Happy 7th Birthday to our rare gem! Phoebe, you have the most unwavering courage and strength, especially for someone so young. Your spirit radiates in everything you do! Your ability to greet each day with wonder and amazement in your eyes is a gift, one I hope you always have! I look at how you handle going through all that you do and you inspire me! You are teaching so many people along your journey and giving a voice to so many! You are a champion, my little fighter! I am honored to be your mommy! I love you puddin!
It has been a while since I have posted an update. With the summer ending we started our marathon of doctors visits to get letters, medical forms and other documentation needed for Phoebe's IEP, as well as just having our regular check up intervals with specialists. We are only a month into school and the visits/work needed to help keep Phoebe safe at school while still giving her as normal of an experience as possible hasn't let up. Add that on top of the other girls starting school, me working, me starting my BSN program and every day life....it's a balancing game. And while I have gotten pretty used to handling it over the past 5 years, when there is a medical emergency like my mom's eye surgery last week, sometimes I just need a moment to breathe. So I do apologize for not keeping you all more up to date on what has been happening with Phoebe. This update may be long but here it goes...
Phoebe is still on a altered school schedule. This year has been trickier than the previous two years, because 1st grade being required in NJ. (kindergarten is not). So a lot of what we have been doing throughout the past few months is getting Phoebe's restrictions documented in letters from her doctors. The reason we are still doing it....Phoebe is rare. There is no blue print or guide to tell us how to manage her care at school. We need to account for every detail of her day including gym, recess, other specials, walking in hallways, walking to the bathroom, using stairs vs using the elevator, having a stroller handy, extra earned recess, PT, OT and what she can do during the times something is going on that she is not able to participate in. We are by no means done figuring this all out for this school year.
What we have figured out so far is Phoebe is not to do gym or recess, and that includes extra earned recesses. Her lung issues are exacerbated by physical exertion, add to that her muscle fatigue and you get her doctors feel that it is not worth the risk for her to participate in these things. Also going along with her lungs and fatigue is Phoebe being accompanied when walking in the hall to go places in the school, including the bathroom. No matter how short of a trip it is, her doctors want someone with her. And, because her specials are on multiple levels of the school, her doctors think it is safest for her to use the elevator instead of the stairs to get to these places. In place of gym, she is receiving her PT and OT so she is not being pulled out of academic portions of the day. She also gets a double special with another first grade class for her second gym period of the week. In place of recess, Phoebe is reading, doing puzzles and playing in an area inside the school. I know some may ask "doesn't she feel left out by missing recess?". I asked Phoebe that very question and her response is "No mommy. I get to play with toys by myself! Anything I want without waiting!".
Now medically, it is even more complex as to what we are doing. We are still waiting to hear from the NIH about our return visit. They have a list of tests they do want to run, but are waiting for final word from the genetics team who has been researching and pouring over her genetic raw data. This way all the tests they want to run are being done on the same visit, instead of orchestrating multiple visits or materials being sent to NJ. While this is frustrating as it has been over 14 months since we were at the NIH, I keep telling myself that it has been 5 years since this began with Phoebe, so we can continue to be (sometimes) patient and wait.
In the meantime in NJ...
Phoebe's immunologist and geneticist are working to get a different type of genetic testing done for Phoebe. Mitochondrial DNA testing. She has been complaining that her legs feel heavy, she is tripping more, her legs are giving out more causing her to fall more, and she is asking for help when going up our stairs in our house because she does not feel safe going up or down them alone. What does this have to do with mitochondrial DNA testing? The mitochondria is the power house of the cell and has it's own DNA. New research is showing disorders associated with mitochondrial DNA. This is a potential lead in diagnosing her hypotonia and muscle fatigue. But new means rare (which is nothing new for us with Phoebe) which means insurance not wanting to approve the testing (again not new for us) because not a lot is known about these disorders or what to do for them. We have only just begun the process of getting approval for this testing, so it is a waiting game while jumping through hoops to get insurance what they are asking for so the test will be covered. Luckily, I have saved all the addresses I need for help if they repeatedly deny the test.
