"Hanging in there"

My typical answer for the general "how ya doing?". And it's true. I logged into this blog for the first time in months a few minutes ago, took a deep breath, and started this. I'm not sure what will come through, but it's time...

We've had a time of monumental change and I needed to retreat to a tiny corner and sit for awhile. Process, love on the littles, and just be. Thoughts have been a blur, and I literally am unable to think 5 minutes into the future anymore (which, I'm finding is quite nice actually).

Everett had his last visit with the hematologist in March. Fully recovered, he is within normal limits for both circulating and stored iron.

Tomorrow, he has his first visit with a plastic surgeon. He has a rapidly growing granuloma on his cheek. It is (upon a dermatologist visit) thought to be one that continues growing rapidly and never resolves, is very unstable (as evidenced by Ev running into David on Sunday or so and bleeding and oozing just about non-stop since), and it needs to be surgically removed (meaning stitches...meaning scarring...on his face...hence the plastic consult). It is thought to be benign and the greatest concerns would be 1. sedation, 2. infection, 3. amount of scarring and 4. reducing chance of regrowth as much as possible. I'll post more here as I know more.

Mary Louise and David had eye check ups in January. They went well - very little change in vision. However, David's left eye has been wandering outward during exhaustion since I can remember. It was corrected with glasses to some degree. But the vision in that eye has declined faster than the right. I am now putting dilating drops in the unaffected eye weekly and patching as much as he'll let me (we play pirates...often...and super heroes...who only need one eye...and when all else fails, I let him watch a show I wouldn't normally allow (Spiderman). My favorite David conversation so far was the first time I put the patch on:

Me: Hey David? Remember those super cool pirate patches we got at the store?
D: Yeah!!
Me: How about you come and choose a color and decorate one. Then we can try it out and be rockin' pirates Bub!
D: OK!! YAY!
ML: I want to wear an eye patch!!
Me: ...
(David put stickers on his patch...I put it on)
D: Hey Mom! I can't see if you cover my eyes!

Sweet baby really doesn't seem to be able to see much out of that left eye...but we're working on it. We go back mid summer for a recheck...hoping for some resolution or at least slowed progression.

Mary Louise has had a couple of visits with her pulmonologist. He will see her again mid summer to hopefully stop all meds completely for awhile. This. THIS. is huge. She is taking 1 puff of Advair daily right now. No nasonex, no adenoid removal, no sinus surgery on the horizon...She runs, she plays, we've used the nebulizers once this year during the winter. Vitamins, probiotics, kicking dairy, elderberry syrup, local honey, nettles based allergy vitamin, shark liver oil immune boosters and the correct (CORRECT) preventative prescriptions. It's what's working. Each little step pushed her to get a little bit better, recover from illness a little easier, faster, stronger. Each brick is so important. And with the doctors seeing that with diligence, this preventative approach is well worth the outcome, perhaps hope will resume (because, let's face it, it is so very needed). The next time they find an ubber obnoxious wild eyed bear momma sitting in their office insisting that "it's just not good enough" and "my baby CAN and WILL be better - I just need to find the way"...perhaps in this critical but relatively typical situation, they can offer something more than "well, this med will work, but it's expensive (without even looking at our ins. coverage)..." or "you know, when people come to me, they usually just want a quick fix script - not a long term diet change".

Mary Louise and David will have their end of the year recital this weekend. David is nervous, ML is hard to contain and has informed David that he must NOT mess up her "big stage". Ha. Stinkers. They will have swimming lessons and a fine motor / handwriting course this summer. and....they will attend a Montessori preschool program 3 days per week in the fall!!! It's very small (15 students total), in home, and they are beyond excited to attend "real" school. I'm nervous...mostly about exposure to illness. But, developmentally, it's time for them. They are begging to try. Having an opportunity to get them into a program like this is a great blessing. So, we'll dive in in a few more months!

We've had another round of birthdays here as well...5 and 2 ya'll...5 and 2. 5 and 2...5 and 2...FIVE AND TWO!!



