Finally at home...

I just counted how many days Karly was in the hospital since Feb. 28...a total of 21 days 15 which were in the ICU. This wasn't much fun from anyone's perspective especially a 19 yr. old. However, Karly was so happy to be able to participate in the Campus Crusade Beach Evangelism. This brings a smile to her face amidst a long, tedious fight for survival. Whenever you get a chance, please ask.

Karly was presented in a large cardiology meeting on March 20. Although there remains to be many opinions about what is best for Karly, it was determined to stabilize her and get her to a heart and kidney specialist in Indiana. We had our one contact from Indiana who highly recommended the advanced heart failure specialist and vascular interventional radiologist specialist that we made contact with. They seemed willing to work with NIH and us in order to get Karly in the best shape we can and God-willing get her ready for the transplant.

The family (which includes Zach) drove Friday night and arrived Saturday morning to NIH. Karly wasn't having a very good day, but she was cheered up and smiley when they arrived. The plan became for Karly and I to fly directly from the NIH ICU to the Cardiac ICU in Methodist on Tuesday morning. The family made the 9 hour drive back to Indiana on Tuesday. Things moved rather quickly when we arrived to Methodist, and I was so grateful for how well the Methodist team received and worked on Karly. Wednesday morning, the vascular surgeon mapped her arteries and determined that ballooning both renal arteries would be best. He was able to successfully balloon both of her 90% blocked renal arteries and immediately got good blood flow into the kidneys. We have seen her BP(with the help of 3 blood pressure medications) hold at a nice pressure. Before, no matter how many drugs we were using her blood pressure wouldn't come down. Her creatinine has come down, and will hopefully keep coming down to a normal level. Her heart should be happier now, and we shouldn't see a worsening. We would love to see an improved heart over time. While all this is great news, it just means she is currently stable. We hope to see an improvement, so that she will qualify for her haplo-transplant. But, really what we hope for is a miraculous healing. No one is very excited about exposing her heart to more chemotherapy or her kidney to toxic drugs. Lord Come!

Now, let me tell you about all the exciting things we saw God provide. I will say that Karly and I were pretty nervous about leaving NIH. We have not had good experiences at other hospitals. When NIH wanted to send us some place else, we were expressively nervous, yet at the same time, gave it all to God. The Bible reading that day, said to "Trust Me and Don't Be Afraid" Okay, that is what we did. Sunday's reading was reminding me about my God of intricate details and overflowing abundance. I had no details, but said okay God has the details in the palm of His hand. The day before we are to leave our place of comfort, the reading was, "This is a time in your life when you must let go." Another reminder of what we do so often, but reminders help me to focus again. As we wait on our flight with 3 hours of delays, I'm reminded that delays are for a reason. Keep trusting. Once on our flight, Karly's tear duct tube all but comes out just as we are strapped in to take off. I'm not allowed out of my seat to find a mirror. I ask if she can just close her eyes and wait only to get no response which I know what that means. I lean over and ask two ladies sitting on the aisles across from me if they have a compact mirror and one does. They ask if everything is okay, because Karly looks visibly slumped over and in discomfort. I tell them a little of what is going on. They share with me that they are both nurses (not traveling together), but willing to help if needed. That is a relief. One of the nurses has just been speaking to Senators and Representatives to increase the funding at the NIH. Now isn't that neat! She also knew a friend of ours who just graduated from the School of Nursing at IWU. Isn't that neat! We finally get to Methodist and everyone is prepared for us...take us straight to her room and hook her up. We immediately are speaking to a doctor and getting a sense of what is going to happen. I had no idea that she was going to have the balloon/stent procedure done on Wed. morning, but knew that it was a possibility. We were just thrust into a whirlwind, but knew God was in control. I loved all the doctors and nurses we encountered...and that is saying something. I was pretty nervous about the procedure, but again God brought her safely out of it. While in the waiting room, a man gave me some insight about parking and staying on the campus. I asked where he was from only to find out that he was from a very small town that my grandpa and dad were from....unusual! This was timely because I had asked to speak to a social worker later that day, but didn't know what I really was after. Meeting this man helped me clarify what I was after. I met with the social worker that afternoon. She started by asking what kind of person Karly is. You cannot describe Karly without speaking about her faith. Immediately, the social worker says I knew it, "When I was in her room, I felt the Spirit of God." I described the trip that she had taken. Again the social worker is getting goose bumps. She had gone on a CRU beach evangelism trip in the 70's very similar to Karly's trip. They were able to share this experience. The social worker was able to get our family a room to stay in for as long as we needed. Fortunately, that was only 2 nights, but we were able to be all together. A friend of ours works at Methodist and got us lunch one day...what a treat! We had encounters with several Christian nurses building blocks of friendship and faith. We did not want for anything. God worked out ALL the details. I had told our people at NIH how nervous I was about leaving them, but was able to share all that God had done.

