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.