Transitioning to Adult Healthcare in Thalassemia

As young adults with thalassemia transition to adult healthcare settings, it is important for them to become comfortable with several new responsibilities, including:

• Scheduling their own medical appointments with the thalassemia provider and any subspecialists.
• Scheduling blood transfusions and necessary medical tests.
• Understanding the reasons why they may need blood transfusions and why they must take particular medications.
• Being familiar with the complete list of medications they take, knowing how to obtain these medications, and keeping track of when medications must be re-ordered.
• Knowing how to contact their medical providers if they have any questions.
• Knowing which emergency room to go to, if needed, and how to contact their regular providers in emergency situations.
• Learning about health insurance and what is involved in obtaining coverage.
• Learning to advocate for their own healthcare needs to help ensure they receive the best possible care.

The earlier the transition process begins for a patient, the more prepared the patient will be in the long run. Many patients start preparing for transition during their early teenage years to allow sufficient time to practice the necessary skills in making their own decisions and managing their own healthcare.

Early teenagers should start to become knowledgeable about their condition and their medications. They can be responsible for taking their own medications. They can also start to identify when a medication refill is needed and know how the medication is obtained.

Late teenagers may also practice making their own healthcare appointments and ordering prescription refills. In addition, they should start to see their doctors alone—for at least part of the doctor’s visit—to gain independence in managing their own healthcare.

Teenagers may have conversations with their doctors about what their privacy rights will consist of once they turn 18 years of age. They may also talk to their parents and healthcare providers about the age at which they would like to make the transition to a new doctor for adult care—or if they will need to find a new doctor if they end up going away for college.

The pediatric provider should be able to recommend one or more experienced adult thalassemia providers in a patient’s local area as they begin to transition to adult care. If they are unable to do so, the Cooley’s Anemia Foundation (CAF), which advocates for U.S. patients with thalassemia, can help patients find an adult provider with thalassemia experience in their local area.

To assess whether the potential new adult provider may be a good fit, patients should contact their office and arrange for a new patient visit. This will give transitioning patients an opportunity to meet the program physician and nurse in order to get a sense of their thalassemia knowledge and experience. It may also allow patients to take a tour of the transfusion and clinic facilities.

It is very important for patients to ask questions during this initial visit regarding their care, so that they know what to expect if they were to become a patient within that practice.

Some important questions to ask of the new adult provider include:

Transfusions
Where are the transfusions administered? What are the hours of operation for the transfusion clinic? What is their protocol for typing and screening, as well as for performing transfusions? Is transfused blood matched for C, E, and Kell antigens? How are transfusion reactions managed? Will it be necessary to see the physician before each transfusion? What are the pre-transfusion hemoglobin target ranges? How would they adjust the transfusion regimen if a patient’s pre-transfusion hemoglobin level was lower or higher than the target range?

Iron overload monitoring and management
How often is serum ferritin monitored? Do they have an MRI machine that specifically quantifies liver iron concentration (LIC) and how often is this testing performed? Is cardiac MRI T2* available, and how often is this testing performed? When putting patients on a new iron chelator, how often do they monitor to see if the medication is effective or if it causes any side effects?

Monitoring for complications
Does the practice have specific subspecialists they can refer patients to for any thalassemia-related complications? These subspecialists might include an endocrinologist, a hepatologist (liver specialist), a cardiologist, an ophthalmologist, an audiologist, and a reproductive medicine physician.

Emergency care
What is the protocol if a patient should develop a fever (for example, stop chelation immediately and contact the hematologist)? Who should patients contact if they feel unwell, develop new symptoms, or experience a transfusion reaction? In the event of an emergency, which Emergency Room are patients referred to, and what protocols are followed regarding their care?

Patient support
Does the practice have a nurse with experience in thalassemia care? Does the practice have a social worker available to thalassemia patients?

Patient services
How many other patients with thalassemia are currently managed in the practice? Do they offer thalassemia support groups?

Optimizing patient care (for patients in smaller centers)
Does the provider consult with a thalassemia specialist in a Thalassemia Treatment Center when needed? Would they consider jointly caring for a patient together with a thalassemia specialist at a Thalassemia Treatment Center? How does the provider keep up-to-date with advances in thalassemia care?

Clinical trials
Can the provider directly enroll patients in clinical trials, or can they refer patients to another provider who can enroll patients in a trial?

Young adults with chronic health conditions are a vulnerable patient population. Sometimes their health conditions worsen as they become fully responsible for managing their own healthcare, especially if they have not been adequately prepared to take on these new responsibilities. This worsening of their health status may be associated with reduced use of medical services and reduced adherence to their prescribed medications – especially iron chelation medicine.