Karly is now at home. She is stable. Her BP is the best we've seen in years. We have an excellent team of doctors in Indiana now as well as in Maryland. We see her heart specialist every week at Methodist. We plan to travel to NIH on Sunday to get her Immunoglobulins replenished and repeat an ECHO. What marvelous news it would be to see her heart function return. Either way, we know that God has got this. Whatever His plan is for her, we are all in. Trusting God is the only way. May you find your complete trust in God also!

Love,
Mom

Karly keeps us on our toes...

Since my last post on February 27, a lot has happened. On Feb. 28, she went for a test called Lower Extremity Arterial Doppler(by the way that was normal). After lying down 30 minutes for the test, she sat up to leave and noticed that it was difficult to tie her shoes, walking to the car, etc. She took a couple breathing treatments at home with no improvement. We called our NIH doctor describing the symptoms, and she sent us to the ER. In the ER, she was put on oxygen, and eventually a CT was done. She had 1 liter fluid around and in her lungs, with an infiltrate present in her lung. They determined that this could resolve on its own and were going to send us home. Our NIH doctor wanted her observed overnight, so we stayed. She was started on some lasix to remove the fluid. Our NIH doctor wanted an ECHO, but BMH wouldn't do an ECHO. After thinking about what could be causing Karly's symptoms and BMH not thinking this was anything beyond an infection, our NIH doc decided she should fly to NIH. She seemed improved, enough so that on Sunday morning, March 2, we could fly to NIH. We are thankful that the fluid was coming off nicely on lasix. After arriving at NIH, a follow up chest x-ray showed there was still some fluid but it was coming off. An immediate ECHO showed she had Congestive Heart Failure. Her Ejection Fraction(EF) had dropped from 50% on January 1, to 35% on March 2. Again, we are so grateful for our team of doctors here for getting to the issues quickly. Our goal that week was to make sure the fluid was off, adjust her meds to control her BP better, and get her to Panama City Beach, Florida with Campus Crusade for Christ. Meanwhile, we were waiting on some tests to determine a possible cause, what role her existing renal insufficiency is playing, and what do we do next.

Panama City Beach was an awesome experience for her. She learned more about God, made awesome friends, and I'm quite sure God used this trip and Karly's story in the lives of many. She had a very difficult time breathing. She couldn't go to some things, had to stop and rest a lot, and learn to cope with breathing difficulties. By the end of the week, she was pretty shot. She was flown directly from PCB to NIH. After assessing her, she was sent to the ICU. Her Ejection Fraction(EF) had dropped to 15-20% in 8 days. Nobody expected that. It is quite curious as to why this is happening. We only have speculation and are still looking for a cause. Meanwhile, this is a long haul. Her fluid overload is HUGE. Her kidney function is severe. Her prognosis unknown. Here comes a big BUT....

On Monday evening the ICU attending told me that one number they had seen earlier was back to perfect. He was rather impressed because this was unexpected. On Tuesday, they put in a Swan-Ganz Catheter which goes through her right atrium, her right ventricle, and into the pulmonary artery. This gives them precise numbers, so they can adjust medications and removal of fluid better. While the fluid level was more than they thought, the heart was better than they thought. In fact, an ECHO was done after the Swan went in, and the EF had come back to 33%. This got a "WOW" from the ICU attending. He said clearly he doesn't understand Karly and this situation. She has also been able to reduce the amount of oxygen she needs. She is still bed-ridden, sore, in pain from severe headaches(probably from high BP and BP medication), and really very sick. We don't know much beyond day to day right now. We do know that her numbers are trending in the right direction. Praise God! I was reminded today to read Psalm 139:

¹ You have searched me, Lord,
    and you know me.
² You know when I sit and when I rise;
    you perceive my thoughts from afar.
³ You discern my going out and my lying down;
    you are familiar with all my ways.
⁴ Before a word is on my tongue
    you, Lord, know it completely.
⁵ You hem me in behind and before,
    and you lay your hand upon me.
⁶ Such knowledge is too wonderful for me,
    too lofty for me to attain.

