Light of our Life: August 2013

Monday, August 12, 2013

Planes

Disney's newest hit, "Planes" is out in theaters now and our little pilot is so excited! Seeing this movie is #1 on his wish list, but for now seeing the retired ones over at our local USAF armament mueseum has been a favorite field trip while he waits. A talented friend graciously snapped these pictures of Samuel so we would have something to submit with his story for the Children's Miracle Network he will be supporting this year.

Walking amidst this avenue of planes, I considered that navigating through life is a little like the guiding of supersonic jets through the clouds. Unlike the well-devised USAF missions however, we follow a flight plan that only reaches us on a moment by moment basis. The aerial view is really both breathtaking and frightening all at once and the pressures from G forces can nearly pull you apart... but who would trade the thrill of the experience for anything in this world?

As we soar through the skyway steering clear of bad weather, we heed the advise of experienced forecasters. We use our tactical weapons sparingly and we keep open lines of communication with the ground towers. We trust the air traffic controller (Our Personal Lord and Saviour) above all to impart divine direction and the coordinates we will need for the journey ahead. We have survived surprise attacks and our parachute has saved us when ejected from a diving free fall.

A daily challenge is handling a payload of cargo too heavy for the amount of fuel our tank can manage, so that means relying on air to air refueling with fellow aircraft. (You Know Who You Are..) Crew fatique, engineering mishaps and equipment failure can easily derail a mission and require a trip to home base but once patched up we taxi back to the runway as fast as we can, ready to get back in line for a chance to fly high until the day our soul permanently joins the heavens and our battered ship retires to the metal graveyard.

We have a lot of curious static on our current flight plan and I guess I need to blog it out eventually but for now am waiting for more information from the recent testing Sam has had done. We have an appointment in a week and hopefully the clouds will part on some unclear symptomology we are picking up on the radar. For now we will 'keep the wings level and true' as they say in the Air Force. Over and Out.

Samuel's Health Challenges

Probable Mitochondrial Disease with Complex 1 and 3 Defects in the OxPhos respiratory chain
Major risk for metabolic decline and acidosis with physiologic and sensory stress.
Frequent Bradycardia (low HR) episodes that have proceeded cyanosis/ low oxygen desaturations and required life saving resuscitations and stimulation. Now happening less frequently with stress avoidance.
Exercise intolerance, endurance concerns and muscular weakness.
Significant GI pareisis and decreased motility causing frequent pain and feeding concerns. Much improved recently with addition of gastric neuro-stimulator device implanted in abdomen.
Fiber 1 and 2 atrophy shown with muscle biopsy.
G Tube fed but now also taking in some nutrition by mouth.
Pyridoxal 5 Phosphate (co-enzyme of B6) dificiency
Nissen/ fundoplication created to ensure no reflux or aspiration of stomach contents into lungs.
Autonomic Dysfunction / Vaso vagal instability which is apparent on multiple levels. From temperature intolerance to circulatory irregularities and his Brady events, all related to nervous system malfunctioning.
Transitionary Air Hunger/ Respiratory Distress
Oromotor Dysfunction
Hypotonia of the trunk area.. now far less obvious than as a baby.
Diffused Encephalopathy at birth but now EEGs indicate typical brain waves.. may be transitional with stress.
Tracheomalacia - airway collapses
PFO (small hole) with right to left shunting that never closed on his heart at birth.
Seizures - well controlled by medication
Developmental, Fine and Gross Motor as well as speech delays, missing most of his milestones but currently and rapidly making strides.