Shoshana Is Four

Life continues to happen around here.  Shoshana's original birthday party had to be postponed because she came down with a case of shingles and spent a week (including her birthday) in the hospital.

The "maintenance" phase of Shoshana's chemo has been otherwise blessedly uneventful.  We are now working with a new home care nursing agency, and they more able to fill the shifts, although they are still spinning up the day shift, and we have had a few nights without a nurse.

We held Shoshana's party this weekend.  Elizabeth wrote a post about the party on the farm blog.

Meanwhile, the girls got to be in a friend's wedding, and their new baby cousin was born!

The night nurse decorated Shoshana's room while she slept.

Mint!

This was the rehearsal.  I was too busy to take pictures during the wedding.

There's that tongue...

...and then this happened.

Cousin Kora

The Trach Is Staying In For Now

I (Tim) haven't had a chance to post an update to this blog in a while, and I don't have a whole lot of time right now, so this will be relatively short and possibly scant on some of the details.

Vincristine

After Shoshana had received her (reduced) doses of vincristine for the delayed intensification phase, we met with the doctors and went over the possible options for vincristine in the future.  The conclusion which we all reached (family and doctors alike) is that Shoshana won't be getting any more vincristine!

• Shoshana's neuropathy from vincristine is so severe that, if she were to take it, even at a reduced dose, it would likely delay and derail her normal course of treatment.
• The European treatment protocols which are similar to the one she is on have eliminated vincristine from this stage of the treatment onward.  The U.S. protocols are in the process of evaluating whether they can do the same thing.

Basically, vincristine is simply being dropped from her treatment schedule, since for her it is doing more harm than good, and the amount of good it could do at this stage for the entire patient population is being re-evaluated.

Praise the Lord!

Recently

Shoshana hasn't been overnight in the hospital in quite a long time, but in the month of May we had 17 clinic visits (which means there were more days that she visited the hospital than she didn't).  It's been great having her home, not only for her sake (and she does so much better at home!), but for Elizabeth and me, and also Abigail.  (Not to mention Kristen, Elizabeth's mom, and the Predmore family, who have been an invaluable help.)  Having her home, we are much less stretched.

Even so, for the past month or so we have been struggling because the two nursing agencies have not collectively been able to provide us with daytime nursing care.  We are switching to another agency (who is hiring away our night-time nursing staff), so hopefully we will get a bit of relief in that regard.

Today

This afternoon, Shoshana went in and was put under anesthesia in order for the ENT doctor to examine her vocal cords and trachea.  The plan was that if everything looked OK, he would observe her overnight with a smaller trach tube, which would be blocked off, so that they could evaluate her breathing overnight, and if everything looked good, the trach would come out.

Shoshana's vocal cords looked like they were functioning normally (Yay!), but unfortunately there is some scar tissue in the tissue surrounding the trachea, which is causing walls of the air passageway to constrict.  (Think of a rubber band or a balloon with a glob of harder rubber that distorts how the rest of it will stretch and move.)  There was some improvement from the last time, but not enough for the trach to come out now, considering the fact that she's still undergoing treatment for cancer.

So, Shoshana's trach will remain in place for at least the next few months, at which point, they will take another look and re-evaluate.  At some point, she will need to have surgery to deal with the scar tissue.  (I wasn't there and it hasn't been explained to me, so the details on that will need to come from Liz--bug her for an update.)

Items for prayer:

• Praise that Shoshana has been doing so well, and has been able to spend so much time at home with her family!
• Ask that Shoshana's scar tissue will be resolved soon, and her breathing pathways restored to normal
• For our home care nursing situation, that we would once again have daytime nursing, as well as nights.
• Thankfulness, for those who have been supporting us, and even occasionally spending "normal" time with us.

