I have been pretty quiet on social media for the last few months. The past two months, I took a complete break from all social media. While we have historically been open about the challenges facing our girls and what was going on in our family, we have kept a lot to ourselves since the beginning of this year. While we entered 2018 with the hopes that it would be a year with less illness, hospital stays, and challenges, that was not to be the case. Maybe the fact that we rang in the new year in the ER should have dampened our optimism, but sometimes you need a little hope to keep going. This year quickly became the worst year of our lives. I am not yet going to talk about Addison and the challenges she faced this year, the changes in her health and prognosis and the effects it has all had on our family. I do want to say that we received so many messages and cards filled with love and support. While we have, for the most part, not responded to them, know that we have read every single one of them and that we greatly appreciate all the love and kind words everyone sent us. In time, I would like to share our story and my version of the last few months. Thanks to everyone for respecting our privacy, allowing us time, and giving us so many acts of kindness. I’m very, very slowly working my way back into a world that has been completely altered.
This update is long overdue, but the past couple of weeks haven’t seen a lot of progress and there has been a lot of discussion about where we go from here. Addison is still in the PICU. She was admitted and intubated on Thursday the 25th and extubated on Sunday the 28th. She has been on bipap since then. She’s had lots of ups and downs. She was fighting a lot of secretions and needed frequent suctioning up until the past few days. She has also had a lot of trouble maintaining her oxygen saturations – she was up to 100% oxygen several times. Her tendency to obstruct her airway has been a huge hurdle. Her bipap settings were weaned down and then increased again a few different times. Looking back over the past week, she has made a lot of progress – it has just been very slow. She is doing trials off the bipap during the day now. She actually had two four hour windows off the bipap yesterday, and she was on room air for most of that time. This was huge because prior to yesterday, she was on a mask with 28-50% oxygen during her time off the bipap. She also frequently wasn’t able to complete even an hour off the bipap for several days. Today, our goal was two five hour windows off the bipap. This morning she made it four hours before her oxygen levels dropped and her breathing became too shallow and she had to go back on the bipap. Currently, our goal is to get her to a point where she only needs to use the bipap at night and can be off it during the day. If she can do well with that schedule, we can go home with a bipap and continue using it only at night and possibly naptime. We would follow up with pulmonary and hopefully be able to wean her off the bipap more after she’s home to (ideally) get her back to using only a small amount of oxygen while she sleeps. She is back on her home feeding schedule and doing absolutely fine with that. This stay has been a lot of one step forward, two steps back and a lot of really difficult days. Of course, balancing time spent at the hospital with time spent at home is always challenging and it becomes even more so after a couple weeks. She’s doing better for the moment, but we don’t know how much longer we will be here. She has been acting more like herself the past few days and has been more alert and smiling at everyone, which is very reassuring. She has so many friends here in the hospital that she is never lacking attention or someone to tell her how cute she is. As always, thanks for all the well wishes and prayers.
I was really hoping that 2018 would be a year with less illness and hospital time, but it hasn’t started out that way. Addison is a girl who always has her own plans. Here’s a quick recap of what Addison has been dealing with over the past couple of months. We brought her in to the hospital Thanksgiving night and she spent two weeks in and out of the ICU. She (as usual) had a cold virus. She was on the vapotherm for a while, started doing better quickly and was transferred to the regular floor. Then, she started having a lot of seizure activity and her breathing worsened again and she was sent back to the ICU for a few days. After two weeks, they sent her home to finish recovering and I finally managed to get a pulse oximeter for home (yay!). She finally started getting back to her normal self after a couple of weeks at home and then she got sick again. I was able to manage her pretty well at home, but after almost two weeks, she just couldn’t shake what she had; I thought she maybe had pneumonia or needed steroids. She obviously needed something I couldn’t give her. We ended up ringing in the new year in the ER. Turns out she did just need some steroids and some deep suctioning. She only spent two nights in the hospital at that time. We finally recovered from that mess, got her back into the routine of PT/OT and preschool. She had a really great week this week – she was finally awake and happy at school. Thursday morning while I was getting her ready for school, she started having trouble breathing. I kept her home and tried inhalers and breathing treatments and oxygen and suctioning and her vest, but her breathing and oxygen sats just keep getting worse. So we were on our way to the ER by 11:30. Long story short – Addison has pneumonia. She tested positive again for the cold viruses she had back at the beginning of the month. Thanks to the pneumonia, they were not able to keep her oxygen levels up with vapotherm or bipap, so she was intubated last night. Her blood pressure was consistently too low, so she got a central line in her neck in order to give medications to bring her BP up and an arterial line to better monitor her blood pressure. She’s got what seems like a million IV medications running. Girlfriend was a hot mess all day and all night, but she seems to have settled down for the past few hours. I had a feeling yesterday that this illness was going to be a bad one and that she was going to end up on the vent. Sometimes, I hate being right. Plan for today is to get her oxygen requirements down and then maybe start weaning some of her vent settings. She’s also got more bloodwork this afternoon to check her electrolytes that were low and replaced this morning. Her blood pressure has normalized for now. She got a lot of fluid overnight and now she needs to get rid of some of that extra fluid, so hopefully she will start peeing some of that out on her own. Basically, we wait and watch and see what our little diva decides to do over the next 24 hours and we go from there.
