Disorders of the Blood and Immune System

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Disorders of the Blood and Immune System

• BCE 542 (Physical Disabilities, Rehabilitation
  and Employment)
• Dr. Dunn
• November 26, 2002

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Disorders of the Blood and Immune System

• Blood Dyscrasias
• Anemia
• Polycythemia
• Agranulocystosis
• Sickle Cell Diseases
• Sickle Cell Anemia
• Hemoglobinopathy SC
• Sickle Thalassemia
• Sickle Cell Trait
• Hemophilia
• AIDS/HIV Spectrum

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Blood Dyscrasias
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Anemia

• Reduced red blood cell count or weakened
  hemoglobin in bloodstream.
• Types
• Aplastic Anemia Malfunction of the bone marrow
  new red blood cells are not produced.
• Hemolytic Anemia Red blood cells destroyed
  faster than the body can produce them.
• Iron Deficiency Anemia The body is unable to
  metabolize iron, or iron intake is insufficient.
• Pernicious Anemia Inadequate intake or poor
  metabolization of vitamin B12
• Symptoms Exhaustion increased heart rate,
  pallor, shortness of breath
• Treatment
• Aims at correcting cause of problem dietary
  changes are often necessary.
• Blood transfusions may be necessary until
  individual can maintain an adequate red blood
  count.

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Polycythemia

• Overabundance of red blood cells, causing
  thickening of the blood.
• Difficulties in circulation can result in
  cardiovascular problems (stroke, heart attack,
  hypertension, congestive heart failure)
• Symptoms and causes are similar to those for
  anemia.
• Treatment aims at correcting cause of the
  problem, thinning the blood (coumadin, increased
  fluid intake).

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Agranulocystosis

• Reduction of neutrophils, a type of white blood
  cell, dramatically reducing the ability to fight
  infection.
• Usually caused by exposure to a variety of
  poisons.
• Medical emergency.
• Treatment Massive doses of antibiotics and
  removal of toxins/detoxification.

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Rehabilitation Blood Dyscrasias

• Usually not a serious concern if brought under
  control and managed.
• When disorder is active, individuals will have
  serious problems with mental and physical
  vitality person should not be placed in
  overly-strenuous activities.
• Unmanageable blood dyscrasias usually result from
  other, more serious disorders which will place
  additional restrictions on the individual.

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Sickle Cell Diseases
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What is a sickle cell?

• Normal red blood cells look like donuts have a
  life span of about 120 days.
• In sickle cells, blood cells have defective
  hemoglobin that wears down after 10-14 days as
  cells wear out they acquire a sickle shape.
• Sickle cells are destroyed more often in liver,
  causing the liver to overproduce bile and place
  more of certain amino acids into the bloodstream,
  causing jaundice.
• Sickle cells also cannot carry oxygen as
  efficiently as normal cells.
• A variety of other complications can develop
  (discussed shortly)

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Sickle Cell Disease Causes

• Abnormal hemoglobin is genetic and hereditary.
• Over 200 types of abnormal hemoglobin, most very
  rare.
• Most common in persons of African descent (1/500
  have disease 1/12 carry the gene).
• Also seen in persons of Mediterranean, Arabic,
  and South Asian ancestry.

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Three most common hemoglobin disorders

• Sickle Cell Anemia Most common form. Usually
  diagnosed shortly after birth peak in death rate
  at ages 2-5 due to hindered ability to fight
  disease. Becomes milder as individual ages.
  Person has an elongated, thin appearance growth
  usually stunted.
• Hemoglobinopathy SC May not show symptoms until
  adolescence/adulthood. Mortality peaks at ages
  40-60, usually due to cardiovascular
  complications (arteriosclerosis)
• Thalassemia Two forms One mimics sickle cell
  anemia and is more serious the other mimics
  hemoglobinopathy SC and is less serious.
• Sickle Cell Trait Individuals who carry one
  normal gene and one sickle cell gene usually show
  no symptoms, but may have some abnormal
  hemoglobin.

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Sickle Cell Crises

• Distinctive manifestations of sickle cell
  disease.
• Four Types
• Vaso-Occlusive or Painful Crisis Sickle cells
  clog and block blood vessels, causing infarction
  of tissue.
• Aplastic Crisis Red blood cells are destroyed in
  the bone marrow as they are created, causing
  lowered blood count.
• Splenic Sequestration Spleen enlarges, trapping
  red blood cells in the organ, lowering blood
  count.
• Hyperhemolytic Crisis Increase in pace of
  destruction of red blood cells.

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Other Complications

• Anemia
• Jaundice
• Cardiovascular concerns (heart murmurs,
  congestive heart failure, heart attack, stroke,
  arteriosclerosis).
• Damage to bones (marrow destruction, loss of bone
  mass, osteoarthritis, osteomyelitis).
• Pneumonia and pulmonary embolisms
• Damage to internal organs
• Damage to skin
• Infections, esp. Encephalitis, Septicemia
• Sexual Dysfunction (Priapism in men or continuous
  erection, causing damage to vascular structures).

