Cystic Fibrosis

Thanks to advancements in care, Cystic Fibrosis patients are leading longer, healthier and more productive lives.

 

Leading the Charge to Conquer Cystic Fibrosis

Cystic Fibrosis, commonly referred to as CF, is a genetic disease that, in part, places patients at risk of lung infections. Mucus builds up in the lungs and other organs, restrict the airways and other pathways, creating complications including lung damage over time. This damage can cause respiratory failure and impact several natural processes in the body.

Because CF is a progressive disease, it’s important to identify the disease in the early stages and seek treatment to assist the body’s functions. CF is usually identified by key symptoms such as shortness of breath, coughing and wheezing, an inability to maintain proper weight and difficulty with bowel movements.

 

happy-dad-holding-child
mother-hugging-happy-child

Treating CF

Care for CF patients can include a variety of treatment options. Total Care RX works closely with referring physicians charged with patient care to ensure strict adherence to each patient's specific program. 

Treatment can include:
The Use of Mucus Thinners

These are inhaled medications that thin mucus in the airways.

CFTR Modulator Therapies

Modulator therapies are designed to correct the malfunctioning protein made by the CFTR gene.

Antibiotics

Because CF patients are more susceptible to infection from bacteria, antibiotics can be an effective option to eradicate bacteria and sustain a patient’s health.

Nebulizer Treatments

A nebulizer generates mist from liquid medications that allow the patient to inhale the solution through the use of a nebulizer. It’s important to pair specific medication with the nebulizer recommended by the manufacturer. When using a nebulizer at home it’s imperative to follow the proper cleaning instructions to ensure a sterile environment. Nebulizers should always be cleaned, disinfected, thoroughly rinsed and air dried. 

Ultimately some CF patients may be candidates for a lung transplant.

CF Facts & Figures

  • Over 30,000 people are living with cystic fibrosis in the U.S. - worldwide the number is between 70,000-100,000. 
  • There are roughly 1,000 new cases of CF diagnosed each year. 
  • Just over 75 percent of people with CF are diagnosed with the disease by age 2. 
  • Because early diagnosis of cystic fibrosis can have a dramatic effect on the future health of newborns, legislation was passed in 2010 in all 50 states, and the District of Columbia, requiring that all babies be screened for CF.  
  • Those that are living with CF need to avoid close contact with others that have the condition as each has a different type of bacteria in their lungs and bacteria that are not dangerous to one person with CF can be extremely dangerous to another.
  • People with CF today are living far longer and more product lives and the proof of that is in the numbers. in 1962 the predicted median survival was 10 years and today living well into one's 40s, 50s and beyond is no longer a rare occurrence for someone with CF.

— Courtesy of Cystic Fibrosis Foundation

happy-family-walking-through-the-fields
old-happy-asian-couple

The Total Care RX Step by Step Approach To Proper Cystic Fibrosis Nebulizer Treatment

Step 1: Bronchodilators

Patients start their treatments with inhaled Bronchodilators such as Albuterol inhalers, which opens up the airways and increases air flow to the lungs.

Step 2: Hypertonic Salines

Hypertonic saline is used to mobilize mucus so it’s easier to cough out.

Step 3: Pulmozyme

Pulmozyme may be prescribed to thin and loosen the mucus to make it easier for patients to clear their lungs.

Step 4

Patients undergo chest physiotherapy, donning an inflatable vest attached to a machine that performs high-frequency vibrations to loosen the mucus.

Step 5

Inhaled antibiotics may be taken again. Some patients will use oral azithromycin or a similar antibiotic from once a day to three times a week to reduce inflammation of the airways.

Step 6

Some CF patients will eventually need to have a lung transplant. Total Care RX will be there every step of the way, from pre- to post-operation, to ensure patients receive the best treatments possible.

Specialty medications Total Care RX dispenses include:
  • Tobramycin 
  • Kitabis Pak
  • Bethkis
  • Pulmozyme

Support Helps The Healing Process

Total Care RX believes strongly that support groups help bring people together and provide a common ground for those suffering from similar medical conditions. The sharing of experiences, feelings and coping strategies can become a major part of the healing process.

Total Care RX is proud to be a Live2Thrive partner as this program offers tremendous support, free vitamins/supplements, copay assistance and much more.

Total Care RX is also proud to be partners with two renown Cystic Fibrosis organizations in the  Cystic Fibrosis Foundation and the Boomer Esiason Foundation. Both of these staples of the Cystic Fibrosis community have always provided tireless support for all people with CF. 

Over 40 Years of Personal & Specialized Patient Care

Total Care RX feels strongly that exceptional patient care goes beyond the prescription.  Patients require the proper education to fully understand and get the most benefit from their medications. This is what being a specialty pharmacist means to us.

718-762-7111 Contact Us