I created this blog when my 6 year old daughter was diagnosed with Nephrotic Syndrome (NS). As an uncommon and difficult to understand condition I wanted a journal, along with a way to let family know how she was doing, and a way to connect with others experiencing the same thing. This blog chronicals the events, emotions, and more she and we as a family went through. The initial episode begins in March 2008. On the side are pictures as well as links to sites I found helpful.
Monday, March 31, 2008
Back Down
Saturday, March 29, 2008
set back?
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
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.
Saturday, March 22, 2008
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!
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.
Friday, March 21, 2008
Improving still
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.
Tuesday, March 18, 2008
Holding Steady
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.
Saturday, March 15, 2008
Feeling Blessed - again
Taste adjustment
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.
Friday, March 14, 2008
Lookin' Good
Hygene Hazards
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.
Thursday, March 13, 2008
Turning the first corner!
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
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
Wednesday, March 12, 2008
Ripple Effect on the Family
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.
Tuesday, March 11, 2008
What did she look like? (a photo journal)
Update on Ada
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.
Nephrotic Syndrome - Our new friend
Angels Around Us
As we headed to the hospital Dad's parent's offered to meet us to pick up the other kids. They took the kids out to dinner and then over to Dad's Sister's house to stay the night. The next morning another sister picked up the kids to play with more cousins. The entire time we were in the hospital, focused on Ada, we did not have to worry for a minute about the other kids. That was such a relief.
As we headed home one of Dad's step-sisters asked us to meet her at a pulloff on the interstate so she could send dinner home with us. Not only did she give us dinner, but also enough groceries to last us a week. That was so thoughtful of her. So much that she picked up were things that Ada can eat without having to worry about the sodium.
When we got home a neighbor across the street offered to order in pizza for the family. Another neighbor called the next day to say they were bringing in a meal for the family; and that day another friend called to say she and a friend were bringing dinner in on Saturday. That was such a blessing because we had such a busy schedule already for Friday and Saturday of things the older kids were suppose to be doing. It was just nice to not have to worry about that thing.
Many friends and family have expressed concern and included us in their prayers. I know Ada is doing so well because of all the prayers in her behalf. In the midst of affliction the windows of heaven truely open and the blessings are poured out upon us so that there isn't even room enough to receive it all.
Heading Home
Saturday, March 8, 2008
A Long Night
When we got back into an exam room we sat and waited for a little while until a nurse came in. They asked for the general story of why we were there. Took a blood pressure, looked in eyes, nose, throat, then said a resident would be in to see us.
The resident came in, asked for our story, looked in her eyes, nose, throat, and felt around her skin and stomach (she gave him the rest of her stomach contents) and then they put her on a no food or water restriction.
He asked lots of questions and seemed frustrated that I didn't know how often she had been peeing, if she was drinking more or less than usual, when her last BM was and if it was loose or firm. I was feeling like a pretty negligent Mom by the time he left.
In the mean time Ada was always talking to anyone that came in, telling them she was all puffy, that her eyes were hazel, and all about her brothers and sisters. She was such a chatterbox that those who came to see her loved visiting.
The nurse came back in, did the blood pressure test again and said the lab would be coming in to take some blood samples. The techs came in to start an IV line and Ada really got scared. Dad held her while they tried to get it in her left hand. But she was so puffy they weren't able to get a line, so after trying and try and trying they finally pulled the needle out, put a bandaide on and tried the right hand. I felt so bad for her.
They were successful in getting the line in her right hand and then took 4 vials of blood to run tests on. Ada was all eyes and ears again wanting to know what and why and everything about what was going on with her blood.
Now a Family Medicine Dr came in and did the same series of eyes, ears, and nose tests, then said they would contact the pediatric Nephrologist on call for instructions. When the Dr came back she said that the Nephrologist suggested Ada be released and we go home and come back in the next day or two to see him in clinic. But because Ada was so miserable and wasn't keeping things down, and since we lived so far away, she decided it would be best to admit Ada into the hospital and work on getting the fluid off to make her more comfortable.
So they started the Admission process (it was now 2 in the am) and gave her an injection, through the IV, of Lasix (a diuretic) to try and get some of the fluid off her. By 3 we were in a room and getting settled. They put us in a room in the NeuroTrama Center. I was given a fold out chair to sleep on, and Dad took the glider. Ada was hooked up to all her machinery to keep her vitals, and hospital staff dropped by to chat with her. They said she was quite famous throughout the emergency floor for her chatty nature and how cute she was.
We were happy to learn she was just to be observed, so as long as the machines stayed hooked up they were just going to let us sleep until morning (for what was left of the night). Gratefully, we fell asleep.
