Shortly after Raya was born, we noticed some pink patches on her skin that looked slightly flaky and which we assumed were just weird newborn skin. When they had not disappeared by morning the next day, I asked a couple of nurses about them and one of them said she had consulted a dermatologist who happened to be in the nursery when Raya was getting her newborn tests, and who had told her that this was just regular newborn rash. Something about the white halos around a couple of the patches continued to bother me, and when we went to Kaiser's newborn club a couple of days after the birth, a nurse felt sufficiently alarmed by the look of the skin that she summoned a pediatrician who then called a pediatric dermatologist who repeated, with a fair amount of confidence, the diagnosis of "newborn rash" (whatever that means). After several more days at home, watching the patches slowly deepen in color, and hours spent surfing the internet, we concluded that Raya likely had a case of the "port-wine stain" birthmark--this was no ordinary rash. A pediatrician we saw around 1 week of age confirmed our self-diagnosis but referred us to a Kaiser dermatologist for a more specific diagnosis and to begin treatment. The dermatologist, who seemed to agree tacitly with the port-wine stain diagnosis, though he never said so explicitly, took pictures to send to Kaiser SF for further consult. By the time we heard back from him, I had already contacted a birthmark specialist at UC Irvine, who told me that this was either a port-wine stain or an early segmental hemangioma, and recommended that we consult a well-known dermatologist at UCSF. His email was the first time we heard the term "segmental hemangioma," which has since become a landmark of our lives. Coincidentally, Kaiser SF also decided that we needed a consult by the same UCSF dermatologist, so at 2.5 weeks we promptly appeared in her office. She had already seen several pictures of Raya that I had emailed her, and it took her about 15 seconds of looking at the real thing to conclude that this was indeed an "infant segmental hemangioma" with a beard distribution. Raya was put on steroids starting immediately, and we were told about all of the possibilities for which we should prepare: that she has a 60% of another hemangioma growing inside her throat and causing, at some point, an airway obstruction; that she was a good candidate for something called PHACES syndrome, which our doctor herself had discovered, and which stands for a constellation of heart/brain/etc. abnormalities that sometimes afflict children with segmental hemangioma; that because the hemangioma encompassed her lip, and the lip was likely to swell at some point, she was at risk for ulceration, which would be painful for all of us to live with. The steroids were being administered in order to keep the hemangioma from growing, and she would be taking them until the H, as I will call it for short, had gone through what is termed its "proliferative stage." That she was likely to come out of this just fine, but it would probably be a long process.
Almost nine weeks later, Raya continues to take the steroids and is so far considered a successful patient. Since she has gone through several weeks when normally the hemangioma would have been growing and/or ulcerating, all with little more than changes in color, the doctors are fairly optimistic. If by 16 weeks there have been no other significant changes, they might consider starting to taper off the steroids. Then, once there is a fair amount of certainty that the hemangioma has finished its entire growth phase, they might begin laser treatments.
In the meantime, Raya has had two visits to a pediatric ENT, who scoped her throat when she was barely four weeks old in order to look for airway obstruction; so far so good, and there is no stridor in her voice indicating that something is growing inside the throat. She also had a heart ultrasound and an EKG, which both showed a healthy heart. She is scheduled to have an MRI at the beginning of February, which will examine her brain, chest, and heart again. Depending on how that turns out, she will receive--or not--the diagnosis of PHACES syndrome.
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