Time passes

Wow, has it really been 2 years since I last posted?  No news has been good news.  She is still on the tacrolimus, 1.5mg 2x daily.  She has only relapsed once since I last posted, and it was because she was sick and missed too many doses of her meds.  I think it is good news that there have been a couple times her protein have risen, but they have gone down on their own within a weeks time.  She has not been back on prednisone for well over a year.

This summer she is working as a lifeguard at a scout camp.  She has done better at remembering to take her medicine, I hope she will keep it up.  It's a leap of faith to let her go for 2 months and be on her own, but she needs to learn to manage it on her own sometime, and she needs to have as normal a life as possible.  The camp is willing to communicate with me and make sure she is able to contact me if needed.  Next appointment in another 6 months for a follow up.

Steady as she Goes

No news is good news, right?  This summer has been pretty uneventful for her.  The Tacrolimus appears to be doing it's job.  The hardest part is making sure she takes her medicine every day.  Her last relapse was because she became lax at taking her meds.

As a child she was really diligent about taking her meds, so I - wrongly - assumed she would be even better as a teen.  But alas, she seems to have lost some of the interest in taking care of her own medical needs.  The doctor said kids can be pretty good at being on top of things, but teens usually are terrible at being responsible for their own care, and that responsibility does not seem to return until around the age of 24.

We are trying to help her by putting signs up in the kitchen and bathrooms to remind her, and using a pill minder so that when we ask if she took her medicine she can't get away with just saying, "I think so."

She's a good kid, I guess it's just hard to have to be responsible all the time for something you wish you didn't have to worry about.

Valentine's relapse

Feb relapse

Good Morning Miss Stay-Puff, looks like you've relapsed again.

I can't believe I have neglected posting updates.  Here is a quick run-down.  Summer went great, School is going well, she continues to be a happy, cheerful girl.  She is excited because having a reprieve from prednisone has given her a chance to grow - now claiming a full 5 feet - Yay!

Miracles happen: Her medication (tacrolimus) was re-classified as preventative, which means it is covered in full by the insurance, pre-deductible.

Dec Pre-relapse

November, doing well so the Dr reduced her tacro because she had made it almost a full year without a relapse.  Guess we may have been a bit premature though, because at the end of December she started spilling protein again.  Called the clinic and she was put on the prednisone relapse protocol.

Unfortunately, right after completely weening off prednisone, she started spilling protein again, so she's right back on prednisone.  We'll visit the Dr in March and re-evaluate if her tacro needs to be raised, or it there are new triggers to consider.  Both relapses coincided with her menstral cycle (a new factor).  Triggers or coincidental coincidance?

Have a Happy New Year!

Update

Ada has responded very well to the Tacro.  She has been in remission all year so far, the longest in a long time.  In June she started spilling again, but only at a 2.  The difficult thing was she was suppose to be going on an LDS Trek - hot, dry weather with miles of walking.

The PA was worried about her going while she was spilling, but after discussing it with the Dr, considering there would be ample medical staff on hand, we - her parents - were also going to be on hand as part of the staff, and she would only be an hour and a half drive from the children's hospital, she received medical clearance to go.

While she continued to spill on trek, she did not worsen and did not become swollen - what a blessing.  After she got home, we went to see her Dr.  We talked, the Dr. verbalized all his thoughts, then said he was inclined to just let it ride for another couple of weeks.  If she didn't improve, then we would have to increase her dosage.

I agreed to this as it felt right.  Sure enough, about 5 days later her protein went back down to negative and she was doing great again.  So glad she is doing so well.

As to the medicine, we did a lot of calling, checking with various pharmacies and our insurance.  We checked all the medication coupon plans.  Eventually we found that by appealing for a change in pharmacy we were able to get the cost of the medicine down to $100.  Although that still seems like a lot, it is pennies compared to the $600 we were originally quoted.

