Spending Rare Disease Day in the Emergency Room. Phoebe's fever is still going over 103.5 and she is not drinking enough to sustain her. Thankful the nurse has worked with Phoebe before and the doctor isn't " thinking horses". As a rare disease patient, it's professionals like these that make all the difference.
While we have successfully managed to avoid the other girls getting the flu or strep (our youngest was diagnosed with flu A, flu B and strep last Tuesday)...Our fear of Phoebe getting hit with one of the other viruses has happened. She woke up today with a temp of 101.8, curled into "that blanket" and went downhill very quickly. Her temp spiked up to 103.4 early this evening. We were able to use the late weekend office hours at her pediatrician to have her checked. No flu. No strep. But an unidentified virus that is crippling her with fever, fatigue and little desire to eat or drink while simultaneously consuming us with fears we know all too well...fears that are our reality with having an immunocompromised child.
Rare Disease Day is 8 days away!! Show how much you #CareAboutRareby going to globalgenes.org/careaboutrare to create your own framed image and #RoarWithPhoebe by sharing it on your social media accounts with #HugsForHope #WRDD201
Phoebe looks forward to the Father/Daughter Sweetheart Dance at her elementary school every year. She always watched her older sisters enjoy the evening by getting dolled up in a pretty party dress with their hair done just right. Last year was Phoebe's first sweetheart dance, as a kindergartner at the elementary school. It was everything she had hoped it would be and more!
With how easily her body tires out and her increasing respiratory and muscle issues, she couldn't stay for the entire dance last year. But this year....as a first grader attending with her younger sister and one older sister....she wanted to try to stay the whole night! And she did!!!
Staying did come with a price though. She pushed herself so far past what her body could handle, despite repeated reminders from her dad to take it easy and slow down, and collapsed when she got home completely exhausted. But with her feeling like a beautiful princess in her special party dress and with her perfect bouncy curls...there was no stopping Phoebe from going all out!
Winter months are proving to be incredibly hard for Phoebe. Coming down with a cold increases her respiratory issues and usually results in middle lobe atelectasis. But with the heightened flu season this winter, Phoebe is at an even higher risk. Though we do our best to keep her as safe as possible, it isn't easy and doesn't prevent her from getting respiratory infections or ending up in the ER.
I had to pick Phoebe up from school today because she was complaining of ear pain in the nurses office. By the time I got to the school I had already made a doctors appointment with the pediatrician. As I pulled into the school I had no idea that her ear pain was only one of the issues we would be facing by the end of the day and that waiting two hours to get into the pediatrician wasn't going to be an option.
In the drive from her school to our home, Phoebe's temperature had spiked from 99.4 to over 101 and she began to scream and cry in pain. With the pediatrician's office closed for lunch and her appointment a few hours away, the ER was the quickest way we could get her seen and out of pain. Unfortunately, as with a lot of the times we are seen in the hospital, the doctors and nurses were not sure how to handle Phoebe's care with her multitude of symptoms and undiagnosed status. They suspected that she had an ear infection but Phoebe's ear canals are smaller than they should be and they did have a good amount of wax in them. Which made it very difficult for them to tell. The doctor did attempt to clean Phoebe's ears out, but was hesitant to do much since she was so unfamiliar with the extensive history Phoebe has and didn't want to cause any more pain or any further issues. The ER was able to ease her pain and help bring down her temperature but insisted we follow up with her pediatrician to be extra thorough and see if there is anything they missed.
We were able to see the pediatrician later in evening, a few hours after what would have been our appointment time and they, being very familiar with Phoebe's anatomy and what to watch for, were able to diagnose the ear infection that was causing so much pain. During the physical assessment they also heard diminished lung sounds in the right lobe and discharge starting to be evident in her right eye. Their prescribed course of treatment.....antibiotics for the ear infection, eye drops for the discharge and albuterol nebulizer treatments, as preventive measures, for the diminished lung sounds.
If today shows anything about Phoebe, it's that she can wake up one way and within a short amount of time spiral downhill quickly. Symptoms are not always present all at once, often times appearing one right after another and very quickly. It's why we are so cautious with her, especially in the cold and flu season.
On the 12th Day of Rare Christmas Phoebe wants you to know...living rare can mean you have to be aggressive, especially during cold and flu season. With a compromised immune system, a simple cold for us is bronchitis or pneumonia for Phoebe. And with her middle lobe being susceptible to collapsing, things can get very dangerous very quickly.
Phoebe is still not presenting with definable symptoms of an illness...no fever, no cough, no vomit..nothing. Her only visibl...e symptom is that she continues to arch her back and take a giant breath in. She has continued to get frequent breathing treatments along with her regularly prescribed inhalers, but it has not made enough of a difference to make us or her doctors feel that we are not battling some type of illness. This morning her doctor informed us that there are a couple of pneumonias circulating throughout the towns and schools, and given Phoebe’s health history it is best to start treating her aggressively.
They have started her on a course of antibiotics for an acute lower respiratory tract infection and want us to continue the frequency of breathing treatments. As her doctor said, her health status and history make waiting this out not a viable option. Waiting versus beginning treatment could mean the difference between heading this storm off at the pass or something terrible happening.
While this course of treatment doesn’t take Phoebe completely out of the woods, we are praying that we are at least one step ahead of what is going on so that she doesn’t get worse.
