Sunday, July 31, 2016

2 Years Old

We just spent Karly's 2nd birthday at the NIH in Bethesda, MD. She received Tyler's life-saving bone marrow 2 years ago on July 25, 2014. That time was full of the unknown, yet we were at peace following God's plan to proceed with this transplant. Little did we know all the benefits that would come of it. There have been many DOCK8 Haplo-transplants since then...mostly with a fantastic outcome. We remember those that didn't make it through their transplant. It saddens us. We start saying, "if only," but quickly dismiss it because it is and always has been in God's hands. His timing is perfect. We stand in awe of Karly's life since the transplant. What a transformation! We've said it before and say it again, "She went from dying to thriving!"

It was an extremely packed week full of appointments and meeting with old and new friends. We feel so blessed every time we meet a new dock8 family. It feels good to be able to share that bond with those that really "get it" and "live it." For Karly, it's a rare opportunity when she can talk with someone who can even remotely begin to understand what a life of terminal/chronic disease is. She would probably say that is her favorite thing about going back to NIH.We get questions like, "What is the worst thing about the haplo-bone marrow transplant," "When do you lose your hair," "When did you go outpatient," and "Where are the best places to go during your outpatient stay?" We pray for them to make it through the transplant and talk of hope.

Karly's tests are improving. Her kidney number, although still high, was the best it's been in 2 years. Her heart numbers are remaining steady. Her BP is great with medication. She dropped one medication for which we are thankful(we were hoping for more to be dropped). Her PFT tests showed numbers she hasn't seen since 2009. Her adrenal insufficiency has been a slow climb over the last two years, but we saw it increase  from 1-8.  The normal number is about 20, so she still has a long way to go (rats). She received 9 vaccines and did super well with all of them. She had her 4 wisdom teeth removed in the OR under general anesthesia for safety reasons. It went well.

                                                                                                                                                                                                                                                                                                                                                                                                               We had fun Wednesday night being escorted by 7 Montgomery County Policemen on motorcycles during rush hour to Toys R Us. It was so cool watching them do their job in front of us. Their was a lot of oohs and awes by the kids and parents. After the kids shopped, we were escorted to a pizza place, then back to the Inn.

The most exciting news is that Karly will be starting the Physical Therapy Assisting Program in August. It will be 2 years long, and she'll receive her Associate's Degree. She longed to do this since her cancer in 2009. When she didn't get into the program last summer, she had to be patient and wait till this year to try again. She re-took the one class she had a "B'" in and received an "A". God's timing is perfect, and her body is ready to withstand the rigors of the program. She is so grateful and beyond excited..which doesn't even begin to describe how she feels. We are so proud of all she has persevered through and the young woman she is. God is using her on the Ivy Tech campus, the Oneighty program, her Bible Studies, and I cannot wait to see what this next chapter brings.

I will end with Karly's life verse. It seems quite appropriate. Proverbs 3:5-6:
"Trust in the Lord with all your heart,
    and do not lean on your own understanding.
In all your ways acknowledge him,
    and he will make straight your paths."

Wednesday, May 25, 2016

2 months before the 2 yr anniversary of her experimental transplant

Hi All,
I have been remiss in keeping you all updated. Since February, Karly has been fighting some kind of infection in or around her eye. We first thought it was caused by her sinus', but after treatment of that, we saw no improvement. Then she tried an eye drop antibiotic...not much improvement. Then a different eye drop antibiotic...again no improvement. She was bleeding out the corners of her right eye, and it was worsening. Finally, we were allowed to see her eye surgeon who had placed her eye tubes prior to her transplant. He confirmed that she was infected in her lacrimal sac. He said that he needed to make a new tear duct that bypasses the old blocked one. He performed a Dacryocystorhinostomy this morning. It is the surgical procedure usually used to treat most cases of blocked tear ducts in adults and rarely in children.This technique creates a new route for tears to drain out through your nose normally again by developing a new connection between your lacrimal sac and your nose. He said she was abscessed and no amount of antibiotic would have worked, so we are grateful that she was diagnosed and now treated. We hope for a speedy, painless recovery. She can't do anything for 48 hours, then about a week of taking it easy. 
Her BP has been high since the beginning of May. Not sure why. We are in a wait and see mode...will it return to an acceptable number or stay high? If it stays high, then the most likely cause is that her renal arteries are blocked again...not what we want. A couple good things with the HBP is that the pressure pushes blood through her kidney better and makes her lasix work better. Bad things are: makes her heart work harder, makes it harder to control potassium intake and possible stenosis. 






