Desire for parenthood and reproductive health knowledge in adolescents and young adults with sickle cell disease and their caregivers.

BACKGROUND/OBJECTIVE: Sickle cell disease (SCD) and hydroxyurea have implications for fertility and reproductive health. The goal of this study was to examine desire for parenthood and reproductive health knowledge among a cohort of adolescent and young adult (AYA) with SCD receiving hydroxyurea and their caregivers at a large pediatric academic center. METHODS: Patients with SCD were approached from September 2016 to July 2017 if they were: (1) 12-20 years old, (2) prescribed hydroxyurea for at least 6 months, (3) proficient in English, and (4) accompanied by a caregiver who was proficient in English and willing to participate.  Participants self-reported sociodemographic characteristics and completed surveys to assess their/their child's desire for parenthood and other life goals, and reproductive health knowledge. RESULTS: Eighteen patient-caregiver dyads completed the study (78.3% of those eligible); 61.1% indicated that they wanted to have future biological children.  Few participants reported receiving information about fertility (16.7% of AYA and 27.8% of caregivers) or birth control (11.1% of AYA and 22.2% of caregivers) from their/their child's health care provider, and the majority had received no information on these topics. Less than half of participants reported that SCD (22.2% of AYA and 50.0% of caregivers) or hydroxyurea (11.1% of AYA and 27.8% of parents) could potentially impair fertility. CONCLUSIONS: Biological parenthood was important to this cohort yet fertility and reproductive health knowledge was low, suggesting that clinicians should prioritize conversations about infertility risk and birth control options with AYA with SCD on hydroxyurea and their caregivers.  More research is needed to identify optimal approaches to these discussions.

Use of Selective Serotonin Reuptake Inhibitor and Midodrine in a Patient With Autonomic Instability 2/2 Compressive Squamous Cell Carcinoma and Pain.

A rare cause of reflex syncope is metastatic cancers involving the head and neck. These can irritate the glossopharyngeal nerve and lead to glossopharyngeal neuralgia with associated syncope. This type of syncope is difficult to treat since it commonly involves both a vasodepressor and cardioinhibitory response, and typically requires removal of the irritative focus. We report a case of a 52-year-old male who presented from home with syncope. He endorsed a 5-week history of progressively worsened positional headaches and dramatic 40-pound weight loss with night sweats over 6 months. In the emergency department, his heart rate was noted to drop into the 20s with associated hypotension 60/31 mm Hg. Heart rate and blood pressure increased with intravenous atropine. Physical examination revealed a large ulcerative lesion in the left tonsillar area. After biopsy of the lesion, a diagnosis of stage IV squamous cell carcinoma of the neck was made; computed tomography angiogram and positron emission tomography/computed tomography confirmed involvement in the posterior tongue extending to the left palatine tonsil in addition to the left jugular chain. The patient was started on cisplatin and radiation therapy, but continued to have episodes of syncope associated with bradycardia and hypotension. After a failed trial of benztropine, the patient was started on sertraline and midodrine with resolution of syncope. This could be a potential treatment option in those with compressive mixed syncope who are not candidates for surgery or chemotherapy or are awaiting definitive treatment.

A Retrospective Review to Determine If Children with Sickle Cell Disease Receive Hydroxyurea Monitoring.

INTRODUCTION: Sickle Cell Disease (SCD) guidelines recommend that patients on hydroxyurea receive monitoring at least every 2-3 months, but it is unknown if this occurs in clinical practice. This study aimed to determine if patients with SCD at Nationwide Children's Hospital (NCH) had at least four, in-person monitoring visits during a 12-month period and if frequent monitoring was associated with hydroxyurea adherence and clinical outcomes. METHODS: We performed a retrospective analysis of children on hydroxyurea for at least 12 months during 2010-2015. Patients' demographics, laboratory studies, prescriptions, and number of hydroxyurea and acute visits were recorded from their 12-month period that met eligibility criteria. Patients were considered frequently monitored if they had ≥4 hydroxyurea visits and adherent if they had prescriptions for hydroxyurea for ≥80% of the days in their 12-month period. RESULTS: Seventy-four children met the eligibility criteria and 57 (77%) had frequent monitoring. The most common reason for not obtaining frequent monitoring was missing a scheduled appointment. A greater proportion of frequently monitored patients were adherent to hydroxyurea (66.7% vs. 17.7%, p<0.001) and they had significantly fewer acute visits (median 1 vs. 2 visits, p=0.032) compared to infrequently monitored patients. CONCLUSIONS: Our study shows that most children on hydroxyurea at NCH received frequent monitoring and that it was associated with improved adherence and outcomes. Our results suggest that frequent in-person monitoring could be an opportunity to identify poorly adherent patients. These data inform our next quality improvement initiative that will maximize adherence to these monitoring guidelines.