Setteling in at home

We got Maddy out of the hospital and home on Tuesday. Been trying to get everything settled in and switched over to Hospice care. There are a lot of little steps involved but it's moving along. It's very hard to think and talk about Hospice care but it has proved to be a fairly positive experience so far. With the exception of a few problems getting Maddy's pain medications to the house on time things are going well so far. I appreciate that the attention has not switch to focus on quality of life instead of simply prolonging life at the expense of happiness.

So the goal now, and I'm sure Ive said it before, is to make Maddy as happy and comfortable for as long as she has left. We are still working with the Transplant doctors to irradiate as much of the antibodies that are attacking Maddy's lungs as we can. This means that we are still giving her the oral anti-rejection meds Prograf, Cellcept, and a low daily dose of the steroid Prednisone. She is also scheduled for monthly IVIG which will hopefully bind up some more of these antibodies and give her a higher quality of life. She is no longer going to accept any treatment that will not make her life better. No point suffering if there is no cure.

Her pain level is still somewhat unpredictable. The long acting morphine is doing it's job for the most part. She gets it around the clock and it keeps the pain in her lungs to a dull 3 or 4 throughout most of the day. Every now and then she has an acute rise in pain that requires her to take a Norco. Yesterday she told me the pain is no longer a stabbing pain but a throbbing ache when it increases. I'm not sure if this is a good or bad sign but I would take throbbing over stabbing most days. The pain is now present in both lungs but her right side still has the majority of it.

Last night she had a pretty bad coughing fit. when she coughed it sounded like a big ball bearing rattling around inside her chest. We are use to hearing stuff rattle around in there from her CF mucus moving days but this was unlike anything we have ever heard. Turns out it was a pretty big mucus plug that she was able to cough up and get out in two pieces. I know this is really gross to read about but my CF community will know that this is something to celebrate. There was no blood in it so we know her lungs are not breaking down at the moment. At least not in a major way. Hopefully this will continue and she will be able to open up some more of her blocked airways so she can breathe easier.

Maddy is still very weak but has been able to be awake all day, every day, since we got home. She still can't make herself food or walk around the house too much but she spends the day in the living room and only spends the night in bed. That is a huge leap forward from last week in the hospital. She is also eating more and more every day. I'm hoping she will be strong enough to start venturing out again soon. We have her wheel chair and just got a new portable oxygen concentrator which will allow us to get out for longer periods of time. I am working on a hack to mount the thing to her wheel chair because it's a bit bigger than we expected it to be. Ha Ha.

Because she is on such heavy pain meds and is still so fragile we can't leave her alone at all. She is both bummed and comforted by this but we are all making the best of it. I'm working out a schedule with family so that I can get to work on a more regular basis and hopefully I will be able to take on my air shift again starting next week. The good thing is that the radio station is less than 5 miles away so I can be back to the house at the drop of a hat.

Not sure what else I can really share here any more. We are working at managing Maddy's pain and halting the progress of this rejection so that we can maximize her quality of life for as many days, weeks, or months she has left. She is doing well and getting stronger but she is still in a very precarious state. Any little setback could be the last setback so we are being very careful and trying to make sure she has as many good days as possible. I will not take for granted one single moment I have to be with my lovely daughter!

Thanks again for all the prayers. Don't expect many updates. If there is a crisis I will try to post something to keep everyone in the know. But otherwise we will take some time away from social media and the web to be here with and for each other as a family.

