2 months free and still counting

Today marks 2 months being prednisone and protien free. The cytoxan is doing its job.

That first picture is from her last relapse in Aug. The other is her current school pic from Nov. She is certainly doing well. Thank you for all the extra prayers in her behalf. Hopefully she won't relapse, but we'll keep you posted either way.

Mark One Month

Today makes a full month that Ada has been protein free without the aid of prednisone or other medications.

Sending our heart felt prayers to those who are in the middle of a relapse.

Kidneys Holding

In spite of the 'flu' yesterday Ada's kidneys are holding in there and protein is at trace. That's a good sign. Thanks for prayers.

In all honesty, we expect there will be an occational relaps, but statistically she should respond better to the prednisone now that she has had a cytoxan course. But it would be great if she never had to deal with this again.

For now our instructions are to test for protein once a week, daily if she is spilling proteins, swelling, or sick. But even then we don't need to worry unless she goes beyond a week with higher protein levels.

1st Test

Ada has been doing great, no protein spills. Today at breakfast she vomited, likely a stomach bug that is going around and has kept her from play dates with friends (hopefully of the 24 hr variety). So now we'll see just how effective the cytoxan is. Wish her luck.

Kids are Kids

The great thing about NS, is although it can be a frustrating disease, as long as it is being treated it doesn't keep kids from being kids.

Another great NS Blog

I'm always on the lookout for information about Nephrotic Syndrome (NS) and especially sites that share the down to earth experiences. Here is a great site if you want so see what another child's experience has been.

http://kid-neydisease.blogspot.com/

1 week free

As of Monday we have been 1 week without any meds and Ada continues to be protein free.

Holding on Her Own

Ada took her last Cytoxan and prednisone 3 days ago on Saturday. (She is still taking prilosec for a couple more weeks to make sure her stomach recovers from the prednisone.)

As of today she is testing negative for proteins. Hurray!! Here's hoping that Cytoxan did the trick. The Neph said to bring her back in 6 mo for a followup. Hopefully we won't see him before that.

We are suppose to check for proteins once a week. We will probably check more often than that - at least for a while. But even if the proteins are up, as long as it doesn't stay up for more than a week we don't need to worry.

We'll keep you posted.

Oct Neph Visit

This is the end of the Cytoxan treatment. Ada has doen very well on it and we have her down to a maintenance dose of prednisone (5mg every other day), Saterday she will take her last dose of both medications, and if all goes well she will stay protien free. She will go back for a check up in 6 mo. because she has been on the Cytoxan.

Just for fun we thought we would take you along on a visit. I appologize for the fashion diva - we have to pick our battles :-)

First thing they ask for is a urine sample. So far we have been able to provide 1, and that was captured at home.

Next comes the weigh in. Check out this scale. Other scales have had hand rails. Keeps the kids from holding onto something and getting a poor reading.

Height Check - This is to make sure she is still growing as prednisone tends to stunt growth.

Blood Pressure Check. Ada never gets a good reading on this machine, because that would require her to HOLD STILL!

A Medical assistant or PA comes into the exam room and gives her a once over and reviews medical history. Then Ada gets a chance to double check everything.

Finally the Nephrologist comes in and does his exam. He is pretty quick and then gives us instructions. Always on a best case scenario. The first visit he said, "Nice to meet you. If all goes well we will never see you again."

Stomach Problem identified

- warning: frank discussion of bodily functions -

I called Ada's NP and we talked about just what sypmtoms she was experiencing, how long etc. She asked if Ada had been having regular bowel movements. (OK, I need to start paying attention to that because medical people are always asking about that :-))

Anyway, I didn't think she had been real regular, but wasn't sure. Her NP suggested that she may be having bowel problems which could include painful gas. (been there, done that, not fun). It seemed to be a good fit with what may be going on - warm baths and back rubs being something that helps and all. She suggested trying miralax to see if that helped, and if Ada wasn't feeling better in the morning to go visit the GP to see if we might be missing something.

So we asked Ada if she had been having normal poops. She said yes, except the other day she had bumpy rock poops. OK, that answers that. At the NP's direction we gave her a dose of miralax (warnings all over the bottle not to use if you have kidney problems without Dr's direction - so "don't try this at home" without Dr approval) and it worked. She slept through the night in her own bed and is feeling much better today.

Don't know if there is any connection between this and the NS, the medications, or if this was just another thing life throws at you and she grabbed it. But glad we have the help we need to get through all this.

Just not feeling well

For the last two days Ada has not been feeling well. She still has her cold with stuffy nose etc, but in the morning and evening she complains of her stomach hurting and feeling like she needs to throw up, but never does. She can get distracted from how she is feeling during the day and will go play with friends for a while, then comes in and lays around the house curled up in a ball, changing positions frequently. She finds most comfort in taking warm baths, and in having someone rub her back, or hanging on me.

She isn't puffy or spilling any proteins, I am just not sure what is up. We have an appt scheduled with the Neph in a week. I guess I will call the clinic and see if she should go in and have her Primary Care Doctor just give her a look over for any obvious problems.

Currently she is on 10mg prednisone every other day, and is also taking the cytoxan (down to the last couple weeks) and previcid (or whatever the insurance is allowing).

It's a Cold

How do I know she has a cold instead of just allergies? She blew in my face and now baby and I know exactly what she is going through :-) It doesn't seem to be knocking her down too much. She doesn't sleep, and complains of her stomach hurting, but mostly she just hangs on me and goes through boxes of tissue. Proteins are staying at trace/negative. So hopefully she will knock this cold before she goes off the Cytoxan and will be able to ween off the prednisone without any trouble.

New Milestone

Actually down to 10mg prednisone. This is the lowest she has ever been on, thanks to the Cytoxan. She currently has a cold, which I think has caused her to have some trace protein readings (which is as good as negative in following NS), but it doesn't seem to be slowing her down too much so hopefully a little extra fluids and homemade soups will have her feeling back to normal soon.

She has been complaining again about stomach aches. Not sure if it is due to the cold/virus she is fighting, or her stomach just being sensitive to the medications. Not too concerned at this point, but I put a call in to the center just to let them know what is going on. Only a few more weeks of the Cytoxan treatment.

Big sister had to have some blood work done Monday. Ada felt pretty brave and important showing her the ropes at the lab :-)

Behind the Scenes

Monday when we went to the lab I took all six kids (I know, crazy). But after they were done with the blood draw this WONDERFUL technician showed the kids how she prepares the slides to do a blood count. She told them she would have loved to take them back to look through a microscope with her except there were too many infectious diseases in the back.

Check out the video of the slide prep!

Oops

When I was recording Ada's protein and meds today I realized that we missed yesterday's prednisone. We got the other meds taken, but she usually takes the pred with lunch and we apparently missed it. Since we are on every other day she took it today when we got home from the MD visit.

Something that has helped us keep track of whether or not she has taken her meds is one of those weekly pill packets that your grandparents use to set out their meds each day. It's nice because if I can't tell if I didn't get her meds or if I just failed to record them, we can look in 5h3 weekly med dispenser to see if she has taken that days meds.

Neph Visit

Had our visit today. Ada actually got them a pee sample today, Yeah! And since Aunt C had an appointment with her Docs that same day she met up with us to go to our appointment.

