On the 4th day of #RareChristmas Phoebe wants you to know....the spoon theory by Christine Miserandino. The theory is simply a story that explains what it is like to live with a chronic illness or disability. A person who is chronically disabled or ill only has a limited amount of expendable energy each day. The spoon theory uses a metaphor of spoons to turn energy into a measurable concept. A person living with chronic illness or disability only has a certain number of spoons in their possession each day, and every small action a person takes can result in a lost spoon. Once a person loses a spoon, it is very hard to get that back until after a full night’s sleep. Simple actions like getting out of bed, taking a shower, walking, and driving can require enormous amounts of energy that people don’t have. These chronic illnesses or disabilities can be visible or invisible. You cannot look at a person and tell if they have a chronic illness or disability or not, and these illnesses are not always physical in nature.
On the 3rd day of #RareChristmas Phoebe wants you to know...living rare comes with limitations. You are not always able to do things other children your age do. You are not always able to experience things your siblings have as they have grown. You are not always able to participate in activities and gatherings with friends and family. Phoebe experiences many limitations each day. And no matter how routine her limitations are, or how "used" to them she has become, she still longs to be just like her sisters, able to do all the things they get to do. #12DaysofRareChristmas
On the 2nd day of #RareChristmas Phoebe wants you to know...living rare measn fighting to stay out of the emergency room. With the excitement of Christmas, looking at the lights, waiting for Santa and the joy of playing with her new toys, Phoebe has a difficult time pacing herself. She doesn't want to miss anything and does not take the rest or fluids she needs. There past few years she has needed trips to the ER the day after Christmas for IV fluids and rest. And while we have not had to make a trip to the ER yet today, she is balancing on the edge of possibly needing to go. So it is with the tricks her team of doctors have taught us that she is fighting very hard to stay at home. #12DaysofRareChristmas
On the 1st day of #RareChristmas Phoebe wants you to know...living rare usually means you have a large team of doctors that you visit frequently. Phoebe has a team of 16 doctos in 12 specialties across 3 states that work on her care. Having a team of doctors that size means she has an appointment with on of them just about every week, sometimes she has multiples appointments a week, and occassionally she is lucky to have a week with no appointments at all. But whether or not we have appointments during the week, we are in constant communication with her team of doctors discussing her case, reviewing results, brainstorming ideas, or updating on her current status. #12DaysofRareChristmas
Phoebe had her repeat CT to check the status of her middle lobe atelectasis and enlarged tracheal lymph node. Even though she still has shortness of breath and difficulty breathing, along with coughing, but we still hoped we would see some improvements since her breathing has been relatively managed on the two inhalers she has been on for the past three and a half months.
When comparing the two CT's side by side, her results proved to be more frustrating than we expected.
This CT was done without contrast (her original CT was done with contrast) so we did not get an image of the enlarged tracheal lymph node. So we do not know if the lymph node is the same size or if it has gotten bigger. Phoebe's lymph nodes enlarge due to hyperplasia (enlargement due to an increased rate of cell reproduction). Because they are enlarged from rapid cell growth and not just inflammed from fighting an infection, they do not reduce in size. They only get bigger. This lymph node will be something we need to find a way to monitor without needing multiple CT's, especially with it being on her trachea.
The CT did show the middle lobe atelectasis has improved. But they did find residual linear bandlike changes in the area. They also found multiple nodules through out both of Phoebe's lungs. The nodules range in size from 1 mm to 3-4 mm each. But what does all this mean?!
The linear bandlike changes are permanent scar tissue caused by the atelectasis. Irritants that we breathe in along with acute infections (like pneumonia) and chronic respiratory diseases (like asthma) all damage our lungs. This damage does not always completely disappear. Much like our skin when we get cut that leaves a permanent scar, showing where the damage was done.
The nodules, are a bit more complicated. Nodules in our lungs can be from irritants that we breathe in that get trapped inside the sacs of our lungs. In adults, the size of the nodules are what signals to a doctor if there is something to be concerned about, as well as determining what further testing is needed and how often to repeat imaging.
In children however....they do not have enough research to indicate any of that. They do not know what the size of the nodules mean in children, or how to safely follow or monitor the sizes of the nodules. The radiation risk to children from having frequently repeated CT's is high enough that the typical course to follow is to do nothing but manage the respiratory symptoms as they appear.
