Choosing Joy through the Storm

 We are still in a season of not the greatest medical stability, but through it all my kids continue to find moments of joy and light. I am always proud of my kids, but never more so than when they continue to shine despite not feeling 100%.

My sweet Alex, my bug...He is so easy to love and so quick to step up for others even when he isn't feeling great. Our relationship is so very special and comes from walking side by side through incredibly hard circumstances for most of his 21 years. We know each other and can read each other better than anyone else in our lives. And best of all we truly enjoy each other's company. We laugh so much when we are together. It is so special to watch him live a life we never dreamed possible and he never takes one moment for granted. He is having a great semester at school. He loves his classes and professors. He is the co-captain of the JV Esports team and a sub on the varsity team as well as commentating the varsity matches. He has been playing some pickleball with friends. And is double majoring in Psychology and History in the honors program. He was also able to enjoy lunch and hear Nic Vujicic speak at school. To say he lives his life to the fullest is an understatement. We have also found an amazing PCP who specializes in transitioning complex medical kids to the adult side medically. We had our first appointment this week and are both feeling so lucky to find a partner to advocate for Alex and make sure he continues to thrive. He has several tests and infusions this week as we make good use of his time during Fall Break!

Alex and Nic

Alex's jersey

Poor Maddy is still struggling a bit. Unfortunately the special urine test that we had hoped to guide treatment and a path to feeling better did not give clear cut answers. She continues to be extremely fatigued as her Kidneys struggle to work properly. After missing all dance and more than half of her rides for several weeks, she has tried to jump back in this week. My heart is sad for her as she is struggling to participate in her favorite activities, but she is adamant she will continue to push through. I treasure the smiles when she is at the barn and her best boy makes her forget how bad she feels even if she can't last as long as she'd like. And in true fashion both Hart and Quigley must be sensing she isn't feeling her best because they have both been extra snuggly with her! 

We did have one exciting development this week...after communicating for several months with Team Impact (TI matches Children with serious illness with college sports teams) she was signed on to the TCU Cheerleading team. While she had initially hoped to be matched with an equestrian team, she is actually thrilled to be with the cheerleading team. TCU Cheer has 41 cheerleaders, but she has a small group of 6-7 that are her group. We attended their practice on Tuesday and she loved every minute. She didn't participate just got to know them and watch, but is hoping her girls teach her a cheer next time! She even has a group chat going with them! Later this week maddy and i are leaving for the biggest horse show in the country, the All American Quarter Horse Congress. She has been looking forward to it for a year. Please pray she feels good and can enjoy her time!

Thank you for loving our family!

Ali

Rough few weeks

 I am not going to lie, this past few weeks has been a little rough. But even on the days when things don't go our way or we have more questions than answers, we know we are held and loved. So we can always find the beauty and the joy regardless of our circumstances.

Alex's port is still leaking, but he feels okay so we are just trying to march along until Fall break as he hates to miss any class time. He is loving his classes this semester. His professors are amazing and he enjoys all of them. He has made some new friends and continues to enjoy all of the "extras" that college has to offer. I am so spoiled that my boy loves to share all the things so I get an inside view into all he is learning and doing. Our relationship has always been close and special and even college has not changed it! This week Bruce and Alex enjoyed a soccer game and he went with his Honors classmates to the symphony. I continue to see so much improvement in his organization and maturity and especially his time management as he has learned to balance work and fun and medical demands and get it all done. 