They ran the basic vitals, weight, BP, and listened to her body. Since it was just before lunch they heard plenty from her stomach :-)

The Nurse Practitioner told us that Ada's white blood cell count was down, but not enough to be concerned with at this point, and that they would give us a call if it did drop too much. Otherwise it looked like she was doing well and we could come back in a month.

The Neph dropped in to take a quick look at Ada and then we were on our way.

Negative - Yeah!

It was Negative this morning. Good, Good, Good.

Bad Urine Dip?

Todays dip was a 1+. I'm hoping it was just a bad sample since it took me till later in the day to get a read off of it. Or maybe something else has caused a temporary bump. We won't panic, just watching a little closer and hoping for a more 'yellow' stick tomorrow. We'll let you know.

Finished 30's

Yeah! We have completed the course of 30mg pred (every other day) without a rise in protein. Today is a day off, and then we start up at 25 mg pred. We haven't been there in a while, so this is good news. Hopefully, with the help of the cytoxan, we can keep on stepping down.

We sure hope all our other NS friends our there are doing well and responding to meds and avoiding relapses. We keep you in our prayers and think of you.

6 months

We have now been "friends" with NS for 6 months. It has been an interesting journey, not entirely a bad one.

We have learned a lot, about NS, kidneys, and ourselves as individuals.

Ada has been unable to get steroid free, so she is currently doing a Cytoxan course (8 week). Once her proteins dropped she has not even had a trace of protein. We are half way through the course this week. She has not had any side-effect problems from the cytoxan that we are aware of.

We are excited at the prospect of gettting her off the prednisone. She has gained a little weight from the prednisone, and her face has the puffy tell tell signs, but otherwise she is healthy and happy. She will have a check in with the Nephrologist next week to confirm that all is going well.

It was interesting to learn that our new family practitioner did an extensive study of the side effects of cytoxan as part of his medical school. He asks about her any time someone from the family comes in.

Unexpected Silver Linings

The saying is that "Every Dark Cloud has a Silver Lining". Would you believe it, we keep finding additional silver linings that make the cloud appear less dark?

A few we have already discovered include; a wonderful community of supportive people, increased faith, increased knowledge, and more.

Another we just discovered is increased health?!? We have to do a health evaluation every year at Dad's work, which determines how much we pay for medical insurance. We are rated on several health indicators based on BMI and blood labs. Points are allocated for each item with 100 points being given for results in the "top" of each catagory.

We usually come in right around 90 pts. Dad was really stressing the tests this year because they have tightened down each financial bracket and he was worried we would end up paying more. Lo and behold when we got his results back we were shocked to see that he had received the maximum 100 points.

The only thing we can atribute this to is a change in diet, the result of Ada's "low sodium" requirements. To go Low Sodium you really have to cut out most processed foods, which also reduces fats, preservatives, and other dietary "no no's". I didn't think it had made that much difference, but appearantly it does.

P.S. I got a 100 pts too, but only because of a Dr waver. Having my blood labs done 3 days post-partum my lab results were way out of wack, and of course my BMI was way off too :-)

So Far So Good

It's only been a couple days into the step down, but so far all protein tests have been completely negative. It's nice to not even have "trace" showing up yet.

Ada is tolerating the medications well and has not been complaining about stomach ache. I'm glad we have her on previcid (or whatever equivelent it is the insurance allows). I wouldn't have thought to ask the Dr about something for her stomach if one of the NS mothers hadn't asked me what Ada was using for stomach upset. So to you NS Moms out there - Thank you for being connected with me!!

She is also tollerating the blood tests. She has discovered that some "pokers" are better than others at finding the right spot to insert the needle. Of course the colorful arm wraps and stickers afterwards help soften the 'poke'.

Stepping down with Cytoxan

We have now had a full week of neg/trace proteins with the help of Cytoxan. Today we did our first prednisone reduction in the step down process. Yeah! We will pray all continues to go as it is suppose to.

Today Ada told me that one of her friends in her church class is having some heart and lung problems (I got all the details a 7 yo can remember). Her friend wears a little monitor on her hip that records heart and lung data for the Drs. They compared blood draw stories. It's good they have companions in their trials :-) It makes the trip more enjoyable.

Setting a Good Trend

We have another negative. So we are tracking right. Down another 2 lbs today, face returning to 'normal' (definitely have the prednisone 'moonface') and ankles are re-appearing. She is also getting her energy back and isn't feeling so sluggish.

The previcid (or whatever equivelent the insurance is approving) seems to be working and she doesn't complain about stomach aches anymore - except that she is now eating more and will overeat if we allow it. Blame the prednisone appetite that tricks you into thinking you are ravenously hungry, even if you just finished a five course meal!

A Good Loss

Once again, no proteins in the urine. And 5 less pounds on the scale as well. (so yes, we were looking at fluid gain, not fat gain from pred.) I will be keeping her weight recorded regularly now so I can definitively say whether she is experiencing weight gain or fluid gain.

Little brother woke up with a fever this morning. Hope it's just molars coming in (non-contagious) as opposed to one of the cold/flus that (contagious) that are appearantly going around. I'm keeping them separated - just in case.

We Have Negative Again

It's been a long time since we have seen that. We are thrilled. (I think Dad could do a happy dance he is so relieved to have those proteins back down - Dad really loves his kids and has been very worried).

I don't know if you saw the comment from our little friend Gray. He's 5 and has been praying for Ada. I don't know that there is much stronger medicine than the prayers of a child. Thank you - and everyone else too. I know we are in good hands, we just aren't always sure what kind of journey we are on each day :-)

Hurray - proteins have dropped!

"this morning Ada was definately trace. I was suprised . Have a great time at church. - N"

I was so happy and relieved to get this e-mail from Sis this morning. The meds are working!! Now that we are pointed in the right direction we hope to be able to complete this trip. Thank you so much for the extra prayers in her behalf.

Good News from Sis - maybe

Ada is having fun with her cousins. Sis e-mailed today and said that Ada wanted to call her dip test a 2, but Sis said it was not a 2, probably a 3 as it didn't look like a 4 to her. So maybe we are starting on our way down. We have had some "It's not a 2, but doesn't quite look like a 4" days, but then the next day it is a definite 4. So hears hoping that we can start having more of those, and even some 2 days, working back down to trace or neg. Usually when Ada drops she drops pretty quick. So we will take any chance for hope that we can.

The Nurse Practitioner who is managing Ada's case called me back Friday. She wanted to make sure Ada is swollen, not just getting fat from over eating. (Ada is swollen, she may be gaining some fat from the Prednisone, but she is also quite swollen, as evidenced by the pitting edema). She asked how much Ada was drinking and was surprised to hear she isn't drinking more. (Ada also has had a limited apetite, always hungry but never eating much). She reminded me about salts and "hidden salts" - salt used in prepared foods - and suggested we keep Ada's fluids restricted, but keep it consistent through the day. Make sure she is sipping, instead of chugging, water.

She is going to run an extra blood test on Monday, when we go in for a white cell count, to check her protein blood level. Maybe that will give us more answers. And hopefully by then her protein level in her urine will start to drop.

Waiting and Hoping

Ada has been on the Cytoxan for now for 4 days, but we have not seen a decrease in the amount of protein she is spilling as yet. We have seen a significant increase in her swelling. She is now carrying between 10 and 15 lbs of extra fluid.

