He has been eating all kinds of new foods including steak, fish, and even crab cakes (Maryland ones of course!) His favorite food is still "Kit Kats" he always says. He even wanted to put that as his favorite food for his preschool assignment! He still takes a *VERY* long time to eat. But, he is eating, and pacing himself was part of our new plan. He gets to call more of the eating shots (with some parental influence).
He still eats most of the day, but we try and get four meals in, and some snacks. He seems to be eating well at Preschool also. I think seeing all the other kids eat lunch is exciting and he wants to eat too. The other children don’t seem to care, or even notice that he eats a bit differently. I try and pack him as “normal” of a lunch as possible… but sometimes it’s nothing but pretzels, a couple noodles, and a Kit Kat bar. I want to make sure he eats whatever I send, so I’m careful about my choices. Thankfully, his teachers are in-the-know. Otherwise, they may think I don’t send enough food for my kid to eat! The drink of course, always comes home. The kid doesn’t like to drink!
Thankfully, we don’t see this face very much anyone...... but rather enjoy seeing the new face!
He still struggles a lot with consuming enough fluids in a day. Because of his visceral hyperalgesia, Ian has increase bowel and digestive discomfort. As I have mentioned in the past, it was best described as Fibromyalgia for the organs. Things we do naturally with digestion (and don't feel) can be very painful for him..... even having the slightest bit of food in his stomach has the chance to cause a lot of pain.
We still give him water through a syringe everyday.... much to my displeasure. He needs to consume over 37 ounces a day in order to keep his bowels happy, and comfortable. That is a lot of fluid for a 36 pound body. Admittedly, I don't always drink that much in a day. It is very hard for him to drink when he isn't thirsty or hungry. I wouldn't want to either. So we try and space it out for him..... with water pretty much every hour either by syringe, cup, or fruit/yogurt. He does love popsicles and Italian ice, which helps a lot in the Spring and Summer months. It is incredibly time consuming.... but, some days are better than others. We very much use our reward system for fluids, just like we did with food.
He did very well this summer with heat tolerance. Two summers ago there was a lot of vomiting and heavy sweating. Last summer was mostly kept under management with a cooling vest handmade from an old friend of mine. This year he had outgrown the vest, but we decided to give it a go without one and see how he did. Thankfully, we had a mild summer without hardly any brutally hot mid-90s and higher days. He only had two instances with vomiting from heat and they were both after being outside almost all day. The more time he spends outside, the more we have to push fluids. We max him out at 44 ounces.
Ian’s Gi system seems to be moving happy along, literally. We have been able to remove his motility meds and he seems to be working within happy management. His gastroparesis is not causing any many troubles at this time. We have been able to keep it well managed with smaller meals and an increased frequency of snacks. As far as we know, he isn’t showing any active reflux. His vomiting os down to once or twice a month. That has been an enormous improvement from the 63 vomits back in February! He tends to vomit mostly with large food quantities, of course. Fluids must be taken into account when it comes to occupying stomach space. Sadly, fluids also take a long time to leave the stomach in motility disorders.
He has been able to keep his bowels happy with the increased water intake. His lower GI system has never had as severe problems as his upper GI, but they are still enough to cause lots of discomfort should they not be taken care of properly. Right now, Ian is not taking any artificial or natural stool softeners or regularity medications.
We recently were able to get rid of two mucus targeted meds. We first switched from the Benadryl to Zyrtec. Benadryl is a mild appetite suppressant. With us letting Ian call more of the shots with eating, we wanted a medication that wouldn't stifle his hunger at all. We did cut a medication out completely. His Periactin is gone. This is the mild anti-histamine that was helping decrease the mucus production that was causing him daily morning retching and vomiting. As it was phased out, Ian had no problems. He is now Periactin free.
Ian seems to be managed well with his emergency respiratory meds. We haven't had to use the nebulizer since February. His rescue inhaler has also only been used a couple times. He is currently on a Pro-Drug everyday inhaler. This means that the drug is taken in, but only activated in the lungs. I know that sounds weird, but some part of all inhaled medications make their way into the esophagus and down the digestive tract. In the past, this was a good thing for Ian because it successfully help manage inflammation from reflux. (Which is why reflux is such a pulmonary disease).