Sticker Shock

We have been very blessed that prednisone has been an affordable drug.  In the past insurance has kept all other medications affordable to us, expensive being over $50.  This year we have a new insurance program, which is not nearly as nice as we use to have.  We were expecting the medication to cost more this year, probably in the $100 to $200 range.  Nothing could have prepared me for the $600 price tag.  I'm going to have to do some looking around and finding out what programs to reduce medication costs are out there.  Wish me luck.

Steroid Dependent

Waiting for the Dr

Well, Ada relapsed again, so the other day we went in to the clinic to consult with her doctor.  Because she responds to the prednisone, but relapses before she is off she is classified as "steroid dependent", which is better than steroid resistant - where the person no longer responds to prednisone.

Bring something to do

The Dr discussed a couple of options.  Cyclosporine, which has the undesirable side effect of excess body hair and is potentially toxic to the kidneys.  Another medicine which I forget the name of that may or may not be successful in treatments as all tests with it have been in teens who are prone to remission anyway.  The final drug, and the one the Dr recommended was Tacrolimus, which is similar to Cyclosporine but without the hairiness.

The expectation is that with the additional help of Tacrolimus Ada will be able to wean off of Prednisone, which will allow her to grow.  After she has been off the prednisone for a year, then she will be weaned off the Tacrolimus, with the hope that her NS will stay in remission.

We will get her started on the Tacrolimus, and then in one month a blood screen will be done to make sure she is at the right dosage and not having other adverse side effects.

I Can't See!

The last year was a pretty typical year.  Occasional relapses, treatment with prednisone, taper, enjoy a couple months off, relapse, repeat.

But last week was a little different.  She was in the final week of a taper when she relapsed again, fast and hard.  She swelled up more than she ever has since her original diagnosis.  According to the scale she was carrying about 20 lbs of surplus water.

The scary part was she told me she couldn't see.  Not that she was going blind, but her vision was really bad.  I called the Drs office and they were concerned about a rare side effect of prednisone where pressure is increased in the eyes.  They also noted a slightly elevated glucose level in her last blood labs and said it could be attributed to that.

First we went to the store to get her some clothing she could wear, because none of her clothing fit and she was very uncomfortable.  Then we went to the hospital for bloodwork.  Finally we tried to find someone who could get her in for an eye exam.  Being the end of the year, that was challenge.

It was a scary time, because vision problems are always concerning, and the idea that we may have to discontinue the use of prednisone would mean needing to look at a different course of treatment.  Thankfully, the optometrist was able to rule out glaucoma and cataracts.  All of her vision problems were attributed to the swelling, and as the swelling started to resolve, so did her eyesight.

Her proteins are now dropping, the swelling is significantly reduced, and her eyesight has returned to normal.  We are grateful for the medical care that is available to us and especially grateful for the many prayers that were offered in her behalf.  We know that they make a difference in her, and our, life.

Happy day

One blessing we enjoy is that Ada is rarely sick, so her NS is seldom triggered by illness.  However, shortly after Christmas she came down with a pretty intense cold, was very congested, and started spilling proteins.  (come to think of it, she is always congested with a relapse, I wonder which is the chicken and which is the egg?)

Anyway, she was spilling up to a 3 (on a scale of 0 to 4+), but never quite made it to a 4, so I decided to just keep watching until the cold was over and see if there was any chance it resolved on it's own.  Low and behold, as the cold subsided, so did the proteins.  Today she announced that her pee test had arrived back down at negative.

We are so excited that it resolved on its own.  I'm hoping this is an indication that she is getting close to outgrowing her NS.  Finger's crossed and counting blessings - but not ready to hold out breath ;)

Update and a dexoscan

I have been derelict in posting Ada's nephrotic events.

After her relapse in early june, she had another relapse in July within a week after she got off the prednisone, so she got to go to our family camp on prednisone, but it didn't stop her from having a great time.