We have a lot of unanswered questions right now. How long should it take for the Cytoxan to kick in? How do we best balance the fluids? My understanding is that she needs to drink plenty of water to flush the Cytoxan through her body, or it can be really hard on the kidneys; but when she is swollen we are suppose to restrict her fluid intake.

I have put in a call to the Neph Center, and they will return my call when they get a few minutes, it usually takes about 24 hrs to get a return call - and it's the weekend again (I wonder if medical people look forward to weekends, or dread them).

Ada and her younger brother and sister left home today with my sister to go visit cousins. I don't think I would let Ada go right now, but Sis is a nurse and knows how to handle meds and charting, she know how to evaluate symptoms; and she also works at a good hospital should anything urgent happen (unlikely). So this gives me a little break to enjoy the new one, and gives the kids a chance to play with their cousins.

We are doing everything we can for Ada. So at this point it is just a matter of waiting for the medication to do it's thing, and pray. Never under-estimate the power of prayer. If anything can help Ada I know that it will be due to the prayers offered in her behalf. And I am extremely greatful that she is not in pain, or suffering, only slightly uncomfortable from being puffy. We sure appreciate all the family and friends who are keeping her, and us, in their prayers. I don't know what we would do without you.

Red Badge of Courage

This is Ada with her 'badge of courage'. Today she went in for her first blood draw. Remembering the IV, which they couldn't get on the first try and had to do again, she was terrified.

Brave is doing what you have to, even though you are scared and don't want to. We had talked about what needed to be done, and as we waited the tears welled up in her eyes and she began to wimper. She had brought a picture with that she had made to give to the person that had to take her blood, just to be nice.

As she sat in the chair she wimpered, but did a good job holding still. I sat in the chair beside her and put my arm around her. She watched as the phlebotomist prepared to take her blood. The lady spoke softly to her and explained every step in advance, giving Ada a chance to ask questions and make sure she understood. Another technician came in to 'help', and held her hand so she could hold still better. They slid the needle in and Ada said in surprise, "But that didn't hurt." In a few seconds she asked when they were going to take the needle out, and the tech said, "right now" as she slid the needle out and placed the cotton in her elbow.

They asked her which color she liked. Ada looked at the pink, purple, blue, and green and said to them, "I like red." The tech opened a drawer and got out a brand-new package of red tape and placed it around her arm. Then they gave her a whole stream of princess stickers and sent her out the door saying how brave she had been.

She is keeping the tape on her arm till Dad gets home so she can show him. I'm glad it went well. That will make the weekly draws much easier.

And the good news is that she has a very healthy white blood cell count, so she is in great shape to start the cytoxin therapy. Yea!!

GRRRRR - Insurance

So we get them home from the consult, discuss options and choices, and head to the pharmacy to fill prescriptions.

One of the prescriptions she received was for Previcid, to protect her stomach from the damage it is taking from the prednisone. (she has been having a lot of stomach upset and complaining frequently of stomach pain)

The insurance denied the prescription and recommended another drug that they want her to take first - That, or we can pay the entire cost of the drug ourselves (over $100/mo).

The pharmacy has put a call in to the Dr's office to see if the alternate drug is an appropriate substitution, but that left us over the weekend without being able to start her on the medication. So the pharmacy offered to fill us through the weekend, which we did, 3 pills for $16.

I am greatful we have insurance, but this is the first time that we have been denied course of treatment as prescribed by a Dr. I'm fine with the insurance offering a lower cost to us if we use a generic drug over a brand name, but when they try to change the Drs prescription without information it is really frustrating.

I really hope I don't have to get good at appealing denied claims to insurance. I have been told never take no for an answer from insurance, but it means filing appeals and submitting claims again. I guess I will get busy educating myself on the process though, just in case.

Treatment Options

Ada is no longer responding to steroid treatment, so additional measures are now needed. Each has it's benefits, and a slew of side effects, to be considered. (At this point there has not been any discussion about the need for a kidney biopsy)

Thankful for all blessings - Ada's Aunt C:-), who also suffers from an auto-immune disease & happens to be a nurse, was up at the hospital for some bloodwork and dropped by to say hi. She was there when the Dr came and was able to ask a lot of questions that we wouldn't have thought to ask, because she is on or will potentially be taking the same medication treatments at some point and time. It was really a blessing because she was able to call me afterwards and explain things so much better and answer questions that I had.

The treatement that is most strongly recommended by the Dr (and after all consideration will be the treatment option of our choice) is a short (2 month), low dose course of cytoxin. Cytoxin is a chemo drug and with that would have the side effects of hair loss, infirtility (will not be an issue for a child this young), and reduced white blood cell count leaving her more suseptable to illness and infections.

This treatment is a one time shot. It would take place in addition to the traditional prednisone treatment and the goal is for it to allow her to complete the weening process, which she has not been able to do up to this point, and eliminate or greatly reduce the relapses. She would not be able to come completely off the prednisone while on cytoxin because while she is on the cytoxin she would need the steroid to aid her immune system. (Isn't it interesting how everything is inter-related)

She would have weekly blood tests to watch her white blood cell count. (That is what Ada is most concerned about - the needles). If her cell count drops too low they would have to stop or delay treatment.

Illness can be a serious concern. Thankfully, because we homeschool, she will not be exposed to the hundreds of other kids who come to school when they should be at home in bed. Also, because she normally does a lot of running and playing out of doors she has a pretty good immune system to begin with. And this is a good time of year, before all the nasty colds and stuff are in full swing. So I'm hoping this all bodes well for her to be able to do this treatment without complications.

Something I do need to ask them about is immunizations. She is current, but her new baby brother and her little sister are due for some immunizations. I need to know if there is a concern of her contracting the diseases from any live vaccines. If so, we will have to talk to the Dr about a modified schedule or temporarily postponing the vaccinations until her immune system is back up to par.

Other options we were given were immune suppressent drugs used most often to counter transplant rejection. If the cytoxin does not work these would be the next steps we could consider. These included:
IV steroid treatment - This treatment would include 6 doses and then a taper process. It would take about 2 years for the treatment to take place.
Cyclosporin - it is a 1 yr treatment, one side effect would include hair growth everywhere except the top of her head, and it has the potential to be toxic to the kidneys.
Celcept - is an option that has not been well studied yet in Nephrotic Syndrome.
Prograf - don't have a lot of information on this either.

So from our understanding we will be doing the Cytoxin (which is actually a brand name and sounds awful - the drug name is cyclophosphamide(sp)) It is a relatively short term treatment. They have seen a lot of success with it, usually allowing the patient to be relapse free for 2 years or longer w/ fewer additional relapses.

The Dr asked if there was any chance we noticed the relapses occuring in combination with something (most children relapse with the onset of an illness). Ada's initial and relapses appear to be completely idiosomatic, occuring without any reason. The only thing I could see that is tripping this would be seasonal allergies, such as hayfever. She has not been sick at all this year - with the exception of this Nephrotic Syndrome (so that is a blessing too.)

Headed for Consult

Ada & Dad headed down to the hospital to meet with the Nephrologist. I really wanted to be there, but physically I was not up to the trip.

So last night was spent briefing Dad on all the details of the log book I keep, where to find information he may need, Making a list of questions, and reviewing together any details from the last week - which Dad is more on top of than I am at the moment.

At this point Ada is getting pretty swollen and is feeling generally uncomfortable. Her appetite has decreased - well, sort of - she is constantly hungry and looking for something to eat, but once she starts eating she doesn't eat very much before her stomach is hurting her. She has been complaining a lot about stomach aches again, whether she is swollen or not.

