An Interdisciplinary Model for Primary Ovarian Insufficiency Care

Transcription

1 An Interdisciplinary Model for Primary Ovarian Insufficiency Care April 21, 2017 Catherine Gordon, MD, MSc Lesley Breech, MD Christina Stewart, LISW-S Deborah Morse, MSN, RN, CPN, CNL

2 Institute of Medicine There is a dearth of clinical programs with the multidisciplinary infrastructure required to provide the full complement of services needed by people with common chronic conditions. (IOM, 2001)

3 Traditional Health Care Poorly organized Overly complex and uncoordinated Wastes resources Leaves unaccountable voids in coverage Leads to loss of information Fails to build on the strengths of all health professionals involved (IOM, 2001)

4 Interdisciplinary Care Avoids potentially dangerous and costly boundary crossing Makes referrals and transitions safer, more effective and less costly Facilitates collaborative clinical research (IOM, 2001)

5 Effective Interdisciplinary Care Members have pre-existing agreements Members assume accountability All parties are connected Patients/ families receive support through the process Members of care team have easy access to one another Facilitates communication Share expertise; reduces stress and potential for burn out (IOM, 2001)

6 CCHMC: Focus on Chronic Conditions 32% of patients receive care for chronic or chronic/complex conditions Provide: exceptional, safe, affordable care Measure and improve outcomes Each division has a dashboard of safety and quality metrics that are tracked monthly Analyze and publish outcomes Share with other hospitals/systems Contributes to advancement of care (CCHMC Centerlink: Anderson Center Chronic Care Improvement Retrieved from

7 Potential needs of patients with chronic illness Dealing with symptoms Dealing with disability Dealing with emotional impacts Accomplishing complex medical regimens Making difficult lifestyle adjustments Obtaining helpful medical care (Wagner, 2001)

8 CCHMC Patient/ Family Focus Groups "All needs are considered and understood, look at my whole life." (medical, financial, social, and developmental). "Give me everything I need and only what I need "Keep me informed and in the loop "Help me identify my go to person" (CCHMC Centerlink Care coordination model Retrieved from

9 Delivery System Design Goals Effective Prepared Pro-active Assures planned evidence-based care Provides appropriate follow up (CCHMC Centerlink Chronic Care Model: Delivery System Design Retrieved from

10 Components of the Chronic Care Delivery System Design Team Delivered Care Standardize Key Processes of Care Pre-Visit Planning Family Centered Culturally, Linguistically and Literacy Appropriate Care Care Coordination (CCHMC Centerlink Chronic Care Model: Delivery System Design Retrieved from

11 Team Delivered Care for POI Adolescent Medicine/Pediatric Endocrine Catherine Gordon, MD Adult/Pediatric Endocrine Sarah Corathers, MD Gynecology Lesley Breech, MD Social Work Christie Stewart, LISW-S Nursing Debbie Morse, MSN, RN, CPN, CNL

12 Nursing Contact ( go to person) for patients and families Coordinates communication between/ among members of the group Pre-visit planning Identify patient/ family goals Initial intake/ assessment Collect previous medical records/ imaging Coordination with providers Who is going to do what? Schedule appropriate appointments/ testing Coordinate follow up

13 Social Work Pre-visit assessment Full psychosocial assessment during visit Anticipatory guidance Referral to support networks

14 Adolescent Medicine/ Endocrinology Assessment for estrogen deficiency Fatigue, insomnia, hot flashes, mood changes Other medical sequelae APS-1 (components of), hypothyroidism, fractures Approach to treatment Height at presentation Final height vs decreased height velocity Transdermal vs. oral estrogen replacement Unopposed estrogen x many months to optimize height Start at 25µg (or lower if young + extreme short stature) Progestin added if spontaneous bleeding Typically at transdermal dose of 75 µg patch

15 Adol Med/Pediatric Endocrine Approach (con t) Primary vs. secondary amenorrhea? Delayed vs. arrested puberty vs. fully developed? Calcium/vitamin D supplementation Bone density at presentation Family history of osteoporosis Fractures? All placed on MVT IU vitamin D Vitamin D treatment doses if 25OHD low Adolescent interview conducted Identify primary concerns of adolescent/parent Meet with patient/parents together for starters Then each individually Communicate findings with social worker

16 Gynecology Pubertal delay vs. arrest Reproductive potential AMH-clinical utility vs. research parameter Long-term hormonal management Role of the IUD Sexual/ intimacy concerns