Karly turned 22 on Monday. She reflected that she will be outliving Kelsey who died at the age of 22. It is both a sad memory and a happy one. She celebrated with her boyfriend, Zach and also her family. We are grateful for every birthday of everyone. We take nothing for granted. We hope you count the cost of life everyday. God has a plan for YOU. Don't waste it. Living life centered on God's purpose for us is amazing. Karly has had an amazingly, God-awed year. Ask her about it sometime when you have time to listen. God is good all the time!

Thank you for standing strong with us throughout the years. I hope to do a better job communicating, but thanks for your patience.
Love,

Mom

Tuesday, February 2, 2016

5 years ago...Kelsey's Homegoing

This is from Kelsey's Memorial page on Be The Match.

Kelsey lived life exuberantly until her death on February 2, 2011. She lived with an undiagnosed primary immunodeficiency disease until 2009 when it was finally diagnosed at the National Institutes of Health as Dock8 Immunodeficiency Syndrome. Having spent 10 years in a research protocol at the National Institutes of Allergy and Infectious Disease, she gave more blood, skin, and cells than anyone with this disease leading to its discovery. Kelsey and her sister Karly were among the first 11 diagnosed with DOCK8 as stated in the New England Medical Journal September 23, 2009.
Her goal was to fight this disease in a rip-roaring manner in hopes of finding a cure for her younger sister, Karly, who has the same disease, and others fighting the same battle. With the knowledge at hand, she moved forward with a Double Cord Blood Stem Cell Transplant Fall 2010. It was the first transplant of its kind for DOCK8 in the world. She developed many complications which ultimately lead to her death. She fought valiantly and gave her body to the Institute to further research in this area. We never found a match for Karly, so the NIH continued to work on Karly's behalf to find an alternative for her and the other DOCK8 patients with no match. Karly became the first Haplo-Bone Marrow Transplant for DOCK8 on this side of the world on July 25, 2014. It was textbook if a textbook had already been written. Her case was writing the textbook. Other Haplo Transplants have occurred since then. We continue to educate people about the National Donor Marrow Program and the ease to join the registry. Our story is told more fully on www.kjkdancingthroughtherain.blogspot.com and www.dock8connection.org.

Kassidy's memories of Kelsey: She was very nice to me and very good at dancing. I remember her teaching me dance. She was outgoing. I wanted to be outgoing like she was. She was very friendly and could make friends with anybody. She wasn't afraid to express how she felt and what she believed in. Kassidy was 9 when Kelsey went home.

Konner's memories of Kelsey: I used to walk into room and find her reading her Bible. She came into my room at night and asked if I had any questions about what I was reading. She encouraged me to dance and learn the splits. One day we did the splits together. Whenever I'm at tap and not striving to do my best, I think about how she would do it and that pushes me on. When I'm not at home, I remember to be nice to people because Kelsey showed kindness to everyone. Remembering her life encourages me to live my life to the fullest. Konner was 11 when Kelsey went home. 

Karly's memories of Kelsey: When you sat by her, she had the tendency to pinch you with her toes, then tickle you. She helped me at the hospital to be brave. We would share a room, so when the phlebotomist came in she would stand behind him and make faces when it was my turn to take my mind off it. She displayed courage, bravery and how to overcome so then I would try to be like her. She seemed perfect to me. She found joy amidst everything. She had the loudest laugh in the room. She was dependable. She knew what to say and how to say it. Karly was 16 when Kelsey went home.

Trent's memories of Kelsey: Cupcakes! Haven't had one as good as hers since then. Trent was 18 when Kelsey went home.

