Cycle 4, Day -4: Steven and His Ears

Today Steven begins his fourth bone marrow transplant, the last one.

Today he receives his last dose of cisplatin, along with amifostine. He suffered a high-frequency hearing loss in his left ear after his third cycle of cisplatin. He has no hearing at all in his right ear, he was born that way.

His ears are mismatched, his right ear is tiny and his left is normal or a little larger than normal. When he was born, it was several months before we were able to determine whether he heard at all, newborn babies can't tell you what they do and don't hear.

A CT scan was performed at 3 months to see what his issues were. There were multiple things going on. On his right side, the malleus was fused and couldn't move. Typically the semicircular canals are inside a bony cavity in the brain. Steven has no bony cavity, his semicicircular canals are sitting in his brain fluid. There are multiple abnormalities in both ears, the little ear on the right has more abnormalities than the left, the right ear problems are not a good candidate for surgery.

He's always been an amazingly calm, happy and easy-going child (unlike his mom). As a baby, I could go into his room and vaccuum when he was sleeping, he wouldn't react. Did that mean he didn't hear? We waited and watched.

At an auto repair shop once when he was a few months old, he flinched in my arms when some metallic heavy object was dropped. Another time he jumped when I slammed the door of the car.

Despite the absence of concrete evidence, I had the feeling that he was hearing my voice, and when he got old enough to be tested, we found the hearing on his left was normal. This was somewhat of a surprise considering all the unusual stuff going on inside his head.

We were told he would have frequent ear infections, and would almost certainly need tubes. Almost all kids with a cleft palate end up needing tubes and Steven was born with a cleft in his soft palate.

But it didn't happen that way. He has probably had no more than half a dozen ear infections in his 9 1/2 years, and no tubes.

As part of his routine medical care, we go to the cleft palate clinic at Children's Hospital in San Diego once or twice a year. It's a 4 or 5-hour event for us, but we cover a lot of ground-- he sees a speech therapist, audiologist, plastic surgeon, orthodontist, ENT, dysmorphologist (a genetic specialist). These doctors have been following him since birth, seeing them is like reuniting with old friends.

One of the doctors who is there on rotation, a plastic surgeon, himself has no external ears, only ear tags, he wears an elastic band to hold his glasses to his head. He examines Steven's ears, tells me things he could do to enlarge Steven's external right ear to more closely match his left ear, but says that overall it is a very nice ear.

We've discussed this with Steven, but Steven is very comfortable in his own skin, and so far isn't interested in any surgery for his ears. When the move "Finding Nemo" came out last year, he liked Nemo referring to his little fin as his lucky fin, Steven's right ear is his lucky ear. Probably the worst thing about it is that when he wears sunglasses, they don't sit straight on his face due to one ear being lower than the other.

We went to the cleft palate clinic 3 days before Steven's brain tumor diagnosis. He was symptomatic for the brain tumor then, a little headache and nausea, but doing OK. As usual, his left ear was perfect, but for the first time his right ear showed an improvement. They fussed with him for an unusually long time, at the end telling me that for the first time in his life, he might be a candidate for a hearing aid, scheduling an appointment with them and also scheduling a CT scan to look at the bones in his ear to see what was different.

The CT scan was scheduled for a Monday, his MRI to see what was causing his headaches and vomiting was scheduled for Tuesday, he had a craniotomy early on a Wednesday morning.

We spoke to his neuro oncologist Dr. Gajjar last week, as expected he advised us to go ahead with a full dose of cisplatin for Steven. He believes in the amifostine, although its not a guarantee. Tyler, one of the four in our little group of boys at St. Jude in the SJMB03 trial, went home last Thursday, he's done, no detectable hearing loss. Jake, the boy one week ahead of us, suffered a stage II loss in one ear after the 3rd cycle, we'll find out next week what the result is after his last cycle. Dylan, the little boy that is one week behind us has no loss after 3 cycles.

My sweet little Steven gets his last dose today.

- Kathleen