That's all I have for now.  Here are some random pictures, since it's been a while:

Pictures

Pain Management and Elevator Music

Shoshana recently started a stage of chemo called Delayed Intensification. In some ways, this stage is considered to be the most difficult, the most intense. Not only does she have four chemo drugs that she has seen before, including frequent vincristine, she gets a new one called cytarabine which causes a lot of nausea. After the first day (Friday) in which she got three chemos, including vincristine, her pain once again got out of control. We brought her back to the hospital on Monday morning because we couldn't give her any more pain medicine despite her obvious discomfort. And the pain medicine we have been using for a while wasn't working as well as it used to.

I told the team that the plan had to change or she wasn't getting any more vincristine. Unfortunately, that would mean a much higher chance of relapse, but she would not be the first case that simply could not tolerate the side effects of vincristine. After many conferences with the chemo team and the pain team we decided there was another option: we increased her gabapentin sharply for three days around the second weekly dose of vincristine (and other drugs, but the big problem is vincristine).

And, as we all know, she doesn't tolerate medications like most people...

We realized she was "off" when she didn't sleep most of Saturday night. Thinking back she had begun shivering the night before complaining of being cold. The on-call fellow said to bring her in to the ER for possible gabapentin toxicity. Once she got there, however, they decided it might be sepsis instead. Her temperatures had been normal, labs were normal, blood cultures wouldn't come back for a few more days. Since there was very little to do at that point other than go back to the lower dosage of gabapentin they sent her home. She was pretty hyper all that day and didn't sleep from 10am to 10pm until I gave her a small dose of melatonin (per Dr.'s orders). She has been doing relatively well since then; the insomnia has resolved and the hyperactivity has made way for a more "normal," silly, super-active Shoshana. Which generally means she is feeling good.

Oh, and that ER visit was the first time I was at work while anything new was going on with Shoshana. I was on my phone a lot. But I somehow still managed to see a lot of patients.

Other than the gabapentin toxicity this last weekend went rather well. Her pain was generally a secondary issue to whatever else was going on. We are hopeful that with a tweak of the gabapentin dosage she will have decent pain control this weekend without going all hyper and insomnolent for a few days. By the way, the typical reaction to too much gabapentin is sleepiness. The same goes for Benadryl but she reacts to that with hyperactivity too.

****
Operation: Harass the Insurance Company

On a related note, we have been working with a second home health agency to take our case since the first is a rather small company which is having difficulty filling our needs. I found out on Thursday that the second agency's petition to be an exception to the "in network" rule was denied.

Now... why is the claim denied? Because someone didn't do their job.

I was on the phone with Blue Cross Blue Shield of Illinois off and on Thursday and Friday trying to get things figured out. Bouncing around departments, many minutes on hold, lots of phone tag and I thought I understood the problem. They somehow got the idea that there were 40 other home health agencies to choose from in my network. I heard that and nearly choked. I doubt there is a 25 mile radius in the United States, let alone the world, that has that many home health agencies, let alone that will take pediatrics, let alone Shoshana's complicated case. Not only that, the request was filed several weeks ago, at least one week before our main day shift nurse put in her three weeks notice. By this time she was doing her last shift and most of the next week was not covered due to her absence. How did it take that long to be denied in the first place?

I tried in vain to motivate the insurance people to make this a top priority because when I don't have a nurse I have to use an ambulance for transport and Shoshana will be admitted quicker and discharged slower without a home health nurse.  This is expensive, people! How is getting a nurse on the case not a priority!

They do nothing.

Monday rolls around and I'm on the phone from 9am to 4pm either on hold, talking to someone, bouncing around departments or waiting no more than an hour for a call back. Oh, and in the middle of this saga our case manager nurse decides to stop answering my calls or responding to my voicemails. Thanks for nothing Marie.

By the time the business day is coming to a close Marie's supervisor FINALLY understands what I'm asking of her (she got hung up on a separate issue) and says I need to talk to a totally different department but now it's too late. And I snapped. What I'm trying is not working and now you're putting my daughter at risk! I had gotten the impression earlier that day that they were not interested in doing their job so I had to do their job to find out the truth about why the "out of network" claim was denied. I asked her to write this down and make sure it is forwarded to the complaints department. I also gave myself permission to use some colorful language if I thought it would help to get my point across.

