Overall, I have to say that we DID receive a lot of answers there. I had sent about a 4 page email to Dr. G ahead of time, per her request, that listed out all of my questions that we did not get to go over during our visit to her office the week before. I went through system by system what my main questions were that i craved answers for, what things we deal with on a daily basis that just never seem to make much sense, but are a real part of our life nonetheless. And when we got there, she told me that she had forwarded my email on to her team of about 5 or 6 residents (i lost track of the entourage with her every day!), and told them “THIS is why these people have traveled across three states to come see us, and THIS is going to be our job this week, to answer as many of these questions that we can find the answers for”.

and sure enough, they all had a pretty clear vision of what we were trying to get help with. As i was able to post about during the first few days – we had specialist after specialist coming in to the room in a near non-stop parade. There were many times throughout the day when I would pick up the phone to call home to talk to the other girls, and in would walk another doctor with another set of questions or testing suggestions. it was truly dizzying, and utterly amazing.

I will try to go system by system and give you the run-down on what we learned:

Eyes:  Although i personally felt like this was akin to trying to stuff a baby octopus in a pair of pantyhose, we somehow were able to dilate kendall’s eyes and have a fundoscopic exam. They tell me that they didn’t see anything of concern at this time. If my darling child had been in any way shape or form cooperative for this exam, i would buy that hook line and sinker. Considering the fact that all three of us were sweating bullets from trying to pin her down for more than point-3 seconds so they could shine the microscope thing into her eyes – I am guessing this one will need to be re-visited next year. (And in case you are wondering why we had to do it at all, many metabolic diseases, including mitochondrial disease, can affect the vision and structures of the eye because of the sheer amount of energy they consume. It has been suggested to us at least 5 times in the past year by 5 different doctors to get this exam done, and Dr G  felt very strongly that we needed to get one sooner rather than later.) Other than her eyes remaining freakishly dilated for the next 48 hours, i think we were able to say that her vision seems to be ok for now. considering her nearly legally blind parents, this is a huge relief!

ENT/Speech: The major thing to come out of this consult is that Kendall is still refluxing, pretty badly, and her swallow has gotten worse at some point in the past few months. The feeding therapist was able to point out that what we have always thought of as just a cute quirk of Kendall eating was actually a pain response to the massive refluxing she is doing. It’s hard to explain in words without showing you, but basically Kendall will drink her bottle a few gulps at a time, and then put her binky in her mouth for a few suck/swallows. We always joke about her being “two-fisted” – having the binky in one hand and the bubba in the other. Apparently it’s not a joke to kendall! The therapist said that she actually has to teach most kids who reflux like this to DO this – to slow their swallow down and suck on something else to help the refluxate stay in place in the stomach. I’ve known that Kendall still has some “reflux issues” when she gets the super bad hiccups that just don’t go away, but having the therapist point out how often she is refluxing when she eats was kind of heart-breaking to see. What makes this even worse to me is that she is doing this through the fundo wrap. Meaning that it is either completely blown, that it is very loose, or that she is refluxing with a ton of pressure to get past the fundo tightening.

The next day she was able to get squeezed in for a video swallow study (nothing short of amazing really – that NEVER happens to us that we can get added in at the last minute!). At this study, Kendall was VERY amenable to trying the different textures they wanted her to try. In swallow studies in the past, including one in march of this year, Kendall has been less than enthusiastic or able to tolerate the different textures – thin liquids, thick liquids, purees, and crunchies. What we have known/assumed is that Kendall used to have dysphagia/aspiration on everything she ate (first few months of her life). After the Vital Stim therapy she went through last spring, she seemd to have an ok swallow with thin liquids (her formula in the bottle with a preemie sized nipple on so that she could control the flow better), did great with crunchies (cereal, crackers, cookies, etc), and was having a hard time clearing thicker liquids/purees from her airway because she just lacks the muscle tone to move them. In March, she showed some “near penetrations” with everything – but we thought it was just because she was pretty sick at the time. (well, that and i was still in a pretty good state of denial at that time because I didn’t want to take her NPO – nothing by mouth – even though it was being suggested to us to help her lungs/airway out in early spring).

And on this “perfect” test – meaning she willingly tried all the textures and i thought was handling them pretty well for her (no obvious choking/gagging like we get a lot of the time) – she was penetrating/nearly aspirating on thin liquids, and doing the near penetration thing with purees. Hard crunchies were, i think, ok – at least they didn’t say anything about it to me. I was too devastated at the thin liquid news to process much past that. I don’t know if I was more disappointed because this is possibly an area of regression (meaning she has gone backwards, lost some skill she previously had), or because she maybe never had the skill in the first place. Either way, dysphagia is still well and alive in our repertoire of ICD-9 (insurance billing) codes. And dysphagia is, in and of itself, a sign of neurological involvement or impairment. But I am pretty good about blocking that last part out. So – final answer – thicken her feeds (with a commercial thickener), and if she is sick or otherwise having a “floppy day”, eliminate oral feeds as much as possible.

GI/Intestinal Motility:  The I-care team came in to meet with us and ask us a few questions regarding Kendall’s motility. While I really do have probably the most questions regarding this area, they seemed a little hesitant to jump in with changes or recommendations at this time with all the other testing we were doing that week. Turns out they were right (based on the fact that her gut did indeed take a nosedive into shutdownsville a mere three days after they came in!) They do want to follow her and work closely with her GI in Milwaukee to figure out what function she does have, and if it is possible that we will be able to get her back onto G feeds, or even full oral feeds at some point in time. I am guessing that for right now, they feel like J-feeds are her best option, and don’t want to do anything to mess up the quasi-homeostasis she is (or at least WAS) currently at. I am hoping that we will get some better direction here, although really, after what we saw happen to her gut from some anesthesia/headache pain, I am not real into making any major jumps or changes anytime soon! So while they didn’t have a whole lot in the way of “answers”, they do seem prepared to jump in when or as needed in order to maintain the function we do have and make appropriate plans from there.

Physiatry/Physical Medicine & Rehab doctor:  This poor doc got to see Kendall on her WORST day – the last one, when she would hardly even put her feet down for the doc, let alone show her the “drunken sailor” walk. In essence, this doc does feel that Kendall’s prognosis will likely be based on her final diagnosis – but did recommend for now that we continue to brace Kendall’s feet to correct her awkward positioning of her feet and hopefully provides some stability to her legs and hips. She also recommended that we continue with the high regimen of therapy that we are currently on, and was pleased that Kendall is already in aqua (swimming) therapy. Other recommendations included a rubber helmet to protect Kendall’s huge noggin from outdoor falls (to which Ben swiftly replied “heck no”), and possibly a medical/adaptive stroller, carseat and high chair in order to provide her with the support she needs to be properly positioned for each of those different activities. Again, she would like to see Kendall for follow up if we do come back to Pittsburgh so she can make more specific recommendations, especially once we get more diagnosis direction.