Lots of sleeping, lots of pain

It's been a long weekend in the hospital trying to manage Maddy's pain. It always seems to be hovering around an 8 or 9 anytime she is awake. As you may remember on Friday Dr. Tuch from the palliative care (PC) team upped Maddy's does and time schedule for the Norco and started her on a long acting morphine at night. She got her first morphine dose Friday night. The first full day on the new schedule (Saturday) Maddy needed Norco every 4 hours without fail. By Saturday night Dr. Tuch had seen that need and chencpged her to morphine twice a day, every 12 hours. So Sunday morning she got a daytime dose of morphine but her pain levels didn't seem to be getting any more tolerable. By mid morning she had to ask the nurse if she could get any other pain medication as well so they gave her Norco on top of the morphine. She also spent the morning and much of the mid day nauseous. Which is terrible because she not eating as it is.
By 2:00 we were able to get the pain under control and her moving enough to get a yogurt smoothie down her. Then it was right back to napping. She got up on her own around 5 and had the tech change her sheets. I was able to get her into my chair for a while during that time. (See above picture) Maddy even said she was hungry while she was sitting up! I ran to Panera to get her a bread bowl with French onion soup. She was able to eat a little more than half of it.
I keep thinking I will open this blog to post an entry and be able to write "Maddy is snapping back and feeling better" but that continues to no longer be the case. It's Monday morning and Dr. Haddad came by about 7:30. When he asked Maddy about her pain she said it was at a 4 or 5, which is good considering all she has had is he morphine last night at 9PM. He told us the goal was to transition her home sometime this week and that Dr. Tuck and the palliative care team would be handling the transition. That's all I have right now. I will wait to post this after I see Dr. Tuch. Hopefully sometime this morning...
It's almost 11 and the PC team has visited. Dr. Tuch said his team will be working with Hospice today in the hopes of getting Maddy home tomorrow. He said he can change the dosage of the long acting morphine (finally found out it's called MS cotton) to 3X a day to avoiding the pain crisis she has been using the Norco for. I told him I thought she was too sleepy all the time and maybe that would be too much. But I didn't want to step on Maddy's toes or keep her from getting the meds she needs. So I asked if he can order it for every 8 hours but if she doesn't need it that often can she just take it every 12 still. He said that sounded like a good compromise. So when it's due at 4:00 today she can decide if she needs it. There is still a concern that hospice will not cover her prograf and cellcept because they are anti-rejection meds. He asked is we had a bit of a stockpile at home and Maddy is sure she does because of all the time she has spent in the hospital lately. He said that will be our work around if hospice doesn't want to cover it.
Maddy was able to sit up and chat with everyone who came in this morning. But still has not been able to eat anything. She said she is afraid it will make her sick if she eats. She also got up and let them change her sheets again but went right back to bed after. She is napping now. I will post another update again tonight or tomorrow when we have answers about hospice and plans to go home.

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Pain Management

After talking with Dr. Tuch yesterday they got Maddy on a schedule of pain management that is getting more tolerable. Having the Norco available every 4 hours instead of 6 is helping. She still can't stay awake for more than a few hours at a time. Getting nourishment into her is also still difficult but she is trying very hard to eat every time she gets up.

Melissa came and hung out yesterday and was here when the lady from Hopsice came around. That was a relief because it was one less time I had to relay the information. The hospice manager gave us all the information and we are thinking about it over the weekend. The trickiest part about it is that they are not designed to provide life extending medications, such as Maddy's anti-rejection meds. She said everything is on a case by case basis but that eventually we would probably have to stop those if we were in the hospice program. There are a handful of other things we have to think about that I really just don't want to go into in this blog. Suffice to say we are taking the weekend to see how Maddy is and make our spdecision on which direction we go in next.

On a brighter note, Ethan and Alex paid Maddy a visit late in the afternoon and into the evening. Maddy was sleeping for much of the time they were here but she got up and chatted with then for about 2 hours before they left. She was in good spirits for much of the visit and didn't start having unbearable pain until toward the end. It was nice to see them making each other laugh for a while. Ethan and Alex promised to come back today and bring Maddy a boba tea. She is looking forward to that.