After a long five month wait, Addison had her hip surgery this morning (Thursday the 21st). Thanks to the many, many abdominal x-rays she had in March due to her feeding tube issues, we found out that her hips were dislocated. We had known for quite some time that she was at risk for this due to her increased muscle tone. She had her first appointment with the orthopedic surgeon in April. After a lengthy discussion with him, and a couple of her other doctors, we decided to go ahead and schedule surgery. We felt that it was definitely worth it to be aggressive. Leaving her hips dislocated could have potentially caused more problems down the road (such as problems with her spine and reduced cartilage in her hip joints – which would have made fixing the problem in the future impossible).
Both of Addison’s hips needed to be relocated. We were very fortunate because she ended up requiring the less involved procedure. Basically, the surgeon only had to adjust the femur to get her hips in proper position, and did not have to do a larger incision to get to her pelvic bones. This also meant that she did not have to have a lower body cast, which definitely makes our lives and her recovery much easier. They lengthened some of her groin muscles so that they don’t pull on her hips.
Surgery itself went well, it only took about three hours (she was orignally scheduled to take about five). She was able to come off the ventilator in the recovery room. She still ended up in the PICU, because it is her home away from home. Seriously though, she has an epidural to help control her pain and with her history of breathing issues, we all figured it would be the safest place for her. She has been fairly stable after surgery. She was originally on plain oxygen, but was having some troubles with airway obstruction and apnea and after trying a few other things, she ended up on the trusty vapotherm. She has a PCA pump in addition to the epidural. She pooped all over herself at the end of surgery, so she’s getting some antibiotics just in case. Addison decided she needed to add a drug allergy to her health history, so right after they hung clindamycin, she broke out in a rash. She earned herself a hefty dose of benadryl and a steroid. She definitely doesn’t like being bothered, but she’s at least fairly comfortable. It was a pretty eventful 3-4 hours after she arrived in PICU, but hopefully she will have an uneventful night as it seems she has finally settled down a bit. Violet and Oriana had a fun day with Grandma and their first demand when we video called was to see Addison. Their biggest concern throughout our discussions about Addison’s surgery has been confirming that we will bring them to visit her. We have, of course, reassured them that we will do so and that they can see her on a video call every day. It’s been a long day, so I’m off to see what sleep I can get on the lovely couch in Addison’s room.
Sooo, I don’t know if y’all heard about this craziness, but the girls turned three
a couple of weeks ago a month ago. I’m way behind on the blog and even on facebook updates; it’s been pretty crazy around here since May, but that’s another post. THREE! How is it possible that these girls are three?! It feels like three years have flown by, yet at the same time it feels like the past three years have taken forever. It was really fun to celebrate their birthday this year, because Violet and Oriana really started to get what it was all about. They were so excited to have a party (parties are their new favorite thing). They are slightly obsessed with the color pink right now, so we had a pink party. Since things have been so crazy, we kept it pretty simple – pink balloons, streamers, plates, cups, utensils, pink icing on the cupcakes. The girls all had pink flower clippies for their hair and we asked their guests to wear pink if they wanted to. They had a great time with all of their favorite people. Plus, they got to eat pizza and cupcakes, so they were pretty thrilled. Mother Nature cooperated and even though we had thunderstorms and hail earlier in the day, it cleared up and we had a beautiful evening for a party. They got lots of wonderful gifts. They have amazing, caring, generous people in their lives and we are extremely grateful.