Immunology/Allergy/ID:  The MAJOR question for this team was “do we need to start IVIG for Kendall?”  IVIG, or passive immunization, has been bounced around by kendall’s docs for almost a year now, with no one being able or willing to pull the trigger on saying yes or no. it’s just been THERE. Yes she probably needs it, but no we don’t want to be the ones to recommend it. The problem is that we really can see how much of a hit her ENTIRE system takes when it is metabolically overloaded or stressed. she has proven that time and time again over the past year with provocations both big and small. And since she has not managed to break her 4 month “out of the hospital” streak from random infections requiring inpatient support, we really need to have a better plan in place with cold and flu season coming up. So after re-testing her immune levels, the pittsburgh immuno team ACTUALLY CAME UP WITH A PLAN. I am still shocked. Well actually, they tried to pull the same “well, mom, its up to you! we could go either way on this!” – and i told that darling little 1st year resident that that was NOT a real answer and she better go scrounge up a quarter somewhere and flip it because i did NOT want to hear her come back in until or unless she had a solid answer. So the head attending for immuno came in to explain things a wee bit better, and said that essentially, her immune levels, the ones her body has to make all on its own, are all still very borderline low. They aren’t just “bumping up” on their own as she gets older/healthier as we had all hoped last year when we discovered her deficiencies in this area. And considering that they were all just barely within range at THIS TIME, when she is about as healthy as we’ve ever seen her – I shudder to think how low they could be going when she is actually trying to fight an infection. Dr. M said that in cases like this where there isn’t a clear scream out for IVIG (meaning her body is making SOME immune response, just probably not ENOUGH for her infection rate) – they typically recommend prophylactic antibiotics – a little dose every day to just help the body fight infections better. Considering that we ARE already doing this, and have been for a few months now – we will know that if Kendall continues to get infections that land her inpatient, we will be making the right call to start the IVIG. The pittsburgh team will send orders over to the infusion center of our choice here locally, and we will get it started as soon as we decide she needs it. Once she starts it, she will be on it for 6 month cycles as we assess her function and ability to fight infections. Once she proves that she is doing a better job of making her own immunities, she can be done with it. This was not my original understanding of how it worked, so this was relieving to me to hear. I thought it was in essence a “once you start you can’t ever really stop” type of deal, and that it would take a few months to have an actual affect on the infection rate. Dr. M was really great about answering all my (stupid) questions and helping us figure out a little better when and where and if we will start the IVIG therapy. For the uninitiated, IVIG is essentially a blood transfusion of the “immunized” blood of many different donors (I realize i am oversimplifying it for those who DO know about it!). It can be very tough for kids’ bodies to handle for this reason, as their body has to work really hard to assimilate all these immunities into their own bloodstream and then start making its own little similar army of immunities. When you first start the therapy, it is very likely that it will need to be run as an inpatient to monitor for bad reactions. It will require monthly visits to an infusion center, where they typically hook little kids up to their chemo. It will require a tramautic poke to search for a vein that will hold for the entire length of the infusion (usually 8-12 hours). I give you all these details not because it’s something we have decided on, but so you understand why it’s not just an easy answer. But it WAS an answer, finally – so for this we are grateful.

Pulmonology:  I am getting super tired now so I am going to try to condense these last few because i am sure i will be back to revisit them in other posts! – Pulmo doc was AWESOME. She was mildly horrified at all that Kendall has had to endure with respiratory infections, but is very encouraged that as of yet, Kendall has not needed intubation for ventilator support during these infections. (so are we!) The biggest news of all came during the bronchoscopy that was performed when Kendall was under for the MRI/MRS on thursday, where dr. A (yes both of our pulmo’s are now Dr. A’s!!!) discovered that Kendall’s left airway, just past her trachea, is in a state of near-collapse on a pretty regular basis. The airway was flopping in on the camera, so Dr. A said that if its that floppy with the semi-hard tube of the camera in there, she can only imagine how hard it is for Kendall to keep it open when she is running around/playing/upright/coughing/fighting infections. this may explain why we see such a “need” for the oxygen support even though her sats look ok – but that may also be purely metabolic in nature. There were a few other non-specific findings – her lungs started bleeding when the camera barely scraped the edge, there was some evidence of atelactasis (I think i spelled that right – lung collapse, basically), some inflammation from aspirations – nothing shocking like the bronchomalacia on the left side. They sucked a bunch of junk (“broncheoalveolar lavage” – isn’t that a fun word???) out of both sides and are seeing if it grows anything fun that might explain the repeated pneumonias – but it is looking more and more like those are from the reflux from below or the bad swallowing from above.

They recommended that we start using a “cough assist” machine – the scream machine! – to help Kendall learn how to cough stuff out of her airways better and hopefully strengthen that left side to stay open a little bit better on its own. We switched up a few of her meds since the albuterol we typically use in her nebulizer when she is getting “junky” with her breathing is a muscle relaxer – apparently NOT what you want to have happen when your airway is already happy to collapse in on itself. So now we have to give her Atrovent in the nebulizer, which our regular Dr. A makes her have when she is inpatient and it turns her into a raving psycho-toddler hose-beast. Can you tell I *heart* this medicine? But – if it works and keeps us out of the hospital, then so be it. Pulmo wanted to follow us weekly I think if she could! But will settle for us following up with her anytime we are in pittsburgh, with at least monthly phone calls/emails to check in with pittsburgh Dr. A.   I really liked this doctor and her team of residents and am hopeful that they can help us get the lung junk stuff under better control for Kendall!

Cardiology: they did not feel there was any evidence of Pulmonary Hypertension at this time, do not feel her PDA is significant due to its size (small), and DO feel like even though her heart is asked to do a lot because her autonomic system likes to go haywire and tell it to beat way too many times per minutes sometimes – that it is a good STRONG heart, and should be able to withstand the pressures put on it. That was good news to HEAR, but the reports listing all her “cardiomegaly” and “left sided hypertrophy” were a little less encouraging. Along with a few other valve “insufficiencies” that were listed as minor – it just means her little heart does work pretty hard every day – and again – this is during her HEALTHY time! I am not sure that we’ve left the PH issue in the dust just yet, but hearing that her heart seems strong at the moment was certainly good! So we will continue on with the intermittent O2 therapy during times of stress and continue to monitor her HR’s.

Neurology:  Dr. Goldstein, the main doc who was running the show for us in pittsburgh, had some really good conversations with me every evening after we had seen everyone else for that day. I will need to blog about the details of these convo’s as well as the genetics consult in a different post as I am still trying to process through all of that info. But basically, it does not appear that she is having seizure like activity RIGHT NOW in her healthy time. Her MRI/MRS showed more of her “abnormal but diagnostic” features of “mystery metabolic disease”, as well as “delayed myelination” – which is just a fancy term for slow=growing brain cells. It explains some of her developmental delays and her struggles to catch up in spite of the huge amounts of therapy – but beyond that doesn’t mean a whole lot right now.

and that’s about all I can remember or type right now…

I am sure i will have more to type tomorrow!

t-crest

I am SO ready to move forward.

right now i am actually ready to move my foot forward onto the janitor who thinks its necessary to move kendalls bed around RIGHT NOW to get all the dirty laundry  - yes  i am sure that the three blankets and one sheet we have in there are of the utmost priority to the linen service. SHE IS ASLEEEEPPPPP!!!!!

Ok, brief rant aside.

Here is somewhat of an update of what is going on. I don’t know or remember what I have put on FB, what i have told some people in person – so I’ll just recap it all here.

Obviously – Plan B was enacted.

Plan B was originally to come have a PICC line placed (a type of more permanent IV that goes in the arm and directly to the heart – but not a PERMANENT permanent Iv. It’s a temporary permanent iv. Got it?) – and once the PICC line was in, we would start her on some PPN (partial parenteral nutrition) or TPN (total parenteral nutrition). The original plan was also that since we can all assume that this is unlikely to be a “quick fix”, that we would come home on the PICC, have home nursing come out and help adjust all these new IV foods/labs/etc, and life would move on.

