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WAINUA is indicated for the treatment of the polyneuropathy of hereditary transthyretin-mediated amyloidosis in adults.

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HOW TO DIAGNOSE

Understand the key symptoms that raise clinical suspicion of polyneuropathy1

hATTR-PN is a rapidly progressive and fatal disease that requires urgent identification2,3

Identify patients with undiagnosed hATTR-PN by recognizing the signs and symptoms

 

NEUROLOGIC EVALUATION IN AMYLOIDOSIS1

 

Autonomic dysfunction

  • Orthostatic hypotension
  • Diarrhea/constipation
  • Urinary retention
  • Erectile dysfunction
 

Sensory symptoms

  • Numbness/pain in feet
  • Inability to sense pain/temperature
  • Impaired balance/falls
 

Motor loss

  • Tripping, foot drop
  • Difficulty with stairs
  • Usually after sensory symptoms
 

Physical examination

  • Muscle weakness
  • Distal sensory loss
  • Reduced or absent reflexes
  • Wide-based unsteady gait

Patients experienced symptom onset between 2.5 to 10 years prior to diagnosis9-11

The scales below can be helpful tools for assessing and documenting the severity of polyneuropathy impairment in your patients with hATTR-PN2

Score Polyneuropathy disability
(PND) description
I Sensory disturbances in extremities; preserved walking capability
II Difficulty walking but no need for a stick or crutches
IIIa Requires 1 stick or crutch for walking
IIIb Requires 2 sticks or crutches for walking
IV Confined to a wheelchair or bed
 
Stage Familial amyloid polyneuropathy (FAP) description
I Neuropathy limited to the lower limbs; walking without help
II Progression of neuropathy in lower limbs; needs assistance when walking; muscles of the hands becoming weak
III Confined to a wheelchair or bed; generalized weakness and areflexia

Patients experienced symptom onset between 2.5 to 10 years prior to diagnosis9-11

Diagnostic assessment tool for identifying patients with hATTR-PN

WAINUA Patients with Polyneuropathy Due to hATTRWAINUA Patients with Polyneuropathy Due to hATTRWAINUA Patients with Polyneuropathy Due to hATTR

Algorithms are for illustrative purposes only.

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Expert consensus and clinical guidelines:

Recommend genetic testing for all patients with ATTR1-8

WAINUA is not indicated for the treatment of cardiomyopathy symptoms.

  • *Small-fiber neuropathy is not detected by conventional NCSs; therefore, a skin biopsy may be performed.1
  • †Monoclonal protein screen should be performed to rule out AL-CM with sFLC, SIFE, and UIFE.1
  • ‡Consider biopsy if cardiac scintigraphy is negative or equivocal and clinical suspicion is high.1
  • §Including peripheral neuropathy (numbness, paresthesia, imbalance) or autonomic dysfunction (orthostatic hypotension, GI symptoms).1

Patients experienced symptom onset between 2.5 to 10 years prior to diagnosis9-11

Recognize the pattern of hATTR over time

See an illustrative case that may reflect your patient's experience

Suspicion of hATTR with mixed phenotype
 Further assessment and testing needed1-5

WAINUA is not indicated for the treatment of cardiomyopathy symptoms

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Consider genetic testing to rule out hATTR-PN

  • *Presence and severity of specific symptoms may depend on the duration and control of diabetes.13
  • †CIDP may present with various phenotypes. This chart describes characteristics of atypical CIDP.19,21
  • ‡Symptoms of motor involvement become more prominent as hATTR-PN progresses.12
  • §GI manifestations, such as chronic diarrhea and constipation, are often considered autonomic symptoms.3,12
  • ||Over 50% of patients develop CTS before diagnosis.15
  • Polyneuropathy due to ATTR can be 10 times more rapid than diabetic neuropathy.29

See how WAINUA targets TTR at
the source

How WAINUA works

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Learn how WAINUA can affect
serum TTR levels

