Care for Adult Thalassemics

by Laurice Levine, MA, CCLS

Thalssemia Outreach Coordinator

Children's Hospital and Research Center at Oakland

Thalassemia is a rare and complex disease which in the past took the lives of children prior to their teenage years. Thankfully, due to improved technology and medical advances, the lifespan for thalassemics now extends into adulthood. Consequently, serious consideration must now be given to adult care. Since thalassemia is a childhood illness, many adults are seen in pediatric hospitals-often, in centers of specialty for thalassemia. Compared to a decade ago, many more patients in thalassemia centers are now adolescents or older. The topic of adult care is especially important, as the adult population of thalassemics is ever-increasing and has now reached 44 percent of the total thalassemia population in the U.S. and Canada. (This number reflects the patients in the United States and Canada that are registered in the Thalassemia Clinical Research Network. Provided by Children's Hospital and Research Center, Oakland, California.) Unfortunately, pediatric hospitals are not fully equipped to meet the changing and complex needs of adults. This article will provide and overview of adult care, a time study, pediatric versus adult settings, challenges, and suggestions for improvement will be discussed. It is vital that the challenges surrounding adult care are addressed immediately-the quality of life for adults living with thalassemia will be improved, and learning from this generation will enhance the lives of the future, as well.

Medical Challenges

The aging process presents new challenges for adult thalassemics and the providers who care for them. Osteoporosis, complex cardiology issues, organ failure, and pain management are not childhood problems. Many of these medical challenges require adult specialists, but unfortunately, these specialists lack specific knowledge of thalassemia. It is difficult to find a provider who has understanding of the whole patient and the complexities of thalassemia care. Fertility and family planning are newer medical needs which are not often addressed, since OB-GYN specialists are not usually found in children's hospitals, and those in adult facilities lack training in thalassemia care. Education and collaboration among specialists is needed in this area for all parties involved.

Employment Opportunities

Many adults with thalassemia are faced with employment challenges. Disclosure and getting time off for treatment are primary issues. A solution for this is evening and weekend transfusion clinics. Changing jobs and self-employment can be daunting for an adult thalassemic due to insurance issues. Solving these issues would require structural changes in our society, government, and insurance coverage.

Medical Insurance

Pre-existing condition clauses, waiting periods, and lifetime caps are especially challenging to adult patients and reflect a social problem that must be addressed. The medical expenses that patients accrue can be grandiose, depending on the type of insurance and co-payments they have. Highly trained social workers can be an asset in efforts to educate patients on coverage and navigating the system.

Death, Loss, and Mortality

A difficult part of being involved in the thalassemia community is the inevitable loss that one experiences. The grief caused by losing friends who share the same disease is insurmountable. Another indescribable tragedy is when two or more siblings have thalassemia, and one dies. The loss of a friend or a sibling is coupled with many feelings, including guilt, blame, and fear. Patients do not frequently discuss their own mortality. Possibly, noncompliance is simply a form of denial until a patients becomes severely ill due to complications from iron overload. Support groups; education on services such as the durable power of attorney; and psychosocial and therapeutic interventions must be made available.

Adult Care

In the next part of this article, a look at adult care and compliance will be the focus. Care includes blood transfusions, Desferal therapy, doctor appointments, taking medications, medically related phone calls, and travel time. Six patients were asked to keep track of the time it took for all of their thalassemia care for one month. The average time these six patients spent on care was 271 hours. Beyond the "basic care," two adults had diabetes, with time for care totaling 755 hours. This included testing blood sugar and wearing an insulin pump. Two patients underwent hospital admissions which totaled 76 hours. The percentage of time for each care category is as follows:

Desferal = 91%

Transfusions = 5%

Appointments = 1%

Phone = 1%

Travel = 1%

Taking medicine = 1%

Pediatric versus Adult Settings - Pros and Cons

All of the specialty centers for thalassemia in the United States are pediatric hospitals. Having expert hematologists who specialize in thalassemia is the greatest benefit of being in a pediatric setting. Other benefits include participating in new medical research, new treatments, and the knowledge of comprehensive care. Exposure to other patients who share the same experiences lends itself to the building of support networks, both in the hospital and within the community.

The primary drawback of adult care in a pediatric setting is facilities' inability to deal with medical challenges that adult patients confront. Fertility, osteoporosis, and adult pain management are not often addressed in the pediatric setting. Another drawback is that not all medical specialties in the pediatric hospital provide adult care. Emergency room care, cardiology, and radiology are a few common examples of this. As a result, adult care becomes decentralized, and comprehensive care is compromised. Adults may see a pediatric hematologist but go elsewhere for all other services. It is difficult to find physicians who have an understanding of all aspects of thalassemia and how it pertains to their specialty. Communication and collaboration among providers is a challenge when care is decentralized.

