Quarterly CF Clinic & Culture Results - October 2017

We made the nearly two hour drive up to Hopkins for her quarterly CF clinic visit.  We left the house at 8 am and returned home by 3:30 pm. 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. All CF patients must also wear masks so they cannot spread harmful bacteria to each other since the waiting room has many cf patients too.) 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. Thus requiring oral antibiotics, impatient IV antibiotics, steroids, 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 symptoms, 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, exercise techniques, etc), 

After meeting with those other members of the team, we finally see her CF Doctor.

There are 4 pulmonologists that make up the pediatric cf clinic but we usually see the same Doctor regularly, as he has followed and  managed Madeline's cf health since we transferred 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. Most CF clinics require an X-ray every 2 years as "routine" and blood draws yearly. She had her X-rays done (last year)

This visit she was due for her yearly blood draw. She took it like a champ!

 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 to the 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 health wise 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.

We also spend a lot of time occupying her younger sister, Audrey who is 2 years old. She doesn't have CF. We try to spend time with the girls in between doctors, nurses, and staff coming in and out of the room since the actual visit last for hours in the same room. 

Overall her visit went very well this time.The CF team usually looks for a healthy weight gain in between cf visits. We constantly work to introduce fatty/ high calorie food to to keep her BMI above 50%. Madeline also works very hard to finish meals and push herself to eat/drink high fat items. Her lung function was similar to her previous visits which we were happy to hear. She also grew an a half an inch and 4 ft, 6 inches tall. Her doctor was pleased with her BMI of 80 and her height/weight ratio being in the 86 percentile. All those extra calories she is getting has been paying off. 

Overall, the cf team was very pleased with her health. Now we must wait for all the lab work and her throat culture to get a better idea of how CF is treating her on the inside. 

UPDATE: Thankfully, it only took 3 days for the results to come back ( usually the wait can be agonizing and longer) and Madeline's throat culture showed normal respiratory flora as well as Staph bacteria. We know the throat culture only gives us a "sample" of what she may harbor in her lungs but, we are thankful for this good news. The staph bacteria is not typically treated in CF patients unless she develops symptoms such as coughing, lower lung function, etc.Thus far, we have not seen any indications that the Staph is effecting her CF health. We are thankful that her throat culture came back good. We always hope for good cultures so we can limit the amount of medicine she is on, hospital admissions, and lung damage. 

We are so proud of Madeline and the amazing CF fight she shows daily. She has grown to be her own advocate for CF and takes more responsibility when talking to her cf doctors about her health. She has learned how to dose her own enzymes ( according to the food she is eating), she reminds others that she needs to wash her hands, and overall is proud to share about her CF when asked. 

We are so thankful for the great care she is receiving at Hopkins and at her new school, Wilson Elementary. Her teacher, ENCORE staff, nurse and the administration there have been so supportive and proactive with Madeline's care. It takes a village to fight CF! Thanks to everyone for the many thoughts and messages as we awaited her results. We appreciate the care you show for Madeline and our family because as we know, CF can take some unexpected turns at short notice.

She's our fighter❤️

CF Walk and Madeline's Marchers

Madeline's Marchers made it to DC for the walk to support Madeine and others with CF! We had a great team this year made of family, friends, and teachers/staff from Madeline's school, The Merit School. We were so thankful for all that showed up, walked with us, and donated from afar. We raised over $8,500 that will to then CFF! We are so thankful! 

It was such a fun time had by all. There was music, plenty of food, kids activities, drinks, and so many people to meet and talk to. We really enjoyed the day- the kids did too!

Madeline's friend, Elin and students/ teachers from the Merit School

We had such a fabulous group of teachers and staff supporting us and Madeline from the Merit School. Mrs. George, the principal as well as Miss Smithson, Madeline's 1st grade teacher came. They brought with them many other teachers that wanted to support Madeline and the CFF from her school.

Madeline's classmates and friend, Amaar

Supporting us!

 The Merit School also ran a book sale organized and started by Miss Smithson, her first grade teacher with help from Mrs. George, the principal. The proceeds from the donated lightly used books went straight to Madeline's Marchers. We had such a great support from parents, students, and teachers from the Merit School for the book sale. Many people purchased, donated books, and helped run the book sale during school, after school, and at other sister schools.

MIss Smithson came up with the idea and organized the sale which brought in close to $700 for our team and the CFF. We are so thankful for the community support of the Merit School. It's a small school of only about 120 students but the outpouring of love, donations, and money raised was amazing! We are so thankful for everyone involved.

 

Miss Smithson and Ms. Davis

( running book sale)

 Madeine loved having her amazing teacher, Miss Smithson at her walk as well as Ms G, Ms Davis, Ms Lamboni, Ms Mayers, Ms. Phillips, Mrs. G, and Mrs. George, the principal. 

We are so thankful for all and what they have  done for Madeline and the CFF.

Madeline and her BFF, Bella

THANK YOU to all involved! We thank you so much....

See you next May at Great Strides 2017!