⁷ Where can I go from your Spirit?
    Where can I flee from your presence?
⁸ If I go up to the heavens, you are there;
    if I make my bed in the depths, you are there.
⁹ If I rise on the wings of the dawn,
    if I settle on the far side of the sea,
¹⁰ even there your hand will guide me,
    your right hand will hold me fast.
¹¹ If I say, “Surely the darkness will hide me
    and the light become night around me,”
¹² even the darkness will not be dark to you;
    the night will shine like the day,
    for darkness is as light to you.

¹³ For you created my inmost being;
    you knit me together in my mother’s womb.
¹⁴ I praise you because I am fearfully and wonderfully made;
    your works are wonderful,
    I know that full well.
¹⁵ My frame was not hidden from you
    when I was made in the secret place,
    when I was woven together in the depths of the earth.
¹⁶ Your eyes saw my unformed body;
    all the days ordained for me were written in your book
    before one of them came to be.
¹⁷ How precious to me are your thoughts, God!
    How vast is the sum of them!
¹⁸ Were I to count them,
    they would outnumber the grains of sand—
    when I awake, I am still with you.

¹⁹ If only you, God, would slay the wicked!
    Away from me, you who are bloodthirsty!
²⁰ They speak of you with evil intent;
    your adversaries misuse your name.
²¹ Do I not hate those who hate you, Lord,
    and abhor those who are in rebellion against you?
²² I have nothing but hatred for them;
    I count them my enemies.
²³ Search me, God, and know my heart;
    test me and know my anxious thoughts.
²⁴ See if there is any offensive way in me,
    and lead me in the way everlasting.

God knows what is happening; we intercede for her; we trust God.  He is good.
He is trustworthy. Thank you for remembering us in prayer.

Love,

Mom

Another update for Karly

Since the last update on Karly a few things have happened. We went to IU Health to see a Vascular Interventional Radiologist. We really like him, and he instantly seemed bonded to our story. He said to consider him a part of our team. What a blessing! He believes she has something rare called Mid-Aortic Syndrome. Even if this is not the correct terminology, we are in pursuit of finding out more of what is happening in her body. She has several arteries and vessels that are severe to critical in narrowing, with some calcified areas as well. Everyone has come to the same conclusion about what to do, and all have decided that stenting wouldn't fix the renals arteries. Because this is more widespread, we have started on the dreaded steroids. For a Dock8 patient, this means that her immune system will be even more immune-suppressed which also means the viruses will be able to wreak havoc on her. What we are praying for is that God would protect her from this and that the steroid would fix all the vessels as soon as it can, so she can come off of it. We hope for her creatinine to come down. It has increased again a lot over the last month. After being on the steroid for a week, we will test the creatinine again to see if it has come down which means the steroid is working. In theory, if the creatinine comes down, then the blood pressure will come down.(Double-edged sword because the steroid can increase the blood pressure and cause swelling). So far, the blood pressure has been okay, with only a little noticeable swelling today in her hands. Also, if the creatinine comes down, she can get the CTA to look at the vessels and arteries around her heart. Right now, the dye used to look at this is a problem, but hopefully it will be okay after the creatinine comes down. These are just some of the things we are trying to achieve in the next month or so. We just keep track of one day at a time.
Thanks for praying with us!
Love,
Mom

3 years later....