Other challenges adults face in pediatric settings include being recognized as a patient with the same rights as a pediatric patient. There is a lack of privacy if and when admitted, and members of the medical staff often do not acknowledge that the adult is a capable of being an integral member of the medical team. If adults are proactive and speak up, they are often labeled as difficult patients.

In adult settings, providers are well trained in adult needs versus pediatric needs. Admissions are not an issue, and patients can be seen by all Services. Many providers are willing to communicate with a patient's thalassemia specialist to provide adequate care to the patient, as well as to assure their participation in new treatments and potential research studies. However, for the most part, this is a challenge due to lack of time and resources in the medical field in general. Lack of communication among providers can compromise patients' care and potentially lower their lifespan.

The transition from pediatric to adult care can be a challenge. It is a medical shift and a cultural shift, because pediatric practice differs greatly from adult practice. As mentioned before, providers lack knowledge of thalassemia, so collaboration with pediatric thalassemia specialists is vital both to provide quality care and keep abreast of new treatments and research.

I spoke with the thalassemics involved in the time study. I asked them what their primary challenges were as a child and what they are as an adult. They were candid in their responses, which are representative of the thalassemia patient community. As children, it was very difficult to learn about and understand thalassemia. It is a complex disease with high-maintenance treatments. All parents would agree how difficult it is to explain to small children why they have to be taken to a hospital on a regular basis and why they have to be stuck with a needle nightly to infuse medicine.

Unfortunately, many children are not taught about thalassemia in a developmentally appropriate way. Providers and parents often speak over their heads, and in an effort to protect them, information is often kept from them. This is detrimental to the building of trust, involvement in care, compliance, and coping. Children quickly learn that they are different and that something is wrong with them. Honest communication is crucial to their understanding and ability to deal with thalassemia throughout their lives. A child life specialist can be an asset to early intervention. Child life specialists can teach children what thalassemia is at their developmental level. The specialists can facilitate medical play and teaching so a child can gain understanding and mastery over thalassemia. By talking about thalassemia early on, children are given the words that they will always need to explain thalassemia to others. Learning how to deal with people when they learn about thalassemia is another challenge that applies to all patients and families. Throughout life, they run into barriers-whether at school, at work, at a doctor's office, in friendships, or in later, intimate relationships.

Other obstacles mentioned were overcoming the fear of needles as a child and absence from school, and later, work. Maintaining necessary energy and drive to continue to stay on top of medical issues and treatment, as well as constant preventative care, is another challenge. The time it takes to care for oneself is exorbitant - thalassemia never ends; there is no break from it, and therefore, it is no surprise that compliance is an issue. Insurance crises can prove to be as challenging as medical crises. Sadly, learning about mortality and life expectancy is another trial. One adult thalassemic said she felt like the worst part of having thalassemia was "dying slowly and periodically having another organ die or become impaired." More difficult to live with than scar tissue and scarred veins are the psychological scars of being reminded daily of one's own mortality throughout one's entire life.

Improving adult care must be addressed by all providers of thalassemia care, both in pediatric and adult settings. Optimal care for adults must be placed as a priority and will require hard work, commitment, and collaboration by all involved. Suggestions for improvement include centralizing care in pediatric settings. This would require collaboration and agreements with upper hospital management and insurance companies, thus lobbying and government mandates may be necessary. In order to centralize care in a pediatric setting, there must be a team of adult doctors in all the necessary specialties, as well as psychosocial services. These specialists will work alongside the thalassemia specialist. Education of all hospital staff regarding adult care would play a primary role in appropriate integration of adults into a pediatric setting. Evening and Saturday transfusion clinics and efficient registration systems would eliminate countless obstacles, such as time and employment issues.

To improve adult settings, a satellite hospital could be designated near a pediatric specialty center. Adult doctors would have to receive specific training in thalassemia in order to provide quality comprehensive care. Routine meetings and collaboration with the pediatric center would be required. Until there is more education in medical school for adult hematologists, improvement in adult settings appears to be more challenging than implementing an adult program in a pediatric setting.

There is no doubt that the brilliant minds of the providers who have worked so hard to keep patients alive until adulthood will be able to collaborate to improve the care of adults in both pediatric and adult settings. Optimal adult care will eliminate unnecessary challenges and will improve quality of life for this generation and many to come.