March Madness Elizabeth Jeanne Style
I haven't posted since the end of February, and that's because March seemed to end as quickly as it began for us. It was a mad, swirling, whirlwind of activity for Elizabeth and for all of us. It was truly our very own version of March Madness. Speaking of that, I didn't catch a single game this year of the March Madness Tournament. I didn't have time. It's a shame, because I find it entertaining, and I usually draft a killer bracket. Anyway, it's for the best because we had more important things to tackle here.
Inpatient Stay for Round Two of Sepsis/ Line Infection:
The first week of March, Elizabeth was admitted to Children's from the ER. She has a central line in her upper right chest, and she receives her TPN/ fluids for nutrition via her central line. There is a lot of care that is involved in taking proper care and using proper sterilization techniques when accessing, connecting a feed or medicine, disconnecting said items, etc. with her central line. When we left the hospital in November with a central line, I was trained at that time on how to do all of the care, procedures, etc. involved for all things central line. Yes, she qualifies for nursing, but most agencies only allow R.N.'s to access or perform any kind of central line care. It is hard to find RN's to staff as many hours as Elizabeth needs to fulfill all of her line care duties. Plus, what if someone calls off, and there there is her whole immune deficiency issue. With her immune deficiency, the less people accessing or doing line care on her the better, that way it is easier to maintain consistency with procedure. So, I decided at the start that I would be the one to do her line care, draw her blood work for labs each week, and change her central line dressing each week. I learned how to do it all, and it has just become part of our routine. I never imagined I would have to draw blood from my child, or have to learn how to put on sterile gloves like a pro, but I had to, and I did, and it is all working out pretty well.
Aside from all of the line care and sterile precautions, one thing that you have to keep an eye on is any type of fever in Elizabeth, even a low grade fever. Any kind of fever, even if other symptoms are present that could explain a fever (i.e. cough, cold, congestion, etc.), is a serious issue when you have a central line, and it is something that you cannot ignore. A central line is basically an open port into your body via a vein/ artery. For example, Elizabeth's central line is tunneled into her upper right carotid artery. It is very easy for bacteria to enter the blood stream when you have a central line for a number of reasons. Most of the reasons why bacteria can enter the blood stream via a central line can be prevented by using proper sterile techniques, but sometimes bacteria can still get in no matter how careful, or sterile, you are when accessing the line. Elizabeth can touch her central line. Celtan can touch it. A toy could touch it. Someone else could accidentally touch her line. Germs are everywhere. The possibilities are really endless. A fever is one of the first warning signs that there is a possible line infection. At the first sign of a fever, it is important to get her to the ER. The earlier you catch a line infection, the better. The longer the line infection sits, the greater the chances that the line infection can turn into full blown sepsis (a blood infection), because the line is a direct access to her blood supply.
Every day, I take Elizabeth's temperature at least twice a day. I also take her temperature any time she feels remotely warm to me. I have thermometers everywhere: in the car, diaper bag, my purse, in several rooms in the house. If I get a temperature of 100.5 degrees or greater, then that is an immediate trip to the ER to rule out a line infection. Elizabeth's immune system issues make her more prone to line infections. She has run a fever, and it was not related to a line infection, however, it isn't worth taking the risk. If the line infection develops into full blown sepsis with her, that is very serious, especially with her immune system. The first week of March, I noticed for two days that her temperatures were hovering in the 99 degree range. The first day they were 99.5, 99.7, and I went ahead and treated her with Tylenol. The Tylenol didn't do anything to keep the fever at bay, and the next day, her temps continued to hover in the 99 degree range. She seemed a little tired, and a little cranky, but she had no other symptoms. Typically, unless her temp reaches that magic number of 100.5, I do not need to call her doctor. She sometimes runs fevers due to her many complex immunological and endocrine issues. However, on the third day, her temp finally breached the 99 degree mark and was 100.4. I called the doctors, and they consensus was that she needed to come in to the ER to be evaluated for a possible line infection. So, we packed up and went to the ER at Children's. My dad came in and picked up Celtan, and Elizabeth and I headed to our home away from home.
My van is always packed and ready to go for hospital trips. It is like perpetually being ready to deliver a baby. I never know what each day will bring. I never know what will pop up any second with her, and it is always better to be cautious. So, we always have to be ready to go. Seriously, I can't even put groceries in the back of my van due to all of the hospital gear.
When you have a central line, and you develop a fever, it is an automatic 48 hour inpatient stay at the hospital. This is because they won't release you until you have 48 hours of negative line cultures when a line infection is suspected. By the time we got to the ER, her temp was already 102, and they started doing blood cultures on her. They take a culture from each of the two lumens/ ports on her central line, and they also take a peripheral culture from another vein in order to determine if the infection has spread into her blood stream. When the infection reaches your blood stream, it is no longer just a line infection, it is now a blood infection, which is called sepsis, which brings all kinds of nasty things with it. They also immediately start you on two big-gun antibiotics that are broad spectrum and basically cover all kinds of bacteria. After the cultures tell them which specific bacteria is growing, then they can tailor the antibiotic regimen to suit the specific bacteria that is growing in her line/ blood stream. In case you are wondering, the two antibiotics that they start with are called Vancomycin and Zosyn. After the cultures were drawn and the antibiotics were started, we sat in the ER and waited for our room on our home floor 7B to be ready. At this point, we knew we would be there at least two days, because you have to stay until they get 48 hours of negative cultures. Sometimes it takes a while for the cultures to show anything, which is the reasoning for the 48 hour rule.