A recent complaint, as of yesterday, is that her eyes are fuzzy. She is having trouble seeing far away. She had gone in to see the eye dr earlier in the year and all was fine, but she is about the same age I was when I had to get glasses. so at this point I wouldn't know whether to blame the swelling, the medicine, or genetics. We'll just have to check that out too.

I guess if there are more questions they will call me. So we will just wait to hear where we are at.

*****

Something I did do is take a urine sample this morning to send down. She has a lot of trouble peeing on command. If we don't get our sample in the morning it is very hard to get later. The last time we went down to the hospital she couldn't give them a pee sample; so I kept the sample cup and used it this morning. I had to use wipes for a clean catch, so it may not be as sterile as it could have been, but at least they will have a pee sample to work with.

Trust in the Lord

This is my new little brother. He was due this Saturday, so Mom was worried about how we would make my appointment at the University Hospital on Friday. Heavenly Father has taken care of Everything. Baby brother arrived just after midnight on Monday Morning, everything went perfect.

Since baby brother is now here we can make arrangements for my Dr appointments and not worry about Mom having him in the middle of nowhere as we travel to and fro.

I sure love my little brother. (Mom says if I keep rubbing his hair he won't have any left.)

Responses to medication

An NS friend asked me how long it takes for Ada to respond to increased prednisone. (Most patients respond within 1 to 2 weeks, but it can take as long as a month.)

After the initial presentation it took 7 days before a drop was noted, and another day before she held at trace to negative.

After the first relapse she responded to the increased prednisone within 5 days, and was trace to neg within 7 days.

After the 2nd relapse (the last one she had) she responded within 7 days, and was consistent trace to negative after 15 days.

So that is good for us to look at. This disease does not have a lot of research, and all treatments have their pro's and con's; so we will have to take everything into consideration. I hope we will have the wisdom to make the best choice for Ada.

Next step - well sort of

Prednisone increased to full dose again. I missed the call back from the clinic, but they left a message asking me to call Monday to schedule an appointment to discuss options with the Dr. That's not an exciting prospective, but since her kidneys are refusing to be cooperative, that's what we need to do.

They wanted to work us in next Friday, but that's the day before my due date, so I'm not real excited about traveling 2 hours away from my OB. Hopefully the baby will come on time and we can take Ada down the following week. But we will work something out.

Relapse #3

Ada began spilling proteins on Monday. By wednesday she was spilling at a 4+ and has not reverted on her own. I called the clinic today and spoke with the nurse practitioner. I gave her the timeline and dosages and urine tests. She will consult with Ada's Nephrologist and get back with me.

Between the Initial presentation and first relapse was 2 1/2 months. Between the 1st and 2nd relapse was 1 1/2 months. Between the 2nd and 3rd relapse (this one) was 1 month. Not exactly the trend we were hoping to see.

I'll let you know what we hear from the nephrologist.

Pineapples don't have salt

Ada's protein test was 4 this morning. As we finished the test and she read the results I said, "No salt today." She said, "It's a good thing pineapple doesn't have salt, so I can eat that."

I love her optimism. It makes things so much easier.

We will watch and wait, make sure this is her trend and give the center a call on Friday.

Wrong way - Back up

Ada is spilling more protein today. She is only at a 2, but we need to change direction and get back to trace and negative. There isn't much we can do to make that happen, it's all up to the kidneys. But we are watching low sodium so that if she does a complete relapse hopefully the swelling can be kept from getting too bad. We'll keep you posted.

Doing well

We're half way through her first step down again, and seems to be doing well. Her proteins fluxuate between neg (0) and 1+, but as long as the 1's are occational and the negatives are the 'norm' then everything is good.

So roughly another week of 30 mg prednisone every other day. Then if all goes well we can step down to the next level.

In the mean time we continue with life. Ada enjoyed two weeks of swimming and did really well. She is sad that we have to wait now till after the swim season for lessons to start up again.

Taper Time

Ada has tested trace to negative consistently for a week now, so we can begin the taper process again. Tomorrow we will begin 30 mg prednisone Every-Other-Day for 2 weeks. We are glad to be tapering again. Hopefully we can make it a little further in the process this time.

Holding off on taper process

Even though it has been a week since Ada responded to the increased medication she continues to have protein leaks up to a one, so we will maintain full dose until she can maintain one week of trace to negative protein leaks. I expect that should be within another week, or at least I hope so. In the mean time she is looking and feeling good and enjoying summer playing with friends.

Negative Again (that's a positive thing!)

Ada's proteins read negative today. So once she can maintain that (or trace) for a week then we will begin the taper process again to try and wean her off the prednisone. In the mean time, by restricting sodium, she has been able to lose a lot of the fluid she had built up. And her stomach is feeling much better. Yeah!

Yeah, responding again.

Ada was excited to report this morning that instead of a 4 her urine protein measured at 1. Hurrah, she is responding to the prednisone again. That is always my big fear is that the medicine will quit being effective for her. So we will hope things hold for the weekend. Then I will call the clinic on Monday for instructions.

I was asked if her stomach discomfort was due to the prednisone or other factors. I think mostly it is from the increased swelling. Her stomach is as firm as my pregnant belly, maybe even more, and that puts pressure on her stomach so she always feels like she has overeaten - but at the same time she always feels hungry. The stomach discomfort is more noticeable first thing in the morning, and later in the evening (I think being busy in the day keeps her mind off it).

If she doesn't take her prednisone with a good meal then she also has stomach discomfort; but I try and have her take it with lunch, eating something first, then spacing each pill out through the meal. Once the swelling is down if she continues to have stomach aches I will ask if we can split her dosage up so she takes half as much twice a day.

Again, Thank you for your prayers and fasting in her behalf.

Holding in there

Although Ada is still spilling lots of protein, at least the swelling is not increasing. She alternates between play and rest, depending on how she is feeling, usually related to how her stomach feels. At night she likes to sleep ont he couch upstairs near us because she gets naseated. When she gets up in the morning she seems to feel a little better.

So at this point we are holding steady, not getting better yet, but not getting worse either, and for that we are thankful.

On a side note:

The other day Ada was visiting a neighbor's garage sale. A shopper asked our neighbor if Ada had Nephrotic Syndrome. This woman explained that she had NS for 14 years as a child. She is now 30+, married, with 3 cute children. She said that for her the relapses tapered off about the time she hit puberty, and eventually went away all together. It was nice to see a success story and gives us good hope for Ada.

Consult with clinic

I talked to the Nurse Practitioner who is overseeing Ada's case at the University Hospital. Of course they are not happy to hear that she is swollen.

After trying to assess her over the phone (which is difficult to do) I was told that I could bring her down for an appointment to be seen, or I could continue to monitor the symptoms and keep in contact with the clinic. Since all they could really do at this point is take a urine sample, blood pressure, and look her over, I have decided to just monitor her here.

Ada is at the full dose of medication and we just need to give her body time to respond. In the past she has responded in about a weeks time. So hopefully she will respond again quickly, which should be by this weekend. But it can take longer, so we will just wait and see and keep an eye out for any dangerous sypmtoms (peritonitis or dermititis).

In the mean time the best thing we can do for her is keep her sodium as low as possible to control the swelling, and limit fluid intake. I worry about limiting fluid intake, but thankfully Dad just installed a swamp cooler, so that should keep her cool, and I have fruits like grapes and apples that can help quench her thirst.