Melissa came back a little after 8 last night to spend the night so I could go home and start getting things ready for Maddy to come home. She reported that Maddy was able to get a good night sleep on the new long acting pain medication Dr. Tuch ordered. Not much luck getting her to eat but that seems par for the course these days. Melissa hung around until a little after 1:00 today so I could run to Ikea and get Maddy a bed. When we moved Maddy chose to go from a futon to a mattress and it was easy for her to just use it on the floor without a frame. If we are going to have nursing help I want her to have a bed up off the floor. So I found a cute little frame at IKEA and hopefully I will be able to get away for a bit tomorrow to put it together.

That's about it. Things are pretty slow at the moment. I'm a bit worried about Maddy not having any energy but I guess that's to be expected. I was really hoping these steroids and antibiotics whould give her a second wind so she could get back on her feet again but so far it's not looking good. Also her tremors are getting worse. I don't know if it's from the steroids or what. Usually she gets them from her anti-rejection meds (prograf to be precise) but they haven't been giving them to her because levels have been too high. I was hoping to ask the doctor about it today but Melissa said he didn't come by, unless she missed him somehow. Guess i'll try again tomorrow.

I guess that's all. As always thanks for the thoughts & prayers.

 

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Palliative Care

I will try to get as much of this right as I can. At this point maybe blogging what we are going through will help someone else who will have to go through this terrible situation in the future.

The Palliative care doctor came around a few minutes ago. He said he spoke to Dr. Haddad and the transplant doctors who said there is nothing more they can do for Maddy at this point. His name is Dr. Tuch and he said his job is to make sure Maddy's pain is managed and help coordinate her decisions from her on out. He asked her to explain about her current pain and tell him what the transplant doctors have told her about her prognosis. He also asked a lot of questions about our home situation and how she thinks she will be able to manage in the comfort of her own home. The goal is to get her out of the hospital if she would rather be at home but he said if she wants to be here thats ok too. She prefers home so the next part of the discussion was how much help she has at home. I voiced my concerns about leaving her alone while I worked if she was on high doses of pain meds. Maddy agreed that she is getting weaker and may need  little more help when I'm not around.

Most of the conversation revolved around Maddy's desire to continue fighting. She wants to continue treatment in the hopes of getting the antibodies under control and the rejection to stop. Dr. Tuch said that she can keep fighting but he wants to make sure she isn't going though a lot of unnecessary pain in the process. He also said it's probably time to consider the possibility that her condition will not improve and that we need a plan to help her be as happy as she can be for as long as she has. He specifically asked her what she wants to do with the time she has left. She said just not be in pain.

After the longest and hardest conversation in my life we cane to a few conclusions. Dr. Tuch is working with the transplant team to adjust her pain medications. He took her off the Ultram because it wasn't helping. He changed her Norco from every 6 hours to every 4 hours as needed. He will also put her on a long acting form of morphine at night so she can get through a whole night of sleep without any pain waking her up. He hopes this will allow her to manage the pain so that she can be up and somewhat active during the day and get a good night rest. He is also calling the Hospice Care team to see what can be done to get Maddy home and get her cared for while she is on these higher dose pain medications. He mentioned that Hospice doesn't usually continue any of the anti-rejection treatments that she is currently on. But he understands that it is important for her to keep fighting so he will work with them to make sure she will still get her anti-rejection meds and if the transplant docs think the Antibiotics and steroids will help then we can continue those as well. He promised to come back later this afternoon to let us know whats up with Hospice.

Maddy was up eating a yogurt smoothie for breakfast when Dr. Tuch came in and we could see that her pain was increasing as the conversation progressed. At the end of our discussion he went right to the nurse to make the medication changes and brought her in to give Maddy her Norco. Shortly after that Maddy asked me if she could put her mask back on and take a nap so she is sleeping again. While the doctor was out Maddy and I had a bit of a conversation about everything thats going on. She was upset that I was upset by all of this. I assured her that I am only upset because she is hurting and that I do not want to see her hurt. I also let her know that I did't think she was giving up and that she shouldn't worry about me. I don't want her to hold on if it means she will be miserable. Especially if none of this pain she is enduring is going to help get her better. So we are going to do our best to make sure she is pain free and can enjoy as much of the time she has left.

