DIAGNOSIS #1 - Petriventricular Leukomalacia
"What in the hell is Petriventricular Leukomalacia?" That is the question Jen and I were asking ourselves in April 2017. We were informed that Rhythm had it following an MRI during a hospital stay to track her brain patterns (EEG) ordered by our neurologist. In fact, the doctor on duty stated it so matter-of-factly that it completely caught us off-guard and forced us into immediate research mode. Up until this point, we thought all of the evil that had ravaged Rhythm was solely because of Infantile Spasms Epilepsy. We were wrong.
The National Institute of Health defines Petriventricular Leukomalacia (PVL for short) as follows:
"Periventricular leukomalacia (PVL) is characterized by the death of the white matter of the brain due to softening of the brain tissue. It can affect fetuses or newborns; premature babies are at the greatest risk of the disorder. PVL is caused by a lack of oxygen or blood flow to the periventricular area of the brain, which results in the death or loss of brain tissue. The periventricular area-the area around the spaces in the brain called ventricles-contains nerve fibers that carry messages from the brain to the body's muscles. Although babies with PVL generally have no outward signs or symptoms of the disorder, they are at risk for motor disorders, delayed mental development, coordination problems, and vision and hearing impairments. PVL may be accompanied by a hemorrhage or bleeding in the periventricular-intraventricular area (the area around and inside the ventricles), and can lead to cerebral palsy. The disorder is diagnosed by ultrasound of the head."
As it turns out, PVL was THE core diagnosis for most everything Rhythm had been experiencing. The root cause. That's why I'm kicking off the deep dive of the various diagnoses she has had with PVL. A look at the cause and symptoms all align.
CAUSE:
Rhythm was born prematurely and had to be resuscitated. In that time, she had a lack of oxygen to her brain. This caused the death of white matter around her ventricles, as confirmed in her MRI.
SYMPTOMS:
1) Motor Disorders - at 5 years old, Rhythm is not yet crawling. While she is now learning to sit up, she does not have the balance to maintain the position yet.
2) Delayed Mental Development - Rhythm is not speaking, although she is very vocal. She has shown the ability to learn things, but at her pace. She is still learning some basics at 5 years old.
3) Coordination Problems - as stated, Rhythm can not maintain balance, but she is trying very hard. She is stronger in using her hands now, but that is a recent development.
4) Vision Impairments - a few doctors have indicated that while Rhythm's eyes are healthy, she likely has Cortical Blindness, which I'll cover in a separate post.
5) Hearing Impairments - Rhythm's hearing is very strong and always has been.
6) Cerebral Palsy - This has also been diagnosed, which I'll cover in a separate post.
7) Infantile Spasms - Any condition that causes damage to the brain can cause Infantile Spasms. This would certainly qualify.
This was a significant piece of information, which unfortunately took us 15 months to get after her seizures started. Now we could tie it directly to her premature birth and the ensuing impacts during resuscitation. In the end, while unfortunate that it all happened the way it did, Rhythm is alive and that means everything.
There is no treatment for PVL at this time. Interventions would be needed, based on severity, to treat the various symptoms.
NEXT POST TOPIC: INFANTILE SPASMS