About the time our room was ready, the Zosyn was finishing up, and it was time to start the Vancomycin. So, they started running the Vanc, and they sent us up to our room. We were greeted in the room as usual by her nurse, and an intake nurse. As I was going over her information with the nurses, which basically is me repeating the same things I say every time I'm there for the billionth time, I noticed that Elizabeth was pretty irritable, and Jason made a comment that she red. It was dark in her room, and we turned on some lights, and when we did we knew that what we saw was an issue. She was bright, flaming, red all over her face, head, neck, shoulders, arms and torso. I had seen this before, and so had the nurses, and we knew this would complicate things. The reaction we were seeing in Elizabeth is known as Red Man's syndrome. It is a reaction that you can have to Vanc, which is technically an allergic reaction. Unfortunately, Vanc is a type of antibiotic that is really useful in treating some pretty tough bacteria, and so having Red Man's as a reaction doesn't mean that they will not give you Vanc if a line infection is suspected. It isn't the same kind of allergic reaction as anaphylaxis, which is a serious life threatening allergic reaction. Red Man's syndrome really is an infusion reaction, and when you have Red Man's syndrome the treatment is to give Benadryl and to slow the infusion rate of the Vanc. They pretty much immediately gave Elizabeth Benadryl, which quickly helped to calm her itching and get rid of the rash. This also meant that from this point forward, Elizabeth would have to be given a dose of Benadryl a half an hour prior to her scheduled dose of Vanc, and it also meant that her infusion rate would have to be slowed down and given over two hours instead of being given to her over only one hour. I can tell you that Red Man's is very unpleasant, because I also have this reaction to Vanc, and so has my sister. Elizabeth has had many times Vanc before, but this was the first time she had ever had a reaction to it.
Once the Red Man's was addressed, we finished doing intake with the nurses. Jason went home, and Elizabeth and I settled in for the night. It was very late, but we went through our usual bedtime ritual in the hospital. We changed into pjs, put on Frozen, and we snuggled and watched the movie. Before long, she was asleep. So, I laid her down, and I laid down on my couch to try and get some sleep.
The thing about the hospital is that you don't get much sleep. In a few hours, I was woken up to the sound of Elizabeth vomiting and choking. I jumped off of the couch and was holding her up with one hand while pressing the call button with the other hand. Then, I started trying to help her clear her vomit. She can't sit herself up when she is sleeping, it goes along with her neuromuscular disease. So, when she gets sick while she is sleeping it can be very dangerous, because she can't sit herself up she can very easily choke and aspirate on her vomit. The nurse came in, and she helped me clean Elizabeth up. They put her on continuous monitor at that point, and they also gave her a medicine to help her with nausea. We were unsure if she was throwing up because of the infection, or as another reaction to the big-gun antibiotics. In my gut, I knew that she was throwing up because she had a line infection and sepsis. This echoed the last time she had a line infection/ sepsis at Christmas. The sudden and quick onset of the vomiting and elevating temperature. Her temp went up to 104.5, and only a combination of IV Motrin and Tylenol were able to bring it down. We gave her a bed bath, and once her fever started to go down, then she fell back asleep. By the time she fell back asleep, it was time for another blood pressure check, and then it was time for another dose of Benadryl and another dose of Vanc. So, neither of us got much sleep that first night.
We both got a little nap in the next morning. When the doctor's came in for rounds, they said that both her line and peripheral cultures were positive for gram positive bacteria, so this meant that she had both a line infection and sepsis. They were going to stop the Zosyn, but continue with the Vanc as it would be the antibiotic of choice to treat the type of bacteria present. They were also continuing the Tylenol and Motrin to help keep her fever down. I went to lunch with my friend, Liz, and was gone from the room for about an hour. Elizabeth was sleeping, so I figured I was safe to go to the cafeteria. When I got back to the floor, I knew there was trouble, as a nurse was standing in her door way intently watching her monitor. She said she was just about to call me when she saw me round the corner. Elizabeth's heart was racing, her respiration rate was rapid, her fever was spiking, and she started throwing up again. She was closely being monitored. Finally, after several medications to help stabilize her, her vitals returned to near normal. She slept soundly as my gray hairs continued to sprout on my head.
The weekend went pretty smoothly. They continued to do blood cultures, and by Saturday we had negative peripheral cultures. This meant that the antibiotics were clearing the infection from her blood stream. We were admitted on a Wednesday. On Sunday, we had negative line cultures, so the antibiotic was also clearing the infection in her line. The Infectious Disease doctors felt it would be beneficial to use Ethanol Locks in Elizabeth's central line as a preventative measure for line and blood infections, so the nurses trained me on how to instill and remove Ethanol locks from her line. We will continue the Ethanol locks indefinitely.
On Monday of the admission, they sent Elizabeth for a Barium Enema to further investigate the presence of a fistula that is connecting between her rectum and vagina/ uterus. She was not at all happy during this test. The test did not show where the fistula was located, which was frustrating to say the least. However, she continues to have stool come from her vaginal area, so there's clearly a pathway, they just can't find it. At this point, she had also picked up a nasty respiratory virus. Her nose was running and she had developed a cough requiring Albuterol. She was scheduled to have her Botox injections in her salivary glands on Tuesday, but the Anesthesiologist would not clear her for the procedure due to her recent sepsis and the current respiratory infection.