Phoebe's pulmonologist saw her today. With how short of breath Phoebe gets, especially when she exerts herself, he is suggesting an exercise test that will be done at Columbia.This will help give us an idea about how her lungs and heart are working, but it will also give us a look at how her body is using the oxygen she is taking in. The test is not too invasive. She will wear a mask on her face while she runs/fast walks. He did his own test with her today to see how she breathing and how her muscles were feeling by having her run back and forth in his hallway. She managed to do 5 laps, which is great, but he could see she the fatigue setting in more with each lap and how her lungs were overworking for her to breathe. He noticed that each lap took longer than the previous one and she began tripping more and more with each lap. So he is conferencing with Phoebe's cardiologist (who happens to work at Columbia as well as SMG) about arranging this exercise test for Phoebe.
All we can do now is wait..... Wait to hear what our next steps are for approval with the mitochondrial DNA testing..... Wait to hear when we go to Columbia..... Wait to hear when we return to the NIH......And wait to get into another appointment with her immunologist because Phoebe has even more enlarged lymph nodes, and they have begun to protrude worse then they have in a while.
As we wait, especially when it gets hard to wait patiently, as it is starting to, I close my eyes and say to myself "Roar". Cause when I open my eyes and see her strength, mine is renewed.
"I got the eye of the tiger, a fighter
Dancing through the fire
'Cause I am the champion, and you're gonna hear me roar
Louder, louder than a lion
'Cause I am a champion, and you're gonna hear me roar!"
Phoebe has been in the extended school year program for the past month through our school district. She has enjoyed the small class size, the work she is doing and seemed to be having a good experience. But last week, that changed. She was upset coming home from school last Thursday and when we spoke to her about why she was upset, asking how her day at school was, she said that her teacher told her she talks like a baby, and that she has to talk like a first grader, so it needs to stop.
Phoebe is very sensitive and her feelings can get hurt easily which can lead to her misunderstanding some things, so I reached out to her teacher for clarification on the matter. When the teacher responded to my email, her reply was "I am sorry if anything I have said has upset Phoebe. This summer, in addition to academics, the class has been working on speaking in an appropriate manner...I will commonly say to the class to...speak like a first grader. Moving forward, we will be mindful of this. Again, I apologize if what I have said has upset Phoebe".
I was shocked and didn't know how to take her reply. So we contacted the head of the special education department who is working this summer and requested she look into this matter further. Her initial response was that while she has sat in on the classroom and has heard the teacher address the whole class in say to not talk like a baby and to talk like a first grader (which did not sit well with us), that she wanted a copy of the exchanged emails and she would definitely look into it further.
When I forwarded her the emails last night, I also included the following note....
"My husband told me he spoke to you today and that you requested I forward the email I sent to (Phoebe's teacher) as well as her response. All of the emails should be below, including my initial email at the beginning of ESY explaining information about Phoebe, her symptoms and the systems affected, and a power point (her school nurse) made for an in-service for the staff at (her elementary school).
When I spoke to my husband during my dinner break at work, he told me that you sat in the classroom when (the teacher) said "we want you to start talking like first graders.".
I struggled for years about the thought of sending Phoebe to public school, concerned if she could physically handle it with her health and mobility issues, along with wondering how she would fit in, if the other children would accept her for how/who she is, and how she would handle facing her limitations and differences to other children her age. As she has gone through Kindergarten, she has become even more aware of how different she is to other children her age, along with her limitations compared to what other 6 year olds can do and handle. She has always known she is different from her sisters (two older and one younger), unable to fully keep up and do things they can do. But it has been hard for her seeing other children her age and realizing just how different she is.
While a small part of me understands wanting a child to stop "talking like a baby" as that child gets older going through elementary school, it is important to remember that every single child is different. And when it comes to children who experience learning disabilities, developmental delays, and other situations that can affect their education and daily lives, you cannot expect them to progress the same way a child who isn't facing those issues would.
I absolutely do not agree with someone, especially a teacher, even more so in extended school year program, telling either a whole class or an individual child they talk like a baby and need to start talking like what grade they are going in to. These children are in this program because they need the extra help so they do not fall behind when the new school year begins. What if these children cannot help the way they talk? What if that is how they, as first graders, talk? There could be any number of reasons for a child to be behind on their speech, and I do not think pointing that out by calling them a baby is any way to encourage or support a child in their attempt at as normal of an academic life as possible.
