Lexicon Pharmaceuticals to Host First Quarter 2021 Financial Results Conference Call and Webcast on May 6, 2021

On April 29, 2021Lexicon Pharmaceuticals, Inc. (Nasdaq: LXRX), will release its first quarter 2021 financial results on Thursday, May 6, 2021 after the markets close (Press release, Lexicon Pharmaceuticals, APR 29, 2021, View Source [SID1234578785]). Management will conduct a conference call and live webcast at 5:00 p.m. ET (4:00 p.m. CT) that day to discuss the financial results and to provide a business update.

Schedule your 30 min Free 1stOncology Demo!
Discover why more than 1,500 members use 1stOncology™ to excel in:

Early/Late Stage Pipeline Development - Target Scouting - Clinical Biomarkers - Indication Selection & Expansion - BD&L Contacts - Conference Reports - Combinatorial Drug Settings - Companion Diagnostics - Drug Repositioning - First-in-class Analysis - Competitive Analysis - Deals & Licensing

                  Schedule Your 30 min Free Demo!

The dial-in replay will be available for 14 days following the call. An audio webcast will be available online at www.lexpharma.com/events, with a webcast replay accessible for 14 days after the call.

Jazz Pharmaceuticals Announces Closing of Senior Secured Notes Offering

On April 29, 2021 Jazz Pharmaceuticals plc (Nasdaq: JAZZ) (the "Company" or "Jazz") reported the closing of its previously announced offering (the "Offering") of $1.5 billion in aggregate principal amount of 4.375% senior secured notes due 2029 (the "Notes") by Jazz Securities Designated Activity Company, a direct wholly owned subsidiary of the Company (the "Issuer") (Press release, Jazz Pharmaceuticals, APR 29, 2021, View Source [SID1234578782]). The Notes will mature on January 15, 2029 and will bear an interest rate of 4.375%. The Notes are guaranteed by the Company and certain of its subsidiaries.

Schedule your 30 min Free 1stOncology Demo!
Discover why more than 1,500 members use 1stOncology™ to excel in:

Early/Late Stage Pipeline Development - Target Scouting - Clinical Biomarkers - Indication Selection & Expansion - BD&L Contacts - Conference Reports - Combinatorial Drug Settings - Companion Diagnostics - Drug Repositioning - First-in-class Analysis - Competitive Analysis - Deals & Licensing

                  Schedule Your 30 min Free Demo!

The Company expects to use the net proceeds from the Notes and borrowings under new senior secured credit facilities (the "New Senior Secured Credit Facilities"), together with cash on hand, to fund the cash consideration payable in connection with the previously announced acquisition of GW Pharmaceuticals plc ("GW") (the "Acquisition"), the refinancing of certain of the Company’s indebtedness (including the Company’s existing senior secured credit facility) and fees and expenses in connection with the foregoing. If (1) the Acquisition is consummated without the Company entering into the New Senior Secured Credit Facilities, (2) the Acquisition has not been consummated on or before August 3, 2021 (or such later date to which such date may be extended pursuant to the terms of the Transaction Agreement, dated February 3, 2021, among the Company, GW and Jazz Pharmaceuticals UK Holdings Limited (the "Transaction Agreement")) or (3) the Transaction Agreement is terminated in accordance with its terms, the Issuer will be required to redeem all of the Notes at a redemption price equal to 100% of their principal amount plus accrued and unpaid interest to, but excluding, the redemption date.

The Notes were offered and sold only to persons reasonably believed to be qualified institutional buyers pursuant to Rule 144A under the U.S. Securities Act of 1933, as amended (the "Securities Act"), that are located in the United States, Canada, France, Ireland, Luxembourg, the United Kingdom, and Bermuda and to certain non-U.S. persons outside the United States pursuant to Regulation S under the Securities Act that are located in Canada, France, Ireland, Luxembourg, the United Kingdom, and Bermuda. The Notes have not been registered under the Securities Act or any state securities law and may not be offered or sold in the United States absent registration or an applicable exemption from the registration requirements of the Securities Act and applicable state laws.

This press release does not constitute an offer to sell or a solicitation of an offer to purchase the Notes or any other securities and does not constitute an offer, solicitation or sale in any state or jurisdiction in which such an offer, solicitation or sale would be unlawful.

ChemoCentryx Reports First Quarter 2021 Financial Results and Recent Highlights

On April 29, 2021 ChemoCentryx, Inc., (Nasdaq: CCXI), reported financial results for the first quarter ended March 31, 2021 and provided an overview of recent corporate highlights (Press release, ChemoCentryx, APR 29, 2021, View Source [SID1234578781]).

Schedule your 30 min Free 1stOncology Demo!
Discover why more than 1,500 members use 1stOncology™ to excel in:

Early/Late Stage Pipeline Development - Target Scouting - Clinical Biomarkers - Indication Selection & Expansion - BD&L Contacts - Conference Reports - Combinatorial Drug Settings - Companion Diagnostics - Drug Repositioning - First-in-class Analysis - Competitive Analysis - Deals & Licensing

                  Schedule Your 30 min Free Demo!