Except that docs really don’t like doing that.

Nor do mommies.

The docs DO like to let little kids lay in the hospital for indeterminate amounts of time until little tummies wake up. Mommies do not.

Mommies like magic pills that don’t exist that make things all better and are the perfect happy medium between “laying in a hospital room” and “bringing a medically complex kid home with a known infection risk”.

Mommies never get their way.

So for right now, we are still stuck. Moving forward in the most miniscule infinitessimallly measurable of steps.  I don’t know what the answer is, but I know Who Does. And I know that He has never let us down before. When things seem to be overwhelmingly dark, the light always shines. When it seems like we are stuck without answers – answers begin to show up. So while I may be barely stifling my frustrations and questions and whining under the surface – I will remain calm. I will wait for the right answer to be revealed here. Because i KNOW. IT. WILL.

Kendall does have the PICC in right now, and she is getting a modified form of “PPN” – we are giving her body a lot of carbs (sugar!!!) and electrolytes, no protein and no fat, through the PICC. She is on 10cc/hr of full strength formula – this is GREAT!!! And while its not going “smoothly” or “perfectly”, it is going. She finally pooped late yesterday afternoon – just a tidge under a week! I never thought i’d be so happy to change a stinky diaper – but it meant things were finally starting to “wake up” a little bit!

I can’t get any of the four nurses i have asked for an extra extension for kendall’s G-tube so that I can relieve the pressure that is building up in there – so now she is just leaking bile and formula out of the stoma (around where the balloon holds her button into place in her stomach). But I think I just finally got one to understand that I am not asking for one from Santa Claus for Christmas, i need it RIGHT NOW.  They are also going to bump her feeds up from 10cc to 20cc and see how she tolerates that! That would be awesome if it works! Because if we can get her up to her goal of 40 cc – I think we can bust outta here!

I am still up in the air about what to do about the PICC. I know she needed it. And right now, even though things are waking up, I know that most of that is because she is getting the fluids/hydration she needs through the IV. I am the teeniest bit worried that once we take her off the IV fluids again, she will start having a rough time again. BUT – i am leaving that in God’s hands. I have way too many other fun things to worry about – like dance auditions for the girls, seeing all my babies all together again, catching up on laundry and cleaning, getting geared up for a great awesome fun year of Kidstown (the kids program I work with as my PT job at our church)!!! And Just Moving Forward with LIFE!!!!

So keep those prayers comin’ for this little monkey’s tummy. It is not real happy with us at the moment, but hopefully it can remain un-angry enough to let us prove to the docs that we can go HOME before we sit here all weekend doing NOTHING!!!

(and now i am hopefully going to go back and update some pittsburgh info – since all of that is now quickly slipping from my memory!)

love and hugs -

t-crest

In spite of the fact that she was clearly pretty weak from her recent hospitalizations, Kendall tried bravely to keep up with Kaylen, until she finally passed out for a four hour nap. Luckily, Kaylen also had a nap during this time, and if THAT happens every day – I will be one productive super trooper indeed! unfortunately yesterday I felt like I should have taken a nap too. Monday night was the 5th nite in three weeks that was spent in my own bed – it was a welcome collapse into bed after getting home from the hospital at almost 11 pm!

As to how Kendall Quinn is doing…well, that remains to be seen. I have been, let’s just call it “distracted” thinking about what Plan B is from her GI doctor. They let me bring her home from the hospital on “feeds” of 5cc/hr. Most preemies can handle more than this into their tummies. It is one dose of medicine, one medicine dropper-ful, every hour, very slowly. It is nowhere near enough to even keep her hydrated let alone fed let alone catch her up on calories which she really desperately needs. She absorbed almost 2 lbs of fluid over the weekend – meaning either that she was WAY more super dehydrated than her labwork even showed, or that it is somehow leaking into her tissues instead of being turned into urine by her kidneys, or – she’s got the world’s hugest bladder right about now! It is probably a combo of all three of those – lumped together under “autonomic dysfunction”. On her best days Kendall’s autonomic system does some crazy stuff, but it usually stabilizes itself, or has adjusted to the craziness, so we don’t mess with it too much (this is things like her body temp, her sugar reaction to certain foods or to “fasting”, peeing too much or not peeing enough). However, after a huge stressor like this weekend was to her (metabolic acidosis), her autonomic system is pretty much haywire.

So they let her bring me home because a.) their only plan was to continue to dump her full of fluids even though they weren’t really coming out as well as they should have, and b.) her pulmo HATES playing GI/metabolic/geneticist to her! He does it out of his love for her and his concern that she get the best level of care as close to home as possible – but he hates it. So – I called our GI, got plan B, and convinced them it was ok for me to bring her home that I could work on feeds just as good at home as I could there. She was interested in some oral eating again, could sit up without puking, and had managed to keep her sugar up for about 4 hours after stopping the IV sugar water. All good signs to me.

Unfortunately – not quite enough for what Kendall’s body NEEDS right now though. I was relatively pleased with her feeding progress yesterday, till I added up her 24 hour intake from stopping the fluids. Between what I was able to pump into her intestines at half her normal rate, and what she wanted to take in orally – she had 16 total ounces. About half of that was pedialyte, meaning she had 8 ounces of “nutrition/calories”. Subtract the 10 ounces that she dumped back out of her g-tube, and we aren’t making a whole lot of progress. Yesterday afternoon she was seeming to perk up a bit more, and we got a few more ounces of full strength formula in (4 to be exact), plus some more pedialyte-diluted formula, and i was able to bump her nite feed up a little higher. If she has a super-excellent day – she might be at half of the calories she needs for TODAY.

Her urine output is back down, the pressure in her intestines is popping open her med-ports (they are little openings on the tubes so you can push meds in without having to unhook the feeding pump – helpful if you have a kid whose motility is good, not so helpful when things are like this!), making her lose even more formula and bile, and she has not had a BM in 5 days. All things making us think that while she has gotten herself out of the metabolic acidosis danger zone – she is still not up to par for what her body needs. Now in a regular kid who had maybe a post-op ileus or even just a touch of gastroenteritis – you can have a little leeway. they will lose weight, sure, because their body will dig into its stores for calorie sources to keep itself going.

With Kendall, its not quite that easy. 1 – she just doesn’t have a whole lot of stores. She has more NOW than she ever has in the past, which is excellent, but her body burned through those real quick with all the vomiting. 2 – her body doesn’t effectively know how to make energy from calories (food) and oxygen on a good day, let alone when its “stressed. That much we do know about whatever metabolic dysfunction she has.