Alex and Bruce taking in a soccer game

Alex and Sam, the Assistant Director of the Honors program and one of Alex's favorite people

This is his second symphony and the Opera House is gorgeous

Oh my poor Maddy...it has been really hard for her this past few weeks. Her kidneys are not working properly (due to her Mitochondrial Disease) and it is taking a toll. She has had all of the symptoms of a UTI, including blood in her urine for most of the past 6 weeks. We have done multiple urine cultures and all but one have come back that it isn't a UTI. So our amazing pediatrician has referred her to nephrology (but can't get in until November) and has continued to order more and more testing. As we are putting the pieces together we know that she hasn't had a "normal" urinalysis since April. During this same timeframe we have watched her endurance decline drastically and fatigue and feeling unwell take over. She is only lasting about 45 minutes for each lesson at the barn (she used to ride 2 hours and would do more if wee let her). She has also had to miss her last two dance classes because she doesn't have the energy to do any more. Dr. Benzick and the nephrologist have been in communication and they believe she has Renal Tubular Acidosis which is somewhat common in Mito. basically, the kidneys are unable to remove acids from the blood into the urine. This makes the blood too acidic and the urine too alkaline which leads to the formation of kidney stones. All testing up to this point supports this diagnosis and an ultrasound yesterday revealed multiple kidney stones. The acidosis is what causes the fatigue and headaches she is experiencing. Unfortunately we need to complete one more test before we can confirm this diagnosis and thus begin treatment. I am so very grateful for Dr. Benzick as he truly has walked beside me and supported my kids from day one. I could not ask for a better doctor to be there for my kids and pick up the ball when we need him to. I am also thankful for Mandy and Joli who love my girl so well and have been extremely flexible as we try to get her feeling better so she can enjoy all her favorite things again! And our best boy, Quigley....he has been one of the few sources of smiles and her time at the barn has been the only time she can distract herself from the pain and fatigue she is dealing with.

In the midst of all this, I have also been undergoing some more testing as some hope of a diagnosis came from the most unexpected of sources...an allergist. I have struggled with awful allergies all my life and finally decided I needed to see an allergist after many years since my last appointment. As I gave him a history of my allergy struggles, but also filled him in on some of my issues over the past few years, he became convinced he  may have an answer. He believes i may have either Systemic mastocytosis or Mast Cell Activation Syndrome both of which can explain the puzzle that hasn't made sense. We are still waiting on the mast cell testing to come back, but in the meantime I had to go off all allergy meds for a week to do allergy testing. My PSA for the week is DO NOT STOP ALLERGY MEDS DURING RAGWEED SEASON!!!! I was a hot mess as my friends at the barn can confirm. I completed testing this week and was allergic to all but 4 of the 56 things they treated me for. So I will be restarting allergy shots soon.

At this time I would ask for prayers for Maddy to feel batter and get back to living her best life and for Alex's port to behave until we can  get it figured out.

Thank you for walking beside us and supporting us during the good and the bad times.

Ali 

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Busy medical week

 The last few weeks have been a bit of a beat down medically, but I always try and remind myself that regardless of circumstance we are held by a loving Father who has a plan and uses all of our trials for his Glory. 

You know I never like to focus on the negative so I am going to start with some good first! Alex just finished the first week of classes. He has thrown himself in full force as he tends to do. He lives each moment to its fullest as he knows our time is not guaranteed better than most! He has 5 classes this semester and thinks he will enjoy them all. He has two professors that were with him on his trip to Boston and understand the extent of his medical stuff more than most. He has been asked to mentor and captain the JV team of his Esports team along with one of his best friends. 

Maddy and I just finished our second week of school and it has been really good. She has worked hard and been extremely well behaved for me. She is also back to her twice a week dance and art classes. And after a little break at the barn while we let Quigley get some new shoes and recover from his hard work at the World Show, we were back to riding yesterday and there were huge smiles all around. Maddy's tutor also reached out to let me know that she feels like Maddy is doing well enough to go back to one day a week tutoring as she no longer needs any intervention in reading and they will just work on spelling and writing. 

Okay here comes all the medical....

