Primary Care for the Cancer Survivor

Question 1

Who is considered a cancer survivor?

Answer 1

• anyone who has been dx with cancer from the time of the initial dx until the end of their life

Question 2

cancers with hghest survival rates?

Answer 2

• female breast
• prostate
• colorectal
• gynecological

Question 3

Sequelae of cancer tx?

Answer 3

• physical/medical: 2nd cancers, cardiac dysfxn, pain, lymphedema, sexual impairment, infertility
• psych: depression, anxiety, uncertainty, isolation, altered body image
• social: changes in interpersonal relationships, concerns regarding health or life insurance, career issues, return to school, financial burden
• existential and spiritual issues: sense of purpose or meaning, appreciation of life

Question 4

What number of survivors of childhood cancer will experience at least one late effect?

Answer 4

• 2/3 of the survivors will experience at least one late effect, and about 1/3 will experience a late effect that is severe or life threatening
• need to emphasize long term f/u care for these childhood survivors (risk of developing these complications doesn’t plateau with time)

Question 5

Key components of establishing optimal healthcare for these childhood cancer survivors?

Answer 5

1) : longitudinal care plan utilizing a comprehensive multidisciplinary tearm approach
2) continuity with single health care provider coordinating needed services
3) an emphasis on the whole person, with sensitivity to the cancer experience and its impact on the entire family

Question 6

What survivors are at the highest risk for late term effects? What do these survivors experience?

Answer 6

• bone tumors: pts commonly experience disfigurement and fxnl limitations caused by amputations and other surgeries. Additional risks: problems with fertility, heart and kidney damage and 2nd cancers related to tx
• CNS tumors: often the most severely affected, particularly if they received radiation, chemo and surgery. Potential difficulties: cognitive impairment, short stature, hearing loss, problems with balance, and coordination, hypothyroidism, thyroid nodules, kidney damage and 2nd cancers
• hodgkins lymphoma:
  pts may experience lung damage, abnormal skeletal growth and maturation, infertility, and hypothyroidism. Females who received chest radiation are at increased risk for breast cancer up to 26% greater than national avg.

Question 7

What are the main disabilities that 60% of ped cancer survivors face secondary to tx later on?

Answer 7

• secondary malignancies
• growth complications
• endocrine complications
• cardiopulmonary complications
• renal complications
• neuropsychological/psychosocial complications

Question 8

How common are secondary malignancies in childhood cancer survivors?

Answer 8

• up to 12% of ped pts will develop a new cancer w/in 20 yrs. 10x increase over the general ped population.

Question 9

Most common tx causes of secondary cancer in ped pts?

What increases the risk of certain malignancies?

Answer 9

• exposure to alkylating agents and radiation therapy are MC causes
• genetic and familial conditions increase the risk of certain malignancies: retinoblastoma, neurofibromatosis, nevoid basal cell carcinoma

Question 10

What are growth complications a result of? Who are at the highest risks for this? What may these pts benefit from?

Answer 10

• Result of direct damage to endocrine tumor
• kids with ALL, brain tumors, orbital tumors and nasopharyngeal cancers who have received radiation are at highest risk
• 90% of pts that receive over 30 Gy of radiation to CNS will show growth hormone deficiency within 2 yrs and 50% receiving 24 Gy
• Kids under 5 at time of radiation are most vulnerable
• these pts may benefit from receiving growth hormone therapy

Question 11

Growth complications related to spinal radiation?

Answer 11

• spinal radiation inhibits vertebral body growth
• asymmetric exposure of the spine may result in scoliosis
• chemo alone may result in attenuation of linear growth: this is usually temporary, as a period of catch up occurs when the drugs are d/c

Question 12

What endocrine complications may childhood cancer survivors face if given cranial radiation?

Answer 12

• in addition to growth hormone deficiency, prepubertal kids given cranial radiation may experience early puberty secondary to premature activation of the hypothalamic - pituitary gonadal axis: this results in premature closure of the epiphyses, which translates to decreased growth and ht (more common in girls)

Question 13

When would thyroid dysfxn be common in childhood cancer survivors? What levels will be abnormal?
Tx?