Tyler's memories of Kelsey:She taught me to like rock and not be afraid of people with tatoos. I loved hanging out in her apartment when we were in college. P.O.D- Goodbye for Now. https://youtu.be/plGETDmXw5g
This song reminds me of Kels. Tyler was 20 when Kelsey went home.



Life with certainty is faith and trust in our God. We didn't choose to live life without her, so there is that sadness that will remain, but confidence....God knows exactly what is next. He knew then, and He knows now.
Love,
Mom






18 months post-transplant

Praising Jesus that her transplant has gone so well! She is still thriving! Her BP continues to stay steady. We have seen her Indiana nephrologist, interventional radiologist, and cardiologist. We have made some changes and as usual it is the balance between heart and kidney happiness. Karly makes daily changes depending on what her body is doing. We leave for the next round of NIH visits on February 24. We haven't received a schedule, but it should be more of the usual. We look forward to seeing our NIH friends and other DOCK8 families.

God faithfully leads us day by day. We still pray for whatever steps God wants us to take each day. I guess it is fair to say that we are quite flexible and able to change a direction quickly. Karly posted some thoughts, and I wanted to share them here in case you hadn't seen them yet.

My thoughts on 1 Corinthians 12...
KARLY J. KOCH·WEDNESDAY, JANUARY 6, 2016
 SO I was just reading these verses in 1 Corinthians and felt inspired to share my thoughts...
Unity and Diversity in the Body
12 Just as a body, though one, has many parts, but all its many parts form one body, so it is with Christ. 13 For we were all baptized by[c] one Spirit so as to form one body—whether Jews or Gentiles, slave or free—and we were all given the one Spirit to drink. 14 Even so the body is not made up of one part but of many.
15 Now if the foot should say, “Because I am not a hand, I do not belong to the body,” it would not for that reason stop being part of the body. 16 And if the ear should say, “Because I am not an eye, I do not belong to the body,” it would not for that reason stop being part of the body. 17 If the whole body were an eye, where would the sense of hearing be? If the whole body were an ear, where would the sense of smell be? 18 But in fact God has placed the parts in the body, every one of them, just as he wanted them to be. 19 If they were all one part, where would the body be? 20 As it is, there are many parts, but one body.
21 The eye cannot say to the hand, “I don’t need you!” And the head cannot say to the feet, “I don’t need you!” 22 On the contrary, those parts of the body that seem to be weaker are indispensable, 23 and the parts that we think are less honorable we treat with special honor. And the parts that are unpresentable are treated with special modesty, 24 while our presentable parts need no special treatment. But God has put the body together, giving greater honor to the parts that lacked it, 25 so that there should be no division in the body, but that its parts should have equal concern for each other. 26 If one part suffers, every part suffers with it; if one part is honored, every part rejoices with it.
27 Now you are the body of Christ, and each one of you is a part of it. 28 And God has placed in the church first of all apostles, second prophets, third teachers, then miracles, then gifts of healing, of helping, of guidance, and of different kinds of tongues. 29 Are all apostles? Are all prophets? Are all teachers? Do all work miracles? 30 Do all have gifts of healing? Do all speak in tongues[d]? Do all interpret? 31 Now eagerly desire the greater gifts.
1 Corinthians 12:12-31