"Momentum builds with each passing quarter, bringing us closer to our goal of bringing novel, precisely targeted medicine to those that need it most," said Thomas J. Schall, Ph.D., President and Chief Executive Officer of ChemoCentryx. "At our R&D Day earlier this month, two world-renowned clinicians outlined the unmet needs in ANCA-associated vasculitis and the data driving their conviction that avacopan could become a landscape-changing therapy. We are well prepared and look forward to providing our views at the FDA Advisory Committee meeting on this topic in just a few days. During our R&D Day we also took the opportunity to establish how our novel small molecule PD-1/PD-L1 inhibitor CCX559 could transcend current limitations in the treatment of cancer. Meanwhile we are progressing toward our next cycle of clinical trials: a Phase III trial of avacopan in patients with severe HS; the initiation of clinical development of avacopan in lupus nephritis, and our first in human studies of the novel orally administered checkpoint inhibitor CCX559 in cancer patients. We look forward to an historic year of 2021 at ChemoCentryx, and we will devote all our energies to making the dream of breakthrough new therapies a reality for patients."

Key First Quarter 2021 Highlights and Recent Developments

In April, the Company held an R&D Day which focused on avacopan in ANCA-associated vasculitis, with a patient describing his journey with the disease, and the Company’s novel checkpoint inhibitor CCX559.

Two leading clinical experts on ANCA-associated vasculitis, Dr. Peter Merkel from the University of Pennsylvania and Dr. David Jayne from the University of Cambridge, explained in detail the widespread effects of this devastating disease and the shortcomings of current therapy with glucocorticoids and other immunosuppressive drugs. Drs. Merkel and Jayne expressed their belief that avacopan could transform the treatment landscape for ANCA-associated vasculitis.

The Company reported new data from the ADVOCATE trial, covering an 8 week period immediately following the 52-week endpoint. During this period from week 52 to week 60, there were 6 relapses out of 158 in the avacopan group and 7 relapses out of 157 in the prednisone group, suggesting a waning of efficacy after drug treatment is stopped. In addition, the estimated Glomerular Filtration Rate (eGFR), which had increased in the avacopan group through week 52, declined in the 8 weeks after patients stopped taking avacopan.

Tausif "Tosh" Butt, who joined the Company in February 2021 as Executive Vice President and Chief Operating Officer, summarized the Company’s preparations and readiness for the

anticipated commercial launch of avacopan shortly after its PDUFA goal date. He reported on primary market research that a survey of 125 rheumatologists and nephrologists indicated that 97% would prescribe avacopan upon FDA approval. The Company plans to focus initially on the estimated eight thousand patients who are newly diagnosed or relapsing with organ or life-threatening disease, out of an estimated eligible patient population for avacopan of twenty thousand patients.

CCX559 was featured in an abstract at the 2021 Annual Meeting of the American Association for Cancer Research (AACR) (Free AACR Whitepaper) in April. The abstract highlighted laboratory and in vivo data demonstrating potent PD-1/PD-L1 checkpoint inhibition. As a next generation therapy, small molecule inhibitors of PD-L1 may have advantageous properties compared to approved monoclonal antibodies, such as better penetration into solid tumors and reduced immunogenicity. The Company plans to initiate Phase I clinical development of CCX559 in Q2 2021.

In February, The New England Journal of Medicine (NEJM) published the results of the Company’s Phase III ADVOCATE trial of avacopan in ANCA-associated vasculitis, in which avacopan achieved statistical superiority in sustained remission at 52 weeks over prednisone containing standard of care. The same issue of NEJM featured an editorial entitled "Avacopan – Time to Replace Glucocorticoids?".

In February the Japanese New Drug Application for avacopan in the treatment of ANCA-associated vasculitis was accepted for review by the Pharmaceuticals and Medical Devices Agency (PMDA), triggering a milestone payment of $10 million to ChemoCentryx from its partner Vifor Pharma.

The Company plans to advance avacopan into Phase III development in patients with Hurley Stage III (severe) HS in Q4 2021. In the Phase II AURORA trial, avacopan demonstrated a statistically significant higher response than placebo in Hurley Stage III patients.

The Company plans to initiate clinical development of avacopan in patients with lupus nephritis in Q4 2021.

The Company plans to schedule a meeting with the FDA to discuss evidence of clinical benefit from the ACCOLADE trial of avacopan in the very rare disorder C3 Glomerulopathy (C3G), for which there are no approved therapies. In the trial, avacopan demonstrated statistically significant improvement in renal function as measured by the pre-specified endpoint of eGFR and in the pre-specified secondary endpoint of C3G Histology Index (HI) Disease Chronicity score, compared to placebo over 26 weeks of blinded treatment, but did not achieve a statistically significant improvment in the primary endpoint of the C3G HI Disease Activity Score. Avacopan was safe and well tolerated in C3G patients.

