Eat Happy!

Eat Happy!

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**The information provided in this BLOG is in no way intended to diagnose, treat, or cure any illness. This information should never replace the advice of a doctor. Please use this information as you see fit. This information will pertain differently to each child, each adult, and each family.**

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Thursday, December 27, 2012

Jurassic Orthotics

Ian picked up his new SMO orthotics last week. The first day, he wore them for one hour. Each day, he has been adding an hour or so. Today, he wore them pretty much the entire time he was awake, about nine hours. He seems to actually enjoy them. He likes to help when we put them on and tried putting them back on after we take them off.

We had been wondering if he would hate wearing them. The orthotist said most children love them because they offer support they don’t get otherwise. He also mentioned that sensory seeking children in particular enjoy wearing them. The deep pressure from the tight fit of the orthotic gives them the extra sensory input they desire. The orthotist was right, Ian certainly does like them so far. Fortunately, he never plays with them once they’re on his feet.

Being the super cool kid that he is, he needed super cool orthotics. We let him pick the pattern for the hard plastic. It was a tough call between the farm animals and the dinos, but the T. Rex prevailed. He picked blue for the velcro straps. The double velcro strap is what secures the orthotic around his foot and ankle. Ian had no trouble showing off his new kicks!

Ian’s case was caught very early. Thanks to other PT issues, we were in the right place at the right time. Since his case was caught so early, his treatment time is about 18 months. That means he should be all finished before elementary school at the latest.

**************************************************************

As a refresher, Ian is wearing the SMO orthotics to help correct pronation. His lower extremities are trying to compensate with his knees. As a result, his thighs and knees are touching, but his calves are apart. He is the opposite of bow-legged if that helps create a visual. Because of the hypotonia (low tone) in his feet, his arch collapses and creates a “flat footed” nature. The foot then collapses inward, creating a situation where the foot rolls in to the center. The knees and legs follow.


Pronation of foot & ankle from Hypotonia

** Ian's orthotics corect his ankle & his foot. **
** This is why "in-shoe" orthotics aren't appropriate. **

Genu Valgum from hypotonia foot pronation
** This is not a photo of Ian **
** But is an excellent visual of his stance. **


Ian's piggies in his super cool dino SMO orthotic

Ian's size 6W Saucony sneakers fitted by Stride Rite
(2 sizes larger and a Wide)
Insole removed for depth

Ian's size 6 Tsukhihoshi Maru orthotic sneakers fitted by Nordstrom
(2 sizes larger, not sold in Wides, but shoe is cut wide)
Insole removed for depth
Sportin' his new kicks!


Saturday, December 22, 2012

RSV 12.13.2012 Hospital Discharge Day & AFTER

Well, now that Ian has been home for ten days, I guess it is finally time to get that last “discharge” blog posted......

Ian came home on Thursday, December 13th. He was given Rxs for two nebulizer medications at home. He will continue with the Flovent as his everyday steroid respiratory medicine. The new nebs are his “go to” respiratory emergency meds. The first is a mixture of his previous Albuterol inhaler, with Atrovent. This is called Duoneb. The Douneb will not only use larger Albuterol particles than the inhaler to help with his lower lungs and small passageways, but the larger Atrovent particles will help with his upper respiratory passages. Ah ha! Inadvertently, this will also help decrease vagal mucus secretions during respiratory distress.

Albuterol-Atrovent Nebulizer Inhalation Solution


The second neb is 3% hypertonic solution....... salt water! I’ve had many parents mention saline nebs before. I had never heard of them and wasn’t sure what made them different. The saline will wick away mucus from the respiratory passages. A little science here.......

**Hypertonic solutions mean that there is a higher concentration of solute proportionately (more salt than water)

**Mucus has a higher osmotic pressure, AKA: is more hypertonic.

**Since areas of high pressure always move to areas of low pressure....... the pressure from the saline neb moves into the respiratory passageways. (High moves to low in order to equalizes the pressure.)............

**This means that water form the hypertonic saline neb moves to the mucus.

Think of it in proportions:

Hypertonic Saline Neb: 4 parts salt, 3 parts water
Mucus secretions: 4 parts salt, 1 part water

Water moves from the saline neb into the mucus until it equalizes

= 4 parts salt, 2 parts water on each side (saline solution and mucus)

The movement of water:
The scenario of the nebulizer solution and mucus is the image on the Right:
Water moves from saline neb into the mucus, adding water to the mucus


As the pressure and osmolarity equalize, the water will move from the hypertonic solution into the mucus. This helps to loosen the mucus and makes it easier to break (cough) apart and expel. It also helps lubricate the passageways of the respiratory track and moisturize them. Some also believe that it helps disturb the stability of the environment of “salt-liking” bacteria causing illnesses and troubles.

3% Hypertonic Solution Saline Nebulizer Inhalation Solution


When Ian uses the nebs, he uses them together. His chronic lung disease in and of itself doesn’t necessarily warrant the regular use of hypertonic nebs. He isn’t prone to continuous thick secretions. Those types of indications often follow a Cystic Fibrosis, or other respiratory secretion illness.

Ian's Nebulizer


Ian was able to stop the oral steroid, thankfully! His artificial steroids were already high enough to suppress his body’s own cortisol production. Typical cortisol levels for someone of Ian’s size, weight, and age are between 3.0 and 19.0. Ian’s were 4.0....... not good. In addition to 4.0 being pretty much at the low end of normal, they were even lower perspectively. When your body is in a time of crisis, ie: being sick, your steroid (cortisol) levels should be higher in order to help fight the infection and keep your body going. If Ian’s were 4.0 when he was sick, I wonder what they would be during times of wellness................. not that we know what that is here in the last four months.