Very Little Movement

Things are looking pretty rotten from this chair I sit in. Yesterday Maddy just slept. She got up and ate a few times throughout the day but other than that unless they came in to give her meds or do something to her she just slept. The pain in her right lung is persistent and the only thing that helps is her pain meds every 6 hours. They are giving her steroids and antibiotics quite often at this point. But it does not look as if she is responding positively to that treatment. Last night the IV in her right arm blew while the steroid was being infused. She woke up at 1AM with her forearm swollen and in pain. The nurse took it out and called in a specialty team to place a new IV. It took two different nurses trying but they finally got it placed and the remainder of the steroid going by 3AM.

I was hoping her sleeping all day yesterday would be restive and give her the strength to get up and moving today. So far no such luck. It's hard to tell if she is just tired because she was up and down most of the night or because she is just getting too weak to go on anymore. This morning we have had a rough time getting her to sit up and take her meds. Usually even when she is tired she can sit right up and talk to someone or take her meds. Not this morning!

We are still waiting on the Palliative care team to come talk to us. I spoke to Dr. Haddad about it this morning and he said he would make sure they came by. I hope Maddy can wake up and communicate enough to make her wishes known to the team. I know what she wants to do in dire circumstances but only she can tell us if this lingering fight is too much. No major organs are failing, except her lungs, so it's just a slow process at this point. I think I've said it already but it's really hard to tell if she is just tired and will bounce back from this episode or of this is it.

I may not even post this but as I sit here in the quiet next to her bed I have to do something to get these thoughts out of my head. I know there are a lot of prayers and well wishes going on out there for us. Honestly the only thing that will help at this point is a miracle. Even if she bounces back from this episode I don't know how much longer that will give her. And if she does make it through this I don't know what kind of quality of life she will have. There is so much uncertainty at this point.

I guess I will post this and try to write again when I know more.

Another Day

Yesterday was a pretty busy day. It took quite a while to get a bed for Maddy at the hospital but that didn't stop the doctor from moving along with her treatment. They took Maddy down for a CT by noonish. I hung back in the clinic because it usually doesn't take that long, but you know what happens when I think something will go smoothly. Maddy texted me that she was still waiting almost 30 min after they took her back. Then when she finally didi get in for the scan they wanted to use contrast and her IV wasn't big enough. So they had to call vascular access to put in a bigger IV. I asked if she wanted me to come find her and she said there was no point. Melissa got to the clinic shortly before Maddy was finished at CT. I took that opportunity to go to work for a bit and then head home to pack Maddy's bag.

The doctor also went ahead and started her IV antibiotics and steroids while she was waiting in clinic for her room. The room was finally ready around 4 but no sooner did she get settled in than they pulled her out for another test. Melissa learned from my mistake and accompanied Maddy while they took her to get an ultrasound of her legs. The docs wanted to make sure she did't have any blood clots that could travel to her lungs and make things even worse. Maddy was not happy with that exam at all. Apparently they have to push really hard in a number of places and she does't like having her legs toughed in the first place. It made her very anxious.

By the time I got back to the hospital Maddy was very worn out. The new pain meds Dr. Floreth put her on keeps the pain in her right lung at bay for the most part. They have her on full telemetry which is always a pain in the butt. Millions of little stickers and wires everywhere makes it kind of hard to get comfortable. But she is making the best of it. She went to bed as early as she could last night, around 10:45 even though they come in at 11 to do her vitals one last time before bed. She was able to sleep most of the night. Her pain meds wore off around 4AM and she woke up and cried out in pain. I helped her get out of her mask and get her O2 back on so she could sit up and wait for the nurse to get her pain meds. That was of course just in time for a bunch of people to come in and start bothering her. They do vitals and a blood draw at 4AM. I think she finally got her meds and back into her mask by 4:30 to sleep again.