It's very hard for me to believe that it's been 3 years since Kelsey passed away. I still remember it like yesterday. The girls think it seems forever ago. What different perspectives. Recently I had them write an essay. The theme was, "Do not sorrow, for the joy of the Lord is your strength." It allowed them to reflect. I loved reading their thoughts.They talked about why it is important to be filled with the joy that we receive through Christ saying, "Living in joy is to our benefit; it makes us stronger and gives God glory. We learn to give comfort when we have received comfort. We give joy when we attain joy. We learn patient endurance when we suffer. God wants us to bring Him glory through our sufferings. God knows about all these things. God is making us perfect through a process of refinement. The next time you face trials trust Him completely. Consider it pure joy that you face the trial, for that very trial will be used by God to develop your perseverance toward maturity." By reflecting, they had time to think about what is facing them next and what response they would have.  You always have a choice...either draw near to God or turn away. I was grateful that their response was drawing nearer to God.  

So what is next? We feel as though it is time to choose the haploid-transplant. Kassidy was tested last month. She does not carry the EBV virus which is pretty essential in Karly's donor. I am currently being tested. Tyler gets tested on Thursday. Trent is going with Karly in 3 weeks to be tested. While I may have all that is needed, I'm not ideal because I have older cells. At this point, we need Tyler or Trent to have all that is needed to be Karly's 1/2 match. Please pray with us. We continue to walk through the doors that become open. We pray that God makes every step clear to us. At the same time we ask that this cup pass us by. BUT in all things we pray for His will to be done and that we glorify in all that we do.

Love,
Mom

Update on Karly

We just returned home from NIH Tuesday evening. She had the endoscopy done Monday; Praise God; it looks better. This means she will not have to take more drugs to get the GI system better. She will still have a risk of internal bleeding, but it is now reduced. It is good enough that she can now move forward to getting both renal arteries stented. This has not been totally confirmed, just the majority consensus. The creatinine is hanging out, and hasn't increased since January 1. Pray it stays put or better yet, improves. Her MRI of the heart was inconclusive, because they couldn't use contrast. At least, at this point, they didn't see anything different than the last time they did it. So for us, this was an upbeat visit. All good news! She will be in another protocol next visit. This is done to study the blood vessels and arteries. It sounds very interesting. They will take a patch of skin from her arm. They can then use it to create blood vessels and cells to study. This could shed some light as to why this is happening to her arteries. Hope they can figure this out. We have not had our consultation with Washington Hospital Center...the doctor was in the Philippines(I think). Anyway, we are hoping to get that in next visit. We are still looking in Indiana also. In the meantime, we have met with the transplant team again. Here's what we know....

It is believed that the best chance for Karly's survival is the haploid-transplant. Karly has decided with much courage and faith that she should be moving in that direction now. With that in mind, Kassidy went with us to give her blood for a transplant work-up. It is imperative that she have the EBV positive antigen antibody. There are several others virus' that we would love for her to have had, so pray that we find these antibodies present in her. If Kassidy is EBV negative, then we may look into Tyler or Trent, maybe me, but they prefer younger cells. Kassidy had the best points of match out of all of us, so it would be best if she has these antibodies. Anybody out there with mono that we can infect Kassidy with...only half-joking. We still pray that God would close doors that we may try to walk through, and swing others wide open with a spotlight shining. It has been rather clear up to this point, and God is certainly leading us.

Continue praying with us. In studying Hebrews, it has rested on me lately that Christ humbly, yet boldly and with confidence prayed to his Father that "the cup of suffering might be taken from Him". He did not waiver in his determination to fulfill the Father's will. The exact verse is from Matthew 26:39, "My Father, if it is possible, may this cup be taken from me. Yet not as I will, but as you will." I have found myself praying this for Karly. We trust Jesus; we trust our Father God; our Faith is strong, and will not be shaken. We continue to stand firm in this.

Love,
Mom

Karly's Story

Dancing through The Rain....