Did you know that there are 44 other home health agencies in my area? I didn't. Neither did my agencies. Because they don't exist. Because someone at your company just punched numbers into their search engine, saw there were 44 hits, and denied the claim. Never mind the fact that some of the hits aren't even home health agencies. Never mind that there were many entries for the same company (four for Gentiva, seven for Multicare, for example). Never mind that THEY DON'T TAKE PEDIATRIC PATIENTS! Out of 44 hits I made 22 phone calls. Some of them only did visiting nurse services such as infusions or wound care, one of them no longer exists, several of the phone numbers on the search engine were wrong so I had to find the numbers myself, and almost all of them don't take peds. Not only that, none of them could suggest an agency that does take peds! My search brought up two relevant hits. One agency is in Bremerton. As in, across the Puget Sound. You need a ferry. To make a two hour commute. I know this because I almost went there a few times for work. The second one is in West Seattle. But do we really have to start the process from scratch when we've already made so much progress with Alliance? They have a nurse waiting to take our case! In the meantime you are putting my daughter at risk for serious complications and you are putting your own pocketbooks at risk! How are you not motivated to fix this? Why am I doing your job?

I unload my chest and took a breath.

I thank her for taking that down then tell her that I expect a call from that relevant department first thing in the morning. That means 6:30am my time. I don't care. This has to be fixed!

They don't call.

Once the heckticness of the morning dies down I call the supervisor again. Miracle of miracles, the out of network request has been reopened and bundled with the review of "medical necessity" already scheduled with the medical director. So if the medical director decides that Shoshana does still need regular home nursing then they will automatically approve Alliance to do some of that work. Hallelujah! It's totally going to be approved, by the way. I'm sure we're going to have several of these medical necessity reviews simply because Shoshana's is a complicated, expensive case. Just for reference: by the time Shoshana came home in December from her three month inpatient saga her care cost just under $1 million.

Let's summarize: Blue Cross Blue Shield of Illinois worked on a case for four weeks, came to a half-assed assessment of the situation, and didn't follow up with anyone. I called constantly for three days before finally getting to the right people, asking exactly the right questions, listening to them repeat themselves over and over again, redirecting their thought processes, and pushing our issue through to the medical director. Oh, and listening to their poor excuse for on-hold music.

BCBS: 0
Me: 298578384846390240

After all of this I feel like I've been in a three day wrestling match and I have the worst elevator music stuck in my head. It was lame, cliche, pathetic, without pride. It looped every three minutes with three unrelated fragments. This could be used as a torture device. It nearly made me hang up from the sheer pain of it.

Enough of that. Let's listen to little girls laughing. Abi had been carrying around Shoshana's "stomp shoes" most of the day and was getting very angry when it wouldn't stay on her foot. So mom put them on and the girls are super cute!... and then Shoshana throws up. This stuff happens really fast. But let's just laugh at the girls for now.

The Grind

Since our last blog post Shoshana was home for seven days before once again having nausea, vomiting, diarrhea, and overall dehydration.

The first day was, again, gut rest then instead of going straight to formula they proposed starting with pedialyte instead. A quick google search confirmed my suspicions that the formulation of pedialyte had ingredients that made me uncomfortable. Namely, the dyes and "natural and artificial flavors," ingredients and labeling ambiguity that are both banned in the UK. It took a bit of back and forth discussion (read: standing my ground) to get the staff to agree to a formulation that included some sort of food such as juice, water, salt, and minerals. Why does that have to be so difficult? All over the world rehydration does a good job in the majority of cases without pedialyte. Where I was in Mexico the prescription for gut issues always included limonada since limes were abundantly available. I managed to convince the team to allow a juice formulation once I said that if that doesn't work I'm more than willing to try dye-free pedialyte. But before we knee-jerk to the industrial formulation which only includes the base chemicals, vitamins, and minerals and "natural and artificial flavors" can we try some food? I understand there are cases in which the body needs a temporary rest from processing foods but still needs base nutrition but I was not convinced this was the time for Shoshana.