So – we are left with Plan B. I am not quite ready to commit Plan B to “paper” yet – those of you who have been through gastrointestinal slowdown/shutdown know what it is. For those who don’t know – let’s just say we may have to head up to milwaukee in the next 48 or so hours to be put on the OR schedule, and come home with a drastically increased medical load. Right now – it’s all up to God and the doctors to decide. I know she needs more than what she is getting right at this moment. I am sure that Plan B would give her a huge boost even beyond just this immediate deficiency. Plan B would enable us to meet with one of the leading motility experts in the country as he happens to be the GI on service this week. Plan B is not a bad plan….it’s just a hard to swallow one. (HA! get it! hard to swallow…cause….kendall isn’t swallowing well…..ok nevermind. too soon…)

All joking aside – it is weighing pretty heavily on my shoulders right now. I am not sure that I will be “happy” either way – if we don’t decide on plan B, i’ll just keep worrying about her and am afraid that plan B will become inevitable at some point in the very near future, perhaps after permanent damage to her minimal gut function has occurred. If we DO enact it – well, it’s just a lot to wrap my mind around. But I think going FOR plan b is probably the best thing to do right now – to ensure that it is the shortest amount of time possible. It will make this WEEK crazy – but hey, i am pretty sure we’re firmly into crazyville already.

So please pray – for our doctor as he makes the decisions and necessary arrangements. For the other girls as they will once again be in upheaval if we have to be admitted in milwaukee for a couple days. For kendall to endure another procedure if that’s what is decided, or to have the strength to endure through another couple weeks of minimal nutrition as we ramp her back up slowly from home. And for me – to retain the shred of sanity I have left. My mind is consumed with this plan and the decisions contained therein. And I would REALLY like to get off this topic already. Or even this merrygoround.

Thank you to all the good friends who have listened to me vent and process and whine through this past week of chaos. Thank you to all of you who pray and lift us up even when I haven’t asked for it. Thank you to our wonderful family who have rearranged their lives this past couple weeks to make sure that we don’t all fall apart. Words are not enough to thank everyone.

Off to see if some dunkin’ donuts’ X-tra X-tra can’t help sort things out a little better…and MAYBE a chocolate frosted. maybe.

t-rock.

sorry – I am going to try to not be overly whiny in this post.

I know so many of you are faithfully praying for our sweet girl and want some updates!

As best as I can recap –

Friday morning when we were due for discharge, Kendall stopped walking, would not bear weight on her legs, and in fact would just crumple into a screaming mess on the floor if we tried to put her down. She was ok if she was laying flat in her bed, but would not make much of an effort to move at all. We dosed her up with tylenol, which was not holding the screaming pain fits off, so we went to IV Tordol (basically it’s the IV version of ibuprofen), which knocked her out for four hours. She woke up happier than she had been all day. we put her down and she walked around so we told the nurse we were ready for discharge. The floor resident said if we were ready to go, we could go. No one came in to examine her or to go over discharge stuff with us. We packed up and were pulling out of the hospital parking lot when kendall started retching (trying to puke, but it hits her fundo and can’t get up). I jokingly said – “no ma’am! Let’s not puke the whole way home!” Oh the propheticness…

Twenty minutes later Kendall violently vomited all over herself and everything in her seat. And let me tell you – if you thought Oreo’s were messy going IN – well, wait till you see them come back out. She had had about one fourth of an oreo on our way out of the hospital and that small bite came back to haunt me for the next five hours! We stopped about an hour away, changed her, hooked her up to drainage bags and pedialyte and oxygen and thought we were good. We thought maybe it was a reaction to the Tordol, or just maybe being overly tired from a long hard week – we thought it was the end of it. But it wasn’t. She continued to violently vomit the whole 8 hour drive home. We stopped counting the times and stopped even trying to get her cleaned up. We just changed out towels and blankets as best as we could. She was definitely hurting.

We knew we were going to pass two children’s hospitals on the way home, but every time we would get near them, it seemed like she was improving. you have to understand how very tired we all were, how we just wanted to get home, how much we missed the other children – we weren’t TRYING to be stupid about Kendall, we just really had no idea which was the lesser of two evils. And plus you’re thinking  – ok surely there’s nothing left in there now, THIS will be the last time she pukes.

We made it home, she laid right down in her crib and went to bed. I slept on the floor next to her crib because by this time she was puking so hard that she would choke/not clear her airway, and I was so afraid to leave her alone. She slept for about four hours, woke up excited to see her sisters, and started puking again. Called Dr. Natalie who gave me a virtual beat-down through the nurse “ YOU KNOW BETTER!!! YOU SHOULD HAVE TURNED RIGHT AROUND!!! WHAT WERE YOU THINKING?!?!?! YOU GET TO THE ER RIGHT NOW!”

So off we went. Good thing I hadn’t unpacked a THING.

i took kaylen with me, Ben had the older girls at the softball tournament, my parents met us at the ER. We were met outside the doors by an angel of a nurse who must have seen how floppy kendall was, or maybe i just looked like as crappy as I felt – but he took kendall and ran us past the line of 30 people waiting to be triaged and into a room. They took one look at the protocol letter i had in my hand and got to work. It was darn near impossible to get a new line started – kendall’s arms and legs are all bruised up like she’s been in a prize fight from losing spots and blowing veins all week. But they got a line in, got fluids started, and I turned off her pump of pedialyte I had been running into her J-tube. And instantly she started to calm down.

She was lethargic and floppy and didn’t want lights on and didn’t want us to touch her or talk to her that whole afternoon. That nite the doctor comes in to tell me that Kendall’s labs were going a little wonky (heading towards metabolic acidosis), and that she had put her on PICU consult – meaning that the PICU doctor would make the calls about Kendall being able to stay on the floor or having to transfer to the PICU for higher level care. Kendall was on an IV fluid containing 5% dextrose, and her sugars were still dropping. She had not made any pee in over 18 hours – she was just in a bad way. At one point I was really super scared for her – she was pale as a ghost, even her lips, felt cold and clammy, and was floppy when i tried to pick up her arms or legs. Her body temp was 95 degrees.

Somewhere in the early morning she started to seem to stabilize, so we stayed out of the PICU, but they were still worried about what she was doing. Dr Aljadeff came in to check on her, took one look at the black bile draining out of her stomach and said in his wonderful accent “ clearly, what we are dealing with here is much bigger than a possible and rare CSF leak”.  wonderful. So we talked about “paralytic ileus”, where the gut THINKS its obstructed, so it stops moving. And yes, in essence, this is what has happened to kendall. We took a KUB (tummy x-ray) – and there is no obvious obstruction, so we can’t just give her an enema and get things moving. We can’t go in surgically and “fix” anything and wake it up. Her body just has to wake it up on its own.

and we have no idea how long that might take.

I tried valiantly to start the pedialyte back up yesterday afternoon – and kendall immediately started in with her pain signs – kicking the bed, writhing around, moaning, crying, grabbing at her head and her chest. So I turned it off. Understand that the rate I am dripping into her intestines is about equivalent to a teaspoon over the course of an hour. We are talking droplets of moisture hitting her tummy – and causing that much of a reaction. She instantly starts making more bubbles in her farrell bag when we do this too – so clearly her whole gut is reacting to the feed attempts.

That is all i know right now. Thank you to my dad and mom who went to the hospital yesterday so I could come get the big girls ready for school to start tomorrow. They are being inducted into the injustices of hospital life – including lab techs who think nothing of flipping on every light on the unit at o’dark thirty to get their lab draw, living off of vending machine food, and the kind of crazy things you start doing to entertain babies when the mind-numbing boredom sets in! so if you see them and they are jabbering non-sense, take it easy on them!

I hear reports from Poppa that Kendall is anxious to get something/anything to EAT right now – so we are trying a sucker to see how that goes! The problem is that she doesn’t understand yet that eating is what is making her feel so bad, and its VERY hard to say no to a kid who hasn’t really eaten anything in a few days, but if we try to put too much into her semi-functional gut, we could make the problem worse, and set her back another day or two! it’s a tough balance.