They got to eat donuts for breakfast on their actual birthday, and then we took them on a walk to the park to run off all the sugar. They also got to go out to eat for dinner, which is one of their favorite things to do and something that we don’t frequently get to do any more. Violet and Oriana got to have their favorite meal from Grinders (chicken fingers) and we experienced the generosity of strangers when the table behind us paid for most of our meal. It was a really nice surprise and we appreciated it very much. It’s so easy to think about all the negative in the world and it is so nice to be reminded of kindness and good things.
I think we managed to give our little ladybugs a pretty awesome birthday weekend. They still talk about it occasionally. It’s so much fun to see all these experiences through their eyes. Thanks to everyone who helped make their birthday great!
Well, the shit show that has been our lives since February continues. Another hospital stay. I’m so tired I can’t even think straight any more. I’m tired of the four walls of this hospital, I’m tired of shitty cafeteria food, I’m tired of our lives being in total turmoil, I’m tired of listening to my child cry, and I’m tired of not getting any answers. I’m not sure I even know what our “normal” lives were like any more. Addi has landed herself in the PICU again – I’m pretty sure it’s her favorite place; it’s starting to feel like our home away from home and we know far too many people here. Which is nice, but getting to know the ICU staff is not really a life goal that you want to strive for.
Addison had her adenoids removed on Friday April 28th. She did great the day of surgery and for a couple of days after. By Sunday night she was crying a lot and acting like she was in pain. She was difficult to get to sleep and she was only comfortable if she was being held. We figured it was just pain from the surgery and she just needed a few days to feel better and for all the inflammation to decrease. Monday through Wednesday were much the same – lots of crying/screaming and difficulty getting her to sleep or settle if she wasn’t being held. Thursday she slept literally all day and only really woke up in time to be fussy around bedtime. Friday I got her to take a short nap in her crib, but she woke up screaming. By the time Saturday rolled around, she was screaming and writhing in pain and was inconsolable. So we brought her to the ER. They found that she had an elevated white blood cell count, but they couldn’t figure out where the infection was – her urine was negative, neck CT was negative, chest x-ray was fine. They gave her morpine, which did nothing and versed, which worked long enough to keep her still for the CT. She had to be put on a little oxygen because all the crying was affecting her breathing and making her sats low. By the time they were finally ready to send us up to the floor, they had pretty much ignored her for a few hours, during which she had been screaming her head off because nothing I did made any difference. By the time we got up to the floor, her breathing was crap (she was retracting and working really hard), her sats were even lower and she needed more oxygen and she was still inconsolable and hysterical. They quickly called the ICU down to come evaluate her and we ended up in PICU within 30 minutes of our arrival on the regular floor. In PICU they could better medicate her and because the crying/screaming was affecting her breathing so much, she ended up on the vapotherm anyways. Basically, they have been sedating her to keep her comfortable while we try to figure out what is going on. She has as needed pain medication and IV drips of two different sedation medications. Thus far, we have not been able to figure out what is causing her pain.
To say that we are extremely frustrated does not even begin to cover it. It’s horrible to watch your child be in so much pain and to not be able to comfort her at all. It’s torture. We have done so many tests, but nothing has been the answer. It has been an incredibly frustrating, exhausting, emotional experience. I just want to find a reason why so we can fix it and get our calm, happy girl back.
