Good Morning Everyone! You may not know this but May is CF Awareness Month. I decided that I wanted to do a post about CF (Cystic Fibrosis) to promote awareness of this disease in the hopes of finding a cure. And what better way to bring awareness than to talk to someone who deals with this disease on a daily basis. It has been a privilege to interview Jessica Michael who is the mother of Cordell, her beautiful son who was born with CF. My hope is that Jess’ interview shines a light on the struggles encountered while battling this disease and can help us push for a cure to be found.
Me: In your own words, what is Cystic Fibrosis (CF)?
Jess: CF is a genetic disease that affects the entire body but mainly the lungs. In short, a person with cystic fibrosis does not utilize salt in their body properly causing their body fluids (like mucus and bile) to be much thicker than someone like you or me. Over time this thick mucus can build up in their lungs causing lung infections. This leads to heart problems as the heart sometimes has to overcompensate for poor breathing. This is why Cordell was born with a bowel blockage. His Meconium (which all babies are born with) was so sticky and thick it couldn’t pass through his intestine and actually caused a bowel blockage that required surgery! About 25% of CF’ers are born with a bowel blockage. Also many CF patients (but not all) have digestive problems because their pancreas does not produce the enzymes which breaks down our food and pulls nutrients and fat from food. Luckily, there are pancreatic enzymes that Cordell can take when he eats to help his body absorb the proper nutrition and fat from his food. Many CF patients struggle with weight gain which is also correlated to poor lung function as well.
Me: How does this disease affect you and your son on a daily basis?
Jess: Cordell is very fortunate with his health. He had a rough start and spent 11 weeks in Johns Hopkins but luckily he has maintained good health since then. He does have to take pancreatic enzymes every time he eats which is difficult as kids tend to graze on food rather than sitting down for meals. It’s stressful on us because we have to make sure we take the medicine everywhere we go and also that no one gives him food without us knowing (ie snacks at church etc). When he was a baby we had to break the capsules open and put them in applesauce and feed them to him on a spoon. We have done this since he was 1 week old. At that time we had to carry applesauce and a spoon everywhere with us as well! Luckily he can swallow the pills now. It’s actually quite amusing to watch a 3 year old take large pills with water. As parents, Keith and I still have to make sure he gets proper nutrition. He needs a high calorie and high fat diet. We have to add coconut oil or butter to a lot of his foods, we eat a lot of avocados and nuts for snacks, and he drinks a Pediasure every day for added nutrition. We also have to give Cordell super vitamins due to his body not absorbing nutrients from his food. Cordell has made a very healthy weight which makes the doctors extremely happy. We know a 2 year old friend with CF who has to have a g-tube in his stomach and get tube feedings several times a day to help him maintain. We are very fortunate we don’t have to go that route. Other than that we just have to keep him active to keep his lungs as clear as possible. We are required to do his vest for 30 minutes 2 times a day. This is a vest we put on him and a machine blows air into it and vibrates it really fast to shake any mucus off his lungs. It’s very hard to get a 3 year old to sit for 30 minutes to do it so we have alternatives such as jumping on the trampoline, running, or manual PT where Keith or I use our hands and basically beat on his chest! It sounds barbaric and I hated doing it to him at first but now I am used to it. Most other CF patients do nebulizer treatments during their vest time but fortunately for us, Cordell has never cultured bacteria in his lungs so we don’t have to do those treatments yet.
Me: What are some of the risks that you have to be aware of and try to avoid?
Jess: GERMS! I mean everyone tries to avoid those but CF patients have a compromised immune system and tend to get sick easier than you or I would. Not only that, but when a CF’er gets sick it can be much more serious. A common cold for you or me can very easily turn into a hospitalization for a CF patient. So we are very careful with hand-washing, etc but also keeping him away from people with a cold or getting over a cold. We also have to be leery of hot tubs, bodies of water with little movement, or anything where bacteria likes to grow. Another reality of CF is that CF patients are not supposed to be in the same room as each other! Apparently it is too easy for them to pass bacteria back and forth to each other. So not only does CF have physical downsides but social as well- CF’ers can’t even hang out with other people going through the same things as them! It’s really sad but social media has helped bridge that gap a little.