Mouth in the middle:




Ma Monkeys...




My greatest lesson: if you see a field of flowers...stop and enjoy...








and...you know...if you're allergy prone, perhaps have the appropriate antihistamine cream on hand post crimson clover dance...always learning...

xo,

Heather

It's (not) all Greek

Remember that time someone told you that your red head / super fair child might have a genetic disorder that typically runs in Greek / Mediterranean families lines and you were all, "huh?!" and they were all, "yeah. I think so." and you said, "so what does that mean?" and they were like, "well, not much...unless he falls in love with one of those olive complected beauties and wants to have babies with her...I mean...he doesn't have any other symptoms of the disease...right?"

Enter description of symptoms:
bone deformities
brain damage
slow to gain milestones
signs of poor oxygenation
frequent severe illness
immune suppression

P.S. - the above isn't from Google - it was actually brought up and discussed during Everett's first and second blood draws.

Then that someone added:
"because his white count looks ok so I don't *think* it's Leukemia...but it might be lead poisoning so let's see how that test comes back...I'll get back with you in a few days on that one"

and then you were like, "wait...I just wanted a 12 month check up and am a little late..."

and they were all, "well, you should probably see a specialist."

Remember that time?

So, you cried and cried and stared at your sweet little *deceivingly* healthy baby for a few days before harassing the specialist's office to give you a dang appointment 5 days after the last blood work was done...because, let's be honest, 3 weeks with all of those possibilities would likely end in you stress eating yourself to death. "Therefore," you said, "the continuation of your family unit depended on you getting your bumpkin in there, like, asap?"

What about that time? Remember that?

Sadly, I do.

Letterhead with "HEMATOLOGIST / ONCOLOGIST" across the top that contained explicit instructions about the exact number of family members (2) that could enter the room with the patient and that they were to be adults - absolutely NO other children allowed during appointments (as well as the intake paperwork for new patients of course) arrived at our house over the weekend. I dreaded filling it out. I dreaded having another doctor contact in my phone. But most of all, I dreaded having a conversation with the man whose name was on that letterhead. I just didn't want him as part of our medical family. I'm not sure how else to describe it. I was shutting down. I just didn't want to hear anymore. Everett looked healthy (still does) and I flat didn't want to know anything else.

But, we went to the appointment.

We arrived in the parking lot of the specialty center, got Everett out of his seat - grinning, but worried as he'd just ridden an hour in the car with *only* his momma and daddy. (Mary Louise and David were home with Mommom who popped over after visiting family in Lafayette.) Kiddo knew something was up. I caught a glimpse of something pink on the parked car beside us: "In Memory of Meghan" with an angel underneath the wording. We were there...this was it.

I walked in and shuffled down the longest isle ever to bring the paperwork to the receptionist. (no seriously, the design of the waiting area, I swear, would insight panic in even the calmest of parents...maybe not...I'm high strung...there it is.)

I turned and went to the restroom. 1 private stall with a suspiciously comfy antique wooden chair in the corner. It appeared completely pristine but wreaked of vomit. This could not be our new home...

We were called back quickly and the nurse was amazingly sweet. Everyone was relaxed and caring. Everett was completely freaked out -but I did manage to get him interested in a few super cool toys that were around...and I'd brought markers - always a win!

The doctor sat down calmly. He looked over the paperwork and test results I'd brought (a good thing because the proper history sure wasn't faxed even though I'd confirmed with our pedi office it had been...they faxed blank results sheets???)

He asked me the following:
Why are you here?
Why were blood tests run in the first place?
What were the circumstances of Everett's birth?
What was his birth weight?
Any concerns before this?
Do you even have Greek or Mediterranean heritage?
How are you giving his medication?
Does he take it well?
How much is he getting?
Are you sure that's the concentration?

