3:30pm. Most CF parents and kids will tell you it's an exhausting day of many "what ifs" and many doctors. It's stressful because you never know what CF may bring to the table. Many tests that are routine are run but, they all factor in to the big picture of CF and give us all a good idea of how her lung health and cf is being appropriately managed.
When we arrive, we sit in the general waiting area. (Madeline is required to wear a mask by her CF doctors so that she is protected from breathing in any germs that may be around the hospital.) Eventually a nurse will call Madeline back to a small room where she will get her vitals.
They weigh her. ( Her BMI needs to be over 50% as far as cf is concerned. Her doctors look for this and calculate her own BMI (as a high BMI is usually correlated to better/ higher lung function). Madeline has to eat twice as many calories to keep her BMI. She drinks two carnation instant breakfast shakes mixed with half and half daily with her meals just to maintain her weight. We also load up on butter on veggies, ice cream, and many other of her favorite high calorie foods to help keep up with the many calories she needs. In order to absorb all the calories she eats, she must also take pancreatic enzymes every time she eats. She usually swallows 5-7 pills during her meal times and a few more for any snacks as her day goes on. It's a fine balance between making sure she always has enough pancreatic enzymes per meals based on the fat/ calorie content alone in each individual meal or she would not absorb all the calories she eats. Thus, not gaining enough weight...it's a cycle and has a learning curve to figure out exactly how many enzymes she needs per meal.
The nurse also measures her height, takes her blood pressure, measures her oxygen levels, and her temperature.
Once all her vitals are done, Madeline waits to be called back to the PFT Lab)
(Pulmonary Function Lab)There they measure her lung function. Usually this gives her CF team of doctors and nurses a good idea of her overall lung health. Her Pfts should be similar each visit and if a decline is noticed it could be an indication of infection, etc.
After PFTs, the clinic nurse usually finds a room where we meet with the pharmacist who goes over each medication Madeline is currently prescribed, adding or changing dosing as needed based on symtpoms, weight changes, etc.
Next, we usually see a slew of doctors from her CF team. Sometimes we meet with them once a year or sometimes more depending on Madeline's needs are what the cf teams feels is best.
Oftentimes, we see a social worker, nutritionist, physical therapist(for breathing technique to cough out mucous, excersize techniques, etc),
After meeting with those other members of the team, we finally see her CF Doctor.
There are 4 pulmonologiat that make up the pediatric cf clinic but we usually see the same Doctor regualrly, as he has followed and managed Madeline's cf health since we transfered to Hopkins CF clinic when she was 2 years old. We love Dr. R!
He usually does a full exam. He listens to her lungs, looks in her ears, nose, and throat. We sit and talk about how Madeline is doing and about any concerns we may have. He discusses her BMI results as well as her PFT test results. All those factor into a piece of how her cf is being managed and how her health may be. Oftentimes, medicine may be added, taken away, new specialist doctors added in due to other issues that may be affecting her cf health. This is usually the time in the appointment when the doctor gives you an overview of what he or she may find concerning, overall good,and what parts of cf need work. Usually a plan going forward is given.
Sometimes these conversations with the doctor require more answers and maybe more tests like blood work, or X-rays to find out even more information.
Last, a throat culture or swab is taken from the very back of the throat. Sometimes Madeline is asked to cough. The swab looks like a long q- tip and is often waved down as far as they can go in the throat prompting cfers to have a gag reflex. Madeline handles this well every appointment but it isn't a "fun thing." The throat culture is usually. " swabbed" and send out in lab to see if any bad cf bacteria may grow on the culture. This helps doctors find and treat possible bacteria that could be wrecking havoc in the lungs. Sometimes that is the best and worst part about cf. A cf patient could look great on the outside healthwise and exhibit very little symptoms but actually be culturing bad bacteria that could cause permanent lung damage over time if not treated. We usually have a wait 3-5 days after the cf appointment for a call or electronic update on the results of the culture. Based on the reading of the culture, a plan by the cf team is usually made as to how to treat the patient if something is grown on the culture. Oftentimes, more inhaled antibotic drugs, extra medicines, and sometimes an inpatient stay at the hospital for IV antibiotics and therapies are requested by the CF team.
Overall her visit went well. Her BMI was 81% and her team was pleased with her good weight gain and ability to keep her BMI above 50%. Her lung function was similar to her previous visits which we were happy to hear since she has been having some ongoing coughing at night and into the morning. This has been an ongoing issue that has many of her team stumped as we aren't sure what is causing it. We have some more doctors to follow up with and specialists to visit to try and figure out why this is happening. Our hope is that it isn't affecting her lung health. We will know soon how the X-ray of her lungs looks in the upcoming days as it's read by her doctor as well as her culture results that will tell us more. Our hope is that her coughing is unrelated to her cf health but oftentimes these things are hard to figure out. Keep my super girl in your thoughts as we await a good culture results ( with no bad bacteria in her lungs) and a good X-ray ( that shows healthy lungs and no evidence of lung damage or infection). Once we get results from these two tests, our CF team will give us a plan of how to move forward in keeping our cf fighter as healthy as possible!
Thank you everyone for all your good thoughts and messages today as we went to clinic. We appreciate it! ❤️
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