 

 
 

 
 

Culture results and X-ray from CF Clinic

We got the results from Madeline's CF Clinic the following week after her clinic. We were thankful to see the Psuedo still hasn't beefing show up on her cultures ( as it has done in the past- see past posts). It's been almost 2 years since she has last cultured pseudo. We are so thankful for that since it is a bacteria that once in the lungs can cause permanent and irreversible lung damage if not controlled. Madeline cultured pseudo at age 2 and used 3 different inhaled drugs over the course of 6 months time to try and rid her body from it. It never worked so we admitted her to Hopkins ( as recommended by her team of doctors) to use IV antibotics to try and kill off the pseudo. Usually IV antibotics is the most aggressive way to treat bacteria, like pseudo that may harm the lungs. After a two week course of antibotics in the hospital, Madeline had one clean culture of no pseudo but only a few months later has pseudo show up again on her culture. We battled pseudo for over a year again with more inhaled antibotics at home to kill off and weaken the bacteria. Thankfully, it seemed to help ward of pseudo. We have been clear.

So what is this new bacteria showing up on her most recent results? We asked this of her CF Clinic team at Hopkins. Streppuccucus pneumoniae is a bacteria that is usually left untreated in cf patients unless they have symptoms - like coughing, lower lung functions,

Etc. Due to Madeline'a chronic night cough the team felt it was nessecary to treat the bacteria with oral antibiotics in thinking that the bacteria could be partly the cause of her cough. 

Her lung function though and X-ray show that this bacteria and chronic night cough has had no affect on impacting her lunch health. We are thankful for these test results. As her X-ray came back as being read as follows:

After learning that her lung health does seems to be impacted by her chronic night cough, we were relieved. Seeing the results of her X-ray, no lung function change, and throat culture gave us a good indication. But, we just like her doctors are still wondering why this night cough has been happening since October and what can he down to stop it. She often chokes on her mucous when she is laying down this waking her up. We needed more answers so we consulted with a pediatric allergist as a start.

We got an appointment to have Madeline go through a full panel of allergy testing to see if maybe some of this chronic night cough has been occurring because of her increasing allergies. She has never had allergy testing done but we know she suffered from

Allergies as she is on 2 medications to treat allergies that have helped relieved he symptoms of sneezing, running nose, and watery/ itchy eyes while she is outside. 

Signing up for allergy testing meant that she had to be off of all of her allergy medicines for 5 days leading up to her appt. we knew this would be challenging.  

In those 5 days, she broke out in hives, had more coughing, sneezing, throat clearing. It was important though that we stick to no allergy medicine so the allergist, Dr Kim could accurately test her skin for all the allergens without the help of any antihistamines in her body.

Rash on her arm

BUT, she made it to allergy appointment day. Dr. KIm ran all the skin tests to see if and what she may be allergies to.

And boy did we learn! Her tests came back as highly allergic to cats, most tree pollens, some weed pollen, and many types of mold. We were given 3 new allergy meds to add to our ever growing list of CF medications to try. It's our hope and understanding from Dr. KIm that her cough could be from a variety of reasons but, as of now she is being treated with these 3 new medicines in hopes that if it is caused my allergies or her sinuses that these medicines will target those issues and hopefully solve or alleviate her chronic night coughing. 

We return in 4 weeks to see Dr Kim to follow up with how theses medications may be working for her and if they aren't what the best next step would be to solving this chronic night cough problem.

Thanks for following Madeline's CF journey! We appreciate your support.

 
 

 

 

 

Quarterly CF Clinic -May 2016

We made the nearly two hour drive up to Hopkins for her quarterly CF clinic visit. It was the rainiest of days with loads of traffic but, we made it. We left the house at 8:30 am and returned home by 

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.

( Madeline's team felt today that getting an x-ray would be beneficial in making sure there weren't any problems going on in get lungs since she's had a lot of night coughing.)

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! ❤️

If you haven't donated yet to the CFF to support Madeline and others with cf, you still can by clicking the link below:

Washington DC 2016: Sandy Long - Cystic Fibrosis Foundation

Every penny counts! Thank you!

CF Clinic- August

We made the trek up to Hopkins.  This would be Madeline's typical CF check up
( usually patients go every 3 months) plus the annual work ups. Usually annual work ups for CF patients include a chest x-Ray ( to see in there is any inflammation, mucous plugs, and to give an other baseline of their lung health via picture), lab work ( usually to monitor vitamin levels since CFpatients don't absorb vitamins easily and have to be on specially formulated cf vitamins, check for liver enzymes- another complication of cf, etc.) We were also told by the clinic that Madeline would continue to " practice" doing PFTs like she did at last clinic visit. Around the age of 5 CF patients begin practicing a blowing technique for PFTs ( or pulmonary function tests). These tests usually gives a more accurate idea of what is going on in the lungs that an xray could not show. Oftentimes PFTs give doctors a variety of numbers that show airflow through and out of the lungs. Once the blowing technique or PFTs are mastered, data often can indicate infection, etc. PFTS are another way to monitor CF and unfortunately another thing to worry about as a cf mom. Numbers are everything. Right now they expect her numbers are normal since she is beginning to understand how to blow inhale and exhale correctly while putting this huge tube in her mouth. I've tried it and it's a ton harder than it looks!