Hopefully her gut really has just decided to wake up on its own. No one at LG has any clue what else to do for her except pumping her full of sugar water. I am afraid to take her out of the hospital prematurely again, because I just don’t know if we will get another IV started if we have to go back in two days or a week. Ok I know I am the one jabbering non-sense right now. that is the udpate as I know it.

I will go back and cover all that we learned last week. It was full of answers, some good, some helpful, some…..harder to swallow. literally.

thank you for the prayers – for the gifts (colleen – I about died at the cuteness of the minnie mouse overload!!!) – for thinking of us – for the emails and facebook posts – for everything.

Keep thinking good tummy thoughts for Kendall! Your prayers are always so appreciated!

T-crest

Seriously – this will be bare bones stuff that I will come back and fill in the gaps on tomorrow.

Today started out by me coming over from RMH (the ronald mcdonald house where i come to sleep for a few hours every nite) to our nurse telling me she had orders to take Kendall NPO cause of her MRI in a few hours. This was further compounded by pulmo coming in to talk to me about the bronchoscopy and having me sign consents – it all made me think we were indeed doing the “big testing” today.

turns out that apparently “MBSS” (modified barium swallow study) and “MRI” look a little too much alike for a night nurse at the end of her shift!

So once we got that all figured out, Kendall was able to eat a quick breakfast (three trix balls, a bite of english muffin, and two bites of pancakes), and then got taken NPO again (this also means tube feeds – it means no food at all. Well, ok NPO means nil per oral …. i digress.

what was on the agenda for today was that she had a one hour EEG (leads hooked to her head to watch for seizures or seizure activity in her brain waves – tons of fun for a very sensory kid), the MBSS to see if she is still aspirating or an aspiration risk, and the EKG on her heart (not to be confused with the ECG or “echo” that was a dismal failure yesterday).

I will get into details tomorrow – I promise – but the long and short of today is that she does not appear to be having any seizure like activity on the EEG – GOOD NEWS!

She can safely swallow purees (which we previously did not think she was swallowing safely) – GOOD NEWS.

we were able to get a calm quiet 20 second picture printed out of her heart from the EKG – GOOD NEWS.

We finally had immuno lay out a plan for us with the IVIG – this is GREAT news!!!! One of the biggest questions we had in coming here.

And – she got essentially a D+ on her thin liquid portion of the swallow study – BAD NEWS. I mean, don’t get me wrong – its not the worst news. It’s just – saddening. If you are lucky enough to know Kendall “in real life”, you have probably seen her happily sucking away on her “bubba” (bottle). (Dear Megan the SLP – I know you hate that she still has the bubba!) It is as much a part of her as her crazy curly hair or her infectious giggle. If she was not able to take almost half of her calories in by “bubba” every day, she would be hurting in a bad way not only for calories, but for hydration. Even hooked up to the pump 24/7, she would still make just over half her daily goal. Plus her ability to walk around uninhibited would be greatly reduced. But – in more than a few conversations over the past couple of days, it is becoming clear that not all is as well in the swallowing/keeping food out of her lungs/keeping food in her stomach arena as we have lulled ourselves into thinking these past few months.

Don’t worry – she can STILL HAVE her bubbas! (I know all of you who aren’t grandparents of KQA were just waiting on the edge of your seat for that announcement).

but – we have to start thickening any liquids she takes by mouth with a super thick clear goop called – watch out this is creative genius right here – “Simply Thick”. I know!!! BRILLIANT!!!

Because I just needed one more thing to do in my day. Now I get to make my daughters bottles the equivalent of trying to drink a chocolate shake through a coffee stirrer. We gave her her first thickened feed tonite and she took one tug on it, pulled it back and looked at me like – “you have GOT to be kidding me, right???? oh well, I’m tired – BOTTOMS UP!!!” The effort gave her a wicked case of the hiccups for no joke an HOUR afterwards, but hopefully she managed to figure out which was her esophagus and which was her trachea while she was sucking like a madwoman on that bottle.

In the long run – I know this is a good thing. It’s just hard to hear it today – because it seems like just one more area she should be “growing out of” her problems, and instead they are just getting worse. But – like I said – it will be a good thing in the long run to have this info because it will help us protect her lungs better, which means hopefully protecting her immune system better, which means hopefully not as many hospitalizations.

We (or at least I – Ben has been at work all week and hasn’t talked to her for more than three seconds) continue to be amazed at Dr. G’s innate sense of being in tune with what Kendall is doing, what things she is looking for, what all these consult reports from different docs coming in hourly every day mean for the bigger picture. She sat with me today for a LONG while and explained through a lot of things, gave me some good perspective. I need to process through it a little more –b ut I will put it all on here soon enough.

for now, I need to get in bed. I am going to need my rest for tomorrow, when the REAL MRI is scheduled for. Tonite we are stressing kendall’s system out (on purpose) in order to get good baseline metabolic labs. It scares the whoopie out of me because this is what we kill ourselves to AVOID on a daily basis at home. But – i know we need this labwork, and I know she is in very capable hands here at the hospital if she DOES crash. She will be getting fluids only – no dextrose, no feeds. But at the first sign of stress, test over, sugar in. So – since no one knows her random little signs better than me, I need to be there. I need to be there in case she starts getting stressed out in her sleep and can’t give us any good signs. I need to be there when they come in to draw labs off her already failing IV without the aid of a tourniquet to get good blood flow. I need to be there when she wakes up to a team of people “doing stuff” to her and she scans the crowd for that one familiar face, mine. so I can sing twinkle twinkle to her even though she is screaming “NO” at me and biting through her binkies in frustration and pain.

so I need my sleep.

more details will come tomorrow. And in case I haven’t put it somewhere else – She is first on the OR list for the morning – 7 am Eastern time she will have an MRI/MRS, spinal tap, bronchoscopy, and then sedated echo. Anesthesia will keep her under while cardio determines route (PDA closure or not), and then that will either be performed right then and there or she will be taken up to recovery.  I have been told that anesthesiologists know all about her prior reactions to certain kinds of anesthetic and that she WILL need a PICU bed if they decide to hang a LR (a solution they like to use in the OR for ????? no one knows why they like it when it causes so many side effects for kiddo’s mostly, and especially for kiddo’s like kendall). This is what happened after her last GA (general anesthesia) – and it was brutal to watch. I know that with some mito/metabolic kids they say no LR as a precaution – it is a proven FACT for kendall that it will cause issues. I am prepared to go in with my boxing gloves on – but I have a feeling that they already know exactly what to do for kendall quinn (from her awesome doctor!)

So – that’s that for tonite.

Thanks so much for all the awesome e=cards you all have sent – they nearly cover one whole wall across from kendall’s bed! SO very heartwarming! and the prayers are definitely being felt! So thank you all!

have a good nite!

terra

So – the first nite I leave her in the capable hands of only the nurse, and this is what I get:

Kendall vomited twice that we know of, possibly a third and fourth time depending on a few suspicious things. I KNEW something was up last nite when she woke up from her nap in the afternoon. It was apparently quite a bit of vomit as I came in to a naked baby and a pile of dirty laundry in the corner (and that was just ours – the rest got sent to hospital laundry!)

In and of itself, this is not a huge deal. The sun rises, the wind blows, and babies vomit.

