Nephrotic Syndrome - Our new friend

I figure it is better to get to know and make friends with this new condition than spend all of our time fighting and struggling with it.

There are a couple of links to the right that can give you more detailed information on Nephrotic Syndrome, if you are interested. From our study our understanding is that this is a lot like an auto-immune disease. It is more a description and management of symptoms than it is a specific disease. In Ada's case they don't know what caused it. Usually some viral or bacterial infection trips the immune system, which then for some reason upsets the kidneys which quit functioning the way they are suppose to.

She isn't in Kidney Failure, but they aren't working right either. They are leaking the protiens into her urine, which they are suppose to keep in the blood stream, and because the protien isn't in the blood stream then her blood isn't retaining the water and the water is leaking into the area between her cells. Causing the swelling.

The swelling is more pronounced in her face in the morning and her belly and feet at night (gravity works). Normally a trim child, she has developed a "buddah belly" and "Auntie Ankles". To manage the swelling she has been placed on a severe sodium restriction, and may possibly need to be placed on a fluid restriction as well if the swelling doesn't go down.

While the swelling (edema) is the visible symptom, the problem is the protien in the kidneys. We have purchsed (after a great deal of looking around) dip sticks that detect the level of protein in her urine. They should indicate trace or negative for protein, but right now she is over 2000 mg of protein. So she tests that each morning and hopefully we will start to see the swelling and proteins go down; but it may 3+ weeks we have been told.

She has been placed on prednisone, the most common drug that seems to work 90% of the time. It will cause problems of it's own, but they tend to go away once she is off. Assuming she responds to the medication then we will just need to watch for a relapse. It may be triggered by an illness, or some hafve suggested allergies may also trigger a relapse.

In a text book case, the medicine works, there are usually relapses, but since you can catch them sooner then the child responds to treatment quicker. The relapses should become fewer and farther between until she outgrows it prior to adulthood.

So the Outlook is pretty good, but it will be a chronic condition which will need to be managed for a while. Not real common, it has been hard to find much information, but some is out there, mostly in England and Canada. As we learn more it is easier to know what to expect and to look forward to her having a normal childhood.

Angels Around Us

Through all of this we certainly have been surounded by angels - mostly in the form of family and friends.

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

After a short sleep the nurses and techs started coming in to do blood pressures, take tests, etc. Ada easily talked to (entertained) them. She was getting pretty comfortable as nothing invasive was necessary.

She was excited to order her breakfast. But then we learned that she was on a sodium restricted diet so we waited for a new menu and then reordered.

Around 11 the Nephrologist came in and looked at her and explained what was going on. I guess seeing much worse situations all the time, he wasn't very concerned and just explained what was happening, gave us an information booklet, and told us we could go home.

But by this time she hadn't gone potty in about 24 hrs, so the nurses and family Dr didn't want to release her until she could go potty. She finally did and around 2pm we were headed home.

A Long Night

The emergency room of a regional hospital is not the speediest method for getting treatment. Although we arrived at 5, we were not ushured into an exam room until around 9:00pm. In the mean time we sat in a folding chair and watched the room fill up. Some people were very upset that they had been waiting for over an hour, and the Nurses kept explaining that people are taken in order of urgency and it is a busy 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

On Sunday, Mar 2, a friend at church expressed concern over Ada and how puffy she was, asking if she had been stung or was allergic to a medication.

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

Sometime between New Year's and President's Day, the Drs assume, Ada had a cold or came in contact with strep and while fighting the illness, her immune system also threw her kidneys out of whack.


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.