Guidelines for Psychological Support to Children and Adolescents with Beta Thalassemia Major

A Cochrane review found no form of psychological therapy in the medical literature so far that would aid patients with chronic illnesses like Thalassemia to cope with the disease (Anie, 2001). Thalassemia is a genetic disease. It is a major healthproblem in Southeast Asia, especially in northeastThailand. It has become the most visible disease withabout 1% of the Thai population suffering from it.Thailand was found to have 600,000 cases of thalassemia.About 40% of the population are carriers, and thesepeople have the possibility of transmitting thalassemic disease by giving an abnormal allele through eachchromosome of 11th or 16th pair chromosome. The phenotypesare mostly expressed by the autosomal pattern of Mendelian law (Fucharoen et al., 1991; Panich, Pornpatkul,& Sriroongrueng, 1992; Weatheral& Clegg, 2001).Thalassemic patients must be cared for continuouslythroughout their lives. In addition, they need to begiven a helping hand by their families for continuoustreatment of regular or occasional blood transfusions and iron chelators. The clinical manifestations dependon severity. These patients usually present withpallor and exhaustion, and are unable to do hard work, depending upon the degree of anemia and their tolerance (Cappellini, Cohen, Eleftherion, Piga, & Porter,2000; Cazzola et al., 1995, 1997). There are indicationsof abnormality in the face and nose. Thalassemic facies may include enlargement of the zygoma, maxillar, frontal, and occipital bones and a flat nose. Patientsalso have enlarged livers and spleens, abdominaldropsy, irregular heart beat or heart failure, infections, and short lifespan (Anie&Massaglia, 2001; Goldbeck,Baving, &Kohne, 2000; Louthrenoo, Sittipreechacharn,Thanarattanakorn, & Sanguansermsri, 2000).Moreover, the disease affects patients and motherspsychologically; for instance, with feelings of unacceptable, loss, grief, and blame (Aydin, Yaprak, Akarsu, Okten, &Ulgen, 1997; Politis, 1998). Furthermore, the impact ofthe sickness results in changes in children’s appearance.Children will be stunted and pale and will have thalassemic facies, which for the parents is a constantreminder of their failure to give birth to a normal child.Because of these factors, many parents are stressedbecause of their child’s sickness. Literature reviews andempirical findings in Western countries indicate thatthalassemia major, as a chronic illness, has a negativeimpact on the quality of life for a mother with a thalassemic child (Caro et al., 2002; Gold beck et al., 2000; Zahed et al., 2002).Additionally, many empirical findings have shownthat Thai mothers sometimes may be overprotective, overindulgent, or over concerned in regard to theirchildren. This results in a stunted process of self development in the thalassemic child. The emotionalstrain on Thai mothers is even greater when theylack knowledge about the disease and support from their society (Suwanpatikorn, 2001; Tedsiri, 1994). Thalassemia imposes a significant intrusion in the lives of the patients and their families. The effects are many, sweeping from financial hardships and absence from school and work, to significant issues with self-image and self esteem. All of these issues have a tremendous impact of the effectiveness of therapy and on the quality of life of the patients. These challenges are further complicated by normal stages of development incurred from infancy to adulthood and by vast cultural differences. This latter point cannot be emphasized enough. Because of the ethnic predilection of thalassemia, the patients come from diverse cultural backgrounds, which are usually different from those of their healthcare provider. Thus, all professionals who provide care and support to these patients must be aware of the cultural, social, developmental, and behavioral issues that affect this diverse population. Medical and psychosocial professionals must also be intimately involved with each other in order to provide optimal care to their patients. Referral to outside providers who have no knowledge or understating of the medical problems is generally ineffective. Behavioral problems have great bearing on compliance with therapy and thus with medical outcome as well as with quality of life for patients with chronic disease.

General Considerations 

Ensure regular assessment of the child’s development through multi-disciplinary review of the children by clinical psychologist, child play team services and social worker at defined milestones and after important medical or social events. This should include:

  • Relationship with parents, peers, siblings
  • Schooling issues
  • Self esteem
  • Identity
  • Coping skills
  • Attitude towards growing up with Thalassemia

Child Play Team Services:

Culturally sensitive play team services are an integral part of a patient’s comprehensive care. Child play team services assure that care is family center and developmentally appropriate for the patient. It is imperative that patients with Thalassemia understand their disease and treatment in order to comply with their rigorous medical regime. Child play team programs in health care settings minimize psychological trauma and promote optimal development of children and their families.

Interventions

  • Through observation and discussion, assess the response of the patient and family to health care experiences and develop a plan to meet their needs and assist with coping.
  • Provide opportunities for gaining a sense of mastery, for play, for learning, for self expression, for family involvement and for peer interaction. This can be achieved in many ways including medical play and art therapy.
  • Provide a positive growth experience for patients. Minimize stress and anxiety for the patient, parents, and siblings.
  • Prepare children and families for health care experiences. Example: conduct a medical preparation prior to a patient’s liver biopsy and splenectomy. This increases overall understanding of the procedure and reduces anxiety.
  • Provide essential life experiences such as play, school, peer-interaction, community events and activities. These experiences commonly take place in a playroom or School room.
  • Create opportunities that strengthen self-esteem and independence.
  • Child play specialist communicates and collaborates with other members of the health care team.