She is still a pretty happy girl, but is lethargic for her and tends to spend a lot of time laying down now. She frequently asks to have her back rubbed and says it makes her stomach feel better. So reading books is a good activity for us, and I don't mind. Neither do the other kids. We are going through several books she wouldn't sit still for before, and many of their favorites. What better activity on a hot afternoon when it is too hot to go out and play anyway.

So that's pretty much where we are at. Wait and watch; pray that she will respond to the medication (the NP says they have some other tricks up their sleeves in case she becomes steroid dependent or non-responsive to the medication.)

Thanks so much for your prayers and caring about us. I will continue to post as we monitor her progress. Hopefully the protein leakage will stop as quickly as it started.

Our love to all,
Ada & family.

Relapse number 2

As you can see, Ada has completely relapsed.

The protein leakage started Sunday and increased rapidly. By Wednesday she was charting her proteins at 4+, as high as our test sticks monitor. She has also noticed that her urine is foamy - one symptom of leaking proteins.

There was no noticeable swelling until Thursday evening. I was looking at her stomach and ankles and thought she might be swelling again.

Friday morning she woke up with the "downs" look and I knew she was swelling. Because it is the holiday I called the nephrologist on call and they suggested we take her back up to full dosage of prednisone (which is only one level this time) and severely limit her sodium. Also limit her fluid intake as long as the swelling increases.

I will call the center on Monday to see what we want to do from there. I am assuming that we will watch for a time to see if she will respond to the stronger dose again. We are hoping and praying. In the mean time, she is pretty healthy and active, although she experiences some stomach discomfort from the swelling and will lay down for a bit when it gets bothersome.

Monitoring

Today her protein level was at a 4+. She also has been complaining about a mild stomach ache. At this point all we can really do is keep watching. I'm not sure if her stomach ache is linked to the kidney problem, or if it is from the amount of fresh fruit she has been enjoying.

Otherwise she looks and acts normal. She has not experienced swelling, which I contribute to her low consumption of sodium. I started making her bread again, and we just leave the salt off the meat. Using Mrs Dash has made that easier. And honestly, her pulled pork beats our wafer ham sandwiches :-)

We'll keep you posted.

Spilling protiens again

The other day Ada started spilling proteins again. Today and yesterday she was at a 3+ (the scale is negative, trace, 1,2,3,&4). She was just coming to the end of her month long treatment of 30 mg prednisone everyotherday and then we were to drop down. So I called the clinic and talked to her case manager.

The first question they always ask is if she has been sick and is she swelling.

She did have diarhea the other day, and is not swelling.

We are to hold her at her current prednisone level and just watch her for the remainder of the week (of course it is a holiday week). Then I will call the clinic back on Monday and we will evaluated any changes and see what to do at that point. Hopefully she is fighting a little stomach bug which is causing the problems.

Of course if she starts swelling or experiencing severe abdominal pain or fevers we are to call the on call physician at the children's hospital.

Keep her in your prayers please.

Visit w/ Nephrologist.

Ada's visit went well. As long as she is responding to the prednisone without suffering from side effects they will continue with the current course of medication, even if she is dependent on it. If she stops tolerating the prednisone or quits growing then they will look at switching course of medicine, most likely to Cyclosporine (or Cytoxin?) for a short course to see if that will work. But at this point, so far so good.

They did ask if she was always so smiley and giggly and hyper, or if the prednisone was having a big impact on her personality. I had to confess that she has always been a whirlwind, but her moods are just a little more intense on the prednisone. Also her eating needs to be watched from time to time, but due to her energy level we don't need to restrict calorie intake much.

Included here is a picture of her just being a normal child on a family outing. Although we are dealing with protein tests, medications, and dietary precautions; there is still plenty of room to just be a normal child.

Tapering Again

Ada's proteins held at trace to negative for a week, so we have begun the taper again. She is now taking 30 mg prednisone every other day. We identify days as "Prednisone days" and "Non-Prednisone days". I don't do this because they are "good days" and "bad days" but because I can check the log and tell Ada what kind of day it is, then she can remind me at lunch time if she has to take prednisone with her lunch. (I am such a space case these days that it is good she can be responsible too)

Positive response to medication

Ada's proteins are holding at trace or negative. That is good. She is doing well and very happy, with plenty of energy to burn. She has been complaining of stomach aches in the evening and I have wondered if this could be a sensitivity to the prednisone. I will have to ask the Dr when we see him again. She did not swell at all that I noticed this time, thank goodness for our little protein test strips. I am glad that the relapse cannot sneak up on us over and over again. Always greatful for little miracles.

Yeah, proteins dropped today

Ada's proteins were recording at a 4 since she spiked. Today they dropped to a 1 (I did the test, so I know it is accurate). So if that holds then we know she is responding to the medication again. If we can get her down to trace/neg for a week then we can begin the taper schedule again. She won't need to stay and the high prednisone dose like she did the first time.

Thanks for prayers and support.

:-( First Real Relapse

Well, Ada has relapsed. But at least with the urine tests we are able to know much sooner and begin treatment before she is all swollen up.

So, we go back to a full prednisone dose until she can be trace/negative for a week, then begin the taper schedule again. If at first you don't succeed - try, try again.

Interesting to note: On Wed, when we were suppose to call if her protein output wasn't down, she came into my room excited to report a negative urine test. It was our hope, but also sounded too good to be true. After some questioning we found that since she couldn't find a pee cup she just dipped into the toilet bowl after she went potty. Got great results, but not accurate.

Holding through Wed

Ada continues to spill proteins without reason (cold, flu, illness). Monday the Nephrologist said to just hold her medication level. They would really like to give her body a chance to respond on it's own without increasing her steroid dosage. So I am to give them a call on Wed and see if they want to give a little more time or if we need to increase her dosage.

We continue the low sodium diet. It won't make a difference on whether her kidneys function or not, but it does minimize the amount of swelling she will experience when her kidneys aren't working.

Bump to Hill

Drat! We didn't go the right direction this morning. Ada's proteins were up to a 3+. She hasn't been this high since the first week we started medication clear back in March.

Today was suppose to be another step down in her prednisone dosage, but I wasn't comfortable with that since she is headed in the wrong direction. So I called the pediatric nephrologist on call (of course it is the weekend when things go south) to get some guidance until Monday. She was very kind and suggested that keeping Ada's prednisone dosage stable would be best over the weekend; and to test the proteins daily, then call if they remain up in the 3/4+ for more than a couple of days, which will put us on Monday, regular business hours, and they would determine whether to increase her dosage. If her protein drops and holds then we can continue the step down.

Of course we are to keep her sodium low and watch for sudden increase in edema, or for a fever. In the event of fever or pain we are to get her to the hospital immediately as this is a symptom of peritonitis, a serious infection. I don't expect we will experience this since we have caught things early.

So that is where we are at this weekend. Watch and Wait. We will of course seek a priesthood administration for her this weekend. Keep her in your prayers. Thank you for your support. Again, we'll keep you posted.

And Holding

OK, her protein tests for the last two mornings have been holding at a 1+. Hopefully over the weekend it will go back down to trace or neg. My concern is that tomorrow, Saturday, is another step down day for her prednisone.

