Parker Rallies Hope for Cystic Fibrosis

06/13/2024

As stated in the past, I don’t post as often these days because Parker is now a teen with his own thoughts about me sharing his fight against Cystic Fibrosis (meaning he doesn’t really want me to share as much anymore, but I sometimes get the ok)

🦠After months of off and on illnesses and 3 rounds of abx at home and 2 rounds of steroid bursts we may hopefully be getting some answers, I hope.

🦠At Parker’s last CF clinic he cultured something brand new for him called, Moraxella catarrhalis. Typically his baseline culture is staph and over the years we’ve seen a few others pop up, like pseudomonas aeruginosa, but this was something that he’s never once cultured so I suspect he likely picked this up at school. Parker typically has no baseline cough and has basically been coughing consistently since December. Now I know a lot of people probably are thinking, but Jeanne, he has CF. To that, I would say while that is true you have to understand what his normal baseline is and why any new changes are concerning. It’s always something I pay attention to and keep clinic informed about. It is possible we may be moving to a new baseline, but I hope not and I think today may have given us a couple possible answers.

👩‍⚕️ After much phone tag with ENT over the last few days they were able to get him in on a sick call to be seen by his new ent, Dr. Kirkham, today. She really was very thorough and went over everything she was seeing upon exam and Parker’s scoping in office.

ℹ️ First he has these red spots on his soft palate. She wasn’t exactly sure what those are caused by but said to keep an eye on them and if they become bothersome or he develops any other symptoms to get him into his p*p. My suspicion is that they are from his CONSTANT throat clearing and coughing for the last week.

ℹ️ Once they started to scope him, they noted how incredibly deviated his septum is, which we’ve always known, but as she explained to me having such a big deviation is really bad for him because his sinuses are not being allowed to properly drain when they do develop infection which only leads to continued infection and overgrowth of bacteria. His passage and cavities were all red and inflamed.

Basically, he’s a walking Petri dish which is not a good thing when you have a lung disease.

He had NO polyp growth which was great, but he is FULL of infection and pus. With that said, she plans on holding off for potentially 6 months to do a revision FESS (functional endoscopic sinus surgery) and a septoplasty. They also took a culture of the crap that’s up in his sinuses and we will find out if what he is currently taking for an antibiotic is enough to cover what is growing but she said she suspects it isn’t and the game plan may need to change depending on those culture results. Those typically take 3 days to come in so I don’t expect to hear anything until next Monday.

The current plan is for him to head back to ENT on December 12 UNLESS I call them and ask for something sooner due to continued sickness. I’m telling you, 2024 so far is reminding me of 2014 when we spent our lives in and out of the hospital and in the feeding program. It’s just not been a good go so far.

ℹ️ Parker has CF clinic on July 2 and we are doing everything in our power to keep him out of going inpatient. We have our family vacation beginning July 19-27 and we are all looking forward to it but having Parker sick at all will put some stressors on us that none of us want to deal with. If we can stay out of the hospital until November 2024 it will have been 3yrs since he was last inpatient. 🤞 While we have been fortunate to not go inpatient he has dealt with many illnesses at home that we’ve been able to treat with oral abx. With as many illnesses as he’s had since December 2023 it’s not a matter of if he will be admitted, but when. Hearing him have a j***y cough is something I have always dreaded because it somehow made cf seem like it was really there attacking his precious lungs and airways, yet I knew all along that was the case… he just didn’t have the cough yet. 🤦🏼‍♀️

Anyhow, I’m sorry I don’t update much these days. Parker’s privacy as he gets older is very important to me and I want him to approve of anything I share about him, especially online. Too many creeps out there lurking.

Please keep our boy in your thoughts. He had a wonderful first year at in person school, but the illnesses have been tough to deal with.

05/05/2024

May the Fourth Be With You!

Day 4 of CF Awareness Month:

ℹ️ Each day, people with CF complete a combination of the following therapies:

✔️Airway clearance to help loosen and get rid of the thick mucus that can build up in the lungs.
✔️Inhaled medicines to open the airways or thin the mucus. These are liquid medicines that are made into a mist or aerosol and then inhaled through a nebulizer and include antibiotics to fight lung infections and therapies to help keep the airways clear.
✔️Pancreatic enzyme supplement capsules to improve the absorption of vital nutrients. These supplements are taken with every meal and most snacks. People with CF also usually take multivitamins.
✔️An individualized fitness plan to help improve energy, lung function, and overall health
✔️CFTR modulators to target the underlying defect in the CFTR protein. Because different mutations cause different defects in the protein, the medications that have been developed so far are effective only in people with specific mutations.

