SixtyFive Roses

Pharmacy intern at PillPack, a pharmacy start up, researching Cystic Fibrosis

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CF Medications

Cystic fibrosis affects the body in many ways, and there are numerous medications that a person with CF takes in order to manage their condition.  Because everyone is affected differently by CF, treatments vary greatly from person to person.  Some common medications that nearly all people with CF will take, either daily or as needed for pulmonary exacerbations, are antibiotics, pancreatic enzymes, multivitamins, Pulmozyme, albuterol, hypertonic saline and anti inflammatory medications.  Newer drugs are emerging, such as Vertex’s Kalydeco, with hopes for further improving treatment and eventually finding a cure in the future.

Antibiotics - prophylaxis against, eradicating, or controlling respiratory infections and pulmonary exacerbations

The specific agent or agents used, the dosage, the route of administration and the length of time taking the antibiotic is highly individualized.

The individualized regimen depends on the strains of bacteria involved, the severity of the infection and previous history of antibiotic use.

Pancreatic Enzymes - Pancrelipase (Creon, Pancreaze, Zenpep) - Replacement for digestive enzymes secreted by the pancreas, the porcine derived lipase, protease and amylase enzymes in pancrelipase enhance digestion in the small intestine

Lipase - breaks down fat

Amylase - breaks down carbohydrates 

Protease - breaks down proteins

Bronchodilators - Albuterol (AccuNeb, ProAir, Proventil HFA, Ventolin HFA)- Inhaled B2-agonists are often administered before chest PT to loosen mucus for airway clearance or are used for bronchial hyperresponsiveness

Dornase alfa (Pulmozyme) - nebulized medication that aids in the thinning of mucous in the lungs so that it is easier to cough up

Large amounts of DNA is released from dead neutrophils that are drawn to the inflammation in the lungs, which increases sputum viscosity

Recombinant human DNase are enzymes that hydrolyse the DNA and separate DNA from proteins

The DNase enzymes break down the proteins, which thins the mucus and improves airway clearance

Ivacaftor (Kalydeco) - targets the defective CFTR protein, a chloride channel present at the surface cells, which is the underlying cause of cystic fibrosis

Potentiates the CFTR protein →  increased open chloride channels → increased chloride transport out of cells

Other Medications and Therapies

Airway clearance

Flutter vest

Chest PT

Nebulized hypertonic saline

Multivitamins

Fat-soluble vitamins - ADEK

Vitamin B12

Zinc

Anti inflammatory medications

Ibuprofen

Steroids

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A brief history of CF. Look at all of the progress that has been made in just the past 2 decades!

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OneRepublic - I Lived

Director: Noble Jones Idea Inspired by Sophie Muller Producer: Taylor Vandegrift, Joseph Uliano Production Company: Wondros To learn more about Cystic Fibros...

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The many manifestations of CF

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chronic illness in a nutshell

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and mental illness

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Life With Cystic Fibrosis - My Outside Perspective

It is very difficult to assess how living with a chronic medical condition, such as cystic fibrosis (CF), affects daily life when you are not the one actually living every day with it for yourself.  However, for healthcare professionals in any discipline, it is important to learn how to empathize with patients in order to better care for them.   In order to gain empathy, you have to be able to place yourself in their shoes, even if just briefly, and need to have an understanding of the symptoms they live with and setbacks they cope with day in and day out to do so.  

A textbook description of CF is that it is caused by the inheritance of a defective CFTR gene that results in disruption in the functioning of chloride channels, resulting in an abnormally thick mucus produced on the outside of the cell, primarily the cells lining the passageways of the lungs and pancreas.  In the lungs, the mucus clogs the airways causing coughing, shortness of breath, decreased exercise tolerance, and increases the risk of acquiring infections.  In the pancreas, the mucus blocks ducts carrying digestive enzymes into the intestines, which interferes with being able to properly digest and absorb nutrients from food and can lead to poor weight gain and growth.

Now that the background is out of the way, I will start with some of my own misconceptions I had before I began doing research into CF and interviewing patients for myself.  Firstly, although I have family with CF, giving me a slight background beyond that of a textbook or a 50 minute lecture in college, my view was that people with CF were so sick that more often than not they could not lead a “normal life”.  I quickly found out that I was wrong, very wrong.  

Yes, those with CF cannot simply forget about their condition.  They undergo nebulizer treatments that can take a couple of hours of each day to complete; they often become short of breath doing routine task; they can find themselves in the hospital for weeks at a time unable to live their life on their terms for a while, sometimes having to go home on weeks worth of IV antibiotics.  However, that does not mean that people with CF do not live fulfilling lives.  They spend the time that is not preoccupied by CF treatments and exacerbations doing things I never imagined they could.  One young woman I interviewed does Crossfit three times a week.  If you are not impressed with that, you must not know that Crossfit is physically challenging to even the most athletic of people, never mind someone who has compromised lung functioning and whose body cannot always absorb essential proteins that are required for building muscle.  In fact, nearly every person with CF that I have interviewed goes to the gym or runs outdoors despite their perceived limitations.  

