Always Late with the Update

Kiran saw his cardiologist a week and a half ago. The fantastic news is his heart is stable from six months ago – no more decline in function. This means Dr. R. is less sure Kiran will need an intervention as soon as he originally thought. However, I had some concerns with noticing increased fatigue. He has been falling asleep somewhat frequently during rest time at school – which, if you know Kiran at all, he has absolutely earned himself the nickname “The Supervisor” at school because he ALWAYS has to know what’s happening around him. And he has been getting tired earlier at night, too, often seeming ready for bed by 6:30!

With all of that in mind, we made the decision to move forward in scheduling an MRI of his heart, to get more information about its current function. This is less invasive than going into the cath lab, obviously, and the cath lab would likely require an MRI of the heart prior anyway, so we aren’t adding any unnecessary steps if we find Kiran does need an intervention.

It all just freaks me out, honestly. His heart has been the least interesting thing about his medical care and daily life for so long…I’m not ready.

Other than that, Dr. M. took a look at his feet again. I get tired of people saying things like “possible future surgery” so I am being super diligent again with the foot stretching and making sure Kiran has the appropriate shoes. Not that my diligence ever stopped, but I continue to do everything possible to avoid any need for future casting or surgery with his feet. Overall, Dr. M. thought things looked pretty good – and we were able to graduate to annual appointments, finally, with him.

Another necessary appointment day in Iowa City in the books. We survived, no terrible news, and the appropriate steps forward for Kiran. One step at a time.

Big Boy – Appt Updates

I am a little late getting this update on here, but the past two weeks have contained a whole lotta life.

Kiran’s first appointment on Thursday was with a nurse practitioner in otolaryngology (ear, nose, throat). This appointment has been in the back of my mind since 2020, when Kiran’s feeding therapist at the time mentioned a procedure that may make a huge difference in Kiran’s saliva production (and therefore, swallowing abilities). I consider it a “due diligence” appointment. The NP was in agreement that Kiran is not a good candidate for medication, because there isn’t a good one that will localize the drying up – meaning his eyes, gi tract, etc. would dry up as much as his salivary glands would. With his vision and constipation issues already, this doesn’t seem like a good option for him. I am relieved, because I have always felt strongly against adding this type of medication. We ultimately decided to follow up with a staff otolaryngologist and got an appointment for next month. She will be able to discuss with us other possibilities for saliva management, which include injections directly in the salivary glands or surgical options.

Honestly, I have a feeling this due diligence will give us good information, but I will likely want to opt out of any procedures at this time. Kiran isn’t having any medical issues with management of saliva – no excess, unproductive coughing, no pneumonia developing – so it probably won’t be worth the risks involved with these other options. But. Due diligence.

Next, we saw his regular ophthalmologist for his vision checkup. And the BIG news of the day is Kiran sat by himself in the chair for almost the entire vision appointment!!! I was tearing up several times throughout, because I can’t believe how much he has grown and matured this last year. He is able to understand a lot more (this is old hat for him at this point, after all), and he is more steady with his body….I just….man, he makes me so proud.

We weren’t able to come off the eye drops as I had hoped. Kiran is on a daily dilating drop because of his microcoria, which is a fancy word for small pupils. Dr. felt his pupils are still pinprick without them (I skipped the eye drops Thurs. morning so she could assess this accurately), but they dilate well with the drops. And especially with the eye gaze devices he is using for communication, she felt it’s important to continue at this time. The hope is his muscles will eventually learn (like they did last time we were on eye drops) to return the pupils to their resting state at that bigger size. Our only other option to address this is surgery, and we all agreed we’d rather just stick to the eye drops for now!

Kiran continues to amaze me every single time we have an appointment day. It was a long one, but he wore his mask like a pro and went with the flow for everything. He is growing up, getting stronger, and finding more independence right before my eyes, yet I still can’t believe it. He’s truly an amazing kid and I’m so happy I get to be on this journey with him.

Authenticity

A random word generator gave me my word for 2022: Authentic. I like it. I specifically like this particular definition: true to one’s own personality, spirit, or character.

I don’t write as much when I feel I cannot be my full, true, authentic self. A personal circumstance over the course of the last year and a half has limited my ability to do so, which is why the blog has been sparse.

I don’t like that I haven’t been writing, but I dislike far more passionately feeling like I have to censor myself.

This is the first day of 2022. I no longer put any weight on the whole concept that a new year will bring new circumstances – same stuff, different day. And yet, 2022 holds the potential for some pretty big things.

Grad school may or may not begin for me this summer. Kiran’s heart may need an intervention this year. And then there is that other large personal circumstance I cannot share, that I would absolutely love many, many prayers for, especially in May.

