At the beginning of last year, Brinlee was continuing with her steroids, but weeks of trying to wean her from them, proved to be an on-going struggle. Last April, she had a terrible week of diarrhea and swelling that started on Easter weekend. She also had a runny nose and cough which because her immune system is compromised, can trigger her kidneys to "relapse." Although there is not ever a good time to be sick, our family almost always gets sick on a holiday or the weekend when being seen in the clinic is not going to happen. So, our only option is to go to the ER. We did start that Easter day at my aunt's home with extended family. Unfortunately, Dave was sick that day and so I was on my own for getting the kids ready. I took them to church and then we stopped back at home to grab the food and off we went. If you are like us, time is always flying by and we struggle to get out the door on time. Needless to say, I was very stressed to rush all three kids into the car and get all the necessities also packed up. After finally grabbing everything and buckling each of them in their seat, we headed out. As we were just about to pull out of our neighborhood, the purple frosted cupcakes I had made and sprinkled, flew off of the front seat and splattered onto the car floor, glove box, and center console. I tried to catch my breath and bite my tongue as I put the vehicle in park and assess the damages. A few tears streamed down my face, knowing those cupcakes were frosted during the very late and sleepy hours the previous night. I wanted to throw in the towel and turn around. It took every last bit of strength to trudge forward to the Easter gathering. I called my dad, telling him I was going to be late, but unfortunately people were hungry so dad told me to take my time, drive slow, and that despite the fact they were going to eat without us, there would still be plenty of food when we got there.
We made it to my Aunt & Uncle's and I just couldn't relax and enjoy the holiday. Brinlee was clingy and I knew she was sick. It felt like every passing minute, she was getting sicker. Either that or I didn't have time to pay attention to just how sick she was feeling during my flurry of rushing that morning. Her eyes were puffy, belly slightly distended, and she intermittently cried out from stomach pains. All I could think about was wanting to get her to the doctor, but debated on whether it would be worth a trip to the ER. We left our family shortly after the Easter egg hunt and arrived home in the late afternoon. Because I felt we may be able to at least get a start on antibiotics to assist her already compromised immune system, I decided to bring her in to our local small town hospital. Explaining all the pertinent information to someone who doesn't know anything about your daughter in a small amount of time, was no easy task. This was a doctor who listened, but didn't understand the entire realm of treatment and countless plans that have been made with her primary doctors. He did a thorough exam of ears, throat, lungs, and belly and determined that she likely had a virus. I asked him to give her a prescription for an antibiotic with a plan to see her primary doctor the next day and it took a lot of convincing. Understandably, he did not want to treat an already over antibiotic treated country without a need, but Brinlee is different. I explained that because she has such a compromised immune system, has a runny nose and cough and is spilling lots of protein (a big sign that her kidneys are struggling), her primary doctors recommended that she would need antibiotics more than any average patient. He continued to deny us the antibiotics and I politely told him that I understood and we would call her primary doctor the next day whom I was confident would give her antibiotics. He waved his white flag and decided to give us enough doses of antibiotics to get her through till the next day.
That night was rough. Brinlee grimaced and knelt over when the abdominal pains struck and then screamed as I cleaned her bottom from numerous loose diapers . The next day, we went to a pediatrician that knew Brinlee, but was not her primary doc since her primary was not in that day. We did stool cultures, labs, and continued her antibiotics along with a burn cream for her poor diaper rash. Her weight was elevated slightly and I planned to keep a close eye on her over the next couple days. That doctor called me the next day and it seemed as though the diarrhea let up a bit and her bouts of pains were not as often as before so I explained that I thought she may be on the mend.
A couple days passed and I was wrong in thinking she may be getting better. Her belly was increasingly larger, she wasn't eating, and was still having belly pain and lots of diarrhea. We went to see her primary doctor and noted that Brinlee had gained approximately 5 pounds that week. This, after not eating and having diarrhea. He recommended we go home, pack our bags, and head to the University hospital ER where her nephrologist works. She was clearly needing interventions quickly. With tears pouring out of my eyes, we both agreed that she had ascites (fluid built up in the belly) and may need to stay overnight to have the fluid aspirated or removed. I was extremely tired and overwhelmed. I felt very sad that my daughter was going through so much and wanted her to feel relief.
We went home, packed our bags in case we were staying at the hospital, and her primary doc called the University ER to tell them what was going on.