Once we got home, Ian was to use the hypertonic and Duoneb for three days, every four hours. We noticed on the second day that Ian’s voice was very hoarse and he seemed to have laryngitis. He also had diarrhea and had small tremors. I know the Albuterol shakes, and this wasn’t them. We assumed Ian was sick, again. I thought for a moment and immediately went to review side effects of the new meds. I know these sites and apps like the back of my hand by now. Under Atrovent, I found: hoarse throat, pharyngitis, diarrhea, and tremors. Voila!!! It was a relief that Ian wasn’t getting sick, but now we were trading in for “sickey” side effects. Thankfully, Ian only needed to use the Duoneb regularly for three days, as he kicked the rest of RSV. Now, he hopefully won’t need it on a continuous basis for quite some time.

****************

The following day, Sandy Hook occurred. Suddenly, I was holding Ian a little tighter, kissing him a little bit more, and snuggling longer. In the midst of grieving for all the children and families, I was also feeling very thankful for the things I have. Suddenly, pharyngitis and the inconvenience of diarrhea didn’t look so bad. We have had times where Ian has almost slipped through our fingers.......... not that it would in any way compare to Sandy Hook! But the thought of losing a child isn’t a foreign idea to us. Sandy Hook aside, many families within our medical support community have angels. Children, like adults, can only fight for so long before their bodies simply surrender. During those scary times, Ian has always come out ahead. I can only hope that he will never find himself in a losing battle.

Tom and I can never express enough love and appreciation for our hugely supportive community. We wish each of you peace, love, and joy during the holiday season. January 1st is right around the corner....... a chance to start the year fresh and new. Best wishes to all of you!


Practicing his coloring skills

Looking a little too "GQ" in this pose!

Thursday, December 13, 2012

RSV 12.12.2012 Hospital Admission Day 4

Ian had another pretty good day today. Last night was the tough part. Even though he was without oxygen on Tuesday, he did need it again last night, as expected. We noticed some time before bed that he was arching and squirming a bit. He hasn’t done much of this at all since back in the reflux good ‘ole days. Tom and I believed it to be RSV related some how. The doctors seemed to think it was feeding related since he seemed a bit more distended in the belly than usual. I agree that it would appear to be that way, and can respect that some of it could be triggered by feeding routines, but this was simply not just “feeding related.” Ian has been having moderate feeding intolerance since...... well, ever! It doesn’t look like this. We see him everyday while he struggles with eating and this is not the child we see.

We stopped his overnight meal immediately for the remainder of the night. Ian *did* stop moving and squirming. Some of that was it was 3A already and Ian hadn’t slept yet. His body simply tuckered out..... another reason why he needed the oxygen overnight. He was already having “other” issues. It was decided to take an abdominal x-ray today to make sure that nothing was going on in the intestines that could be causing issues. I requested a chest x-ray. They agreed to do both. The x-rays came back with normal results.

Of course as soon as he fell asleep at 3:30A or so, it was only an hour before Tom was up and leaving for work. Respiratory therapy was in at 5:30A to do his treatments...... he didn’t budge. Ian usually fights these pretty hard right now. Sitting in your rocker at home with a book, mom, and dad while doing nebs is hardly the same as doing them in a hospital bed, with new equipment, and scary doctor people in masks. I also slept through the treatments. I slept beside Ian all night and have no recollection of them doing the treatments..... and nebs aren’t quiet.

Ian doing nebs two months ago with an asthma attack

Ian's nebulizer mask: Ian loves purple!!

Another reason I stayed in the bed close to Ian is for oxygen delivery. His cannula had irritated his nose so badly over the last three days that it would no longer rest in his nose. The swelling was too bad. As a two year old with swollen nostrils, dry from all the hospital air, having nosebleeds from coughing too hard, and skin so sore and frail, he wasn’t even letting the cannula rest under his nose. We all know Ian would never wear a mask overnight. They did decide to let him do a “blow-by” or a “flow-by.” This is when a mask or tubing of some kind is placed near and in front of the patient’s mouth and-or nose. It allows the “blowing” of air int he direction of the breathing patient. The doctors here do not recommend that as a typical oxygen supply because of its inability to wean. It cannot simply be titrated down by number or percentage.

I needed to stay awake enough to make sure the mask stayed nearby. The nursing station can see his saturation levels up front, but every mom wants to make sure herself that her child is getting enough oxygen. There wasn’t much sleeping going on last night. I am hoping tonight will be better.

The day was a good one. I was interested in what the pulmonologist would say about the previous night. He didn’t really have much input on the events. He did say that Ian sounded much clearer in his lungs. He felt as though tomorrow, Thursday, would be our discharge day as long as things continued to go well. He said maybe, if Ian had an excellent and long enough nap, with good saturations, that he could go home tonight. We never heard anything even after Ian had a great nap without oxygen and discharge did not happen.

As it would turn out, it was for the better anyway. More on that in a minute. Ian enjoyed the rest of the day playing, coloring, and watching videos...... same old, same old. While he was napping, Santa, Mrs. Clause, and Frosty came by to visit. Volunteers were visiting children and passing around gifts. I was sad that Ian slept through the visit, but he was sleeping so peacefully after a long night, I couldn’t help but be so happy he was resting finally. One of the nurses came in saying Merry Christmas and placed a pile of presents on the bed for Ian. There was a stuffed animal frog, a learning and musical plush soccer ball, and a Little People Farm set with a video included!

We were very surprised! Ian has been presented with so many gifts throughout his hospital admissions. We have enough blankets, stuffed animals, art supplies, toys, and keepsakes to make a special playroom out of them. Ian has been admitted to the hospital 13 times, including the NICU. I bet we have something special form at least 10 of those visits. The admissions have been in six different hospitals and I know we have gifts from all of them. While the gifts are from all different people and organizations, lots of them come from little old ladies that knit, crochet, and quilt these beautiful blankets for the children. Ian has a handful of them. One hospital even calls them “Project Linus.”

Toward the end of the day, Ian started getting fidgety. He was feeling well enough to want to be out of the bed and moving all around. He was getting into *everything!* The only attachment he had remaining was the pulse-oximeter for this blood oxygen levels. The nurses agreed that he could take it off and play for a few hours. When he went back to sleep, it had to go back on his toe. While we still weren’t allowed to leave the room, Ian liked helping close the door when people came in and out. He seemed excited just to be able to move around freely without his “pulse-oxygen leash.”