So, no cause for panic yet, just - Holding at 1, praying for a drop, watching for an increase. . . . and waiting, and watching, and hoping, and waiting (how does that song go?).

On a support site they suggested not watching proteins daily or you can drive yourself crazy. but I would hate to be testing weekly and see a spike and not know if we had a bump or if we were into a trend. So for now we choose to drive a little crazy :-)

Protein Bump

Ada was very worried yesterday when she did her protein test and it was up to a 2+ (it has been holding at neg with an occational trace). Since she didn't get her test done until afternoon I told her not to worry about it and we would just make sure we did the test first thing in the morning and would be extra careful about sodium just in case.

(protein leakage tends to increase in normal bodies after they have been active or when fighting illness)

This morning she tested at a 1+. She has bumped up to a 1 for a couple of days before, so we will just be watching closer until it comes back down. We don't need to worry unless it maintains this level for a week, or she spikes to a 3 or 4+ for more than a day or two.

We'll keep you posted

3rd Prednisone reduction, 2 more to go

Stepped Ada down on her prednisone again yesterday, from 25 every other day to 15 every other day. She is getting excited to be done. (crossing fingers and hoping all continues well).

Saw the primary care doctor the other day just to do routine exam. Her blood pressure is good, no swelling, or proteins or blood in the urine. Told us we don't need to some back other than normal well child checkups as long as she continues to do well. He did encourage us to make sure she gets vitamins and fluoride as the prednisone interferes with calcium absorption in teeth and bones.

Who is Molly?

If you read any of the comments to posts in this blog, you will see a few from "Molly's Mum."

Through blogs I have met Molly & her Mum. Molly is a beautiful 3 1/2 yr old girl who has been fighting Nephrotic Syndrome for a year now. Her journey has been similar to Ada's, but she leads out a year ahead. Although she responded well to the treatment, she has relapsed a couple of times and is now looking at a biopsy and full steroid treatment again.

Molly is a real trooper, and has been through so much already as she began life in an orphanage in China. Her story is not only one of NS, but also of the miracle of adoption by loving parents.

So as you pray for Ada, take a moment to remember the other many children of God around the world who are also in need of extra prayers.

Prednisone Reduction

Yesterday we were able to reduce Ada's prednisone from 30mg every other day to 25 mg every other day. She will only take 4 doses of this and then we will reduce again to the next level, assuming we have no setbacks.

A few days ago her protein levels slipped up to a 1+ for the day, but then immediately fell back down to negative and have held there for the las 3 days, so I am learning not to get excited when her proteins raise up to a one. Our instructions say we don't need to contact the Dr unless she holds between a 1&2 for a week, or raise to a 3-4 for more than 2 days.

My understanding is anything can cause a temporary raise in proteins, from a slight cold or allergies, to testing the urine after she has already been up and about for a little bit.

So everything is looking good at this point. We'll keep you posted as we continue the step-down process.

And Holding

All's good. Still toggles between trace and negative from day to day. I think that is suppose to be pretty normal. Maybe we should have another child track for a week or two and see just what is "normal". Good opportunity to teach the kids about having a "control" in a science experiment. Not that you can "control" anything about Ada :-)

Steady Progress

Ada has been on 30 mg of prednisone every other day now for 2 weeks without any negative effect. She continues to show only neg or trace proteins. She is active and otherwise healthy, and doing well with schoolwork.

I am amazed at the amount of personal responsibility a now 7 year old (happy birthday Ada) can take for their own care. When Grandma and Grandpa took her out shopping for her birthday, Grandpa picked himself up a package of malt balls. She decided she wanted to get some too, but then asked to see how much sodium was in them. She took the package from Grandpa to read the nutrition lable and then told them, "Oh, 150 salt. I can only have 100 salt." So they went and found lifesavers instead (zero sodium). She does the same with breakfast cereal and other food items brought into the house. Good Girl, Ada

Prednisone side effects

I thought I would comment on the side effects we have seen in Ada since she has been on Prednisone.

The first thing we noticed was the constant hunger. She never feels full and is always hungry. To help I try to keep lots of fruit and veggie snacks around. But she will sneak bread given the chance.

The second side effect is an upset stomach. I don't think she was eating as much with the prednisone as she should be. So I told her she had to quit downing the handful of pills all at once and had to eat a bite of food before and after each pill. That seems to have helped.

Finally, her energy level. When grandpa heard she would be placed on a steroid (the prednisone) he said, "Are you sure you want HER on a performance enhancing drug??" Of course prednisone is not the same type of steroid as those abused by the athletes, but it does seem to increase the energy level. Ada has always been an energetic child, but now she is really intesnse. So when she is happy, she is giddy. When she is feeling silly she gets crazy. When she is frustrated she becomes very rude, and when she is upset she has trouble calming down. We have a corner for her to sit in until she can "control the prednisone". I know it is hard for her, but she must learn that no matter how you are feeling, you can't let the drug control you. She handles that pretty well and is usually back in control after a short timeout.

Otherwise she is our same fun little girl, always on the move and into everything.

1 month of treatment - Photo Log

I thought it would be good to show some photos of how Ada is progressing. First, here is a picture showing Ada before, at diagnosis, and 1 month of treatment.

click on any of the pictures to see them better.

before NS, at diagnosis, 1 month of treatment. On the last picture she looks pretty normal. If you look close along her jaw line you can see some of the visual effects of the prednisone, looks like she is starting to develop a "double chin"


Here are updates of some of the other pictures we have shown.
Her torso: full edema, 1 mo treatment (normal)


Her feet: with edema, 1 mo treatment (normal)


The dip stick test results: blue/green shows proteins, yellow shows lack of proteins.

Addressing the Low Sodium Diet

From the beginning I understood that the reason for the low sodium diet was to reduce edima and pressure on her body. So at the visit with the specialist I asked about the need to continue the low sodium diet now that her edema is gone and the protein leakage is controled. This is what I learned.

While Ada is on prednisone it will cause her to retain salts and water, which will cause an elevated blood pressure and unnessesary wear on her heart and other organs. So as we drop the prednisone we can be less strict in the sodium restriction.

Muscle Spasms

Ada has started getting muscle spasms in her fingers and toes. Similar to a charley horse, the muscles or tendons or something cramp up, causing the fingers or toes to become stiff and pull in one direction. When they are bad they can be painful up to her elbow or knee.

The family Dr suggested it could be a potassium difficiency due to the low sodium diet and suggested just keeping plenty of bananas in the house. The specialist said this is something they see that they can't explain. He said they have tried all sorts of things but don't see a consistent response, but it seems to go away after a time.

For now, when she does get a cramp we have found that working the area, having her open and close her hands or walk on her feet, seems to work out the cramp and then she is fine for a time. They do not happen too frequently, probably less than daily, and are more curiosity and nuisance than a problem.

Aren't these bodies of ours a curiosity?

Follow up with Nephrologist at 1 month

We just got back from our first follow up with the Nephrologist (kidney specialist) at the University Hospital.

Great News. They are thrilled with how quickly she has responded to the medication and continues to have days with negative and trace proteins. We start the weaning process to get her off the medication, which should take until the first of June if all goes well.

The Nephrologist said that we will see occational bobbles where her proteins will go up a little, especially if she is fighting a cold or allergies, or has been especially active, and not to worry unless they stay at a 1 or 2 for a week or if they elevate to a 3 or 4 plus; or if she shows signs of edema (swelling).