They discharged us on Tuesday, but due to issues with delivering the Vanc to our home in time for her 4 pm dose, we had to stay in the hospital until her 4 pm dose was completed. So, we were finally discharged around 7 pm. It was good to be home after being in the hospital for nearly a week. We were discharged and sent home on Vanc every 8 hours for the next 10 days, Ethanol locks in her lumens every day, and also changes to her feeding schedule. She had gained too much weight too quickly, and so they wanted to back off on her fluid intake. When we went in to the hospital, she was on TPN 22 hours a day, and she was on G-Tube feeds for 20 hours a day. When we were discharged, she was now on G-Tube feeds for 18 hours a day, and TPN for 12 hours a day. Between having to give the Benadryl a half an hour before each dose of Vanc, the Vanc running for two hours each time it was given and being time sensitive so it has to run at midnight, 8 am and 4 pm, establishing a schedule for Ethanol locks, and connecting/ disconnecting feeds, it was quite tedious. But, we managed and are still managing, without nursing.
When you have a central line, and you develop a fever, it is an automatic 48 hour inpatient stay at the hospital. This is because they won't release you until you have 48 hours of negative line cultures when a line infection is suspected. By the time we got to the ER, her temp was already 102, and they started doing blood cultures on her. They take a culture from each of the two lumens/ ports on her central line, and they also take a peripheral culture from another vein in order to determine if the infection has spread into her blood stream. When the infection reaches your blood stream, it is no longer just a line infection, it is now a blood infection, which is called sepsis, which brings all kinds of nasty things with it. They also immediately start you on two big-gun antibiotics that are broad spectrum and basically cover all kinds of bacteria. After the cultures tell them which specific bacteria is growing, then they can tailor the antibiotic regimen to suit the specific bacteria that is growing in her line/ blood stream. In case you are wondering, the two antibiotics that they start with are called Vancomycin and Zosyn. After the cultures were drawn and the antibiotics were started, we sat in the ER and waited for our room on our home floor 7B to be ready. At this point, we knew we would be there at least two days, because you have to stay until they get 48 hours of negative cultures. Sometimes it takes a while for the cultures to show anything, which is the reasoning for the 48 hour rule.
About the time our room was ready, the Zosyn was finishing up, and it was time to start the Vancomycin. So, they started running the Vanc, and they sent us up to our room. We were greeted in the room as usual by her nurse, and an intake nurse. As I was going over her information with the nurses, which basically is me repeating the same things I say every time I'm there for the billionth time, I noticed that Elizabeth was pretty irritable, and Jason made a comment that she red. It was dark in her room, and we turned on some lights, and when we did we knew that what we saw was an issue. She was bright, flaming, red all over her face, head, neck, shoulders, arms and torso. I had seen this before, and so had the nurses, and we knew this would complicate things. The reaction we were seeing in Elizabeth is known as Red Man's syndrome. It is a reaction that you can have to Vanc, which is technically an allergic reaction. Unfortunately, Vanc is a type of antibiotic that is really useful in treating some pretty tough bacteria, and so having Red Man's as a reaction doesn't mean that they will not give you Vanc if a line infection is suspected. It isn't the same kind of allergic reaction as anaphylaxis, which is a serious life threatening allergic reaction. Red Man's syndrome really is an infusion reaction, and when you have Red Man's syndrome the treatment is to give Benadryl and to slow the infusion rate of the Vanc. They pretty much immediately gave Elizabeth Benadryl, which quickly helped to calm her itching and get rid of the rash. This also meant that from this point forward, Elizabeth would have to be given a dose of Benadryl a half an hour prior to her scheduled dose of Vanc, and it also meant that her infusion rate would have to be slowed down and given over two hours instead of being given to her over only one hour. I can tell you that Red Man's is very unpleasant, because I also have this reaction to Vanc, and so has my sister. Elizabeth has had many times Vanc before, but this was the first time she had ever had a reaction to it.
Once the Red Man's was addressed, we finished doing intake with the nurses. Jason went home, and Elizabeth and I settled in for the night. It was very late, but we went through our usual bedtime ritual in the hospital. We changed into pjs, put on Frozen, and we snuggled and watched the movie. Before long, she was asleep. So, I laid her down, and I laid down on my couch to try and get some sleep.
The thing about the hospital is that you don't get much sleep. In a few hours, I was woken up to the sound of Elizabeth vomiting and choking. I jumped off of the couch and was holding her up with one hand while pressing the call button with the other hand. Then, I started trying to help her clear her vomit. She can't sit herself up when she is sleeping, it goes along with her neuromuscular disease. So, when she gets sick while she is sleeping it can be very dangerous, because she can't sit herself up she can very easily choke and aspirate on her vomit. The nurse came in, and she helped me clean Elizabeth up. They put her on continuous monitor at that point, and they also gave her a medicine to help her with nausea. We were unsure if she was throwing up because of the infection, or as another reaction to the big-gun antibiotics. In my gut, I knew that she was throwing up because she had a line infection and sepsis. This echoed the last time she had a line infection/ sepsis at Christmas. The sudden and quick onset of the vomiting and elevating temperature. Her temp went up to 104.5, and only a combination of IV Motrin and Tylenol were able to bring it down. We gave her a bed bath, and once her fever started to go down, then she fell back asleep. By the time she fell back asleep, it was time for another blood pressure check, and then it was time for another dose of Benadryl and another dose of Vanc. So, neither of us got much sleep that first night.