We had to go see the nephrologist in early Aug so they could evaluate her.  Because she had experienced 3 relapses over the summer, with only a week break from the prednisone, it was time to start talking stronger medication.  We discussed the alternatives, and I didn't really feel good about any, except the new one that the Neph honestly doesn't think works, he just thinks it is a coincidence because it is usually tried on teens, who tend to outgrow NS anyway.

They would really like to get her off prednisone, but we talked some more.  The 2nd relapse this summer we thought we could attribute to her receiving vaccinations so shortly after a relapse, and the 3rd relapse happened at the same time she experienced a cold.  So they figured they would go ahead and give her one more shot at getting  off the prednisone on her own.

In the mean time they are trying to decide whether to be concerned about her grown, if the prednisone is inhibiting it.  As you can see from the picture, she is a bit shorter than her sister, who is 2 years younger than her (yes, that causes a bit of turmoil for her and strife between them).  However, we think she is just on a slower growth pattern.  Her older brother was short until his later teens, and she has several cousins who are 2 years apart in age but the same height.  Ada continues to have a steady growth curve, and her height is not abnormal for her age, she is just on the shorter end, and her sister is on the taller end for her age (and also experiencing a growth spurt of 6 inches in 18 mo!)

The conclusion is that a dexoscan was scheduled (bone density scan) and we are suppose to make sure she gets plenty of calcium.  Since she isn't a big milk drinker, I have her take a Tums daily.  I wish I had taken pictures of the bone scan.  She had been worried about it, but it was super easy and completely painless.  She just lay on a table while the scanner rotated around her, but it wasn't a closed tube like an MRI, it was a curved arm that moved up and down over her body and around the sides.  She just had to lay still while the arm was moving.  Not too much of a trial, but if she had to do it when she was a toddler it would have been more of a challenge.

We were so excited that this week, the first of January, made 5 months medication and protein free.  Unfortunately, the other morning she was looking a little puffy and a pee test showed she was spilling again at a 2+.  Since the entire family are down with colds, we are going to just watch and see if the protein improves after she gets better.  It doesn't usually work that way (but then illness isn't usually her trigger, we don't know what is) but it has once  before, so we will be hopeful.

Well, that's our update.  Last year wasn't bad, and we will hope for better this next year.  Wishing everyone else a happy and protein free New Year!

Up, Down, like a merry-go-round round

Ada went off prednisone the first week of June, just in time for girls camp.  She had a whole 2 weeks off and then she got sick - something along the lines of strep, but not strep.  Anyway, she started spilling protein and swelling up, so I called the University today and they said back on prednisone she goes, just in time for scout camp next week.

In all this I am so grateful that the biggest disruption in her life is the need to watch her sodium intake and remember to take prednisone during the EOD schedule.  Otherwise, she is able to go about life as normal, playing, learning, and growing as any child should.

Relapse, 2nd go

So Ada didn't go back down on her own and I called the clinic.  They had us go to the local hospital for a urine and blood culture.  We received a call within a couple of hours verifying that she had actually relapsed and we would need to start over on the treatment.  We were to make sure she was protein free for 5 days before beginning the taper process.  So she was back at full strength daily for two weeks, and now we are tapering again.  Hopefully we will make it all the way through and have a successful weaning process.

Oh well, at least she is having a great time with swimming lessons, playing with friends, and learning.  It could be so much worse, and should eventually fade away into a memory.

Vaccination bump

I took Ada to the Dr the other day for a routine physical and she was given a Tdap booster.  Today her proteins bumped up to a 2.  I called the clinic and they will have us stay on the every-other-day until her proteins are back down for a week.

In hind sight - Would I not do the vaccination?  No, but I probably would have waited until she had been in remission for a week or so - I think?

Dr Visit and Consult

It had been a while, so we made a run down to the Hospital for a face-to-face visit with the Dr.

We were lucky to get the first visit of the day, so the office was running on time!

