How do I transition to adult sickle cell care?

Moving from a pediatric to an adult sickle cell disease (SCD) doctor is a massive milestone. It is completely normal to feel anxious about leaving the medical team you have known your whole life. The key to a safe and smooth transition is shifting from relying on your parents and pediatric team to actively taking the reins of your own healthcare. By starting early and building a concrete adult care plan, you can protect your health and avoid dangerous gaps in your treatment.

Why Preparation is So Important

The years when young adults transition to adult care are a critical period for your long-term health ^[1]. The risks of severe complications and early mortality can increase during this time, making it vital to stay on top of your care ^[2]. Several factors make this shift challenging:

• Learning a New System: Pediatric clinics often have centralized teams who handle everything for you. Adult care is more fragmented, and you will need to learn how to manage appointments and insurance on your own.
• Insurance Shifts: Changes in coverage—such as aging out of Medicaid or a parent’s plan—can cause sudden gaps in care if not planned for ^[3].
• Specialist Shortages: There is a nationwide shortage of adult hematologists specializing in sickle cell disease ^[4]. If an SCD specialist isn’t available in your area, you may need to find a general hematologist who is willing to consult with an SCD center of excellence.

Essential Steps to Prepare

Preparation should ideally begin in your early teen years, but it is never too late to start ^[5]. Take the following actionable steps to protect your health during this change:

1. Build a “Portable Medical Summary”

Adult providers will not know your history. You need a comprehensive summary of your health to give to any new doctor ^[6]. This document should include:

• Your specific SCD genotype (e.g., HbSS, HbSC, HbS beta-thalassemia).
• Your baseline lab values, particularly your standard hemoglobin (the oxygen-carrying protein in blood) and reticulocyte (immature red blood cell) counts when you are healthy.
• History of complications, such as past acute chest syndrome, leg ulcers, or organ involvement like pulmonary hypertension ^[7].
• Specialized screening results. In pediatrics, Transcranial Doppler (TCD) ultrasounds are standard to check brain blood flow; in adult care, your team will likely transition to using MRIs or MRAs to monitor ongoing brain health.
• Fertility status, which is especially important to track if you take disease-modifying therapies like hydroxyurea that can impact fertility, or if you have previously done fertility preservation ^[8].

2. Master Your Medications and Insurance

In the adult system, it is your responsibility to remember medications, request refills, and handle pharmacy issues. Adherence to medications like hydroxyurea often drops during the transition due to these logistical hurdles ^[9]. Work closely with your pediatric team to learn how to navigate prior authorizations with your insurance and ensure you have a seamless supply of hydroxyurea before your first adult appointment ^[10].

3. Establish a Concrete Pain Management Plan (and Prepare for the ED)

Adult emergency departments (EDs) operate very differently than children’s hospitals. Unfortunately, many young adults face systemic bias and “drug-seeking” stigma when seeking acute pain relief in adult EDs. Having a documented plan is your best defense ^[11].

• Outpatient Plan: Know exactly which medications (and doses) work for your daily or mild chronic pain.
• Emergency Protocol: Have a written ED clinical pain pathway signed by your hematologist. National Heart, Lung, and Blood Institute (NHLBI) guidelines state that you should receive pain medication (opioids) within 60 minutes of registering at the emergency department during a vaso-occlusive crisis ^[12].
• Advocating in the ED: If you face skepticism from a triage nurse, stay as calm as possible. Present your written protocol and say, “I am having a sickle cell crisis. My hematologist has written this protocol for my care. Please call my hematologist immediately to coordinate.” Bring a trusted family member or friend to advocate for you, and do not hesitate to ask for the hospital’s Patient Advocate or Charge Nurse if your protocol is being ignored.

4. Build Your New Care Team

Do not wait until you are sick to find an adult doctor. Ensure your pediatric hematologist communicates directly with your new adult provider to hand off your care ^[10]. If possible, schedule your first adult intake appointment before your final pediatric visit so you do not experience a gap in coverage. Additionally, medical transitions are emotionally taxing; ask for a referral to a therapist or counselor who understands chronic illness to help support your mental health during this change.