On the 11th Day of Rare Christmas Phoebe wants you to know...“cold and flu germ-laden droplets may remain infectious for several hours, depending on where they fall. Germs generally remain active longer on stainless steel, plastic and similar hard surfaces than on fabric and other soft surfaces. Other factors, such as the amount of virus deposited on a surface and the temperature and humidity of the environment, also determine how long cold and flu germs stay active... outside the body.” (Mayo Clinic). We are in the midst of the cold and flu season, which makes protecting those who do not have a strong enough immune system to protect themselves that much more essential to do. Some ways that we can protect the immunocompromised and prevent the spread of infection include:
-Hand washing with soap and water. This is the number one way to help prevent the spread of infection. This will help prevent the germs from being on your hands and being passed to objects and people you touch.
-Cough and sneeze into your elbow. This will help prevent the germs from being on your hands and passed on to the people and objects you touch.
-If you are sick, stay home! My girls schools have guidelines of when to keep them home from school. These guidelines are not only for your own safety but also for the safety and health of those around you. These school guidelines are ones that we should all use in our lives...including when we need to go to work or have plans with family/friends...not just when deciding if our children should go to school. And not just for protecting the immunocompromised. These guidelines protect us all!
“Guidelines for if you should keep your child home from school:
*Unusual fatigue, paleness, lack of appetite, confusion and/or irritability
*Children should be fever free (<100°F) for 24 hours without fever reducing medication before returning to school.
*Children should be free of vomiting and diarrhea for 24 hours before returning to school
*Acute cold or sinus issues
*Inability to sleep most of the night because they don't feel well or wake not feeling well
*Sore throat, especially if combined with a fever or swollen neck glands. If your child has been diagnosed with strep, he/she needs to be on antibiotic for 24 hours prior to returning to school
*Drainage from the eyes
*Rashes unrelated to heat or other known non-contagious cause. If you child has a rash, a note from the physician stating that the rash is not contagious is warranted”
On the 10th day of Rare Christmas Phoebe wants you to know….living rare makes your health unpredictable. With Phoebe, she can start the morning feeling good and acting fine. But things can quickly take a turn and start to snowball downhill. And usually there is no real warning that things are going to turn bad so abruptly. While we do know what symptoms to watch for and specific “Phoebe clues” to keep an eye out for, more often than not when things go bad with her they go bad fast and seemingly out of left field. Today is the perfect example.
Phoebe’s middle lobe of her right lung is susceptible to collapsing. Middle lobe syndrome (as it is called) is common in patients who have a history of hyperplasia...which Phoebe has (every lymph node biopsy has come back as hyperplasia). Hyperplasia is when there is an increase in the number of cells in an organ or tissue due to cell proliferation (multiplying)...why Phoebe’s lymph nodes are so large.
Phoebe is at risk of the middle lobe collapsing randomly because she has hyperplasia, but she is highly susceptible to it happening when she is sick. And she has a tell tale sign that we are able to see when she is experiencing her middle lobe starting to go down. When she goes to take a breath she arches her back and takes a giant breath in, doing everything in her power to get the oxygen she needs into her lungs.
The scary part of this (aside from watching her take breaths like that) is when she is not displaying any symptoms of being sick and starts to breathe like that. It’s times like that, like today, when we do not know if it is happening because of the hyperplasia or if it is happening because she is sick and just hasn’t started to show symptoms.
And with her immune system being what it is, we are now in a battle to try to keep her home and out of the hospital. Her doctors share our concerns and cautions about sending her to the emergency room when things start to go like this with her. Exposing her to the germs in the ER and the inpatient unit (if they admitted her) increases her risk of respiratory distress and failure.
If you watched Phoebe get off her bus at 3:20 this afternoon, you would have never guessed that this would be the turn of events we would experience this evening.
So for now, we are administering frequent breathing treatments with constant monitoring until we can get to the doctor first thing in the morning, praying more unpredictable events do not happen in these wee hours of the morning.
On the 9th day of Rare Christmas Phoebe wants you to know…living rare scares you. It is not just the lack of a diagnosis or the lack of treatments that scare you. It’s seeing how bad things can get and not knowing if that’s the worst of it or if it could get much much worse. It’s not being able to explain to your child why they are going through what they are. It’s feeling like you aren’t doing enough...fighting enough...to get your child the services they need. It’s having to trust other people to provide care for your child (doctors, hospitals) when they have never cared for a rare undiagnosed patient. It’s feeling unheard when you need to keep explaining your child’s medical history, symptoms and risks. It’s wondering if the letters from the doctors will be followed or brushed off. It’s feeling like your fears and concerns are being dismissed by others. It’s seeing your child pushing themselves beyond their limit because they want to be like the other kids. It’s trying to give your child as normal of a life as possible while keeping them as safe and healthy as possible. It’s everything you know and everything you don’t know that scares you.
*photo from our November trip to Kozair's Christmas Village..each of our girls put in money and rang the bell
On the 8th day of Rare Christmas Phoebe wants you to know…living rare makes it hard to plan. On January 1st, people talk about the resolutions they made for the new year….things they plan to do, plan to give up, plan to not do...how they plan to make the new year better than the last.
Phoebe’s health limits us in the plans we make and the plans we keep. Too many times we have made plans and at the last minute had to cancel or leave early because her health couldn’t handle the experience. It is not easy as a parent to see the disappointment on any of the girls faces when we aren’t able to do something or experience something fully. And while we do our best to still allow the other girls to experience as much as they can, it is a delicate balance that is seemingly impossible to walk. On one hand you don’t want Phoebe to feel left out because she isn’t able to go or has to leave early, while on the other hand you don’t want the other girls to feel they are missing out because they can’t go or have to leave early because Phoebe isn’t feeling well.
All we can do is hope...hope that if we make plans we are able to keep them...hope that we can keep the delicate balance of all the girls being able to experience and do as much as possible...hope that people will understand if we have to cancel last minute or leave early...hope that Phoebe can do more than she did last year
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.