The Dr was pleased that she is responding to the prednisone and her growth is steady.  He wants her getting plenty of dairy for calcium.  She is suppose to outgrow these relapses, but she will probably relapse again.  As long as we are catching the relapses and she is responding to the prednisone we don't really need to be concerned.  We are only looking at stronger treatment if she reaches the point where she either doesn't respond or is dependent on the prednisone.  We are OK if she gets 4 months or more off of prednisone.

4 months is better than 2 months - right?

Well, I had been really optimistic and believed that Ada would make it at least a year without spilling proteins again.  But thankfully she had 4 months protein and prednisone free.

Have notified the clinic.  Will watch her protein level daily (it is currently at a 3+) and report back next week.

I was so optimistic that I had not been riding Ada to get those pee tests done each week, so who knows how long she has been leaking.  This morning the tell tell signs of facial edema were obvious and I demanded a pee test, refusing to let her shrug it off (I can't blame her for being tired of the routine).  The stick was dark.

Oh well, praying for the best and realizing she has good medical care we are thankful for.

Cheers!

State of the Ada address

We are coming up on the end of 5 years with NS.  Ada is doing pretty well.  She had a relapse earlier this year, but has been prednisone and protein free since early November.  She is growing into a delightful young woman.

We have not noticed any significant side effects from the treatments she has been under.  She is shorter than many of her friends, and even her younger sister, but we do not know if that is a result of the prednisone or the genes she happened to inherit. She has cousins who are shorter than their younger siblings, and while she is short, she is not disproportionate.

Ada does not allow NS to slow her down.  She is active with her friends, learning to swim, and becoming a fabulous little pianist.  We are hoping this will be the year she so goes protein and prednisone free.  We hope you will be protein and prednisone free as well.  But if not, keep your chin up and hang in there, hopefully that day is coming.

On the Road Again

So it is good to be on a smooth road again.  Ada successfully weened off the prednisone 2 weeks ago and has stayed protein free.  I expect she will relapse again, I hope she won't, and we will be grateful for each day, week, month, and year? we are granted.

Wishing you all a good Holiday Season and longer time between relapses (100+ years :) )

Relapse #??

Yup, it's a relapse.  I called the clinic last week to let them know she was spilling proteins.  They told me to watch her for another couple of days and if she hadn't gone down give them a call back on Friday.  They hadn't gone down.  She was at a 3+ when I called the clinic back.  They weren't thrilled but what can you do.

They sent a prescription into our local pharmacy for more prednisone and put her on the fast taper.  60 mg daily until she shows trace for 3 days, then 40 every other day for 4 weeks, then off.  (this is a new schedule that gets the kids off prednisone faster.  I don't know if it works better, worse, or the same.)

So, she is being good about the low sodium to keep her swelling down and testing daily.  Her relapse spread over a longer time period, so maybe things are changing and after the taper she will be protein free for a while.  Time will tell. (I suspect not, but am always hoping!)

We made it 9 months!

See that right eye, more almond shaped?  That is the classic look of a relapse.  A week ago her morning pee test was up to a 1.  During the week it bumped up to a 2, but went back down.  This morning she was at a 3.

We are still keeping our fingers crossed that her kidneys will self correct, but I suspect not.  We'll keep you posted.  Till then - Keep Smiling!

Happy Mother's Day

I thought I better give a quick update.

I last posted that Ada had come down with a minor cold/flu and with that we found her proteins up in the 2+ range.  I had her take care of herself so she could get better and we watched and prayed.

Blessing of blessings, as the illness faded away so did the protein level.  Her body recovered on its own.  (It is not uncommon for anybody to spill proteins when their body is stressed, so this was not a relapse!)  What a wonderful Mother's day present, for Ada to be protein free for a week again.