The Company maintained a strong balance sheet, with reported cash, cash equivalents and investments of $424.2 million at March 31, 2021.
First Quarter 2021 Financial Results
Revenue was $10.4 million for the first quarter of 2021, compared to $6.0 million for the same period in 2020. The increase in revenue from 2020 to 2021 was principally attributable to the $10.0 million milestone from Vifor for the February 2021 acceptance of the Japanese NDA for avacopan in the treatment of ANCA vasculitis.
Research and development expenses were $23.4 million for the first quarter of 2021, compared to $19.3 million for the same period in 2020. The increase from 2020 to 2021 was primarily attributable to the manufacture of commercial drug supply in anticipation of the launch of avacopan for the treatment of ANCA vasculitis and costs associated with the advancement of CCX559, the Company’s orally-available small molecule checkpoint (PD-1/PD-L1) inhibitor. These increases were partially offset by lower Phase II related expenses due to the completion of patient enrollment of the avacopan AURORA Phase IIb clinical trial in patients with HS and the discontinuation of further clinical development of CCX140 in FSGS in 2020.
General and administrative expenses were $16.3 million for the first quarter of 2021, compared to $8.8 million for the same period in 2020. The increase from 2020 to 2021 was primarily due to higher employee-related expenses, including those associated with the Company’s commercialization planning efforts, and higher professional fees.

Net loss for the first quarter of 2021 was $29.7 million, compared to net loss of $21.7 million for the same period in 2020.
Total shares outstanding at March 31, 2021 were approximately 69.7 million shares.
Cash, cash equivalents and investments totaled $424.2 million at March 31, 2021. The Company expects to utilize cash, cash equivalents and investments in the range of $145 million to $155 million in 2021.
Conference Call and Webcast
The Company will host a conference call and webcast today, April 29, 2021 at 5:00 p.m. Eastern Time / 2:00 p.m. Pacific Time. To participate by telephone, please dial (877) 303-8028 (Domestic) or (760) 536-5167 (International). The conference ID number is 3963565. A live and archived audio webcast can be accessed through the Investors section of the Company’s website at www.ChemoCentryx.com. The archived webcast will remain available on the Company’s website for fourteen (14) days following the call.

Vertex Reports First-Quarter 2021 Financial Results

On April 29, 2021 Vertex Pharmaceuticals Incorporated (Nasdaq: VRTX) reported consolidated financial results for the first quarter ended March 31, 2021 and reiterated full-year 2021 guidance for product revenues (Press release, Vertex Pharmaceuticals, APR 29, 2021, View Source [SID1234578780]).

Schedule your 30 min Free 1stOncology Demo!
Discover why more than 1,500 members use 1stOncology™ to excel in:

Early/Late Stage Pipeline Development - Target Scouting - Clinical Biomarkers - Indication Selection & Expansion - BD&L Contacts - Conference Reports - Combinatorial Drug Settings - Companion Diagnostics - Drug Repositioning - First-in-class Analysis - Competitive Analysis - Deals & Licensing

                  Schedule Your 30 min Free Demo!

"In CF, our goal is that all eligible patients have access to and can benefit from CFTR modulators. In the first quarter, we continued to make significant progress towards this goal, and in so doing again delivered strong revenue and earnings growth," said Reshma Kewalramani, M.D., Chief Executive Officer and President of Vertex. "Beyond CF, we have also seen continued significant progress across our broad pipeline, including advancement of VX-548 to Phase 2 in acute pain, initiation of the Phase 1/2 clinical trial with VX-880 in type 1 diabetes and completion of enrollment and dosing in our Phase 2 proof-of-concept study with the AAT corrector, VX-864. The recent amendment of our agreement with CRISPR Therapeutics for the CTX001 program further enhances our leadership position in cell and genetic therapies and we look forward to completing enrollment of our ongoing trials for CTX001 in sickle cell disease and beta thalassemia this year and bringing this first-in-class treatment to patients with these devastating diseases as soon as possible."

1

First-Quarter 2021 Financial Highlights

Three Months Ended March 31,

%

2021

2020

Change

(in millions, except per share amounts)
Product revenues, net
$
1,723

$
1,515

14%
TRIKAFTA/KAFTRIO
$
1,193

$
895

SYMDEKO/SYMKEVI
$
125

$
173

ORKAMBI
$
219

$
234

KALYDECO
$
186

$
213

GAAP Operating income
$
888

$
720

23%
Non-GAAP Operating income
$
1,003

$
877

14%

GAAP Net income
$
653

$
603

8%
Non-GAAP Net income
$
781

$
674

16%

GAAP Net income per share – diluted
$
2.49

$
2.29

9%
Non-GAAP Net income per share – diluted
$
2.98

$
2.56

16%

Product revenues increased 14% compared to the first quarter of 2020, primarily driven by the uptake of KAFTRIO in Europe and continued performance of TRIKAFTA in the U.S. Net product revenues in the first quarter of 2021 increased 6% to $1.25 billion in the U.S. and increased 43% to $470 million outside the U.S., compared to the prior year.
GAAP and non-GAAP net income increased compared to the first quarter of 2020, largely driven by strong growth in total product revenues.
Cash, cash equivalents and marketable securities as of March 31, 2021 were $6.9 billion, an increase of $265 million compared to $6.7 billion as of December 31, 2020 primarily driven by strong revenue and profitability offset by the repurchase of our common stock authorized under our stock repurchase plan.
2