We have been waiting since the middle of October for today's appointment with Phoebe's GI doctor. The moment we became aware of her weight loss, Phoebe's team of doctors and I wanted to get her into her GI to see if she has a malabsorption problem. Especially given that she has such a good appetite and is eating a high number of meals a day.
As soon as the doctor came in the room she pulled up Phoebe's spread sheets to show her history of percentile rankings of heights and weights taken by all the doctors at Goryeb. (And since Phoebe has 10 specialists at Goryeb, there is a very thorough history in her records). What she noticed was drastic, steady declines in her height, weight and BMI since March of this year.
Up until March, you saw Phoebe's normal growth pattern. She would fluctuate a pound or two, plataeu for a little, but the record showed an incline. Everything after March showed nothing but decline. The BMI percentile was the most alarming to the GI doc. In March Phoebe was above the 50 percentile for BMI. But at today's appointment, she was below the 30 percentile. And she has not grown in height enough to warrant that kind of dramatic drop.
True to Phoebe form though, her blood work did not give us a clear cut reason for her weight loss. What happened next is one of the reason's why I love Phoebe's team of doctors so much and why, despite still having no answers, I know we have the best team working on Phoebe's care.
Phoebe's GI doctor said "children with chronic pulmonary, cardiac or neurologic problems have been proven to have a harder time gaining weight let alone maintaining their weight. Because of the extra work these systems have to do to work properly and the difficulties they can experience, the amount of calories a healthy 6 year old would consume are not enough for these children."
I have always kept Phoebe's entire team up to date on everything she goes through, tests other doctors order and findings other doctors find. I have always felt her entire team needs to be as up to date on everything as much as possible. Because of this, the GI doctor was aware of the pulmonary issues Phoebe has been facing, most especially in the past 5 months.
This doctor could have easily looked at us and said that since the problem did not appear to be specifically GI related, she was signing off on this issue. Instead, this doctor (like the others on Phoebe's team) looked at the whole patient, all the systems of the body, all the complications and difficulties Phoebe has, and tried to connect the dots of how all these other systems and difficulties play a role in her weight loss.
Phoebe's CT results, shortness of breath, difficulty breathing, and poor pulmonary function test results could be a major contributing factor to Phoebe's weight loss. And by following her spread sheet, the more difficulty Phoebe was having with her lungs/breathing, the more we saw her weight drop.
The GI doctor suggested we do two things. The first is a stool test to see if Phoebe is losing too many fats, Though she is predicting this test will come back normal, she wants to make sure we cover all our bases given Phoebe's history. The second is a food diary to calculate the caloric intake over three days. The diary will then be submitted to a dietician who will analyze what Phoebe ate while taking her vast medical complications and history into consideration. The dietician's recommendation will hopefully give us a better game plan than "eat 7, 8, 9 meals a day".
We submit the completed diary tomorrow evening. Since it's a holiday weekend, I don't believe we will hear anything until the end of next week, at the earliest.
A special holiday brunch was held by Jack's Kids today, complete with a special visit from Santa!! The love, care and dedication everyone at Jack's Kids puts into each child and their families is touching and inspiring!! To spend even a few minutes with these amazing people who volunteer their time to help ease the struggles of families facing serious childhood illness and/or disabilities, you know they live by their motto "It's all about the kids"!
Everyone at Jack's Kids has become our family and we are so blessed to have them in our lives and that Phoebe is a Jack's Kid!!
At the holiday brunch all the girls received a special gift from Sweet Dreams 4 a Cure! Personalized handmade pillowcases!! Dreamcases were created "in hopes of providing sweet dreams to anyone receiving treatment due to an illness" and their family members!
To learn more about Jack's Kids and Sweet Dreams 4 a Cure, click on their names above!
Photos: 1 - Santa greeting the girls with gifts
2 - Santa Photo
3 - Dreamcases from Sweet Dreams 4 a Cure
An evening full of Christmas festivities!
We started our night at the Holiday Lights Parade! Emergency vehicles are decorated with lights, inflatables and wrapping! Last year was our first attending this event, and the girls loved it so much we have made it an annual tradition! The girls giggled and waved as Santa led the parade! As the truck's lights lit up the road, the joy and excitement of the holiday beamed in the girls eyes!