CF is a challenging illness and is different from patient to patient even if they have the same genetic mutations.

💵 Please help us continue to fund the search for a cure by donating to our 2024 Great Strides walk:

I'm walking in my local Great Strides event to help find a cure for all people living with cystic fibrosis. Will you support my fundraising goal?

05/03/2024

CF Awareness Day 3:

ℹ️Genetics and Diagnosis

🧬 Cystic fibrosis is a genetic disease. People with CF have inherited two copies of the defective CF gene — one copy from each parent. Both parents must have at least one copy of the defective gene.

People with only one copy of the defective CF gene are called carriers, but they do not have the disease. Each time two CF carriers have a child, the chances are:

25 percent (1 in 4) the child will have CF
50 percent (1 in 2) the child will be a carrier but will not have CF
25 percent (1 in 4) the child will not be a carrier and will not have CF

The defective CF gene contains a slight abnormality called a mutation. There are more than 1,700 known mutations of the disease. Most genetic tests only screen for the most common CF mutations. Therefore, the test results may indicate a person who is a carrier of the CF gene is not a carrier.

Diagnosing cystic fibrosis is a multistep process, and should include a:

✔️Newborn screening
✔️Sweat test
✔️Genetic or carrier test

Clinical evaluation at a CF Foundation-accredited care center:

Although most people are diagnosed with CF by the age of 2, some are diagnosed as adults. A CF specialist can order a sweat test and recommend additional testing to confirm a CF diagnosis.

Parker was diagnosed with CF by 3wks old after his newborn screen flagged him with DF508 and a sweat chloride test confirmed he did in fact have CF on November 15, 2010. He was later found to have a rare mutation for his 2nd gene.

Paxton is a carrier of our rare gene 3199del6 which will be important for him to know when he goes to start his own family some day.

💵 Donate today to help make a difference in the lives of our CF fighters:

I'm walking in my local Great Strides event to help find a cure for all people living with cystic fibrosis. Will you support my fundraising goal?

05/02/2024

Day 2 of CF Awareness Month:

ℹ️ People with CF can have a variety of symptoms, including:

✔️ Very salty-tasting skin
✔️Persistent coughing, at times with phlegm
✔️Frequent lung infections including pneumonia or bronchitis
✔️Wheezing or shortness of breath
✔️Poor growth or weight gain in spite of a good appetite
✔️Frequent greasy, bulky stools or difficulty with bowel movements
✔️Nasal polyps
✔️Chronic sinus infections
✔️Clubbing or enlargement of the fingertips and toes
✔️Rectal prolapse
✔️Male infertility

Parker, deals with a lot of sinus issues including polyp growth for which he’s had numerous polypectomy surgeries for. Unfortunately starting school in person for 2023/2024 has led to numerous respiratory infections but we’ve been able to keep him from going inpatient with abx at home and tons of extra treatments. He is overall doing really well though and we are so grateful for the support we’ve received over the last 13yrs. Your donations help drive critical research to extending the lives of our cf fighters.

💵 Please make a tax deductible donation today at:

I'm walking in my local Great Strides event to help find a cure for all people living with cystic fibrosis. Will you support my fundraising goal?

05/01/2024

Day 1 of Cystic Fibrosis Awareness month: As nearly everyone knows by now, our son, Parker, was diagnosed with CF at 3wks old in 2010.

❓What Is Cystic Fibrosis?

ℹ️ Cystic fibrosis is a progressive, genetic disease that affects the lungs, pancreas, and other organs.

There are close to 40,000 children and adults living with cystic fibrosis in the United States (and an estimated 105,000 people have been diagnosed with CF across 94 countries), and CF can affect people of every racial and ethnic group.

In the lungs, the mucus clogs the airways and traps germs, like bacteria, leading to infections, inflammation, respiratory failure, and other complications. For this reason, avoiding germs is a top concern for people with CF.

In the pancreas, the buildup of mucus prevents the release of digestive enzymes that help the body absorb food and key nutrients, resulting in malnutrition and poor growth. In the liver, the thick mucus can block the bile duct, causing liver disease. In men, CF can affect their ability to have children

🙌🏻 Today, because of improved medical treatments and care, more than half of people with CF are age 18 or older. Many people with CF can expect to live healthy, fulfilling lives into their 30s, 40s, and beyond.

💵 Donate today to help us continue the search for a cure: 🫶

I'm walking in my local Great Strides event to help find a cure for all people living with cystic fibrosis. Will you support my fundraising goal?