Answer 13

• common in those receiving brain/neck radiation
• this can manifest as early as 6 months and as late as 7 yrs
• they will usually have normal thyroxine levels with abnormal TSH: they should be considered for thyroid replacement therapy b/c persistent stimulaiton of the thyroid from an elevated TSH may predispose them to thyroid nodules and carcinomas

Question 14

Endocrine complications:
Gonadal dysfxn from radiation can result in what in childhood cancer survivors? What pts are at the highest risk for this?

Answer 14

• result in azospermia, low testosterone levels and delayed sexual development:
• pts who receive testicular radiation as part of therapy for ALL, abdominal radiation for Hodgkinds or total body radiation are at highest risk
• ovarian dysfxn from radiation cn result in failure to undergo menarche, increased FSH and LH levels and low estrogen

Question 15

What may girls receiving craniospinal radiation for ALL develop and what are they at increased risk for?

Answer 15

• may develop delayed menses and are at risk for early menopause
• women who have received abdominal radiation and develop uterine vascular insufficiency or fibrosis of the abdominal and pelvic musculature or uterus may have an increased risk of perinatal death or low-birth wt, premature infants, and their pregnancies should be considered high risk

Question 16

What are the cardiopulmonary complications survivors of childhood cancer may develop? What drugs cause these?

Answer 16

• several chemo agents are known to cause cardiopulm dysfxn:
  Bleomycin, nitrosoureas, cyclophosphamide, methotrexate: these may also cause pulmonary fibrosis ( restrictive lung disease - will have decreaed CO diffusion and small lung volumes)
  Anthracyclines (Doxorubicin): CHF - myocytes are destroyed and lead to inadequate myocardial growth as the child ages. Arrhythmias are also common due to changes in the electrical cell structure

Question 17

Radiation therapy to the mediastinal region has been linked to what? What kids are at the greatest risk?

Answer 17

• linked to increased risk of CAD and chronic restrictive pericarditis
• kids tx under the age of 4 with mediastinal radiation or anthracycline are at the greatest risk

Question 18

Renal complications in childhood cancer survivors? What drugs causes these?

Answer 18

• msot long term complications stem from chemo
• cisplatin may cause an abnormal glomerular filtration rate and persistent tubular dysfxn
• cyclophosamide can cause hemorrhagic cystitis which can lead to increased chance of bladder CA
• ifosfamide can cause fanconi sydnrome (proteinuria, glycosuria, phosphaturia with hypophosphatemia) which may result in clinical rickets (hypophosphatemic rickets as opposed to nutritional rickets) if adequate phosphate replacement isn’t provided

Question 19

Neuropsych complications of childhood cancer survivors? Who are at the highest risk? What pop is more susceptible?

Answer 19

• pts who received cranial radiation for ALL or brain tumors are at highest risk
• severity depends on dose, size and location of radiation field, child’s age when radiation was admin and gender
• girls appear to be more susceptible to CNS toxicity b/c they have more rapid growth development during childhood
• main effects of CNS radiation appear to be related to visual processing speed, visual motor integration sequencing ability and short term memory

Question 20

Psychosocial complications for childhood cancer pts?

Answer 20

• absence from school, frequent medical appts, hospitalizations may lead to social isolation
• adolescent survivors may demonstrate an increased sense of physical fragility and vulnerability manifested by hypochondria or phobic behaviors

Question 21

Medical and psycho-social goals for childhood cancer survivors?

Answer 21

• medical: early recognition and tx of late effects - improve quality of life and decrease healthcare costs
• psycho-social: est. a new normal - social life and school, work

Question 22

What are the outcomes of over screening and under screening?

Answer 22

• over: undue fear, unnecessary screening, high costs
• under: missed late effects (potentially life threatening), missed early intervention, missed chance to minimize morbidity, in the long run will have higher healthcare costs

Question 23

What should every survivor know about their cancer?