The gut rest and slow rehydration had an unexpected side effect: Shoshana ate! Like, constantly! Not big amounts but she was almost always putting something in her mouth. This did not happen last time. She even ate a few things that she hadn't touched since before her diagnosis such as oranges, PB&J, and granola.

As we continued to brainstorm what could be causing Shoshana's latest gut issues Dr. Labriola, our pediatric oncology naturopath, suggested a few things and the hospital nutritionist concurred: we changed the hemp milk to rice milk and we purposed to feed her with rest breaks and a few spoonfuls of yogurt at the beginning of each tube feeding to support the digestive flora. This seemed to help since she continued to want to eat even as we worked our way up to the goal calories per day. Within a few days she improved greatly and we made it home on Friday afternoon. Other than an extra dose of pain meds and going to bed early she seemed normal.

Tim and I got to sleep in the same bed at the same time for the first time in a week... for one hour. The night nurse knocked and informed us that Shoshana was very uncomfortable all evening and has been mounting a fever over the last 30 minutes and the latest reading was 38.5C/101.3F. In all the time Shoshana has been neutropenic this is only the first or maybe second time since she has gone over 101F. She runs low 97's like I do and a change of two degrees is enough to make anyone feel crummy. I asked Tim to take her to the ER because I was so wiped out and sleep deprived and the weekend is usually his shift anyway.

By the way, that sets a new record! Discharged at 2pm, re-admitted 12 hours later. That is not the shortest time between trips to the hospital but it is the shortest time between admits. At least she got to have a bath with little sister which they both thoroughly enjoyed.

Now she tests positive for rotavirus which is known for viral respiratory sorts of infections. She is on two broad-spectrum antibiotics which put her at high risk for another round of C-diff. Once the microbiology report comes back tomorrow they will be able to change those to more specific drugs. She had a lot of pain overnight and more secretions which make the doctors think of a general tracheitis but at this point it doesn't make much difference whether it is a mid-airway infection or lower airway infection. Over the day she has improved, complaining of less pain, less secretions, and generally feeling "better" though sometimes she would say "bad".

It is possible she could be discharged late tomorrow afternoon but it depends on the microbiology report. It usually takes 48 hours but sometimes more, sometimes less. It depends on her general symptoms too.

We have an appointment with the clinic on Tuesday to evaluate her readiness for the next stage of chemo called Delayed Intensification with a planned start on Friday. That means adding a nasty drug called doxyrubicin, restarting steroids (yay...), methotrexate, PEG-asperaginase, and vincristine WEEKLY for FOUR WEEKS. Am I anxious about this? Guess. Please be praying for her handling of those drugs. She has gotten at least one dose of each of those in the past but in some ways this stage is even harder to handle than the first stage of treatment. A lot of hydration fluids are required on multiple days to protect the kidneys, lots of labs to monitor, white blood cells hovering around zero, and possible red blood cell or platelet transfusions. Not to mention the pain and nerve damage from the vincristine. This will be the second time she is scheduled for so much vincristine in so little time and we know how that ended. Obviously, she has a trach tube in place so, theoretically, her airway is "stable" but there has literally never been a case like Shoshana related to vincristine.

In my research I've learned that there are only six other known cases of survived vincristine toxicity, one of which was a girl. Only one of them (a boy) needed a trach for a short time after extubation. None of them had the pain and nerve side effects Shoshana is experiencing. One of the reassuring things about leukemia treatment is that because it is so common and has been around for so long we have a lot of data to help us decide how to treat her. Unfortunately, all that data only goes so far.

Please pray for us. For sleep. For peace. For pain relief. For protection from drug side effects.

Thank you.

Playing with medical supplies

Eating more!