Cultural sensitive psychological services make up a critical part of all comprehensive care plans for patients with chronic illness. In particular, those with rare chronic diseases that havehigh morbidity and require time intensive therapy and medical intervention such as thalassemia. A psychologist will provide consultation to the MDT about the ongoing assessment and intervention for children and their families to ensure that their psychosocial needs are addressed. This includes supporting others involved in the child’s care on a regular basis to help children and families cope with issues related to the morbidity of thalassemia and fear of dying. Children who had complex psychosocial needs flagged up by the MDT should seen by a psychologist for direct assessment, case formulation and intervention.

Interventions

  • Evaluation of general functioning and adaptation to chronic illness and to local culture.
  • Evaluation of ability to comply with and cope with medical regimen.
  • Assistance and intervention with issues of compliance and coping styles should be considered for every patient.
  • Organization and direction of therapeutic groups for adolescents.
  • Organization and direction of therapeutic groups for adolescents. • Evaluation and referral to psychiatrist is indicated if serious psychological difficulty or psychiatric disturbances were identified.

Social Services:

Social services that meet the needs of the patients in a culturally sensitive way are critical for patients with chronic disease that requires an inordinate amount of resources.

Interventions

  • Evaluation of functioning in the community and school.
  • Provision for adequate social and medical services.
  • Services for assessment of medical need and financial assistance.

Exercise and physical therapy

There are three clinical classifications of beta thalassemia in common use according to the Joint Center for Sickle Cell and Thalassemic Disorders.

1. Thalassemia minor may have no symptoms at all and does no damage to the body.

2. Thalassemia intermedia can affect your body and may sometimes require transfusion. It can also starve the cells of oxygen enough to interfere with normal bone growth.

3. Thalassemia major can require transfusions for survival, and often interferes with normal physical development. There are similar classifications for alpha thalassemia. Since these are clinical classifications, there are no absolute lines between the groups.

Bone Formation

Thalassemia results in a lack of oxygen in the cells due to the defective hemoglobin. Cells require oxygen to function and to reproduce. Bone formation requires oxygen, calcium, phosphorus and vitamin D as well as some other substances and hormones. Oxygen starved bone cells do not develop correctly and deformity can result. Even if all the other elements are present, if the oxygen is not, the bones cannot grow and harden. Improving the circulation through exercise can help as does treating the thalassemia.

Low Bone Mass

If you have thalassemia, you must take measures to keep your bones strong. Your doctor can arrange for periodic bone density tests, assess your nutritional status, and decide if you need to take any precautions with exercise. Your part includes cooperating with your doctor’s advice, not smoking, following a nutritious diet with enough calcium and vitamin D and participating in regular exercise. The pamphlet, “Low Bone Mass in Thalassemia”, recommends weight bearing exercises to strengthen bones. For adults, it suggests “brisk walking, jogging, running, aerobics, step classes, dancing, and circuit training”. Exercise can help prevent fractures and bone deformities. However, be sure to do only the exercises that your doctor approves.

Bone and muscle

Brittle bones will increase the risk of fracture during many types of exercising but especially with heavy weight lifting and resistance training.Exercising on a regular basis is an important point for maintaining healthy growth and development. İt improves oxygenation and muscle development if enough hemoglobin ispresent.For strong bones, brisk walking, jogging, running, aerobics, step, dancing, circuit training, weight bearing exercises will be suggested according to the person’s medical condition. If the person is anemic, enough oxygen will not be supplied to muscles during exercise, resulting in tiredness and muscle pains. Thalassemic patients may be suggested to receive transfusions 2 days before known physical activity programs.

Heart and lung

Heart problems and restrictive type of lung problems are main hindrances against exercise capacity and mostly occur due to iron overload.Studies regarding lung function are contradictory. Cooperetal demonstrated a restrictive pattern in some patients that tended to correlate with age- duration of the disease. Keens et al. described abnormalities in pulmonary function tests suggesting small airways dysfunction which could be due to iron deposits in peripheral airways.Several authors suggested the possibility of pulmonary vascular involvement by microthrombi. Then most studies have revealed either an obstructive but most frequently a restrictive pattern of lung pathology. C. Cracowski et al. (1998) investigated lung function, exercise testing and echocardiography a few days after transfusion and found a significant impairment in exercise capacity in adult thalassemic patients which was not observed in children. No evidence was found in favour of pulmonary vascular or parenchymal involvement responsible for exercise limitation. The abnormal cardiac response was suggested to contribute to the exercise limitation observed.There are some reports that immediately after transfusion, for thalassemic patients with a history of airway obstruction, had a worsening of their airwayobstruction after transfusion. This was postulated to airway narrowing due to airway wall vascular engorgement or edema induced acutely and aggravated after transfusion. (Santamaria et al. 1994, Villa et al.1996) Many studies didn’t find a correlation between respiratory function impairment and body iron content (Dimopoulou et al. 1999, AM Li et al.2002).

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