Answer 23

• what kind of cancer they had
• what kind of chemo and radiaton they had and how much
• any adjunct therapy
• name of their oncologist/radiation oncologist

Question 24

Routine monitoring for pts that underwent CNS radiation?

Answer 24

• growth needs to be closely monitored, (ht, wt, growth velocity, scoliosis screening, possible growth hormone testing) and neuro psych screening yearly

Question 25

Routine monitoring for pts that underwent chest radiation?

Answer 25

• thyroid monitoring, PFTs, cardiac workup for cardiac toxicity, breast CA monitoing in females

Question 26

Routine monitoring for pts that underwent abdominal/pelvic radiation? Male and female specific?

Answer 26

• renal: UA, CMP, eGFR, BP, renal US, possible cytoscopy
• males: monitor testicular size, semen analysis, test. levels, FSH and LH levels
• females: detailed menstrual hx, FSH, estrogen levels,uterine US

Question 27

Monitoring for cardiac toxicity based on risk levels?

Answer 27

• low risk(just need one to meet criteria): took less than 250 mg of anthracyclines, no radiation, and you were older than 5 at time of tx
• moderate risk (just need one): radiation to chest or neck, had more than 250 mg of anthracyclines but less than 400 mg, or pt was less than 5 with tx
• high risk (just need one): anthracyclines plus radiation to the chest, dose was over 400 mg, or pt had pre-existing cardiac disease plus antrhacycline tx

Question 28

Adult cancer survivors are at increased risk for what?

Answer 28

• recurrence of original cancer and development of second primary malignancies as result of cancer therapy and other RFs
• prolonged monitoring and tx are warranted for long term SEs of surgical, radiation or cytotoxic agents

Question 29

Goals fo f/u care for these adult cancer survivors?

Answer 29

• prevent premature mortality
• prevent or detect early physiologic or psychosocial sources of morbidity
• mange (or refer for management) of co-morbidities
• screen for 2nd cancers

Question 30

What are the components of surveillance after breast cancer?

Answer 30

• monthly self-breast exam, clinical breast exm q 6 months for 5 yrs and then annually, mammogram annually
• surveillance for secondary cancers:
  increased risk for ipsilateral and contralateral breast CA, and CRC
• PE: lymphedema, premature menopause, osteoporosis, uterine CA
• psychosocial: distress about risk of recurrence, sexuality, body image, depression, anxiety
  other considerations: assess age at dx and family cancer hx, consider referral for BRCA 1&2 mutations, annual pelvic exam, screen for CRC and cervical cancer, pneumococcal and flu vaccines, assess psychosocial fxn

Question 31

What are the components for surveillance after prostate cancer?

Answer 31

• look for recurrence: clincial eval, PSA q 6 months for 5 yrs, and then annually, DRE annually
• surveillance for secondary cancers: increased risk for bladder CA
• PE: sexual dysfxn, bowel or urinary incontinence, radiation proctitis, or diarrhea
• psychosocial: depression, sexuality
• other considerations: assess age at dx and family hx of CA, consider referral for genetic counseling and assessment if strong family hx, CRC screening, pneumococcal and flu vaccinations, assess psychosocial fxn

Question 32

Surveillance components after CRC?

Answer 32

• surveillance for recurrence: CEA, clinical exam q 3 months for 2 yrs, and then q 6 months for 3-5 yrs, CT scanning q 3-6 months for 2 yrs and then q 6-12 months for a total of 5 yrs, colonoscopy after one year and then at 3 yrs, and then q 5 yrs
• survey for 2nd cancers - CRC at diff site
• PE: ostomy care, rectal incontinence, radiation proctitis, or diarrhea, adhesions
• psychosocial: sexuality, body image, depression
• other considerations: assess family cancer hx for FAPm HNPCC, refer for genetic counseling and assessment, breast and cervical cancer screening, pneumococcal and flu vaccinations, assess psychosocial fxn

Question 33

Risk of late effects anf impact of long term effects decreases or increases with time?

Answer 33

• INCREASES with time!!!