Phoebe has global developmental delay (among all of her other health issues). Her younger sister, who will be 5 on September 3, and has always progressed at a pace similar to my two oldest daughters. She speaks and understands at a higher level than Phoebe does, and has for years. I would never think to point out to Phoebe how she is talking "like a baby". That, to me, would only hurt her because it is pointing out and emphasizing the differences between her and her younger sister.
If there is a concern for the way Phoebe speaks, why would a recommendation not be made by (her teacher) for speech therapy rather than comments like that? (Phoebe was getting speech therapy through her IEP) I would think, if it was noticed that her speech is not where a first graders would/should be, a better way to approach the situation would be to reach out to the parent and request the services that would be most beneficial to be put in to place."
We are still waiting to hear back regarding this matter. But as much as I wold like to say this situation has not confirmed some of my fears about Phoebe attending public school I would be lying. Though this is the first situation we have experienced like this in her two years as a student of our towns school district, it most likely will not be the last.
This will, however, most definitely be something that I bring to the attention of her case manager, school nurse, guidance counselor, teacher and principles of her elementary school when we all meet to discuss our options and finalize our plan for her school year as a first grader.
Phoebe got to throw out the first pitch at one of the Jack's Kids 25th Annual Jack Gardner Softball Tournament Games! She also was picked to pull the winning ticket for the 50/50 raffle back at the lodge later that afternoon!
To be a part of this organization, for Phoebe to be a Jack's Kid, is such a blessing to myself, to Phoebe, to our entire family.
Hank, a policeman in my township, met Phoebe back in 2014 at the Bernards Township PBA Golf Outing. A short time after the golf outing, I ran into Hank at a local bank and we started to talk about Phoebe. It was during this conversation that he mentioned to me that he was on the committee for an organization called Jack's Kids. He explained all about what they do for children like Phoebe, how they help families who face challenges like we face, and how they could help our family. He gave me his contact information and encouraged me to to contact him with any questions I may have, or help I may need both while filling out the paper work and while waiting to hear from Jack's Kids.
The love, support, encouragement, friendships and family that have come into our lives from being a part of Jack's Kids, is something I will never be able to thank Hank enough for.
On Tuesday, with a temp going up to 102.4, Phoebe's on-call immunologist wanted her seen in the ER. Phoebe was diagnosed with strep throat. They gave her her first dose of antibiotic and were able to bring her fever down to 100.4.
Today is a different story. Phoebe officially doesn't have just strep. She has a viral infection on top of strep (possibly more). We had her follow up from her ER visit today with the pediatrician. She thinks Phoebe caught the virus first, which gave the strep the chance to run wild while masking the virus worsening.
She has been congested, sneezing, coughing, and wheezing. And she is frequently doing the grunt she does (described by her pulmonologist as a forced PEEP), which is a sign her middle lobe is being affected (middle lobe syndrome is common in people with hyperplasia). Her fever has not broken below 100, even with alternating tylenol and motrin.
Infections are a huge concern for people who are immunocompromised. With alterations in the normal defenses to fight against infections, It becomes a constant battle to try to keep them from getting sick, or trying to stay one step ahead when they do get sick. Things can spiral out of control quickly, numerous infections can happen at once, and available options for treatment become more weighted.
Bacterial infections can receive antibiotics. But sometimes, the antibiotics don't work. You find yourself having to switch to a different antibiotic, sometimes more than once, trying to find the right one that will help control and eliminate the source.
Viral infections, there are no antibiotics for them. All you can do is support the systems that are affected by treating the symptoms while it runs it course, hoping to head off any worsening progression. And unfortunately, when you already have a difficult time fighting off infections, worsening is inevitable.
So for now, Phoebe is continuing her ten day course of antibiotics for the strep and doing nebulizer treatments three times a day to support her lungs, while continuing her normal daily medications/inhalers. Her pulmonologist goes away for vacation starting this weekend for the first two weeks in July. Thankfully, he was able to squeeze us in tomorrow morning at 10 am.
As a mom, there are many firsts that you look forward to experiencing with your child. Their first smile, first tooth, first word, first step, first haircut, first birthday....stepping stones on a child's way to growing towards their first day of Kindergarten. The firsts go by quickly. In what seems like a blink of an eye. Before you know it, you are remembering when from years ago, even though they still like only yesterday.