First-Quarter 2021 Expenses

Three Months Ended March 31,

2021

2020

(in millions)
Combined GAAP R&D and SG&A expenses
$
648

$
631

Combined Non-GAAP R&D and SG&A expenses
$
530

$
477

GAAP R&D expenses
$
456

$
449

Non-GAAP R&D expenses
$
379

$
337

GAAP SG&A expenses
$
192

$
182

Non-GAAP SG&A expenses
$
151

$
140

GAAP income taxes (1)
$
168

$
55

Non-GAAP income taxes
$
207

$
184

GAAP effective tax rate

20%

8%

Non-GAAP effective tax rate (1)

21%

21%

Combined GAAP and Non-GAAP R&D and SG&A expenses increased compared to the first quarter of 2020, primarily due to the expansion of Vertex’s pipeline in CF and other disease areas and incremental investment to support the global launches of Vertex’s medicines.
GAAP income taxes and the GAAP effective tax rate increased compared to the first quarter of 2020 due a non-recurring discrete tax benefit recognized in the first quarter of 2020 and Vertex’s increased operating income.
Non-GAAP income taxes increased compared to the first quarter of 2020 primarily due to Vertex’s increased operating income.

Full-Year 2021 Financial Guidance
Vertex today reiterated its full-year 2021 financial guidance, except for its expectations for combined GAAP R&D and SG&A expenses, which increased by $900 million as a result of Vertex’s amended collaboration with CRISPR announced in April. Vertex’s guidance is summarized below:

Current FY 2021

Previous FY 2021

Product revenues
Unchanged

$6.7 to 6.9 billion

Combined GAAP R&D and SG&A expenses (2)
$3.8 to 3.95 billion

$2.9 to 3.05 billion
Combined Non-GAAP R&D and SG&A expenses (2)
Unchanged

$2.25 to 2.3 billion
Non-GAAP effective tax rate
Unchanged

21% to 22%

3

Key Business Highlights

Cystic Fibrosis (CF)
Vertex anticipates that achieving new approvals and entering into additional reimbursement agreements for our current CFTR modulators will increase the number of CF patients treated with our medicines and continue to grow our CF business in the years ahead.

Key progress in 2021 includes:
•New approval received for TRIKAFTA (elexacaftor/tezacaftor/ivacaftor and ivacaftor) in Australia for people with CF ages 12 years and older who have at least one F508del mutation.
•Post-marketing application filed with the European Medicines Agency (EMA) for the expanded indication of KAFTRIO (elexacaftor/tezacaftor/ivacaftor and ivacaftor) to include children with CF ages 6 through 11 years.
•TRIKAFTA/KAFTRIO is now approved and reimbursed or accessible in 12 countries outside the U.S., including Denmark, Germany, Ireland, Israel, Switzerland and the countries within the UK.

R&D pipeline
Vertex continues to progress a broad pipeline of potentially transformative small molecule, cell and genetic therapies aimed at serious diseases. Recent and anticipated progress for key pipeline programs is noted below:

Beta Thalassemia and Sickle Cell Disease
•In April, Vertex and CRISPR Therapeutics amended their collaboration for the CTX001 programs in beta thalassemia and sickle cell disease. Under the terms of the revised agreement, Vertex will lead worldwide development, manufacturing and commercialization of CTX001. The revised agreement provides Vertex with 60% and CRISPR Therapeutics with 40% of program economics. At closing, CRISPR Therapeutics will receive a $900 million upfront payment with the potential for an additional $200 million milestone payment upon CTX001 regulatory approval.
•The CTX001 program employs a non-viral ex vivo CRISPR gene-editing therapy for the treatment of transfusion-dependent beta thalassemia (TDT) and sickle cell disease (SCD). This approach aims to edit a person’s hematopoietic stem cells to produce fetal hemoglobin in red blood cells, which has the potential to reduce or eliminate symptoms associated with the diseases.
•Enrollment and dosing are ongoing in the clinical studies for CTX001 and more than 30 patients have now been dosed to date. Completion of enrollment in both studies is expected in 2021.
4

•In April, the European Medicines Agency (EMA) granted Priority Medicines Designation (PRIME) to CTX001 for TDT. The program has previously been granted Regenerative Medicine Advanced Therapy (RMAT), Fast Track, Orphan Drug and Rare Pediatric Disease designations from the U.S. Food and Drug Administration (FDA) for both TDT and SCD. CTX001 has also been granted PRIME designation for SCD and Orphan Drug Designation from EMA for both TDT and SCD.

Alpha-1 Antitrypsin (AAT) Deficiency
•Vertex is evaluating multiple compounds with the potential to correct the misfolding of Z-AAT protein in the liver, in order to increase the systemic levels of functional AAT. Misfolded Z-AAT protein is the root cause of AAT deficiency and the Vertex small molecule corrector program targets both the liver and lung manifestations of the disease.
•Patients enrolled in the Phase 2 proof-of-concept study for the Z-AAT corrector, VX-864, have completed the 28-day dosing period. The study includes a 28-day safety follow-up period which is ongoing, and results are expected in the second quarter of 2021.

APOL1-mediated Kidney Diseases
•Vertex is evaluating the potential of inhibitors of APOL1 function in people with APOL1-mediated kidney diseases, including focal segmental glomerulosclerosis (FSGS).
•Enrollment is ongoing in a Phase 2 proof-of-concept study designed to evaluate the reduction in proteinuria in people with APOL1-mediated FSGS after treatment with VX-147. Results from this study are expected in the second half of 2021.