Once Ian was settled for bed, we had everything hooked back up for him. Sadly, his saturation levels started dropping once he fell asleep. We did some repositioning and shifting, but they weren’t coming up. The general consensus seems to be that it is ok for them to drop into the 80s, as long as they bring themselves right back up within a few seconds. When it stays in the 80s, then oxygen is needed.

We brought the mask back out for the “flow-by.” It seemed to help Ian and we used it for about 30 minutes. Then, I started weaning it away from his face. A few times, it dropped again, but after shifting him a bit, he started improving. It has been 90 minutes now and he has not dipped below 90, with no oxygen. I am hoping this is a sign of a good night to come. I pray each time I see the numbers drop that they’ll come back up. Lots of moms will tell you that they don’t get all worked up or bothered by dips in numbers unless they become a big deal...... and to a major degree, that is true. But, every mother notices when those numbers drop, every time. I would be surprised to hear of a parent who didn’t.

An example of Ian's monitor: pulse (70), oxygen level (100%), and blood pressure (116/56)

We still do not have anymore labs back from the blood work over the last week. We have a small handful of information, but nothing largely critical. I am hoping that we will be able to go home with Ian tomorrow. I miss our home. Whenever Ian is admitted through the ER, as lots of children are, I never go back home. I stay with Ian and Tom runs back home for the stuff that we don’t already have immediately packed for ER rush trips. For that reason, I never remember what shape the house is in, what was left in the washer and dryer, how many dishes were in the sink, or how many piles of papers and bills are on the desk. The mental shift renders all of that information as jurassic and long forgotten.

As the morning rolls in, I will do my best to keep the updates coming. There is something about once discharge comes, the updates get tougher to do. I would like to take another moment to express how thankful we are for each one of you. Ian was watching Muppets Christmas Carol today. One of the song lyrics said “If you need to know the measure of a man you simply count his friends.” It instantly made me think of how we are who we are as a family because of all of you. Whether you are abel to rad this message or not, you are how we are able to move along each day. The knowledge, support, empathy, and genuine love is amazing.

I often think of people I have never met and children I have never hugged when Ian is having troubles. I can picture them in my head and I know they are either having a tougher time, had a similar experience, or are praying for Ian. When a parent from my support groups says their little one pointed to Ian on the computer monitor and asked about him, I am overwhelmed. I then hear sometimes, that the child will say they will pray for him or that they feel sad he is hurting. 

“If you need to know the measure of a man you simply count his friends.”

It is truly restorative to know this support exists. In a world of rushing, narcissism, and disconnection, those small acts of love in the smallest of children are the ones that bring it all back together. We love all of you every day. Thank you.

Wednesday, December 12, 2012

RSV 12.11.2012 Hospital Admission Day 3

Monday night was certainly better than Sunday night here in room #558. Ian slept a little more peacefully and was able to wean down to three liters of oxygen per minute. Sometimes, one of the biggest problems with Ian getting a good night’s sleep is us. Trying to sleep two adults in a hospital room with a two year old may sound easy. Those of you who do it, know it is anything but that! One ends up in bed with the child and one on the “pull-out” of whatever type it might be. Well, two year olds never stay in one place and all all-over-the-bed!

Ian sweats like crazy and makes huge wet rings on sheets and pillow cases, causing whomever is sharing the bed to roll into them and become equally disgusting! Sometimes, you even venture there and then notice it isn’t sweat..... your child has just peed through the diaper because of all the extra fluids..... even MORE disgusting. One hospital we visit has a pull out double bed for the parents, pergo laminate hardwood floors, two tvs (child and parent) and a gorgeous tiled bathroom. ALL hospital rooms should be like these. It is very “home-y” feeling.

Plus, when you spend over 30 nights (34 so far to be exact) in a hospital as a parent, you learn to realize what is truly important and what really doesn’t matter. Such as..... soft sheets and pillow----- important........ being able to change into *real* clothes and get dressed for the day----- not important....... having decent food available----- important....... (although you never get to eat it hot, so the correct temperature of food becomes totally irrelevant) having time to check your email for midnight because of bad wifi (which always happens)------ not important. Each experience is different, but over time, the good and the bad start to stand out.

Aside from people coming in every 20 minutes for something between 3am and 8am, the night was good. Medical staff really does need to get it together at these special times. Especially since Ian had blood drawn at 6:45A for steroid testing. However, when he woke up, he was as fresh as he would have been on any other day. He spent the morning smiling and giving kisses and high-fives. We colored and watched videos together. Grandpa was here again today, Tuesday, and was a great help to ME! Trying to accomplish *anything* while alone with a two year old in the hospital is impossible. You would think again, that being in a confined space would make it easier, but...... certainly not!

Ian is still in isolation because he is contagious. Meaning, none of us can venture out of the room except to leave and enter the hospital. ICK! It gets really claustrophobic in hospital rooms under isolation, especially with stagnant, dry, smelly hospital air. I’m painting quite a pretty picture huh? To be honest, being in the hospital has become one of the safest feelings for Tom and I. While being one of our least favorite feelings, knowing that you are somewhere that your child can be taken care of is very reassuring........ assuming you trust the doctors and the hospital........ been there, done that bad road too.

The pulmonologist and respiratory therapist were able to remove Ian’s oxygen this afternoon. Around 3P he was good to go and held his saturation levels up above 92 the whole afternoon. For those who might not know (since this is a general blog, support group, facebook update) that means that 92% of hemoglobin (oxygen transportation portion of the red blood cell) is oxygen. It gets much more technical, but that simplifies it quite a bit. Ideally, the saturation is 100%. Below 94% is unacceptable in most cases, and usually below 92% is a problem. When entering the hospital, Ian was between the upper 70s and mid 80s...... extremely unacceptable and depriving his brain of vital oxygen.