Proteins

Ada woke up feeling sick the other day: mild fever, upset stomach, generally unwell.  She slept most of the day.  Today she still felt a little off but got up anyway and started feeling better through the day.  I told her to go take a pee test so we could keep an eye on things.  In the past she has never had a relapse associated with an illness.  I guess this time is different.  Although she wasn't spilling any proteins on Monday, she is today at a 2+.  Hopefully as the illness resolves itself her body will resolve the proteins too.  It isn't uncommon for anybody to spill some proteins when their body is sick or stressed.  We'll post updates as they come.

On the positive side, This is the first sign of any proteins since last November.

Heading into 2012

(always a smile!)

Although no news is good news, I thought I would share a little about how Ada is doing.

She went off prednisone on Thanksgiving Day 2011 and has been doing well.

She tolerates the prednisone pretty well, but this last time that insatiable appatite took over and she gained some excess weight. I'm pretty sure she will work it off in normal play, but I am trying to help her retrain her eating habits.

I'm attaching a few photos to show that nothing slows her down. Even though we would like this disease to resolve itself (which the Nephrologist assures us it will in time) she isn't allowing herself to become a victim of the disease.

a hike with dad ice-skating with sis.

2011 update

Thanksgiving Day we completed the weaning process off of prednisone. 2 weeks now and we are protein free. I think the Dr made a good call to treat her as if we were starting from the beginning. Do I expect her to relapse? Honestly, yes, I think it will happen again, but I'm hoping we'll see a good year before it does happen.

Again, the expectation is that while she will relapse they will become less and less frequent until they don't happen anymore. So while a relapse is a set-back, it doesn't mean we aren't making progress.

Update

The adventure continues. . .

We were sailing through things pretty good. She weened off the prenesone and was protein free again. We were now testing weekly and I was glad because we were headed to a weeklong woodbadge and family camp and I didn't want to mess with pee sticks and dietary concerns.

So what happened :-)

A couple week before we headed to camp she swelled up like the pillsbury dough boy. A protein test showed she was up at a 3+. We tracked it for a week and called before the weekend hit. Back on prednisone. I was worried she wasn't going to come down because it was taking so long, but she did, and with severe sodium restrictions the swelling went away.

So - now what to do about camp? We packed up her meds, plenty of fresh fruits and low sodium snacks, got a copy of the camp menu so we knew ahead of time where restrictions were needed, and took off to the camp.

As far as diet was concerned, since the swelling was gone I just had her use prudence and not gorge herself on chips and hot dogs - had her load up on the fruits and veggies instead, but gave her permission to enjoy what was offered.

As far as activity was concerned - it didn't slow her down a bit. She went hiking and sailing, she played games and made crafts. She made new friends and had a wonderful time.

NS is a pain - well, actually there isn't a lot of pain involved as long as we are on top of the edema, so it's more of a nuisance, and we are still hoping remission will return, but in the mean time life is going on and she is a very happy, well adjusted child.

Although we have not finished the weaning process her proteins have begun to elevate again. The are holding at a 2+, which makes me suspect that when she weans off she will fully relapse. But for now we are not increasing the med and are just watching to see what happens.

Lots of prayers and love and fasting are appreciated. They keep assuring us that eventually this will go away on its own, and until then we just need to manage it. So we will keep managing. I'll see if I can get a couple of pictures of her posted so you can see just what an active happy child she is 4 years later.

well - it's been a while. Ada went a year and a half without any problems, then last summer she had a relapse. She did a run of prednisone and was OK for another 4 months. At the first of the year she did another course of prednisone, but shortly after she went off she has relapsed again.

She is going back on prednisone, a higher dose this time, with a quicker taper. We'll cross our fingers that it will hold for a while so we don't have to talk about more of the intense auto-immune drugs.

One other thing - she has not had any illness to trigger this, but she is congested from allergies. We are going to aggressively treat her allergies and see if that will help keep her from relapsing again.

Official Relapse

After over a year and a half we now have an official relapse.

At the end of June her proteins spiked, but they came back down on their own, which is great. But early July they went back up again and have stabilized at a 3 now for a couple of weeks.