Many patients who undergo the transition to adult care report the difficulty of leaving their pediatric providers, with whom they have had long-standing relationships. Sometimes these patients feel abandoned by their pediatric team, even though they are aware that hospital rules sometimes prohibit pediatric providers from treating and/or admitting adult patients into a pediatric hospital if necessary.

Transitioning patients may also feel distrustful of the new adult care team, who may spend less time assisting them than their pediatric providers did and may therefore appear to be “less invested” in the patient’s care. Processes and procedures in the adult clinic/hospital may also be different from what the patient experienced in the pediatric center, which can take time to adjust to.

Finally, the transition to adult care comes at a time of life when young adult patients are undergoing many other life transitions, all of which may leave the patient feeling overwhelmed.

Going off to college can be an exciting time of life and a key step in developing independence for young adults. Because their health needs are of critical importance, many college-bound patients find it helpful to visit the campus student health center as well as a nearby medical center with thalassemia expertise when they go on tours of potential colleges. Patients who need help finding an experienced thalassemia physician/medical center at or near their college of interest are welcome to reach out to the Cooley’s Anemia Foundation (CAF), which is in contact with thalassemia physicians throughout the U.S., for assistance.

Once a patient has identified a college or university which offers an academic program that suits them, they can begin to investigate the thalassemia treatment center options that are nearby. If the college is located near an experienced thalassemia treatment center, patients can reach out to them to see if they are able to accept new patients. Many pediatric programs will accept college students, who may remain with the program through college graduation. However, if there isn’t a large, well-established thalassemia center nearby, then patients may still be able to get excellent thalassemia care while in college by partnering with a local hematologist for ongoing care (transfusions, monitoring for iron overload, chelation monitoring, and referrals for local subspecialty care). This care can be supplemented by visiting a specialized thalassemia treatment center that cares for a large number of thalassemia patients for a comprehensive annual evaluation once per year. The highly experienced thalassemia center will review the patient’s health records, care plan, and latest test results and will collaborate with the local hematologist to optimize the patient’s care in order to keep the patient healthy for the long term.

Another important step is for patients to figure out which type of insurance coverage they will have in college and whether this will be accepted by the thalassemia clinic that is at or near the college. It will then be helpful to connect the new thalassemia provider at college with the pediatric provider so that the medical records can be transferred and the physicians can speak about the patient’s healthcare needs. Patients may want to remain in contact with their pediatric thalassemia program until they feel that they have established a good relationship and management plan with their new adult care team. In fact, patients attending college may continue to see their original pediatric providers when they are home on holiday breaks or for summer vacation.

Patients may find that their adult care providers do not have as much experience with thalassemia as their pediatric providers did. Patients should know that they may still be able to get very good care in such a situation, but this will depend on the patient knowing the recommended care guidelines for thalassemia and advocating for themselves if the new care team is not aware of these guidelines. The Cooley’s Anemia Foundation has some Thalassemia Management Checklists available on their website on topics such as managing transfusion therapy, monitoring iron overload, and monitoring deferasirox therapy, which may help with this. These checklists can be found here.

Also, patients may want to prepare for the possibility of an emergency by letting trusted people – such as their roommate, college residence advisor, and/or friends – know about thalassemia and how it may affect their health. It can also be helpful for patients to stay connected to their family and friends from back home – even though they may have moved far away from them – as they can continue to be a strong source of support. Friends from the thalassemia community can be another key source of understanding and support, regardless of where patients end up living. Finally, the more patients can do to maintain a healthy lifestyle (getting a proper amount of sleep each night, eating healthy foods, and exercising) while in college, the better they will feel as they begin to manage on their own for the first time.

Patients become legal adults at 18 years of age and are therefore legally responsible for their own care. At that point, the doctor will talk directly to the patient – not to his or her parents – about the patient’s health. In fact, parents/guardians cannot access the patient’s medical records and need the patient’s approval to be in the room at the doctor’s visit. Confidentiality between a patient and his/her doctor is legally known as the Health Insurance Portability and Accountability Act (HIPAA). HIPAA is a federal law that gives a patient legal rights over his/her health information and sets rules and limits on who can look at and receive his/her health information. Any health-related information found in a patient’s medical records, conversations with providers about the patient’s care, as well as patient information in insurance providers’ computer systems and billing information are all protected under HIPAA. The patient’s consent is required for anyone to access this information. As an example, transitioning patients may be asked to provide consent prior to sharing their information with their new adult thalassemia doctor. Parents will also not be given any information directly from the adult thalassemia doctor without the adult patient’s permission.