Overall, Ian had an AWESOME day!  Big improvements and happy.  He is so strong!

Snuggles

"CHEESE"

Books with Grandpa

Once bedtime came, we knew things would change. Respirations (breaths) slow down during sleep and patterns change. Oxygen levels often drop. This happened *a lot* with Ian back in the NICU. And, I remember myself, after delivering, I was hooked to lots of equipment, including a pulse oximeter to measure this. A threshold level is set. When it reaches whatever the setting is, the monitor alarms. Each time I would finally fall asleep, the saturation levels would drop, and the alarm would immediately wake me again. With an luck, sweetness, and good nurses...... alarms can be silenced on your side of the monitor. With Ian, we always ask that they be ON.

This means when Ian’s blood saturations drop below an acceptable level, he alarms. Which..... we hear...... Ian sleeps. The flashing red box makes it easy to see the numbers in the middle of the night. There are different alarms for oxygen, heart rate, respirations, heart contractions, and more. As experienced parents, we know them all. I know enough to hear the respiration alarm at 2A and know that if I only hear it beep once or twice, the leed wasn’t in the right spot when Ian rolled over. However, I can tell you that no matter how many times it drops and comes back up..... a parent’s heart skips a beat when the blood saturation alarm goes off. Sometimes it’s one beep, sometimes for minutes..... but every time..... parents notice.

As Ian fell asleep tonight, his oxygen started as OFF. We knew the instant he fell asleep. His eyes closed, he took a few breaths, then slowed down. twenty seconds later, the number started dropping. Thirty seconds in, we knew he needed the oxygen. We gave it a few “rustles” of Ian to make sure it wasn’t positioning on a fluke. He is sitting at 1.5 liters right now as I type. being half of last night’s dose, that’s pretty darn good....... 3 days into an RSV diagnosis, especially a severe one with originally partially collapsed lungs. I have no idea how these little kids do this!!

Tomorrow will be another day..... hopefully another day with oxygen! If all continues to go well with his continued breathing treatments and downgraded oxygen levels around the clock, we may be abel to go home Thursday. I’m guessing Friday maybe... who knows. Ian needs to prove he can keep his saturations up both during the day and at night, and still accepting his breathing treatments well.

There were a lot of labs run this morning and last week. We have some results in, but it doesn’t make much sense to discuss them lab-by-lab. Once we get all the labs returned, we can get into that fun stuff. One shows a significant result, but most are within normal ranges.

Thank you everyone for always keeping Ian and Tom and I in your thoughts. He truly does soak-in all those prayers. He pulls strength from what seems like no-where. I know where it comes from....... everywhere! He has kept “reserves” too for times like these when he needs a little extra umph! I will keep everyone updated as the days pass. Thank you again!

Tuesday, December 11, 2012

Beaner-Bot & His MiMi- RSV Admission

Beaner-Bot & His MiMi...

Dear Ian: 

I wish you weren't so far away, 
And having such a tough, hard day. 
I wish that I could hug you tight, 
And tell you stories before "Good nights." 
I miss your giggles, and "uh-oh"s, 
Your eyes, your cheeks, your little toes. 
I can't wait until we are feeling well, 
Boy that day will be so swell! 
But until then just know you'll be, 
Constantly loved by your MiMi... 

I love you little buddy!! See you soon I hope! ♥ Meems 



Dear MiMi: 

I love my MiMi when she’s near, 
It’s oh so great to have her here. 
I like to call her A-Mi-Mi, 
Cuz she’s my special aunt you see. 
We take long naps and snuggle tight, 
She always kisses me goodnight. 
And when I’m sad and have bad dreams, 
I curl up close and hug my “Meems.” 
But when she is so far away, 
In my heart she’ll always stay. 

I miss you Meems..... but I’ll see you, in my dreams! ♥ Beaner 


***For never was a story of more love you see............ Than Beaner-Bot and his MiMi!***


Monday, December 10, 2012

Growth Hormones, Metabolic and Mitochondrial Diseases


In addition to the RSV, we spoke with his Endocrinologist at CHOP today and will be running cortisol and ACTH labs in the morning, along with some labs to look for metabolic and mitochondrial disorders based on his issues. It is best to test these when you are sick. The cortisol will simply show an elevated level..... indicating that it would normally be lower. They would want it to be a more "
normal" level now if he is deficient regularly.

We will be beginning growth hormones in January (after the paperwork is completed). It is based one is Intrauterine Growth Restriction before birth and his small for gestational age at birth (measured 26 weeks even though he was 29 weeks).

Ian has had pretty much less than 10% catchup growth. it should be closer to 90-95%. We have reviewed the risks and have decided that based on his systemic issues as well as the growth issues, he would benefit from extra "growth" per say. It can help organs grow, change appetite, and even help with immunity in some cases. Ian is a good candidate to start earlier.

We did get some results from the bone age x-ray done at CHOP about 2 weeks ago. he is measuring a little behind. No exact range was given, but the way he mentioned, I would guess about 4 months or a little more. He said this mostly is to supply a good baseline for future bone age x-rays.

They will be running Ian's labs in the morning. The geneticist has some she would like to run while he is sick for metabolic and mitochondrial disorders. Also, the Endo will be running the cortisol labs. The cortisol and ACTH should be higher since his body is in crisis. A "normal" value would indicate that typically his body is much lower and would indicate deficiency. The concern is that Ian is on many steroids for his issues, that it would suppress natural adrenal gland steroid production biologically.

While not a HUGE deal at this point.... when he comes off the prescription steroids, he will then be deficient, and would most likely need supplementation. It is all up in the air right now. We are also waiting for the immunity labs to come back to show any immune system deficiency. Antibody levels may be low, and not recognizing previous illnesses. instead of protecting them, they float around and aren't doing their job...... or there aren't enough to keep up with the workload.

Mitochondrial diseases and metabolic diseases sometimes overlap in category. Fatty acid oxidation disease is a type of mitochondrial disease. This occurs in a small group of HELLP syndrome born children.