Type 1 Diabetes (T1D)
•Vertex is evaluating a cell therapy designed to replace insulin-producing islet cells in people with T1D. Vertex is pursuing two programs for the transplant of these fully-differentiated functional islets into patients: 1) transplantation of islet cells alone, using immunosuppression to protect the implanted cells and 2) implantation of the islet cells inside a novel immunoprotective device.
•In March, the U.S. FDA granted Fast Track Designation and Vertex initiated a Phase 1/2 clinical trial for VX-880, the islet cells alone program, in people with T1D.

Pain
•Vertex is evaluating selective small molecule inhibitors of NaV1.8, a genetically validated, novel target for the treatment of pain, with the goal of preventing pain signals traveling from the sensory
5

nerves to the central nervous system. Vertex has previously demonstrated clinical proof-of-concept with a small molecule investigational treatment targeting NaV1.8, VX-150, in multiple pain indications including acute pain, neuropathic pain and musculoskeletal pain.
•VX-548, a selective NaV1.8 inhibitor, demonstrated favorable safety, tolerability and pharmacokinetic profiles in Phase 1 studies. In these studies, the molecule exhibited a favorable profile at doses considerably lower than those required with our previous NaV1.8 inhibitors.
•VX-548 is expected to advance into Phase 2 proof-of-concept studies for acute pain in the second half of 2021.

Investments in External Innovation
•In April, we entered into a research collaboration with Obsidian Therapeutics, Inc., or Obsidian, aimed at the discovery of novel therapies that regulate gene-editing for the treatment of serious diseases. This collaboration enables us to leverage Obsidian’s cytoDRiVE platform technology to discover gene-editing medicines whose therapeutic activity can be precisely controlled using small molecules.
6

Non-GAAP Financial Measures
In this press release, Vertex’s financial results and financial guidance are provided in accordance with accounting principles generally accepted in the United States (GAAP) and using certain non-GAAP financial measures. In particular, non-GAAP financial results and guidance exclude from Vertex’s pre-tax income (i) stock-based compensation expense, (ii) revenues and expenses related to collaborative milestones and upfront payments, (iii) gains or losses related to the fair value of the company’s strategic investments, (iv) increases or decreases in the fair value of contingent consideration, (v) acquisition-related costs and (vi) other adjustments. The company’s non-GAAP financial results also exclude from its provision for income taxes the estimated tax impact related to its non-GAAP adjustments to pre-tax income described above and certain discrete items. These results should not be viewed as a substitute for the company’s GAAP results and are provided as a complement to results provided in accordance with GAAP. Management believes these non-GAAP financial measures help indicate underlying trends in the company’s business, are important in comparing current results with prior period results and provide additional information regarding the company’s financial position that the company believes is helpful to an understanding of its ongoing business. Management also uses these non-GAAP financial measures to establish budgets and operational goals that are communicated internally and externally, to manage the company’s business and to evaluate its performance. The company adjusts, where appropriate, for both revenues and expenses in order to reflect the company’s operations. The company’s calculation of non-GAAP financial measures likely differs from the calculations used by other companies. A reconciliation of the GAAP financial results to non-GAAP financial results is included in the attached financial information.
The company provides guidance regarding combined R&D and SG&A expenses and effective tax rate on a non-GAAP basis. The guidance regarding combined GAAP R&D and SG&A expenses does not include estimates associated with any potential future business development activities. The company does not provide guidance regarding its GAAP effective tax rate because it is unable to forecast with reasonable certainty the impact of excess tax benefits related to stock-based compensation and the possibility of certain discrete items, which could be material.
7

Vertex Pharmaceuticals Incorporated
First-Quarter Results
Consolidated Statements of Operations
(in thousands, except per share amounts)
(unaudited)

Three Months Ended March 31,

2021

2020
Revenues:

Product revenues, net
$
1,723,305

$
1,515,107

Other revenues
1,000

Total revenues
1,724,305

1,515,107

Costs and expenses:

Cost of sales
192,329

162,497

Research and development expenses
455,973

448,528

Sales, general and administrative expenses
192,077

182,258

Change in fair value of contingent consideration
(3,900)

1,600

Total costs and expenses
836,479

794,883

Income from operations
887,826

720,224

Interest income
1,465

12,576

Interest expense
(15,678)

(14,136)

Other expense, net (3)
(52,653)

(61,130)

Income before provision for income taxes
820,960

657,534

Provision for income taxes
167,822

54,781

Net income
$
653,138

$
602,753

Net income per common share:

Basic
$
2.52

$
2.32

Diluted
$
2.49

$
2.29

Shares used in per share calculations:

Basic
259,369

259,815

Diluted
261,916

263,515

8

Reconciliation of GAAP to Non-GAAP Net Income
First-Quarter Results
(in thousands, except per share amounts)
(unaudited)

Three Months Ended March 31,

2021

2020
GAAP net income
$
653,138

$
602,753

Stock-based compensation expense
115,174

115,706

Decrease in fair value of strategic investments (3)
52,295

44,870

(Decrease) increase in fair value of contingent consideration (4)
(3,900)