But for kendall, this could mean the start of any number of random, probably complicated, things. It means her gut motility (movement of food through her GI tract) is totally tanked, building up enough pressure to blow all that food past her fundo (the surgery she had last summer to keep food IN her stomach). What she didn’t throw up of her nite feed, which the nurse and I both assume was the majority of it, ended up coming out into her farrell (drainage bag that is attached to her G/stomach port). So the charge for the mystery illness that is now part of this mystery diagnosis begins.

The one good thing is that her nurse definitely got a crash course in “Kendall101″! She actually said, I was going to wash all her clothes but then I thought, she’ll probably end up being allergic to the detergent and i’ll just make a big deal even bigger!  I had to laugh at that because it’s SO TRUE. It would be just like kendall to pull a random contact dermatitis to the hospital detergent, have a horrific reaction which scared everyone, and then never be allergic to detergent again.

So now that everyone is a believer in the fact that Kendall can pull random stuff for no rhyme or reason, things are definitely movin’ right along! They called the IV team (THEY HAVE A TEAM DEDICATED SOLELY TO STARTING LINES IN KIDS!!!!! I know this is probably par for the course at most children’s centers – but i am personally in shock at it still!), they were able to start a line – but its in her hand where she has the most scar tissue and where she tends to infiltrate the most. We’ll see – I am holding out hope that this one might last for a whole day! She keeps holding out that arm to me and whining – like “ok mom, take it out now please!”

They then proceeded to take almost one ounce of blood from kendall (a little over 20 cc’s) – it’s never a comforting thing to hear a nurse walk out of your child’s room going “if you need four people to help with one kid’s blood draw, they ordered WAY too much blood!” I also heard our nurse basically accost the pulmo who came in to check on us and say “if you are thinking of ordering bloodwork you have another think coming – NO WAY can we take more blood out of that kid today!” That made me laugh out loud too!

so now she isn’t really tolerating any feeds (she wanted a bottle but it all came pouring right back out her G-port), she doesn’t even want a sucker – which is saying a lot for how crappy she must feel. We are trying the “bubba” (bottle) again, but she has assumed the falling asleep/leave me alone position – turned on her side with blanket pulled tightly over her face.

I am hoping this doesn’t cause too many bumps in the road with everything else they want to do.

here’s the scorecard as of 9 am, Tuesday morning:

Pulmo – whole team (doc, resident, student) in to consult/history and make suggestions

We have done her morning meds (nebulizer treatment and Qvar inhaler) AND we started the cough assist machine this morning. If you’ve ever seen the movie Monsters Inc – you know the scream machine? yeah…..it’s that. It’s a big mask that fits over her nose and mouth and essentially takes deep breaths for her – kind of like a bi-pap machine – but the end goal is that it makes her cough. And sure enough it DID! I was actually shocked to hear a REAL cough out of her! Of course now she is all junky sounding, so hopefully she keeps coughing or its just all going to settle back down in her lungs – but YAY! Finally something to help us help her clear all that crap out of her lungs.

Pulmo is also going to be pushing hard to get into the OR on Thursday during the other procedures in order to do the bronchoscopy (camera into her lungs). We have needed to do this for a while, So I hope it all works out logistically.

Opthamology also came by yesterday afternoon to do an eye exam. They say things are mostly normal looking in there (they dilated her eyes – they are STILL dilated this morning!), but the right eye was definitely giving them trouble. this is in line with the fact that kendall’s right side everything seems to not really keep up with her left. But I don’t know what it all actually means yet. Hoping Dr. G has some thoughts when she gets all the reports to put together.

while we were doing the above two things, the GI team and the Cardio team came by, and I heard were hoping to come by this morning and do a consult, possibly and echo for Kendall. So we’ll see what happens there. We are at least much farther ahead with the team discussions/multiple consults than I thought we would be at this point.

So that’s what’s up right now. Will update more later – possibly with some pictures soon!

If you didn’t see the facebook update – they have this neat thing here where you can send Kendall an e-card. They print them all and deliver them to our room so we can hang them up! I know a few of you sent them yesterday and i haven’t seen any yet – but the morning is still young and we are up on the 8th floor.

Here is the link to that if you are so inclined:

http://www.chp.edu/CHP/ecards

Thank you for checking on us!

terra

After getting a phone call this morning at 9 to “come in immediately”, we waited in admitting for 2 hours for them to find an open bed! (This is typical of most inpatient direct admits – it was the call that threw me off!)

So now we are all checked in and are on our unit, 8B. We are in a “sleep study room”, which is very nice – very large! – and according to the nurse “actually has a view, so enjoy it!” Because we may or may not be moved out of this nice room in our own little corner suite with only one other room by us, and out onto the main floor, where it is loud and busy and chaotic. But considering how packed to the gills the hospital appears to be at the moment, i am kinda hoping we get to stay here!

Kendall has had her baseline vital statistics taken, we’ve met our nurse, and – this is the shocker – WE HAVE SEEN DR. GOLDSTEIN ALREADY!!!!! I am in shock. We have also seen her senior resident who came in to get our medical records notebook. I handed her the 5 inch binder haphazardly organized with papers spilling out everywhere and told her to go have fun! I think she thought I was kidding…

So – they have ordered consults from Optho (eyes), Pulmo, Cardio, Immuno, GI and I-care (intestinal/motility), Genetics – and of course Dr. G and her team (neuro). Watch me pass out in shock if we actually see ANY of them today. Things just don’t move that quickly in hospitals.

Their EMU (epilepsy monitoring unit) is also packed to the brim, so we may not do the video part of the EEG, but just get her hooked up to leads and see what kind of clinical correlations we can get. Now – for the record – I don’t think she is having seizures at all. She has had one EEG (20 minute outpatient) that was totally normal. I think she has weird “electrical discharges” that CAN happen if she is otherwise stressed out metabolically (which for Kendall can be anything from constipation to tachycardia/autonomic dysfunction to just eating too much fruit or something equally as benign) – But I don’t think I would classify them as seizures at all. More like zone outs or possibly staring spells that last just a few seconds too long for my comfort zone, but you know, nothing serious.

Dr. G wants to be absolutely SURE that they aren’t seizures though, hence the vEEG. If she is able to get down onto the EMU (where they have the video part available) – I think Ben or I can’t be in the room with her overnite – but it’s hard to get a straight answer on that from people up here, who don’t work down there. That’s ok.

We did get a room at the RMH (Ronald McDonald House) – so we will go check into that later today. That is a HUGE PRAISE! Now, I can and probably will stay in the room most of the time with Kendall, but having a separate area to go decompress in, take a real shower, do some laundry, etc will be so very welcome.

So that’s what I know right now.

Kendall has a slight fever – nothing too high, but higher than her norm, and a little higher than her typical “metabolic fevers”. And its cold in this room. She has been a little mucusy the last few days, and sounded just on the verge of junky last nite when we got to the hotel, so i am hoping that something isn’t settling in to her lungs from all the crazy travel this week. She is leaking gross stuff out her GJ stoma and it is bright red/angry looking because of it. At least we’re at the best place for her to start pulling “Kendall-isms”!

Thank you so much for all the thoughts and prayers.

If I haven’t passed out from shock (if we actually do see other teams/consults today) – I will try to update later!