More on all those details as they come up.

Mitochondrial diseases


http://www.umdf.org/site/pp.aspx?c=8qKOJ0MvF7LUG&b=7934627

Fatty acid oxidation metabolic diseases


http://en.wikipedia.org/wiki/Fatty-acid_metabolism_disorder

Mitochondrial Fatty Acid Oxidation Diseases


RSV 12.10.2012 Hospital Day Update

Ian had a so-so day today. It was very much like yesterday, only with some better management. The pulmonologist was not thrilled with his oxygen saturation levels, but they were acceptable. Ian remained on 4 liters of oxygen per minute until late this afternoon. The pulmonologist was happy enough with the treat,nets he had been receiving to lower the rate to 3 liters.

Ian has been receiving breathing treatments in the form of racemic epinephrine nebs, hypertonic saline nebs, and easy-pap device, and supplemental oxygen. He has started to leave the nasal oxygen cannula alone, with the exception of when people move it around or reposition it. Most of the time, it remains in the right spot.

Every 4 hours, the respiratory therapist comes in for about 20-30 minutes. The epi nebs come first and take about 10 minutes or so. Ian is used to the make at home, but is much more scared and sad here, understandably. Then, they use the easy-pap. It is a breathing device that looks like a spacer for inhalers. In a few sets of about a minute or so, he breathes with the device, which creates positive pressure to help inflate the collapsed parts of his lungs. After both treatments, they listen to his lungs again, and suction his nose with the most powerful "boogie-sucker" in the entire world! I would actually love having one of these at home..... Of course, Ian hates this as well. But it removes a tremendous amount of junk.

After that, he sounds much better. The "snarffy" noises are gone and he breathes deeper. Then they do the hypertonic saline nebulizer medication. This medication has a high salt concentration to help reduce the viscosity or thickness of secretions. It helps them move secretions better in order to help break them apart. It is then easier to expectorate them and increases breathing efficacy.

Hypertonic solutions are ones with higher salt concentrations within the cell. When immersed in an isotonic (equal) solutions, such as body fluids for the most part, water from the isotonic cells rushes into the hypertonic cells in an effort to balance the equilibrium. The hypertonic solution breaks up what are called disulfide bonds in the mucus...... Loosening the mucus as a result.

Ian's blood oxygen levels are much better after that, and he is more comfortable. These are ordered every 4 hours..... After about 3, he starts picking up a little trouble. The treatments make him cough because of the sensations they create by the anatomy changes in his lungs and trachea. They are not "made" to make him cough, but do as a side effect. When he coughs, it is much wetter and his is able to move secretions around much better.

Ian does not spit these out for probably various reasons...... He is 2, with oral aversions, and previous reflux..... So he fights it. He swallows most of them, creating a fairly gross mucusy stool. Not much fun either. He has not shown much change in activity level or energy today. He has little interest in getting down off the bed and seems pretty weak. He likes watching his videos, coloring, reading books, playing with his toys....... And most of all, snuggling!

Now that he is sleeping, the oxygen levels are going down again. It is unsure at this point if he will need to go up on the oxygen levels again. We heard day 4 is usually the worst, and that is most likely tomorrow based on his time frame of symptoms. Tomorrow is another day.... And things change by the hour it seems sometimes. We are continuing to keep good thoughts of our little fighter who is so much braver than I could ever dream. We are so proud of him!

RSV 12.9.2012 Hospital Overnight Update

Ian's overnight update:

Ian is not doing well overnight. Overnight is typically worse, but seeing as though his case is diagnosed as severe, it makes it so much harder. He is back up to 4 liters of O2 and is still having a moderate amount of trouble. His chest x-rays showed partial atelectasis, partial collapsed lung, in both lungs.... Making it even harder for him to breath.

He has a lot of secretions and exacerbates them by his hypersensitive vagus nerve and all the coughing. He doesn't expectorate the secretions because of the dysphasia sensation and oral aversion, so he ends up swallowing them. With the dysphasia, this usually induces more coughing. Thankfully, the x-rays did not show fluid buildup in the lungs.... So, at this point he seems to be holding off from the pneumonia. RSV is one of the most common causes of pediatric pneumonia.

The respiratory therapist is doing an easy-pap treatment to try and help open the collapsed parts of his lungs. If he reaches 5 liters and is unable to respond to the easy-pap or other therapies (which are unknown to us at this point) they consider intubation.

Ian still seems very uncomfortable and sickly. For Ian, this is rare even when he is sick. This is the sickest we have seen him and he seems to be really struggling. Yesterday during the day was better, but we were told that sometimes they get worse, and then usually improve. Ian's allergy tests for immunity have not returned yet. They were drawn on Tuesday of last week. Hopefully, we can get those results soon and the doctors can assess he needs based in his level of immunodeficiency.

Thank you again for all the thoughts and prayers. With Ian's previous prematurity, chronic lung disease and the reactive airway disease-asthma, we are certainly not falling into a fantastic category. Most children do not need to be admitted for RSV. Of those admitted, only a little less than half really need to have oxygen. Ian is on a pretty high dose right now and we are praying that it is an overnight-sleeping exacerbation.

Atelectasis:http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001130/

Sunday, December 9, 2012

RSV Admission Day

Ian has been admitted to Fairfax hospital for RSV. Hospital admission #9 for 2012. Ian has been sick for almost a week now and things went down hill yesterday pretty quickly.

The stomach virus he had made a turn for the worse. Overnight, his blood oxygen levels measured accurately in the upper 70s and low-mid 80s..... Very unacceptable!

He is on 4 liters of oxygen which is pretty high. He is still able to get all his fluids and food through his G-tube for now and has no IV. If he stays stable, we can probably dodge one.

It's going to be a good number of days before he can go home safely. After all the respiratory issues in the last 4 months, I wouldn't be surprised if they send Ian home with oxygen this time.

Please send lots of hugs and positive thoughts for our sweet little boy who has been sick since early September..... One thing after another, with only a week break over Thanksgiving, thankfully! ;)

We are waiting for a room and I'll make sure to keep everyone updated.





