Reply To: Sickle Cell Non-compliance

  • Jennifer Young

    February 11, 2022 at 4:10 pm

    Thanks for opening the conversation about adherence, a topic that can be hard at times.

    Having worked within the sickle cell community as an APRN for 14+ years I have come to realize that adherence, or lack of adherence,  has so many pieces and parts. One thing I try to do is to better understand the barriers to lack of adherence. Here are some of the questions that I find to be helpful in this

    “What is it about your routine, situation, family structure that keeps you from ‘x’?”

    “Tell me about a typical day in your family and where medication administration may or may not fit.”

    “What is the biggest challenge to getting to clinic/keeping TCD appts/etc.?”.

    I try to ask questions that are open and compassionate “in a given week how many times would you say you/your child may miss a dose of PCN VK/HU/etc.?” This gives some permission to saying that a dose is missed.

    I also work to better understand the patient/family’s knowledge, goals of care and interest in the recommended care. “Is the recommended medication/testing/etc. something that you really want to do? It is okay to say no so that we can discuss this matter openly and honestly”.   

    At times I have also had to step back and remember that for some families preventative care, whether clinic visits, annual screening studies or daily medication administration, may  takes a backseat to the daily life needs that must be met; food and water, safe housing, work and the list goes on, which can make it hard for some families to manage the issues that seems less pressing.

    There are times that I work with patients/families who are struggling with adherence by having them come to clinic more frequently for smaller, shorter pieces of education and steps in the plan. At these visits I may work with the patient/family to set one goal between that visit and then next visit and then celebrate the patient/family’s success when I see them the next time and the goal was met, or even attempted.

    For some patients/families connecting them to another patient/family with sickle cell disease may be helpful, of course if all parties are interested and willing.   

    I don’t know that my thoughts have helped to answer your question, but I share from my experience that working through adherence challenges can be hard work that at times feels like an uphill battle, but I encourage you not to give up, because seeing the patient/family ultimately thrive and be well is so worth the work. ��

    Thank you for caring for our patients and families with sickle cell disease!!



    Jennifer Young, MS, CPNP-AC, CPHON

    Hem/Onc Lead APRN

    Sickle Cell & Thalassemia Nurse Practitioner

    Nationwide Children’s Hospital

    Columbus, Ohio 43228



    Original Message:
    Sent: 2/4/2022 10:48:00 AM
    From: Andrea Haggard
    Subject: Sickle Cell Non-compliance

    Hello all,

    I am interested in how other facilities are handling sickle cell patients and non-compliance. Specifically, concerning refugee patients from the Congo. How are your facilities overcoming language and cultural barriers to promote compliance? Are you seeing the same trends in non-compliance at your institution?  I am looking for better ways to help serve this population. 

    Thank you,

    Andrea Haggard APRN 
    Kentucky Children’s Hospital

    Andrea Haggard, APRN,RN
    Lexington, KY
    United States