I want to take a moment today, to just remind myself and anyone reading this: We are still here. Look at ALLLLLL the hard the past two years have brought, and we are still standing. Beaten, worn, perhaps forever changed – many of us, myself included, have experienced unexpected loss, perhaps related to the ongoing pandemic and perhaps not. Relationships have been challenged, broken – oof. I mean, it has been rough.

I want to spin positivity, but the truth is – I’m not convinced I am better for it. The experiences of these past two years haven’t necessarily all shaped me into a better person. I’m grappling with a lot of darkness in my heart. But I haven’t lost sight of my priorities – my son, my family, beloved friends – my desire to bring more love and good into the world than anything else.

I think my point is simply this: It is okay that we are just here, trying our damnedest to be the best people we can be in this impossible life we are living. And just a reminder that others don’t have to always understand – and that sometimes people aren’t meant to be ours for the duration.

In the meantime, the people who are walking with us – man, this life is precious. I always say “It’s a long road ahead, but we journey together” in order to capture the daunting nature the road I’m traveling often has. But I could just as easily say it’s a short road that could come to an end at any time. So the one thing about me that I hope never changes is this: I am going to love with everything I have.

Here’s to the darkness not winning. Happy 2022.

tired

I’m tired.

This has been a season of difficult decisions, one after the other. A season of assessing risk and making tough choices, when there is no easy answer to be found.

I’m tired.

This has been a time during which I have been forced to assess the people in my life, and as it turns out “my people” are few and precious. And unexpected.

I’m tired.

Every time I see the light at the end of the tunnel, it turns out to be another damn freight train coming at us.

I’m tired.

I am making the best decisions I am capable of and I own every single one, and yet it is also somehow on me to carry the decisions others are making.

I’m tired.

I don’t want to do this anymore. I don’t want to make another single difficult decision.

I will. Because that’s what I do. I am Kiran’s mama, and we have come too far for me to stop assessing risk and making hard decisions now. He gets his second shot next week, giving him a level of protection he hasn’t had. He’s back in school. We will spend time with extended family this Christmas, Omicron-willing (that’s the freaking freight train, and I am grieving grieving grieving).

And I am so damn tired.

I got this.

We had Kiran’s annual waiver assessment meeting yesterday afternoon. One of the questions that was asked was on the scale of 1-10, how confident are you in your ability to provide care for Kiran. I answered 10.

Six years ago, days before he was born, I would have answered -800. Five years ago, just a month and a half after his open heart surgery, maybe I would have answered a 3.

But, days shy of 6 years into this journey, I answered a 10.

There are lots of reasons for this. The one I will share is that I DO recognize that in the past six years, a lot has been thrown our way. And every single thing, I have handled. I have figured out. I have learned. I have moved forward and done what it takes to keep Kiran safe and healthy and living his best life.

I am his mom. But even beyond that, I am his caregiver. And no matter what is coming, what has already happened allows me to know this one truth: I. Got. This.

I just need to remember.

the way things are

I know people have strong feelings about a lot of things right now. I have chosen to keep quiet about my strong feelings about things, because I have run out of capacity, lately. My philosophy has just been to keep to myself and take care of my family.

We have sacrificed a lot in the last year and a half, in order to keep Kiran safe. A lot of people haven’t understood the level of sacrifice we chose, and that’s okay, mostly. The whole point is that he’s still here. I cannot – and do not wish to – fathom a reality where that is not so.

Making the decision to keep Kiran home this fall and start his kindergarten year online/virtually was one that I have been grieving, deeply, ever since we made it. There is no doubt in my mind or heart that it was the right decision to make, but I really didn’t want this for him. If I’m being honest, I didn’t really want it for ME, either. I am not a teacher, certainly not a special education teacher, nor am I a physical therapist, occupational therapist, speech-language pathologist (Yet!)…and I just really didn’t want to be in this position again.

There is a lot going on with health mitigation in the political and legal world in Iowa right now, especially as it relates to public education. I doubt I have to explain it here, but basically, this week, our school district could once again legally make the decision on whether or not to put a universal mask mandate in place. And our superintendent and school board members came through for kids like Kiran, who absolutely deserve – and need – to have a safe environment where they can learn in person. The vote was unanimous – masks will once again be required in all buildings by all people.

Again, I know people have strong feelings. But hear me when I say this: This is LIFE-CHANGING for Kiran and my family. We have quietly sacrificed and gone about our business and done our best educating him from home since March 2020. I could continue to do so, with success. I already see how much he is learning, especially with his virtual learning groups in the special education classroom. But, the screen fatigue and frustration behaviors I started to see toward the end of the school year last year, I am already seeing this week – week 4 of school.

So. It is time. His dad and I discussed quite a bit today about how we move forward and navigate the transition to in-person school for Kiran. With masking in place and good news about the vaccine on the horizon for kids his age, the benefits outweigh the risk. Monday, we officially start the process.