Once there we went to the main hospital to get a chest x-ray This was a baseline x-ray. Usually CF patients get routine chest x-rays every 1-2 years to check for infections and abnormalities in the lungs.

waiting for x-ray

xray of lung

waiting to be called back to for stats

Stats check (weight, blood pressure, oxygen levels, pulse, and height)

Showing off her Elsa sticker she got from doing stats

blood draw

PFTs

waiting to see doctor

Dr. R and Madeline

Overall Madeline had a great visit. She is very tall - over 46.5 in inches which puts her at 93 percentile for height, 52 pounds at the 90th percentile for weight, and a BMI of 83%. We were pleased to see that her weight is staying in despite how tall she is continually growing.


Her x-ray came back as "normal" and she was incredibly brave during her blood draw- never crying. Her lab reports came back last week. They ran about 10 reports and close to ten viles of blood for various vitamin level tests ( since cf patients don't absorb vitamins as well as non cfers), liver enzyme levels, etc. thankfully all her vitamin levels and blood tests came back within normal range. We also got a call that she is still culturing "very light pseudo" . So no change in plans for her in the future. The clinic was very happy with her standings now. We will continue on with Tobi every other month. Starting kindergarten in September will definitely  make our schedules busy will full time school and numerous treatments. She's a trooper though and we are so thankful for her good health right now.
We know that CF can take a turn for the worst at a minutes notice so celebrating the healthy times are super important to us.

We hope as Madeline begin Kindergarten next week that her health continues to soar like it has in the past. We are nervous yet excited for another school year!

We would love for you to join our team or donate to our Great Strides Walk. Please click the link below to help support Madeline and others with CF. We thank you so very much!

http://fightcf.cff.org/goto/madelinesmarchers

Quarterly CF Clinic

We made the drive Friday morning to CF Clinic for Madeline's quarterly visit. Our last visit was at the end of January. Since we are cycling through TOBI every other month, this seemed like a good time to head for a checkup- April is our " off" TOBI month. It's usually a 2 hour trek to Hopkins so Madeline asked to watch "Frozen" in the car.  This may have been the 456,763,177 time she has seen it but, it did help the car ride seem quick for her.

Watching "Frozen" on the way to clinic 

First, we usually check in upon arrival and wait for the nurse to get Madeline's stats. Usually the nurse gets  her O2, height, weight, blood pressure, and respiratory rate. We are usually ushered back back out to the waiting room to sit until Madeline's name is called into the CF Clinic.

We bring an assortment of iPads, games, movies, etc to keep Madeline busy since these appointments usually take close to 2 hours from start to finish.

  

After stats... It's ipad time 

And more waiting for the pharmacists, nurse, respiratory tech, and doctor to 

go over Madeline's care at clinic. There are always so many people to consult with while we sit in one room at CF Clinic

Waiting for cf team 

After going over the many meds that Madeline takes with the pharmacist, adjusting 

dosages, and discussing any changes this is what our updated medication list looks like on a daily 

basis. Madeleine always takes her meds like a champ. No complaining and a lot of pill swallowing 

take places. She swallows over 17 capsules a day....

Madeline's med list

After meeting with the pharmacist, we met with the PFT tech.

She worked with Madeline this time "practicing" her blowing technique for her future 

pulmonary function test. Usually clinics start using the data from these tesst around the age of 6. 

So until she turns 6 Madeline will come to the lab each visit to practice. PFTs usually give doctors a 

good idea of how well her lungs are functioning and sometimes help discover what exactly may be affecting the lungs by the readings on her pft test. It's another diagnostic tool that cf clinics use to 

kept Madeline's lung health monitored. Usually when lung function drops there may be cf bugs, 

infections, etc happening. Madeline enjoyed checking out the pft lab and practicing. The doctor and pft tech noted that she did well for her very first time. They saw expected PFT values for a child her age just starting.

 

We came back to the room and waited for the doctor to do an exam.

And more waiting...

The doctor came in and listened to her lungs, looked in her ears, felt her stomach, discussed how she has been feeling, and finally took a throat culture. We visited with the nurse too. Both the doctor and the nurse were pleased with Madeline's progress.

Madeline gained 2 pounds between last visit in January and now. This put her at the 90th percentile for weight. She grew an inch since January and measures 45.5 inches now which puts her at 92nd 

percentile for height. Her BMI is now 85%. The doctor was very happy with these stats. They encourage cf patients to gain 1-2 pounds between clinic visits and maintain a BMI above 50% since lung function is directly tied to a higher BMI.

Overall, there were no changes to her cf medical plan. It's always a good sign that the doctor is very happy with how Madeline's health is progressing. No change = positive visit.

Now, we just wait for the culture results to come in next week. 

We are hopeful that no new bugs  will show up besides her typical " light pseudo".

We have our fingers crossed that  the results will be what we expect.

Please keep your fingers crossed for us.

Thanks again for everyone that thought of us as we traveled to clinic. All the Facebook comments of support are always appreciated. 

Cheers to a good clinic day. We celebrate each good one!