I find it so amazing how God has made the body, and how He has made the Body of Christ. I just finished a semester of Anatomy & Physiology, and while it’s a hard subject to understand, I am fascinated with it! I think about how every little thing is necessary in the body because of its specific purpose. Without just one atom, cell, chromosome…etc., the body functions differently or is compromised. For example, my heart started failing when my renal arteries started to narrow and dysfunction due to DOCK8. When the heart is given resistance through narrow pathways, it feels the need to increase the flow of blood more forcefully.  As internal vessel pressure increases, the heart works harder. So the narrow resistance made my heart work entirely too hard, and eventually my heart compromised its shape to make sure it was performing to the level it needed to. The structure change caused functional difficulty which is heart failure. Even my small arteries are important for my body to function.  Another example is the production of a tiny little hormone called cortisol or hydrocortisone. Produced by the adrenal glands, this little guy has a huge job with handling the body’s stress. Being that I am adrenally insufficient (where my adrenal glands do not produce cortisol), I have to provide myself with the correct amounts of cortisol every day by taking it in pill form. I learned that stress can take over very quickly when the body doesn’t have enough cortisol to cope. I found myself in a high stress situation where I needed more cortisol, but didn’t know how much cortisol I really needed. I became very sick, debilitated, and could not do any normal day tasks. This little guy means life or death in some situations, and I am taken aback when I realize how perfectly each aspect of the body was designed. I can’t believe that such an intricately, complex design can just happen out of nowhere. What I can believe, though, is a loving Creator who spent time thinking about every little detail, thinking about all the different personalities as he laced every part together, and the tears of joy streaming down his face when his design is complete. Every single part is just as important as the other, and it is the same in the Body of Christ. Every member of the body of Christ is absolutely necessary. We were all made especially different for specific purposes. With all of our different talents, skills, and abilities, we are all being used in God’s interwoven plan to produce something bigger and better. If we were all made the same, we would have the same purpose and then only one task is getting accomplished. God loves us more than that, though. He has an individualized, thought out plan for each one of us, and He desires to use each one of our plans in His masterpiece. We get to be a part of his wondrous plan. He didn’t have to let us in on this, but He loved us so much that He couldn’t resist. How amazing is it that such a supreme, omega God would yearn for us to be in His presence, and to be used as tools in His blueprint?

Tuesday, November 17, 2015

After the bilateral renal angioplasty...

Karly's Bilateral Renal Angioplasty went so much better this time in terms of pain. The pain is what she was dreading the most. The doctor came in, and we shared how much pain she was in during the procedure in March 2014. He said he couldn't do much about it, because they needed her alert to take deep breaths at certain times... but he would try. I guess that meant she would be pretty alert. Well, Praise Jesus, all was accomplished, alert enough to take deep breaths and pain free enough to be happy about it. Her recovery went well...6 hours flat on her back...not much pain...only when they pushed on it. Her problems came later. Karly's adrenal glands don't work and haven't for years. We literally give her the adrenaline her body needs. Going into the procedure she must have an elevated dose so her body won't go into shock. Saturday went well until her body needed more adrenaline. She had a bad headache, nausea, vomiting, little appetite, was weak and very fatigued. After speaking to her NIH doctor Sunday morning, we gave her another elevated dose. She felt a little better again, but all symptoms still present. Yesterday she was still about the same. Tonight, we have seen a little break through. Now she is just taking her normal dosing. The next issue we found was her BP came down nicely, but because her body has been acclimated to the high BP, the nice new BP was causing her to feel bad, nauseous, and a whoosh in her head. She was on 4 BP medications. She stopped 2 immediately after the procedure. Her BP this morning before any medications was a little low for her...not most people. Her BP stayed down all day only taking 1 BP medication. She was so used to walking down the hallway and see her BP rise. Now, activity does not make it rise. Praise Jesus! And Praise Jesus she is only on 1 BP drug again. She finally was able to eat a meal tonight. Her body seems to be finally adjusting to the new changes.

We travel to NIH on Monday-Wednesday. She will see an Allergist, Endocrinologists, get vaccines, and get an ECHO to make sure her heart has made it through the last 3 months of high BP and strain. It will be a quick trip(at least we hope so). It will be good to see our special doctors! We always look forward to Christ-led encounters. Please pray for our trip and God's best!

Love,
Mom

Tuesday, November 10, 2015

15 months post-transplant

Karly is doing phenomenal. She is serving our Lord and Savior everyday. She is our beloved daughter. She's an amazing sister. She is Zach's girlfriend.  She is taking 2 classes. She is going to meetings that involve Ball State Cru leadership. She's helping pioneer Cru at Ivy Tech. She is leading a new Small Group at Ivy Tech and is in one at BSU. She is a survivor. She's a miracle. She is thriving! Praise the Almighty Father for the work He he has done and is doing in her life!