View TTR suppression

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FOOTNOTES

Afib, atrial fibrillation; AL-CM, light-chain cardiac amyloidosis; ATTR, transthyretin-mediated amyloidosis; ATTR-CM, cardiomyopathy of transthyretin-mediated amyloidosis; ATTRwt, wild-type transthyretin-mediated amyloidosis; CIDP, chronic inflammatory demyelinating polyneuropathy; CTS, carpal tunnel syndrome; EMG, electromyography; GI, gastrointestinal; hATTR, hereditary transthyretin-mediated amyloidosis; hATTR-PN, polyneuropathy of hereditary transthyretin-mediated amyloidosis; HFpEF, heart failure with preserved ejection fraction; NCS, nerve conduction study; PCP, primary care provider; PN, polyneuropathy; sFLC, serum free light chain; SIFE, serum immunofixation electrophoresis; SNAP, sensory nerve action potential; TTR, transthyretin; UIFE, urine immunofixation electrophoresis.

REFERENCES

  1. 1. Kittleson MM, Ruberg FL, Ambardekar AV, et al; Writing Committee. 2023 ACC Expert Consensus Decision Pathway on comprehensive multidisciplinary care for the patient with cardiac amyloidosis: a report of the American College of Cardiology Solution Set Oversight Committee. J Am Coll Cardiol. 2023;81(11):1076-1126.
  2. 2. Adams D, Ando Y, Beirão JM, et al. Expert consensus recommendations to improve diagnosis of ATTR amyloidosis with polyneuropathy. J Neurol. 2021;268(6):2109-2122.
  3. 3. Gertz M, Adams D, Ando Y, et al. Avoiding misdiagnosis: expert consensus recommendations for the suspicion and diagnosis of transthyretin amyloidosis for the general practitioner. BMC Fam Pract. 2020;21(1):198.
  4. 4. Alcantara M, Mezei MM, Baker SK, et al. Canadian guidelines for hereditary transthyretin amyloidosis polyneuropathy management. Can J Neurol Sci. 2022;49(1):7-18.
  5. 5. Heidenreich PA, Bozkurt B, Aguilar D, et al. 2022 AHA/ACC/HFSA Guideline for the Management of Heart Failure: A report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines. Circulation. 2022;145(18):e895-e1032.
  6. 6. Fine NM, Davis MK, Anderson K, et al. Canadian Cardiovascular Society/Canadian Heart Failure Society joint position statement on the evaluation and management of patients with cardiac amyloidosis. Can J Cardiol. 2020;36(3):322-334.
  7. 7. Brito D, Albrecht FC, de Arenaza DP, et al. World Heart Federation consensus on transthyretin amyloidosis cardiomyopathy (ATTR-CM). Glob Heart. 2023;18(1):59.
  8. 8. Arbelo E, Protonotarios A, Gimeno JR, et al; ESC Scientific Document Group. 2023 ESC Guidelines for the management of cardiomyopathies. Eur Heart J. 2023;44(37):3503-3626.
  9. 9. Aus dem Siepen F, Hein S, Prestel S, et al. Carpal tunnel syndrome and spinal canal stenosis: harbingers of transthyretin amyloid cardiomyopathy? Clin Res Cardiol. 2019;108(12):1324-1330.
  10. 10. Cappelli F, Zampieri M, Fumagalli C, et al. Tenosynovial complications identify TTR cardiac amyloidosis among patients with hypertrophic cardiomyopathy phenotype. J Intern Med. 2021;289(6):831-839.
  11. 11. Barroso FA, Coelho T, Dispenzieri A, et al; THAOS Investigators. Characteristics of patients with autonomic dysfunction in the Transthyretin Amyloidosis Outcomes Survey (THAOS). Amyloid. 2022;29(3):175-183.
  12. 12. Nativi-Nicolau JN, Karam C, Khella S, Maurer MS. Screening for ATTR amyloidosis in the clinic: overlapping disorders, misdiagnosis, and multiorgan awareness. Heart Fail Rev. 2022;27(3):785-793.
  13. 13. Feldman EL, Callaghan BC, Pop-Busui R, et al. Diabetic neuropathy. Nat Rev Dis Primers. 2019;5(1):42.