We both got a little nap in the next morning. When the doctor's came in for rounds, they said that both her line and peripheral cultures were positive for gram positive bacteria, so this meant that she had both a line infection and sepsis. They were going to stop the Zosyn, but continue with the Vanc as it would be the antibiotic of choice to treat the type of bacteria present. They were also continuing the Tylenol and Motrin to help keep her fever down. I went to lunch with my friend, Liz, and was gone from the room for about an hour. Elizabeth was sleeping, so I figured I was safe to go to the cafeteria. When I got back to the floor, I knew there was trouble, as a nurse was standing in her door way intently watching her monitor. She said she was just about to call me when she saw me round the corner. Elizabeth's heart was racing, her respiration rate was rapid, her fever was spiking, and she started throwing up again. She was closely being monitored. Finally, after several medications to help stabilize her, her vitals returned to near normal. She slept soundly as my gray hairs continued to sprout on my head.
The weekend went pretty smoothly. They continued to do blood cultures, and by Saturday we had negative peripheral cultures. This meant that the antibiotics were clearing the infection from her blood stream. We were admitted on a Wednesday. On Sunday, we had negative line cultures, so the antibiotic was also clearing the infection in her line. The Infectious Disease doctors felt it would be beneficial to use Ethanol Locks in Elizabeth's central line as a preventative measure for line and blood infections, so the nurses trained me on how to instill and remove Ethanol locks from her line. We will continue the Ethanol locks indefinitely.
On Monday of the admission, they sent Elizabeth for a Barium Enema to further investigate the presence of a fistula that is connecting between her rectum and vagina/ uterus. She was not at all happy during this test. The test did not show where the fistula was located, which was frustrating to say the least. However, she continues to have stool come from her vaginal area, so there's clearly a pathway, they just can't find it. At this point, she had also picked up a nasty respiratory virus. Her nose was running and she had developed a cough requiring Albuterol. She was scheduled to have her Botox injections in her salivary glands on Tuesday, but the Anesthesiologist would not clear her for the procedure due to her recent sepsis and the current respiratory infection.
They discharged us on Tuesday, but due to issues with delivering the Vanc to our home in time for her 4 pm dose, we had to stay in the hospital until her 4 pm dose was completed. So, we were finally discharged around 7 pm. It was good to be home after being in the hospital for nearly a week. We were discharged and sent home on Vanc every 8 hours for the next 10 days, Ethanol locks in her lumens every day, and also changes to her feeding schedule. She had gained too much weight too quickly, and so they wanted to back off on her fluid intake. When we went in to the hospital, she was on TPN 22 hours a day, and she was on G-Tube feeds for 20 hours a day. When we were discharged, she was now on G-Tube feeds for 18 hours a day, and TPN for 12 hours a day. Between having to give the Benadryl a half an hour before each dose of Vanc, the Vanc running for two hours each time it was given and being time sensitive so it has to run at midnight, 8 am and 4 pm, establishing a schedule for Ethanol locks, and connecting/ disconnecting feeds, it was quite tedious. But, we managed and are still managing, without nursing.
MRI Results:
At the end of February, Elizabeth was scheduled to have a series of MRIs to look at her brain and her abdomen. There was a scheduling issue, and they did not have time to complete all of the MRI's ordered, so they did the brain and a pelvic MRI. We had the results the next day.
The brain MRI showed that she still has several forms of congenital brain damage/ anomalies, which are unchanged since the MRI last fall. It is good that there have not been any changes in the damage already present, however the damage/ anomalies there are responsible for many of her health, developmental and growth issues. There is no way to fix or correct them. They will always be there. They will never go away or get better. We just have to hope that they don't get worse. Just to review, her brain MRI shows partial agenesis of the corpus callosum, white matter loss, undulating ventricles, small and cupped optic nerves, periventricular leukomalacia, asymmetrical ventricles, and thinning of the corpus callosum just to name a few. She continues to be followed by Dr. Hoda Abel-Hamid in Neurology at CHP for these findings, as well as for her Congenital Myasthenic Syndrome.
The pelvic MRI did not locate a recto-vaginal or recto-uterine fistula, however the radiologist noted that the MRI even with contrast is not sensitive enough to pick up a very tiny fistula. So, they decided then to do the Barium Enema for another look, which they completed when she was inpatient for the sepsis. They are currently consulting a Pediatric Gynecologist to determine if there are any additional tests that may show the fistula.
The abdominal MRI and Enterography was not completed due to a scheduling issue. We were in radiology for so long that day, because she had to be put under general anesthesia for the MRIs, that we missed our scheduled genetics appointment. So, we had to reschedule it. The appointment with Genetics is actually today (March 29th), and we will discuss the recent Whole Exome Sequencing findings regarding her having a mutation of the SON-gene.