He went over the blood work step by step with me with this funny little smirk and SUPER calm demeanor the whole time. Then he said those golden words, "here's why I think Everett is just severely iron deficient...and why Thalassemia isn't likely"

Reasons stated:
He had a severely traumatic birth which included extreme stress and bleeding
He was slightly premature which means he missed out on at least 3 more weeks of iron stores he would have gotten from you (Heather) during a full term pregnancy

Me: so what about his age? Isn't it true that most 1 year olds are at least slightly anemic?

Dr.: yes - growth during the first year literally uses everything your body has...when else are you going to double (almost quadruple) your weight? Never...I mean, if you were 150 pounds and you suddenly grew to 450 pounds, you'd probably be anemic too, yes?

The doctor continued:
I see you gave the iron supplement for 2 weeks and saw minimal improvement. Everett is 9.9 kg today. The CORRECT low dose that I start severely anemic children at is 3mg / kg.

(Everett has been getting 15mg total per day. He SHOULD have been getting twice that per his weight.)

Please start him on 30mg and we'll recheck him in 3 weeks. Expect to keep him on the iron supplement for 3 months AFTER his blood work comes back at low normal - which I would expect to be at the 3 week mark. We can go up on his iron supplement dosage again if needed. He can have as much as 6mg /kg. I suspect his iron stores are nonexistent at this point so the 3 months following normal result should help his body to recover from this. What he is experiencing is something I would consider to be as normal per his history, age and growth. His red cells will remain small and deformed until he has healthy iron stores again (and wouldn't, alone, indicate Thalassemia).

See you in 3 weeks.

I wanted to hug him. Literally. Hug. I didn't. But I thought about it...and thought about yelling, "WTF" really loudly as well. Instead, I just sighed, kissed Everett on the forehead, hugged him closer and smiled at Brent.

The doctor also asked me if Everett was the baby of the family and if Mary Louise and David ever had anemia. (they didn't believe it or not) He said he did a study in 1995 and 9 out of 10 anemic children were the youngest in the family. He couldn't find the reason - it just was.

*Sigh*. I seriously couldn't make this stuff up if I tried. We'll go back to that same office in 3 weeks for repeat blood work. As long as it's normal, I believe we'll have another recheck 3 months later then done.

Thank you so much, again, for all who have checked on us and offered gracious thoughts and prayers. It is all very very much appreciated. I promise.

xo,
Heather

Just Smile

Sometimes, there's nothing to do but smile - one of those big inappropriate smiles that comes from the depths of a mother's crazy soul (um. let's face it. Mother's are a breed all their own. We're all a bit off...some more than others clearly...but anyway...back to inappropriate smiling...) After the inappropriate smiles come the ear piercing cackles. The cackles sometimes consist of audible curse words - so cover your ears little ones - ear muffs. After the cackles comes the heart breaking silence. Just a sea of nothing between the fog so thick one can't even breathe and that final acceptance and clarity.

I am on the verge of cackling - so we're ok right now. 

Everett had his blood work rechecked today. What I thought would be a few minutes and a whine or 2 from a finger stick turned into an hour wait because the doctor wanted to make sure to see us personally (we only had a nurse's visit on the schedule so we had to be fit in). Once in the room, the nurse was overly formal with the computer work, really quick and efficient with the finger stick, then painfully slow at getting the results...probably not - but that's what my neurotic little heart processed while waiting and throwing a half ton of gold fish crackers at the big kids while they drew murals on the table paper and learned phonics Ipad style. (how's that for a nice fat run on sentence?)

(before I move on, it should be noted that Everett is OK. The following is what we've found out, how I found out and what considerations need to be kept in mind from here until we see the pedi hematologist. After that visit, some of this may change. However, the diagnosis is very likely correct from what I've been told.)

