Aiden was born, he had a dark purple birthmark on the right side of
his face. The attending pediatrician at the hospital told me that Aiden
probably had what is called a port-wine stain (PWS), but could not give
me a definite diagnosis or information.
After arriving home from the hospital, I did some extensive research
on PWS. What I found was that this was not simply a birthmark or cosmetic
issue, but a medical condition that would require early diagnosis, evaluation
and treatment to prevent further complications. Some of the information
I came across was devastating. I found that because Aiden’s PWS
involves the first and second branch of the trigeminal nerve (the upper
eyelid/forehead), Aiden's lesion was a marker for a rare condition called
Sturge-Weber Syndrome (SWS) and that he would most likely develop glaucoma
I immediately made an appointment for Aiden to see an ophthalmologist
who confirmed that Aiden had a pressure of 40 (10 is normal) in his
right eye (the eye affected by the PWS). Aiden was prescribed seven
different eye drops and after they failed to lower the pressure, it
was decided that Aiden needed a surgery called Trabeculotomy (performed
at 2 ½ months of age in 2004). We made frequent post-op visits
to make sure the surgery worked and we were happy to hear that the pressure
in his right eye had dropped significantly. However, after an eye exam
under anesthesia in 2006, we were told the pressure was increasing and
Aiden was put back on eye drops. The drops are currently keeping his
pressure at an acceptable level.
Aiden goes to the Beckman Laser Institute in Irvine approximately every
eight weeks to receive laser treatments to lighten his port-wine stain.
He began treatments when he was only four months old. He has had 15
treatments to date and the results are amazing!
Aiden was diagnosed at three months old as having Sturge-Weber Syndrome
after having his first seizure. One night, Aiden was very fussy, which
was not typical, and I noticed his left foot and hand twitching rhythmically.
We rushed him to the emergency room where it was confirmed that Aiden
was having focal (coming from one side of the brain) seizures. They
ran CT, MRI and EEG tests and the result was positive for SWS. The right
side of his brain has an overabundance of blood vessels just like in
his skin. The excess blood vessels on the brain had caused calcification
and atrophy (shrinking) which was causing the seizures. He had a couple
of “stroke-like” seizures which caused him to have a weakening
and partial paralysis on the left side of his body (called hemiparesis).
We stayed in the hospital for two weeks trying to find a medication
that would control his seizures. We were sent home on four different
anti-epileptic drugs (Phenobarbital, Tegretol, Dilantin and Depakote).
That combination worked for almost six months when the seizures returned
and landed us back in the hospital. By that time, Aiden had been weaned
off of all meds except Depakote. A new drug called Topamax was prescribed
and after another two weeks in the hospital, we were able to go home.
Aiden made it almost two years seizure free until just before Christmas
2007, he started having brief focal and absence seizures again. This
would happen up to four times a day. I called his neurologist right
away and he increased Aiden’s dose of Topamax. After two days
of the increased dose, the seizures stopped. I took Aiden in to see
the neurologist for follow-up and he said that I should seriously consider
a surgery called hemispherectomy because SWS is a progressive disease
which means Aiden’s brain would continue to get worse over time.
He gave me the number of a neurosurgeon at UCLA so we could take the
next step in determining if Aiden would be a candidate for the surgery.
We went to UCLA for pre-surgical testing in April, 2008. They did a
PET scan and extensive MRI, MRA, and MRV scan of Aiden's brain as outpatient
procedures. Then, we were admitted for 7 days for EEG/Telemetry to map
and record seizure activity. We then met with Dr. Mathern, the surgeon
who would be performing the hemispherectomy. He said all the tests showed
that there was only right-side brain involvement which made Aiden a
candidate for the hemispherectomy. The procedure would involve removing
the right hemisphere of Aiden’s brain and hopefully put an end
to his seizures. He explained that the removal of the severely damaged
brain tissue would better allow the healthy hemisphere to perform its
duties. 80% of patients who undergo this surgery have seizure control
with little to no medication.
The surgery was scheduled for August 21, 2008. That day was the hardest
day of my life. I had to kiss my baby goodbye knowing that kiss might
be the last one I would give him. He was in the O.R. for 12 long hours.
Having my family there for support was crucial. To our amazement, Aiden
was awake when we were finally allowed to see him in the ICU. The nurse
told us he had been moving his arms and legs, which to me, was a miracle.
The morning after surgery, I was sitting next to Aiden, waiting for
him to wake up. When he did, I sang to him the song I’d always
sing to him in the morning and he smiled at me and I knew at that moment
everything was going to be alright.
Aiden recovered from his surgery so fast and since there were no resulting
complications, he was allowed to go home after only seven days in the
hospital. When he returned home, we continued to see progress and the
best part, no seizures!
Aiden has had physical and occupational therapy since he was 5 months
old because of severe developmental delays. He didn’t learn to
crawl until he was almost 3 years old. He took his first steps when
he turned 4. He can’t speak or chew food. For these issues, he
has speech and feeding therapy. His developmental delays are believed
to be caused by brain damage due to Sturge-Weber Syndrome and the seizures
he had suffered. He continues to have therapy everyday and is making
strides. He can now walk without assistance, is learning to feed himself
and making more vocalizations. Although he is still behind developmentally,
I feel that the hemispherectomy was the best choice for him. He is much
more aware of the world around him and he is a happy, healthy boy. Aiden's
future is a bright one and I know he will make a difference in this
It is an honor to be able to share Aiden’s story and our experience.
It is gratifying and humbling to help people who are going through similar
situations and facing decisions that will transform their lives.
-Rachel (Aiden's mom)