With each first, comes the hopes and plans for the next. Dreaming of what the next day, week, month, and year will bring. Planning the next birthday party, arranging next weekends play dates, deciding on where to go for the next vacation, picking out the next size up clothing, wondering about the next school year, anticipating what first you will be celebrating.
Today, we celebrated a first with Phoebe. One we hoped she would get to experience, but were not sure she would be able to.
Her health has always made navigating school a tricky topic. It is difficult being able to find the right balance of time for her to be in school each day without it being too much to where her body and immune system cannot handle it. Making sure each day is beneficial to her education, supplying the necessary physical and occupational therapies she needs, arranging the early pickup times so ensure she doesn't 1) over do it, risking injury and 2) feel left out, excluded and different because she has to sit on the sidelines while her friends go to recess and gym because she is not cleared for those activities, all while making sure accommodations are set for any situation that can present itself can become overwhelming, stressful, leave you in a perpetual gray area of not knowing what to do.
The community we live in, and the school district the girls are in, is one of the main reasons Phoebe was able to have today. It has been their acceptance, concern, encouragement and compassion for Phoebe's situation that has helped Phoebe experience this first year at LCS. They have taken a special approach to handling her special needs, and have helped to create a very fluid, team built plan. A plan that addresses all of Phoebe's conditions, symptoms, restrictions, and concerns while still giving her a Kindergarten experience that she can benefit from and feel proud of.
The Kindergarten class of LCS (class of 2029!) had a moving up to 1st grade ceremony. All the Kindergarten classes participated in a processional to the stage where they recited poems, sang songs and had a video montage of the children's first year of school. Phoebe's eyes lit up like 4th of July as she walked down the center aisle to take her place on stage. She was so excited to be included in an event for her entire grade. I have never seen her smile so big!
And for as big as her smile was, mine was just as big. Tears gently rolled down my cheeks, most especially as I watched the video montage of the children's year at LCS and saw just how much Phoebe was able to be a part of.
There is still one week left of this school year, which means we have begun wondering what next year's school day will look like for Phoebe. But we will leave the wondering for tomorrow. Today, we are celebrating our almost 1st grader and a first we are so happy she was able to be a part of!
Every year we make a point to go on a family vacation. With everything that Phoebe and our family face throughout the year, it is important to us to make the time to go away together, reconnect with each other, and enjoy quality family time without the normal bustle of doctors and tests. And every vacation, we always go to the same place....Disney World.
But why? Why do we always go to Disney? And the answer is simple. Disney World has the best and most accommodations for children like Phoebe.
The stroller as a wheelchair tag keeps Phoebe safe. With having hypotonia and falling unexpectedly, her safety is always a top priority. Having this tag allows Phoebe to prevent injury and muscle fatigue by letting her remain in the stroller in the lines to meet the characters and ride the rides. On the rides that have a separate disability entrance, she is able to safely enter the attraction and have access to transfer assistance when it is needed.
The DAS allows us to get a return time, comparable to the standby wait time, for the character or ride. With the return time we are able to wait in an uncrowded area or in air conditioning. With having difficulty regulating her temperature, overheating and spiking temperatures, this pass allows us to help keep Phoebe cool and keep her from overheating. She is able to stay out of the heat and direct sun while still waiting her turn to see her favorite princess or ride her favorite ride.
The DAS also gives us the opportunity to control the crowd size around Phoebe. With her being immunocompromised and us knowing we cannot prevent all encounters with things that could make her sick, we do everything we can to limit exposure without isolating her. This pass gives us that. Instead of standing in an over packed line stuffed full of people, we are able to wait in a more open area, minimizing her contact with germs and pathogens but still being able to enjoy the sites, shops and attractions.
We also follow accommodations we have set in place with the help of her doctors to keep Phoebe as safe and healthy as possible during our trip down to Florida and while we are there.
We normally go during the girls school spring break. Because the summer months in Florida get brutally hot and Phoebe has difficulty regulating her temperature, overheating and spiking temperatures, we cannot attempt to go then. By going in April to mid May, during their spring break, it is warm enough to enjoy some beautiful weather but not too hot that it would make it intolerable and unsafe for Phoebe.