About a year ago, Ian was diagnosed with mild Adrenal Insufficiency. This means his Adrenal Glands do not produce quite as much natural steroid as normal. The more artificial steroids you are exposed to (systemic, inhaled, illness prescriptions, etc.) the higher the chance of you not producing as much on your own. When your body takes in artificial steroids, your body suppresses natural release. But, when the artificial ones are removed, your body doesn't always pick up the slack, leaving you deficient. This is usually from chronic steroid use (good or bad). The new Pro-Drug inhaler we switched to keeps steroids out of the blood stream as much as possible.... preventing his body from suppressing natural steroid production as much as we can.
Also endocrine related... we visited them in June. If all goes well with illness avoidance and nutritional growth, we may be able to do a trial off the growth hormone (or “Super-Juice” as we call it). It is hard to tell if nutritional progress or growth hormone, or both, have been contributing to his growth success. Ian has Small-For-Gestational-Age and Intrauterine-Growth-Restriction diagnoses. He was previously Failure To Thrive and is also currently under a Short-Stature diagnosis. All four of these diagnoses put him at a lifetime disadvantage to “catch-up” “typical” growth patterns for a child. It is hard to know if good nutrition would be enough to keep him on tract or not, without trying it first.
With Ian currently in the 50% for weight and 25% for height, the next year or so is a good time to take that chance. Those numbers seem great, and they are, but they are only great because of all the help he is getting from food and hormones. It is hard to tell if he could maintain without hormonal supplementation. He is not growth hormone deficient, but rather the normal amount he produces isn’t adequate for him to grow. His linear growth curve (steady pattern) is beginning to even out now, meaning he is falling into his pattern and we want to keep him there. We can always restart the growth hormones if we needed them.
Speaking of illness, since Ian hasn’t been sick, we haven’t needed to give him steroid supplements to help him recover. I know it sounds like a catch-22, and it is with steroids. Ian doesn’t make enough steroids to fight off infections most of the time because of his adrenal insufficiency. This is because of past high steroid exposure in his much needed medications. This, in turn, makes him steroid deficient when he comes off the illness-helping steroids….. but, then that deficiency makes him more prone to infections. So, illness steroids are great to help you get well in recovery, but can hurt you by predisposing you to future infections. Thankfully, Ian has been steroid free for nine months now. Last year, we used them for over 30 days of the year because he was sick so often.
Ian just received his fourth pair of SMO orthoses for his shoes. This is crazy growth for feet! We buy his sneakers large, so they do tend to last him the length of the orthoses. He does like wearing them. They give him pressure and stability, which he likes. As a mom who *loves* being barefoot, I have to try and remember to at least wear flip-flops around the house. Ian always wants to be “bare-piggy” when mommy is. Especially when we snuggle on the couch with a blanket, he always wants to take his shoes and socks off, and I don’t blame him. He needs to wear them all but about one hour of when he is awake, which doesn’t leave much “bare-piggy” time.
|New SMOs and Sneakers - September 2014|
The orthoses help lift his arches and straighten his legs. With his Hypotonia, his arches collapse and it makes standing uncomfortable over time. He will have to continue wearing these as his legs and feet grow in order to mold the bones in the right place. He is responding well, but still collapsing without the support of the orthoses.
Ian still has mild-moderate obstructive sleep apnea. The exact cause is fairly unknown. It is most likely from mucus and nasal passage obstruction. He has not had another sleep study since removing the mucus medications, but will have one this month. He does seem restless at night, but this could also be from his Periodic Limb Movement Disorder.
Periodic Limb Movement Disorder is typically caused by low iron. Not anemia, but low Ferritin iron in the bloodstream. A certain amount of iron is needed to keep the Central Nervous System running smoothly. With Ian’s low Ferritin, he has CNS disruptions when he sleeps. The average person has up to five leg movements an hour at night, none of which are enough to wake you up. Ian has over 24 an hour, and over 2.5 of those every hour wake him up enough to disturb his sleep.