While CF is something they have to modify their life around having, whether is it carving out a few hours a day for treatments or living in homes without stairs to make everyday life a little less physically taxing, having CF is not what defines these people.  It has been shown that those with chronic illnesses are more susceptible to succumb to anxiety and depression, which is understandable; however, nobody I have spoken to or who has sent me anonymous feedback has told me that emotional distress holds them back from living their lives.  I was impressed by this because I for one can work myself up into having a full-fledged panic attack over the most trivial of things.  I admire these people who can cope with setbacks and can appreciate every day.  One woman I interviewed said it perfectly, “Every day I wake up is a bonus day.”  The positive outlooks of everyone I interviewed has really put living every day to its fullest into perspective.

Having CF is not something that most people with this disease want to allow to hold them back, but it does not come without its struggles or times of feeling defeated, beaten down and like CF is winning.  One young man I interviewed wanted to clear up the misconception that he was lazy.  He wanted to work more than anything, but he had to devote so much of his time to treatments and was so weak from a recent infection that holding down a job was nearly impossible.  I am nearing graduation and often worry about securing a job and being independent, and speaking with someone my own age who is trying to also be autonomous himself and has so many more obstacles to overcome was eyeopening.  I cannot imagine how difficult it can be when you want to achieve something and have the willpower to do so but physical limitations make it that much more difficult and cause others to cast unfair judgement.

Being a pharmacy student really makes no difference in what I have learned about CF.  I have a background in medicine, which makes me know why IV and nebulized therapies can be time consuming and what specifically is happening physiologically, but I think that anyone can see that undergoing these treatments, being short of breath and having digestive issues can be a hindrance to daily life.  My biggest takeaway so far is that CF is a serious illness, but it is not what defines those who have it nor do they want it to define them.  People with CF have to modify their lifestyle around their physical boundaries and treatments, and they do have genuine limitations and need certain accommodations at times, but they tend to want to extend themselves as far as they can to the threshold of their own limits to live their lives to the fullest.

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About Me

I am Meaghan Flynn, a 6th year PharmD student at MCPHS University.  I grew up north of Boston in Tewksbury, MA but was fortunate enough to live in and experience the city for five years during my time in college.  Yes, my upbringing in the Boston area does mean that I have a love for all things Boston, and I will most likely wear black in mourning when Tom Brady is no longer the quarterback of the Patriots.  When I am not studying or working ridiculous hours I enjoy being outside as much as possible whether it is running, hiking, kayaking or just spending time at the beach (even during winter).  Boston has my heart, but I absolutely love traveling.  I take any opportunity I can to visit new cities and experience different cultures.  I hope that these experiences and my willingness to learn from others will help me in my career as a healthcare provider.

I have always had a passion for science, and although I did not necessarily know exactly what pharmacy entailed when I was applying to colleges at the age of 17, I knew I wanted to work in healthcare.  I felt as though pharmacy was the best fit for me in regards to what arena of medicine I would chose as a career.  Six years later I know what a pharmacist can ultimately do, but I have yet to decide exactly what avenue I wish to pursue.  That is what I love about pharmacy though; if you put in the effort, you do not have to be complacent with the typical retail or hospital pharmacist roles.

I currently work at CVS in Harvard Square, which has taught me a great deal about working in a busy retail pharmacy.  I also have gained experience working with a very diverse patient population from all walks of life and from across the globe.  However, because I already have insight into retail pharmacy and continue to work at CVS, I did not add a single conventional community pharmacy rotation as any of my 240 rotation selections last February.  I truly wanted to use my time on APPE rotations to learn as much as possible about things I did not or could not have learned while in my first five years of pharmacy school.  

What made the rotation at PillPack stand out to me was that it was so different from other rotations that were listed and because I do truly enjoy writing.  To be honest, I did not look into the company as a whole until I had already submitted it as one of my first choices simply based off of the rotation description given.  I was pleasantly surprised when I researched the company a little and talked to classmates and found out that PillPack was so innovative compared to the typical depiction of pharmacy most people have in their minds.

I have chosen to spend my six weeks at PillPack researching cystic fibrosis (CF).  I have a personal investment in the condition since I have numerous family members with CF, but I have never actually put much thought into how to overcome barriers to adherence that this patient population faces.  Since there is currently no cure, people with CF are chronically on a vast number of antibiotics, pancreatic enzymes, inhalers and other medications, and they could benefit greatly from PillPack’s services.

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Bryan Warnecke, a 15 year old from Colorado with CF, shared his story in OneRepublic's music video I Lived, which focuses living every day to the fullest.

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Cystic fibrosis is a recessive disorder, which means that a child must inherit the mutated gene from both parents in order to have the disease. If both parents are carries of the CF gene, they have a 25% chance of their child having CF.

If a child only inherits one copy of the mutated gene, he or she will be a carrier of CF. Carriers do not actually have the disease, but they can pass it on to their children.

Reference - http://learn.genetics.utah.edu/content/disorders/singlegene/cf/ 

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The phrase "65 roses" is another way of saying cystic fibrosis. In 1965, a young boy with cystic fibrosis overheard his mother talking about the disease on the phone. When she said the words “cystic fibrosis”, he thought she said “65 roses”. The mother was touched by her son’s mistake because he saw something beautiful in a disease that can often be quite ugly. The phrase is now a registered trademark of the Cystic Fibrosis Foundation, which adopted the rose as its symbol.

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Just breathe