  14. 14. Waddington-Cruz M, Wixner J, Amass L, Kiszko J, Chapman D, Ando Y; THAOS Investigators. Characteristics of patients with late- vs. early-onset Val30Met transthyretin amyloidosis from the Transthyretin Amyloidosis Outcomes Survey (THAOS). Neurol Ther. 2021;10(2):753-766.
  15. 15. Karam C, Dimitrova D, Christ M, Heitner SB. Carpal tunnel syndrome and associated symptoms as first manifestation of hATTR amyloidosis. Neurol Clin Pract. 2019;9(4):309-313.
  16. 16. Poli L, Labella B, Cotti Piccinelli S, et al. Hereditary transthyretin amyloidosis: a comprehensive review with a focus on peripheral neuropathy. Front Neurol. 2023;14:1242815.
  17. 17. Lin X, Yarlas A, Vera-Llonch M, et al. Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis. BMC Neurol. 2021;21(1):70.
  18. 18. Adams D, Coelho T, Obici L, et al. Rapid progression of familial amyloidotic polyneuropathy: a multinational natural history study. Neurology. 2015;85(8):675-682.
  19. 19. Lewis RA, van Doorn PA, Sommer C. Tips in navigating the diagnostic complexities of chronic inflammatory demyelinating polyradiculoneuropathy. J Neurol Sci. 2022;443:120478.
  20. 20. Zis P, Sarrigiannis PG, Rao DG, Hewamadduma C, Hadjivassiliou M. Chronic idiopathic axonal polyneuropathy: a systematic review. J Neurol. 2016;263(10):1903-1910.
  21. 21. Fisse AL, Motte J, Grüter T, Sgodzai M, Pitarokoili K, Gold R. Comprehensive approaches for diagnosis, monitoring and treatment of chronic inflammatory demyelinating polyneuropathy. Neurol Res Pract. 2020;2:42.
  22. 22. Vrancken AFJE, Notermans NC, Wokke JHJ, Franssen H. The realistic yield of lower leg SNAP amplitudes and SRAR in the routine evaluation of chronic axonal polyneuropathies. J Neurol. 2008;255(8):1127-1135.
  23. 23. Lehmann HC, Wunderlich G, Fink GR, Sommer C. Diagnosis of peripheral neuropathy. Neurol Res Pract. 2020;2:20.
  24. 24. Natesan V, Kim SJ. Diabetic nephropathy - a review of risk factors, progression, mechanism, and dietary management. Biomol Ther (Seoul). 2021;29(4):365-372.
  25. 25. Bansal V, Kalita J, Misra UK. Diabetic neuropathy. Postgrad Med J. 2006;82(964):95-100.
  26. 26. Van den Bergh PYK, van Doorn PA, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force-second revision. J Peripher Nerv Syst. 2021;26(3):242-268.
  27. 27. Visser NA, Vrancken AFJE, van der Schouw YT, van den Berg LH, Notermans NC. Chronic idiopathic axonal polyneuropathy is associated with the metabolic syndrome. Diabetes Care. 2013;36(4):817-822.
  28. 28. Visser NA, Notermans NC, Degen LAR, de Kruijk JR, van den Berg LH, Vrancken AFJE. Chronic idiopathic axonal polyneuropathy and vitamin B6: a controlled population-based study. J Peripher Nerv Syst. 2014;19(2):136-144.
  29. 29. Berk JL, Suhr OB, Obici L, et al. Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial. JAMA. 2013;310(24):2658-2667.

IMPORTANT SAFETY INFORMATION

WARNINGS AND PRECAUTIONS

  • Reduced Serum Vitamin A Levels and Recommended Supplementation WAINUA leads to a decrease in serum vitamin A levels. Supplement with recommended daily allowance of vitamin A. Refer patient to an ophthalmologist if ocular symptoms suggestive of vitamin A deficiency occur.

ADVERSE REACTIONS

Most common adverse reactions (≥9% in WAINUA-treated patients) were vitamin A decreased (15%) and vomiting (9%).

INDICATION

WAINUA injection, for subcutaneous use, 45 mg is indicated for the treatment of the polyneuropathy of hereditary transthyretin-mediated amyloidosis in adults.

 

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