Eat Happy!

Eat Happy!


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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

Fatty acid oxidation metabolic diseases

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.