At 7:31pm tonight Emmett turned 2 years old! And yesterday at 1:30pm we were given an “official” diagnosis.
Periventricular leukomalacia (PVL)
This. This is what Emmett has. This is what I’ve been repeating in my head since yesterday, over and over again.
Taken from MedLine Plus:
Periventricular leukomalacia (PVL) is a type of brain injury that affects infants. The condition involves the death of small areas of brain tissue around fluid-filled areas called ventricles. The damage creates “holes” in the brain. “Leuko” refers to the brain’s white matter and “periventricular” refers to the area around the ventricles.
PVL often leads to nervous system and developmental problems in growing babies, usually during the first to second year of life. It may cause cerebral palsy (CP), especially tightness, or increased muscle tone (spasticity) in the legs.
Babies with PVL are at risk for major nervous system problems, especially involving movements such as sitting, crawling, walking, and moving the arms. These babies may need physical therapy.
A baby who is diagnosed with PVL should be monitored by a developmental pediatrician or a pediatric neurologist, in addition to the child’s regular pediatrician.
Taken from Wikipedia:
Two major factors appear to be involved in the development of PVL: (1) decreased blood or oxygen flow to the periventricular region (the white matter near the cerebral ventricles) and (2) damage to glial cells, the cells that support neurons throughout the nervous system. These factors are especially likely to interact in premature infants, resulting in a sequence of events that leads to the development of white matter lesions.
The initial hypoxia (decreased oxygen flow) or ischemia (decreased blood flow) can occur for a number of reasons. Fetal blood vessels are thin-walled structures, and it is likely that the vessels providing nutrients to the periventricular region cannot maintain a sufficient blood flow during episodes of decreased oxygenation during development. Additionally, hypotension resulting from fetal distress or cesarean section births can lead to decreased blood and oxygen flow to the developing brain. These hypoxic-ischemic incidents can cause damage to the blood brain barrier (BBB), a system of endothelial cells and glial cells that regulates the flow of nutrients to the brain. A damaged BBB can contribute to even greater levels of hypoxia. Alternatively, damage to the BBB can occur due to maternal infection during fetal development, fetal infections, or infection of the newly delivered infant. Because their cardiovascular and immune systems are not fully developed, premature infants are especially at risk for these initial insults.
It is often impossible to identify PVL based on the patient’s physical or behavioral characteristics. The white matter in the periventricular regions is involved heavily in motor control, and so individuals with PVL often exhibit motor problems. However, since healthy newborns (especially premature infants) can perform very few specific motor tasks, early deficits are very difficult to identify. As the individual develops, the areas and extent of problems caused by PVL can begin to be identified; however, these problems are usually found after an initial diagnosis has been made.
The extent of signs is strongly dependent on the extent of white matter damage: minor damage leads to only minor deficits or delays, while significant white matter damage can cause severe problems with motor coordination or organ function. Some of the most frequent signs include: delayed motor developments, vision deficits, apneas, low heart rates, and seizures.
Additionally, infants with PVL may not be able to assume the same positions for sleeping, playing, and feeding as premature or full-term children of the same age. These developmental delays can continue throughout infancy, childhood, and adulthood.
Premature infants often exhibit visual impairment and motor deficits in eye control immediately after birth. However, the correction of these deficits occurs “in a predictable pattern” in healthy premature infants, and infants have vision comparable to full-term infants by 36 to 40 weeks after conception. Infants with PVL often exhibit decreased abilities to maintain a steady gaze on a fixed object and create coordinated eye movements. Additionally, children with PVL often exhibit nystagmus, strabismus, and refractive error.
Occurrence of seizures is often reported in children with PVL. In an Israel-based study of infants born between 1995 and 2002, seizures occurred in 102 of 541, or 18.7%, of PVL patients. Seizures are typically seen in more severe cases of PVL, affecting patients with greater amounts of lesions and those born at lower gestational ages and birth weights.
Another common but severe outcome of PVL patients is the development of epilepsy. The link between the two is not entirely clear; however, it appears that both genetic and early environmental factors are involved. One study estimated that 47% of children with PVL also have epilepsy, with 78% of those patients having a form of epilepsy not easily managed by medication. Many of these affected patients exhibit some seizures, as well as spastic diplegia or more severe forms of cerebral palsy, before a diagnosis of epilepsy is made.
So, there you have it. Lots of info, I know. If you made it through reading that, thank you.
Both neurologists seem to agree that this happened sometime during fetal development. That is more of a rarity. PVL most commonly occurs in premature and low birth-weight infants. I know I haven’t gotten around to writing Emmett’s birth story here yet, I’ve been meaning to do that. In short, Emmett was born at 41 weeks and was 8lbs 9oz. I laboured for 18 hours, pushed for 2.5, attempted (and failed) forceps delivery and then cesarean. It wasn’t a very easy labour and delivery, to say the least. Which is why I’ve been hesitant to write about it. Even though it happened 2 years ago now, it’s still very fresh in my mind like it was yesterday. And when I really get to thinking about it, I feel anxious, nauseous, sad, angry, etc…
Usually PVL is caused by decreased blood flow or decreased oxygen flow to the back of the brain. She also mentioned it could have been caused by low blood sugar, too. I also came across this, “Additionally, hypotension resulting from fetal distress or cesarean section births can lead to decreased blood and oxygen flow to the developing brain.” As far as we know, Emmett did not show any signs of fetal distress. But again, and I’ve mentioned it before, I wonder if something was missed.
This isn’t Emmett’s MRI scan, I should have asked if I could have taken a photo, but at least it will provide you with a bit of a visual.
The white areas on both sides, where the arrow is pointing, is the area of where the scar tissue is located. Emmett’s was not that bright white or large, however. But those areas are supposed to be dark/black.
Since getting the diagnosis, I’ve been feeling surprisingly relieved. What’s been making us question everything for the past two years finally has a name. There’s an explanation. I can’t begin to tell you how difficult it was not knowing what was going on, what was causing his seizures, his visual impairment, his developmental delays… it just feels like a huge weight has been lifted. It feels like I’ve just exhaled for the first time in 2 years. We know what we’re facing and we know that what we’ve been doing for Emmett has been what he needed. We just need to continue his therapies to make him be the best he can be!
Today, I am so thankful for that little boy. I am so thankful to share him with Ryan. I am so thankful to have a husband that absolutely adores his little boy. Ryan is absolutely wonderful with Emmett. I’m thankful that we finally have an answer to the endless amount of questions we’ve had. Last year I felt a little sad on Emmett’s birthday. I kept going back to when he had his first seizure. To the NICU. To the ER. Back to the NICU. Trying to recover from a cesarean. Test after test with no answers. Sad that I had no idea what was in store for any of us. Even though the memories of his birth are still fresh in my mind, this year it feels a lot sweeter. I’m proud of how far I’ve come, how far we’ve all come. Emmett works so hard, I’ve never seen such determination. And I know there are some very trying times ahead, but I can’t help but feel so thankful for it all tonight.