After the parade we headed to the second annual BH Winter Walk! The event had so many wonderful activities for the kids to do! You could build your own snowman with Elsa, have a snowball fight in the Living Snow Globe, strike poses in the Photo Booth, touch the animals at the Petting Xoo and even stop by to say hi to a live Reindeer!
It truly was a magical first Saturday of December!
Photos: 1 - Waiting for the Holiday Lights Parade to begin
2 - Phoebe with her Snowman that she made at BH Winter Walk
3 - Playing in the Living Snow Globe at BH Winter Walk
4 - Visiting the Reindeer at BH Winter Walk
I know I haven't posted any updates about Phoebe's health since the beginning of the month. Part of the reason has been we have been in a stand still waiting for results before determining next steps, and I want to update you all as best as I can each time. But we have a very busy week of appointments this week, so here it goes...
Phoebe continues to still lose weight despite eating as much as she is. We took her in for a weight checks each week since the beginning of the month, and each time she steps on the scale she shows at least a 1 lb weight loss. To date, she has lost a total of 5 lbs. Because of this continuous loss the doctors drew blood to run a bunch of labs.
Her labs....as confusing as ever. She has some levels that are high and some that are very high. She also has some levels that have been low her entire life but are now in range. Collectively her team of doctors don't know what to make of these values and changes. What they all can agree on is that if you were to look at individual levels they point to specific diseases. But combining these levels to be read as a whole, she remains a medical mystery. This could be her immune disorder, or it could be something else. But with these individual lab results coupled with the weight loss, this cannot be ignored. So they want us to go back to the doctors office where this all began 4 years ago...The Valerie Fund.
We go to the Valerie Fund on Thursday, to see Dr Halpern. It's been a little more than a year since we have seen him in person. That seems to be the never ending pattern. We never go longer than this before something pops up sending us back to him. It was slightly comforting but equally heart breaking to hear "How is Phoebe?! We miss her but I was hoping we wouldn't hear from you to make an appointment" when I called to schedule her visit. Over the four years with the Valerie Fund we have grown close with the staff, as I am sure each parent who brings their child there does. It was a collective unspoken heartbreak that we were there again, no closer to answers, but being fully aware of her history and the great risks it carries.
On Friday, we go to Phoebe's pulmonologist. We scheduled this appointment in September for two purposes. 1) We had changed her inhalers in September, and they wanted to give her 3 months to see if there was any improvement in her symptoms. 2) We were to have a repeat CT and compare it to the images from the NIH to check the size of the tracheal node and see if the atelectasis improved, worsened or has remained the same. The CT was to take place on Sunday morning. Key word...was.
Since the original CT was done at the NIH, insurance was not involved. The NIH is government funded and they do not go through insurance at all. When we first saw her pulmonologist here in NJ he ordered an X-ray to see if we could see what the CT showed, hoping that would eliminate her needing the repeat CT (less exposure with an X-ray than a CT, and they didn't want to do another CT if we could see it on an X-ray). Well, this bit us. Because the X-ray was done through insurance, and true to Phoebe didn't show us anything, we got the call Friday morning that insurance was denying the CT.
Insurance feels the CT is unnecessary since there were no findings on the X-ray. The office tried to quickly calm me by telling me the doctor was appealing this decision and immediately requesting a peer to peer, it will just take time to hopefully have it approved. We are keeping our appointment on Friday to regroup regarding her inhalers and have a face to face discussion about the next step in appealing insurances denial.
So there I was, Black Friday shopping, crying about insurances decision. Heart broken that the inhalers have not substantially helped, knowing all this time at least part of her lung shows collapse, knowing she is fighting a cold and developing a bad cough because of it. Insurance can look at her entire medical record (we have had the same company since she became ill) and still deny necessities.
But as I was falling apart, something caught my attention. The song playing at the store I was in. ..."Fight Song". It was the reminder that I needed at that exact moment in time. We have fought every step of the way for the past four years. Though we don't want to be, we are used to this fight.
The true author of this blog is my daughter Phoebe. This is her life. I am blessed beyond words to be her mommy and to be able to share her story with you.