The Freaking Flu
The night we were discharged from her inpatient stay with the sepsis, the flu struck our household. At 4 am, I heard the horrendous sound of Celtan vomiting. He threw up EVERYWHERE. I did laundry for three days. I scrubbed my whole house that next day from top to bottom with bleach. You could smell the bleach from outside of my house, and I had to walk down my stairs sideways for three days, because my legs hurt so bad from stepping up and down off of the step ladder that day while disinfecting my house. I was just done with germs and wanted them gone. Thankfully, neither Jason nor I got this flu, and it didn't seem to last long. Celtan was actually the only person who had it, and it was gone within 48 hours. However, for those 48 hours it was violently coming out of both ends. Elizabeth also had nasty diarrhea from the Vanc, and she also had thrown up, but it was because of the respiratory virus she picked up in the hospital, and she was coughing so hard it was making her throw up. Her diarrhea continued for another two weeks. For the next ten days, I was not only in the throws of an intense antibiotic regimen for Elizabeth, but every single day for ten days I had to strip one of my kids beds at least once a day due to a poop or puke incident. To say I was "over it" was an understatement. I don't for the life of me understand why it is so hard to get to a bathroom when you can walk and have to throw up, however, Celtan just can't seem to get there. Oh well, the flu is gone from here. I think it got them message from the bleach attack.
Other Appointments:
Elizabeth did have her botox injections in her salivary glands last week. While she was under anesthesia for the procedure, they also checked out her central line, as we were not getting any blood return from it. They were not able to repair her line, it had clotted and crystallized, so they had to replace her central line. She did well with both procedures. She never even needed medicine for pain once we were discharged, which was awesome. Elizabeth is super, super tough. I will take out her stitches around her new line sometime next week.
Elizabeth has also been going to "driving lessons" every Tuesday at the Children's Institute in Pittsburgh. In February, we ordered her a Power Wheel Chair, and the insurance approved several weeks of lessons. The thought process is that by attending the driving lessons, it will help her to be ready to maneuver around in her chair when it arrives. She is doing fairly well with the lessons. She knows how to use the joystick to move the chair, but it is very much when she wants to, and if she wants to, and what her attention is focused on. I'm hopeful that she will improve as we continue lessons. The power wheel chair will be a huge step in allowing her to be able to interact more in her environment.
We also had her IU evaluation this month for her IEP for preschool. An entire IU team came to our house to do her evaluation. It was very productive. Elizabeth is waiting for the evaluation to be completed so that her IEP meeting can be scheduled, and she can begin preschool at the Western PA School for Blind Children in May when she turns three. She will not only attend preschool there, but she will also receive all of her therapy there as well. Right now, we have 10 hours of therapy each week in our home for her that is given by a team of six therapists. Some of these therapists have been with us for over two years when we started Early Intervention for Elizabeth. It is sad to think that very soon, their work with Elizabeth will be done, because you can only receive EI services until the child is three. I will never be able to thank them enough for everything that they have done for Elizabeth and for our family.
We also had an I-Care appointment in March that was super hopeful and productive. The medicine that Elizabeth is on to help get her gut working again is working, and despite the potential for nasty side effects we aren't seeing any of those (so far) in Elizabeth. Since she is on a super low dose, we are going to continue to give her the medicine, even though she has been on it for much longer that is typical for protocol. When you have a child like Elizabeth, you are often faced with decisions where there is no win-win option. Everything is truly a cost-benefit analysis. Do the benefits of the medicine, procedure, etc. outweigh the costs? In this situation, the benefits that this drug has brought for Elizabeth have been overwhelming. This medicine has stimulated her brain-gut connection, and her stomach and intestines are now tolerating feeds. She also has passed a swallow study, is eating by mouth (small amounts) again, and is showing an interest in eating and progress as we train her to eat by mouth again. These are all great things. This medicine gave us an enormous amount of hope when we had very little because she was in intestinal failure, was not gaining weight on the TPN, and was not tolerating the TPN very well. We know that she will not be able to be on this medicine forever. We know that eventually the odds are not in her favor and eventually she will develop one of the nasty side effects, and we know that once she develops these side effects they are permanent and irreversible despite stopping the medication. We know that when the time comes to take her off of the medicine that this brain-gut connection will likely not remain stable, and we will be back to where we started with little options at that point. Right now, we are grateful that it is working, and we will just enjoy the ride, enjoy the progress.
Speaking of progress, she has gained weight so nicely, and has been maintaining her weight with the reduction of TPN from the inpatient stay for the sepsis, and her lab results have all been good, and the retraining her to eat and drink by mouth is coming along slowly but surely, and she has an pretty serious interest in wanting to eat by mouth, and because of all of these things she is no longer on TPN. Instead, at our I-Care Clinic appointment, Dr. Al-Issa, who I adore, switched her off of TPN and now for eight hours a night she receives normal saline IV fluids with 5 % Dextrose (D5) via her central line, she also is connected to G-Tube feeds for 18 hours a day, and she we are continuing to advance her diet by mouth as tolerated. They also took out her G/J Tube in clinic and put a G tube in it's place. We jokingly stated that now she is a "Straight Up G" again, with the change in the G-Tube. My need to find humor in all things is overwhelming, and the straight up G thing is funny. In fact, a friend, Megan Guntrum, made a hilarious pic about it. Elizabeth has been able to maintain her weight these past few weeks on just the normal saline with D5 in lieu of the TPN, and I am waiting to hear from her doctor, but this could me big things for Elizabeth. Like, that maybe she won't need any kind of IV fluids to help her maintain her weight and stay healthy and hydrated, and this means that we may be able to pull her central line. There are always a lot of unknowns, but there is always a lot of hope. This medicine has given us hope.