Making faces :)

C-Diff, Methotrexate, and Pneumonia

The title pretty much sums up the last two weeks here. Two weeks ago Shoshana started vomiting not very often, but a large amount each time. After a day and a half of this I knew she was getting dehydrated so we brought her in to the clinic for fluid bolus and evaluation. After the first fluid bolus, still no urine. So they ordered a second bolus. Well after the second fluid bolus she finally wet her diaper but not with much. The doc hemmed and hawed about whether to keep her or send her home and Shoshana finally did it: she performed in clinic! She threw up 650ml which was everything fed into her stomach since the last vomit. The nurse and I pointed out that that is how much she was regularly throwing up because, apparently, when we say "a lot" it doesn't translate into anything important until you measure it and get something ginormous like "around 600mls." The doc heard how much emesis was measured, lifted his eyebrows, and decided to keep her. Even though I was on the fence at that point I was relieved that we wouldn't be going home with an ongoing hydration problem without any answers as to why.

The next few days were a rotating trial of gut rest, slow gut trial, throwing everything back up, rinse, repeat. Finally, some watery diarrhea prompted a stool test and Clostridium difficile was diagnosed. It is likely the natural gut bug was allowed to grow out of control because of an earlier round of antibiotics combined with her low immune system. Once out of control it is also highly contagious and easily tips the internal balance for other patients in the healthcare setting. It was around seven days after her first symptoms that she finally started to have a little net gain through her stomach. As soon as that happened we agreed to start her fourth round of high dose Methotrexate etc. which is the last one for this phase of treatment. Her gut continued to progress and she stopped dropping weight.

Then the respiratory secretions got bad. Her pain exploded, vital signs started changing, she was cranky, her lungs didn't sound right (though they have sounded poorly in the past for various reasons) but every test we did was basically negative. Some minor signs of infection were seen under microscope from her trach secretions but not nearly enough to explain her symptoms. There was only one thing left to try before requesting transfer to the ICU. She got scoped by ENT and, lo and behold, he saw pus in her right bronchus! Hallelujah, we have a diagnosis! Apparently there is an urban legend among doctors that it is possible to diagnose pneumonia before it is visible on a chest X-ray. Well, there you go medical researchers! It is possible. And I expect to be notified in the near future of yet another case study.

But this all goes to confirm her previous patterns of having severe symptoms before something can even be diagnosed. She sure keeps us on our toes! Sometimes I sigh and wonder why she has to be so special. Can't she just be "standard" for something? That would make everyone's job easier and would probably mean a faster and easier recovery from this damn cancer.

Oh, and we're not even done. The day before Shoshana came home Abi was a little extra cranky and I had a sore throat. The next day we both spiked fevers and wanted to do nothing but lay down and sleep. And the pain. OH THE PAIIIIN. I hate the flu I hate the flu I hate the flu. This is the fourth time I have ever had the flu. The first time was at the age of 22, two months after getting my first ever flu shot because it was a job requirement. Since then I've been forced to get a second flu shot (again a job requirement), again got the flu a month later, but this year I've had it twice without a preceding flu shot. I've worked in healthcare that whole time and traveled three continents before age 22. I don't think it's a coincidence. But that is a blog for a different time.

Shoshana has been home for two days and doing well. Abi is so giggly now that her sister is around and so interested in everything Shoshana does that she is rather annoying. I hope they can both learn some social norms through this. Abi, Shoshana needs space. Shoshana, Abi is so happy to see you!

We haven't taken many pictures recently so here are only two:

 Still pretty wiped out but enjoying her new shipment of hats.

Beginning to feel better. Yes, she has lost weight. 2.5 kilos.

Please also keep our friends Rachel, Merle and their two boys Benton and Wilson in your prayers. Last I heard they were still stuck at Children's trying to manage Benton's pain without overdosing him. Mostly they just want to go home and be in a familiar environment during the coming transition. My heart breaks for them.

Pain and other things

We had a follow up appointment and lab draw today to see if Shoshana's white blood cell counts were high enough to hit her with more chemo or not. Finally! We can begin her third round of inpatient heavy duty Methotrexate infusion. Since she has been taking longer and longer to "recover" from those treatments they are going to decrease her dose to 80% based on how sensitive she is to all the other chemos as well. It makes sense that she would be sensitive to more than one. 