Saturday, December 8, 2012

Short Stature: Petite, Hormonal, Genetic, or all of the above?

Ian has always been on the petite side. He was born by emergency C-section from maternal severe HELLP syndrome. Although Ian was born early because of my issues, Ian had been having issues of his own. Since he was 20 weeks gestationally, he was measuring small. At birth he measured 26 weeks gestational age, even though he was 29 weeks. At 930 grams, 13.5 inches, and a head at 26cm, Ian was our little peanut. Not much has changed in the peanut department.

Ian continued having growth issues in the NICU. Thankfully, he was able to come home at 35 weeks gestational age. It just so happens, he was only 3 pounds 13 ounces. Miraculously, he didn’t need any monitors, machines, or equipment. We have been very fortunate. Since being home, Ian has been growing in hops and skips instead of leaps and bounds. While making net gain, the teeter-totter growth has placed Ian far behind in the preemie “catch up growth” department.

Once Ian’s reflux-motility was under control in December of 2010, he grew wonderfully until June of 2011. Since then, he has been all over the place. The reflux surgery and G tube in September 2011 helped for about five months or so. Since March of 2012, Ian has gained about 1.5 pounds. One and a half pounds in 10 months is very little.

We have been working with many doctors, therapists, and dietitians on Ian’s food and over nutrition. Ian has been able to keep his blood glucose stable since a couple months after the initial reflux surgery. His side effects have been significant. For that reason, it has been hard getting enough calories into his little body. Since he cannot tolerate the osmolarity (density), volume, or delivery of many formulas, finding a good one has been troublesome. Playing with feeding schedules complicates things even more.

The thing is, a lot of times anyone can pin why Ian isn’t growing, ie: illness, surgery, feeding intolerance, volume intolerance, blood glucose intolerance. The problem is that it is simply one thing after another that is keeping him from having the chance to grow........ or so we thought. 



Apparently, that may not be all that is going on. Many people have asked us in the past if “they” (doctors) think that all of Ian’s troubles are due to his prematurity. We always reply yes..... because we had always been led down that belief. As it turns out, there might be other contributing reasons. Ian’s Endocrinologist and feeding therapist both mentioned on separate occasions a syndrome called Russell-Silver (RSS). RSS is a form of primordial dwarfism- meaning proportional growth in body parts and organs. RSS is characterized by short stature without catchup growth, normal head size, triangular face, low set ears, and other physical characteristics.

RSS has both genotype and phenotype. Genotype is how the DNA presents. Phenotype is how the appearance presents. RSS can be genetic but doesn’t have to be. It can occur randomly. There are genetic tests that can diagnose about half of RSS cases, but not all of them. RSS children and adults have many phenotypes. Some show more physical characteristics than others. Some have mostly size and systemic characteristics. Since there is no perfect definitive diagnostic test for all cases, many diagnoses are a clinical judgement call.

Distinguishing characteristics of RSS kids from other Small for Gestational Age kids (SGA) are: 
1) Body asymmetry: one side from another
2) Inadequate catchup growth from infancy to age two
3) Persistent low weight for height ratio
4) Lack of interest in eating enough calories
5) Hypotonia
6) Broad forehead
7) Large head size for body (head on normal growth range)
8) Small chin and possible tooth crowding
9) Downturned corners of the mouth, thin upper lip
10) High arched palate
11) Low set ears
12) High pitched voice as child
13) Inward curving of the fifth finger
14) Webbing of second and third toes
15) Abnormal openings of the penis- Hypospadius
16) Cryptorchidism- undescended testicles
17) Cafe-au-lait birth spots
18) Dimples in posterior shoulders and buttocks
19) Narrow, flat feet
20) Scoliosis

Ian has #2, 3, 4, 5, 6, 7, 8, 10, 11, 14. This is 10 out of the 20 characteristics....... enough worth looking into. The 10 noted are ones that were noted by clinicians, not just us. There are also characteristics of SGA kids in general that are seen more often in RSS kids:

1) Fasting hypoglycemia and mild metabolic acidosis
2) Intestinal movement abnormalities: reflux, delayed gastric emptying, slow intestinal movement
3) Blueish tinge to the whites of the eyes
4) Frequent ear infections
5) Congenital absence of second premolars
6) Delay in gross and fine motor control
7) Delay in speech
8) Kidney abnormalities
9) Delayed bone age
10) Early public hair and underarm hair
11) Early puberty
12) Classic or neurosecretory growth hormone deficiency
13) ADD and special learning disabilities

Ian has #1, all of 2, 4, some of 6, 7, and possibly 9 and 12. Again, ones mentioned by doctors or therapists and also noted by Tom and I. And yet again, worth looking into.

There are certain diagnostic criteria for a clinical diagnosis, independent of a positive genetic test. The PubMed criteria are listed below:


Three of the four major criteria must be met (which Ian does) 

OR 

Two major and two minor (Ian has three major, two minor) 

He also happens to have three of the five supporting criteria 


As you can see, if totally based in clinical diagnostic criteria, Ian certainly fits into the group. However, it isn’t *always* that easy....... but then again, worth looking into. There are a few doctors in the world who have made RSS their life’s work. The prevalence is 1 in 75,000-100,000 births. Because of the rarity of the condition, finding specialist is hard to do. There happens to be one nearby in Manhattan at Mt. Sinai Hospital. The lady there is an Endocrine Geneticist. Families travel from all over the world to meet with her and confirm or rule out a diagnosis. For some, it isn’t that easy. We were once told that children “grown into their phenotypes” as they age for a while. That being said, genetic diagnoses are often a work in progress if it doesn’t jump-out-at-you right away.

We have scheduled to see Dr. Harbison in Manhattan in January 2013. All of Ian’s growths and requested documentation has been sent ahead of time in order to escalate the process. I addition, we have also met with a clinical geneticist here in Virginia to establish some genetic testing. The trouble is, while looking into genetic testing for short stature, RSS isn’t the only concern. There are other genetic conditions for short stature and other characteristics. Ian has been tested for some of those conditions.