We know a crap ton of things that this is not. It is not:
- Reflux, EGD was normal, plus she’s been on two reflux medications for over a month now
- Seizures, EEG was not changed
- Even though her hips are dislocated, ortho says they are not causing this level of discomfort
- Intestinal obstruction or twisting or constipation
- Kidney stones
- Any random fractures in her lower extremities
- Increased fluid/pressure in her brain
- An abscess or infection in her neck from her adenoidectomy
- Low vitamin B12 level
There are two main theories we are working with now. The first is keppra rage. Apparently keppra can make you angry/irritable. This is treated with vitamin B6, so they started that a couple days ago. To me, she acts more like she is in pain, but I guess we will try it for a few days and see what happens. There is no information out there on how long people are on keppra when this “rage” starts. I asked the doctor to look into it and she was unable to find anything. When I attempted to google it, all I could find were a bunch of forums of people who are/had been on keppra discussing it, but nothing scientific. I need scientific. The second working theory is that with the damage she had to the white matter of her brain (way back in her NICU days), she could have trouble processing sensory input – everything is just too much for her to pay attention to/sort through so she is basically overwhelmed by it. There is talk of doing an MRI to see if there have been any changes since her last one, which was a year and a half ago. However, that doesn’t really change the treatment plan, so we are holding off for now. Finally, the only part of her body that hasn’t been x-rayed or imaged at all is her arms, so I asked about getting x-rays of her upper extremities just to be sure there is nothing going on there. We have been in the PICU for a week and it feels like we are no closer to the answer. She is still on the vapotherm because when she gets worked up, her breathing becomes a problem; we have been able to wean her flow a little over the past couple of days and she has been on room air for the past couple of days as well. At this point, I just want to find the cause so we can figure out how to treat it and get back to our normal. It’s crazy when you start hoping something is going to come back abnormal. She’s been miserable for so long, I would love to see her happy, content, and smiling again. As always, thanks for all your thoughts, prayers, and kind words. If you have taken the time to text or message Ryan or I, know that it is greatly appreciated.
Here’s a quick summary of what has been going on with Addison over the past week. I am going to attempt to hit the highlights and not get too detailed. She was extubated last Saturday (the 18th). It was a rough first few hours off the ventilator, but she worked through it. She was on vapotherm until late Wednesday night, and has been on room air since early Thursday morning. They had to treat her for withdrawal from morphine and versed; she is no longer on a narcotic, but is still receiving Ativan. It is being weaned and she will be off it soon. They have also been weaning her off the medication for pulmonary hypertension, as our insurance would not approve it and her echocardiogram was normal. She probably had some component of pulmonary hypertension exacerbated by her viral illness, but it is not a problem that they think she needs medication for at this point.
They have been questioning from the beginning of her stay whether or not she is silently aspirating her tube feeds. The doctors felt like when they restarted her tube feeds, her respiratory status worsened (they also weaned her vapotherm a lot that day, so who knows for sure). They were pushing for us to convert her g-tube to a j-tube (which bypasses the stomach and goes straight into the intestines and means continuous feeds). We were not convinced that aspiration was the problem, considering she has been doing great with her feeds at home and doesn’t regularly vomit or get pneumonia. I put my foot down and told them they had to prove to me that aspiration was truly a problem before I would consider the j-tube, as it would make our lives that much more challenging and I’m not willing if it is not necessary. We finally got them to consult GI. It basically came down to this: whether she is having delayed gastric emptying and reflux/aspiration all the time or whether it is simply due to her illness, we cannot be sure. So we decided to try a medication that helps her stomach empty faster. That was started Friday afternoon and Saturday morning she was started on slow continuous feeds. She did well for a while, but then she vomited early Sunday morning. The GI doctor suggested that we try to increase the dose of her medication and go slower with continuous feeds Sunday as she wouldn’t be able to get converted to a j-tube until today (Monday) anyways. He thought we might as well try it while we were waiting – if it helped great, if not fine. She only lasted a few hours before she vomited everything back up, so she will get a j-tube placed today. GI feels that it will be temporary, just until she fully recovers from her illness and then we will be able to trial and hopefully resume g-tube feeds. Right now, the j-tube is the safest thing for her. It will prevent aspiration and allow her to get the nutrition that she needs.
Pulmonary also finally saw her and they think she has asthma, so they have started her on an inhaler to prevent asthma attacks, and she will have a rescue inhaler and possibly steroids at home for when she does get sick. We are also looking into getting some home equipment to help us manage her at home when she’s ill and maybe keep her out of the hospital a little bit more. Violet and Oriana have been able to see and talk to Addison on video calls, which has made them super happy. They still want her home of course. Hopefully we will be transferred out of the ICU today once she gets her j-tube since she will just need monitored for 24 hours to ensure she tolerates her feeds through it. It has been a very long two weeks for all of us, but there is a light at the end of the tunnel. Unless something (else) crazy happens, it is now only a matter of days until we can all be home together again. Once again, thank you for all the thoughts, prayers, and positive vibes. They make us feel so loved and we definitely needed that over the past couple of weeks.