As we ween her off, hopefully the proteins will stay down and this will be her last visit to the nephrologist; There is a good chance she will probably have at least one recurrence, but they are hopefull that with her positive response that she will be one of the lucky ones who only ever have one episode. But if not, at least now we know what to look for and will be able to catch it early.

She continues to be really good about taking her pills and following the low sodium diet (fear of blood tests or the idea of a kidney biopsey really motivate her to be diligent).

We have been so blessed, and again thank you for your prayers, interest, and kindness.

Back Down

Ada's proteins held at 1 over the weekend, but today, monday, they are back down at a trace. So we just keep monitoring, watching the sodium, and praying that she will continue to improve.

set back?

Ada has been testing negative for proteins for the last week, really encouraging us. But a couple of days ago she crept back up to trace, not a concern; and now is at a 1 (on a Neg, Trace, 1,2,3,4 scale).

She does have a cold, so maybe that is just par for the course. At this point we don't need to do anything different unless her proteins stay up for a week, or raise up to a 3 or 4 for a couple of days. And we have an appointment with the nephrologist the end of next week. He will be able to know at that point if we can continue as planned weening her off the prednisone or if she will need to stay on it a little longer.

So here is hoping that her cold clears up the the proteins go back down.

Give us this day our Daily Bread

Boy this phrase has new meaning. Every time I turn around it is time to make bread again. I really enjoy making bread; but depending on the homemade bread creates a greater demand than I have had before.

I guess I could buy bread for the rest of the family and just make Ada's; but everyone else prefers the homemade bread too - even without the salt.

I have read stories of the pioneers, and rising each morning to make the daily bread. I thought maybe that was just because things didn't keep, and maybe that was the case, but with a good size family it really is easy to eat up several loaves of bread in a day or two, thus time to make bread again.

But the kids are good helpers and enjoy rolling the rolls and forming the loaves. And Ada loves her bread over everything else.

Low Sodium Options

The other day we had a school field trip to the zoo. Because it is a good couple of hours in driving I wanted some snacks for the kids, which Ada would be able to enjoy. Fruits are all good, but they also needed a bread or some kind of carb. All the standards were out, pretzels, crackers, etc. So I sent Dad to the store to find some low sodium snacks.

Dad came back from the store with some low sodium chips and rice-cakes. The chips were suppose to be fruity, but they were so sweet that after one chip none of the kids was willing to eat any more. I always thought of ricecakes akin to eating styrophome, but I have to admit that the apple cinimon rice cakes were pretty good, and compared to my pretzels weren't actually any dryer. We had a great time and enjoyed the snacks on the way home.

This weekend is Easter and we have family coming in to help celebrate. I knew the traditional ham was out, but didn't expect the turkey to also be out. Since turkeys are now injected with juices, depending on brand the turkey was 450 - 650 mg of sodium per 4oz serving - that's before gravey and everything. Fresh turkeys are a little pricey so I looked around for other options. Ada and I found a whole chicken that was only 60mg. So for Easter this year the family will all enjoy a turkey, and Ada will have her own chicken, which Dad has begged her to share with him.

Most easter candy is low sodium though, except malted eggs :-) Happy Easter

Negative for Proteins!

Yeah! Today Ada tested negative for protiens. After the first week, when her proteins initially dropped, she tested negative for one day. For the last week she has only been showing a trace of proteins, although yesterday it was a close call between trace and negative. Today it was Negative!! Here's hoping it holds.

The other day when we came out of the Drs. with news that her blood pressure had dropped, Ada looked at me and asked, "Do you think I'm doing better because so many people are praying for me?" I told her, "Absolutely!" She smiled big. I told her today that since so many people are praying for her to get better and she is being blessed that she needs to remember to pray for everyone else that they will receive the blessings they are in need of. I am grateful for the opportunity she has to learn about the power of prayer.

Improving still

Another Dr Appt. Her blood pressure was down closer to what they want to see. It was 110 over something. If I understand right, they want the upper number to be 100 or less, for a child her age, hight, weight, and gender.

They also want a pee test to check for blood in the urine; but the last two times she hasn't been able to produce. So they sent home a container for next week so we can catch it when it comes.

Ada continues to look good and is very active. She is tolerating the medicine and it is holding her proteins at a trace. She is getting a pretty good appetite, but an eating schedule is helping with that.

Holding Steady

Ada's Protiens are holting steady at just a trace, which is good. Her edema (swelling) is completely gone, which is also good. And she isn't looking dehydrated today.

Unfortuneately, her blood pressure remains high. It's not so high that it needs to be medicated yet, and the best way to treat her high blood pressure at this time is to continue to maintain a low sodium diet. So we are keeping it low, but not as low as we did initially.

Feeling Blessed - again

Today Ada came to me and said she didn't like how her eyes looked now. They looked pretty sunken and dark. I am hoping it is just part of the adjustment as the syndrome reverses itself and her body tries to find equelibrium again.

In looking for more answers I can to this site by another mother of a child with Nephrotic Syndrome. As I read her story I count each blessing since things were so much worse for them, and am grateful for how things were instead of how they could have been.

http://www.geocities.com/rausha/neph.html

Taste adjustment

Quick note: There was only a trace of protein this morning, so we are keeping a good trend. Edema continues to go down. She has her belly button and ankles back.

The other day Dad and I went out to dinner for a much needed date. We went to one of our favorite restaurants and ordered a favorite meal. I was surprised the next morning to find my tongue feeling thick and dry, and my whole self feeling generally sluggish from the sodium in the meal. I didn't realize you could become so sensitized so quickly.

Lookin' Good

We are so excited!! The treatment appears to be working. Her urine tested negative for proteins this morning. Hurray! Also, much of the swelling in her body has gone down. There is still some edema, but she is getting her ankles and knees back. And we have Ada's laughing eyes back. She is still a little puffy in the cheeks, but I suspect those are the prednisone cheeks (moonface) that will go away once she is weened off the prednisone. Hurray!

Now that she is doing so well and the edema is under control I think we can go up to the original sodium restriction. Which should be cakewalk now.

Hygene Hazards

One problem that we have encountered, which I hadn't anticipated, has been bathroom hygiene. Because Ada's stomach, pelvis, and thighs are so puffy it make it very hard to properly clean after going to the bathroom.

Also, we noticed her "running" in a side to side waddle because her legs would rub together and get sore. She was starting to get very raw in that region.

To combat this I got out an old peri-care bottle from a past pregnancy and taught her to "wash" and dry after going to the bathroom. This eleviated much of the soreness and made it easier to take proper care of herself.

Turning the first corner!

Hurray! We are so excited. Yesterday the edema seemed to be down considerably in her face around her eyes, although it was still significant in her abdoman, pelvis, and legs. Today the protein level in her urine showed it starting to drop a little. Of course it will take a couple of days to show a trend, but we are very hopeful.

The hospital had told us that a few kids started to show improvement in as little as a week (it has been a week to the day); but average was 3 weeks with some taking as long as four.

I know she is improving so quickly because of the prayers by so many in her behalf and our diligence in following the dietary recommendations. Thank you everyone for your prayers. They are making a difference.

Reducing Sodium - trickier than you think

To manage the edema and help the kidneys recover Ada was placed on a low sodium diet. I quickly learned that "No added salt" did not equal "low sodium"



The first days after we came home from the hospital Ada continued to increase in puffiness. The skin around her eyes was so tight I called the hospital, worried about infection, but because the skin was not warm and she wasn't running a fever they said it was just the edema and we needed to restrict sodium just as much as we could. I made it my personal goal to keep her sodium between 300 & 500 mg a day.