Speaking of sensitivity, she is currently on a 30% dose of Vincristine in an attempt to reduce the neuropathic pain side effect but we're not seeing a significant improvement since dropping the dose from 50%. We saw the pain team yesterday and the decision was to increase the Gabapentin beyond the recommended maximum dose since she isn't showing signs of side effects of that drug yet. After this next dose of Vincristine we will have a more in depth conversation on whether to stop the drug altogether (which would greatly increase her chances of relapse) or figure that she is one of those rare people who have severe side effects with any Vincristine whatsoever and give her a higher dose while focusing harder on managing her pain.

The pain side effect has become such an issue that we once again brought Shoshana to the ER last weekend simply because her pain was out of control and there was nothing more we could give her at home. That was while on two narcotics known as Methadone and Dilaudid as well as Gabapentin which is technically an antieleptic but is the mainstay drug for neuropathy. Besides the glottic stenosis, glottic edema, and tracheostomy, the pain itself is considered a severe side effect. Sometimes she wakes in the middle of the night with severe pain, sometimes, like this morning, she is simply grumpy for a while before admitting she has pain. The goal is that she wouldn't need Dilauded more than 4 times a week. At this point we're at 7-8. Unfortunately, since she is already on a pretty intense pain management regimen any increases to her pain medication will need to be really slow unless she is inpatient where they can monitor her very closely.

Please also pray for a former roommate and his family. They just finished a set of full body scans this week ater their last ditch effort to stop the spread of his rare neuroblastoma. Unfortunately, the scans show cancer all over his body. The mom says they are going home for the weekend and having fun before making any more decisions about his care. They are believers with two sons and they live in Yakima. I cried and gave her a hug through she didn't cry with me. She says that she is still numb from the news last night and hasn't cried about it yet. My heart is breaking for them. She is such a sweetheart and we have a lot in common. Our kids were going through a lot of the same struggles when we met and I was encouraged by her several times. Please pray with me for them.

On a lighter note, here are some pictures of what Shoshana has been busy with recently:

Lunch with a different former roommate (second from the right). She met their new puppy afterward.

Playing with Abi and daddy. I love Abi's expression in this picture. They sure have the best daddy in the world!

At the entrance to the Seattle Children's Museum. She had a great time.

Normal-ish-ness

Oh, wow, it's been a month since I updated here last!

It's been so nice to have Shoshana at home.  We have had several all-day clinic appointments, a short (scheduled!) hospital stay, and a few trips to the emergency room, but compared to the last few months, it was downright normal and relaxing.  We even made it to church together a few times.

We have home nursing care at night, and during the day most days (except Sundays and every other Saturday).  This enables Elizabeth and I to sleep at night (in the same bed!).  It's been great to have the girls together.  They love to be with each other, and Abigail especially misses Shoshana being in her room at night when she's gone.  Abigail is walking now.

Shoshana's neuropathy pain is still an issue, even on the lower doses of vincristine.  Over the past two weeks or so, it has been getting worse.  Last night, after a clinic appointment in the morning, and a lumbar puncture in the afternoon (with a trip to the Children's Museum in between--her first trip to anywhere but the hospital or church since diagnosis!), she woke up from her evening nap in extreme pain, and spiked a fever.  Liz and I brought her in to the emergency room.  They are increasing her pain meds (again), and she seems to be doing okay this afternoon.

She is due to be admitted to the hospital for another round of IV chemo anyway, and would have been earlier this week, but her blood counts were not high enough to start the chemo.  Her blood counts are high enough today, but with all that's going on, they want to see her sustain that for another day before staring the round.  If not, we will have to go home, come in to the clinic on Monday, and then be admitted on Tuesday.  Bouncing back and forth between home and the hospital is no fun for anyone.

Specific items for prayer:

• That Shoshana's neuropathy pain will resolve quickly.  
• That the hospital stay and IV chemo will go smoothly.
• That Shoshana will remain in remission from cancer.  Forever.
• Please continue to pray for our family: nuclear, extended, and our much bigger family of supportive friends.  I praise the Lord for all He provides!

 I know you all just visit this blog for the pictures, so here you go: