There is no evidence of histoplasmosis in Ryman’s bone marrow so far!!! Some of the cultures take a while but hematology delivered it as good news! Not only is it not present but they saw tons if new red blood cells being made. So what ever was going on last week is straightening out! We are so so grateful for this news. So the plan as of now is to continue IV antibiotics and hopefully watch her get better! We did have a rough night and start to today. She is having digestion issues and an xray showed lots of air/inflammation in the bowels. If things do not clear up in the next few days they may do a scope to investigate if there is anything going on. She is eating great and was being a little silly and laughing tonight which makes us so happy. Thank you so much for the continued prayers!
Not only are we on hospital time but we are on hospital holiday week time. Things move slow in a hospital setting when you are just hanging out and getting antibiotics. Its a good thing though, things are calm today. Ryman got to stop the Nupragen shots the night before last and she actually walked a little bit yesterday. They think she was having some bone pain. So no bone scan for the time being. We still haven’t heard anything from the biopsy and spinal tap. Hopefully some news tomorrow. We did have some really good labs this morning that showed Ryman making lots of reticulocytes, which are immature red blood cells. So she is making blood again! Such a good sign. Hopefully no more transfusions will be needed. The feeding tube and nutrition seems to be helping move things along. We did find out that the granulomas they found in the liver biopsy that are due to histoplasmosis were present in the biopsy from July as well. She just wasn’t having any of the symptoms to think anything of it since rejection was present. We then immune suppressed her like crazy due to the rejection which gave way for the fungal infection to flourish. The doctors said it is a really slow progressing infection so she has probably had it for a long time. We also found out that Ryman will remain on just a detectable amount of immune suppression medication for as long as possible. It could be 6 months, 1 year or forever. This is great for her being able to not get sick as much but bad for her if her immune system decided to fight her liver. So we are praying that we will be able to balance the histoplasmosis medication and the immune suppression enough to not reject the liver but be able to fight the infection. She will be on oral antibiotics when we go home and will remain on them for 1 year or more. I feel like Ryman is improving but it will be a slow process. At least seven more days of IV antibiotics inpatient is the plan as of now.
I also just want to say that the support and prayers are so appreciated. The coffee, food, balloons, stuffed animals, toys, humidifier, magazines, house work and company are more than we could ask for. We are so thankful tonight for the friends and family we have. The prayers and love are simply something we will never be able to express the power of through words. You all make this journey one that we can travel in joy regardless of the circumstances. I was reminded today of how close we truly were to loosing Ryman last year. Yet during the storm, Jeremy and I never felt the burden. You all carried that for us, there is really no other way to explain it. One of our liver family’s lost their little girl today. She also had CPS-1 and was a little over a year old. I sat today and wondered why these babies have to go so young. Why do others go and some stay. Some go and save others in doing so. It all seems so crazy to me. We live in a fallen wold and I know we will never understand the whys of death. Proverbs 16:9 says,” In heart a man plans his course, but The Lord determines his steps.” We have no control of how many steps we will have. I have thought a lot lately about the saying, live each day as if it were your last. How amazing would the world be if we actually did that. I’m not sure if that is even possible for the human race. We have so many distractions that stand in our way. To wake up and know, this is my last day to be here with my family and friends. How different I would live that day. But our minds and our ability to live life in the present makes that so hard. I would spend way less time on my phone and more time playing with Ryman and expressing my love to Jeremy. Yet as I think about it I almost want to cry, would I just sit and hold them both all day? I think its hard with life full of material things to stop and remember that we only need him. Things really are that simple yet we live in such a time that it is so hard to keep focus on that.
Even if you can’t live the entire day as if it were your last, then let’s try to live at least one situation or moment as so. Even if its just telling someone how much you appreciate them and love them. Love you all, Vital check time so I’m going to go give Ryman a big hug and kiss!
I’ll leave you with some of those moments captured by an amazing photographer a couple months ago. I’ll have to share her story later. Thank you Tara Day for these reminders of how we should spend each day. Joyful!
Ryman had her bone marrow biopsy and the lumbar puncture yesterday.
Her spinal fluid was clear and had no red or white blood cells so that is a good sign. We will not know about bone marrow for a couple days but might get a prelim report before then. They said they would be surprised of it wasn’t in her bone marrow so we expect to see it there. Otherwise we start looking for another reason she isn’t making new red blood cells. The MRI did have a few spots but not from the infection. They think its possibly from pre CPS1 stuff or fat that develops around nerves until age 2 but they do not have scans here to compare too. I think we did one at Georgetown so they are sending it to Dr. Yazigi to compare if so. But no infection there so for this treatment that is a really good thing. She got an NG tube yesterday to start extra food (Boost) just to catch up on nutrition. She and Jeremy hate the tube but I think its a good thing for now. Her albumin is low causing some fluid in her abdomen and lack of consistent nutrition (NPO for the past there days on and off) has added to her mobility issues so her stomach is so swollen. She has been really lethargic for days now and had me really worried. She seemed a little more responsive last night but is back to being weak and sleepy this morning. I pray that it’s just exhaustion from fighting the infection. Still no fevers so that is a good sign. We are probably looking at 4 weeks of IV antibiotic and 1 year of oral antibiotic therapeutic dose and then probably a couple years of a low dose antibiotic. Insane! But if that’s what will get her better than bring it on.
I am thankful today that Ryman is still here with me and that I get to be by her side each day. Sometimes its hard to be thankful when she is so sick but I know that it is a blessing to get each day with her regardless of the circumstances.
My devotional said “Thankfulness is not some sort of magic formula; it is the language of Love, which enables you to communicate intimately with Me. A thankful mind-set does not entail a denial of reality with its plethora of problems. Instead, it rejoices in Me, your Savior, in the midst of trials and tribulations. I am your refuge and strength, an ever-present and well-proved help in trouble.”
He definitely is a well-proved help in trouble. Teaching me daily to lean and trust.
Ryman is in Day 6 of the antibiotic treatment and hasn’t improved so they are doing some more test today just to make sure they aren’t missing anything. She was able to get a PICC line placed so we are thankful for that and hasn’t had to get anymore sticks. Well except the shot she has to get in her thigh every night. It’s a medication that helps boost your white blood count. She had a CT scan, X-ray and ultrasound yesterday. They all looked okay, the GI doctor said he saw a few areas of non specific stuff that they think is just from the infection and she has a small pocket of fluid in her abdomen so we will keep and eye on those. They even checked her ammonia yesterday because she was so malaise. It was completely normal. They will do some additional test tomorrow morning on her liver to make sure its doing its job. Today is going to be a big one though. She is scheduled for a brain and abdomen MRI, a bone marrow biopsy and a spinal tap at around 2 or 2:30 today. We would appreciate prayers that these go smoothly and that Ryman isn’t in too much pain after the procedure. Also for good results. Histoplasmosis can go to your bone marrow and can effect your central nervous system or cause brain lesions. So we want to rule those out. The scans will allow them to know if the infection has settled anywhere and they can focus on site treatment if it has. Thank you all for the love and the suppose during this rough time. We love you all!
Ryman is currently getting a PICC line. The IV has worked great but for the 2 weeks of IV antibiotics she will need the lines needs to be a more long term stable one. She had a rough night on Saturday. She kept spiking a fever every 6 hours and seemed to feel worse. Her blood count continued to drop so she had another transfusion last night. It is likely that all of these symptoms are from the Histoplasmosis. She got her second dose of the IV antibiotic last night and her fever spread to 10 hours. She slept great last night and hasn’t had a fever since 4pm. However her sugar was 306 this morning, so not sure what that is about. Potentially from all the IV fluids she received over night. They were going to recheck it while she is out for the PICC. I got to hold her as they pushed the medication into ghee IV to make her fall asleep. Its crazy how fast that stuff works. Her eyes rolled back and she was out. Hopefully its a good nap. There were talks of Ryman being flown back to Georgetown today if she did not improve and the fevers persisted. We are hopefully that she will feel much better today and that we will be able to stay her for treatment but we are willing to do anything we need to get her better. We should know more later today and I will update. Prayers that this antibiotic is doing its job. She also got a shot in her thigh last night of a medication that is suppose to help boost her bodies ability to make white blood cells. Prayers that it works and she can fight this thing fast.
We are back at the hospital. The team called last night and said that one of Ryman’s urine culture came back positive for histoplasmosis. Wondering what that is:
Histoplasmosis is an infection caused by a fungus called Histoplasma. The fungus lives in the environment, particularly in soil that contains large amounts of bird or bat droppings. In the United States, Histoplasma mainly lives in the central and eastern states, especially areas around the Ohio and Mississippi River valleys. The fungus also lives in parts of Central and South America, Africa, Asia, and Australia.
People can get histoplasmosis after breathing in the microscopic fungal spores from the air. Although most people who breathe in the spores don’t get sick, those who do may have a fever, cough, and fatigue. Many people who get histoplasmosis will get better on their own without medication, but in some people, such as those who have weakened immune systems, the infection can become severe. Crazy, right? Even crazier that we figured it out.
We were suppose to get admitted tomorrow night and start treatment on Monday but Ryman woke with a 103 fever. We are suppose to bring her in if she has even a fever of 100.3 under the arm. So 103 and we are getting to the hospital fast. We are waiting for our room on the floor but we got an IV in on first try! Thank goodness Chris was here, he is our favorite IV therapy guy. He is usually a night shift guy so the fact that he was here is so amazing. PICC team isn’t in on the weekends so we though we would have a really traumatic time getting an IV stared today. They did a chest X-Ray and it looked clear. Did labs and blood culture and just finished the catheter for a urine culture. We will most likely do a few days of regular antibiotic BUT we will start amphotericin (iv med for the histo). Once better after a few days, we can switch to an oral medication called voriconazole. Per Dr. yazigi her Georgetown doctor. We are looking at a week inpatient at least. Histoplasmosis is endemic to this area and our doctors see it in immune compromised patients a lot. So its totally treatable and we are just so thankful that we have something to actually treat. There are still lots of other labs pending but as of now we will treat this. Her blood count came back at a 7.9 so she may end up getting transfused today. We will be on our downtown apartment #8302 if anyone wants to visit. If you are healthy of course ;). We will be lowering her prograf from a goal of 8 to 2 or 3. So big time prayers that this allows her to fight this and not reject in the meantime.
“I have told you these things, so that in me you may have peace. In this world you will have trouble. But take heart! I have overcome the world.”
It was a long day of waiting around yesterday but we are home tonight! Tuesday night was really rough, Ryman only slept 4 hours and not consistently. It was lots of screaming and obvious discomfort. It’s really tough when there are so many variables and she can’t tell us what is wrong. Whether it was a headache from the medication wearing off, pain from the procedure or the fact that she just really needed to use the bathroom she was definitely not happy. The night and day nurses had to listen to the screaming too. On Wednesday they finally gave her some Tylenol on her way to have a chest and abdomen X-Ray. After both of those and an ultrasound to look for bleeding from the procedure she was out. Napped for 3 whole hours and I made doctors talk to me in the dark in order to not wake her up. The X-Rays and ultrasounds all looked great. We had one set of labs from her IV that came back with a really low blood count again. They called in the PICC team to use an ultrasound to guide the needle and get a new sample without using the IV. So about the 15th stick since last Monday. The second sample was much better and her blood count hadn’t changed much since the day before. That was a very good sign that things weren’t getting worse. Her liver labs looked a little better too. After the prelim report identified no rejection they stopped her Cellcept medication and lowered her prograf immunosuppression medication. We are hoping this will allow her body to fight whatever the culprit is. We were consulted by Infectious disease and hematology. Around this time last year we were inpatient for some digestive issues Ryman was having. They did a scan then to eliminate Post Transplant Lymphoma Disease. Its a form of lymphoma that is a rare side effect of the immune suppression drugs that transplant patients take for life. It is a side effect in the sense that their bodies can not fight the cells that can turn into lymphoma. PTLD can cause bone marrow suppression and since that is one of the symptoms that Ryman is having they wanted to consult Hematology to rule it out. Cellcept can also cause bone marrow suppression. Viral or infection can also cause the same thing to happen. The biopsy did show granulomas which are common in infection or viral cases. So in the end we got to come home today after taking lots of blood from Rye to send out and check for lots of different viral, fungal and bacterial infections. She will have a TB skin test done on Tuesday when we go back for labs. It’s still up in the air about doing a bone marrow aspiration but it can be done as an outpatient patient so they let us check out of our downtown alarm ermine. We may never know the cause of this but as long as she keeps improving and she can fight it without fighting her liver we are fine not knowing. It was a long week but our girl is sleeping peacefully in her crib and mom and dad are enjoying not having to be the crazy parents who insist that no one is allowed to stick their daughter unless they are part of the PICC team and have an ultrasound machine.