A day in the life of Marlee is nothing short of eventful, fulfilling, and exhausting. We start early, we play hard, we don't skip a beat, and we do it all with a smile on our face. Each day we spend approximately 4 hours of our day focused on our Cystic Fibrosis Care. Below we have included the different medications taken and treatments done, all to help manage her CF.
Medications
We currently administer seven different medications for Marlee.
Pulmozyne
This medication helps make mucus thinner and looser, which can make it easier for Marlee to clear lungs.
Orkambi (Age 2 to 6)
Orkambi is a combination of two compounds — lumacaftor and ivacaftor. These compounds target the defective chloride channels. Together, they maintain the balance between salt and water, keeping mucus from clogging the ducts of organs. To be most effective this medication should be taken with a high fat food.
Albuterol & Sodium Chloride
These are given to Marlee thru her nebulizer. This treatment is used to improve lung health in patients with cystic fibrosis (CF). The current consensus is that the treatment generates an osmotic gradient that draws water into the airways and increases airway surface liquid (ASL) volume.
Creon - currently takes 20 to 24 pills per day
Creon (a form of digestive enzyme) is used to help Marlee digest food normally because her pancreas does not make enough enzymes due to cystic fibrosis. She has to take this before every meal and every snack.
Vitamins & Salt
Marlee takes these orally each day. The vitamin contains additional water soluble vitamins like vitamin B and vitamin D. The salt is necessary to replenish the extra salt lost when Marlee sweats (hence the reason her Aunt calls her Salty Nugget).
Treatment Time
Marlee completes "treatment" twice a day. Her treatments currently consist of 15 minutes in her SmartVest while doing nebulizer treatments. Her SmartVest is a high-frequency chest wall oscillation inflatable vest that is attached to a machine. The machine mechanically performs chest physical therapy by vibrating at a high frequency. The vest vibrates the chest to loosen and thin mucus.
Morning Treatment - 15 minutes in her vest, plus a nebulizer treatment to include albuterol and sodium chloride.
Evening Treatment - 15 minutes in her vest, plus a nebulizer treatment to include Pulmozyme.