Results
Twenty-nine respondents participated in the survey. The characteristics
of the respondents are shown in Table 1. The majority of respondents
(51.7%) were PHOs from North America, with pediatric cardiology
(34.5%) being the second largest group. One respondent was from Asia.
Of the 28 responses from North America, 16 states were represented. At
respondents’ institutions, the majority of patients with IH are treated
by PHOs and dermatologists.
A summary of key responses is shown in Table 2. Hypothetical cases and
management scenarios are shown in Table 3. From the responses, several
specific observations and several generalizations can be made. The
decision to observe or treat is subjective. Shared decision making with
the parent(s) is commonly employed. When treatment is deemed indicated,
propranolol is the most commonly used first-line treatment. Respondents
are more likely to treat IH on the head or neck than the trunk or
extremities. For uncomplicated patients at least 5 weeks old by CGA,
most are treated as outpatients. The decision on which patients to admit
for monitoring varies widely and includes indications such as those with
PHACE syndrome; those less than 5 weeks CGA; patients with ulcerated,
painful, or bleeding hemangiomas; and patients with perceived high risk
social situations. Many respondents will treat infants less than 5 weeks
CGA, especially if an IH is on the head/neck and/or causing problems
(such as ulceration or bleeding). A majority (83%) would monitor those
patients (< 5 weeks CGA) more closely than older patients
(> 5 weeks CGA), usually (58.6%) in the hospital.
For infants > 5 weeks CGA who are otherwise healthy
and have a reassuring history and exam, the majority (63%) of
respondents administer the first dose of propranolol under medical
supervision (e.g. in the clinic or hospital). The period of observation
ranges from 1 hour to overnight, and longer for high risk/complicated
patients. Heart rate and blood pressure are the most common values
monitored. Most respondents (74%) obtain a liver ultrasound if 5 or
more cutaneous IH are present. Approximately 40% of providers do not
have an upper age limit for treatment. For patients without PHACE
syndrome or other risk factors for toxicity, a minority of respondents
obtain lab work, an electrocardiogram, an echocardiogram, or a
cardiology consult prior to starting treatment. The majority (88.9%) of
respondents take baseline photographs for the patient’s electronic
medical records. Most clinicians (71%) prescribe generic propranolol
rather than trade name propranolol (Hemangeol®). Propranolol is
perceived to be very easy to obtain including coverage by the patient’s
insurance and/or only a small copay. The most common goal dose for
propranolol is 2 mg/kg/day, divided twice a day, and the full dose is
most commonly reached (via escalating dose) at 2 weeks of therapy. The
perceived discontinuation rate of propranolol due to side effects is
less than 10%. Respondents infrequently (7.7%) switch from one form of
propranolol (generic to trade name, or vice versa) due to side effects.
On-therapy and off-therapy practices vary considerably. A minority of
providers (26.9%) have started propranolol using a telehealth encounter
only. However, a majority (64%) are using telehealth for routine
follow-up visits. A majority of respondents (63%) provide oral syringes
and instruct the parents on the prescribed dose. A variety of
educational tools are used, although few specific websites are
recommended. Most respondents (57.7%) discontinue propranolol at
approximately 1 year of age for typical cases (although each patient is
considered unique). When discontinuing propranolol, the majority of
respondents (53.8%) taper it, typically over 2 to 4 weeks; 26.9% let
the patient outgrow the dose and then stop it at some point in the
future; 15.4% discontinue it abruptly. The majority of respondents
(65%) reassess patients in clinic at least once after discontinuing
propranolol, presumably to assess for rebound growth.