1,600

Collaborative revenues and expenses (5)
650

36,250

Acquisition-related costs (6)
2,820

2,883

Total non-GAAP adjustments to pre-tax income
167,039

201,309

Tax adjustments (1)
(38,961)

(129,608)

Non-GAAP net income
$
781,216

$
674,454

Net income per diluted common share:

GAAP
$
2.49

$
2.29

Non-GAAP
$
2.98

$
2.56

Shares used in diluted per share calculations:

GAAP and Non-GAAP
261,916

263,515

Three Months Ended March 31,

2021

2020
GAAP operating income
$
887,826

$
720,224

Stock-based compensation expense
115,174

115,706

(Decrease) increase in fair value of contingent consideration (4)
(3,900)

1,600

Collaborative revenues and expenses (5)
650

36,250

Acquisition-related costs (6)
2,820

2,883

Non-GAAP operating income
$
1,002,570

$
876,663

9

Reconciliation of GAAP to Non-GAAP Revenues and Expenses
First-Quarter Results
(in thousands)
(unaudited)

Three Months Ended March 31,

2021

2020
GAAP total revenues
$
1,724,305

$
1,515,107

Collaborative revenues
(1,000)

Non-GAAP total revenues
$
1,723,305

$
1,515,107

Three Months Ended March 31,

2021

2020
GAAP cost of sales
$
192,329

$
162,497

Stock-based compensation expense
(1,431)

(1,361)

Non-GAAP cost of sales
$
190,898

$
161,136

GAAP research and development expenses
$
455,973

$
448,528

Stock-based compensation expense
(72,802)

(72,687)

Collaborative expenses (5)
(1,650)

(36,250)

Acquisition-related costs (6)
(2,820)

(2,678)

Non-GAAP research and development expenses
$
378,701

$
336,913

GAAP sales, general and administrative expenses
$
192,077

$
182,258

Stock-based compensation expense
(40,941)

(41,658)

Acquisition-related costs (6)

(205)

Non-GAAP sales, general and administrative expenses
$
151,136

$
140,395

Combined non-GAAP R&D and SG&A expenses
$
529,837

$
477,308

Three Months Ended March 31,

2021

2020
GAAP other expense, net
$
(52,653)

$
(61,130)

Decrease in fair value of strategic investments (3)
52,295

44,870

Non-GAAP other expense, net
$
(358)

$
(16,260)

GAAP provision for income taxes
$
167,822

$
54,781

Tax adjustments (1)
38,961

129,608

Non-GAAP provision for income taxes (7)
$
206,783

$
184,389

GAAP effective tax rate
20
%

8
%
Non-GAAP effective tax rate (7)
21
%

21
%
10

Condensed Consolidated Balance Sheets
(in thousands)
(unaudited)

March 31, 2021

December 31, 2020
Assets

Cash, cash equivalents and marketable securities
$
6,923,968

$
6,658,897

Accounts receivable, net
977,551

885,352

Inventories
298,863

280,777

Property and equipment, net
986,123

958,534

Goodwill and intangible assets
1,402,158

1,402,158

Deferred tax assets
815,890

882,779

Other assets
710,506

683,311

Total assets
$
12,115,059

$
11,751,808

Liabilities and Shareholders’ Equity

Accounts payable and accrued expenses
$
1,659,876

$
1,560,110

Finance lease liabilities
572,856

581,476

Contingent consideration
185,700

189,600

Other liabilities
716,373

733,807

Shareholders’ equity
8,980,254

8,686,815

Total liabilities and shareholders’ equity
$
12,115,059

$
11,751,808

Common shares outstanding
258,829

259,890

11

Notes and Explanations

1: In the three months ended March 31, 2021 and 2020, "Tax adjustments" primarily related to the estimated income taxes related to non-GAAP adjustments to pre-tax income including (i) stock-based compensation (including an adjustment for excess tax benefits related to stock-based compensation), (ii) decreases in the fair value of the company’s strategic investments and (iii) collaborative milestone payments. In the three months ended March 31, 2020, "Tax adjustments" also included a non-recurring discrete benefit to the company’s provision for income taxes of $50.4 million, relating to the write-off of a long-term intercompany receivable, that the company excluded from its Non-GAAP measures.
2: The company’s increased combined GAAP R&D and SG&A expenses guidance reflects the expected effect upon closing of the company’s contemplated transaction with CRISPR, which was announced in April 2021. The difference between the company’s full-year 2021 combined GAAP R&D and SG&A expenses and combined non-GAAP R&D and SG&A expenses guidance relates primarily to $1.12 billion to $1.17 billion of R&D expenses related to existing and contemplated collaboration agreements and $430 million to $455 million of stock-based compensation expense. The guidance regarding combined GAAP R&D and SG&A expenses does not include estimates associated with any potential future business development activities other than the company’s contemplated transaction with CRISPR.
3: "Other expense, net" includes net losses related to changes in the fair value of the company’s strategic investments and from sales of certain investments.
4: During the three months ended March 31, 2021 and 2020, the change in the fair value of contingent consideration relates to potential payments to Exonics Therapeutics’ former equity holders.
5: "Collaborative revenues and expenses" in the three months ended March 31, 2021 and 2020 primarily related to collaborative milestone payments.
6: "Acquisition-related costs" in the three months ended March 31, 2021 and 2020 related to costs associated with the company’s acquisition of Exonics Therapeutics in 2019.
7: The company released its valuation allowance on the majority of its net operating losses and other deferred tax assets as of December 31, 2018. As of December 31, 2020, the company had utilized substantially all of its remaining federal net operating losses. As a result, a larger portion of the company’s tax provision will represent a cash tax payable beginning in 2021, subject to continued utilization of certain tax credits.