Let me be clear, that just because she is tolerating feeds now does not mean that she will forever and always tolerate feeds by mouth or into her gut. This in no way means that she does not have intestinal failure or continued gut motility issues. This in no way means that she will never be on TPN again, nor does it mean that she will never need a central line or a port again once the decision is made to pull her current one, nor does it mean that we are out of the woods in terms of intestinal functioning. This also does not mean that she is anywhere near the point of not needing a feeding tube, and truthfully it is highly unlikely that she will ever have her feeding tube removed. This is just current progress, the current state of affairs, and it can change at any moment, without warning, so we are left with no choice but to continue to hope that this progress continues as long as it possibly can continue. We see I-Care every month, so we will be seeing them again in April.
Speaking of progress, she has gained weight so nicely, and has been maintaining her weight with the reduction of TPN from the inpatient stay for the sepsis, and her lab results have all been good, and the retraining her to eat and drink by mouth is coming along slowly but surely, and she has an pretty serious interest in wanting to eat by mouth, and because of all of these things she is no longer on TPN. Instead, at our I-Care Clinic appointment, Dr. Al-Issa, who I adore, switched her off of TPN and now for eight hours a night she receives normal saline IV fluids with 5 % Dextrose (D5) via her central line, she also is connected to G-Tube feeds for 18 hours a day, and she we are continuing to advance her diet by mouth as tolerated. They also took out her G/J Tube in clinic and put a G tube in it's place. We jokingly stated that now she is a "Straight Up G" again, with the change in the G-Tube. My need to find humor in all things is overwhelming, and the straight up G thing is funny. In fact, a friend, Megan Guntrum, made a hilarious pic about it. Elizabeth has been able to maintain her weight these past few weeks on just the normal saline with D5 in lieu of the TPN, and I am waiting to hear from her doctor, but this could me big things for Elizabeth. Like, that maybe she won't need any kind of IV fluids to help her maintain her weight and stay healthy and hydrated, and this means that we may be able to pull her central line. There are always a lot of unknowns, but there is always a lot of hope. This medicine has given us hope.
Let me be clear, that just because she is tolerating feeds now does not mean that she will forever and always tolerate feeds by mouth or into her gut. This in no way means that she does not have intestinal failure or continued gut motility issues. This in no way means that she will never be on TPN again, nor does it mean that she will never need a central line or a port again once the decision is made to pull her current one, nor does it mean that we are out of the woods in terms of intestinal functioning. This also does not mean that she is anywhere near the point of not needing a feeding tube, and truthfully it is highly unlikely that she will ever have her feeding tube removed. This is just current progress, the current state of affairs, and it can change at any moment, without warning, so we are left with no choice but to continue to hope that this progress continues as long as it possibly can continue. We see I-Care every month, so we will be seeing them again in April.
Good and Fun Things
We were treated to tickets to Frozen on Ice from the Izzie's Gifts of Hope Foundation. We had a great time, and Elizabeth of course loved the show. So did Celtan. We all enjoyed it. We are huge Disney fans! We have received tickets for several events through Izzie's Gifts of Hope. It is an amazing organization, and if you're ever looking for an organization to donate to, I can tell you that they do amazing things for special needs families, and it would be a worthwhile donation. We cannot thank Izzie's Gifts of Hope enough for all of their generosity.
Also, we were recently notified that Elizabeth was nominated and will be granted a wish from Make-A-Wish. We are very excited about this. Right now, they are assembling a team of volunteers that will handle all of the details of Elizabeth's wish. Once the team is assembled, they will send the team of volunteers to meet Elizabeth, and so that she can make her wish. Stay tuned for more details.
Some Things I Need to Say
First, let me say again that we are super grateful for all of the kindness and generosity that people in our families and community continue to extend to us on this journey. You have no idea how much the kind words, messages, cards, gifts for the kids, gifts for the family, little anonymous surprises, the continued financial support from a few of the local churches and some local individuals, all of it means to us. I've been writing thank you notes for over a year now. I know I'm missing someone, missing groups, missing individuals, missing families that have been generous to us. It's not intentional. The generosity has been so overwhelming, and it has been very difficult to keep track of among everything else. We do what we can to pay all of this forward. Please know that you have lifted us up when we are weary, have helped us to go on when we think we can't, and have helped us to continue to hope when we felt there was none. Your prayers have truly helped Elizabeth out of many scary things, and they continue to help her. We truly, truly, truly appreciate it.
While so many have been so supportive to us, I still maintain that you cannot truly understand the joy and the sorrow this type of journey brings unless you live it every day. No matter how hard I try, no matter how "good" I am at telling you her story, no matter how many questions I answer, there is just no way to adequately describe this life. We wouldn't trade it for the world. We don't know how we do it, but we also don't know any other way. We are just doing what needs to be done to care for our daughter, and our son who has also suffered in many ways because of this.