We travel with a diaper bag full of supplies. Supplies we know we will need like all of her medications and letters from her main doctor that itemize her symptoms and direct her care. And supplies we have just in case like a fully stocked first aid kit, ibuprofen and portable fan. The bag stays with us at all times, easily accessible. We also make sure our first order of business entering any Disney property is to locate the first aid station and the fastest routes to get there.
Instead of flying, we take the 17 hour drive down. It is a long and sometimes tedious drive, but in the case of an emergency it is easier to seek treatment being on the road than if we were in the air on a plane. It also helps limit the contact with the germs Phoebe would encounter on the plane.
Going to Disney is the one week out of the year that Phoebe doesn't feel different. Instead, she feels like she is exactly like her sisters. She gets to do everything they do without being told she can't or feeling left behind. It is the one week her limitations don't stand in her way. Disney is a place of magic, where dreams do come true. Especially for Phoebe.
That is why every year we will take our tax return and make the drive down to Florida. Because Phoebe deserves to feel that she is like everyone else, even if it is just for one week out of the year.
Phoebe's tonsils were removed when she was 2 because of her obstructive sleep apnea. They grew back within a year. We were told to wait and watch, that we wouldn't do anything until they started to cause complications again.
Her pulmonologist doesn't want to wait anymore. With all the lung and breathing problems she has been having this year, her snoring getting worse, how hard it is to wake her in the mornings from sleeping so restlessly, and how easily she tires out during the day, he's ordered a sleep study.
I was not aware of how many wires and what all was exactly involved in a sleep study. But even though I was a little nervous watching them hook everything up, Phoebe remained calm and still through the whole process. She didn't flinch or resist at all. And once everything was all hooked up and secured where it needed to be, she laid down, held my hand and fell asleep.
Phoebe slept through the whole night and was eager to go home when we woke up this morning. All she wanted to do was wash the paste out of her hair (they attached the probes to her head) and go to school. Her strength, bravery and determination continue to amaze me!
Now we wait and prepare.
Wait...wait for the results from the sleep study, praying they give us some insight or ways to help Phoebe get the rest and sleep she needs. We are so used to hearing "I don't know", "all we can do is treat the symptoms" and "let's watch and wait", that any help or information, no matter how small, would be a relief.
Prepare... prepare for the fight with insurance. We were switched to a new plan with a new company the beginning of April and it has been horrible! All the other fights we have had with insurance companies to cover services for Phoebe were nothing compared to what we face now. This new company will not cover anything until the deductible is 100% met, and once it is they only cover a percentage of the cost. What's worse, most of what Phoebe regularly goes through isn't covered and if it is, our cost is at least 4 times what it used to be. And that is just for what she has had done in the first month of this new insurance.
Phoebe had a pulmonary function test on Friday. The technician was quite impressed with her. She was the first kid the tech saw who was willing, happy even, to sit in the booth and shut the door to do the tests.
Phoebe's pulmonologist ordered extra tests given how bad her lungs got when she was sick last month. Now we wait for the results and to hear whether the pulmonologist wants to make any changes to her inhaler dosages or medication types.
Phoebe was finally able to return to school this past Wednesday. She was home from school for just under one month. Having the three viruses she did (RSV, coronavirus and human rhinovirus/enterovirus) really took their toll on her. Especially when she developed pneumonia on top of them.
When this week started she was only experiencing her "normal". But her normal takes it's toll on her, even without the added strain of an infection. Phoebe's normal includes swelling, fevers, fatigue, shortness of breath, muscle weakness, aches and pains...among other things. But sending her to back to school after coming off of being how sick she was, we were expecting her to be more tired and experience more flare ups.
And we were right. Come Thursday afternoon, Phoebe was completely exhausted and her flare up was in full swing. Even though she is on an abbreviated schedule regularly at school (three and half hours of school a day), and she had only been back for two days, her body was worn out, swollen and sore.
But true to the fighter she is, Phoebe insisted on going in to school today. She has missed her class, her teacher, and being able to go to school like her sisters that she wasn't going to let anything stop her. She did come home today and curl up in her pajama's underneath "white", her blanket she clings to when she isn't feel well.
Since it is now the weekend, she has a couple of days to rest and recover from her few days back at school. Hopefully her symptoms remain as her flare ups, and don't progress into anything more.
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.