When we arrived at the ER doors, the front desk staff were expecting us and we were immediately brought to a room. Because this is a University, there are many student interns and in our case this is who greeted us in the room. You could tell he was fresh from the classroom. He of course asked us what brought us there and after I explained Brinlee's long week of belly pains, endless diarrhea, FSGS, and weight gain, he smiled pointed at her extremely bloated abdomen and says "Well, it's clear what's going on. You can see it from here. She full of stool." It took just about every ounce of strength inside me to not immediately tell him to get a trained doctor in the room. I took a deep breath and said, "well, her doctor and I are pretty sure she has ascites with all of the symptoms that she's experiencing and the fact that she's not eating and yet having constant stools, we are pretty sure it's fluid and not stool." I didn't tell him I am a nurse. I wanted him to treat me like he would anyone else, but boy this was a tongue biting moment. He left the room, likely to consult with his "upper" and came back to the room with a plan. He was going to first do a bedside ultrasound to quickly clear up the matter of whether or not stool was filling her belly. The very first area of her abdomen that he placed the ultrasound probe, he jumped the gun and quickly said, "Not much fluid here." He then proceeded to check her entire abdomen and did see what he called "a bit of fluid." Many labs were drawn and an x-ray was also done. After a couple hours of tests, he and his colleagues came into the room with the final status and plan. He stated, "Well, the good news is, she doesn't have much stool in her belly." My tongue was starting to hurt at this point. He continued, "It does appear that she has some fluid in her abdomen and with her protein levels where they are, we talked with the Nephrologist who recommends she start a medication called Furosimide, also known as Lasix." Before he could even start telling me about the medication, I nodded and said "Yep, ok." He paused, then stated, "You've heard of this before? Has she been on it in the past?" Well, so much for keeping my cover... I sighed and said, "No she hasn't, but I'm a nurse so I'm aware of the medication." In that moment, I think he had a flash back to the moment he confidently pointed to her abdomen and was convinced she had stool in her belly. Funny to think he would think that our doctor would send us to the University for stool in the abdomen. Her steroid dose increased yet again and she started Lasix. We luckily were able to go home that night and hoping to finally be on the road to remission.
A couple weeks passed and we went to see her Nephrologist. It was determined that Brinlee had likely built up a tolerance for the steroids and thus they were not effective any longer at that time. It was time to start her a new medication. Our options were between 2 different medications. The first option was an anti-rejection medication that's side effects included excess hair growth all over the body which would require shaving, including her face and could also cause her gums to enlarge and need to be surgically decreased. Her other option which we chose was Tacrolimus. It's main side effect which is fairly rare is diabetes. It is basically a medication that helps stop her body from attacking her kidney and is thus used mainly for patients who have transplants as an effort to help prevent the body from rejecting the "foreign" organ. It's a medication that she will need for life since her body will always want to fight her kidneys. I will say that getting this medication covered by insurance was not easy by any means. It's a very expensive medication and there is not a generic option from my understanding. Literally numerous calls to numerous insurance people led me to feel frustrated and yet grateful that this was really our only major insurance issue. To think about all the people who don't have insurance or have poor coverage really made me try to be patient and thankful for what we have. At that same appointment we learned that there will likely come a day when her kidneys no longer function the way they need to and she will need a transplant. The doctor explained that some people with FSGS carry a mutation in their blood which if present, significantly decreases the persons chances of dealing with FSGS after a transplant. Those who do not carry the mutation will almost immediately start spilling protein related to FSGS on the operating table right after a transplant. It's an expensive test to have done to find out if she carries the mutation, so it is typically not done until a transplant is needed. So, we pray she has this mutation, but most of all we pray her kidneys she has now will be strong enough for many, many, many years to come. That day, we also started her on a blood pressure medication because her blood pressure was 140's/90's. Luckily she needed that only temporarily. After nearly 13 months, Brinlee was finally weaned from Prednisone. She still may need it for short term (like a week) when her kidneys have relapses, but not months, like before.
I truly did not realize how puffy the steroids had made her until looking back at the pictures now. I knew she was puffy and had the typical "moon face" that steroids cause, but never really thought it was to the extent that it actually was. On a very humid, hot 4th of July family reunion, one of my great aunts looked at Brinlee and exclaimed in an obvious tone, "well she's healthy!" I calmly explained that she was actually quite sick and her puffy face was from the steroids she was on.
She turned 3 yo last month and it's amazing how far she has come. She is too smart, spunky, naughty, funny, sweet, and confident! I love her to pieces and am sooo very grateful for all of the people who care about her and pray for her.