Ian has been on iron therapy for over a year now and it is slowly climbing. She would like his blood level to be over 100, which is still low. We started at 23 and moved to 33 six months later. Six months ago, Ian’s was at 44. She said it would take a long time for the iron to accumulate in his body. We have another eval with a sleep study this month and I am anxious to see the results. Ian is also still using his ComfyLift bed for treatment. We will be receiving a new twin size mattress for him very soon. He has outgrown the one that used to fit in his toddler bed (converted crib). It has been sitting solo on the floor for months now and his feet hang off the end of it because he is now 38 inches tall. The bed helps elevate his upper body, as to open his breathing passages and help with sleep apnea. It also serves as a wonderful tool to decrease nighttime reflux and GI discomfort. Ian *loves* the bed!! We are looking to build a super-cool frame for the new one so it can still sit low to the floor. He wants to decorate it with cars and planes wall decals!
|Ian's ComfyLift bed|
Ian’s eyesight is doing quite well. We visit her once and year and the last time we had an appointment, he was seeing above average. With Ian having a steroid prescription, we do need to continue seeing her once a year, even with “perfect” eyesight. Steroids can increase the pressure in your eyes. With Ian being an ex-preemie, he is predisposed to things like this. He loves wearing pretend glasses and sunglasses, just like mommy and daddy!
Ian had a great dental check-up this summer. Still surprising to me, with over 1200 vomits (not counting silent reflux which we cannot see), Ian has NO ACID EROSION on his teeth! I cannot help but feel a huge amount of thankfulness that I knew everything I did while Ian was suffering so badly from rflux and vomiting. I attribute his xylitol toothpaste and Sonicare toothbrush for helping avoid acid erosion on his enamel. We also still use xylitol CariFree gel every night. I smear a large pea-sized amount on his teeth, gums, and cheeks when he crawls into bed.
Ian is currently on “consult” with speech therapy. This means that he is at age-appropriate levels (there’s that AGE APPROPRIATE word agin…. music to my ears!). We speak with her once or twice a month via email or over the phone as a “catch-me-up” type thing. We can ask questions and go over expectations as he develops. He will not be discharged because as a child with past concerns, he could fall behind yet again. It is so much harder to get in than to stay there and do some mini-consults here and there. It is with the public school system and he is working with the lady who works at the Kindergarten/Elementary school he will attend in a few years.
We still visit our Diagnostician every six months when we see our GI. He takes the chance to overlook everything we are doing and can evaluate. He helps us decide who we need to see and who we can put on reserve. He also is great at helping guide us toward our next steps, as he can see a better view of the overall picture. He has been an incredible asset to Ian’s care. If you have access to a Diagnostics or Complex Care department, I highly recommend getting yourself a “Dr. House!”
Since April, we have been able to remove seven daily medications!!!!! He now only takes three in the morning, and three at night. We remain on the Prevacid (for reflux), large dose of Multivitamin for added iron, and Alvesco (Pro-drug inhaler for Asthma/Reactive Airway Disease) in the mornings. At bedtime, he takes Iron (for Periodic Limb Movement Disorder), Zyrtec (for morning mucus suppression), and Growth Hormone.
Ian just turned four on September 3rd and he started his 3rd year of preschool the following week. He started preschool the week before his 2nd birthday. We wanted him to see other children eating and talking. It has made a huge impact on his progress. He is an only child, and playing with the other kids at school is a very fulfilling experience for him. He always tells me about how he played with “all his friends” when I ask what he did that day. He has so much fun attending their birthday parties and loves giving them all group hugs! He is doing very well and his teachers have been extremely pleased with his developmental growth and learning skills.
|2012 - 2013 - 2014|
We still have a lot of physicians and specialists on reserve but have not needed to see the: Audiologist, Allergist, Behavioral therapist, Clinical Geneticist, Metabolic Geneticist, Immunologist, Occupational Therapist, ENT, Pediatric Surgical Reflux Clinic, or Physical Therapist in almost a year now. That’s amazing!
To sum up, we are so proud of our little boy! He is making great medical and developmental advances everyday. I always say “this is my favorite age.” I hope it keeps getting better and better. While we still have a lot on our plate to manage and keep current, it is nice to know we can keep some things on the back burner. I’m hopeful that as he grows, his body will grow and improve as well. Thank you always for all the love, prayers, and support. We are a very blessed family and I am thankful to have all of you as part of our journey!