I'm am so sorry to leave you all in the dark. I was thinking that maybe it was just more of the same for you to keep hearing about. BUT, Karly has to deal with this everyday, and the prayers of the saints are needed. I went back to where I had left off. She was having difficulty getting IV's in every three weeks, so finally she had a port placed last summer. This is been a source of great relief. The timing of course was awesome resulting in its need to perform so often. She has needed IV antibiotics and fungal meds...about every 4 months since then. For about a year we have been watching her BP and Creatinine rise. Didn't quite know what to make of it. For the last 3 months, they have been rising rather rapidly. The BP med she was on wasn't working, and the extra fluids weren't helping. Dr Freeman, in her wisdom, had a MRA done of her kidneys. It shows she has Bilateral Renal Arterial Stenosis. Her Messenteric artery is blocked or narrowed, her EKG is below normal, and the internal carotid arteries near the opthalmic artery are narrowed bilaterally. She doesn't have Atherosclerosis or Fibromuscular Dysplasia(FMD) which are the two reasons this might happen. Our best guess right now it that is DOCK8 related, but unknown etiology. The ways to treat this are high dose steroids for 3-6 months, ballooning, or stenting. The steroids would suppress her immune system so much that the viruses would rage out of control. The ballooning is temporary, but would it suffice(we don't know). Stenting is the way of thinking right now. Nobody is excited about stenting a 19 yr. old because this is a permanent procedure. The next issue is the aspirin and Plavix she would need to be on after the procedure. Her GI system is fragile with severe eosinophilic disease. This could cause internal bleeding. She has been on a swallowing steroid treatment to put it directly where is needs to be which seems to have given her some relief for the eating/swallowing issue. The next step is to look inside her GI system again to see if there has been enough improvement to take these meds. If not, they would increase this steroid in preparation to take the meds. We will be visiting a large hospital(Washington Hospital Center) in Maryland next visit to speak with their team about the stenting surgery. Our team at NIH wants to be nearby to keep their eyes on our girl. I have not given up hope that it could be done in Indiana. How will we know what to do or where to do it? My prayer is that God would slam the doors shut as we try to walk through them. We don't have the luxury of timing(at least we don't think so), so we will keep moving forward and try to discern which path we are walking. We desperately want the path that God is choosing for us...not what seems right, easy or convenient. We are trusting ultimately on God to make our path straight.

It is becoming more obvious that moving into a transplant will be sooner now than later. The two options remain the same: Haploid-Transplant(using the mostly closely matched sibling) and the 9/10 unrelated mismatched in a very bad place( having to do with Graft Versus Host Disease) with survival less likely. At this point, we are feeling better about the Haploid-transplant, but there are still unknowns to be thought out. This type of transplant has not been done in the United States for this disease. It has been done at NIH for another immunodeficiency disease with success to date about 67%(roughly 2 out of 3). Johns Hopkins likes these types of transplant for cancer although it has its share of relapse. We wouldn't have to worry about relapse, but rejection and viral reactivation. I know this sounding overwhelming, but we are taking one step at a time without getting ahead of ourselves. We come to Our Lord Jesus to rest in His presence and experience His peace.

 People ask me how she is doing. It is hard for her to answer, and it is hard for me also. It isn't a quick answer. I was remembering this the other day....Kelsey used to answer, "I'm living." Karly showed me a reading that seemed perfect to her the other day. It is from Jesus Today, by Sarah Young. "TRUST ME IN TIMES OF CONFUSION-WHEN things don't make sense and nothing you do seems to help. This type of trust delights Me, because I know it is real. Invite Me to enter into your struggles-to be ever so close to you.Though other people may not really understand what you're going through, I understand perfectly. Find comfort in knowing you're not alone in your struggles. I am with you, watching over you continually. Long-term trials can drain you of energy and hope, making it hard for you to keep trusting Me. But, I have given you a wonderful Helper, the Holy Spirit, who never runs out of strength. You can ask for His help, praying: "I trust You, Jesus; help me, Holy Spirit." Instead of trying to resolve all your problems, simply rest in My Presence. Trust that there is a way forward, even though you can't see it. I am providing a good way for you, though it is bumpy at times. When the road is rough, cling all the more tightly to Me. As your soul clings to Me, My right hand upholds you.
"I am with you and will watch over you wherever you go, and I will bring you back to this land. I will not leave you until I have done what I have promised you." Genesis 28:15
"When the Helper comes, whom I shall send to you from the Father, the Spirit of truth who proceeds from the Father, He will testify of Me." John 15:26
"My soul clings to you; your right hand upholds me. Psalm 63:8

Thank you for praying with us!
Love,
Mom