I am posting the most recent video explaining briefly some of the journey just before transplant and following transplant.


video


Her bilateral renal angioplasty is this Friday, Nov. 13 at 9am. Please be praying with us. Her BP is really high without any medication. She is taking 4 drugs to get it down and by morning, she starts all over again. We don't exactly know what we wish to happen, but it is to have God's best. If this angioplasty takes the BP down to normal again, we'd be quite happy. But then, is this a lifetime of angioplasty in her future? If they find that the arteries are not blocked and the cause of this high BP, then what ramifications will she be looking at? We already know that she has Stage 3 Chronic Kidney Disease at the age of 21. So you see, we really don't know what we wish for, but praying God's will be done. Thank you for praying with us!
Love,
Mom

Tuesday, September 8, 2015

1 yr post-transplant appointments

The weeklong appointments proved to be informing and interesting. It was the usual run-through of CT's, MRI's, dermatology, dental, eye, dexa scan, allergy, nephrology, pulmonary, echo, stress test, bone marrow biopsy, gynecology, cortisol stem test, fluids, ENT, 1 yr. vaccines, IVIG,  and consults with doctors. We had a few surprises; she has some chronic GVHD in two places that we were unaware of. Fortunately, it is asymptomatic most of the time. It can flare as it did in her mouth. She had sores in her mouth that looked ulcerative at one point, and I took photos of them. They eventually didn't hurt anymore, but didn't go away either. At least now, we know what they are. We can treat with an oral steroid gel when it happens again. Patients with cGVHD are at a significantly increased risk for developing oral cancer and should undergo an oral cancer screening at least once a year. Since oral cGVHD can look similar to early oral cancer, it is best to be seen by a specialist who is familiar with these conditions. Periodic biopsies of suspicious lesions may be necessary. Karly now has osteoporosis of the spine with a few other areas not that far behind. With all that she has been through with chemo, radiation, lack of calcium, steroids, this is not a surprise. The thing now is to treat it the best we can. With the lack of hormones in her body, we are adding something like a combi-patch. This will help with hormones and the osteoporosis. Some of the tests we were hoping would be improved like the arteries, ECHO, and the PFT, but they are remaining the same. This is okay. We have patience. Some were improved like her skin...she didn't have one spot of diseased skin for a biopsy....oh rats...but she let them do it anyway for research. She's just awesome like that. Her allergy testing showed she was more allergic testing positive to milk(again), and also to Timothy Grass and soy. She's also had some weird lip, tongue, cheek swelling when eating things she clearly isn't allergic to, yet, is she? Her blood work was looking very good. Her eyes are dry, but not thinking it is GVHD at this time. She failed her Cortisol Stem Test. She has adrenal insufficiency. She wears a bracelet to alert the public in the event of a trauma or stressed situation; she can go into adrenal shock as I understand it. We have an emergency dose at home and will carry one with us. She was trying to wean the hydrocortisone this past week, but going down with just the one increment caused her fatigue and bad headache and joint pain. We will try to wean next semester when she might have a break from school. Her MRI of the abdomen still shows very little flow to her kidneys. She did another renal ultrasound last week showing things are pretty much okay, at least they think so. However, since she clinically seems to be worsening, they have decided to do another bilateral renal angioplasty. We hope that this procedure will cause her blood pressure to go down and she can come back off of the 3 other drugs she's been put on. Her Cardiopulmonary Stress Test was pretty good. She has a mild aerobic impairment, she reached her maximum cardiac/cardiovascular capacity, and she has a mild diffusion impairment. All to mean her heart causes her to not go as far or as much as she would like to go. Things to pray for: the angioplasty goes smoothly, blood pressures return to normal status, come off other BP drugs, her weight will decrease, she will stop holding fluid, she doesn't get flares from GVHD, her food allergies go away, her body starts making cortisol so she doesn't have to take it anymore, and they find the right mix of hormones which will help several things. This almost sounds like a long list, right? In retrospect...this list is very short and manageable. She is grateful beyond words to be so much better. She doesn't seem to let these things get her down. She goes one day at a time and jumps, hurdles, or crawls over whatever she comes up against. God has been faithful time and time again. He will not fail her. She is a miracle! Thank you Jesus!