After 5 years of no real changes in Bruce's testing, this summer brought a few red flags. There was growth in the lesions on his kidney along with potential corresponding lab changes. His oncologist felt it was imperative to send him back to the urologist. After an appointment and some further labs, the urologist decided to take a watch and see approach and will see Bruce again after his next scans in December. He also had another colonoscopy where they removed two more polyps. We do not have results back yet, but his colorectal surgeon said he is pretty sure they will come back pre-cancerous and that he will never to go longer than 3 years between colonoscopies for the rest of his life. He does not think any further treatment will be necessary, but will reconvene after biopsy results come back.

If you have been reading our blog for a while some of this will be a repeat as I explain central lines. Alex's port (central line) is a necessary life sustaining tool. It is also what will most likely be the cause of his death one day. Central lines are "permanent IV's" that are inserted into large veins and end in the atrium of the heart. Alex has had a central line since he was 3 and it is the source of his nutrition and many meds. We also use it for all blood draws as Alex's vein access is almost non existent (like having to be stuck 12-15 times and often still no blood). While Alex's line is necessary it also carries major risk. Every time you use the line it is a sterile procedure and re-access and dressing changes require completely sterile protocol (similar to what is done in surgery). An infection causes sepsis and sometimes septic shock. Infections can happen either by mistake (not being totally sterile when handling the line) or translocation which has often been the case for Alex. In Alex's case his GI system is so diseased that bacteria can leak into his bloodstream and it likes to attach to a foreign substance AKA his line.  Okay so now here is what is going on... Alex's port is leaking at skin level. It is hard to keep his dressing on (which is necessary to keep it sterile) and also leaves his skin around the port wet which could breed bacteria. The week before school started we reached out to surgery. They did some testing and according to them the port is "working". They think his skin is so weak due to 18 years of port accesses and that is at the root of the problem. However, the leaking continues so we had to do multiple dressing changes this week instead of just one and has Alex and I both concerned. We will re-access his port today and hope it improves. We are trying to figure out the best way forward to keep him safe, but also not have to miss large amounts of class time. 

My Maddy Moo...you would never spend time with her and have any idea about all she has going on. I am not sure how she accomplishes all she does, but I am amazed by her grit, determination, and commitment to doing what she loves despite any challenges. I will start with her joints. We had an appointment with her physical medicine and rehab doctor (who is amazing). Maddy's ankles and knees are in bad shape. her joint instability is significant enough that her muscles are no longer firing like they should. She often has her joints give out on her (especially on stairs) and she has some level of chronic pain in all these joints. We are not quite ready to tackle her first major knee surgery so her doctor is going to have us use a NMES machine to try and force those muscles to contract and not lose any more muscle mass. We should have our appointment soon to get our machine set correctly and learn where we need to place the electrodes so we can do this multiple times a week at home. Maddy also continues to experience lightheadness, dizziness and migraines due to her POTS and neurocardiogenic syncope. She is on 4 medications and while it is a bit better it is still a daily issue that makes everyday tasks difficult. The biggest issue right now is some bladder/kidney stuff. She has struggled with bladder stuff for the past several years, but we have never been able to really get answers on the cause and therefore a treatment. However, some new, more concerning symptoms have been going on since May. The urologist has kind of thrown her hands up so I made the decision to take her to our beloved, amazing pediatrician because I knew he would get to the bottom of it. He ran 4 tests last Monday and we are waiting on all results before we decide next steps, but so far the 3 that have come back are not normal. I am hoping Dr. B can come up with a plan and we can get her feeling better soon.

As for me, I haven't had a minute to focus on my issues. There is no treatment (that doesn't cause more issues) for my biliary reflux and bile induced gastritis, but the medications I am on have made it manageable. I am EXHAUSTED despite sleeping 7-8 hours a night. I am unsure if it is my chronic anemia or my thyroid levels or something else, but I am hoping to get some labs soon to see if I can find a way to be less fatigued day in and day out.

If you made it this far, thank you for reading all of that. We would covet all prayers for our family and especially the kids as they try to continue to Choose joy and live life fully while managing a disease that is relentless and forces their bodies to betray them at every turn.

Ali

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