It will be a process. I am not sure what all it will take, but we anticipate a couple weeks before everything is lined up and ready. Because this was the kindergarten transition and Kiran is starting in a new school building, a lot of the in-person transition stuff wasn’t taken care of at the beginning of the year. Health documents need updated and g-tube education needs to take place. I don’t know what the associate situation will be, if they have someone or will need to hire someone to be one-on-one with him. I just know I plan to do everything I can to make the transition go smoothly.

Also, thinking about the possibility of this the other night, I cried. I cried a lot. This is no ordinary transition into kindergarten, not in any sense. Kiran has been home with me all day, most days, for a year and a half. As hard as it’s been…as many days as I have just wished for ten minutes to be alone…as overwhelmed and exhausted as I have been trying to wear alllllll the many hats to help Kiran thrive…

I am going to miss it. I am going to miss HIM. I have never forgotten to stop and take the time to appreciate how lucky I have been, to get this front row seat to his education. I have watched so many tiny steps of progress and seen so many concepts click for him after so much work…it has been such an amazing experience. Like it or not, I am going to miss a lot of those moments now. They will happen all day every day at school, and I may or may not hear about them later. And even though it was never “supposed to be this way”, this past year and a half, it was. It was this way. And it makes me sad knowing it must now go to the way it “should have” been.

It is absolutely what Kiran needs, wants, deserves. I am thrilled for him, and I know that he will grow so much learning alongside his peers. There are just some things, like true real life social interactions with others, we can’t replicate at home. He will be so excited. He loves his people.

But one of these soon here days…whenever it is time, and I drop him off for his first day of in-person kindergarten….

I am going to be a puddle. A giant, weeping mess of a woman. And it will be so, SO hard to let go.

It’s a long road ahead, but we journey together. This part of our journey is coming to an end, and it is time to navigate a new path. I’d like to say I’m ready – I’m not – but I do know, without a doubt in my head or my heart, it is time.

Heartiversary – Also, School

I still can’t believe it has been five years. It hits differently, this year. Seeing all the memories pop up on Facebook, knowing we are nearing another heart intervention.

But I am so thankful he is here.

The last two weeks have been incredibly difficult. Life has been pulling me in millions of different directions, and my plate is overflowing with it all. There is personal stuff – loss, goodbyes, things worth grieving simply because it’s a path I never wanted to be on.

But there is also school. And this time, I don’t mean mine.

Kiran’s general education curriculum is an online curriculum not actually associated with his school district. So far, there is no real teacher interaction, no seeing other students (supposedly, story times are coming). There are just videos and online practice and lots and lots of workbook pages. As in way more workbook pages than I think any kindergartner should have to do. I get it – with everything being online, there has to be some way to assess knowledge. As you can imagine, this is not at all geared toward Kiran’s learning abilities.

And the frustrating thing is, even though I have reached out – twice now – to the appropriate people within the district to get some accommodations for Kiran, all I have been able to successfully do is opt him out of PE and Arts/Crafts. I am still waiting on direction, and the online teacher (again, not affiliated with K’s school district) just keeps telling me she can’t make any changes until she gets direction from the district.

So. I have been doing my best to get creative and make assignments more accessible to Kiran, changing how we do things so he can participate at his skill level. I think I’m pretty good at it, and I just leave notes for his online teacher every time we submit an assignment so she knows how we worked on that particular assignment. My biggest complaint isn’t that I don’t have help with this part – I feel confident I am helping Kiran learn – my biggest complaint is it’s just too much content and too much work for us. I’ll be following up a third time and making some more requests.

It doesn’t help that this is Kiran’s transition into kindergarten and many members of his IEP team are trying to do assessments virtually in order to know how to update his IEP at the end of this month. I have been inundated with forms and questionnaires and interviews and meetings – there is SO much on our calendar for September, especially since all of Kiran’s big waiver meetings happen this month as well.

AND (oh, you thought maybe that was all he had going on!?!) we just started his eye gaze communication device trial on Thursday, so that adds a whole new piece of equipment and goal we must diligently work on.

I have to say – his IEP team members have been amazing. All of the IEP supports and therapies are happening virtually, and I couldn’t be more pleased with how that is going already. Even just almost two weeks in, they are getting to know Kiran and working with me to make the virtual setting beneficial. Between everything they do and everything I am doing, Kiran IS learning. It’s amazing to see, and I am so proud of him.

I’m also exhausted.

I don’t have a lot of grace for myself, and honestly, there have been numerous times these last two weeks that I have wondered why I didn’t just send him to school. Feeling like it’s all too much, I can’t do this…and then he successfully chooses the red marker when asked. Feeling like there’s no way I can help him navigate this new communication device…and he already has success with it during our first virtual session with his school SLP.

