Page 5 - CUA Best Practice Report: Pediatric hemorrhagic cystitis

BPR: Pediatric hemorrhagic cystitis

HC and 0/5 protocol patients died, so this may not reflect time 85‒90 minutes) for 5‒6 days a week at 2.1‒2.5 atm
the full duration of HC. with either 100% oxygen only or a mixture of 100% oxygen
Recommendation: There is limited evidence on the posi- and chamber air dives. Complete resolution of HC in these
tive benefits of using PPS in pediatric patients with HC, studies has reportedly ranged from 70‒94%. A median of
therefore, further studies are needed prior to broad imple- 10‒13 sessions were needed to yield complete resolution
mentation (Level 4 evidence, Grade D recommendation). of HC. Savva-Bordalo et al also noted a strong correlation
between the time from HC onset to the initiation of HOT
Estrogen and the time from HOT to HC resolution (r=0.70; p<0.05).
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In this study, three patients did end therapy early due to
Conjugated estrogen has been used in the management of ear barotrauma, pressure intolerance, and claustrophobia,
HC, either in oral or IV form. It is thought to treat HC by indicating that patients with a history of claustrophobia and
promoting stabilization of small blood vessels, suppressing diving intolerance should avoid HOT. Patients with active
the immune response, and facilitating the repair of injured cancer, active viral infection, or Fanconi’s anemia are also
tissues. 36,37 Potential side effects that have been proposed or advised to avoid HOT. 6,44
observed, however, include liver dysfunction, hypercoagu- Recommendation: HOT has been proven to be safe, effec-
lability, hypertension, facial flushing, feminization in males, tive, and relatively low-risk in treating pediatric patients
and malignant transformation, a grave risk in patients with with HC. Its widespread use, however, may be limited due
pre-existing histories of malignancy. Heath et al evaluated to cost and access to facilities with HOT (Grade 3 evidence,
10 children with HC following high-dose chemotherapy and Level B recommendation).
SCT who received estrogen. The treatment was started via
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IV and transitioned to oral after 2‒3 days; liver function tests Invasive treatment
(LFTs) were monitored every 48 hours. Duration of therapy
ranged from 5‒23 days, resulting in reductions in HC symp-
toms in 90%, although two patients required repeated IV and Prostaglandin (E1/E2)
oral dosing after initially being partial or non-responders.
One patient had existing liver GVHD and developed eleva- Several reports exist on the use of prostaglandin (PG) E for
tion in LFTs and, therefore, therapy was stopped. Mousavi the management of HC, although the exact mechanism of
et al’s study of oral estrogen noted no benefit in the man- action is not widely understood. In a review of practice,
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agement of HC. This randomized, case-control study of Cesaro et al noted that intravesical PGE2 use at the discretion
56 adult and pediatric patients post-SCT demonstrated an of the physician for severe and/or refractory HC post-SCT in
insignificant downgrading of HC in treatment relative to children resulted in 37% success, although success was not
41
control groups. well-defined. The therapy was diluted, instilled intravesi-
Recommendation: There is conflicting evidence regard- cally, and then allowed to dwell for 1‒2 hours. Instillation
ing benefit vs. harm with estrogen therapy. Additional pro- was repeated daily until HC resolution or for at least one
spective, randomized, controlled studies are indicated to week until being considered unsuccessful. No patient expe-
clarify its use in managing HC (Level 4 evidence, Grade D rienced complications or intolerability necessitating therapy
recommendation). withdrawal despite concerns over its potential for inducing
bladder spasms or flushing. Trigg et al noted more positive
Hyperbaric oxygen therapy (HOT) results in their pediatric post-BMT study with intravesical
45
PGE1, as PGE2 was not commercially available in the U.S.
HOT has wide-reaching uses within urology, particularly in All children were sedated during therapy, which targeted a
the management of radiation-induced hematuria, but has therapeutic dwell time of one hour. HC was eliminated in
also recently come to be recognized as a potential thera- 5/6 patients over a seven-day course, and in some cleared
peutic option for pediatric patients with post-BMT HC. It is in as little as 24 hours. No apparent side effects were noted.
thought to assist in the resolution of HC by inducing neo- Recommendation: PGs may be of modest benefit as an
vascularity and permanent tissue healing in the damaged intravesical therapy in pediatric patients with HC with ure-
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bladder tissue. Following a positive case report by Furness thral catheters, although additional studies are needed to
et al, where the only notable side effect was bilateral otitis clarify its efficacy and if sedation or general anesthesia is
media, multiple institutions have reported case series on needed to administer the therapy due to concerns for its
their similarly positive results. 41-43 Though the exact protocols potential to cause painful bladder spasms (Level 3 evidence,
of HOT therapy varied, it was typically initiated once HC Grade C recommendation).
reached grade II‒III and patients had failed prior conserva-
tive therapies. Patients underwent multiple dives (total dive

CUAJ • November 2019 • Volume 13, Issue 11 E329