In many ways, I don't want you to understand. While I wouldn't trade Elizabeth and all of her problems for the world, I also wouldn't wish this kind of life, this kind of heartache, this kind of journey on even my worst enemy. It is hard. It ages you. It ruins relationships with family and friends. It destroys marriages. I destroys you financially. It shatters dreams. It breaks your heart in more ways than you can count. It brings you sadness and feelings of hopelessness and helplessness beyond your wildest dreams.
There are many joys that it brings to you, as well. Joys that I cannot describe you in words. The littlest bits of progress are exciting in a mind-blowing way. The things you take for granted in your typically developing children mean so much more with a child like Elizabeth. The way this journey changes your perspective on life is nothing short of amazing. It has changed me, it has changed us, in many ways for better. I see the world differently. I see life differently. I see friends and family differently. It makes you appreciate things big and small even more. It makes you love a little deeper, smile a little brighter, find humor where there shouldn't be any, play a little harder, and just live every moment for what it is worth. We are blessed because of this. The blessings outweigh the burdens.
In saying that, Elizabeth has been making progress lately. She looks better than she has in a long time. These are all things that we are over the moon about. However, this is just a phase we are in. We know that even though we want desperately for all of her health issues to be a thing of the past, we wish that it didn't have to be her, that will never be the case for Elizabeth. I every day ask for God to take this from her and give it to me. Let me be the one to suffer, not her. It just isn't fair. It isn't right. It isn't that we have lost faith. It isn't at all that we have lost hope. It is just simply the reality of the situation.
Elizabeth doesn't have a disease that you can medicate or cure. The root of all of her health and developmental issues lie in things that cannot be corrected, or changed, or made to work no matter how hard you hope, no matter what kind of medicines or procedures you trial, no matter how good your doctors are, no matter how strong your faith is, nothing can fix what has caused all of these issues. Nothing. That's the hard truth of it. We know that. We accept it. We delight in the good periods of health and progress, and we face the periods of unknowns and uncertainty and sickness and decline as bravely as we can. We continue to give her the best care, get her to the best doctors, we continue to hope when we are told there is not hope, we continue to enjoy this blessing that we have been given. There is no other way.
Even though she is making progress now, she looks good, and all of those things, she still has many health issues, many developmental issues, and we are no where near out of the woods with Elizabeth. Things change and can change quickly. She has not been cured. This is not the end of the journey. There is no clear end in sight, except for an end that we can't fathom right now. I am to blame for this false perception that many people seem to have about her well being. I'm just as guilty as everyone else about posting "ideal" pictures of her where she looks great, or writing posts that lean on the positive side instead of writing the bitter truth all of the time. These things can easily bring false hope, incorrect impressions of her well being, and I apologize for that. I also am not saying that I have lost hope, or that I don't believe in miracles. I do. I live with a miracle every day. I will always hope. I will always pray. I will always believe that some doctor, some day will find some say to stop the mutations that plague my sweet girl in their tracks. However, you can't survive a journey like this without being realistic.
The reality is that Elizabeth has a series of genetic mutations that are responsible for her many complex medical and developmental issues. Her genes are mutated. Her DNA isn't coded correctly. This has caused major issues with Elizabeth's major body systems, down to the cellular level, and there is no way to fix that, or unmutate the mutation, or whatever. As much as we want and hope for it to be that simple, for it to be curable or fixable, it just simply is not. That is the reality. There are medicines that can help ease and manage some of the issues. There are doctors that know some of what to anticipate and what to expect and how to treat things as they come up with Elizabeth. But, more often than not, they don't know exactly what is going on, or how to explain what is happening, and this is mostly because the genetic mutations that she has are extremely rare. She doesn't have a common disease, or even an uncommon disease that can be treated and cured and healed by medicine or a procedure.
This is our reality. No matter how good she looks. No matter how much progress she is making. This is our reality. There is no way to cure this. There is no way to fix it. There is no way to end it. There is no way to stop the progression, although we can slow it in some ways temporarily. I don't talk about this often, because I don't want the focus on Elizabeth to be sadness or pity. I want her to be seen as the beautiful, sweet, miracle that she is every single day. We live this reality every day. The balance between knowing the reality and cherishing the blessing is delicate, but it is possible. The good days and the bad, they're all worth it. She makes it worth it. Elizabeth has more strength in her tiny body that almost all of the grown adults I know. She is the most amazing human I know. She is our blessing that outweighs any burden. No matter what the outcome, no matter what comes next, no matter what the next minute or hour or day or year brings, she makes it all worth it. I continue to be amazed by her, and I continue to maintain that her journey has a story to tell.
So, we tell her story, as we live it. We could choose to focus on all of the negative things, but we don't. We choose to know the reality of her situation, of her mutations, but we choose to operate with hope. We follow this path with her knowing that when you're the 5th known case in the world with your specific mutation/ genetic disorder that even the most highly trained professionals don't have all of the answers. We know that we don't have all of the answers. We know that we probably never will. What we do know is that we have been given a great blessing. We have the rare opportunity of watching a miracle unfold, beat odds, and bring beautiful surprises to our lives every day. She is our blessing, our miracle, our princess. The blessings on this journey always outweigh the burdens. Always, always, always.Thank you for following, praying, and for supporting our Warrior Princess. It means so much to us!