Feeling like what if he’s not learning as much as he would have had I sent him…and then thinking what if he wasn’t here to learn at all if I had sent him? Today, on his fifth heartiversary, I got notice that he may have been exposed to Covid…had he been in the school building. And I know I would be having a much different kind of night, tonight, if we had chosen differently for our family.

It is hard. He is learning. It is exhausting. He is safe. For me, this is the path we were meant to walk right now. And this, too, shall pass.

I’ll probably miss it.

Heart and Brain

In some of the mice studies for the genetic diagnosis Kiran has, it has been noted that two of the areas most affected are heart and brain. None of the other children that have been studied with this diagnosis have reported heart defects, so Kiran is unique in that way. But the brain abnormalities reported in others, along with the diagnosis of intellectual disability, are similar.

We just happened to see both Kiran’s cardiologist and neurologist on Wednesday this week. Gotta love those trips when you leave at 5 am. Kiran did not know what to make of me waking him up so early!

Neurology was our last appointment of the day, and it went exactly as I had hoped. We have only seen neurology a few times in Kiran’s life, because we have no reason to be seen regularly. We were following up with her for his unilateral flushing issue. And she agreed with the hematology/oncology doctor, that we have been chasing a cause for this long enough. We have essentially ruled out any serious cause, and Kiran has seen every specialist regarding this issue to do so. So we just accept that it is idiopathic, which means “of unknown cause”. It is just another thing that makes Kiran a unique, special little boy. Unless other symptoms pop up or it starts bothering him or increases in frequency greatly, we don’t need to follow up. One more specialist off the scheduled docket!

Our first appointment of the day was Kiran’s echo, and we got a special treat! Our favorite echo tech, who is retired but comes in to help on occasion, was there and available, so we got to have him for Kiran’s echo! But our new favorite requested echo tech is leaving next month, so we will have to find a new favorite once again. Still, it was so nice to catch up and have the calm, expert experience first thing in the morning.

Cardiology did not go as I had hoped. I think after years of hearing “Everything is stable, see you next year!”, I was lulled into a comfortable space, even though my brain knew that Kiran would eventually require interventions. It is nothing we weren’t expecting, and in fact, is exactly what we have been monitoring. The pressure gradient in the conduit that was placed between his right ventricle and pulmonary artery has increased, somewhat substantially. However, there is no indication the right ventricle is having to work harder yet, which would be an increase in size – a growth of that muscle, so to speak. Because of that, it is not something we need to urgently address. Dr. R said Kiran will probably need a cath lab intervention, to balloon the stent, in the next 12-18 months. The ballooning helps to increase the size of the space so blood can flow more easily, lowering the pressure. It essentially prolongs the life of the valve, because we don’t want to have to put in a bigger one (which is open heart surgery) until we absolutely have to. The bigger/older he is when we replace it, the fewer we will have to replace over the course of his life.

So, we do need to keep a closer eye on things, so we will see cardiology again in six months, rather than waiting an entire year. At that point, if things are stable, we will go another six months. If the pressure gradient has increased a lot and/or we see right ventricle wall growth, then it will be time for the intervention.

So the positive here is it is exactly what we knew was going to happen, and we have a plan in place to address it. This is nothing surprising and is just part of having these procedures with conduits placed. He’s in great hands with his cardiologist, who I trust completely. I was so thankful for his calm guidance and explanations. He is always good at taking the time.

It’s a long road ahead, but we journey together.

Big Diagnostic News!

I was not anticipating a phone call this morning that would bring relief along with a complex emotional reaction after years – almost six, to be exact – of wondering and hoping for answers.

I’ll back up a bit. Two years ago, Kiran underwent whole exome sequencing, which is the most thorough genetic testing available, I think even still, but certainly two years ago. His genetic report came back with a misspelling on RAB11B. At that time, this misspelling was considered a “variant of unknown significance”, which basically means there wasn’t enough data at that time to say anything about it for certain. They would not say that the misspelling was responsible for Kiran’s medical issues.

Our genetic doctor at the time speculated that it made sense that it was – but cautioned that we couldn’t know for sure just yet – but it was a misspelling that was being actively studied.

Today, I got confirmation that they have indeed upgraded this misspelling to a “pathogenic variant”. This basically means that they now have enough information to definitively say that those with this misspelling have certain medical and developmental characteristics that are caused by it.

Kiran is still unique with his heart defect, as none of the other (3-4) known kids have complex heart issues. But this means we can look more confidently at the two studies we have to get an idea of what is in Kiran’s future. And hopefully, now that the misspelling got its upgrade, more people will be found that share the diagnosis.

I love definitive answers and new information, and it’s nice to have another piece to the puzzle – the biggest piece – as to why Kiran is who he is.