On January 29, members of the Indiana Sickle Cell Advisory Committee and the Indiana Sickle Cell Consortium participated in Sickle Cell Advocacy Day at the State Capitol. Several IHTC staff members and patients attended. The focus of the day was to educate legislators on the need for funding for programs and services for adults in Indiana with Sickle Cell Disease. Currently, the state funds two programs at IHTC for patients: the Sickle SAFE Program, for ages 0-3, and the SCORE Program, for ages 3-21. IHTC staff members and other consortium members agree that the state needs to do more for adults. The day’s agenda included speeches from several stakeholders in the sickle cell community, including patients, parents of patients, medical professionals, and even Representative Greg Porter, who has long been a sickle cell champion. Several other legislators visited the event or sent their staff members to gather information. We have plans to make 2019 Advocacy Day even bigger and better! Keep watching this space for more information!
A person inherits genes that produce hemoglobin from his/her parents. It is important to identify people with hemoglobin trait so they will be aware of their risk of having children with sickle cell disease. If one parent has sickle cell trait and the other parent has normal hemoglobin, there is a 50 percent chance with each pregnancy that the child will be born with sickle cell trait. If both parents have sickle cell trait or another hemoglobin trait, there is a 25 percent chance with each pregnancy that the child will have sickle cell disease or another hemoglobin disease.
People with sickle cell trait usually do not have any disease symptoms. However, it is possible for a person with sickle cell trait to have complications of the disease under extreme conditions, such as:
I had some familiarity with Sickle Cell Disease (SCD) when my son was born in November 1996. I was told that he had Sickle Cell Trait (SCT), which he inherited from me. SCD became a part of my family December 25, 2002 with the birth of my twin daughters. One of my daughters had the trait and the other had the disease (HbSS). I didn’t know much about the disease at that time, but I would soon become inundated with information through independent learning and education by doctors and others in the sickle cell world.
At that office visit, we received the name of a sickle cell educator, a referral to the hematology clinic at Riley Hospital for Children, and an address/invite to the Martin Center’s sickle cell support group. Our family was armed and ready for battle! We did not know it at the time, but these resources would prove to be extremely valuable to us and ultimately part of our extended family. They were our front-line supporters as my daughter began to experience complications related to SCD, endure hospitalizations, and require extensive care and support.
My purpose for sharing this backdrop is to encourage anyone reading this article to make sure that there is proper coordination of care for any sickle cell patient in your life: your child, your patient, your friend, your student, or anyone else. This begins with having the right team on board to support the patient’s needs.
My daughter’s team is extensive, beginning with our biological family and spiritual support. Her medical team includes her primary care physician, dentist, and hematologists at the Indiana Hemophilia and Thrombosis Clinic (IHTC). She also has access to wrap-around care provisions at IHTC including Physical Therapy, Dental Hygienist, Phlebotomy, Dietary, and Social Services. Her hematologists collaborate with other service providers including pulmonary care at Riley Hospital, optometry care, and radiology for her annual Transcranial Doppler scans, just to name a few. IHTC is also the bridge between home and school, for educating and collaborating with school nursing staff and teachers to ensure our daughter has the best support possible.
As you can see, this team is extensive. The number one best advocate for a child with SCD is the parent or caregiver with whom the child resides. This advocate must always be alert to ensure the care team is aware and ready. Recently, my daughter’s dentist referred her to an endodontist to remove all four of her wisdom teeth. We consulted with the endodontist and scheduled the surgery. Immediately after the office visit, I reached out to three people at IHTC: her hematologist, her primary nursing contact, and our social/educational advocate to advise them of the upcoming surgery and understand their requirements from a sickle cell perspective.
I quickly learned that care coordination is critical. Her hematologist reached out to me immediately with questions and copied the dental hygienist on staff. They both followed up with me again, then reached out to the endodontist directly. Timing was great because my daughter had a clinic appointment coming up before the dental surgery. We were able to have an on-site consult with her IHTC care team, and receive resources/instructions for her post-op care. This resulted in a smooth process and recovery.
Don’t take anything for granted. Proper care coordination is critical to ensure that those with Sickle Cell Disease have the best available treatment, care, support, and possibility for effective recovery.
Jacquelyn Robinson, MSM, M.Ed., PHR, GPHR
Parent and advocate for Sickle Cell patients and families; founder of SCACURE Networks, Inc.