In many ways, I don't want you to understand. While I wouldn't trade Elizabeth and all of her problems for the world, I also wouldn't wish this kind of life, this kind of heartache, this kind of journey on even my worst enemy. It is hard. It ages you. It ruins relationships with family and friends. It destroys marriages. I destroys you financially. It shatters dreams. It breaks your heart in more ways than you can count. It brings you sadness and feelings of hopelessness and helplessness beyond your wildest dreams.
There are many joys that it brings to you, as well. Joys that I cannot describe you in words. The littlest bits of progress are exciting in a mind-blowing way. The things you take for granted in your typically developing children mean so much more with a child like Elizabeth. The way this journey changes your perspective on life is nothing short of amazing. It has changed me, it has changed us, in many ways for better. I see the world differently. I see life differently. I see friends and family differently. It makes you appreciate things big and small even more. It makes you love a little deeper, smile a little brighter, find humor where there shouldn't be any, play a little harder, and just live every moment for what it is worth. We are blessed because of this. The blessings outweigh the burdens.
In saying that, Elizabeth has been making progress lately. She looks better than she has in a long time. These are all things that we are over the moon about. However, this is just a phase we are in. We know that even though we want desperately for all of her health issues to be a thing of the past, we wish that it didn't have to be her, that will never be the case for Elizabeth. I every day ask for God to take this from her and give it to me. Let me be the one to suffer, not her. It just isn't fair. It isn't right. It isn't that we have lost faith. It isn't at all that we have lost hope. It is just simply the reality of the situation.
Elizabeth doesn't have a disease that you can medicate or cure. The root of all of her health and developmental issues lie in things that cannot be corrected, or changed, or made to work no matter how hard you hope, no matter what kind of medicines or procedures you trial, no matter how good your doctors are, no matter how strong your faith is, nothing can fix what has caused all of these issues. Nothing. That's the hard truth of it. We know that. We accept it. We delight in the good periods of health and progress, and we face the periods of unknowns and uncertainty and sickness and decline as bravely as we can. We continue to give her the best care, get her to the best doctors, we continue to hope when we are told there is not hope, we continue to enjoy this blessing that we have been given. There is no other way.
Even though she is making progress now, she looks good, and all of those things, she still has many health issues, many developmental issues, and we are no where near out of the woods with Elizabeth. Things change and can change quickly. She has not been cured. This is not the end of the journey. There is no clear end in sight, except for an end that we can't fathom right now. I am to blame for this false perception that many people seem to have about her well being. I'm just as guilty as everyone else about posting "ideal" pictures of her where she looks great, or writing posts that lean on the positive side instead of writing the bitter truth all of the time. These things can easily bring false hope, incorrect impressions of her well being, and I apologize for that. I also am not saying that I have lost hope, or that I don't believe in miracles. I do. I live with a miracle every day. I will always hope. I will always pray. I will always believe that some doctor, some day will find some say to stop the mutations that plague my sweet girl in their tracks. However, you can't survive a journey like this without being realistic.
The reality is that Elizabeth has a series of genetic mutations that are responsible for her many complex medical and developmental issues. Her genes are mutated. Her DNA isn't coded correctly. This has caused major issues with Elizabeth's major body systems, down to the cellular level, and there is no way to fix that, or unmutate the mutation, or whatever. As much as we want and hope for it to be that simple, for it to be curable or fixable, it just simply is not. That is the reality. There are medicines that can help ease and manage some of the issues. There are doctors that know some of what to anticipate and what to expect and how to treat things as they come up with Elizabeth. But, more often than not, they don't know exactly what is going on, or how to explain what is happening, and this is mostly because the genetic mutations that she has are extremely rare. She doesn't have a common disease, or even an uncommon disease that can be treated and cured and healed by medicine or a procedure.
This is our reality. No matter how good she looks. No matter how much progress she is making. This is our reality. There is no way to cure this. There is no way to fix it. There is no way to end it. There is no way to stop the progression, although we can slow it in some ways temporarily. I don't talk about this often, because I don't want the focus on Elizabeth to be sadness or pity. I want her to be seen as the beautiful, sweet, miracle that she is every single day. We live this reality every day. The balance between knowing the reality and cherishing the blessing is delicate, but it is possible. The good days and the bad, they're all worth it. She makes it worth it. Elizabeth has more strength in her tiny body that almost all of the grown adults I know. She is the most amazing human I know. She is our blessing that outweighs any burden. No matter what the outcome, no matter what comes next, no matter what the next minute or hour or day or year brings, she makes it all worth it. I continue to be amazed by her, and I continue to maintain that her journey has a story to tell.
So, we tell her story, as we live it. We could choose to focus on all of the negative things, but we don't. We choose to know the reality of her situation, of her mutations, but we choose to operate with hope. We follow this path with her knowing that when you're the 5th known case in the world with your specific mutation/ genetic disorder that even the most highly trained professionals don't have all of the answers. We know that we don't have all of the answers. We know that we probably never will. What we do know is that we have been given a great blessing. We have the rare opportunity of watching a miracle unfold, beat odds, and bring beautiful surprises to our lives every day. She is our blessing, our miracle, our princess. The blessings on this journey always outweigh the burdens. Always, always, always.Thank you for following, praying, and for supporting our Warrior Princess. It means so much to us!
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