Other labs have also been run for him. Metabolic panels are a concern due to maternal HELLP syndrome. There can be a correlation between HELLP in the mother and Fatty Acid Oxidation Disorders (inabilities to digest and process fats). Ian has some of thees symptoms, but not a lot Also, mitochondrial labs are on the table. Ruling out metabolic and mitochrondrial diseases rules out a large chunk, at least for the here-and-now. Ian has had the metabolic and mitochondrial tests done, or is having them within the next few days. We do not have any results at this time. In addition, Ian had some allergy and immunity panels run the other day. Immunity and antibodies, along with allergies can identify other concerns. It sure would be nice if there was a way to collect enough blood to be able to run ANY test needed. Ian’s growth hormone testing will help determine is path of hormonal therapy.

Ian has visited with both his clinical and metabolic geneticist, and his allergist in hopes of taking care of a lot of these labs. We are hoping that Dr. Harbison, as an Endocrine Geneticist, will be able to help take control over both geneticists and endocrinology. I’m not sure if that will happen.

Despite what might be going on genetically, if anything....... Ian is certainly a candidate for growth hormones. This comes from his lack of catchup growth, as well as his SGA diagnosis and also failure to thrive diagnosis. He is getting ready to have his cortisol and ACTH (growth hormone stimulation test) labs done soon. Last week, Ia had a bone age x-ray taken. By looking at the radiograph, the “age” of a child can be determined. if the child isn’t measuring what they *should* be based on their age....... they show a delayed bone age. The bone age can also let doctors know how close a child or teen is to the end of growth. it can even help determine the estimated overall height of a child or teen. We do not have any results back on Ian’s bone age x-ray yet.

Overall, there are many short stature and failure to thrive issues that can be investigated. While we are hoping that none of them will pertain to Ian, only time (and a little testing) will be able to tell. For Tom and I, two type A, definitive, black-or-white type people, this is frustrating. Clinically, we are told that getting a diagnosis helps get faster treatment, makes treatment easier with insurance, and helps “name” what you are going through. We are also told that in most cases, a syndrome is described. A syndrome simply means a collection of symptoms. Well, symptoms are things that are simply treated, or used as part of a condition. Needing a diagnosis doesn’t necessarily keep you from treating whatever the symptoms are. 

More information about growth deficient conditions can be found at The Magic Foundation.

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~



We don’t know what Ian’s test results will show, or what any Geneticist or Endocrine Geneticist will decide concerning genetic diagnoses. Regardless, we have options with Ian and we have an excellent team working with him. Thanks to great family, friends, and the wonderful support groups on Facebook, we have quite a support team going on here. Each of Ian’s issues has a group online of mommies and families that are there to offer knowledge, support, and experiences to others. I am truly grateful for each of them!

I have to admit, sometimes they know more than the doctors. It’s like going to a doctors appointment with every die-hard-advocating-mommy you can get your hands on! Pretty awesome! As we know more, I will be able to elaborate on the issues. Right now, things are up in the air. The only genetic/growth issue that is really front and center on the table right now is RSS (and his already diagnosed SGA).

No matter what, Ian is our little peanut. Despite a diagnosis, or lack of one...... he will always been the miracle child that gave us an opportunity to do work worth leaving a mark. Standing up for Ian has proven to be the most self-challenging and difficult task of my life...... but it has also been the honor of a lifetime.


A Triple CHOP

Thursday was a day of productive appointments. Ian visited the Diagnostics and Complex Care department, the GERD clinic for troubled Nissen kids, and Endocrinology. Back-to-back morning appointments kept us on our toes. Each had something to offer Ian’s plan of care.

DIAGNOSTICS/ AKA: Dr. House!!

The first appointment of the day was with Diagnostics. Excited to see our “Dr. House” for the first time, we waited impatiently. We didn’t have to wait long as we were the first appointment of the day. Dr. Pasquariello and Dr. Magnusson have been at the clinic for a very long time. Dr. Pasquariello was appointed in 1963. We had the opportunity to meet with Dr. Magnusson. Our goal was to look at Ian from a Wholistic point of view. With Ian’s multiple diagnoses and issues, we thought having a global view of everything would be beneficial....... and man was it! We were impressed.

He had a lot of good things to say. He was able to point out a few things we are doing that are helpful and/or necessary. He also gave us some good advice on things that are most likely NOT issues and what can be discarded. Most importantly to us, he was able to rule out almost everything from an all-encompassing standpoint. The only unifying diagnosis would be Russell-Silver Syndrome (RSS). I will have a specific Russell-Silver BLOG post very soon....... but Genetics Home Resource describes RSS as:

Russell-Silver syndrome is a growth disorder characterized by slow growth before and after birth. Babies with this condition have a low birth weight and often fail to grow and gain weight at the expected rate (failure to thrive). Head growth is normal, however, so the head may appear unusually large compared to the rest of the body. Affected children are thin and have poor appetites, and some develop low blood sugar (hypoglycemia) as a result of feeding difficulties. Adults with Russell-Silver syndrome are short; the average height for affected males is about 151 centimeters (4 feet, 11 inches) and the average height for affected females is about 140 centimeters (4 feet, 7 inches). 

Many children with Russell-Silver syndrome have a small, triangular face with distinctive facial features including a prominent forehead, a narrow chin, a small jaw, and down-turned corners of the mouth. Other features of this disorder can include an unusual curving of the fifth finger (clinodactyly), asymmetric or uneven growth of some parts of the body, and digestive system abnormalities. Russell-Silver syndrome is also associated with an increased risk of delayed development and learning disabilities. 

RSS is something we were already thinking about. After two different providers brought it up and two others confirmed plausibility, it has been on our minds. More on that later.......... 