Me: Is there a cure currently, or one being worked on by medical research?
Jess: There is currently NO CURE for Cystic Fibrosis. In the last 30 years the life expectancy of a CF has increased by 23 years due to medical advancements and treatments. It increases by 6 months to a year each year. Most recently, the CF Foundation has come out with a new medication called Orkambi last year that is the first FDA approved drug that actually tried to counteract the underlying genetic defect that causes the disease rather than just treating the symptoms. This was a HUGE step towards a cure and there are more studies coming down the pipeline. Unfortunately, Cystic Fibrosis gets no federal funding from the government since it is considered an “orphan” disease, meaning not enough people are affected by it, even though there are about 33,000 cases in the United States. Most of the funding comes from fundraisers and private donors along with some grants from pharmaceutical companies.
Me: What are some of the misconceptions about Cystic Fibrosis (CF
Jess: When people look at Cordell or my sister-in-law (who also has CF), they look “normal” and don’t really understand what the big deal is…they downplay the illness. CF is an extremely serious disease and one cold can kill someone with CF in an instant. My sister-in-law has lived a healthy life but she has had multiple procedures to remove polyps in her nose, had her gallbladder removed, had PICC lines, and now a permanent port because of all the blood work CF patients get done regularly. Kristen had a G-tube for a year of her life and has had to do countless treatments. It’s frustrating for people to just look at your son, who you are fighting so hard on a daily basis to keep them healthy and alive, and say “well he looks fine.” We spend hours doing treatments and spend a small fortune paying for medications to keep this kid up and running normal. My friends will complain when they have to give their child 10 days worth of antibiotics or they have to do a nebulizer or some other treatment every night for a week and I just laugh, because that is our life every single day. And we don’t get a break or a vacation from it. I also want to add that when I was pregnant with Cordell and they suspected Cystic Fibrosis due to our family history and they could see the bowel blockage in the sonogram that abortion was brought up several times during our pregnancy. Luckily, Keith and I are very educated on the disease because of his sister and we are hopeful for treatments and a cure in our lifetime so that was not an option for us. Now, I look at Cordell and watch him play with his siblings, or laugh at a movie, or run around the yard chasing the dog and I couldn’t imagine ending his life just because he was going to require a little more time and effort than a healthy child. He is such a blessing to our family and has taught us all about love, perseverance, patience, and sacrifice. I cannot imagine my life or my family without him and it breaks my heart to think some parents are terminating pregnancies because of this disease. I am not naïve – it is a full-time job to have a child with a chronic illness and it is not for the faint of heart, but parenthood in general is HARD! It’s the hardest job in the world but it comes with the greatest reward as well.
Me: What do you want people to know about living with Cystic Fibrosis (CF)?
Jess: Be kind. Educate yourself and develop an understanding of the disease if you are around anyone with CF, because the disease is quiet. Someone with CF may look and act normal and healthy to you but they have a lot going on inside. I remember seeing a post on a Cystic Fibrosis website of a 25 year old with CF who had a handicap parking sticker and someone actually wrote her a very nasty note and left it on her car accusing her of taking a handicap spot from someone who truly needed it and telling her she should be ashamed of herself because on the outside she looks like a healthy 25 year old but on the inside her lung function is 27% and it is painful for her to breath let alone walk distances especially in the heat.
I hope that Jess’ insight into living with CF has inspired you to help get the awareness out about this disease. Please share this post and visit the below sites to help support the fight against CF!
Great Strides: Cystic Fibrosis Foundation Fundraiser: http://fightcf.cff.org/site/TR/GreatStrides/154_Maryland_Baltimore?team_id=47943&pg=team&fr_id=4912
Thank you Jess Michael for sharing your personal journey fighting every day against this disease!
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