Our dear pedi entered. I tried to read her. She's a hard read. Then she sighed. She's much easier (to read) when she sighs. Then she looked at Everett and I started to cry. She said Thalassemia and a ba-jillion questions entered my mind but I couldn't focus on even half of one. She said Leukemia was pretty much ruled out because Everett's white cells have been stable. But, his blood work had only marginally improved. (I know, you're still on Leukemia right? ruled out...try to let it go)

For the medical pros and curious among us: Everett has been on ICAR for exactly 2 weeks today - 15mg / 1.5 mls ; 1.5 mls daily

7/25/13 - first CBC

Lead: 5.1

WBC 7.5

HGB 8.6

HCT 27.8

MCV 60.5

MCH 18.9

PLT 285 (low normal)

MPV 7.2

8/8/13 - second CBC (no lead repeat - moderate range in first screen)

WBC 8.9

HGB 9.2

HCT 29.5

MCV 62.9

MCH 19.7

PLT 209

MPV 7.5

normal ranges:

WBC 6-17

HGB 10.5 - 13

HCT 33-38

MCV 70-84

MCH 23-30

PLT 250 - 600

MPV 8-11

Essentially, this means Everett is anemic and his red cells are very small. There is still some concern that an iron deficiency anemia is contributing to the abnormal labs. But the general feeling is that Everett has Thalassemia Trait (or Minor). This means he carries and is chemically affected by the genetic mutation that is Thalassemia but he does not physically express the disease process. He's heterozygous for one of the forms of Thalassemia. (I invite you to review your high school Punnett Square genetics lessons *grin*). 

Concerning? yes. End of the world as we know it? no. He's still the healthy smiling sweet poop a doop (as Granny calls him) he's always been - and he'll continue to be so. Right now, this means we need to know his blood work will always appear very concerning - always irregular. We might have to have checks every so often so that we can know his normals in case a situation should arise where such information would be important (blood transfusion parameters and processing). He will also need to keep this in mind if he chooses to have children (in 30 to 45 years...I'm softening ha). If his spouse (um. hoping - ha) should carry the same genetic trait, the baby could have Thalassemia Major which is devastating. 

Why are we so shocked? This is a genetic mutation that tends to run in Greek and Mediterranean blood lines. Umm...if we have relatives in Greece, be forewarned to make a bed Sug because I'd like to come and visit! Furthermore, you may or may not have noticed the whole super white kid / fair hair, light eyes, freckled face thing we have going on here...this was just not even close to being on our radar.

So, right now, we are waiting on a call from the pediatric hematologist. (Because, you know, we don't have that "ologist" in our contacts just yet.) He will (likely) run further (more specific) testing which will hopefully offer a more complete picture on our little Everett. If this is Thalassemia Trait, Everett would not need further care or any treatment at this time- there's nothing we can do and his body seems to have compensated well for his deficiencies. We will simply have the information if or as we need it.

We have Mary Louise's allergy testing at the end of next week and an ENT check up (where we will likely learn if an adenoidectomy will be recommended (please...please no)). So, I'll be updating on our family medical status quite a bit for awhile...

Many many many thanks for the check ins, thoughts and prayers...sometimes all I can do is smile...or maybe cackle (ha!).

xo,

Heather

Medical updates

David is doing very well - no real updates or concerns right now. He continues to use his preventative inhaler (Qvar) before bed and takes his vitamins, probiotics and elderberry syrup in the morning. That's it! (yay!)
**************
Mary Louise is in the middle of check ups and meetings with a few specialists. (Pulmo, ENT and allergist)

We had a visit with the pulmo last week and I am very happy to report that we have been able to reduce her preventative medications to just one puff of Advair daily until September. We will go back up to twice daily then as it will be RSV and flu season and she'll need the extra protection. She'd had immune function testing during her last visit with him and he reported everything was normal and very stable so her respiratory condition is uncomplicated by secondary factors.

We are participating in a few activities this fall(ballet classes for one), but must remain very mindful and weigh our winter outings carefully to protect Mary Louise and David as best we can. Their immune systems are becoming more specialized and will continue to develop over the next few years. Preventing any further serious illness or hospitalizations is one of our top priorities as doing so can offer our sweet big kids the very best long term heath outcome.

