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.