Ben just brought me a caprese salad and a coke – HEARTS!!!! So glad he could be here with us this week! I love that he knows exactly what to get me out of the 392 choices at the cafeteria!

t

So this post will be just the details of the appointment – or as best as I can recount the conversation without too much editorial comment. That will come later. Either later on in this post or in another post.

So our appointment was scheduled for 1 pm, but this morning the nurse called to change our appointment to 3 pm so that the doctor would have more time/all the time we wanted with her. I will refrain from the editorial comments here about what a mess this was, but let’s just say that when you are calling to leave a message for someone that has a somewhat urgent component to it, like say an out of state doctor appointment occurring within the next few hours, LEAVE YOUR NAME AND PHONE NUMBER. That is all.

It ended up working out well because a.) I would have hogged up that much time anyways, and b.) Kendall actually took a nap ( I know! in someplace NOT her room! Unheard of!!!).

In nothing short of a miracle, we found the hospital. This crazy city of one way streets and construction every other block – oh wait, I am reserving editorial comments for later. Anyways, we found it. Thank you Jesus for your guidance.

We were called back for our appointment right away after we registered (probably because it took me forever to park and get up to finding the clinic), and we didn’t even fill out the paperwork because Dr. G came right in (her name is Dr Goldstein, but i am just used to referring to her as Dr. G, so, there you go. ok that was the last editorial comment).

Dr. G came in with a stack of papers and file folders, welcomed us and said she felt like she already knew Kendall and was SO glad to meet her in person. I gotta tell ya, NOTHING warms the heart like hearing that from a doc! She had clearly done her homework on the paperwork I had sent over back in March. She had made notes on our lab tests, and had a few other pages of her own notes. She said she had a lot of questions for us, and I said that worked for me to start with hers and then go on to mine.

Each question she would ask would inevitably lead to one of my “issues” i had wanted to discuss, so we’d go back and forth in this manner going body system by body system. It was amazing to hear MY THOUGHTS echoed out of this doctor’s mouth. For instance, she said ” I noted in the paperwork that there is a lot of talk about IVIG, and that her immune testing is all relatively {not good} (I’ll get in trouble from my mom if i use the word she really said here), but WHY is she not on IVIG yet?”  I was dumbfounded. I have wondered this same thing myself many times! Not that we WANT Kendall to need IVIG infusions, but in the face of what we are up against with the active immunizations not working, and the fact that we deal with an average of a bacterial infection of some kind every 8 weeks or so, and that those TYPICALLY end up with kendall needing inpatient support, why isn’t more being done to support her?

There were about five or six instances where this kind of thing happened – we talked about the MRI results report not making anyone feel warm and fuzzy (my thoughts exactly), her possibly needing a cough assist device (again, discussed many times, never actually ordered), her possibly needing a port/access of some kind in the future (second verse, same as the first), and the fact that there is little testing to support our theories/treatment of Kendall’s gut function, because we all ASSUME/have good confidence that it’s just {not good}. Just about everything i have ever questioned/researched/been frustrated with in regards to Kendall and her issues, Dr G brought up and her thinking was just so right on with what I have always felt.

After about a half hour of this, she just kind of stopped and said “Clearly there is a LOT going on here, and I am just going to tell you, it is going to take a while to get to the bottom of all these assorted pieces with the different people involved in her care. Is there any way you can come back next Monday to be admitted and just let me do it all here inpatient?”

My mouth still hurts from where my jaw dropped open and hit the floor.

Her options were actually “Next Monday, or the week of Christmas, and I just don’t think she’s going to make it to Christmas without some good solid direction from the testing we need to have run.”

so we picked next week. * the reason those were the only two options are because those are the weeks that Dr. G is the attending neurologist on the floor. Any other week would be some different doctor, not familiar with Kendall, and with drastically different goals for outcome.*

Now if you have ever had the privilege of having a medically complex kid, you know EXACTLY what i mean by “getting things done inpatient”. As long as its not a holiday weekend, you will get done in about three days what would take about six months if you did it all “outpatient”. Getting to run testing inpatient means you get answers/results/help on a much accelerated timeline. (I know – not always, but in general, you get way more done, right???) so for HER to suggest this to me,  I knew she wasn’t fooling around with it. I told her I needed to run it by Ben, but that given what we have had to go through this past almost two years, we were pretty much ready to do whatever was needed to get Kendall the help/answers to live life to the fullest.

I called Ben and told him what was up, and he said “well, I guess it works out then that I am supposed to be in pittsburgh next week”. I could not believe it. now I don’t believe that everything is going to magically fall into place just because THIS happens to be one major coincidence. But – to my heart and mind – it was just one way of feeling reassured that we were doing the right thing.

Dr G started the paperwork for the admit right there and told me that while she had many more questions for us, we would definitely be going through all of them next week. She gave me her email address and a list of things I should try to get from the hospitals/her docs before we come and told me to send her any questions I still had so that we could finish getting a solid game plan in place.

And that was the end of the appointment.

It was just….

A breath of fresh air. A huge infusion of hope. Exactly what I NEEDED to hear.

sure I went in hoping we would get THE ANSWER – whatever that is. But that isn’t what I NEEDED. What I needed was to feel like I was being heard, that everything I have noticed or tracked or brought up to docs for the last 21 months wasn’t for nothing.

That i wasn’t crazy.

And i mean, the jury is still out on that last one in MANY ways – but today, I got confirmation that at least with regard to my baby, I am not crazy.

She is beautiful and she has bright blue eyes and she gets most of her nutrition through a tube in her intestines and she likes to make her heartrate do funny things at random times that scare the bejeezus out of me – and she has a metabolic condition that is causing every cell of her body to misfire.

SHE IS DOING GREAT - and we hope to keep her that way.

We do not know what to call her metabolic condition yet. Dr G had printed off page after page, chart after chart of the “metabolic cycle”, urea cycle disorders, Krebs cycle disorders, fumarate something or others – my head started spinning at the sheer volume of info she had compiled. The bottom line is this – what we DO KNOW right now is that the cycle that each cell has to go through in order to turn food and oxygen into energy is not happening correctly for Kendall. EVERYTHING in her testing points to this as a definite concrete thing.

If you picture the metabolic cycle as a figure 8, the bottom half of the cycle is what we’ll call “metabolic” and the top half of the 8 is “mitochondrial”. The bottom half is supposed to complete its little circle and then hand off its by-product to the top half, which creates “energy”. Energy to digest food, fight infection, receive messages from your ears and eyes, swallow food correctly, keep your brain functioning, keep your heart pumping, etc etc etc. Everything needs energy. Still with me?

For those of you who know kendall, you will see where we hit the crossroads. Many of those things don’t happen, or at least don’t happen properly, because there’s not enough energy.

so that right there is enough to say – yes, kendall has a mitochondrial metabolic disorder. Or a metabolic mitochondrial disorder.

The problem is – that’s not enough to actually HELP Kendall. If we can pinpoint WHERE the breakdown is occurring in that figure 8, we may be able to provide what her body is lacking, plug in a cog or a sprocket in the form of a supplement or a medicine that will help the process keep moving.

where we are running into problems is that we can’t pinpoint it just yet. Last fall when we thought she had the genetic defect for the MMA – methymalonic acidemia – we thought THAT was the missing cog. And sure enough – the b12 shots helped IMMENSELY.  But it turns out, kendall doesn’t “genetically” have that defect. That was just one part of the assembly line that had a kink. As we worked that kink out – it became apparent that there were more kinks.

