Notes to the Financial Statement

NOTE 1 – CORPORATE INFORMATION

Orphazyme A/S (the “Company”) is a late-stage biopharmaceutical company.  The Company is a limited liability company publicly traded on Nasdaq Copenhagen with headquarters in Copenhagen, Denmark. The company’s American Depositary Shares are listed on the Nasdaq Global Select Market in the U.S. The group consists of the headquarters in Copenhagen, Orphazyme A/S; a wholly-owned subsidiary in the U.S., Orphazyme U.S., Inc.; and a wholly-owned subsidiary in Switzerland, Orphazyme Schweiz GmbH (together “Orphazyme” or “the Group”).  

Following receipt of a Complete Response Letter from the FDA for arimoclomol for NPC, a restructuring program reducing our global workforce by approximately two thirds was initiated and finally concluded by July 31, 2021. Employees in the Group’s subsidiaries and in headquarters were affected. Following our restructuring, we have strategically maintained medical and commercial personnel to strengthen our footprint in areas with the most significant market potential for NPC.  We have maintained a core team in the U.S. to effectively act on any actions agreed with the FDA; and we have maintained a core team in Europe and the UK to continue to support our early access programs and establish our commercial presence in those respective countries.

NOTE 2 – BASIS OF PREPARATION AND UPDATES TO THE GROUP’S ACCOUNTING POLICIES

Basis of preparation

The interim condensed consolidated financial statements for the six months ended June 30, 2021 have been prepared in accordance with IAS 34 Interim Financial Reporting as issued by the International Accounting Standards Board (IASB) and as adopted by the EU, and additional Danish disclosure requirements for interim reports of companies listed on the Nasdaq Copenhagen.

The interim condensed consolidated financial statements do not include all the information and disclosures required in annual financial statements and should be read in conjunction with Orphazyme A/S’ latest consolidated annual financial statements as of December 31, 2020. These interim condensed consolidated financial statements have been prepared in accordance with the going concern assumption (Note 3).

COVID-19

At this time, there is no material impact directly related to COVID-19 on the Group’s consolidated financial statements, including the judgements and estimates applied. Specifically, Orphazyme is not currently conducting any clinical trials and in the process of closing out the existing trial extensions following the disappointing results of the Phase 2/3 clinical trial related to IBM and the Phase 3 clinical trial related to ALS. However, as Orphazyme is assessing the potential path forward with the FDA following receiving a complete response letter from the FDA on arimoclomol for the treatment of NPC and is actively pursuing the European regulatory approval, other parts of the business and operations may be adversely impacted by the effects of COVID-19, for example: our third-party manufacturers and other third parties; the productivity of our staff; ability to attract, integrate, manage and retain qualified personnel or key employees; our global supply chains and relationships with vendors and other parties; significant disruption of global financial markets; and reduced ability to secure additional funding. We will continue to monitor the COVID-19 pandemic and its potential impact on our business and financials.

Exhibit 99.1

Updates to the Group's accounting policies

The accounting policies used in the preparation of the interim condensed consolidated financial statements are consistent with those used in the preparation of Orphazyme’s annual consolidated financial statements for the year ended December 31, 2020, with any additions included in the respective notes below.  No new or amended standards became applicable for the current reporting period; and consequently, there is no resulting impact on the interim condensed consolidated financial statements of the Group. 

Significant accounting estimates and judgements

In addition to the significant accounting estimates and judgements disclosed in note 1.4 of the consolidated financial statements for the year ended December 31, 2020, Management has prepared these interim condensed consolidated financial statements under the going concern assumption (Note 3); and the company has estimated the revenue recognized using the ‘expected value’ method (see Note 4).

NOTE 3 - GOING CONCERN

On June 18, 2021, Orphazyme announced that it received a Complete Response Letter (CRL) from the U.S. Food and Drug Administration (FDA) following the FDA’s review of the new drug application for arimoclomol, a heat shock protein amplifier intended for the treatment of Niemann-Pick disease type C (NPC).  As a result of arimoclomol for NPC not being approved by the FDA, Orphazyme cannot start the commercialization of its drug product in the U.S., which would have contributed to revenue and to reduced operating loss. Furthermore, Orphazyme was not able to obtain a priority review voucher in 2021.

Receiving the CRL from the FDA and the resulting consequences for our cash position introduces a going concern risk and consequently, Management has made an assessment of Orphazyme’s ability to continue as a going concern for the twelve-month period following the balance sheet date of June 30, 2021.  In connection with this assessment, several uncertainties have been identified and, as a result, mitigating actions have been initiated.

The following significant uncertainties were identified as of June 30, 2021:

Exhibit 99.1

In response to these uncertainties, the following mitigating actions have been taken:

With the uncertainties listed above, there can be no assurance that Orphazyme will achieve or sustain profitability or positive cash flows from operations. If the Group is unable to attract additional funding, future operations will need to be significantly scaled back or discontinued. These conditions raise substantial doubt about the Group’s ability to continue as a going concern. These interim condensed consolidated financial statements do not include any adjustments that might result from the outcome of this uncertainty.  Based on the mitigating actions described above, Management is of the view that a going concern assumption is appropriate as of June 30, 2021.

NOTE 4 – NET REVENUE

Orphazyme recognizes revenue in accordance with IFRS 15 Revenue from Contracts with Customers and, as a result, follows the five-step model when recognizing revenue: 1) identifying a contract; 2) identifying the performance obligations; 3) determining the transaction price; 4) allocating the price to the performance obligations; and 5) recognizing revenue when the performance obligations have been fulfilled.

Net revenue comprises revenue from the sale of arimoclomol for the treatment of NPC under the remunerated early access compassionate use program (“nATU”) in France. An early access compassionate use program is a program giving specific patients access to a drug, which is not yet on the market. Only drugs targeting serious or rare indications and for which there is currently no appropriate treatment are considered for early access compassionate use programs.  Further, to be considered for the early access compassionate use program, the drug must have proven efficacy and safety and must either be undergoing price negotiations or seeking marketing approval. Orphazyme is currently pursuing regulatory approval from the European Medicines Agency (EMA).

Revenue is recognized when the drug products are sold to the customer, i.e., at the time when control over the drug product is transferred to the third-party customer.

Exhibit 99.1

Under the French nATU, the manufacturer can set its own price for the drug products until a price agreement with the authorities is in place. Any excess in the price charged by the manufacturer compared to the price agreed with the health authorities once the drug product is approved in France must be repaid. Orphazyme is therefore recognizing net revenue in accordance with IFRS 15 Revenue from Contracts with Customers using the ‘expected value’-method, which is based on probability-weighted consideration of different agreed-upon sales prices. The estimated net revenue is recognized less VAT and other sales related taxes. A long-term liability is recognized for the amount estimated to be repaid.

For the six-month period ended June 30, 2021, Orphazyme recognized net revenue of DKK 13.2 million (DKK 0 for the six-month period ended June 30, 2020), which is net of certain estimated repayment amounts that may become due following potential approval of our drug product from the French authorities.  The estimated amount to be repaid is accrued as a long-term liability under the line item Other non-current liabilities.

Trade receivables in the amount of DKK 26.3 million are recognized in the balance sheet at the total invoiced amount less any expected credit losses. Due to the nature of the revenue transactions, expected credit losses are very limited.

Cost of goods sold relating to products sold under this French nATU is recognized as research and development expenses. As the drug products sold have not yet received approval for commercial sale, any related inventory is impaired and written off (Note 5).

NOTE 5 – RESEARCH AND DEVELOPMENT EXPENSES

Research and development expenses of DKK 264.7 million (June 30, 2020: DKK 167.0 million) include employee costs for research and development personnel (i.e. salaries, bonuses, employer contributions to pension schemes, share-based compensation); external costs for further development of our pipeline, including ongoing clinical trials and clinical pharmacology registration trials; and pre-launch inventory costs. Further, research and development expenses include costs of goods sold relating to products sold under the French early access compassionate use program (Note 4). For the six-months ended June 30, 2021, an impairment charge was recognized for intangible assets and right-of-use assets (Note 8).

Included in total research and development expenses is an amount of DKK 37.7 million attributable to restructuring activities, i.e. clinical trial close-out costs (DKK 36.3 million) and employee redundancies (DKK 1.4 million).

NOTE 6 – GENERAL AND ADMINISTRATIVE EXPENSES

General and administrative expenses of DKK 214.2 million (June 30, 2020:  DKK 78.6 million) include pre-launch costs of DKK 123.8 million (June 30, 2020: DKK 44.1 million) associated with establishing a commercial organization and the escalation of launch preparation activities, including hiring a commercial team in our subsidiaries in the U.S. and Switzerland and medical affairs activities to further engage with the scientific community through communication and education programs.  Furthermore, included in general and administrative expenses is DKK 61.8 million (June 30, 2020: DKK 34.5 million) relating to salaries for administrative employees and Executive Management, remuneration to the Board of Directors, share-based compensation costs, audit fees, legal costs and investor relations costs; as well as an impairment charge related to software used in pre-launch and administrative activities (Note 8). Included in these amounts is DKK 12.1 million attributable to restructuring activities, i.e. employee redundancies (DKK 11.4 million) and termination of onerous contracts (DKK 0.7 million).

Exhibit 99.1

NOTE 7 – SHARE-BASED COMPENSATION COSTS

Please refer to Note 2.5 of the Group’s consolidated financial statements included in the 2020 Annual Report for a description of the share-based compensation programs and the accounting policies and estimates applied. The activities in the respective programs are outlined below.  The respective share-based compensation programs allow the vesting of awards through the employment release date.  As such, there are no forfeitures to be disclosed or expense to be reversed due to the termination of employees as a result of our restructuring.

2021 Long-term incentive program (“2021 LTIP”):  

Similar to the 2017 LTIP, the 2019 LTIP and the 2020 LTIP described in Note 2.5 of the consolidated financial statements for the year ended December 31, 2020, the 2021 LTIP offers all permanent employees of Orphazyme a certain number of Restricted Share Units (RSUs) contingent on continued employment and Performance Share Units (PSUs) depending on the development of the Company’s share price.  Although the grant date of these awards was in April 2021, management has determined that the service commencement date of the participants was the earlier of January 1, 2021, or the employee’s employment date, and consequently the compensation expense is recognized from the service commencement date.  

The fair value of the 2021 LTIP awards was estimated separately for the RSUs and PSUs using a Black-Scholes model and a Monte-Carlo simulation, respectively, at the grant date of April 21, 2021. The following inputs were used in the valuation models:

The risk-free interest rate has been estimated based on Danish government bonds with similar maturities.  Expected volatility has been determined based on Orphazyme’s own historic volatility.  Based on the fair value of the 2021 LTIP, an expense of DKK 6.0 million was recognized for the six-month period ending June 30, 2021.  

In addition, for the six-month period ending June 30, 2021, an aggregate expense of DKK 5.1 million was recognized for the 2020 LTIP, 2019 LTIP and 2017 LTIP for the six-month period ending June 30, 2021 compared to DKK 10.1 million recognized for the six-month period ended June 30, 2020.  

In January 2021, the Matching Shares from the 2020 LTIP fully vested and were issued to the participants in exchange for the nominal value of DKK 1 per share. This resulted in cash received of DKK 170,131 and a capital increase of the same number of shares.

Exhibit 99.1

As described in Note 2.5 of the consolidated financial statements for the year ended December 31, 2020, the Phantom programs are cash-settled and their fair value is re-assessed at each reporting date.  Management used a Monte-Carlo simulation model with the following inputs to estimate the fair value as of June 30, 2021:

The risk-free interest rate has been estimated based on Danish government bonds with similar maturities.  Expected volatility has been determined based on Orphazyme’s own historic volatility.  Based on the fair value of the awards on June 30, 2021, a credit of DKK 10 thousand was recognized for the six-month period ended June 30, 2021 compared to an expense of DKK 0.2 million recognized for the six-month period ended June 30, 2020.

In March 2020, the 2019 RSUs granted to the Board of Directors in 2019 fully vested. As of June 30, 2021, all but one board member exercised the fully vested RSUs.

Also in March 2020, the 2020 RSU program was announced, granting the Board of Directors an aggregate of 15,177 RSUs under similar terms and conditions as the 2019 RSUs described in Note 2.5 of the consolidated financial statements for the year ended December 31, 2020. In September 2020, a new RSU incentive program was announced (2020-2 RSU program), which comprised 22,993 RSUs in total, including an on-boarding grant to a new board member in accordance with the Company’s remuneration policy. The 2020-2 RSU program runs in parallel with the 2020 RSU program and board members can only exercise RSUs under one of the programs.  As of June 30, 2021, these RSUs were fully vested but unexercised.  

In May 2021, an aggregate of 30,450 RSUs were granted to the Board of Directors under the 2021 RSU program under similar terms and conditions as the 2019 RSUs described in Note 2.5 of the consolidated financial statements for the year ended December 31, 2020. The 2021 RSUs fully vest in March 2022.

The risk-free interest rate has been estimated based on Danish government bonds with similar maturities. Expected volatility has been determined based on Orphazyme’s own historic volatility. Based on the fair value of the 2021 RSUs on June 30, 2021, an expense of DKK 0.3 million was recognized for the six-month period ended June 30, 2021. In addition, an amount of DKK 0.3 million was recognized for the 2020 RSUs for the six-month period ended June 30, 2021 compared to DKK 0.1 million recognized for the six-month period ended June 30, 2020.

As part of the license agreement with KLSDC and UCL described in Note 3.1 of the consolidated financial statements for the year ended December 31, 2020, consideration to KLSDC and UCL is payable in shares of the Company (“Bonus Shares”) each January and is based on incurred costs reported by KLSDC and UCL for the previous year. Subsequently, it was agreed, that as an alternative to the issuance of shares, a cash equivalent of the value of the Bonus Shares may be paid instead. As at December 31, 2020 the aggregate costs incurred by KLSDC and UCL amounted to USD 0.5 million (DKK 3.2 million). A total of 22,553 Bonus Shares (“2021 Bonus Shares”) were issued to KLSDC based on the average 30-day closing price of Orphazyme’s shares and an amount of USD 0.3 million (DKK 1.6 million) was paid to UCL. At the time of the share issuance and the cash transfer, the equity reserve was decreased by DKK 3.3 million, which represents the market value of the shares issued and the cash paid.   

NOTE 8 - IMPAIRMENT OF ASSETS

As disclosed in Note 3.1 of the consolidated financial statements for the year ended December 31, 2020, Orphazyme entered into a license agreement with KU Center for Technology Commercialization Inc., University of Kansas, Kansas Life Sciences Development Company, Inc. (KLSDC) and UCL Business PLC (UCL) in 2017, granting Orphazyme the right to develop and commercialize products under all data generated in the course of the Phase 2/3 clinical trial on arimoclomol for the treatment of IBM worldwide. In March 2021, it was announced, that the phase 2/3 trial evaluating arimoclomol for the treatment of inclusion body myositis (IBM), a progressively debilitating muscle-wasting disease, did not meet its primary and secondary endpoints. As a result, Orphazyme has recognized an impairment loss of DKK 7.5 million corresponding to the remaining carrying amount of the license agreement. The impairment loss was recognized under research and development expenses.

Further, as also disclosed in Note 3.1. of the consolidated financial statements for the year ended December 31, 2020, Orphazyme entered into an exclusive license agreement with the University of Miami in 2019, granting Orphazyme a global royalty-bearing, exclusive license to all data, know-how, inventions and technology generated by the University of Miami and certain other institutions in a Phase 2 clinical trial of arimoclomol in ALS with the A4V SOD1 mutation to research, develop, make, use or sell certain

Exhibit 99.1

pharmaceutical products or processes containing arimoclomol. In May 2021, it was announced, the ORARIALS-01 pivotal trial of arimoclomol in amyotrophic lateral sclerosis (ALS) did not meet its primary and secondary endpoints to show benefit in people living with ALS. As a result, Orphazyme has recognized an impairment loss of DKK 0.5 million corresponding to the remaining carrying amount of the license agreement. The impairment loss was recognized under research and development activities.

In addition, Orphazyme has impaired leased laboratory equipment classified as a right-of-use asset in the amount of DKK 3.2 million.  This amount is recognized under research and development activities.  

Lastly, Orphazyme has impaired software and other assets recognized within intangible assets at a total amount of DKK 2.6 million.  The impairment loss was recognized under general and administrative expenses, as the assets were used in pre-launch or administrative activities.  

NOTE 9 – PROVISIONS FOR RESTRUCTURING COSTS

Provisions for restructuring are recognized when the plan for the restructuring has been prepared, and the employees affected have a valid expectation that the restructuring program will be fulfilled. Provisions for onerous contracts are recognized to the extent that obligations under the contract exceed the economic benefit from the contract.

In June 2021, Orphazyme announced a restructuring program, including a reduction of the global workforce by approximately two thirds and terminating contracts and agreements not deemed necessary in the continued pursuit of regulatory approval in Europe and in the U.S. A total amount of DKK 49.8 million has been recognized as restructuring costs, of which DKK 12.9 million relates to the reduction of the global workforce and DKK 36.9 million relates to termination of contracts and agreements.  As a result of the phase 2/3 trial evaluating arimoclomol for the treatment of inclusion body myositis (IBM) and the ORARIALS-01 pivotal trial of arimoclomol in amyotrophic lateral sclerosis (ALS) not meeting the primary and secondary endpoints, several contracts with CROs and other vendors have been terminated.

The restructuring costs were expensed under research and development expenses or general and administrative expenses, depending on their nature (see Notes 5 and 6).

NOTE 10 – FINANCIAL LIABILITIES

As disclosed in Note 3.6 of the consolidated financial statements for the year ended December 31, 2020, the structured debt facility with Kreos (“Loan Agreement”) entered into in August 2019 includes a Facilitation Fee that is due and payable by Orphazyme at the sole discretion of the lender. The Facilitation Fee is an amount equal to the greater of (i) 10% of the aggregate amount of the amount borrowed and (ii) the percentage increase in the Company’s share price between the 30-day volume-weighted average share price on the date of the Loan Agreement and the closing share price on the day immediately preceding the date of the payment request notification by the lender applied to the aggregate amount of amounts borrowed. The variability arising from the change in Orphazyme’s share price is not closely related to the host debt instrument characterized mainly by interest rate and credit risk. Therefore, the embedded equity-linked amount is separated from the host debt instrument and accounted for as an embedded written call option at fair value through profit and loss. The call option is measured at fair value at level 2 in the fair value hierarchy using a Black-Scholes option valuation model.  In measuring the fair value, various observable and unobservable inputs are required. Observable input mainly relates to the market price of Orphazyme’s shares, and risk-free interest rate. Unobservable inputs mainly relate to the expected volatility of Orphazyme’s share price and the term of the option.

The change in fair value of the call option is recognized as a finance expense in the statement of profit or loss. Based on the fair value of the call option on June 30, 2021, an expense of DKK 0.6 million (June 30, 2020: DKK 0.7 million) was recognized for the period.

As disclosed in Note 4.8 of the consolidated financial statements for the year ended December 31, 2020, in February 2021, the Company issued 170,131 Matching Shares to participants in the 2020 LTIP. In February 2021, 58,000 ordinary shares were issued to the former CEO, Kim Stratton, and also in February 2021, 22,553 Bonus Shares were issued as part of consideration payable to KLSDC relating to the license agreement (see Note 7d above).

As discussed in Note 7 above, in March 2021, certain members of the Board of Directors exercised restricted share units and the Company issued 3,854 shares in exchange for DKK 0.2 million.

Exhibit 99.1

Following the above activity, the total nominal share capital of the Company as of June 30, 2021, was DKK 34,952,241, representing 34,952,241 ordinary shares each with a nominal value of DKK 1.

In January 2020 the Company issued 20,650 shares as part of consideration payable to KLSDC and UCL relating to the license agreement.

On February 7, 2020, Orphazyme completed an offering of 7,032,937 shares in a directed issue and private placement and raised gross proceeds of approximately DKK 745 million and net proceeds of approximately DKK 694 million. The net proceeds of the directed issue and private placement is expected to support the U.S. and European filings for approval of arimoclomol for the treatment of Niemann-Pick disease Type C (NPC), as well as preparations for commercial launch.

The transaction consisted of a directed issue and private placement of up to 3,961,264 new shares of a nominal value of DKK 1 each (the “New Shares”) and a private placement of up to 3,071,673 existing shares of a nominal value of DKK 1 each (the “Existing Shares” and together with the New Shares, the “Offer Shares”) at an offer price of DKK 106 per Offer Share, as determined by the Board of Directors of the Company through a book-building process (the “Offering”). The New Shares were issued without pre-emption rights for existing shareholders.

The offering of Existing Shares was facilitated by a share loan to the Company from related parties Novo Holdings A/S and Orpha Pooling B.V. (the “Lending Shareholders”) pursuant to a stock lending and subscription agreement with an obligation for the Company to redeliver new shares of an equivalent number as the Existing Shares borrowed by the Company from each of the Lending Shareholders (the “Replacement Shares”), which were issued without pre-emption rights for existing shareholders. The Lending Shareholders did not participate in the Offering and were only facilitating the loan of the Lending Shares for purposes of the Company’s offering of Existing Shares in the Offering.

In April 2020 certain members of the Board of Directors exercised their 2019 RSUs and the Company issued 6,543 shares in exchange for DKK 0.4 million.

Following the above activity, the total nominal share capital of the Company as of June 30, 2020 was DKK 27,044,929, representing 27,044,929 ordinary shares each with a nominal value of DKK 1.

*Recalculated retrospectively as a result of Bonus Shares issued subsequently to June 30, 2020.

Basic loss per share is calculated by dividing the net loss attributable to ordinary shareholders for the period by the weighted-average number of ordinary shares outstanding during each period. Diluted loss per share is calculated by dividing the net loss by the weighted-average number of ordinary shares outstanding during the period increased by the dilutive effect of the assumed issuance of any outstanding share-based awards. Due to the fact that Orphazyme has incurred losses for each period presented, the potential shares issuable related to outstanding equity awards have been excluded from the calculation of diluted loss per share as the effect of such shares is anti-dilutive.  Therefore, basic and diluted loss per share are the same for each period presented.  

As disclosed in Note 4.3 of the consolidated financial statements for the year ended December 31, 2020, in February 2021, Bonus Shares were issued to KLSDC under the terms of the license agreement entered into in October 2017. Basic and diluted loss per share for the comparative period presented has been adjusted retrospectively to include these Bonus Shares in the number of weighted average shares outstanding for the six-months ended June 30, 2020.

NOTE 13 - COMMITMENTS AND CONTINGENCIES

There have been the following material changes in commitments and contingencies compared to year-end 2020.

Contractual obligations towards Contract Research Organizations (CROs)

As a result of the phase 2/3 trial evaluating arimoclomol for the treatment of inclusion body myositis (IBM) and the ORARIALS-01 pivotal trial of arimoclomol in amyotrophic lateral sclerosis (ALS) not meeting the primary and secondary endpoints, several contracts with CROs and other vendors have been terminated. A provision in the amount of DKK 36.3 million has been recognized to cover the onerous part of these contracts.

Contingency related to early access compassionate use program in France

Net revenue comprises revenue from the sale of arimoclomol for the treatment of NPC under the early access compassionate use program (“nATU”) in France. Under the French nATU, the manufacturer can set its own price for the drug products until a price agreement with the authorities is in place. Any excess in the price charged by the manufacturer compared to the price agreed with the health authorities once the drug product is approved in France must be repaid. Orphazyme is recognizing net revenue in accordance with IFRS 15 Revenue from Contracts with Customers using the ‘expected value’ method. The ‘expected value’ method is based on probability-weighted consideration of different agreed-upon sales prices. A long-term liability is recognized for the amount estimated to be repaid. However, Orphazyme has a contingent liability if the final price agreed with the health authorities is less than the price estimated by Orphazyme, which has been recognized as revenue.

Exhibit 99.1

Putative class action lawsuit

On July 9, 2021, a putative class action lawsuit was filed against the company and certain of its current and former directors and officers in the United States District Court for the Northern District of Illinois. This lawsuit alleges that certain representations about arimoclomol in the Company’s U.S. IPO offering documents and in subsequent public statements were false and misleading, in violations of U.S. securities. Orphazyme does not believe these claims have merit and intends to vigorously defend itself against the lawsuit.

NOTE 14 – RELATED PARTIES

Orphazyme’s related parties are Orphazyme A/S’ subsidiaries, Orphazyme U.S. Inc. and Orphazyme Schweiz GmbH, Board of Directors, Executive Management, and close members of the family of these persons.  Effective April 1, 2021, Christophe Bourdon was appointed Chief Executive Officer and a member of the Executive Management.

During the six-month period ended June 30, 2021, remuneration to Executive Management and the Board of Directors was made and share-based compensation awards were granted to Executive Management and the Board of Directors in line with the Company’s remuneration policy.  There were no material related party transactions during the first half of 2021.

NOTE 15 – SUBSEQUENT EVENTS

The most recent French Social Security law of December 14, 2020 (Article 78- La Loi de financement de la sécurité sociale, FSSL) for the healthcare plan of 2021, implemented changes to the existing ATU (remunerated early access program) system. The ATU program has now been grouped into two subgroups: An early access authorization program (Accès précoce aux médicaments, EAP) which includes the cohort ATU and a compassionate access program (Accès compassionnel aux médicaments, CAP) which includes nominative ATU, among others. The reforms will come into force no later than July 1, 2021 and ATUs with an expiry date after this date will not be eligible for renewal and will have to reapply.

Exhibit 99.1

Statement by the Board of Directors and the Executive Management

The Board of Directors and the Executive Management have today reviewed and approved the interim financial report of Orphazyme A/S for the period January 1 - June 30, 2021. The interim financial report has not been reviewed or audited by the Company’s independent auditors.

The interim financial report for the period January 1 - June 30, 2021 has been prepared in accordance with IAS 34 Interim Financial Reporting. The accounting policies used in the interim financial report are consistent with those accounting policies used in Orphazyme’s 2020 Annual Report with the additions described and additional Danish disclosure requirements for interim reports of listed companies.  

In our opinion, the interim condensed consolidated financial statements give a true and fair view of Orphazyme’s assets, liabilities, and financial position at June 30, 2021 and of the results of its operations and cash flows for the period January 1 - June 30, 2021. Furthermore, in our opinion, Management’s Review gives a true and fair account of the development and performance of the Group’s activities.

Copenhagen, August 31, 2021.

Board of Directors

Georges Gemayel
Chairman of the Board

Bo Jesper Hansen
Deputy Chairman of the Board

Carrolee Barlow

Catherine Moukheibir

Martin Bonde

Stephanie Smith Okey

Executive Management

Christophe BourdonAnders Vadsholt
Chief Executive OfficerChief Financial Officer

Exhibit 99.1

Disclaimer

This company announcement may contain certain forward-looking statements within the meaning of the Private Securities Litigation Reform Act of 1995, including in respect of the timing of the company’s clinical trials and the results thereof, anticipated regulatory developments and approvals for the company’s product candidates, the company’s anticipated operating performance, financial position and ability to operate as a going concern. Although the Company believes its expectations are based on reasonable assumptions, all statements other than statements of historical fact included in this company announcement about future events are subject to (i) change without notice and (ii) factors beyond the Company’s control, including adverse developments in the company’s clinical program, actions by regulatory agencies, effects of the global COVID-19 pandemic, technical and scientific developments in the indications that the company’s product candidates are designed to treat, regulatory developments and the impact of the Company’s restructuring. These statements may include, without limitation, any statements preceded by, followed by, or including words such as “target,” “believe,” “expect,” “aim,” “intend,” “may,” “anticipate,” “estimate,” “plan,” “project,” “will,” “can have,” “likely,” “should,” “would,” “could”, and other words and terms of similar meaning or the negative thereof. Forward-looking statements are subject to inherent risks and uncertainties beyond the Company’s control that could cause the Company’s actual results, performance, or achievements to be materially different from the expected results, performance, or achievements expressed or implied by such forward-looking statements. In light of these risks and uncertainties, and other risks and uncertainties that are described in the Risk Factors section and other sections of the Company’s Annual Report on Form 20-F, filed with the Securities and Exchange Commission (SEC) on March 2, 2021, and other filings that the Company makes with the SEC from time to time (which are available at http://www.sec.gov), the events and circumstances discussed in such forward-looking statements may not occur, and the Company’s actual results could differ materially and adversely from those anticipated or implied thereby. Although the Company’s forward-looking statements reflect the good faith judgment of its management, these statements are based only on facts and factors currently known by the Company. As a result, you are cautioned not to rely on these forward-looking statements. Except as required by law, the Company assumes no obligation to update these forward-looking statements publicly, or to update the reasons actual results could differ materially from those anticipated in the forward-looking statements, even if new information becomes available in the future.