Due to a new baby coming and an unexpected family funeral, I had postponed notifying the clinic, hoping she would resolve on her own. She was not swelling, or sick, and I took her to the Dr for a blood pressure check and it was good, so I continued to put off calling. But today I decided I needed to notify the clinic and find out where we go from here.

Her nurse, Debbie, was very understanding. We talked about where we are at, how things have gone, and then determined that the best thing to do at this point is to put her back on the prednisone, using a relapse schedule, and we would schedule an in-clinic appointment after things have settled down a little around here after the new baby comes (hopefully this week).

So, back on the "icky medicine". Glad she can swallow the pills instead of having the liquid.

Keep hands and feet inside the ride at all times

Looks like Ada is back on the ride. A month ago she was looking 'funny' so I had her do a test (she has been clear for so long that we were testing about every other week or so, especially since I was running out of test strips). She tested at a 4.

It was going into the weekend so we just ordered new strips, limited the salts, and watched her. After a couple of days the numbers started going down and by the new week she was at a 1 or 2, so I decided to take a 'watch and see' approach. By the end of the week she was consistently trace, and even had a couple of negatives.

For the next while she was

No change - still watching

The other day Morg wondered if Ada was puffing up, her eyes looked a little funny. So we did a dip stick test (I'm almost out of those guys again). Trace. She looks good today and her sticks still say trace. trace is good. Happy active child. A little short, but that could be genetics as much as the prednisone.

Update

So Ada is back in auto-pilot.

After a couple days of 1 or 2 she shot up to a 3+ for a couple of days. But I also noticed her urine was very dark, brownish. I didn't know if that was her being dehydrated (has she been drinking much lately - I haven't a clue) or were more serious things going on?

So after consulting with our family Dr we decided to push fluids (nothing drastic, just making sure she was getting a reasonable amount of fluids) and see what happened.

The next day her urine was light colored, pale yellow, and her protein back down to trace. We were able to continue the same pattern for a couple of days.

So she is definitely spilling some proteins - but only a trace, which is unconcerning to the nephrology clinic. But we have learned that if she gets dehydrated the proteins will become more concentrated in her urine. Likewise, if she drinks more water they will be diluted. But as long as she is drinking a reasonable amount of water that should give us our best indication of how she is doing.

So do I know what is going on - not really. I hope it will clear up completely in time. But at least for now she is able to go about normal activity and we continue to know what to watch for.

Ha Ha Jokes on me

So, of course, Ada did a pee check last night after I responded to a comment the other day from someone asking how many times Ada had relapsed since her cytoxan and the stick was dark, a 3+. I decided not to panic, she has been running trace with an occasional 1 and the clinic said she was fine as long as she didn't stay at a 1 or 2 too long or spike to a 3/4.

So we checked again this morning and she was still a 3+, pushing a 4. So back on the low sodium kick while we wait and watch. Won't be too big of an adjustment as we maintained a lot of the habits from before - will just miss the convenience of store bought breads.

Hopefully her body will self correct. If not, I guess she will have to go back on a prednisone treatment. Fortunately most of her friends have responded much better to the prednisone after their cytoxan treatment and so that bodes well for Ada.

And I thought/hoped we were done for good. We'll see.

Good News

Talk to the clinic (well, left phone messages back and forth). The good new is that as far as they are concerned trace is as good as negative, so as long as her 1s are occasional and she doesn't spike above a 2 then we can just keep an eye on her. No swelling or any other problems. I'm glad we don't have to worry about prednesone or anything else like that. So on with a normal life, knock on wood.

Who knows??

So Ada continues to test trace, 1, trace, more than 1 but not 2, trace, nothing high, but not negative either. I meant to call the clinic, but we just kept going and I never got a few minutes with the phone. Do I worry, or just keep an eye out? Hopefully I can get ahold of the clinic next week.