As Dr G explained it to me today (and those of you who are way more knowledgeable in this than me, please correct me if i am mis-speaking here) – we may not ever be able to distinguish for Kendall if her “kink” is in the top half or the bottom half of that figure 8 cycle. They are both affecting the other. She may have a primary mito defect that is causing a lack of energy to the bottom half of the cycle, thereby not allowing the top half to ever function properly because, well, it doesn’t function properly. She may have a metabolic defect that prevents energy from getting to that top half so it doesn’t have enough energy to force the bottom half to do its job right. There are literally dozens of parts to the bottom half cycle alone, and Kendall’s testing points in at least one dozen of those directions!

So – that’s what we are going to attempt to narrow down next week.

We will also be having the sub-specialty parade in our room:

Pulmonology, Cardiology, Genetics, Endocrinology, Immunology, Opthalmology (i can’t spell that word tonite), Intestinal Care, GI, and of course Neurology (Dr. G) will all be coming in to give us “second opinions”, or at least opinions that are cohesive under the metabolic mitochondrial disorder genre. Right now, we have most of those people on our “team” already – but none of them are really working TOGETHER, with one big picture in mind. Dr. G has certain docs in each of these specialities who she knows KNOW her metabolic kiddos. They know that what might be normal, or abnormal, for a kid with any other issues might not be what works for a metabolic kid. They know that there are different threshholds for metabolic kids.

And they know how to make all of that work together for what is best for each child individually.

Now this doesn’t mean we are done with our docs back home. It just means that NOW we will have some direction for them all! I know that Kendall scares the whoopie out of the majority of them because, well, metabolic stuff is a scary thing to mess with, and because they just don’t know what to do with her. That is part of what is so amazing about Dr G – when I would describe part of what I see happening with Kendall, she could FINISH THE SENTENCE FOR ME. She got it. She got that if “x” was starting to happen, “y” would be the final result. I am still in shock and awe, sorry.

What we KNOW will be happening next week: Kendall will need good veinous access all week for certain tests that Dr G wants to run. I laughed out loud when she said that and told her that i hoped they could get it all in one shot because they would have an average of ten hours from the first IV placement til they needed a new one. And again – she said that didn’t surprise her given kendall’s history. So – we MAY end up going for a PICC placement first thing upon admitting. She will more than likely be on a Holter monitor for her heartrate stuff for a couple of days, and will more than likely end up with a video EEG – which is so many levels of fun to imagine that it will take its own post. You know kendall? my child with sensory issues who hates anyone touching her head or face? They will have to hook her up to about 20 leads all over her head, with sticky tape/glue stuff, and LEAVE THEM THERE for three days straight. I think I might rather be mauled by small woodland creatures….And then at the end of the week, once everyone else has evaluated her in her “regular” state, she will be put under GA for a sedated MRI and spinal tap, and possibly a few other procedures as may be recommended by the other team members. This may or may not include her PDA closure, a chest CT/bronchoscopy, GI testing, etc.

our insurance is going to hate us.

And there are about eightyfourthousand loose ends we still need to tie up.

For instance, do we bring the girls with us? It’s the week before school starts. I have countless other appointments next week that will need to be rescheduled. I have to WORK at some point. I need to bedazzle the girls’ backpacks. Dance tryouts are coming up. We have no idea if we can stay in the Ronald Mcdonald House yet, and no way can we afford a hotel for a week. Ben has to be at his work monday through wednesday nites which means that I have to leave Kendall alone in the hospital at nite so i can stay with the other girls at the RMH – IF we get in there.

Blah.

I can’t think of that – its going to make my head explode.

I have probably already made all of you all’s heads explode with all this info.

So that’s that. you got a little more than “just the facts”. I am still sorting out “how I feel” about it all. Mostly peace. It’s a super good feeling to feel like you are being HEARD. And that you aren’t crazy. And that maybe, just maybe, there’s hope on the horizon. I mean, there’s ALWAYS hope, but it is harder to see at some times than others.

Remember my favorite movie? Anyone, anyone?

Here’s part of why I love it – one of my favorite quotes:

“Hope is a good thing, Red, maybe the best of things. And no good thing ever dies.”

Full of hope tonite,

terra

So please don’t take it personally dear friends and wonderful family. By this time next week, I will be in a much different, hopefully better, headspace. I will have a direction to set the navigational beacons in, and I will gather up the chicks and lead them towards that direction.
Thank you for sticking by me through it.

And now I need to go figure out how I am going to get kendall, all her medical equipment, me and at least one laptop on the plane and come in under our 40 lb cargo weight limit. Because the laptop and my makeup bag alone are about 10 lbs…
this should be fun!

t

(PS – I know some of you have so many questions about where we are going and why pittsburgh and why the small plane and what are we hoping to learn in this appointment – and I promise that I will get to that. Just not tonite! But feel free to leave any questions in the comments and I will do my best to answer them!)

But the way that I found this out – oh my gosh, one of the funniest things I’ve ever witnessed in a long time. And I see a LOT of funny stuff every day!

so, my mom and I are driving down Weber after picking up pizza for dinner. Mistake #1. For those not local enough to appreciate the absolutely effed up stretch of former asphalt that is Weber Road, let me just say, on a GOOD day it makes you want to stab your eyeballs out with a dull spoon. Right now it is all ripped up while they re-pave. Except they’re not actually re-paving it yet, they’re just moving orange barrels around in a random pattern every day, clogging up an already clogged up road even further. I tell you all this so that you understand how PAINFULLY SLOW traffic was parked which is what allowed me to see the poor turtle in the middle of the road.

So I tell my mom to hurry up and get out of the car and pick up the turtle (I actually thought it was a frog). She hops out and leaves her door open to find the amphibious creature, when we actually start crawling forward. She bends down JUST as her open door is about to whack her in the head. I am sure the people behind us were fully convinced they were on Candid Camera because it had to be a hilarious sight to see. So she gets it in the car and we start looking at it going – what IS that? Because while it had a hard shell on its back, it was squatty and fat like a toad. Then it started peeing. And the pee REEKED like all manner of decaying things.

So my mom is screaming which is making me scream and this turtle is like hissing at us and trying to crawl across the dashboard (where my mom threw it when she started screaming) and i am screaming at her to catch it catch it and she is screaming that she can’t and that she is going to puke from the smell/stench of the turtle-thing pee and that i better clean her car out.

anyways – i thought the girls would like to see it, and we were almost home, so she played lion tamer on the turtle with two sheets of paper, which made him even more pissed off. which made him…..you know…piss. more. stinkily.

We carry the turtle up the front steps proudly and call the girls out to see it and my dad real nice and calm-like is all “That’s really not the kind of turtle you wanna be holding on to Mary Jane….” and we’re all “why? its cute! it’s a baby! It even still has its tail!!!” And the girls are all “I wanna see it! Let me hold it!” And my dad says “well yeah, that cute little tail is how you know its a snapper”. My mom resumed with the screaming and flings it off the porch into the bushes.

So now we have a snapping turtle living in our front yard. I am quite sure Ben will appreciate this as he tries to do yard work this weekend. It keeps things exciting.

anyways – i continued to laugh about this scenario well into the wee hours of the morning yesterday. I just wanted to share it here. In case it makes you laugh too.

and plus, you know, to tell you the important info that turtles with tails are bad.

you’re welcome!

Terra the Turtle Whisperer

           La Tortuga