Dr. Haddad came in at 7AM and did't even wake her up. Just gave her a listen and said to let her sleep. I asked him how the CT looked and he said "a lot of damage and inflammation from the rejection". He never seems very hopeful anyway but this seemed even less hopeful than usual. We still haven't heard from anyone about hospice care. I don't know if they wait to send them in just before we are ready to go home or if someone will come by today to talk to us about it.

Maddy got up for a little while around 9 to do her breathing treatments. I was able to get her to drink a yogurt smoothie while she was up. The nurse also got her morning meds done and hung another IV antibiotic. It seems they are running every 6 hours on one of them and every 8 hours on the other. It's about 10 and Maddy is napping again. She didn't want to put the mask on so I told her I would watch her breathing and let her rest without it for an hour or so. As long as she doesn't sleep too long without it there is not worry about the CO2 building up too much. I'm guessing she will be asleep most of the day soI will make her put it back on again before too long.

As for her spirits, Maddy is just very tired. She is hoping this will help get her back on her feet again and give her another chance to live for a little while at least. She hasn't given up and told the doctors to bug off just yet so thats a good sign. Last night she was complaining that the creepy crawly feeling from the steroids was kicking in. She hoped the pain meds they have her on would help her get past that and be able to rest and so far that seems to be the case. There is nothing worse than the feeling steroids gives us! I have experienced it on small doses so I can't imagine what Maddy puts up with when she is on these high doses. At least this time around she is able to rest.

I think thats all for now. I'm just going to stay close and be available for what ever she needs. I'm so thankful that we live so close to the hospital now! and for all my awesome co-workers and boss who cover things for me when this craziness goes down. And of course I am very thankful for all the prayers and good vibes coming from everyone near and far. Maddy is still fighting so we all should too!

Chest pain

Shortly after I posted yesterday's update I got a text from Maddy saying he right side of her chest hurt really bad. So bad she felt she may have had part of her lung collapse. I put in a call to the doctor and rushed home. She was able to breathe but couldn't take a deep breath. She was still coughing s lit and every time she coughed she would breathe in deep and said it felt like someone was stabbing her. We checked her O2 saturation and that was at 99%. It took quite a while for the doctor to call back. He said if she is saturating ok then try taking some pain meds and come see him tomorrow morning. If the pain is too much to take then go to the ER. The doc called in a pein medication for her to CVS and she soldered through the pain for the rest of the day.

Maddy said the pain has been happening a little since she started using the machine. Usually she would wake up with a sharp pain in one side or the other but it would go away after a short while. Yesterday she got up around 10 with the pain and it just never went away. She texted me by 11:15 but it took until 1:30 to get through to the doctor then 3:00 before we actually got any pain meds into her.

Up and to the doctor this morning by 7:15. They just did a pulmonary function test, which was almost impossible for Maddy to do because she can't take a deep breath. She got an indreadable 11% of predicted! No surprise there. I'm hoping it's just that low because she can't take a really deep breath. Waiting on the doctor to come in and see her now then decide what kind of imaging to do. The nurse mentioned they may have to do a scan for blood clots. I hadn't even thought about the possibility of a pulmonary embolism.

Dr. Floreth came in and said that they were going to get a chest X-ray. He asked if the pain meds were helping and she said not really. He told us that his goal was to make sure she had the pain management that she needed. He mentioned that where her lung functions are they may have to call in Hospice to help her manage the pain and have someone to care for her at home. They gave her two Norco before sending her up for the X-ray. After eye X-ray I checked with her and she said the pain is a little better.

Doc just came in and said the X-ray was abnormal. No collapsed lung but there is a lot of information in her lower left lung. Either infection or rejection. He wants to put her in the hospital and get a CT scan to see if he can get a better look at what going on. It's too dangerous to do a bronchoscopy and take biopsies at this point so we are going to have to try and treat this from scans as best we can. He wants to start her on some steroids and antibiotics and hopefully she can cough up a good sample so they can check for infection.

He also told her that she has the right at any time now to say "I'm done with hospitals" and he will send her home with hospice. He thinks they can knock this out a bit and get he back on her feet. But he will call in hospice to come talk to us while she's in and start working on a plan in case they can't get her better.

I'll update again tonight or tomorrow after things have settled in a bit or when we know more.

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One Week Out

I'm having trouble coming up with a good title for this blog entry. I don't usually update when Maddy is not in the hospital but thought I should this time. I wouldn't say things are going exactly smoothly at home but it's still better than being in the hospital. Many people are asking me how Maddy is doing and thats a really hard question to answer. Most times I say "taking it one day at a time", but that doesn't really give anyone a picture of her health. So here is the rundown one week out.

The A-Vap machine, or what ever of the three names people call it at any give time, is working well for the most part. We had a few bobbles the first few days but have most of those kinks worked out. Maddy still panics a little every time she has to put it on. She says it makes her feel like she is suffocating, and of course that it her worst nightmare. It also ends up blowing air into her stomach throughout the night. She wakes up with a painfully bloated abdomen. Usually this would be no big deal but they made her get a procedure done shortly after her transplant to keep her from having acid reflux into her lungs. Here is the blog entry from that day.  Basically they put a zip tie around the top of her stomach to keep the acid from regurgitating into her esophagus and then down into her lungs. Problem is that zip tie keeps everything down there. Maddy physically can not burp! So each morning to get the air out of her stomach she has to make herself gag and dry heave the air out of her stomach. Sorry to be so graphic but this is how she has to live now.

Speaking of her stomach, that is the next issue she is having. Not sure if it's all the air being pumped in there each night or just that fact that she hasn't been able to eat right since mid January but she is not doing well on the nourishment front at all. One of the big things the Co2 poisoning did to her is eat away at a bunch of her muscle and what little fat stores she had remaining. She was already very underweight because of all the infections and this latest episode has done her in. We are having trouble finding food she can tolerate. Everything seems to hurt her stomach. I'm lucky to get 600 calories into her on any given day. On top of that she just has no appetite and when she does try to eat, something always seems to go wrong. Take last night as an example...

Her cough has increased quite a bit lately. Yesterday it was a dry cough, seemed like she just had a tickle in her through that would not go away. I'm not talking just a few hours either, she was coughing non stop... every waking moment... of the entire day! These dry coughing fits always make her gag as well, so all day her stomach was heaving as if she were puking but she wasn't. She was barely able to choke down a shake at lunch. By the time I severed her dinner she couldn't stop coughing long enough to get in one bite. She literally sat there with the plate on her lap for two hours trying to eat. She finally gave up and asked for something lighter and in the next 45 minutes of trying was only able to get down two bites.

I don't want to be pessimestic but it's getting harder and harder to see some kind of silver lining. But even through all of these setbacks Maddy seems willing to continue the fight for every breath. I'm sure there is much more I could write about here but I'm having trouble getting it all down at the moment. As always thanks for the prayers and please continue.

Lets go home!

 

Maddy's new ventilator came yesterday so we get to go home today! I didn't want to post anything until we talked to the doc this morning just to be safe. He just came in and gave us the all clear so now we just have to wait for paperwork. In the pic above you can see Maddy's new machine on the left compared to the hospital one on the right. Much more portable and easy to deal with. Obviously! The new mask is much better than the generic hospital one as well. It fits much better, has a smaller hose and a swivel connector so she can move easily. I'm sure she won't have any trouble tolerating this at home.

So that's the plan. Use the ventilator at night so she doesn't get Co2 build up again. IVIG every month to get those suckers down. Maybe photopheresis in the future and also maybe thymoglobulin again. Dr. Floreth said they don't like to do the thymo more than once a year and it's just now been a year since she had it. So they will watch her levels and if they are not going down start using the more hard hitting treatments.

Thanks again for all the prayers and good vibes.

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