Ponytail

Now that my hair is long enough again, I have been wearing a ponytail a lot lately. No time for a real haircut ( I get my bangs trimmed at Cookie Cutters when Adam goes) and it's just convenient.  I don't think Adam likes it.  He points at it and says "Hair sticking out, Mommy?"  "Well...kind of, Adam." "Ooohhh".

AFP and ultrasound

Adam had his quarterly stuff done on Monday.  He was SOOOO good.  He watched some of "Land Before Time" and ate his lollipop during his ultrasound so it went super fast.  Then he didn't even cry for his arm poke!  He squirmed a tiny bit but I was able to restrain him myself unlike previous times where they had to have someone else hold his arm while I got the rest of him.  We always know before we leave that his ultrasound is clear otherwise the radiologist would come in and talk to us.  We only have been having to wait a couple of days for his AFP results (it used to take weeks when we went through the Geneticist) and it is down to 2.6!!!  Woo Hoo!  When I saw Dave write it down, I thought it was a 26 and I started to panic but there's a decimal point in there.  Only a year and a half left of arm pokes!!

And in case you forget why we have to do all this...
From the screening protocol -

Wilms tumor is the most common cancer in children with BWS, occurring in about 5-7% of all children with BWS. Most children develop Wilms tumor prior to their fourth birthday; however children with BWS can develop Wilms tumor up to 7 or 8 years of age. By 8 years of age 95% of all Wilms tumor have occurred. J Pediatr 1998 Mar; 132 (3 Pt 1): 377-

The second most common cancer in patients with BWS is hepatoblastoma. Similar to Wilms tumor hepatoblastoma can be identified by abdominal ultrasound. However, an abdominal ultrasound does not view the entire liver. Fortunately there is a marker for hepatoblastoma and this is alpha-fetal protein (AFP). AFP is a protein that is made in the liver of unborn infants. At birth the AFP levels are high and gradually decline to adult levels by 10 or 11 months of age. However, most infants with hepatoblastoma do not have a declining AFP measurement, rather their AFP level increases rapidly. 

Hepatoblastoma is also a fast growing cancer and because of the fast growth, we recommend an AFP  measurement every 6 weeks and ultrasound of the liver and kidney every 12 weeks. The ultrasound of the liver can be done at the same time as the ultrasound of kidney. Unlike Wilms tumor, the risk of hepatoblastoma drops off after 4 years, so we would recommend screening with AFP up to 4 years of age. We don’t see any value in conducting a liver ultrasound after four years of age.

As with all screening programs there is a risk benefit ratio that has to be determined by each parent and physician for the child with BWS. Our recommendations to screen with AFP up to age 4 years and ultrasound up to 8 years is based on the observation that most but not all hepatoblastoma and Wilms tumor will occur by these ages. The decision to screen beyond these ages is an individual one, where the benefits must be weighed against the risks. The major risk of screening is a misdiagnosis of cancer resulting in inappropriate surgery. We have had three such incidents. We conducted a cost effective model describing the costs and benefits of screening for cancer in this population;  although not perfect, the model coupled with the data strongly favors screening for Wilms tumor and hepatoblastoma based on the above mentioned data.  Med Pediatr Oncol. 2001 Oct;37(4):349-56.