The doctor spoke with me at length -discussing our plans socially and medically to formulate the best road for Mary Louise. It was a very positive visit and he is pleased with our decisions and progress so far. He asked that we consider a beach vacation (we have one planned) as swimming in salt water works wonders for asthmatic patients with seasonal allergies - helping their sinuses to clear and reducing inflammation. (ha - I can't say I mind that sort of medicine!)We will have another check up in 3 months.

P.S. precious girl is 30 pounds 15 ounces as of last week. WHAT?!! we have a 30 pound kid in this house!!
**************

Everett went in for his (late) 1 year check up last week. It was a circus with all 3 kids in need of some sort of immunization. We vaccinate - but do a very mixed schedule. Mary Louise and David reacted, in some way, to every shot they had - typically fever and pain, general malaise - so we only give 2 at a time now. It works for us. Our doctor is supportive. But it means I am trucking back and forth to the office a few times extra to get things done. Anyhow...

This was the very first time Mary Louise and David have reacted - emotionally - to shots. Keep in mind, these kids have been poked and prodded more than some terminally ill patients I've encountered. They have a history of not even flinching - much less fussing - for shots or blood draws.

Yeah - those heart breaking quiet days are apparently over. Those 2 were WILD. So much so that I'm pretty sure the office breathed a sigh of relief when we were finally able to cart the screaming banshees out of the door. (Gracious thanks to Mommom for helping with the wrangling). Mary Louise tried to escape at one point. David tried to kick me in the face a few times. I stood wide eyed and drenched in sweat when all was said and done. Good times.

Everett has a concerning spot on one of his top teeth so we'll be making his first appointment with the dentist next week.

He is on the petite / normal side at 20 pounds 15 ounces and just over 30 inches long. But he's growing just the same.

The doctor did a routine CBC (general bloodwork) and lead screening. The lead screening takes a day or 2 as the lab does them in batches. I didn't hear anything yet so it's either normal or I'll hear something Monday.

The CBC was very concerning. Everett has moderate anemia. The cause is, as of now, unknown. We have started a prescription supplement (though the insurance company lists it as an OTC drug so they will not cover it and I have no appeal recourse as the classification is preventing the coverage...could someone please explain that to me?! It's not very expensive - so that's not the issue - it's the principle: We pay for the policy. The policy should cover NEEDS. This is a need. It should be covered. I've spoken to 2 pharmacists. There is no OTC med that comes close to this and this is, in fact a prescription medication.). We will have repeat blood work done in 2 weeks time. The expectation is that, if he is simply iron deficient, the lab values will show notable turn around. If not, further testing is warranted and there are a couple of pathological processes to rule out.

I wish I could say that I am not worried at all...but I am terrified. The idea that something might be awry with that boy makes me ache inside, makes my brow furrow uncontrollably, makes my lips draw into a permanent slight frown and my shoulders tense. I want to shield him (and Mary Louise and David) from all that makes life difficult at times. I haven't succeeded with any of them of course - that want was poo-poo'd from the moment their cells became an organized mass for goodness sake. But the want is still there. It's not just that though...this is Everett:



It boggles my mind that anything could even be off with that child - much less "moderate to severe"ly off. It just doesn't make sense.

Truth be known, I thought about asking for repeat blood work right there - thinking they might have accidentally switched the vials somehow...I just don't understand...

So, we are giving the medication as prescribed (ICAR) and focusing on an iron rich solid diet as much as possible. I spoke with the lactation consultant where the kids were delivered about whether or not increasing my iron would help. She couldn't find any particular research stating it would, but said it would make sense that me taking in more iron would increase the iron in my milk. So, I'm do all I can with regards to my diet as well as Everett still nurses a considerable amount of time (though I can't say how much volume he's getting...) (oh, and because this is a hot topic in breastfeeding discussion groups: Breastmilk is not considered a "high iron" food. However, the iron there is much more easily absorbed which makes it nutritionally sound and, in most cases, supplementation of vitamins and minerals is unnecessary.)

In 2 weeks *sigh* we will have more answers...and hopefully not too many more questions. I'll, of course, update here...

xo,
Heather