BioMarin Announces First Quarter 2021 Financial Results and Corporate Updates

On April 29, 2021 BioMarin Pharmaceutical Inc. (NASDAQ: BMRN) (BioMarin or the Company) reported its financial results for the first quarter ended March 31, 2021 (Press release, BioMarin, APR 29, 2021, View Source [SID1234578779]).

"We mark the start of 2021 with strong financial results, and a number of exciting regulatory decisions ahead this year." said Jean-Jacques Bienaimé, Chairman and Chief Executive Officer of BioMarin. "The potential approval in Europe this summer of vosoritide, the first potential therapeutic option for children with achondroplasia, will set the stage for our next significant phase of growth, especially considering the EMEA region is 3 times larger than the U.S. market. To date, the interactions with the review team at the European Medicines Agency have been very positive. Next, we plan to re-submit the application for valoctocogene roxaparvovec gene therapy, to treat hemophilia A, to the EMA in the second quarter, to be followed by potential approval of vosoritide in the United States later this year."

Schedule your 30 min Free 1stOncology Demo!
Discover why more than 1,500 members use 1stOncology™ to excel in:

Early/Late Stage Pipeline Development - Target Scouting - Clinical Biomarkers - Indication Selection & Expansion - BD&L Contacts - Conference Reports - Combinatorial Drug Settings - Companion Diagnostics - Drug Repositioning - First-in-class Analysis - Competitive Analysis - Deals & Licensing

                  Schedule Your 30 min Free Demo!

Mr. Bienaimé continued, "We are well positioned to deliver strong results for the full-year 2021, and reaffirm our financial guidance provided early this year. This takes into consideration the uneven ordering patterns in our dynamic and global commercial business, as well as continued uncertainty outside of the United States from COVID-19, and further underscores the essential nature of our products to the people who rely on them. Taken together, our financial strength, including positive cash flows from operations of $113.5 million in the first quarter, combined with near-term opportunities from potential new products and a robust early-stage pipeline, we believe that 2021 will be pivotal to BioMarin’s evolution."

Financial Highlights:
•Net Product Revenues for the first quarter of 2021 decreased to $467.8 million, compared to $489.0 million for the same period in 2020. The decrease in Net Product Revenues was primarily attributed to the following:
•Lower Kuvan product revenues primarily due to known generic competition in the U.S.; partially offset by
•Higher Vimizim product revenues primarily due to timing of orders from Europe, Middle East and North Africa; and
•Higher Palynziq product revenues primarily driven by a combination of revenue from more U.S. patients achieving maintenance dosing and new patients initiating therapy.

•GAAP Net Income decreased $64.0 million to $17.4 million for the first quarter of 2021 compared to $81.4 million for the same period in 2020. The decrease was primarily due to the absence of the gain on sale the Firdapse commercial assets totaling $59.5 million in the first quarter of 2020 and a decrease in gross profits due to the lower Kuvan product revenues due to generic competition in the U.S. This decrease was partially offset by lower selling, general and administrative (SG&A) expenses primarily due to a decrease in foreign currency exchange losses in the first quarter of 2021.

•Non-GAAP Net Income for the first quarter of 2021 decreased to $104.4 million compared to Non-GAAP Income of $116.5 million for the same period in 2020. The decline in Non-GAAP Income for the quarter, compared to the same period in 2020, was primarily attributed to lower gross profits, partially offset by lower SG&A expenses primarily due to a decrease in foreign currency exchange losses in the first quarter of 2021.
Commercial Portfolio (Naglazyme, VIMIZIM, Brineura, Palynziq, Kuvan and Aldurazyme)
•Naglazyme, VIMIZIM and Brineura maintained robust patient compliance in the quarter and continue to grow based on strong underlying demand.
◦Patients on commercial Naglazyme and Vimizim therapy both increased approximately 10% year-over-year.
2

