CF Milestones: Celebrating Achievements and Managing Expectations
Last updated: March 2021
In addition to all the milestones one faces with their child, the treatment and management of cystic fibrosis includes pivotal moments for patients. While they may not be the traditional milestones most families celebrate, like taking first steps or learning to ride a bike, we view them as key moments in her treatment plan and as a way to exercise independence. Each milestone presents expectations and adjustments, both for our daughter Margo and for us as caregivers.
Margo is currently three years old. Below are some of the important milestones she has experienced so far.
Because we learned Margo’s CF diagnosis prenatally, we had already been dealt the nerves and uncertainty most parents experience while waiting for the results of her sweat test. We had the test performed when she was two months old to comply with our clinic policies and as a necessary step for an official CF diagnosis. However, while awaiting the results did not cause stress, the actual sweat test was no picnic. I remember holding Margo as she cried for five minutes per arm during the test.
It was an incredibly uncomfortable process for her and I couldn’t explain what was happening or offer her any comfort other than hold her close and give her kisses. While she doesn’t remember this moment, it was a fundamental moment for her as a CF patient.
Graduating from taking her enzymes by spoon with applesauce to swallowing the pills whole was a big deal for Margo. It gave her independence, a new skill to practice, and the ability to eat her food quicker. It was a big deal for us as caregivers, too. Even as Margo grew and taking her medicine in applesauce became second nature for us, it could still be a time-consuming and cumbersome process.
When she was about two, we taught her a trick of sticking the pill at the top of a fruit pouch and squeezing it into her mouth, where she would swallow the food and pill together. It definitely took some practice. Initially, we had to watch her very carefully to make sure she didn’t chew on the pill and that she was actually swallowing it. Today at three years old, she can swallow her pills with water, but she still enjoys doing it with one of her favorite pouches. We often try to give her pills with a high-fat pouch containing coconut or ones full of fruits and veggies for added nutrients.
At our initial CF clinic appointments, Margo’s team discussed Orkambi, which at the time was still in clinical trials for children and had just been approved for patients six years and above. We learned that based on its trajectory, it was very likely that Margo could start taking it when she turned two. Lo and behold, she took her first dose soon after her second birthday.
While we were thrilled for this medicine, it was challenging to introduce yet another medication to a toddler. We quickly learned that Orkambi is very bitter and masking it with food really is a necessity. Through trial and error, we initially found success with chocolate pudding. That lasted about six months and she began rejecting it completely. We changed to chocolate syrup and that works very well for her and for us. The next step in our process will be teaching her how to feed it to herself with the bowl, spoon, and syrup.
Getting her first vest
As I’ve become more familiar with the CF community, I’ve learned that each clinic has a different philosophy on when to introduce the vest as a form of chest PT and airway clearance. As a general rule, our clinic recommends waiting until the patient is around three, which is when we got Margo her first vest. This definitely depends on the child and the caregiver. As a family, we have been successful with manual PT, especially as Margo became more adjusted to the process and routine. It was sometimes difficult when she was a baby to get her into the various positions and keep her content throughout the 24+ minutes, but we got through it.
We introduced the vest as a tool— another way to teach independence and ownership of her treatments. I want her to be able to choose whether she does the vest or “beat beats,” as she calls manual PT. To be honest, right now she still prefers the latter. While the vest allows her to get up and play with her toys, I think she is very aware of the fact that she’s hooked up to a machine versus laying on her dad or mom’s lap. We all like the physical connection manual PT brings. However, as I write this, I am nine months pregnant with our second baby. With our new addition quickly arriving, the family dynamic will change and I think we will all value the option of the vest.
We celebrate all of our daughters CF milestones
There have been other big CF moments in Margo’s life. Some we prefer to forget and hope they don’t return, like her first 28-day course of Tobramycin to combat pseudomonas when she was eight months old. Others will be joyous events, like when she is able to take Trikafta. With each milestone we pass, we try to instill a sense of independence into her, but I am happy that she knows we are fighting alongside her the entire way.
Have you participated in clinical or market research before?