The Adventure Begins
Aha, I thought. So I started giving her Benedryl, but after 24 hrs it still wasn't making any difference. I spent some time online looking up puffy (a very vague description to look up.) What I found was a reference to edema, when the body retains water. A specific type of edema is "pitting edema" and is evident when you press your thumb against the persons skin for a couple of seconds and a divit remains. That was easily done with Ada and explained what was happening to Ada's legs, also her arms we found. So I called and made an appointment with the Dr for Friday.
While we were waiting I kept searching on the internet for causes of edema. What I was finding was not encouraging, mostly information about major organ failure. I mentioned this to my sis-in-law, who has been in the medical field, and she suggested that I call the Dr back and speak to a nurse instead of the receptionist to findout if the Dr was comfortable in waiting till friday. I wasn't certain if this was necessary as it appeared to be going on so long. I kept searching on the internet and found information about edema on the Mayo Clinic web site. It described the symptoms and complications and possible causes. It also gave a list of when to contact the Dr. Ada did not have difficulty breathing, but it also mentioned abdominal swelling. I checked Ada over and sure enough, her belly was swollen up like a balloon, the belly button closing up. So I called the Dr and spoke to the nurse, who set up an appointment for the next day (wednesday).
After a LONG morning we got in to the Dr and they wanted a urine sample, which took forever because as part of the disease she was not processing liquids, and there were so many other things to be interested in than going potty. But we finally got a sample and then she was all over finding out how they did the tests (dip stick). They also took her blood pressure again, it was high for her age, so she wanted to know just how they took the blood pressure. Then a medical student came in to do the exam all over again while the Family Dr spent some time on the phone calling specialists and looking things up on the internet.
Finally he came in and told me that he wasn't certain what was going on but that her kidneys were not functioning right and he suspected she had something called Nephrotic Syndrome (or minimal change syndrome) and needed to be seen by a pediatric nephrologist (kidney dr). He told us that we could wait and get an appointment in the next couple of days, but he was worried about wasting valuable time if her kidneys were shutting down and suggested we make arrangements for the other kids and get her straight to the local Regional Pediatric Hospital's Emergency Room. So we went straight from the Dr office to pick Dad up from work, got home, had a friend help give her a priesthood blessing, told each kid to pack pj's and a change of clothes in their pillow and get in the van. We dropped off a few responsibilities to others on our way out the door, not knowing if we were looking at 24 hrs or several weeks away from home, and headed out on the road for the pediatric hospital.
On the way we encouraged the kids to watch movies (on the little TV things we had picked up at christmas while stranded by snowed out roads) and we made calls to get the older kids excused from lessons they were suppose to be at that night, called family to let them know kind of what was up, and ask for prayers.
Grandma & Grandpa met us at the hospital to take the other kids to Dad's sister's house to stay the night. By this time there was so much pressure in her abdoman that she had vomited in the van, and again in the waiting room.
We got signed in, and managed to provide another urine specimin. Now Ada is feeling very bloated and stiff and is not feeling well or acting like herself. We also learn at this time that she is carrying somewhere between 5 and 10 lbs of water with the bloating. At 5pm they have finished all the initial check in and we are told to take a seat and they will call us back as soon as a room is available.
How it all started
Most of Ada's symptoms were so gradual, that it took hind sight to link them all together.
Several weeks ago I noticed Ada looked kindda funny when she woke up in the morning. I described it to my sister that "she looks like she is down's (syndrome) in the morning". But I knew you don't GET down's, so I chalked it up to typical growth and development where the face changes from a toddler to a child. Honestly until I looked back through pictures I didn't realize how much change was taking place. (Even at the hospital, when they first saw her medical personel would ask, "So she looks pretty normal, are you sure she is puffy?" Then when they would feel her legs they would say, "Oh yeah, she is pretty tight.")
Then a couple weeks ago when she kept trying to get out the shorts outfits I noticed she was getting "pudgy". Because she is plenty active and eats appropriately I decided she must be putting on some babyfat that young girls often do building up to puberty. I didn't want weight to become a focus for her, especially at this age.
About that same time she came and showed us a "dent" in her leg. It was a smooth, deep, groove in her leg. Her leg felt "tense" so I massaged it for a while and the dent went away. I figured she had a muscle cramp and it was knotted up enough that the knot was visible and had pulled the area around it funny. Actually, it was from her sitting with one leg over the other for a while and the pressure actually dented her leg. That was the "pitting edema", which we would learn about later.
It wasn't until Sunday, the 2nd of Mar, that everything began to snowball.