◦Patients on commercial Brineura therapy increased by more than 30% year-over-year.
•Order timing for both Naglazyme and VIMIZIM are expected to be more concentrated in the first half of 2021 as compared to the second half of 2021.
•Palynziq top-line results in 2021 are expected to increase approximately 35% based on the mid-point of full-year 2021 guidance as compared to full-year 2020 results.
•Palynziq growth in European, Middle East and African regions (EMEA) has been impacted by ongoing challenges due to COVID-19 with Palynziq revenue from EMEA expected to increase when PKU clinics have more freedom to operate and start additional patients.
◦The number of U.S. Patients on commercial Palynziq therapy increased more than 20% year-over-year and is expected to continue to grow as U.S. PKU clinics re-open over the coming quarters.
•Loss of Kuvan U.S. market share, due to the introduction of generics following BioMarin’s loss of U.S. market exclusivity, is consistent with expectations.
•Aldurazyme contributions were driven by the timing of product released and transfer of control to Genzyme, who is responsible for marketing Aldurazyme worldwide.
Late-stage Regulatory Portfolio (Vosoritide and valoctocogene roxaparvovec)
•In Europe, with respect to vosoritide for the treatment of achondroplasia, BioMarin is in the final stages of the review procedure ahead of the anticipated June 2021 Committee for Medicinal Products for Human Use (CHMP) opinion. Assuming a positive CHMP opinion, the European Commission could potentially grant marketing authorization for vosoritide in the third quarter of 2021.
•In Europe, with respect to valoctocogene roxaparvovec for the treatment of severe hemophilia A, based on positive pre-submission feedback in the current quarter, BioMarin reaffirms its plan to submit the Marketing Authorization Application with one-year results from the Phase 3 GENEr8-1 study to the European Medicines Agency (EMA) in June 2021.
•In the U.S., BioMarin provided the U.S. Food and Drug Administration (FDA) with the two-year results from the Phase 3 extension study with vosoritide to supplement the New Drug Application (NDA) already under review. As anticipated, the FDA designated this submission as a major amendment to the application, thus extending the Prescription Drug User Fee Act (PDUFA) target action date by three months to November 20, 2021 to provide time for a full review of the submission.
◦Also in the first quarter of 2021, the FDA pre-approval inspection of BioMarin’s Novato facility for the manufacture of vosoritide drug substance was completed, representing another important milestone as vosoritide advances through the review process.
•During the current quarter, the FDA reiterated their recommendation that BioMarin submit two-year follow-up safety and efficacy data on all study participants from the GENEr8-1 study to support their benefit/risk assessment of valoctocogene roxaparvovec. BioMarin is targeting a Biologics License Application (BLA) submission in the second quarter of 2022 assuming favorable results, followed by an expected 6-month review procedure by the FDA.
◦The FDA granted Regenerative Medicine Advanced Therapy (RMAT) designation to valoctocogene roxaparvovec. The RMAT designation is complementary to Breakthrough Therapy Designation, which BioMarin received in 2017.
3

Earlier-stage Development Portfolio (BMN 307, BMN 255, BMN 331, DiNA-001, Allen Institute Collaboration)
◦BMN 307: Dose escalation in PHEarless, the Phase 1/2 study of BMN 307 continues based on encouraging Phe lowering and safety profile observed in study participants who were treated with the lowest dose.
◦BMN 255 for a subset of chronic renal disease: On January 11, 2021 BioMarin announced that it had filed an IND in 2020 for BMN 255, a small molecule for a subset of chronic renal disease. BMN 255 was driven by genetic discoveries for both mechanism and for identifying individuals for treatment.

◦BMN 331 gene therapy product candidate for Hereditary Angioedema (HAE): IND-enabling studies are ongoing with BMN 331 for the treatment of HAE, BioMarin’s third gene therapy candidate. BioMarin plans to leverage its broad expertise in developing gene therapies for severe hemophilia A and PKU to improve efficiencies in the development process of BMN 331. BioMarin is on track to file an IND for BMN 331 mid-year 2021.

◦BMN 351 for Duchenne Muscular Dystrophy (DMD): IND-enabling studies are underway with BMN 351, an antisense oligonucleotide therapy that has demonstrated dystrophin expression levels of 30-50% of wild-type levels in the quadriceps in a DMD mouse model treated at 18.7 mg/kg/week for 13 weeks (measured 2 weeks following last administration). If results from the ongoing pre-clinical studies are supportive, BioMarin anticipates filing an IND for BMN 351 in the first half of 2022.

◦DiNA-001 for MYBPC3 hypertrophic cardiomyopathy (HCM): Pre-clinical studies are underway with DiNA-001 following a collaboration announced in 2020 with DiNAQOR, a gene therapy platform company, to develop novel gene therapies to treat rare genetic cardiomyopathies. DiNAQOR received an undisclosed upfront payment and is eligible to receive development, regulatory and commercial milestones on product sales in addition to tiered royalties on worldwide sales.

◦On April 28, 2021, BioMarin and the Allen Institute announced a collaboration to create new gene therapies aimed at rare genetic diseases of the central nervous system (CNS). The goal is to combine the Allen Institute’s leadership in large-scale genomic science in the CNS therapeutic area with BioMarin’s expertise in developing transformational gene therapies.
2021 Full-Year Financial Guidance (in millions, except %)

Item

2021 Guidance * (reaffirmed)
Total Revenues

$1,750

to

$1,850
Vimizim Net Product Revenues

$570

to

$610
Kuvan Net Product Revenues

$250

to

$290
Naglazyme Net Product Revenues

$365

to

$395
Palynziq Net Product Revenues

$210

to

$250
Brineura Net Product Revenues

$120

to

$140

Cost of Sales (% of Total Revenues)

23
%

to

25%
Research and Development Expense

$645

to

$695
Selling, General and Administrative Expense

$725

to

$775

GAAP Net Loss

($130)

to

($80)
Non-GAAP Income (1)

$170

to

$220