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Sickle Cell Disease Treatment

• There is no cure for sickle cell disease.
• Treatment
• Transfusions in certain circumstances.
• Avoidance of infections (can bring on sickle cell
  crisis)
• Management of complications
• Prevention (Genetic Counseling)

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Sickle Cell Disease Rehabilitation

• Disease is unpredictable planning may be
  difficult.
• Chronic fatigue is the major physical limitation.
• Physical capacities vary widely--all persons with
  sickle cell can do sedentary/light work, but
  others may be able to perform medium to very
  heavy work.
• Heavy lifting may cause complications in some
  persons.
• Environment should be adequately oxygenated not
  cold (increases cell sickling).
• Disease causes thirst person should have ready
  access to drinking water and toilet.
• Stress may bring about crises--may need to be
  avoided.
• Complications of the disease can impose
  additional functional restrictions.

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Hemophilia
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Causes of hemophilia

• Hemophilia is an insufficient ability of the
  blood to clot, caused by a lack of one or more of
  13 clotting factors present in the blood.
• Two major types
• Hemophilia A (Classic Hemophilia) Lack of
  clotting factor VIII accounts for 80 of cases
• Hemophilia B (Christmas Disease) Lack of
  clotting factor IX accounts for 19 of cases.
• Hemophilia A and B are caused by a sex-linked
  inherited gene and are seen only in males.
  Hemophilia in women is exceedingly rare and is
  only brought about as a result of a number of
  disorders such as Von Willebrands Syndrome,
  which make up 1 of hemophilia cases and can
  occur in either sex some female carriers of
  hemophilia genes may show some mild symptoms.

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Hemophilia Symptoms, Complications, Treatment

• Inability to clot can make even minor wounds
  life-threatening.
• Hemoarthroses Spontaneous bleeding in the
  joints causes swelling, pain, loss of range of
  motion. Can lead to osteoarthritis.
• Risk of hemoarthroses can make exertion and
  prolonged sitting/ standing difficult.
• Treatment Replacement of clotting factors with
  those derived from human donors.
• Can be done in the home.
• Very expensive
• Person is at heightened risk of developing
  blood-borne diseases and infections.

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Hemophilia and Rehabilitation

• Sedentary and light work should be considered,
  although some persons can tolerate medium
  exertion.
• Dangerous work should be avoided for obvious
  reasons.
• Psychosocial implications Children can grow up
  sheltered, overprotected can be emotionally and
  vocationally immature.

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Human Immunodeficiency Virus Spectrum

• Note HIV Spectrum is now the preferred term to
  refer to this disorder, rather than AIDS.

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HIV and the Body

• The human immunodeficiency virus attacks the
  immune system by way of T-cells, rendering the
  body incapable of fighting off infections.
• We are exposed to viruses, germs, and fungi which
  our immune system normally can fight off easily.
• If the immune system is weakened, these organisms
  can easily infect the body, however.

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Opportunistic Infections

• Pneumocystis Carinii pneumonia Caused by a
  parasite common in the body.
• Toxoplasmosis Brain infection Caused by
  microorganisms commonly carried by cats.
• Oral Thrush/Candidiasis Caused by candida
  fungus painful, raised white blothches in
  mouth/throat. A form of yeast infection.
• Kaposis Sarcoma Opportunistic cancer,
  manifested in red or purple blotches in the skin.

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Testing for HIV infection

• Opportunistic infection may indicate HIV
  infection calls for testing
• ELISA test Initial test for HIV.
• Western Blot A confirmatory test administered if
  the ELISA test shows positive result for HIV
  infection.

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Course of Disease

• Old classification
• HIV infection showing no symptoms
• AIDS-Related Complex (ARC) Bouts with
  opportunistic infections increasing in severity.
• AIDS/Full Blown AIDS Massive infections leading
  to death.

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Course of the Disease

• New classification, four groups based on t-cell
  count
• The HIV Spectrum
• 1 Evidence of recent infection (usually flu-like
  symptoms)
• 2 Asymptomatic, but infected.
• 3 Swollen lymph nodes over extended period of
  time.
• 4. Opportunistic infections.

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Treatment of HIV

• There is no cure for HIV infection. Treatment
  attempts to support immune system, treat
  opportunistic infections.
• HIV now may be regarded as a manageable disease
  in many persons, much like diabetes, because of
  new drug treatments.
• Combinations of drugs (drug cocktails) have
  allowed persons to remain relatively symptom free
  for longer and longer periods of time.
• Drug cocktails may have serious side effects
  (nausea, lethargy, development of abnormal facial
  fat).

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HIV Rehabilitation Concerns

• HIV is an extremely difficult disease to catch.
• Unreasonable fear and loathing remain the
  greatest obstacles to employment for persons with
  HIV.
• Persons with HIV may have no functional
  limitations for many years--often, no
  rehabilitation is needed.
• As disease progresses, and individual becomes
  more ill, functional limitations will be present.
  Often, these may be so severe or swift as to
  prevent the individual from participating in
  rehabilitation.
• HIV may more often be a secondary concern in
  rehabilitation, rather than a primary disability.
• Persons in rehabilitation who have HIV should
  avoid crowds and be cautious if employed in
  health care occupations--not because they can
  infect others, but because others might infect
  them with communicable diseases.