Russell-Silver Syndrome- MAGIC Foundation

GERD CLINIC

Our second appointment of the day was with general surgery, the GERD clinic. We are always happy to visit Dr. Blinman and our Dietitian Robin. CHOP has been a lifesaver, literally. After everything they have done for us over the last year, Ian is in a far better place. Twelve months later, we were still dealing with retching. While this wasn’t the type of retching the drops oxygen levels, it was significant enough to elicit sweating, distress, and changes colors to red and purple. No one wants to see their child in that much distress, everyday.

On a recent visit to Ian’s pulmonologist, the retching came up in discussion. There are many pulmonary manifestations of reflux. With all the throat irritation, vagus nerve irritation, and esophageal irritation that can cause chest congestion and lung inflammation, the connection between pulmonary and reflux is well known. Also, ear, nose, and throat disorders can present form the irritation and congestion/inflammation/mucous.

Pulmonary manifestations of gastroesophageal reflux disease

Our pulmonologist has always been an excellent resource. There seems to be a large overlap in some of Ian’s team members, and large gaps in some others. We decided to try Ian on the erythromycin again. He previously used it from age three months to nine months. We stopped because it simply stopped working. It seems that there is a tolerance effect that occurs. Typically, there is a protocol of an “on-off” schedule to help keep the medication working.

Erythromycin, as you can remember, is an antibiotic...... an antibiotic that causes stomach cramping and diarrhea. GI has made that side effect “therapeutic.” By administering in a low dose, the stomach contractions that usually produce cramping still occur, but on a much lower scale. These contractions help the stomach empty quicker. This has often been used as a therapy for delayed gastric emptying. When drugs are used for this effect, they are called Prokinetics.

What is a Prokinetic?

Almost three weeks ago, Ian started a regimen of erythromycin. Ian has not retched since we started the medication. Tom and I hate to even talk about the great news since we are always shot down weeks later. We are hoping that since Ian had good results before, it can happen again. 



While not the original intentions of the CHOP clinic, they were willing to help work with us. The clinic’s philosophy is to use food and supplements, instead of medications, to help with symptoms. It seems as though we have been continuing to struggle. The erythromycin has helped Ian’s motility. By helping the food “move along,” he is accepting his meals more easily.

The dose of the erythromycin needed to be titrated to the lowest effective dose. Since erythromycin typically causes diarrhea from abdominal cramping (such as when taking it as an antibiotic), a minimal dose is desired. Ian has been doing very well so far. Soon, we will be taking an “off” time before restarting.

Ian’s calories were increased overnight so that he can get some “extra” calories before we visit the DAY program at Children’s Hospital of Richmond. Ian is in need of more calories. Hopefully, he can get some growth out of them.

ENDOCRINOLOGY

The last visit of the day was with Ian’s Endocrinologist. We were following up with him concerning Ian’s growth and failure to thrive. We are in the process of reviewing growth hormones. Ian would not begin until age three or four. Despite where we end up with Russell-Silver syndrome, Ian will most likely be undergoing a course of growth hormones.

He ordered a Bone-Age x-ray. This will help them identify where he is in the growing stages..... and can be compared to others later. It can also determine the ultimate growth of a person. This is useful for growth hormones as well as other growth disorder determinations. We completed the Bone Age while we were at CHOP. The Endocrinologist also ordered the Russell-Silver genetic testing.

What is a Bone Age x-ray?

An example of a bone age radiograph at 24 months of age
There are multiple options available when it comes to meeting Endocrinologists and Geneticists in this arena. There is an Endocrinologist in New York City specializing in Genetics, Russell-Silver in particular. Patients travel from all over the world to see her. Many children with this growth syndrome do not show an all-or-nothing yes or no answer. Her expertise includes making a knowledgable diagnosis, or exclusion. More information will follow in the RSS BLOG. A certain percentage, about 10-15% will show on genetic testing, but the other cases are based on clinical diagnoses.

The Endocrinologist at CHOP also ordered testing to evaluate Ian’s cortisol steroid levels. Ian is currently on Flovent, Flonase, regularly and Triamcinolone cream occasionally. The trouble is that being on a certain amount of steroids can suppress your body’s natural steroid production. A blood test will look at current levels. We will take care of this test later on. It must be done between 5A and 8A due to the body’s natural production of steroids.



After a full day of meetings, we headed back to Virginia. It felt good to be home!

Friday, November 30, 2012

My Thanksgiving Butterball

Thanksgiving is a large holiday for our families. As long as I can remember, I have been doing the “double dinner.” Growing up we always visited both sets of grandparents. When I got older, one set of grandparents came to live with us......... and so came all the family Thanksgiving guests. As a child, there were thirty or more people hustling all about for the holiday. Having the party move our to our home was a blast...... at least as a kid with little cooking responsibility!

I was always excited to share the Thanksgiving mania with my children. Last year was the first year we had the opportunity to do that. Being able to make it tradition this year was even better. Having him as part of both family dinners is amazing. Fortunately, our families are both within a 90 minute drive from us and each other.

Watching Ian play with Thanksgiving feast is pretty awesome. He is very interested in picking up the kernels of corn and touching the mashed potatoes. His actual Thanksgiving dinner was Cheez-Its and Cheese Curls. A true feast for my little butterball.

There were a few beautiful days around the holiday and we were able to take Ian outside. I was able to catch some great family moments on camera.

Contemplating life with Grandpa

Running around

Watching the Parade

Playing in the leaves

Horsing around with mommy

Family Photo

Slide time

Tunnel

Sporting my penguin hat

Playing with glowing battery candles

sesamestreet.org

Grandpa, Nonna, and Uncle J

World Prematurity Day 11.17.2012

New York, New York

World Prematurity Day is a day to remember the more than one million babies who died this year worldwide, from being born too soon. Tom and I are very blessed to have our little preemie miracle with us everyday. I wanted to share some of the photographs our family created to honor the special day. I also wanted to share some awesome photographs of the world recognizing the special day.









Canada

Prague

Italy

Turkey

Australia

Macedonia

Poland

Italy

Dublin