17 Standardize Key Processes of Care Standard POI Order Set Basic tests: -FSH -LH -Estradiol -AMH -DHEA-S -Prolactin -T4, free -TSH -Chem-10 panel (with Ca, Mg, Phos) -25-OH vitamin D -PTH -Pregnancy screen (urine or blood) Antibody tests: - Ovarian - Adrenal - 21-hydroxylase -Thyroid -TPO, anti-thyroglobulin Genetic tests: - Karyotype (if not obtained previously) - FMR1 analysis - Galactosemia testing Imaging: -DXA -Whole body -Spine -+/- Hip -Bone age x-ray -Pelvic ultrasound

18 Case Study: AB History of Present Illness 15 yo female Amenorrhea Poor breast development Insomnia

19 Case Study: AB History Allergies NKMA Medications No current meds Past Medical History Headaches Past Surgical History No pertinent past surgical history

20 Case Study: AB Pertinent Family History Mother Hyperthyroidism (diagnosed after first pregnancy) Osteoarthritis Maternal Grandmother Hyperthyroidism Diabetes Osteoarthritis Maternal Aunt Lupus

21 Case Study: AB Physical Examination Ht: 163 cm Wt: 63.8 kg BMI: kg/m2 BP: 102/63 mmhg Breast: Tanner II Pubic hair: Tanner III

22 Case Study: AB Initial Testing by PCP Labs FSH: 68.9 mlu/ml, 71.6 mlu/ml LH: 14.4 mlu/ml Anti-mullerian hormone: <0.003 ng/ml DHEA-S: mcg/dl T4 Free: 1.2 ng/dl, 1.3 ng/dl TSH: 0.54 mclu/ml, 0.94 mclu/ml TSH w/ Free T4 Reflex: mclu/ml Karyotype: 46, XX

23 Case Study: AB Follow up Testing prior to POI Team appointment Labs Estradiol: 4.6 pg/ml Prolactin: 5.4 ng/ml PTH: 38 pg/ml T4: 11.6 mcg/dl TSH: 1.24 mclu/ml Glucose: 85 mg/dl 25-hydroxyvitamin D: 13.9 ng/ml 21-Hydroxylase Autoantibodies: IU/mL Anti-Nuclear Antibodies: negative Thyroglobulin AB: <10.0 IU/mL Thyroid Peridoxidase Antibody: 9.1 IU/mL Fragile X Molecular Analysis: No FMR1 mutation identified

24 Adolescent Med/ Ped Endocrine: AB Estradiol weekly mg/ 24 hr patch Bone Age DXA (Total Body, Lumbar Spine)

25 Gynecology: AB Discussion of parenting options Consultation regarding impaired fertility Sexuality concerns

26 3 month follow up w/ Adol Med: AB Reviewed Bone Age Chronologic Age: 16 years (192 months) Estimated Bone Age: 13 years (156 months)- 4.6 standard deviations below the mean Reviewed DXA Whole body (Z- score of -2.3) Lumbar spine (Z-score of -3.4)

27 AB: Dexa Scan

28 AB: Dexa Scan

29 3 month follow up w/ Adol Med: AB Med Check No menstrual period + breast tenderness, perceived increased breast size No breast or vaginal d/c Perceived decrease in headache frequency Declined GYN follow up at this time Plan Increase dose to 0.05 mg/ 24 hr patch Ergocalciferol 50,000 IU once weekly x 8 weeks Calcium carbonate 500 mg daily x 8 weeks Increase dietary calcium for bone health Follow up in 3 months

30 Case Study: KY HPI 32 yo female Menarche at age 14 with regular periods until age 25 Irregular menses, lack of energy since age 25 surprised by the finality of the diagnosis as presented by her local physician

31 Case Study: KY History Allergies NKMA Medications Lo-Loestrin PMH Allergic rhinitis Headache PSH No pertinent past surgical history

32 Case Study: KY Pertinent Family History Mother Osteoporosis Thyroid cancer Maternal Grandmother Osteoporosis

33 Case Study: KY Physical Examination Ht: 175 cm Wt: 67.8 kg BMI: kg/m2 BP: 127/63 mmhg LMP: January, 2015

34 Case Study: KY Initial Testing Labs FSH: mlu/ml LH: mlu/ml Testosterone: 30.9 ng/dl TSH: 3.11 uu/ml Transvaginal Pelvic Ultrasound Both ovaries wnl No adnexal masses Endometrial stripe: 3.76 mm

35 Adult Endocrinology: KY Recommendations: Screening/ monitoring for bone health Genetic testing for Turner s, Fragile X Screening for autoimmune conditions Increase EE in OCP

