A lot has been happening

My apologies for the length of time since the last post. So much has been happening, mostly with Maya medically, that there has not been much time or energy left to blog.

Maya has been having several problems with her NG tube. Her skin is so sensitive to all of the adhesives used, as well as the NG tube material itself, that she gets an oozing red sore under the tape w/in a few days. Changing the tube to the other nostril weekly to alleviate the rash on one side temporarily solved the problem, but has stopped being effective as the interval now required to change the tube is to short to allow enough healing of the other side's rash. Also the procedure to remove/replace the tube is traumatizing to Maya. In addition, she has been developing aspiration (at least in part) based pneumonia due to aspiration of either foods, stomach acid or saliva, despite treatment with acid-blocking medication. The NG tube is a foreign body that does not allow for full closure of the spinchter at the entrance of the esophagus into the stomach, so it allows a path for acid to reflux back up the throat, possibly then over and down into the lungs.

To reduce the NG tube problems, we are having a P.E.G. tube/port placed into her stomach on Friday which will allow food/liquids access as well as samples of stomach acids via a port in her side. She will still be able to eat solids, but liquids will be through this port, just like the NG tube. At the same time she will have a bronchoscopy to examine her lungs and trachea to look for structural abnormalities and to test her mucous to see if they can find a cause for her continued infections.

In addition we have noticed, as well as her pediatricians, that she has significant bowing of the tibia, more pronounced near her ankles and this is happening at the same time her growth has slowed or stopped (since spring). Tests for Vitamin D deficiency have paradoxically led to a finding of an extremely high 1,25 Dihydroxy Vitamin D (Active form of Vitamin D), which can be caused by, in order of liklihood: Sarcoidosis, Tuberculosis, Hyperparatyroidism, hereditary syndromes and various inflammatory diseases. So far we have a negative skin test for TB, but that is common in children - a follow series of three day's of stomach acid samples will be upcoming via her port. She also has negative tests for hyperparathyroidism. Sarcoidosis tests will be performed soon, as well as genetic testing.

I have to run now, time to take Brianna to Taekwondo. Her are a few pictures of Maya's NG tube and rash:

-Chris

Good news and bad news today with Lung/Sinus CT Scans

Today Victoria and I woke up at 3:30 AM and hit the road by 4 AM with a sleepy Maya to have two CT studies performed under general anesthesia. One was an examination of her sinus passageways to look for blockages or other reasons why she is so prone to sinus infections. The other, the chest CT study was to look for something Victoria and I were dreading and haven't talked to many people about...blebs. Our concern was that they would be large, widespread and possibly cancerous requiring major surgery, etc. to try to correct.

We had Maya under sedation right on time at 7:30 AM, and her studies were over in about 45 minutes. We then were able to walk over to the Ear, Nose and Throat clinic and consult with her assigned Dr. on short notice to interpret her sinus study. Maya was found to have normal sinuses with some "mucousal swelling" in her front sinus passageways, which was to be expected with a current sinus infection and/or with an NG tube adding to irritation. So basically it was good news that nothing was awry with her sinuses, but it was disappointing not to have a physiological and simple reason for her recurrent infections.

So then we had a long window of time until our 4:30 PM appointment with our assigned pulmonary doctor, which we filled by taking Maya to a TGI Friday's about 8 blocks away. Maya had not eaten since the night before, and was so hungry she ate a 1/2 rack of ribs and all of her fries. She then played a bit in the public square out front watching the large flock of pigeons and occassionally walking towards one. When a pigeon would take flight it would startle her, the first time she even fell on her butt as a result. It was very funny and I have video I will eventually post of it, but no time now.

When the time came to consult with the pulmonologist, Victoria and I had butterflies in our stomaches expecting ominous news. What actually happened is that the doctor and a visiting pulmonary doctor from Barcelona, Spain took some time to go over Maya's full history. While this was happening we both felt the doctor was unprepared and had not reviewed the CT scan, which was true, but the doctor redeemed himself by getting up to speed while briefing the visiting doctor as he pulled up the CT images. His initial opinion was that no blebs were visible, which the Spanish doctor agreed with, so he refocused the consultation on what he felt was relevant - a current pneumonia-like state in which Maya has areas of mucous caused by aspirated fluids, and a resultant or leftover state of collapsed lung tissue from her surgery. He also noted what was most likely a hiatal hernia that he was surprised was not discovered in earlier swallow studies. In either case, he said she needs chest "percussion" to be delivered by a qualified pulmonary doctor and we agreed to have him contact our pulmonologist in Hartford. Apparently we can be trained to do this ourselves. He also said the cause of her current infection was aspiration of fluids (saliva, water, etc.) and that Gastro-Esophageal Reflux Disease (GERD or acid reflux) was the largest contributor to this issue. He said the NG tube exacerbates the GERD, as does the hitherto undetected hiatal hernia, and he reviewed our doseage of Rantinidine (Zantac) and found it to be appropriate, but increasable if need be. He said the #1 priority needs to be controlling stomach acids and any fluids from entering the lungs, and the NG tube makes that nearly impossible. He introduced the idea of a G-port tube directly into her stomach, which I was not ready to entertain yet as it felt to invasive, but after consulting nurse friends sounds like a better idea to allow her esophagus, vocal chords and lungs to heal. Of couse that may mean no intake of any food or liquid via eating, simply all food pumped into her stomach, which is another major step downhill for us, which we hope will be reversable once her vocal chords heal, but with no guarantee. This also will likely further delay her ability to return to school, which was tentatively set for next week. That suck bad, as she needs social interaction badly.

On the bright side, we have qualified for Birth-3 programs and will be getting speech and possibly occupational/physical therapy to help Maya to get to age appropriate milestones in areas that she is suffering in.

So we have to wait a week before we have Boston Pulmonology's full report and can breathe easy regarding the bleb issue, which we will then have reviewed by CHOP in Philadelphia. In the meantime we get to look forward to consulting with and coordinating between: ENT Doctors in Boston and one in Hartford, Pulmonologists in Boston, Hartford and Philadelphia, Gastroenterologists in Boston and Hartford, Speech and physical therapists at home, a visiting nurse and our primary care physicians, ensuring they all recieve the proper and timely reports from each other while we work to find someone to take the role of an impartial case manager to coordinate and facilitate grop information exchange and consensus.

So that's where we are - tomorrow (or ASAP thereafter) we need to visit CCMC in Hartford to get Maya "modified (seated) percussion treatment" to loosen mucous and to learn to deliver it ourselves, as well as discussing the G-tube option benefits given here comprimised pulmonary state. And of couse figuring out day care for Maya that will accomidate her medical needs.

I am learning to adjust quickly and just be greatful that Maya is happy and affectionate and funny. We love both our kids and they are great gifts here to teach us so much about responsibility, altruism, sacrifice, gratitude and humility. I hope that all of Maya's conditions are temporary and will heal in the next year, but if they don't, we'll research long and hard into the best options to get her the best quaity of life given her medical limitations.

Thanks to all of you that have sent encouraging comments!

-Chris