Inilah DIRIKU....
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.
Understand Me ? NO !