The charts included (click on to increase size) show how much sodium is in natural and processed foods. Pretty much anything in a can or box was out. Also out was anything made with salt, baking powder, or baking soda. We took everything high sodium out of the cupboards and put it down in the pantry. It served us well that I was use to making a lot of things from scratch.



My biggest concern was bread. One slice of store bread is 160 mg sodium, almost half of what I wanted her getting. So priority number one was to make our own bread. I switched from butter to shortning, left the salt out completely, and found the lowest sodium milk I could. Doing that we had bread down to 10 mg per roll.



We filled the house with fresh fruits and vegitables for her to have for snack, and started cooking everything without salt, or season salts.



I also found a Baking Powder that is sodium free. It was kinda pricey, but it let us put pancakes back on the menu. I also got a recipe for tortillas that had no sodium, and were surprisingly easy to make. So now we can make veggie wraps.

I learned that yogurt, sour cream, and cream cheese made good substitutes for mayo and dips, and I also learned to make vinegrettes to use in place of salad dressing.

One more thing we did. We switched off our water softener on the cold line to the kitchen sink. I was surprised to learn how much sodium that puts in our drinking water. Not a big concern, except when we are trying to limit sodium.


Doing all this we have been able to keep her diet in the low 300's. And it must be working, because the edema is reversing and the protiens are starting to drop. Hurray!!

Once she is doing better, we will ease up and return to the hospital recomendation of under 2000 mg a day. But that should be cakewalk compared to what we have been doing. And I am convinced if we hadn't restricted her sodium so much she would have been hospitalized for pressure on her heart or lungs due the increasing edema.

Mayo Clinic has some great recipies for low sodium (or low fat, or gluten free for that matter) which I look forward to trying. http://www.mayoclinic.com/health/healthy-recipes/RE99999

New Daily Routine

While for the most part we try to keep Ada doing all her regular activities, a few new things have been fit into her daily schedule.

Each morning she has to do a urine catch and dip. The sticks weren't easy to find, but a good pharmacist checked all over the area for us and was able to help us get them. They show us how much protein her kidneys are leaking. It should read negative, but right now she is reading high, although in this picture it is the first time the color isn't quite so intense.

At noon time she gets to take her prednisone. Since she was able to swallow pills they sent her home with pills instead of liquid. So at lunch she is suppose to take 4 1/2 pills with food. She likes to take them with the rolls I made her.

She is getting pretty good at taking them, and in this picture is trying to take two at once.

Also, since she needs to make sure she isn't always eating (the prednisone makes a person ravenously hungry) and to monitor how much sodium she is getting, she has flexible eating schedule where we log everything she eats, how much sodium is in it, and how much fluid she has taken in.

Ripple Effect on the Family

While Ada's condition has made a huge impact in her life, it has also had a ripple effect on the entire family. As parents we can add credibility to the idea that stress has a negative effect on the immune system. After things calmed down a little we both found ourselves battling colds.



Ada has an older brother and sister. They have been invaluable in keeping the family functioning. From helping get food on the table, to cleaning the house, to playing with the younger kids. But it is asking a lot for pre-teens to fill the roles of adults, and they are now ready for a break, and taking it. We have to work with them to keep them focused on school work. But they will come around.



The greatest impact has been on Ada's little sister and brother. While they don't identify that they feel neglected, it quickly became evident in their behavior that they were having a difficult time with the amount of attention we, and others, were focusing on Ada. Especially little sister, who has alway thought herself a twin with Ada. At one point I overheard little sister say, "I wish I was fat and poofy too!"



As soon as we recognized what was going on we made some quick adjustments to make things as normal and "fair" as possible. The school routine was quickly re-established for the older ones. For the younger ones Dad began taking them out on extra dates. He would help them buy things that matched what Ada had been given by friends so they could all play together. They each have their own water bottle, like Ada, to monitor how much they drink. They get straws, and they get to eat the same things she eats.

What did she look like? (a photo journal)

The edema (swelling) developed in Ada very gradually. Every time a new Medical person saw her they questioned if there was actually any edema until they were able to feel her legs and stomach. I thought it would be good to post some pictures so you can see the change over time. You can click on any picture to see it up closer.

Here are some pictures of her the end of summer. You can see she is normally a petite child.

Here is a comparison of her just before Christmas, February, and today, March. Then I also included a picture that shows her first thing in the morning. You'll notice by her eyes which side she sleeps on each morning.

This picture below shows the edema (swelling) in her abdoman. These pants have an adjustable eleastic waist. They were cinched tight at Christmas time and still were loose. In this picture I have just let them out as much as they will go and she still appears to have "donelaps" disease (my belly done lapped over my belt)

Update on Ada

All the posts I have made on this blog so far have been about what has happened, and a lot of people are asking "How is she doing NOW". I got an e-mail asking about ada and decided to just post the reply straight to the blog. Here it is:

Hi there, (got an e-mail from Dad's sister-in-law and thought I would send an update out to all since the blog is a little delayed)

Ada is stable (I guess that's the best word for it - she isn't in any danger at this point, but she isn't doing better either, and it may be a while). The swelling is still increasing. She has to work to keep her eyes open. (I'll be placing some pictures on the blog http://www.adaneph.blogspot.com/)

We have her sodium limited to 300 mg a day, which is taking some work. They have said that if she keeps swelling we will have to limit her water intake to under 24 oz a day, but I don't think she is drinking that much now.

If you didn't know her you would just think she was an obese child who is very active (she was out riding her bike yesterday). She has no activity limitations and is actually encouraged to do anything she would normally do. Toward evening she walks like she has been on a horse way too long, and she has "auntie ankles" which get uncomfortable at night. I think the hyperactive part of the prednisone is kicking in, like she needed that :-) And she is getting the prednisone appetite so she is constantly hungry. Time to get more fruits and veggies in the house.

I guess the swelling isn't a concern unless it starts putting pressure on her lungs or other vital organs (which would be indicated by pain beyond discomfort). Also, anytime you have fluid pooling in the body there is increased risk of infection (which would be indicated by a fever). So at this point we are good there.

So I guess we have done the "hurry up", and now we will do the "wait" for the protiens and swelling to start going down. Ada is in really good spirits and taking this all in stride. She is a pro at downing the prednisone (4 1/2 pills once a day), doing a urine sample, and reporting her food and water intake. She also is pretty good at understanding which foods she can and can't eat right now, and is good about asking. If she wants something she can't have then we start looking for something she can that is appealing to her. I made her a special batch of rolls that are really low sodium and she loves snacking on those. She is a big bread person and it was killing her to not be able to have any bread.

As for the rest of us - other than being tired, even with plenty of sleep, I am doing well. Mostly I am just busy enough that I don't notice the pregnancy, especially since I'm not awkward yet. We are hoping to know the results of Dad's sleep apnea test by the end of this week and can get him on the Cpap. The other kids are doing well, although little brother and sister had been acting out with the attention focussed so much on Ada. But we have made an effort to direct more attention their way and they seem to be doing better. So at this point we are just getting back on a normal schedule/routine, and monitoring Ada.

Hope you are well, glad spring is peaking it's head out.

Love.