribed alongside other treatment options, including inhaled bronchodilators to help relax the muscles and widen airways before performing ACT, antibiotics to fight infection, and certain medications that help thin mucus so it’s easier to loosen.
Being able to provide relief at home is a huge benefit for any person with CF, but especially for children. Being rushed to hospital during a flare-up disrupts learning and social activities and means that it’s harder to live a more regular routine. What’s more, being able to clear the airways in the comfort and familiarity of their own home is a much more reassuring experience for patients of all ages.
Home caregivers can also support people living with CF in a variety of different ways, depending on how the disease affects them. If cystic fibrosis has damaged the person’s sweat glands, they may have a port to which an IV can be attached to deliver fluids and minerals.
Families also often rely upon a PCA to help with bathing, staying on top of nutrition, and other self-care tasks, as well as housekeeping and providing transportation to doctor appointments during flare-ups.
This Cystic Fibrosis Awareness Month, Best Care is here to offer you guidance and information about how cystic fibrosis home care services can greatly improve the quality of life for children and adults living with this debilitating disease.
Stay Connected to Best Care
Best Care is ready to help family caregivers across Minnesota with support and guidance to provide quality home care for their child living with CF. Contact our team of home care professionals to learn more.