36 Gynecology: KY Small risk of spontaneous pregnancy Importance of reliable contraception Combined oral contraceptive pills IUD Consistent use of condoms AMH level Referral for REI ovarian stimulation oocyte retrieval donor eggs

37 Case Study: KY Follow up Testing Labs Estradiol: 2.1 pg/ml Prolactin: 9.2 ng/ml T4: 1.2 ng/dl TSH: mclu/ml Vitamin D: 36.6 ng/ml 21-Hydroxylase Autoantibodies: Thyroglobulin AB: <10.0 IU/mL Thyroid Peridoxidase Antibody: 6.7 IU/mL Anti Mullerian Hormone: <0.003 ng/ml Prolactin: 9.2 ng/ml

38 Case Study: KY Dexa Scan

39 Case Study: KY Medication Adjustments Change to 20mcg pill After 2 weeks, pt c/o nausea, fatigue, inability to concentrate, dizziness, dry skin, heavy eyes Self returned to LoLoestrin, requested change to patch After review of risks and benefits, change to transdermal + daily progestin estradiol patch mg/ 24 hr patch twice weekly + norethindrone 0.35 mg tab daily After 3 months, increased transdermal estradiol patch mg/ 24 hr patch twice weekly + norethindrone 0.35 mg tab daily

40 Follow Up: KY Final increase to mg/24 hr patch twice weekly plus norethindrone 0.35 mg tab daily Transition to local provider for additional management, follow up

41 Case Study: LL HPI 21 yo, sexually active female Diagnosed w/ POI in 2008 at age 13 years Delayed puberty Elevated FSH (100mlU/mL), low LH (14mlU/mL) Amenorrhea Initially started on transdermal estrogen patches, three weeks on/ one week off per month Transitioned to OCPs after initiation of vaginal bleeding

42 Case Study: LL History Allergies: NKDA Medications Microgestin 1.5/30 one tab PO QD PMH 2005 (age 11 y): Euthyroid chronic lymphocytic thyroiditis Enlarged thyroid (0.6 cm cystic area on right isthmus) Normal TSH and T4 w/+ TPO antibodies x2 Negative adrenal/21-hydroxylase antibodies x (age 13 y): absence of pubertal development on routine yearly/ sports physical 2008 (age 14 y): elevated FSH, low estradiol, amenorrhea 2009 (age 15y): started on OCPs and had W/D bleed

43 Case Study: LL History (cont.) PSH No pertinent past surgical history Pertinent Family History Negative for DVT, PE, osteoporosis, early menopause, miscarriages, infertility, hirsutism

44 Case Study: LL Initial 2008 Labs Chromosomes: 46, XX TSH: uu/ml Free T4: 1.09 ng/dl LH: 14 mlu/ml FSH: 100 mlu/ml Estradiol: 1.8 ng/dl DHEA-S: 43 ug/dl Prolactin: 6.3 ng/dl 21 Hydroxylase Autoantibodies: 0.01

45 Case Study: LL Physical exam Ht 172 cm Wt 65.2 kg BP 113/68 LMP 11/23/2016 BMI kg/m2 Breasts Tanner V, normal contour

46 Case Study: LL Testing Immediately Prior to Office visit (on COCs) AMH: <0.015 Estradiol: 7.0 FSH: 4.1

47 Adolescent Med/ Endocrinology: LL Bone density

48 Gynecology: LL Hormone replacement therapy Transdermal vs. Oral Small risk of spontaneous pregnancy OCP IUD Condoms Fertility Donor eggs Sexuality

49 Gynecology: LL HRT w/ transdermal patch + progesterone Estradiol patch MG/24HR patch twice weekly Levonorgestrel IUD - 20 MCG/24 HR

50 Opportunities Research Collaborations Support Groups Referral Sources

51 Patient/Family Experience Assessment Validated Questionnaire Results

52 Next Steps

53 Questions

54 References CCHMC Centerlink: Anderson Center Chronic Care Improvement. Retrieved from CCHMC Centerlink - Care coordination model. Retrieved from CCHMC Centerlink Chronic Care Model: Delivery System Design. Retrieved from Institute of Medicine (IOM). (2001). Crossing the quality chasm : a new health system for the 21st century. Washington, D.C. : The National Academies Press. Wagner, E. H., Austin, B. T., Davis, C., Hindmarsh, M., Schaefer, J., & Bonomi, A. (2001). Improving chronic illness care: translating evidence into action. Health Affairs (Project Hope), 20(6),