Cystic Fibrosis Nursing

Cystic Fibrosis Nursing

What is Cystic Fibrosis? 

Cystic Fibrosis is an inherited disorder that causes severe damage to the lungs, digestive system and other organs of the human body.

Cystic Fibrosis damages cells that are responsible for generating mucus, digestive juice and sweat. These types of secreted fluids are defined as slippery and thin; however, for people with Cystic Fibrosis, the fluids are thick and sticky. As opposed to carrying out their intended functions of lubrication, the secretion from these fluids plugs up the passageways, ducts, and tubes particularly in the pancreases and the lungs.

Cystic Fibrosis Symptoms, Causes, Treatment

The signs and symptoms vary depending on the severity of the disease. People with Cystic Fibrosis have high levels of salt in the sweat and mostly the signs and symptoms affect the digestive and the respiratory system. Adults diagnosed with this condition have high chances of atypical symptoms including; recurring pneumonia, infertility, and pancreatitis.

Respiratory Signs and Symptoms

The thick and sticky mucus linked with Cystic Fibrosis clogs the tubes carrying air to and from the lungs and is associated with the following signs and symptoms;  Cystic Fibrosis Nursing

• stuffy nose or inflated nasal passage
• persistent lung infection
• Intolerance to exercise
• breathlessness
• Wheezing
• production of thick mucus through persistent coughing

Digestive Signs and Symptoms

The thick mucus also has the ability to block the tubes carrying enzymes used in the digestive process from various parts such as small intestine and pancreas. As a result, the intestines lack the capability to absorb nutrients from food and this can be identified with the following signs and symptoms: Cystic Fibrosis Nursing

• severe constipation
• blockage of intestine particularly among the newborns
• poor growth and weight gain
• Greasy stools that may be characterized with foul-smelling

Causes Cystic Fibrosis

Cystic Fibrosis is mainly caused by defection or mutation in the genes which causes alterations in the proteins responsible for regulating the movement of salts in and out of the cells. Cystic Fibrosis Nursing

This leads to sticky and thick mucus in the reproductive, digestive and respiratory systems and increased concentration of salt in the sweat.  In addition, various defects can take place in the gene which is termed as the gene mutation that is linked with the rigorousness of the disorder.

For children to experience this condition, they have to inherit a copy of the gene from each parent for the disease to develop. However, in case the kid only inherits one copy, they cannot develop Cystic Fibrosis condition. Interestingly, such kids will be carries and they will possibly pass the gene to their children.

Treatment of Cystic Fibrosis

Though no cure has been established for Cystic Fibrosis, there are various treatments that can be used to relieve the symptoms and minimize complication risks. Cystic Fibrosis Nursing

Some of these medications include;

• prescription of anti-biotic to help get rid of lung infection and prevent an attack from other infections in the future
• medication on muscle thinning to make muscles less stick and maintain then thinner
• use of Nonsteroidal anti-inflammatory drugs (NSAIDs) including; indomethacin and ibuprofen to reduce fever and pain associated with Cystic Fibrosis. Cystic Fibrosis Nursing
• Use of Bronchodilators to help relax the muscles that are around the tubes carrying air to and from the lungs
• Bowel surgery and lung transplant.
• Chest physical therapy

Major Cystic Fibrosis Nursing Care Plans

The major nursing care plans for Cystic Fibrosis include the following; preventing complication, ensuring there is adequate nutrition, articulating the importance of sufficient dietary and fluid intake, promoting measures that will enhance of pulmonary secretions, and ensuring there is adequacy of oxygenation in the lungs.

The Role of the Cystic Fibrosis Nurse Specialist

Cystic fibrosis nurse specialist plays the following roles;

Advocacy: due to the many demands made by the patient and the CF team, family and patient advocacy have been established to be a vital role of the nurse specialist. This ensures patient satisfaction and well-being.

Clinical management: the nurse specialist must assume a central role in decision making of the patient and monitoring care. Besides the practical responsibilities, the day to day care of the nurse specialist is to ensure they are responsibly aware of the treatment modalities that are used on the management of CF by ensuring that each patient gets an optimum can that matches personal needs of the patient.

Nurse specialist also acts as a link between community, family and patient services in a healthcare facility that has multi-disciplinary teams. Cystic Fibrosis Nursing

Advice and support: professional colleagues, families, and patients all benefits from support, advice, and liaison that is offered by the nurse specialist. This can be done through counseling, consistent carer, and confidante.

Cystic fibrosis nurse specialist also plays a central role in enhancing effective communication since problems are well resolved more efficiently through communication pathways put in place for all parties. In addition, cystic fibrosis nurse specialist also educates patients, carers, and families and takes a central role in education research and management. Cystic Fibrosis Nursing

Cystic Fibrosis Nursing