STEVEN JAMES YRAY-WASILIK
Mitochondrial Related Health Summary
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Born: 3/24/98 in Houston, TX
Died: 6/9/03 at Texas Children's Hospital, Houston, TX
Father: Stanley Ward Wasilik
Mother: Joyce Charmagne Yray

Status Update: Steven passed away on June 9, 2003 following a long hospitalization chronicalled in the main journal. Following Steven's hospitalization in the fall of 2002 for pneumonia, his muscle weakness and lack of motor control was more apparent than before. As he had "rehabbed" well several times, we remained hopeful. Unfortunately, almost four months later, Steven had not regained the ability to walk, had difficulty sitting upright and crawled only with effort. His fine motor skills had regressed substantially, and he had difficulty picking up, holding and maintaining control of objects. While he never developed any significant speech skills, he sometimes formed single, recognizable words. He continued to attempt to verbalize up until his final months. He was fed strictly via g-tube and received medication mixtures every four hours. He generally recognized people and was receptive to and gave affection, although it seemed in later days that sometimes it took him some time (several minutes) to recognize family members if he hadn't seen them recently.

Condition:

Mitochondrial disorder. Unconfirmed through genetic analysis; consensus diagnosis based on symptoms.
Unconfirmed Cytochrome-C-Oxydase production deficiency (borderline low levels only)
Unconfirmed Succinate Dehydrogenase production deficiency (borderline low levels only)
Unconfirmed mDNA depletion (Initially suspected based on enzyme levels noted)
Have exhausted current DNA testing technology. No re-testing likely within next two years.
Prognosis unknown.

Symptom History:

Seizures. Onset age one month. Identified at 3 years as myoclonic type with associated myoclonis.
Gross motor skills delayed
Fine motor skills delayed
Motor skill regression evident
Speech and comprehension skills limited or absent
Cognitive skills delayed
Mild hearing loss
Balance difficulty and uneven gait
Repeated, severe respiratory infections
Generalized muscle weakness

Other General Status:

Gastronomy tube fed
Seizures generally well controlled by medication in last 18 months of life.
Very susceptible to respiratory and other infectious diseases


Medications:

Valproic Acid (Depakote)
Ativan
Zonisamide (Zonigram)
Clorazapate
Zantac
Enzyme Co-Factor Q10
Levo-Carnitine
Abluterol
Robinal

Other:

Fails standard Barium swallow study
Slight, MRI-detected atrophy in some portions of brain at 4 years
Significant, atrophy in all portions of the brain evident via MRI just prior to death.
EEG normal
Liver function normal
Kidney function normal until just prior to death
Heart function normal until just prior to death
Ragged red fibers not detected
Lactic Acid normal
Leigh's syndrome negative
MELOS, MERF and MAD negative
Co-proporphyria negative
1/2 sister in good health
Social response and recognition skills generally normal, although recognition skills slowed over time.